MCID: FCT005
MIFTS: 52

Factor Xiii Deficiency

Categories: Blood diseases, Rare diseases

Aliases & Classifications for Factor Xiii Deficiency

MalaCards integrated aliases for Factor Xiii Deficiency:

Name: Factor Xiii Deficiency 12 74 52 25 36 54 43 15 71
Hereditary Factor Xiii Deficiency Disease 12 6
Fibrin Stabilizing Factor Deficiency 52 25
Congenital Factor Xiii Deficiency 52 58
Deficiency, Laki-Lorand Factor 12 25
Fibrin-Stabilizing Factor Deficiency 58
Factor Xiii Deficiency Disease 12
Deficiency of Factor Xiii 25
Deficiency, Factor Xiii 39

Characteristics:

Orphanet epidemiological data:

58
congenital factor xiii deficiency
Inheritance: Autosomal recessive,Not applicable; Prevalence: <1/1000000 (Europe); Age of onset: All ages; Age of death: normal life expectancy;

Classifications:

Orphanet: 58  
Rare haematological diseases


External Ids:

Disease Ontology 12 DOID:2211
KEGG 36 H00945
MeSH 43 D005177
NCIt 49 C98941
SNOMED-CT 67 50189006
ICD10 via Orphanet 33 D68.2
UMLS via Orphanet 72 C0015530
Orphanet 58 ORPHA331
UMLS 71 C0015530

Summaries for Factor Xiii Deficiency

Genetics Home Reference : 25 Factor XIII deficiency is a rare bleeding disorder. Researchers have identified an inherited form and a less severe form that is acquired during a person's lifetime. Signs and symptoms of inherited factor XIII deficiency begin soon after birth, usually with abnormal bleeding from the umbilical cord stump. If the condition is not treated, affected individuals may have episodes of excessive and prolonged bleeding that can be life-threatening. Abnormal bleeding can occur after surgery or minor trauma. The condition can also cause spontaneous bleeding into the joints or muscles, leading to pain and disability. Women with inherited factor XIII deficiency tend to have heavy or prolonged menstrual bleeding (menorrhagia) and may experience recurrent pregnancy losses (miscarriages). Other signs and symptoms of inherited factor XIII deficiency include nosebleeds, bleeding of the gums, easy bruising, problems with wound healing, bleeding after surgery, and abnormal scar formation. Inherited factor XIII deficiency also increases the risk of spontaneous bleeding inside the skull (intracranial hemorrhage), which is the leading cause of death in people with this condition. Acquired factor XIII deficiency becomes apparent later in life. People with the acquired form are less likely to have severe or life-threatening episodes of abnormal bleeding than those with the inherited form.

MalaCards based summary : Factor Xiii Deficiency, also known as hereditary factor xiii deficiency disease, is related to thrombasthenia and hemangioma, and has symptoms including prolonged umbilical cord bleeding An important gene associated with Factor Xiii Deficiency is F13A1 (Coagulation Factor XIII A Chain), and among its related pathways/superpathways are Complement and coagulation cascades and Response to elevated platelet cytosolic Ca2+. The drugs tannic acid and Benzocaine have been mentioned in the context of this disorder. Affiliated tissues include myeloid, liver and brain, and related phenotypes are abnormal umbilical stump bleeding and reduced factor xiii activity

Disease Ontology : 12 An inherited blood coagulation disease that is characterized by easy bleeding, has symptom prolonged umbilical cord bleeding, epistaxis, bleeding of the gums, menorrhagia, recurrent miscarriages, abnormal scar formation and wound healing, and hemarthrosis, and has material basis in autosomal recessive inheritance of mutation of the F13A1 or F13B gene, which encodes Factor XIII, formally known as fibrin stabilizing factor.

NIH Rare Diseases : 52 Factor XIII deficiency is an extremely rare inherited blood disorder characterized by abnormal blood clotting that may result in abnormal bleeding. Signs and symptoms occur as the result of a deficiency in the blood clotting factor 13, which is responsible for stabilizing the formation of a blood clot. In affected individuals, the blood fails to clot appropriately, resulting in poor wound healing. Blood may seep into surrounding soft tissues , resulting in local pain and swelling. Internal bleeding may occur; about 25 percent of affected individuals experience bleeding in the brain. FXIII deficiency is usually caused by mutations in the F13A1 gene , but mutations have also been found in the F13B gene. It is usually inherited in an autosomal recessive fashion. Acquired forms have also been reported in association with liver failure, inflammatory bowel disease , and myeloid leukemia .

KEGG : 36 Factor XIII deficiency is a rare autosomal recessive disorder characterized by defective cross-linking of fibrin and poor resistance to fibrinolysis. The severity of the bleeding tendency varies from benign symptoms like excessive bruising to life threatening bleeding emergencies such as intracranial hemorrhages. In plasma, FXIII circulates as a pro-transglutaminase composed of two catalytic A subunits and two non-catalytic B subunits. Symptoms that are almost pathognomonic for A subunit deficiency are umbilical bleeding in the neonatal period and repeated fetal wastage in pregnant females.

Wikipedia : 74 Factor XIII deficiency occurs exceedingly rarely, causing a severe bleeding tendency. The incidence is... more...

Related Diseases for Factor Xiii Deficiency

Diseases related to Factor Xiii Deficiency via text searches within MalaCards or GeneCards Suite gene sharing:

(showing 276, show less)
# Related Disease Score Top Affiliating Genes
1 thrombasthenia 30.0 F5 F3 F2
2 hemangioma 29.9 VWF F3 F2
3 eclampsia 29.8 SERPINE1 SERPINC1 F2
4 endocarditis 29.8 SERPINE1 SERPINC1 F2
5 hemophilia 29.7 F9 F8 F7
6 purpura 29.7 VWF SERPINC1 F3 F2
7 placental abruption 29.7 SERPINC1 F5 F2
8 liver cirrhosis 29.5 SERPINF2 SERPINC1 F3 F2
9 active peptic ulcer disease 29.3 VWF F7 F3 F2
10 acquired hemophilia 29.1 F9 F8 F5 F3
11 thrombophilia due to thrombin defect 28.9 SERPINE1 SERPINC1 F8 F5 F3 F2
12 compartment syndrome 28.9 SERPINC1 F8 F7 F3 F2
13 intracranial thrombosis 28.6 VWF SERPINC1 F8 F5 F3 F2
14 acute myocardial infarction 28.5 VWF SERPINF2 SERPINE1 SERPINC1 F7 F3
15 von willebrand's disease 28.2 VWF F9 F8 F7 F5 F3
16 vascular disease 28.0 VWF SERPINE1 SERPINC1 F8 F5 F3
17 thrombocytopenia 27.6 VWF SERPINE1 SERPINC1 F9 F5 F3
18 hemarthrosis 27.4 VWF MCFD2 F9 F8 F7 F3
19 disseminated intravascular coagulation 27.0 VWF SERPINF2 SERPINE1 SERPINC1 F9 F7
20 blood coagulation disease 26.8 VWF SERPINF2 SERPINE1 SERPINC1 F9 F8
21 afibrinogenemia, congenital 26.7 VWF SERPINF2 SERPINE1 SERPINC1 MCFD2 F8
22 hemorrhagic disease 26.6 VWF SERPINF2 SERPINE1 SERPINC1 F9 F8
23 pulmonary embolism 26.5 VWF SERPINF2 SERPINE1 SERPINC1 F9 F8
24 thrombosis 25.7 VWF SERPINF2 SERPINE1 SERPINC1 F9 F8
25 factor xiii, a subunit, deficiency of 11.9
26 factor xiii, b subunit, deficiency of 11.9
27 rare hemorrhagic disorder 10.6
28 prothrombin-related thrombophilia 10.4 F2 F13A1
29 plasminogen activator inhibitor-1 deficiency 10.4 SERPINF2 SERPINE1
30 amaurosis fugax 10.4 SERPINE1 F5
31 fainting 10.3 VWF F8
32 paracetamol poisoning 10.3 F5 F2
33 alkhurma hemorrhagic fever 10.3 F8 F3
34 arteritic anterior ischemic optic neuropathy 10.3 F5 F2
35 achenbach syndrome 10.3 F3 F2
36 pediatric angiosarcoma 10.3 VWF F8
37 renal pelvis squamous cell carcinoma 10.3 F3 F2
38 autosomal recessive disease 10.3
39 emphysematous cholecystitis 10.3 F3 F2
40 femoral neuropathy 10.3 F3 F2
41 hemopneumothorax 10.3 F3 F2
42 lymphangiosarcoma 10.3 VWF F8
43 gastric hemangioma 10.3 F3 F2
44 ankylosing spondylitis 1 10.3 F3 F2
45 hemopericardium 10.2 F3 F2
46 epidural abscess 10.2 F3 F2
47 thyroid angiosarcoma 10.2 VWF F8
48 fibrinolytic defect 10.2 SERPINE1 SERPINC1
49 generalized atherosclerosis 10.2 VWF SERPINE1
50 cold urticaria 10.2 SERPINF2 SERPINC1
51 cerebral falx meningioma 10.2 F3 F2
52 intracranial hypotension 10.2 F3 F2
53 heparin-induced thrombocytopenia 10.2 SERPINC1 F3
54 acute leukemia 10.2
55 catastrophic antiphospholipid syndrome 10.1 SERPINE1 F5 F3
56 von willebrand disease, type 2 10.1 VWF F8
57 acute hemorrhagic encephalitis 10.1 F3 F2
58 vulvar angiokeratoma 10.1 SERPINC1 F3
59 thoracic outlet syndrome 10.1 SERPINC1 F2
60 leech infestation 10.1 SERPINC1 F3
61 cryptogenic cirrhosis 10.1 SERPINE1 F5 F2
62 analbuminemia 10.1 F3 F2
63 coronary stenosis 10.1 VWF SERPINE1 CPB2
64 abducens nerve disease 10.1 SERPINC1 F2
65 exophthalmos 10.1
66 argentine hemorrhagic fever 10.1 SERPINE1 SERPINC1
67 medulloadrenal hyperfunction 10.1 VWF SERPINE1 F3
68 ceroid lipofuscinosis, neuronal, 5 10.1 VWF F7
69 cerebral sinovenous thrombosis 10.1 F5 F3 F2
70 mediastinitis 10.1 F5 F3 F2
71 branch retinal artery occlusion 10.1 SERPINC1 F2
72 angiodysplasia 10.1 VWF F8 F3
73 hantavirus pulmonary syndrome 10.1 F3 F2
74 von willebrand disease, type 3 10.1 VWF F8
75 mastoiditis 10.0 SERPINC1 F2
76 papillary adenofibroma 10.0 VWF F3 F2
77 ebola hemorrhagic fever 10.0 F5 F3
78 sticky platelet syndrome 10.0 SERPINE1 SERPINC1 F5
79 intracranial hypertension, idiopathic 10.0 F3 F2
80 central retinal artery occlusion 10.0 SERPINC1 F2
81 splenic sequestration 10.0 VWF F3 F2
82 limb ischemia 10.0 VWF SERPINE1 F2
83 rheumatoid arthritis 10.0
84 leukemia 10.0
85 glomerulonephritis 10.0
86 paraplegia 10.0
87 meningococcemia 10.0 SERPINE1 SERPINC1 F5
88 primary thrombocytopenia 10.0 VWF F3 F2
89 legg-calve-perthes disease 10.0 SERPINE1 SERPINC1 F5
90 platelet aggregation, spontaneous 10.0 VWF SERPINF2 SERPINC1
91 spinal cord infarction 10.0 SERPINC1 F5 F2
92 sudden sensorineural hearing loss 10.0 SERPINC1 F5 F2
93 severe pre-eclampsia 10.0 SERPINE1 SERPINC1 F5
94 portal hypertension 10.0 VWF F3 F2
95 prothrombin deficiency, congenital 9.9 SERPINC1 F5 F2
96 blue toe syndrome 9.9 SERPINC1 F3 F2
97 factor v and factor viii, combined deficiency of, 2 9.9 MCFD2 F8 F5
98 lateral sinus thrombosis 9.9 SERPINC1 F3 F2
99 waterhouse-friderichsen syndrome 9.9 SERPINC1 F3 F2
100 cavernous sinus thrombosis 9.9 SERPINC1 F3 F2
101 lemierre's syndrome 9.9 SERPINC1 F3 F2
102 anuria 9.9 F3 F2
103 hepatic infarction 9.9 SERPINC1 F3 F2
104 intracranial sinus thrombosis 9.9 SERPINC1 F3 F2
105 retinal vascular occlusion 9.9 SERPINC1 F5 F2
106 intracranial embolism 9.9 SERPINC1 F3 F2
107 splenic infarction 9.9 SERPINC1 F3 F2
108 hepatic coma 9.9 SERPINC1 F3 F2
109 homocystinuria 9.9 SERPINC1 F8 F5
110 osteonecrosis 9.9 SERPINE1 SERPINC1 F2
111 esophageal varix 9.9 SERPINC1 F3 F2
112 papilledema 9.9 SERPINC1 F3 F2
113 nonarteritic anterior ischemic optic neuropathy 9.9 SERPINC1 F5 F2
114 alpha-1-antitrypsin deficiency 9.9 SERPINE1 SERPINC1 F2
115 heart conduction disease 9.9 SERPINC1 F3 F2
116 intermediate coronary syndrome 9.9 VWF SERPINC1 F3
117 hydranencephaly 9.9
118 coronary heart disease 1 9.9 VWF SERPINE1 F7
119 cerebral amyloid angiopathy, cst3-related 9.9
120 autoimmune disease 9.9
121 neutrophil migration 9.9
122 strabismus 9.9
123 myelofibrosis 9.9
124 papilloma of choroid plexus 9.9
125 congenital hemidysplasia with ichthyosiform erythroderma and limb defects 9.9
126 leukemia, acute myeloid 9.9
127 leukemia, acute lymphoblastic 9.9
128 leukemia, acute lymphoblastic 3 9.9
129 spastic paraplegia, intellectual disability, nystagmus, and obesity 9.9
130 aspiration pneumonia 9.9
131 inflammatory bowel disease 9.9
132 follicular lymphoma 9.9
133 infective endocarditis 9.9
134 aphasia 9.9
135 colitis 9.9
136 clostridium difficile colitis 9.9
137 inguinal hernia 9.9
138 chronic myelomonocytic leukemia 9.9
139 polycystic kidney disease 9.9
140 covid-19 9.9
141 b-lymphoblastic leukemia/lymphoma 9.9
142 osteomyelitis 9.9
143 tonsillitis 9.9
144 hydrocephalus 9.9
145 henoch-schoenlein purpura 9.9
146 arteriovenous malformation 9.9
147 alternating exotropia 9.9
148 exotropia 9.9
149 male infertility 9.9
150 pancytopenia 9.9
151 hepatitis a 9.9
152 corpus luteum cyst 9.9
153 ehlers-danlos syndrome 9.9
154 squamous cell papilloma 9.9
155 papilloma 9.9
156 severe acute respiratory syndrome 9.9
157 gingivitis 9.9
158 temporal lobe epilepsy 9.9
159 plasmacytoma 9.9
160 arthropathy 9.9
161 gastritis 9.9
162 infertility 9.9
163 kidney disease 9.9
164 b-cell lymphoma 9.9
165 peritonitis 9.9
166 vasculitis 9.9
167 mechanical strabismus 9.9
168 encephalitis 9.9
169 keloid disorder 9.9
170 47,xyy 9.9
171 autoimmune encephalitis 9.9
172 cardiac rupture 9.9
173 cryofibrinogenemia 9.9
174 myeloid splenomegaly 9.9
175 splenomegaly 9.9
176 head injury 9.9
177 spinal cord injury 9.9
178 avascular necrosis 9.9
179 rare coagulation disorder 9.9
180 cholesterol embolism 9.8 SERPINC1 F7 F2
181 atrial heart septal defect 9.8 SERPINC1 F3 F2
182 von willebrand disease, type 1 9.8 VWF F8 F3 F2
183 venous insufficiency 9.8 VWF SERPINE1 F5 F2
184 posterior cerebral artery infarction 9.8 F9 F3
185 synovial angioma 9.8 F8 F7 F3 F2
186 polycythemia vera 9.8 VWF F5 F2
187 dysfibrinogenemia, congenital 9.8 SERPINC1 F5 F2 F13A1
188 livedoid vasculitis 9.8 SERPINF2 SERPINE1 SERPINC1 F2
189 hepatic veno-occlusive disease 9.8 SERPINE1 SERPINC1 F8 F3
190 giant hemangioma 9.8 SERPINF2 SERPINC1 F3 F2
191 lipoprotein quantitative trait locus 9.8 VWF SERPINE1 F3 F2
192 fournier gangrene 9.8 SERPINC1 F8 F3 F2
193 ischemic colitis 9.7 SERPINE1 SERPINC1 F5 F2
194 adhesive otitis media 9.7 F9 F7
195 sagittal sinus thrombosis 9.7 SERPINC1 F5 F3 F2
196 thrombotic thrombocytopenic purpura 9.7 VWF SERPINF2 SERPINC1 F3
197 peripheral vertigo 9.7 SERPINC1 F5 F3 F2
198 antithrombin iii deficiency 9.7 SERPINC1 F5 F3 F2
199 carotid artery thrombosis 9.7 VWF SERPINE1 SERPINC1 F3
200 qualitative platelet defect 9.7 VWF F7 F3 F2
201 congenital disorder of glycosylation, type ia 9.7 SERPINC1 F9
202 placenta disease 9.7 SERPINC1 F5 F3 F2
203 intermittent claudication 9.7 VWF SERPINE1 SERPINC1 F2
204 bilirubin metabolic disorder 9.7 F9 F3 F2
205 marantic endocarditis 9.7 VWF SERPINC1 F3 F2
206 mitral valve stenosis 9.6 VWF SERPINC1 F3 F2
207 autoimmune disease of cardiovascular system 9.6 VWF SERPINC1 F3 F2
208 varicose veins 9.6 VWF SERPINC1 F5 F2
209 endocardium disease 9.6 VWF SERPINC1 F3 F2
210 blood protein disease 9.6 VWF SERPINC1 F3 F2
211 moyamoya disease 1 9.6 VWF SERPINC1 F3 F2
212 takayasu arteritis 9.6 VWF SERPINC1 F3 F2
213 hemolytic-uremic syndrome 9.6 VWF SERPINC1 F3 F2
214 thrombocytosis 9.6 VWF SERPINC1 F3 F2
215 atherosclerosis susceptibility 9.6 VWF SERPINE1 F3
216 atrial fibrillation 9.6 VWF SERPINC1 F3 F2
217 acquired hemophilia a 9.6 F9 F8 F5 F3
218 purpura fulminans 9.5 SERPINF2 SERPINC1 F5 F3 F2
219 retinal vein occlusion 9.5 SERPINE1 SERPINC1 F5 F3 F2
220 x-linked recessive disease 9.5 VWF F9 F8 F3
221 homocysteinemia 9.4 VWF SERPINE1 SERPINC1 F5 F2
222 hepatic vascular disease 9.4 VWF SERPINE1 SERPINC1 F3 F2
223 placental insufficiency 9.4 VWF SERPINE1 SERPINC1 F3 F2
224 portal vein thrombosis 9.4 SERPINE1 SERPINC1 F7 F5 F2
225 essential thrombocythemia 9.4 VWF SERPINE1 SERPINC1 F3 F2
226 cerebral palsy 9.4 SERPINE1 SERPINC1 F7 F5 F2
227 pulmonary hypertension 9.4 VWF SERPINE1 SERPINC1 F3 F2
228 budd-chiari syndrome 9.4 SERPINC1 F7 F5 F3 F2
229 cardiovascular system disease 9.3 VWF SERPINE1 SERPINC1 F7 F3
230 post-thrombotic syndrome 9.3 SERPINE1 SERPINC1 F8 F5 F3 F2
231 protein s deficiency 9.3 SERPINE1 SERPINC1 F8 F5 F3 F2
232 brachydactyly, type d 9.3 VWF MCFD2 F9 F8
233 alpha-2-plasmin inhibitor deficiency 9.2 SERPINF2 SERPINC1 F8 F7 F3 F2
234 dic in newborn 9.2 SERPINF2 SERPINE1 SERPINC1 F7 F3 F2
235 central retinal vein occlusion 9.2 VWF SERPINC1 F8 F5 F3 F2
236 antiphospholipid syndrome 9.2 VWF SERPINE1 SERPINC1 F5 F3 F2
237 cardiac tamponade 9.2 F9 F8 F7 F3 F2
238 hellp syndrome 9.2 VWF SERPINE1 SERPINC1 F5 F3 F2
239 peripheral vascular disease 9.2 VWF SERPINE1 SERPINC1 F5 F3 F2
240 acute promyelocytic leukemia 9.2 VWF SERPINF2 SERPINE1 SERPINC1 F3 F2
241 coumarin resistance 9.2 MCFD2 F9
242 acquired von willebrand syndrome 9.2 VWF F9 F8 F7 F3
243 factor x deficiency 9.2 F9 F7 F5 F3 F2
244 hemophilia a 9.2 VWF F9 F8 F7 F3
245 sneddon syndrome 9.2 SERPINC1 F9 F8 F5 F2
246 protein c deficiency 9.1 SERPINE1 SERPINC1 F9 F5 F2
247 cerebrovascular disease 9.1 VWF SERPINE1 F8 F7 F5 F2
248 blood platelet disease 9.1 VWF SERPINC1 F8 F7 F3 F2
249 coronary thrombosis 9.1 VWF SERPINE1 SERPINC1 F7 F3 F2
250 factor xii deficiency 9.1 VWF SERPINC1 F9 F5 F3
251 diabetes mellitus 9.1 VWF SERPINE1 SERPINC1 F8 F3 F2
252 patent foramen ovale 9.0 VWF SERPINE1 SERPINC1 F5 F3 F2
253 hypertension, essential 9.0 VWF SERPINE1 SERPINC1 F7 F3 F2
254 hypothyroidism 9.0 VWF SERPINE1 F9 F8 F3 F2
255 retinal artery occlusion 8.9 SERPINE1 SERPINC1 F9 F8 F5 F2
256 intracranial hypertension 8.9 SERPINE1 SERPINC1 F9 F5 F3 F2
257 glanzmann thrombasthenia 8.9 VWF F9 F8 F7 F3 F2
258 bernard-soulier syndrome 8.9 VWF F9 F8 F7 F3 F2
259 diabetes mellitus, noninsulin-dependent 8.8 VWF SERPINF2 SERPINE1 F9 F8 F7
260 heart disease 8.8 VWF SERPINE1 SERPINC1 F8 F7 F3
261 vitamin k deficiency bleeding 8.8 SERPINC1 F9 F8 F7 F3 F2
262 factor vii deficiency 8.8 SERPINC1 F9 F8 F7 F3 F2
263 pre-eclampsia 8.8 VWF SERPINF2 SERPINE1 SERPINC1 F8 F5
264 vein disease 8.6 VWF SERPINE1 SERPINC1 F8 F7 F5
265 thrombophlebitis 8.4 VWF SERPINF2 SERPINE1 SERPINC1 F8 F7
266 prothrombin deficiency 8.3 SERPINC1 MCFD2 F9 F8 F7 F3
267 hemophilia b 8.3 VWF SERPINC1 F9 F8 F7 F3
268 inherited blood coagulation disease 8.3 VWF SERPINC1 F9 F8 F7 F5
269 pulmonary artery disease 8.3 VWF SERPINF2 SERPINE1 SERPINC1 F8 F7
270 factor v deficiency 8.2 VWF MCFD2 F9 F8 F7 F5
271 factor viii deficiency 8.2 VWF MCFD2 F9 F8 F7 F5
272 thrombophilia due to activated protein c resistance 8.2 VWF SERPINF2 SERPINE1 SERPINC1 F9 F8
273 factor xi deficiency 8.1 VWF SERPINC1 F9 F8 F7 F5
274 stroke, ischemic 8.0 VWF SERPINF2 SERPINE1 SERPINC1 F9 F7
275 thrombophilia 7.5 VWF SERPINF2 SERPINE1 SERPINC1 F9 F8
276 myocardial infarction 7.5 VWF SERPINF2 SERPINE1 SERPINC1 F9 F8

Graphical network of the top 20 diseases related to Factor Xiii Deficiency:



Diseases related to Factor Xiii Deficiency

Symptoms & Phenotypes for Factor Xiii Deficiency

Human phenotypes related to Factor Xiii Deficiency:

58 31 (showing 26, show less)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 abnormal umbilical stump bleeding 58 31 hallmark (90%) Very frequent (99-80%) HP:0011884
2 reduced factor xiii activity 58 31 hallmark (90%) Very frequent (99-80%) HP:0008357
3 umbilical cord hematoma 58 31 hallmark (90%) Very frequent (99-80%) HP:0030657
4 bruising susceptibility 58 31 frequent (33%) Frequent (79-30%) HP:0000978
5 spontaneous hematomas 58 31 frequent (33%) Frequent (79-30%) HP:0007420
6 cerebral hemorrhage 58 31 frequent (33%) Frequent (79-30%) HP:0001342
7 oral cavity bleeding 58 31 frequent (33%) Frequent (79-30%) HP:0030140
8 intramuscular hematoma 58 31 frequent (33%) Frequent (79-30%) HP:0012233
9 joint hemorrhage 58 31 frequent (33%) Frequent (79-30%) HP:0005261
10 gingival bleeding 58 31 occasional (7.5%) Occasional (29-5%) HP:0000225
11 epistaxis 58 31 occasional (7.5%) Occasional (29-5%) HP:0000421
12 bleeding with minor or no trauma 58 31 occasional (7.5%) Occasional (29-5%) HP:0011889
13 menorrhagia 58 31 occasional (7.5%) Occasional (29-5%) HP:0000132
14 poor wound healing 58 31 occasional (7.5%) Occasional (29-5%) HP:0001058
15 post-partum hemorrhage 58 31 occasional (7.5%) Occasional (29-5%) HP:0011891
16 persistent bleeding after trauma 58 31 occasional (7.5%) Occasional (29-5%) HP:0001934
17 prolonged bleeding after dental extraction 58 31 occasional (7.5%) Occasional (29-5%) HP:0006298
18 prolonged bleeding after surgery 58 31 occasional (7.5%) Occasional (29-5%) HP:0004846
19 delayed onset bleeding 58 31 occasional (7.5%) Occasional (29-5%) HP:0040232
20 prolonged bleeding following circumcision 58 31 occasional (7.5%) Occasional (29-5%) HP:0030137
21 ecchymosis 58 31 occasional (7.5%) Occasional (29-5%) HP:0031364
22 recurrent spontaneous abortion 58 31 occasional (7.5%) Occasional (29-5%) HP:0200067
23 inflammation of the large intestine 58 31 very rare (1%) Very rare (<4-1%) HP:0002037
24 hepatic failure 58 31 very rare (1%) Very rare (<4-1%) HP:0001399
25 myeloid leukemia 58 31 very rare (1%) Very rare (<4-1%) HP:0012324
26 subcutaneous hemorrhage 58 Frequent (79-30%)

Symptoms:

12 (showing 1, show less)
  • prolonged umbilical cord bleeding

MGI Mouse Phenotypes related to Factor Xiii Deficiency:

45 (showing 3, show less)
# Description MGI Source Accession Score Top Affiliating Genes
1 cardiovascular system MP:0005385 9.85 F13A1 F2 F3 F5 F7 F9
2 homeostasis/metabolism MP:0005376 9.83 CPB2 F13A1 F13B F2 F3 F5
3 mortality/aging MP:0010768 9.4 CPB2 F13A1 F2 F3 F5 F7

Drugs & Therapeutics for Factor Xiii Deficiency

Drugs for Factor Xiii Deficiency (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(showing 12, show less)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
tannic acid Approved Phase 3 1401-55-4
2
Benzocaine Approved, Investigational Phase 3 94-09-7, 1994-09-7 2337
3
Fibrinolysin Investigational Phase 3 9004-09-5
4 Fibrinolytic Agents Phase 3
5
Thrombin Approved, Investigational
6
Protein C Approved
7 Antithrombins
8 Antithrombin III
9 Antifibrinolytic Agents
10 Thromboplastin
11 Plasminogen
12 Factor VIII

Interventional clinical trials:

(showing 13, show less)
# Name Status NCT ID Phase Drugs
1 A Prospective, Multicenter, Open-label, Phase 3b Study of Human Plasma-Derived Factor XIII Concentrate in Subjects With Congenital Factor XIII Deficiency Completed NCT00885742 Phase 3
2 A Prospective, Multicenter, Open Enrollment Study of Human Plasma-Derived Factor XIII Concentrate in Subjects With Congenital Factor XIII Deficiency Completed NCT00945906 Phase 3
3 A Multi-Centre, Open-Label, Single-Arm and Multiple Dosing Trial on Efficacy and Safety of Monthly Replacement Therapy With Recombinant Factor XIII (rFXIII) in Subjects With Congenital Factor XIII Deficiency Completed NCT00713648 Phase 3 catridecacog
4 A Multi-Centre, Open-Label, Single-Arm, and Multiple Dosing Trial on Safety of Monthly Replacement Therapy With Recombinant Factor XIII (rFXIII) in Subjects With Congenital Factor XIII Deficiency Completed NCT00978380 Phase 3 catridecacog
5 A 12 Week, Multicenter, Pharmacokinetic and Safety Study of Human Plasma-Derived Factor XIII Concentrate in Subjects With Congenital Factor XIII Deficiency Completed NCT00883090 Phase 2
6 A Phase 1 Escalating Dose Study of the Safety and Pharmacokinetics of Recombinant Factor XIII in Patients With Congenital Factor XIII Deficiency Completed NCT00056589 Phase 1 catridecacog
7 Cross-sectional Study on Prevalence of Coagulation Factors Deficiency in Children Attending Assiut University Children Hospital ( a One Year Study) Unknown status NCT03273998
8 Risk Factors for Perioperative Pulmonary Embolism in Neurosurgical Patients in Respect of Factor XIII Activity Unknown status NCT01106937
9 Major Burns Coagulation and the Role of Factor XIII: A Descriptive Study Unknown status NCT03188913
10 Clinical Research Study of Factor XIII Concentrate From Human Plasma Fibrogammin P in Patients With Factor XIII Deficiency Completed NCT00640289 Fibrogammin P
11 Impact of Acquired FXIII Deficiency on Morbidity and Mortality Among Hospitalized Patients Completed NCT04416594
12 Incidence of Wound-healing-abnormalities in Major Abdominal Surgery - Association With Factors of Coagulation Completed NCT00735579
13 Factor XIII and Other Biomarkers in ST Segment Elevation Myocardial Infarction Active, not recruiting NCT03523624

Search NIH Clinical Center for Factor Xiii Deficiency

Cochrane evidence based reviews: factor xiii deficiency

Genetic Tests for Factor Xiii Deficiency

Anatomical Context for Factor Xiii Deficiency

MalaCards organs/tissues related to Factor Xiii Deficiency:

40
Myeloid, Liver, Brain, Testes, Kidney, Eye, Whole Blood

Publications for Factor Xiii Deficiency

Articles related to Factor Xiii Deficiency:

(showing 473, show less)
# Title Authors PMID Year
1
Congenital factor XIII deficiency: a commentary on 'Homozygous intronic mutation leading to inefficient transcription combined with a novel frame-shift mutation in F13A1 gene causes FXIII deficiency'. 61 6
21633364 2011
2
Novel Y283C mutation of the A subunit for coagulation factor XIII: molecular modelling predicts its impaired protein folding and dimer formation. 6 61
11380452 2001
3
Truncated mutant B subunit for factor XIII causes its deficiency due to impaired intracellular transportation. 6 61
11313256 2001
4
Two novel and one recurrent missense mutation in the factor XIII A gene in two Dutch patients with factor XIII deficiency. 61 6
11167856 2001
5
Molecular mechanisms of type II factor XIII deficiency: novel Gly562-Arg mutation and C-terminal truncation of the A subunit cause factor XIII deficiency as characterized in a mammalian expression system. 61 6
9531593 1998
6
Type I factor XIII deficiency is caused by a genetic defect of its b subunit: insertion of triplet AAC in exon III leads to premature termination in the second Sushi domain. 6 61
8639893 1996
7
Mutations causing coagulation factor XIII subunit A deficiency: characterization of the mutant proteins after expression in yeast. 61 6
7727776 1995
8
Genetic heterogeneity of factor XIII deficiency: first description of unstable A subunits. 61 6
7236530 1981
9
Homozygous intronic mutation leading to inefficient transcription combined with a novel frameshift mutation in F13A1 gene causes FXIII deficiency. 6
21512576 2011
10
The Arg703Trp missense mutation in F13A1 is a de novo event. 6
19438481 2009
11
Mutations in coagulation factor XIII A gene in three Turkish patients: two novel mutations and a known insertion. 6
12100162 2002
12
Identification and characterization of two missense mutations causing factor XIIIA deficiency. 6
10027709 1999
13
A novel insertion mutation (1286insC) in exon 9 of the factor XIII-A subunit gene. 6
9712293 1998
14
Four novel mutations in deficiency of coagulation factor XIII: consequences to expression and structure of the A-subunit. 6
8547636 1996
15
Deficiency in the A-subunit of coagulation factor XIII: two novel point mutations demonstrate different effects on transcript levels. 6
8025280 1994
16
Factor XIII ABristol 1: detection of a nonsense mutation (Arg171-->stop codon) in factor XIII A subunit deficiency. 6
7918041 1993
17
Two genetic defects in a patient with complete deficiency of the b-subunit for coagulation factor XIII. 6
8324218 1993
18
Deficiency of coagulation factor XIII A subunit caused by the dinucleotide deletion at the 5' end of exon III. 6
1644910 1992
19
Identification of a point mutation in factor XIII A subunit deficiency. 6
1353995 1992
20
A familial factor XIII subunit B deficiency. 6
2334637 1990
21
Identification of eight novel coagulation factor XIII subunit A mutations: implied consequences for structure and function. 61 54
20179087 2010
22
Clinical audit of inherited bleeding disorders in a developing country. 61 54
20090222 2010
23
Menorrhagia and reproductive health in rare bleeding disorders: a study from the Indian subcontinent. 54 61
18803553 2009
24
Cerebral thrombosis associated with heterozygous factor V Leiden mutation and high lipoprotein(a) level in a girl with factor XIII deficiency. 61 54
17473580 2007
25
Clinical and prognostic role of plasma coagulation factor XIII activity for bleeding disorders and 6-year survival in patients with chronic liver disease. 61 54
16448455 2006
26
[Molecular mechanisms of two novel mutations of factor XIII gene resulting in hereditary coagulation deficiency]. 61 54
14720426 2003
27
Transjugular liver biopsy is safe and diagnostic for patients with congenital bleeding disorders and hepatitis C infection. 61 54
14511303 2003
28
Identification of an inframe deletion and a missense mutation in the factor XIIIA gene in two Turkish patients. 54 61
12801297 2003
29
Rare inherited coagulation disorders in India. 61 54
9283899 1996
30
Gynecological and obstetric outcome in the French cohort of women with factor XIII deficiency. 61
32360976 2020
31
Autoimmune factor XIII deficiency with unusual laboratory and clinical phenotype. 61
32311817 2020
32
Severe bleeding in a patient with Factor XIII deficiency and COVID-19. 61
32516478 2020
33
Concurrent hematoma and venous thrombosis in a patient with autoimmune acquired factor XIII deficiency. 61
31132215 2020
34
Factor XIII Deficiency in Western Afghanistan due to a Novel F13A Gene Mutation. 61
31136071 2020
35
Factor XIII deficiency in two Spanish families with a novel variant in gene F13A1 detected by next-generation sequencing; symptoms and clinical management. 61
32060721 2020
36
Novel deep intronic mutation in the coagulation factor XIII a chain gene leading to unexpected RNA splicing in a patient with factor XIII deficiency. 61
31914974 2020
37
Acquired Factor XIII Deficiency Inducing Recurrent and Fatal Bleeding, Description of a Case. 61
32099500 2020
38
Congenital factor XIII deficiency: comprehensive overview of the FranceCoag cohort. 61
31414482 2020
39
Factor XIII deficiency does not prevent FeCl3-induced carotid artery thrombus formation in mice. 61
31989092 2020
40
Acquired factor XIII deficiency in two patients with bleeding events during veno-venous extracorporeal membrane oxygenation treatment. 61
31834529 2019
41
Expression and CpG island methylation pattern of MMP-2 and MMP-9 genes in patients with congenital factor XIII deficiency and intracranial hemorrhage. 61
31469059 2019
42
Factor XIII deficiency leading to preseptal haematoma post-strabismus surgery. 61
31727636 2019
43
Congenital Factor XIII Deficiency With the Presence of Inhibitor: A Case Study. 61
31764516 2019
44
Acute kidney injury is associated with low factor XIII in decompensated cirrhosis. 61
30967339 2019
45
Benefits of thromboelastometry for monitoring replacement therapy in patients with severe inherited factor XIII deficiency: 3 illustrative cases. 61
31361371 2019
46
Valproic Acid-Induced Coagulopathy. 61
31201069 2019
47
Penile prosthesis implant for primary erectile dysfunction in patient with Klippel-Trenaunay syndrome complicated by consumptive coagulopathy: A case report. 61
31393387 2019
48
Factor XIII deficiency with intracranial haemorrhage. 61
31451452 2019
49
Phenotype and genotype of FXIII deficiency in two unrelated probands: identification of a novel F13A1 large deletion mediated by complex rearrangement. 61
31340840 2019
50
Factor XIII mutation spectrum in Iranian patients with hereditary factor XIII deficiency: Detection of 3 novel mutations. 61
30578706 2019
51
Identification of novel pathogenic F13A1 mutation and novel NBEAL2 gene missense mutation in a pedigree with hereditary congenital factor XIII deficiency. 61
30935919 2019
52
Massive recurrent post-tonsillectomy bleedings revealing a transient factor XIII deficiency in a 10-year-old boy. A case report. 61
31388547 2019
53
Use of Catridecacog in a patient with severe Factor XIII deficiency undergoing surgery. 61
30915205 2019
54
Recurrent Hematomas following a Revision Total Hip Arthroplasty in Acquired Coagulation Factor XIII Deficiency. 61
31396426 2019
55
Rotational Thromboelastometry for Assessing Bleeding Complications and Factor XIII Deficiency in Cardiac Surgery Patients. 61
30198311 2018
56
Acquired factor XIII deficiency: A review. 61
30446212 2018
57
State of the art in factor XIII laboratory assessment. 61
30087086 2018
58
Novel Factor XIII variant identified through whole-genome sequencing in a child with intracranial hemorrhage. 61
30404926 2018
59
Segmental uniparental disomy as a rare cause of congenital severe factor XIII deficiency in a girl with only one heterozygous carrier parent. 61
30702381 2018
60
Pathogenicity analysis of variations and prenatal diagnosis in a hereditary coagulation factor XIII deficiency family. 61
29307277 2018
61
Immunotolerance approach to refractory CNS bleeding in a patient with congenital factor XIII deficiency and acquired alloantibody. 61
29790627 2018
62
Recombinant factor XIII A-subunit in a patient with factor XIII deficiency and recurrent pregnancy loss. 61
29665207 2018
63
Acquired Factor Xiii Deficiency: An Uncommon But Easily Missed Cause Of Severe Bleeding 61
30489053 2018
64
Identification of a novel nonsense mutation leading to congenital factor XIII deficiency. 61
29604433 2018
65
[Factor XIII-guided treatment algorithm reduces blood transfusion in burn surgery]. 61
29269148 2018
66
Intracranial Hemorrhage: A Devastating Outcome of Congenital Bleeding Disorders-Prevalence, Diagnosis, and Management, with a Special Focus on Congenital Factor XIII Deficiency. 61
28898896 2018
67
Molecular Basis of Congenital Factor XIII Deficiency in Iran. 61
27879471 2018
68
Factor XIII deficiency diagnosis: Challenges and tools. 61
29027765 2018
69
Molecular diagnosis of factor XIII deficiency, data from comprehensive coagulation laboratory in Iran. 61
29095761 2018
70
Choroid Plexus Papilloma and Factor XIII Deficiency: Case Report. 61
30176663 2018
71
Thromboelastometry Identified Alteration of Clot Stabilization and Factor XIII Supplementation Need in a Patient with Decompensated Liver Disease Undergoing Liver Biopsy. 61
30228917 2018
72
A large case series on surgical outcomes in congenital factor XIII deficiency patients in Iran. 61
29028293 2017
73
Lobar Hemorrhage Induced by Acquired Factor XIII Deficiency in a Patient with Cerebral Amyloid Angiopathy. 61
28801214 2017
74
De Novo Precursor B-Lymphoblastic Leukemia/Lymphoma With Double-Hit Gene Rearrangements (MYC/BCL-2) Presented With Spinal Cord Compression and Acquired Factor XIII Deficiency. 61
32300395 2017
75
Factor XIII deficiency in south of Tunisia. 61
28704210 2017
76
Is Extracorporeal CO2 Removal Really "Safe" and "Less" Invasive? Observation of Blood Injury and Coagulation Impairment during ECCO2R. 61
28187047 2017
77
Defective α2 antiplasmin cross-linking and thrombus stability in a case of acquired factor XIII deficiency. 61
28516512 2017
78
The first case report of a patient with acquired factor XIII deficiency in the context of autoimmune encephalitis. 61
28664679 2017
79
Detection of Factor XIII deficiency: data from multicentre exercises amongst UK NEQAS and PRO-RBDD project laboratories. 61
28406553 2017
80
Recombinant factor XIII prophylaxis is safe and effective in young children with congenital factor XIII-A deficiency: international phase 3b trial results. 61
28581691 2017
81
Fracture neck of femur in Factor XIII deficiency: Was better outcome possible? 61
29417025 2017
82
Long-term prophylaxis in patients with severe congenital factor XIII deficiency is not complicated by inhibitor formation. 61
27306330 2017
83
Diagnosis, clinical manifestations and management of rare bleeding disorders in Iran. 61
27894217 2017
84
Autosomal recessive inherited bleeding disorders in Pakistan: a cross-sectional study from selected regions. 61
28388959 2017
85
Factor XIII Deficiency and Thrombocytopenia Are Frequent Modulators of Postoperative Clot Firmness in a Surgical Intensive Care Unit. 61
28503124 2017
86
Molecular genetic analysis of ten unrelated Iranian patients with congenital factor XIII deficiency. 61
28013530 2017
87
Rotational thromboelastometry can detect factor XIII deficiency and bleeding diathesis in patients with cirrhosis. 61
27634287 2017
88
Treatment with Recombinant Factor XIII (Tretten) in a Pregnant Woman with Factor XIII Deficiency. 61
28432284 2017
89
Original tandem duplication in FXIIIA gene with splicing site modification and four amino acids insertion causes factor XIII deficiency. 61
27427785 2017
90
Developing the First Recombinant Factor XIII for Congenital Factor XIII Deficiency: Clinical Challenges and Successes. 61
27556350 2017
91
Impact of Rare Bleeding Disorders during Pregnancy on Maternal and Fetal Outcomes: Review of 29 Pregnancies at a Single Center. 61
28166593 2017
92
Autoimmune acquired factor XIII deficiency due to anti-factor XIII/13 antibodies: A summary of 93 patients. 61
27542511 2017
93
Factor XIII deficiency enhances thrombin generation due to impaired fibrin polymerization - An effect corrected by Factor XIII replacement. 61
27902939 2017
94
Disseminated intravascular coagulation with positive D-dimer: a controversial clinical feature in severe congenital factor XIII deficiency in southeast Iran. 61
26836269 2016
95
Comparison of 2 Methods of Clot Solubility Testing in Detection of Factor XIII Deficiency. 61
27522062 2016
96
Blood coagulation factor XIII and factor XIII deficiency. 61
27344554 2016
97
Recurrent Post Tonsillectomy Secondary Hemorrhage in Patients with Factor XIII Deficiency: A Case Series and Review of Literature. 61
27843132 2016
98
Diagnosis of factor XIII deficiency. 61
27077776 2016
99
Laboratory Diagnosis of Factor XIII Deficiency in Developing Countries: An Iranian Experience. 61
27346867 2016
100
Guidelines for laboratory diagnosis of factor XIII deficiency. 61
26588445 2016
101
Acquired coagulation factor XIII deficiency: a case report. 61
26588447 2016
102
Mild factor XIII deficiency and concurrent hypofibrinogenemia: effect of pregnancy. 61
26575494 2016
103
Once daily ledipasvir/sofosbuvir fixed-dose combination with ribavirin in patients with inherited bleeding disorders and hepatitis C genotype 1 infection. 61
26315711 2016
104
Genetic basis of severe factor XIII deficiency in a large cohort of Indian patients: Identification of fourteen novel mutations. 61
26852661 2016
105
Morbidity and mortality in a large number of Iranian patients with severe congenital factor XIII deficiency. 61
26692088 2016
106
Laboratory Diagnosis of Factor XIII Deficiency, Routine Coagulation Tests with Quantitative and Qualitative Methods. 61
27215067 2016
107
Medical and Surgical Management of Postpartum Hemorrhage in a Woman with Factor XIII Deficiency. 61
27635271 2016
108
Long Term Follow up Study on a Large Group of Patients with Congenital Factor XIII Deficiency Treated Prophylactically with Fibrogammin P®. 61
27642336 2016
109
Is Embolization an Effective Treatment for Recurrent Hemorrhage After Hip or Knee Arthroplasty? 61
26220895 2016
110
Effects of Factor XIII Deficiency on Thromboelastography. Thromboelastography with Calcium and Streptokinase Addition is more Sensitive than Solubility Tests. 61
27648200 2016
111
Establishment of a prenatal diagnosis schedule as part of a prophylaxis program of factor XIII deficiency in the southeast of Iran. 61
26703985 2016
112
First cases of severe congenital factor XIII deficiency in Southwestern Afghanistan in the vicinity of southeast of Iran. 61
26226252 2015
113
Identification of acquired coagulation disorders and effects of target-controlled coagulation factor substitution on the incidence and severity of spontaneous intracranial bleeding during veno-venous ECMO therapy. 61
25823366 2015
114
[Coagulation factor XIII – Pathophysiology, clinic and therapy of factor XIII deficiency]. 61
26650946 2015
115
Factor XIII deficiency and head trauma: management and therapy. 61
26116182 2015
116
[Identification of genetic defects in a Chinese pedigree with factor XIII deficiency: case report and literature review]. 61
26477763 2015
117
Challenges in implementation of ISTH diagnostic algorithm for diagnosis and classification of factor XIII deficiency in Iran. 61
26099358 2015
118
Association between expression of MMP-2 and MMP-9 genes and pathogenesis of intracranial hemorrhage in severe coagulation factor XIII deficiency. 61
25544259 2015
119
Factor XIII deficiency revealed by spontaneous intramedullary hemorrhage: confirmation of a severe mutation. 61
25828969 2015
120
[Acquired von Willebrand syndrome in a patient with immune thrombocytopenic purpura]. 61
26256928 2015
121
Successful treatment of severe gastrointestinal manifestations of Henoch-Schonlein Purpura and factor XIII deficiency using cryoprecipitate transfusion. 61
30805443 2015
122
Nonhemophiliac musculoskeletal pseudotumor. 61
31724593 2015
123
The Story of Serum Prothrombin Conversion Accelerator, Proconvertin, Stable Factor, Cothromboplastin, Prothrombin Accelerator or Autoprothrombin I, and Their Subsequent Merging into Factor VII. 61
25973586 2015
124
Effect of Social Factors on the Highest Global Incidence of Congenital Factor XIII Deficiency in Southeast of Iran. 61
26155604 2015
125
Identification of two novel missense mutations causing severe factor XIII deficiency. 61
25832324 2015
126
Factor XIII deficiency in Iran: a comprehensive review of the literature. 61
25615432 2015
127
Diagnosis and management of severe congenital factor XIII deficiency in the Emergency Department: lessons from a "model" family. 61
25369590 2015
128
Central nervous system bleeding in pediatric patients with factor XIII deficiency: a study on 23 new cases. 61
25001244 2015
129
[Study on the molecular mechanisms of a novel large deletion of FXIIIA mRNA in a new hereditary factor XIII deficiency]. 61
25778889 2015
130
Acquired factor XIII deficiency after desensitization as a potential contributor to postoperative bleeding: more than meets the eye. 61
25265989 2015
131
Efficacy and safety of prophylactic treatment with plasma-derived factor XIII concentrate (human) in patients with congenital factor XIII deficiency. 61
25377187 2015
132
The use of recombinant factor XIII in a major bleeding episode of a patient with congenital factor XIII deficiency--the first experience. 61
25471755 2015
133
Pharmacokinetics and safety of plasma-derived factor XIII concentrate (human) in patients with congenital factor XIII deficiency. 61
25458735 2015
134
Novel and recurrent mutations in the F13A1 gene in unrelated Korean patients with congenital factor XIII deficiency. 61
25004025 2015
135
Neoplasm-induced bleeding in inherited, heterozygous FXIII-A deficiency. 61
26540128 2015
136
Clinical manifestations and management of life-threatening bleeding in the largest group of patients with severe factor XIII deficiency. 61
25230816 2014
137
Successful treatment of chronic disseminated intravascular coagulation using recombinant human soluble thrombomodulin in a dialysis patient with dissecting aortic aneurysm. 61
25501411 2014
138
Recurrent episodes of severe bleeding caused by congenital factor XIII deficiency in a dog. 61
25356716 2014
139
Development of anti-factor XIII antibodies in a patient with hereditary factor XIII deficiency receiving therapy for chronic hepatitis C. 61
25333572 2014
140
Fibrinogen but not factor XIII deficiency is associated with bleeding after craniotomy. 61
24871873 2014
141
Recurrent spontaneous splenic rupture in a patient with congenital factor XIII deficiency. 61
24351968 2014
142
Congenital factor XIII deficiency in Pakistan: characterization of seven families and identification of four novel mutations. 61
24329762 2014
143
Factor XIII deficiency in Henoch-Schönlein purpura - report on two cases and literature review. 61
25182395 2014
144
Congenital afibrinogenemia in a new born: a rare cause for bleeding. 61
24509333 2014
145
Investigation of a link between raised levels of pepsinogen in blood as a mediator of in-vitro clot lysis in acid and a cause of abnormal factor XIII screening tests. 61
24440916 2014
146
A new era of therapy for congenital factor XIII deficiency. 61
25003489 2014
147
Factor XIII deficiency management: a review of the literature. 61
24401950 2014
148
Intracranial hemorrhage pattern in the patients with factor XIII deficiency. 61
24149912 2014
149
Severe congenital factor XIII deficiency caused by novel W187X and G273V mutations in the F13A gene; diagnosis and classification according to the ISTH/SSC guidelines. 61
24286209 2014
150
Factor XIII Assays and associated problems for laboratory diagnosis of factor XIII deficiency: an analysis of International Proficiency testing results. 61
24497117 2014
151
Ovarian haemorrhage: a rare presentation and diagnostic dilemma in factor XIII deficiency. 61
24640817 2014
152
Acquired factor XIII deficiency: still a clinical challenge in the era of novel therapy. 61
24354481 2014
153
Polymorphism of thrombin-activatable fibrinolysis inhibitor and risk of intracranial haemorrhage in factor XIII deficiency. 61
24354489 2014
154
Factor XIII deficiency: complete phenotypic characterization of two cases with novel causative mutations. 61
24118344 2014
155
Spontaneous epidural hematoma in a child with inherited factor XIII deficiency. 61
23619114 2014
156
Symptomatic factor XIII deficiency with normal urea solubility test. 61
25651747 2014
157
Molecular pathology of rare bleeding disorders (RBDs) in India: a systematic review. 61
25275492 2014
158
Coagulation factor XIII deficiency. Diagnosis, prevalence and management of inherited and acquired forms. 61
24503678 2014
159
[A neonate with umbilical cord bleeding]. 61
25004785 2014
160
[A 79-year-old patient with severe bleeding after colon surgery and normal global coagulation parameters]. 61
24240605 2013
161
Thromboelastometric detection of clotting Factor XIII deficiency in cardiac surgery patients. 61
23962029 2013
162
A child with acquired factor XIII deficiency: case report and literature review. 61
23607876 2013
163
Spontaneous acute cerebral hematoma in a child with factor XIII deficiency. 61
23697960 2013
164
Congenital factor XIII deficiency in women: a systematic review of literature. 61
23992439 2013
165
The perioperative course of factor XIII and associated chest tube drainage in newborn and infants undergoing cardiac surgery. 61
23668424 2013
166
Rare bleeding disorders in children: identification and primary care management. 61
24127475 2013
167
Pregnancy complications and obstetric care in women with inherited bleeding disorders. 61
24102860 2013
168
The role of fibrin matrices and tissue factor in early-term trophoblast proliferation and spreading. 61
24041635 2013
169
Procoagulant platelets form an α-granule protein-covered "cap" on their surface that promotes their attachment to aggregates. 61
23995838 2013
170
Factor XIII deficiency: an update. 61
23929307 2013
171
Hyperfibrinolysis and acquired factor XIII deficiency in newly diagnosed pediatric malignancies. 61
23753026 2013
172
A common F13A1 intron 1 variant IVS1+12(A) is associated with mild FXIII deficiency in Caucasian population. 61
23508224 2013
173
Post-infectious acute glomerulonephritis and transient coagulation factor XIII deficiency associated with acute parvovirus B19 infection in a young woman--reply. 61
23683879 2013
174
Post-infectious acute glomerulonephritis and transient coagulation factor XIII deficiency associated with acute parvovirus B19 infection in a young woman. 61
23683878 2013
175
Laboratory testing for bleeding disorders: strategic uses of high and low-yield tests. 61
23480172 2013
176
Biology of Factor XIII and clinical manifestations of Factor XIII deficiency. 61
22928875 2013
177
An investigation of the spectrum of common and rare inherited coagulation disorders in north-eastern Iran. 61
23114518 2013
178
Acquired factor XIII deficiency: a therapeutic challenge. 61
23306660 2013
179
New developments in the management of congenital Factor XIII deficiency. 61
23761984 2013
180
Current understanding in diagnosis and management of factor XIII deficiency. 61
24575291 2013
181
Relevant bleeding diathesis due to acquired factor XIII deficiency. 61
24169946 2013
182
Acute abdomen in a young girl with factor XIII deficiency: Perianesthetic issues. 61
23495278 2013
183
Occurrence and recurrence of spontaneous chronic subdural haematoma is associated with a factor XIII deficiency. 61
22541133 2013
184
Tocilizumab induced acquired factor XIII deficiency in patients with rheumatoid arthritis. 61
23936360 2013
185
The rare inherited coagulation disorders. 61
23109366 2013
186
Factor XIII deficiency presenting with intracerebral bleed. 61
23314446 2013
187
Successful use of recombinant factor VIIa in a child with Schoenlein-Henoch purpura presenting with compartment syndrome and severe factor XIII deficiency. 61
23103728 2013
188
Corifact™/Fibrogammin® P in the prophylactic treatment of hereditary factor XIII deficiency: results of a prospective, multicenter, open-label study. 61
23439001 2012
189
Hemorrhagic-acquired factor XIII deficiency associated with tocilizumab for treatment of rheumatoid arthritis. 61
23070535 2012
190
Molecular modeling predicts structural changes in the A subunit of factor XIII caused by two novel mutations identified in a neonate with severe congenital factor XIII deficiency. 61
22633530 2012
191
A longitudinal prospective study of bleeding diathesis in Egyptian pediatric patients: single-center experience. 61
22610136 2012
192
Difficulties and pitfalls in the laboratory diagnosis of bleeding disorders. 61
22726086 2012
193
[Bleeding and coagulation disorders in tonsillectomies]. 61
22398952 2012
194
Coagulation management in patients undergoing mechanical circulatory support. 61
22910089 2012
195
Acute promyelocytic leukaemia associated Factor XIII deficiency presenting as retro-bulbar haematoma. 61
22483775 2012
196
[Spontaneous intracerebral haemorrhage secondary to congenital factor XIII deficiency]. 61
22465647 2012
197
Recombinant factor XIII: a safe and novel treatment for congenital factor XIII deficiency. 61
22451421 2012
198
Acute Abdomen in a Young Girl with Factor XIII Deficiency Perianesthetic Issues. 61
23543997 2012
199
Further evidence of heterogeneity of gene defects in Italian families with factor XIII deficiency. 61
21812861 2012
200
Reduced difference of α₂-plasmin inhibitor levels between plasma and serum in patients with severe factor XIII deficiency, including autoimmune hemorrhaphilia due to anti-factor XIII antibodies. 61
22205503 2012
201
[Molecular mechanisms of Arg77Cys missense mutation and Arg174stop nonsense mutation of factor XIII A gene causing severe factor XIII deficiency]. 61
22169303 2011
202
Management of pregnancy and delivery in women with inherited bleeding disorders. 61
21852211 2011
203
Induction of hemodialysis therapy in a case with factor XIII deficiency. 61
21710188 2011
204
Factor XIII Deficiency in Siblings: Importance of Prophylactic Replacement. 61
22942571 2011
205
Novel aspects of factor XIII deficiency. 61
21738029 2011
206
Postoperative bleeding in a patient with normal screening coagulation tests. 61
21804371 2011
207
Mutations in coagulation factor XIII subunit A in severe factor XIII deficiency patients: five novel mutations detected. 61
21658166 2011
208
An update of the mutation profile of Factor 13 A and B genes. 61
21640452 2011
209
How to deal with medical and social aspects of bleeding disorders--preparing women and the family in developing countries. 61
21692929 2011
210
Successful long-term replacement therapy with FXIII concentrate (Fibrogammin(®) P) for severe congenital factor XIII deficiency: a prospective multicentre study. 61
21481176 2011
211
As many as 12 cases with haemorrhagic acquired factor XIII deficiency due to its inhibitors were recently found in Japan. 61
21264450 2011
212
[Role of thrombelastometry for the monitoring of factor XIII. A prospective observational study in neurosurgical patients]. 61
21152676 2011
213
Posttraumatic subgaleal and orbital hematoma due to factor XIII deficiency. 61
21284470 2011
214
Factor XIII deficiency related recurrent spontaneous intracerebral hemorrhage: a case and literature review. 61
20950938 2011
215
Spontaneous regression of the inhibitor against the coagulation factor XIII A subunit in acquired factor XIII deficiency. 61
20941461 2010
216
FXIII deficiency due to base exchange Thr 449 (ACT) --> Ile (ATT) in exon 11 of the factor 13A gene. A cause of bleeding? 61
20680227 2010
217
Spinal anaesthesia and caesarean section in a patient with hypofibrinogenaemia and factor XIII deficiency. 61
20345422 2010
218
Congenital factor XIII deficiency caused by two mutations in eight Tunisian families: molecular confirmation of a founder effect. 61
19937244 2010
219
Long-term prophylaxis in patients with factor XIII deficiency complicated by intracranial haemorrhage in Iran. 61
19878334 2010
220
Factor XIII deficiency as a potential cause of supratentorial haemorrhage after posterior fossa surgery. 61
19557304 2010
221
Novel human pathological mutations. Gene symbol: F13A1. Disease: Factor XIII Deficiency. 61
20108427 2010
222
Spontaneous rupture of the spleen in Factor XIII deficiency: A report of two cases. 61
22802765 2010
223
Factor XIII deficiency: report of two cases. 61
21189773 2010
224
In vitro inhibition of factor XIII retards clot formation, reduces clot firmness, and increases fibrinolytic effects in whole blood. 61
19762725 2009
225
Subgaleal hematoma presenting as a manifestation of Factor XIII deficiency. 61
19255750 2009
226
Factor XIII Deficiency. 61
19598071 2009
227
Acute and diffuse postoperative bleeding after free latissimus dorsi flap--Factor XIII deficiency: a case report and review of the literature. 61
19114970 2009
228
Severe bleeding complications caused by an autoantibody against the B subunit of plasma factor XIII: a novel form of acquired factor XIII deficiency. 61
18955560 2009
229
Factor XIII deficiency. 61
19141159 2008
230
Factor XIII deficiency (fibrin stabilizing factor). 61
19097552 2008
231
Factor XIII deficiency in children--clinical presentation and outcome. 61
18803895 2008
232
Intracranial haemorrhage in patients with congenital haemostatic defects. 61
18637845 2008
233
High incidence of vaproate-induced coagulation disorders in children receiving valproic acid: a prospective study. 61
18600085 2008
234
An unusual clinical presentation of factor XIII deficiency and issues relating to the monitoring of factor XIII replacement therapy. 61
18600098 2008
235
Cranial hemophilic pseudotumor associated with factor IX deficiency: case report. 61
18284939 2008
236
[Thrombelastometric detection of factor XIII deficiency]. 61
18338137 2008
237
Corpus luteal hemorrhage: an unusual manifestation of congenital factor XIII deficiency. 61
18397286 2008
238
Factor XIII deficiency: a differential diagnosis to be considered in suspected nonaccidental injury presenting with intracranial hemorrhage. 61
18057145 2008
239
Effects of recombinant activated factor VII on thrombin-mediated feedback activation of coagulation. 61
18277134 2008
240
[Characterization of a large deletion that leads to congenital factor XIII deficiency]. 61
18411802 2008
241
Management of acute myocardial infarction in a patient with factor XIII deficiency using prophylactic factor replacement therapy. 61
17961171 2008
242
Molecular characterization of five Italian families with inherited severe factor XIII deficiency. 61
18028394 2008
243
Spontaneous splenic rupture in a patient with factor XIII deficiency and a novel mutation. 61
16456856 2008
244
Phenotype-genotype correlation in eight Polish patients with inherited Factor XIII deficiency: identification of three novel mutations. 61
17880458 2007
245
[Identification of a novel mutation of F (13) A gene in a pedigree with factor XIII deficiency]. 61
18246815 2007
246
The influence of intrinsic coagulation pathway on blood platelets activation by oxidized cellulose. 61
17274026 2007
247
[Factor XIII deficiency in a newborn]. 61
17451919 2007
248
International registry on factor XIII deficiency: a basis formed mostly on European data. 61
17549292 2007
249
[Congenital factor XIII deficiency required high-dose factor XIII concentrate in late pregnancy]. 61
17571589 2007
250
Congenital factor XIII deficiency in Switzerland: from the worldwide first case in 1960 to its molecular characterisation in 2005. 61
17594539 2007
251
Congenital blood coagulation factor XIII deficiency and successful deliveries: a review of the literature. 61
17371605 2007
252
[Congenital factor XIII deficiency in the south of Tunisia]. 61
16530351 2006
253
Safety and pharmacokinetics of recombinant factor XIII-A2 administration in patients with congenital factor XIII deficiency. 61
16556896 2006
254
Case report of an acquired factor XIII inhibitor: diagnosis and management. 61
17252037 2006
255
Effect of four missense mutations in the factor XIII A-subunit gene on protein stability: studies with recombinant proteins. 61
16479194 2006
256
Mutations in coagulation factor XIII A gene in eight unrelated Indians. Five novel mutations identified by a novel PCR-CSGE approach. 61
16525586 2006
257
[A novel genetic defect in a Chinese family with inherited coagulation factor XIII deficiency]. 61
16792911 2006
258
Factor XIII deficiency causes cardiac rupture, impairs wound healing, and aggravates cardiac remodeling in mice with myocardial infarction. 61
16505171 2006
259
Identification of a novel mutation combination in factor XIII deficiency: genetic update to the first reported case in the United States. 61
16513532 2006
260
Characterisation of six novel A-subunit mutations leading to congenital factor XIII deficiency and molecular analysis of the first diagnosed patient with this rare bleeding disorder. 61
16543965 2006
261
A novel compound heterozygous mutation in the F13A gene causing hereditary factor XIII deficiency in a Chinese family. 61
16409483 2006
262
Prophylaxis in rare coagulation disorders -- factor XIII deficiency. 61
16616323 2006
263
Factor XIII deficiency: new nonsense and deletion mutations in the human factor XIIIA gene. 61
16330458 2005
264
Melanoma associated with subacute primitive fibrinolysis. 61
16268882 2005
265
Congenital blood coagulation factor XIII deficiency and perinatal management. 61
16026274 2005
266
Recurrent miscarriage syndrome and infertility due to blood coagulation protein/platelet defects: a review and update. 61
15678268 2005
267
A case of deficiency of plasma plasminogen activator inhibitor-1 related to Ala15Thr mutation in its signal peptide. 61
15650551 2005
268
Prophylactic and perioperative replacement therapy for acquired factor XIII deficiency: reply to a rebuttal. 61
15634290 2005
269
Seven novel mutations in the factor XIII A-subunit gene causing hereditary factor XIII deficiency in 10 unrelated families. 61
15456491 2004
270
Factor XIII deficiency in south-east Iran. 61
15357772 2004
271
Prophylactic and perioperative replacement therapy for acquired factor XIII deficiency. 61
15140148 2004
272
Acquired factor XIII deficiency due to an inhibitor: a case report and review of the literature. 61
15136243 2004
273
Factor XIII deficiency: a rare cause of repeated abortions. 61
15094990 2004
274
Factor XIII deficiency associated with valproate treatment. 61
14738427 2004
275
Phenotype-genotype characterization of 10 families with severe a subunit factor XIII deficiency. 61
14695539 2004
276
Problems relating to the laboratory diagnosis of factor XIII deficiency: a UK NEQAS study. 61
14675096 2003
277
Prevalence of factor XIII deficiency in patients presenting with a bleeding disorder in Pakistan. 61
14675114 2003
278
Deficiency of factor XIII gene in Chinese: 3 novel mutations. 61
14604285 2003
279
A case of giant expanding cephalhematoma: does the administration of blood coagulation factor XIII reverse symptoms? 61
12900121 2003
280
Pattern of symptoms in 93 Iranian patients with severe factor XIII deficiency. 61
12911609 2003
281
Valproate induces reversible factor XIII deficiency with risk of perioperative bleeding. 61
12859294 2003
282
Combined occurrence of von Willebrand's disease and factor XIII deficiency: a case report. 61
15022915 2003
283
Inherited bleeding disorders in Indian women with menorrhagia. 61
12614371 2003
284
Batroxobin-induced clots exhibit delayed and reduced platelet contractile force in some patients with clotting factor deficiencies. 61
12871496 2003
285
Surgery in severe factor XIII deficiency: report of a case of epilepsy neurosurgery and review. 61
12558790 2003
286
Factor XIII deficiency mistaken for battered child syndrome: case of "correct" test ordering negated by a commonly accepted qualitative test with limited negative predictive value. 61
12447966 2002
287
Unusual presentation of factor XIII deficiency. 61
12199683 2002
288
Clinical and laboratory features of congenital factor XIII deficiency. 61
12070580 2002
289
[Identification of two novel mutation in two Chinese hereditary coagulation factor XIII deficiency families]. 61
12015062 2002
290
Delayed umbilical bleeding--a presenting feature for factor XIII deficiency: clinical features, genetics, and management. 61
11826242 2002
291
[Clinical course and management of severe congenital factor XIII deficiency]. 61
12193985 2002
292
Recurrent bleeding following rhinoplasty due to factor XIII deficiency. 61
11698876 2001
293
Physiopathology and regulation of factor XIII. 61
11487042 2001
294
Identification of a new Leu354Pro mutation responsible for factor XIII deficiency. 61
11168522 2001
295
Viral markers and use of factor products among Finnish patients with bleeding disorders. 61
11136380 2001
296
Factor XIII deficiency causing mutation, Ser295Arg, in exon 7 of the factor XIIIA gene. 61
11057855 2000
297
Identification of a new mutation (Gly420Ser), distal to the active site, that leads to factor XIII deficiency. 61
11073170 2000
298
[Cephalohemoatoma as the first manifestation of congenital factor XIII deficiency]. 61
11083967 2000
299
Factor XIII deficiency and postoperative hemorrhage after neurosurgical procedures. 61
11118574 2000
300
Maternal blood coagulation factor XIII is associated with the development of cytotrophoblastic shell. 61
10833374 2000
301
Bleeding risk and reproductive capacity among patients with factor XIII deficiency: a case presentation and review of the literature. 61
10674253 2000
302
Reduced levels of coagulation factor XIII in patients with advanced tumor disease. 61
10690608 2000
303
Molecular and genetic mechanisms of factor XIII A subunit deficiency. 61
10805274 2000
304
Factor XIII deficiency. 61
10583246 1999
305
Spectrum of hereditary coagulation factor deficiencies in eastern province, Saudi Arabia. 61
11924110 1999
306
[Congenital factor XIII deficiency in pregnancy. A case report]. 61
10638168 1999
307
[An infant with umbilical cord and intracranial hemorrhage--severe factor XIII deficiency]. 61
10517131 1999
308
[Primary myelofibrosis with fatal mesenteric arterial thromboembolism caused by antiphospholipid syndrome]. 61
10496039 1999
309
Prenatal diagnosis in factor XIII-A deficiency. 61
10212091 1999
310
Low levels of fibrin-stabilizing factor (factor XIII) in human Plasmodium falciparum malaria: correlation with clinical severity. 61
9988331 1999
311
[Is the use of factor XIII for delayed wound healing in patients with head-neck tumors of value?]. 61
10036675 1998
312
Factor concentrates for the treatment of factor XIII deficiency. 61
9814641 1998
313
Arg260-Cys mutation in severe factor XIII deficiency: conformational change of the A subunit is predicted by molecular modelling and mechanics. 61
9609521 1998
314
Novel deletion and insertion mutations cause splicing defects, leading to severe reduction in mRNA levels of the A subunit in severe factor XIII deficiency. 61
9531026 1998
315
SD Plasma in TTP and coagulation factor deficiencies for which no concentrates are available. 61
9789534 1998
316
[Recurrent hematomas and normal standard hemostasis tests]. 61
9750687 1998
317
Molecular mechanisms of mutations in factor XIII A-subunit deficiency: in vitro expression in COS-cells demonstrates intracellular degradation of the mutant proteins. 61
9241733 1997
318
Factor XIII deficiency due to a Leu660Pro mutation in the factor XIII subunit-a gene in three unrelated Palestinian Arab families. 61
9241732 1997
319
Recurrent ulcerations on both legs since early childhood due to a factor V gene mutation. 61
9187855 1997
320
Congenital factor XIII deficiency: a patient report and review of the literature. 61
9007348 1997
321
Coagulation factor XIII in pregnant smokers and non-smokers. 61
9144031 1997
322
Application of HUMF13A01 (AAAG)n STR polymorphism to the genetic diagnosis of coagulation factor XIII deficiency. 61
8972004 1996
323
New mutations causing the premature termination of translation in the A subunit gene of coagulation factor XIII. 61
8865519 1996
324
ETRO Working Party on Factor XIII questionnaire on congenital factor XIII deficiency in Europe: status and perspectives. Study Group. 61
8989825 1996
325
Therapeutic factor XIII preparations and perspectives for recombinant factor XIII. 61
8989827 1996
326
Gene defects in congenital factor XIII deficiency. 61
8989822 1996
327
Factor XIII deficiency: pathogenic mechanisms and clinical significance. 61
8989826 1996
328
The normal and abnormal genes of the a and b subunits in coagulation factor XIII. 61
8989821 1996
329
Claw toes correction and factor XIII deficiency--a case report. 61
8668067 1995
330
Molecular basis of inherited factor XIII deficiency: identification of multiple mutations provides insights into protein function. 61
8555083 1995
331
Factor XIIIA Calgary: a candidate missense mutation (Leu667Pro) in the beta barrel 2 domain of the factor XIIIA subunit. 61
8547093 1995
332
[Leukocytoclastic vasculitis and factor XIII deficiency]. 61
7637414 1995
333
Optimisation of a new continuous UV assay for the determination of blood coagulation factor XIII activity in human plasma. 61
7918847 1994
334
[Intradural hematoma of the foramen magnum associated with factor XIII deficiency]. 61
7863159 1994
335
Congenital factor XIII deficiency. 61
8005932 1993
336
Deficiency of plasma plasminogen activator inhibitor 1 results in hyperfibrinolytic bleeding. 61
8481516 1993
337
Hereditary factor XIII deficiency. 61
8253495 1993
338
Factor XIII deficiency in Pakistan. 61
8230654 1993
339
[Factor XIII deficiency as the cause of postoperative hemorrhage]. 61
8147170 1993
340
Hemophiliacs--a picture from a developing country: Karnataka, a south Indian state. 61
7886595 1993
341
A reversed activity staining procedure for detection of an acquired antibody against factor XIII in a girl with factor XIII deficiency. 61
1419823 1992
342
Congenital factor XIII deficiency. 61
1428130 1992
343
[Factor XIII deficiency: blood coagulation defect in pregnancy]. 61
1414069 1992
344
[Pre-hospital diagnosis of nosebleed in children]. 61
1788893 1991
345
Intraspinal hemorrhage in a child with factor XIII deficiency. 61
1758779 1991
346
Platelet and coagulation studies in Ehlers-Danlos syndrome. 61
1911298 1991
347
Congenital factor XIII deficiency: two case reports. 61
1938825 1991
348
Congenital factor XIII deficiency. A family report. 61
1816054 1991
349
Low prevalence of antibodies against human immunodeficiency virus in Finnish haemophiliacs. 61
1862639 1991
350
Factor XIII deficiency in adult polycystic kidney disease. 61
1896103 1991
351
Clinical pharmacokinetics of a placenta-derived factor XIII concentrate in type I and type II factor XIII deficiency. 61
1984679 1991
352
Factor XIII-deficiency in the blood of venous leg ulcer patients. 61
1676216 1991
353
Congenital factor XIII deficiency associated with von Willebrand disease. 61
2220766 1990
354
[Stenosing ureteritis and factor XIII deficiency in anaphylactoid purpura]. 61
2284881 1990
355
Factor XIII deficiency and intracranial hemorrhages in infancy. 61
2206163 1990
356
[Intracranial hematoma accompanying bleeding tendency: therapeutic practice and analysis of literature]. 61
2203982 1990
357
Congenital factor XIII deficiency with treatment of factor XIII concentrate and normal vaginal delivery. 61
2358199 1990
358
Congenital factor XIII deficiency: report of a case and literature review. 61
2697742 1989
359
Spontaneous intracerebral hematoma in a adolescent with factor XIII deficiency. Case report. 61
2613496 1989
360
[Anesthesia for a patient with Ehlers-Danlos syndrome and factor XIII deficiency]. 61
2585722 1989
361
The subunit composition of factor XIII proteins in normal and factor XIII deficient plasma and serum analysed by line immunoelectrophoresis. 61
2714001 1989
362
Congenital factor XIII deficiency: a case report and review of literature. 61
2687169 1989
363
Congenital factor XIII deficiency with multiple benign breast tumours and successful pregnancy with substitutive therapy. A case report. 61
2620871 1989
364
[Acquired factor XIII deficiency and chronic myeloid splenomegaly]. 61
2741236 1989
365
Inherited factor XIII deficiency. 61
2763783 1989
366
Hemostasis in Crohn's disease: low factor XIII levels in active disease. 61
3201134 1988
367
Clinical experience with a pasteurised human plasma concentrate in factor XIII deficiency. 61
3388293 1988
368
Intracranial hemorrhage in congenital deficiency of factor XIII. 61
3189718 1988
369
[Acute malignant myelofibrosis complicated by factor XIII deficiency, protein C decrease and skin necroses]. 61
3312074 1987
370
Successful pregnancy in a woman with congenital factor XIII deficiency treated with substitutive therapy. Report of a second case. 61
3607295 1987
371
Hereditary factor XIII deficiency. 61
3679479 1987
372
A new family with congenital factor XIII deficiency showing a deficit of both subunit A and B. Type I factor XIII deficiency. 61
2891592 1987
373
[Detection of factor XIII deficiency in acute leukemia with resonance thrombography]. 61
2448208 1987
374
Type I and type II disease in congenital factor XIII deficiency. A further demonstration of the correctness of the classification. 61
3779113 1986
375
Factor XIII as a modulator of plasma fibronectin alterations during experimental bacteremia. 61
2878087 1986
376
Arthropathy associated with factor XIII deficiency. 61
3718569 1986
377
Male fertility in factor XIII deficiency. 61
3457710 1986
378
A contribution to the pathology of acquired plasma factor XIII deficiency. 61
4071061 1985
379
[Surgical treatment of intracranial hematoma with congenital factor XIII deficiency. Case report]. 61
2417150 1985
380
Congenital factor XIII deficiency: type I and type II disease. 61
4005184 1985
381
Neonatal factor XIII deficiency. 61
3995866 1985
382
Tooth extraction in two patients who had a congenital deficiency of factor XIII. 61
3156225 1985
383
A case of congenital factor XIII deficiency. 61
2581864 1985
384
On the retraction of collagen and fibrin induced by normal, defective and modified platelets. 61
2993136 1985
385
Defective fibrin crosslinking in acute leukemia. 61
6531757 1984
386
Congenital hemorrhagic disorders in Jordan. 61
6333734 1984
387
Differential binding of plasminogen to crosslinked and noncrosslinked fibrins: its significance in hemostatic defect in factor XIII deficiency. 61
6232970 1984
388
Effect of crosslinking on the structure of solubilized fibrin degradation products in whole plasma. 61
6232329 1984
389
Hereditary factor XIII deficiency: report of four families and definition of the carrier state. 61
6696852 1984
390
A fluorescent spot test for coagulation factor XIII. 61
6731823 1984
391
[Pathogenesis of tracheal stenosis following long-term intubation of patients with multiple injuries]. 61
6654713 1983
392
Acquired factor XIII deficiency with chronic myelomonocytic leukemia. 61
6579877 1983
393
Factor XIII levels in five families of patients with inherited factor XIII deficiency: support for an autosomal recessive inheritance. 61
6636036 1983
394
Determination of factor XIII activity and of factor XIII inhibitors using an ammonium-sensitive electrode. 61
6138878 1983
395
Factor XIII and its deficiency - review literature and the case report of a Thai boy with congenital factor XIII deficiency. 61
6864142 1983
396
Factor XIII deficiency associated with Klippel-Weber disease, platelet dysfunction and cryofibrinogenemia. 61
6305083 1983
397
[Blood clotting factor XIII substitution in acute leukaemia: result of a randomized and controlled study]. 61
6754328 1982
398
A case of factor XIII deficiency in an adult male. 61
7130118 1982
399
[A report of two cases with congenital factor XIII deficiency]. 61
7176097 1982
400
A familial hemorrhagic diathesis in a Dutch family: an inherited deficiency of alpha 2-antiplasmin. 61
6177359 1982
401
[Wound healing disorders and factor XIII deficiency after tumor operations in the floor of the mouth]. 61
6949755 1982
402
Factor XIII deficiency in antibiotic-associated pseudomembranous colitis and its treatment with factor XIII concentrate. 61
6982198 1982
403
Factor XIII deficiency. 61
7337960 1981
404
Congenital factor XIII deficiency. 61
7320002 1981
405
[Acquired factor XIII deficiency and clinical surgery]. 61
7207164 1981
406
[Factor XIII deficiency]. 61
7256966 1981
407
Prophylaxis in factor XIII deficiency. 61
6105415 1980
408
[Clinical laboratory characteristics of the severe form of hereditary fibrin-stabilizing factor (factor XIII) deficiency]. 61
7413627 1980
409
An electrophoretic and quantitative analysis of coagulation factor XIII in normal and deficient subjects. 61
7426441 1980
410
Factor XIII deficiency. 61
6103467 1980
411
Fibrin cross-linking in congenital factor XIII deficiency. 61
7400341 1980
412
[Spontaneous splenic rupture in a patient with congenital factor XIII deficiency]. 61
7389494 1980
413
[Congenital factor XIII deficiency. Report of a new family (author's transl)]. 61
7423340 1980
414
A specific, fluorescent activity staining procedure applied to plasma and red blood cells in congenital factor XIII deficiency. 61
6155132 1980
415
[Studies on congenital factor XIII deficiency and detection of the carrier in his family (author's transl)]. 61
537170 1979
416
Factor XIII. 61
514066 1979
417
An acquired inhibitor to factor XIII A case report. 61
483113 1979
418
Factor XIII deficiency. A family study by measurement of factor XIII subunits A and S. 61
118616 1979
419
[Factor XIII deficiency due to lack of both the S and A subunits. (Classification of factor XIII deficiency in 2 groups)]. 61
395620 1979
420
Congenital factor XIII deficiency in a neonate. 61
728710 1978
421
[Fibrinogen and fibrin structure in patients with cirrhosis of the liver (author's transl)]. 61
706517 1978
422
[Factor XIII deficiency]. 61
691462 1978
423
Subunits A and S inheritance in four families with congenital factor XIII deficiency. 61
638074 1978
424
Studies on factor XIII antigen in congenital factor XIII deficiency. A tentative classification of the disease in two groups. 61
77814 1978
425
[Parodontal alterations caused by a deficiency of fibrin-stabilizing factor (factor XIII deficiency) (author's transl)]. 61
154262 1978
426
[Severe factor XIII-deficiency. Studies on subunits and turnover of the fibrin stabilizing factor (author's transl)]. 61
597618 1977
427
Congenital factor XIII deficiency. 61
610333 1977
428
Factor XIII deficiency in BALB/c mice with plasmacytoma. 61
908006 1977
429
[Factor XIII deficiency in burns]. 61
902534 1977
430
[Factor XIII deficiency in adults with acute leukemia: results of a substitution therapy with factor XIII (author's transl)]. 61
269298 1977
431
[A clinical study of congenital factor XIII deficiency (author's transl)]. 61
904084 1977
432
A tentative classification of factor XIII deficiency in two groups. 61
413309 1977
433
Congenital deficiency of factor XIII with normal subunit S and lack of subunit A. Report of a new family. 61
410213 1977
434
[Longtime therapy of congenital factor XIII deficiency using factor XIII concentrate]. 61
1000087 1976
435
Bleeding disorder with abnormal wound healing, acid-soluble clots and normal factor XIII. 61
1037151 1976
436
Glanzmann's thrombasthenia in a Melanesian. 61
1072174 1976
437
[A case of congenital factor XIII deficiency and acquired factor XIII deficiency (author's transl)]. 61
945384 1976
438
[Studies on abnormal protein in seven patients with congenital factor XIII deficiency (author's transl)]. 61
945383 1976
439
Factor XIII deficiency: report of a case complicated by splenic rupture. 61
1272449 1976
440
Platelet aggregation in congenital factor XIII deficiency. 61
826089 1976
441
[Blood coagulation factor XIII and fibrin stabilization (author's transl)]. 61
765611 1975
442
Rheological properties of fibrin clots. Effects of fibrinogen concentration, Factor XIII deficiency, and Factor XIII inhibition. 61
1176815 1975
443
Proceedings: Factor XIII concentrate in the long term management of congenital factor XIII deficiency. 61
810913 1975
444
Factor XIII deficiency in two Melanesian families from Papua New Guinea. 61
1058680 1975
445
[Importance, diagnosis and substitution in acquired factor XIII deficiency in the postoperative course]. 61
50985 1975
446
[Acquired factor XIII deficiency and postoperative aseptic wound healing disorders]. 61
1217233 1975
447
Factor XIII deficiency: a genetic study of two affected kindreds in Finland. 61
4811819 1974
448
Immunological studies of coagulation factor XIII. 61
4199604 1973
449
[Simplified radiologic factor XIII determination and its clinical use in congenital factor XIII deficiency (I)]. 61
4718816 1973
450
[Congenital factor XIII deficiency]. 61
4708547 1973
451
Documentation of the plasma factor XIII deficiency in man. 61
4508922 1972
452
[Substitution treatment of factor XIII deficiency with a new factor XIII concentrate]. 61
5011493 1972
453
[Congenital factor XIII deficiency: studies in an infant and his family]. 61
5016722 1972
454
[Case of factor XIII deficiency]. 61
5130081 1971
455
Factor XIII deficiency. A rare haemorrhagic disease. 61
5288877 1971
456
Genetic aspects of factor XIII deficiency. 61
5159534 1971
457
Blood factor XIII deficiency: review of literature and report of case. 61
4935503 1971
458
A clinical and family study of factor XIII deficiency in a New Zealand family. 61
5284554 1971
459
Epsilon-(gamma glutamyl) lysine in fibrin: lack of crosslink formation in Factor 13 deficiency. 61
5279517 1971
460
[Plasma transglutaminase activity in congenital total and partial factor XIII deficiency (a contribution to the problem of factor XIII determination)]. 61
5577969 1971
461
[Factor XIII deficiency in various patients with acute leukemia]. 61
5155938 1971
462
[Presence of a plasma antigen indispensable to the stabilization of fibrin in 2 cases on congenital factor XIII deficiency]. 61
5511868 1970
463
[Case suspected to be factor XIII deficiency]. 61
5535825 1970
464
[Case of factor XIII deficiency]. 61
5535826 1970
465
Congenital factor XIII deficiency and increased fibrinolysis. A case report. 61
5431447 1970
466
[2 patients with congenital factor XIII deficiency. Contribution to the problem of factor XIII determination]. 61
5426213 1970
467
Congenital factor XIII deficiency. Report of 2 cases. 61
5271545 1969
468
Factor XIII deficiency. Treatment with monthly plasma infusions. 61
5819238 1969
469
Ocular complications of factor XIII deficiency. 61
5774246 1969
470
Ocular complications of factor XIII deficiency. 61
5312901 1969
471
[Spontaneous intracerebral hematoma in a familial congenital coagulation disorder (factor XIII deficiency)]. 61
5406277 1969
472
Treatment of factor XIII deficiency with cryoprecipitate. 61
5710469 1968
473
[Postoperative complications due to acquired factor XIII deficiency]. 61
4173875 1967

Variations for Factor Xiii Deficiency

ClinVar genetic disease variations for Factor Xiii Deficiency:

6 (showing 1, show less) ‎‎
# Gene Name Type Significance ClinVarId dbSNP ID GRCh37 Pos GRCh38 Pos
1 F13A1 NM_000129.3(F13A1):c.563G>T (p.Trp188Leu)SNV Pathogenic 634901 rs1561673120 6:6266799-6266799 6:6266566-6266566

Expression for Factor Xiii Deficiency

Search GEO for disease gene expression data for Factor Xiii Deficiency.

Pathways for Factor Xiii Deficiency

Pathways related to Factor Xiii Deficiency according to KEGG:

36 (showing 1, show less)
# Name Kegg Source Accession
1 Complement and coagulation cascades hsa04610

Pathways related to Factor Xiii Deficiency according to GeneCards Suite gene sharing:

(showing 7, show less)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.01 VWF SERPINF2 SERPINE1 SERPINC1 F9 F8
2
Show member pathways
12.68 SERPINC1 F9 F8 F7 F5 F3
3
Show member pathways
11.94 VWF SERPINF2 SERPINE1 SERPINC1 F9 F8
4
Show member pathways
11.61 F9 F7 F2
5 11.6 VWF SERPINF2 SERPINE1 SERPINC1 F9 F8
6 10.67 F9 F7 F2
7 10.48 SERPINF2 SERPINE1

GO Terms for Factor Xiii Deficiency

Cellular components related to Factor Xiii Deficiency according to GeneCards Suite gene sharing:

(showing 11, show less)
# Name GO ID Score Top Affiliating Genes
1 extracellular exosome GO:0070062 10.04 VWF SERPINF2 SERPINE1 SERPINC1 F9 F2
2 extracellular region GO:0005576 9.97 VWF SERPINF2 SERPINE1 SERPINC1 F9 F8
3 extracellular space GO:0005615 9.96 SERPINF2 SERPINE1 SERPINC1 F9 F8 F7
4 endoplasmic reticulum lumen GO:0005788 9.8 SERPINC1 F9 F8 F7 F5 F2
5 blood microparticle GO:0072562 9.71 SERPINF2 SERPINC1 F2 F13A1
6 Golgi lumen GO:0005796 9.63 F9 F7 F2
7 endoplasmic reticulum-Golgi intermediate compartment membrane GO:0033116 9.61 MCFD2 F8 F5
8 platelet alpha granule GO:0031091 9.48 VWF F5
9 platelet alpha granule lumen GO:0031093 9.43 VWF SERPINF2 SERPINE1 F8 F5 F13A1
10 serine-type peptidase complex GO:1905286 9.37 F7 F3
11 collagen-containing extracellular matrix GO:0062023 9.28 VWF SERPINF2 SERPINE1 SERPINC1 F9 F7

Biological processes related to Factor Xiii Deficiency according to GeneCards Suite gene sharing:

(showing 19, show less)
# Name GO ID Score Top Affiliating Genes
1 ER to Golgi vesicle-mediated transport GO:0006888 9.88 MCFD2 F9 F8 F7 F5 F2
2 negative regulation of endopeptidase activity GO:0010951 9.75 SERPINF2 SERPINE1 SERPINC1
3 negative regulation of peptidase activity GO:0010466 9.74 SERPINF2 SERPINE1 SERPINC1
4 platelet activation GO:0030168 9.73 VWF F8 F2
5 platelet degranulation GO:0002576 9.73 VWF SERPINF2 SERPINE1 F8 F5 F13A1
6 COPII vesicle coating GO:0048208 9.7 MCFD2 F8 F5
7 hemostasis GO:0007599 9.7 VWF SERPINC1 F9 F8 F7 F5
8 fibrinolysis GO:0042730 9.67 SERPINF2 SERPINE1 F2 CPB2
9 acute-phase response GO:0006953 9.65 SERPINF2 F8 F2
10 positive regulation of blood coagulation GO:0030194 9.63 SERPINE1 F7 F2
11 blood coagulation, intrinsic pathway GO:0007597 9.62 VWF F9 F8 F2
12 negative regulation of plasminogen activation GO:0010757 9.61 SERPINF2 SERPINE1 CPB2
13 positive regulation of collagen biosynthetic process GO:0032967 9.58 SERPINF2 F2
14 regulation of blood coagulation GO:0030193 9.55 SERPINC1 F2
15 positive regulation of positive chemotaxis GO:0050927 9.54 F7 F3
16 positive regulation of platelet-derived growth factor receptor signaling pathway GO:0010641 9.51 F7 F3
17 blood coagulation, extrinsic pathway GO:0007598 9.49 F7 F3
18 negative regulation of fibrinolysis GO:0051918 9.46 SERPINF2 SERPINE1 F2 CPB2
19 blood coagulation GO:0007596 9.36 VWF SERPINC1 F9 F8 F7 F5

Molecular functions related to Factor Xiii Deficiency according to GeneCards Suite gene sharing:

(showing 5, show less)
# Name GO ID Score Top Affiliating Genes
1 serine-type peptidase activity GO:0008236 9.5 F9 F7 F2
2 peptidase inhibitor activity GO:0030414 9.43 SERPINF2 SERPINE1 SERPINC1
3 serine-type endopeptidase inhibitor activity GO:0004867 9.33 SERPINF2 SERPINE1 SERPINC1
4 serine-type endopeptidase activity GO:0004252 9.26 F9 F7 F3 F2
5 protease binding GO:0002020 9.02 VWF SERPINF2 SERPINE1 SERPINC1 F3

Sources for Factor Xiii Deficiency

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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