MCID: SCK005
MIFTS: 54

Sickle Cell Disease

Categories: Immune diseases, Blood diseases

Aliases & Classifications for Sickle Cell Disease

MalaCards integrated aliases for Sickle Cell Disease:

Name: Sickle Cell Disease 24 25 55 43 3
Sickling Disorder Due to Hemoglobin S 25
Sickle Cell Disorders 25
Hemoglobin S Disease 25
Anemia, Sickle Cell 73
Scd 25

Classifications:



External Ids:

ICD10 33 D57

Summaries for Sickle Cell Disease

MedlinePlus : 43 What is sickle cell disease? Sickle cell disease (SCD) is a group of inherited red blood cell disorders. If you have SCD, there is a problem with your hemoglobin. Hemoglobin is a protein in red blood cells that carries oxygen throughout the body. With SCD, the hemoglobin forms into stiff rods within the red blood cells. This changes the shape of the red blood cells. The cells are supposed to be disc-shaped, but this changes them into a crescent, or sickle, shape. The sickle-shaped cells are not flexible and cannot change shape easily. Many of them burst apart as they move through your blood vessels. The sickle cells usually only last 10 to 20 days, instead of the normal 90 to 120 days. Your body may have trouble making enough new cells to replace the ones that you lost. Because of this, you may not have enough red blood cells. This is a condition called anemia, and it can make you feel tired. The sickle-shaped cells can also stick to vessel walls, causing a blockage that slows or stops the flow of blood. When this happens, oxygen can't reach nearby tissues. The lack of oxygen can cause attacks of sudden, severe pain, called pain crises. These attacks can occur without warning. If you get one, you might need to go to the hospital for treatment. What causes sickle cell disease? The cause of SCD is a defective gene, called a sickle cell gene. People with the disease are born with two sickle cell genes, one from each parent. If you are born with one sickle cell gene, it's called sickle cell trait. People with sickle cell trait are generally healthy, but they can pass the defective gene on to their children. Who gets sickle cell disease? In the United States, most of the people with SCD are African Americans: About 1 in 13 African American babies is born with sickle cell trait About 1 in every 365 black children is born with sickle cell disease SCD also affects some people who come from Hispanic, southern European, Middle Eastern, or Asian Indian backgrounds. What are the symptoms of sickle cell disease? People with SCD start to have signs of the disease during the first year of life, usually around 5 months of age. Early symptoms of SCD may include Painful swelling of the hands and feet Fatigue or fussiness from anemia A yellowish color of the skin (jaundice) or the whites of the eyes (icterus) The effects of SCD vary from person to person and can change over time. Most of the signs and symptoms of SCD are related to complications of the disease. They may include severe pain, anemia, organ damage, and infections. How is sickle cell disease diagnosed? A blood test can show if you have SCD or sickle cell trait. All states now test newborns as part of their screening programs, so treatment can begin early. People who are thinking about having children can have the test to find out how likely it is that their children will have SCD. Doctors can also diagnose SCD before a baby is born. That test uses a sample of amniotic fluid (the liquid in the sac surrounding the baby) or tissue taken from the placenta (the organ that brings oxygen and nutrients to the baby). What are the treatments for sickle cell disease? The only cure for SCD is bone marrow or stem cell transplantation. Because these transplants are risky and can have serious side effects, they are usually only used in children with severe SCD. For the transplant to work, the bone marrow must be a close match. Usually, the best donor is a brother or sister. There are treatments that can help relieve symptoms, lessen complications, and prolong life: Antibiotics to try to prevent infections in younger children Pain relievers for acute or chronic pain Hydroxyurea, a medicine that has been shown to reduce or prevent several SCD complications. It increases the amount of fetal hemoglobin in the blood. This medicine is not right for everyone; talk to your health care provider about whether you should take it. This medicine is not safe during pregnancy. Childhood immunizations to prevent infections Blood transfusions for severe anemia. If you have had some serious complications, such as a stroke, you may have transfusions to prevent more complications. There are other treatments for specific complications. To stay as healthy as possible, make sure that you get regular medical care, live a healthy lifestyle, and avoid situations that may set off a pain crisis. NIH: National Heart, Lung, and Blood Institute

MalaCards based summary : Sickle Cell Disease, also known as sickling disorder due to hemoglobin s, is related to hereditary persistence of fetal hemoglobin-sickle cell disease syndrome and acute chest syndrome, and has symptoms including abdominal pain, angina pectoris and chest pain. An important gene associated with Sickle Cell Disease is MIR144 (MicroRNA 144
It was demonstrated that increased miR-144 is associated with reduced NRF2 levels in homozygous sickle cell disease (HbSS) reticulocytes and with decreased glutathione regeneration and attenuated antioxidant capacity in HbSS erythrocytes, thereby providing a possible mechanism for the reduced oxidative stress tolerance and increased anemia severity seen in HbSS patients. Taken together, the findings suggest that erythroid microRNAs can serve as genetic modifiers of HbS-related anemia and can provide novel insights into the clinical heterogeneity and pathobiology of sickle cell disease. ), and among its related pathways/superpathways are Factors involved in megakaryocyte development and platelet production and O2/CO2 exchange in erythrocytes. The drugs Ribavirin and Hydroxyurea have been mentioned in the context of this disorder. Affiliated tissues include bone, bone marrow and lung, and related phenotypes are Increased shRNA abundance and cardiovascular system

Genetics Home Reference : 25 Sickle cell disease is a group of disorders that affects hemoglobin, the molecule in red blood cells that delivers oxygen to cells throughout the body. People with this disorder have atypical hemoglobin molecules called hemoglobin S, which can distort red blood cells into a sickle, or crescent, shape.

Wikipedia : 76 Sickle cell disease (SCD) is a group of blood disorders typically inherited from a person\'s parents.... more...

GeneReviews: NBK1377

Related Diseases for Sickle Cell Disease

Diseases related to Sickle Cell Disease via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 255)
# Related Disease Score Top Affiliating Genes
1 hereditary persistence of fetal hemoglobin-sickle cell disease syndrome 33.0 BCL11A HBB HBG1 HBG2
2 acute chest syndrome 32.7 HBB VCAM1
3 splenic sequestration 31.7 BCAM HBB
4 hemoglobinopathy 31.0 EPO HBA2 HBB HBD HBG1
5 sickle cell anemia 30.5 BCL11A EPO HBA2 HBB HBG1 HBG2
6 malaria 30.4 EPO G6PD HBA2 HBB SELP VCAM1
7 hemoglobin c disease 30.2 HBB HBD
8 hereditary spherocytosis 30.2 G6PD HBB HBG1
9 glucosephosphate dehydrogenase deficiency 30.0 G6PD HBB HBG2
10 arteries, anomalies of 29.4 PGF SELP VCAM1
11 deficiency anemia 28.6 EPO G6PD HBA2 HBB HBG2
12 hemolytic anemia 28.3 EPO G6PD HBA2 HBB HBG2
13 alpha-thalassemia 27.8 EPO G6PD HBA2 HBB HBG1 HBG2
14 thalassemia 27.3 BCL11A EPO HBA2 HBB HBD HBG1
15 beta-thalassemia 26.7 BCL11A EPO G6PD HBA2 HBB HBD
16 sickle cell disease associated with an other hemoglobin anomaly 12.0
17 schnyder corneal dystrophy 11.5
18 superior semicircular canal dehiscence 11.5
19 fatty liver disease 11.5
20 sickle beta thalassemia 11.4
21 cardiac conduction defect 11.3
22 fetal hemoglobin quantitative trait locus 5 10.9
23 endosteal hyperostosis, autosomal dominant 10.9
24 osteomesopyknosis 10.9
25 moyamoya disease 1 10.9
26 hemoglobin zurich 10.6 HBA2 HBB
27 methemoglobinemia, beta-globin type 10.6 HBA2 HBB
28 splenic disease 10.6 EPO HBB
29 heinz body anemias 10.5 HBA2 HBB
30 hypochromic microcytic anemia 10.5 HBA2 HBB
31 acute erythroid leukemia 10.5 EPO HBB HBG1
32 neonatal anemia 10.5 EPO HBA2
33 priapism 10.4
34 hemoglobin lepore-beta-thalassemia syndrome 10.4 HBB HBD
35 hemoglobin d disease 10.4 HBB HBD
36 acquired methemoglobinemia 10.3 G6PD HBG2
37 congenital methemoglobinemia 10.3 G6PD HBG2
38 retinal vascular occlusion 10.3 PGF SELP
39 malignant secondary hypertension 10.3 HBB HBD
40 immune-complex glomerulonephritis 10.3 SELP VCAM1
41 pulmonary hypertension 10.3
42 glutathione peroxidase deficiency 10.3 HBA2 HBB SELP
43 osgood-schlatter's disease 10.3 HBB HBD
44 aging 10.3
45 cerebritis 10.3
46 mild pre-eclampsia 10.3 PGF VCAM1
47 kluver-bucy syndrome 10.2 HBB HBD
48 endotheliitis 10.2
49 hereditary persistence of fetal hemoglobin-beta-thalassemia syndrome 10.2 HBB HBG1 HBG2
50 hemosiderosis 10.2

Graphical network of the top 20 diseases related to Sickle Cell Disease:



Diseases related to Sickle Cell Disease

Symptoms & Phenotypes for Sickle Cell Disease

UMLS symptoms related to Sickle Cell Disease:


abdominal pain, angina pectoris, chest pain, edema

GenomeRNAi Phenotypes related to Sickle Cell Disease according to GeneCards Suite gene sharing:

26
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Increased shRNA abundance GR00327-A 8.92 EPO G6PD HBD HBG2

MGI Mouse Phenotypes related to Sickle Cell Disease:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 cardiovascular system MP:0005385 9.23 BCAM EPO G6PD KCNN4 PGF SELP

Drugs & Therapeutics for Sickle Cell Disease

Drugs for Sickle Cell Disease (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 388)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Ribavirin Approved Phase 4 36791-04-5 37542
2
Hydroxyurea Approved Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable,Early Phase 1 127-07-1 3657
3
Deferiprone Approved Phase 4,Phase 2,Phase 3,Phase 1 30652-11-0 2972
4
Iron Approved Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable 7439-89-6 23925
5
Ketamine Approved, Vet_approved Phase 4,Phase 3,Phase 2,Not Applicable 6740-88-1 3821
6
Proguanil Approved Phase 4,Phase 1,Phase 2,Not Applicable 500-92-5 4923
7
Morphine Approved, Investigational Phase 4,Phase 3,Phase 1,Phase 2,Not Applicable 57-27-2 5288826
8
Deferoxamine Approved, Investigational Phase 4,Phase 2,Phase 3 70-51-9 2973
9
Deferasirox Approved, Investigational Phase 4,Phase 2,Phase 3 201530-41-8 5493381
10
Peginterferon alfa-2a Approved, Investigational Phase 4 198153-51-4 5360545
11
Peginterferon alfa-2b Approved Phase 4 99210-65-8, 215647-85-1
12
Guaifenesin Approved, Investigational, Vet_approved Phase 4,Phase 1,Phase 2,Not Applicable 93-14-1 3516
13
Sulfadoxine Approved, Investigational Phase 4,Phase 3,Phase 1,Phase 2 2447-57-6 17134
14
Pyrimethamine Approved, Investigational, Vet_approved Phase 4,Phase 3,Phase 1,Phase 2 58-14-0 4993
15
Hydromorphone Approved, Illicit Phase 4,Phase 3,Phase 2 466-99-9 5284570
16
Codeine Approved, Illicit Phase 4,Phase 1,Phase 2 76-57-3 5284371
17
Amodiaquine Approved, Investigational Phase 4,Phase 1,Phase 2 86-42-0 2165
18
Fentanyl Approved, Illicit, Investigational, Vet_approved Phase 4,Phase 2 437-38-7 3345
19
Ibuprofen Approved Phase 4,Phase 2,Not Applicable 15687-27-1 3672
20
Dihydroartemisinin Approved, Investigational Phase 4 71939-50-9 6918483
21
Piperaquine Approved, Investigational Phase 4 4085-31-8 5079497
22
Desogestrel Approved Phase 4 54024-22-5 40973
23
Etonogestrel Approved, Investigational Phase 4 54048-10-1 40976 6917715
24
Ketorolac Approved Phase 4 74103-06-3, 66635-83-4 3826
25
Heroin Approved, Illicit, Investigational Phase 4 561-27-3 5462328
26
Vitamin D Approved, Nutraceutical, Vet_approved Phase 4,Phase 3,Phase 2,Phase 1 1406-16-2
27
Vitamin D3 Approved, Nutraceutical Phase 4,Phase 3,Phase 2,Phase 1 67-97-0 6221 5280795
28
Ergocalciferol Approved, Nutraceutical Phase 4,Phase 3,Phase 2,Phase 1 50-14-6 5280793
29
Citric Acid Approved, Nutraceutical, Vet_approved Phase 4,Phase 2,Phase 1,Not Applicable,Early Phase 1 77-92-9 311
30 Analgesics Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable
31 interferons Phase 4
32 Respiratory System Agents Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable
33 Central Nervous System Depressants Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable
34 Micronutrients Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable
35 Chelating Agents Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable
36 Chlorpheniramine, phenylpropanolamine drug combination Phase 4,Phase 1,Phase 2,Not Applicable
37 Narcotics Phase 4,Phase 3,Phase 1,Phase 2,Not Applicable
38 Neurotransmitter Agents Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable
39 Analgesics, Non-Narcotic Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable
40 Analgesics, Opioid Phase 4,Phase 3,Phase 1,Phase 2,Not Applicable
41 Anesthetics Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable
42 Anesthetics, Dissociative Phase 4,Phase 3,Phase 2,Not Applicable
43 Anesthetics, General Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable
44 Trace Elements Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable
45 Anesthetics, Intravenous Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable
46 Nucleic Acid Synthesis Inhibitors Phase 4,Phase 3,Phase 2,Phase 1,Early Phase 1,Not Applicable
47 Vaccines Phase 4,Phase 3,Phase 2,Not Applicable,Early Phase 1
48 Peripheral Nervous System Agents Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable
49 Ergocalciferols Phase 4,Phase 3,Phase 2,Phase 1
50 Vitamins Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable

Interventional clinical trials:

(show top 50) (show all 641)
# Name Status NCT ID Phase Drugs
1 Interferon and Ribavirin Treatment in Patients With Hemoglobinopathies Unknown status NCT00887081 Phase 4 PEG-IFN alpha2a or PEG-IFN alpha2b and Ribavirin
2 Comparing Acute Pain Management Protocols for Patients With Sickle Cell Disease Completed NCT02222246 Phase 4 Hydromorphone (Standardized, weight-based dosing);Morphine Sulfate (Standardized, weight-based dosing);Hydromorphone (Patient Specific dosing);Morphine Sulfate (Patient Specific dosing)
3 Future of Spermatogenesis in Men With Sickle Cell Disease Medically Treated Completed NCT01609192 Phase 4 Hydrea® (hydroxyurea )
4 Rejuvesol® Washed RBC in Sickle Cell Patients Requiring Frequent Transfusions Completed NCT02731157 Phase 4 Rejuvesol
5 Evaluation of the Impact of Renal Function on the Pharmacokinetics of SIKLOS ® (DARH) Completed NCT02522104 Phase 4 Siklos
6 Intranasal Fentanyl for Initial Treatment of a Vaso-occlusive Crisis Completed NCT01482091 Phase 4 Fentanyl Citrate;Normal Saline
7 Pilot Study for Patients With Poor Response to Deferasirox Completed NCT00749515 Phase 4 Deferoxamine;Deferasirox
8 Efficacy and Safety of Ferriprox® in Patients With Sickle Cell Disease or Other Anemias Recruiting NCT02041299 Phase 4 Deferiprone;Deferoxamine
9 Enhancing Preventive Therapy of Malaria In Children With Sickle Cell Anemia in East Africa (EPiTOMISE) Recruiting NCT03178643 Phase 4 Proguanil Oral Tablet;Sulfadoxine/Pyrimethanine-Amodiaquine (SP-AQ);Dihydroartemisinin-Piperaquine (DP)
10 Contraception in Women With Sickle Cell Disease Active, not recruiting NCT02594462 Phase 4 etonogestrel-releasing implant contraceptive
11 Risk Clinical Stratification of Sickle Cell Disease in Nigeria, Assessment of Efficacy/Safety of Hydroxyurea Treatment Active, not recruiting NCT02149537 Phase 4 hydroxyurea
12 Long-term Safety and Efficacy of Ferriprox® in Iron Overloaded Patients With Sickle Cell Disease or Other Anemias Enrolling by invitation NCT02443545 Phase 4 Deferiprone
13 Mobile-Directly Observed Therapy on Adherence to Hydroxyurea Enrolling by invitation NCT02844673 Phase 4 Hydroxyurea
14 Does IV Acetaminophen Reduce Opioid Requirement in Pediatric Emergency Department Patients With Acute Sickle Cell Crises? Not yet recruiting NCT03541980 Phase 4 Acetaminophen;Normal saline
15 A Randomized Phase IV Control Trial of Single High Dose Oral Vitamin D3 in Pediatric Patients Undergoing HSCT Not yet recruiting NCT03176849 Phase 4
16 Ibuprofen and Opioid (Morphine or Diamorphine) for Acute Pain in Sickle Cell Disease - Sickle With Ibuprofen & Morphine Terminated NCT00880373 Phase 4 Ibuprofen;Placebo;Diamorphine or Morphine
17 Pilot Study on the Effects of Intravenous Ketamine on Acute Pain Crisis in Patients With Sickle Cell Disease Terminated NCT00252122 Phase 4 Ketamine
18 Ketorolac Versus Ibuprofen to Treat Painful Episodes of Sickle Cell Disease Terminated NCT00115336 Phase 4 Intravenous Ketorolac;Ibuprofen
19 Immunogenicity and Safety Study of Pneumococcal 7-Valent Conjugate Vaccine in Sickle Cell Disease Infants. Terminated NCT00368186 Phase 4
20 Assessment of Opioid Analgesia in Sickle Cell Withdrawn NCT00513864 Phase 4 Dextromethorphan;Codeine;Morphine
21 Endothelial Function in Patients With Sickle Cell Anemia Before and After Sildenafil Withdrawn NCT00937144 Phase 4 Viagra (Sildenafil);placebo
22 Transfusion Alternatives Pre-Operatively in Sickle Cell Disease (TAPS) Unknown status NCT00512577 Phase 3
23 Ketamine as an Adjuvant Therapy for Acute Vaso Occlusive Crisis in Pediatric Patients With Sickle Cell Disease Unknown status NCT02801292 Phase 3 Ketamine
24 Vascular Function Intervention Trial in Sickle Cell Disease Unknown status NCT01718054 Phase 2, Phase 3 Chloroquine
25 Study Of Efficacy,Safety of Combined Deferasirox and Deferiprone Versus Combined Deferiprone and Desferal In Conditions of Iron Overload Unknown status NCT01511848 Phase 2, Phase 3 DFP (ferriprox) and deferasirox (ICL 670);DFP, DFO
26 Evaluation of the Lung Capillary Blood Volume in Children With Sickle Cell Disease Completed NCT00560261 Phase 3
27 Evaluation of Purified Poloxamer 188 in Vaso-Occlusive Crisis of Sickle Cell Disease (EPIC) Completed NCT01737814 Phase 3 Saline;MST-188
28 L-Arginine and Sickle Cell Disease Completed NCT01142219 Phase 3 L-arginine;Placebo
29 N-Acetylcysteine in Patients With Sickle Cell Disease Completed NCT01849016 Phase 3 N-Acetylcysteine;Placebo
30 Hypnosis to Manage Pain and Symptoms in Patients With Sickle Cell Disease Completed NCT00393250 Phase 3
31 A Phase III Safety and Efficacy Study of L-Glutamine to Treat Sickle Cell Disease or Sickle βo-thalassemia Completed NCT01179217 Phase 3 L-glutamine;Placebo
32 Apixaban in Patients With Sickle Cell Disease Completed NCT02179177 Phase 3 Apixaban;Placebo
33 Steroid Treatment for Sickle Cell Pain Crisis Completed NCT00263562 Phase 3 Methylprednisolone plus prednisone taper
34 Evaluation of Repeat Administration of Purified Poloxamer 188 Completed NCT02449616 Phase 3 MST-188
35 Study Evaluating 13-valent Pneumococcal Conjugate Vaccine (13vPnC) in Children With Sickle Cell Disease Completed NCT00918580 Phase 3
36 Sulfadoxine- Pyrimethamine Versus Weekly Chloroquine for Malaria Prevention in Children With Sickle Cell Anemia Completed NCT00399074 Phase 3 sulfadoxine pyrimethamine
37 Phase III Randomized Study of Poloxamer 188 for Vaso-Occlusive Crisis of Sickle Cell Disease Completed NCT00004408 Phase 3 poloxamer 188
38 Ameliorating Attention Problems in Children With Sickle Cell Disease (SCD) Completed NCT01411280 Phase 3 methylphenidate
39 Multicenter Study of Hydroxyurea in Patients With Sickle Cell Anemia (MSH) Completed NCT00000586 Phase 3 hydroxyurea
40 Intravenous Magnesium for Sickle Cell Vasoocclusive Crisis Completed NCT01197417 Phase 2, Phase 3 Intravenous Magnesium Sulfate;Normal Saline Placebo
41 Hydroxyurea to Prevent Organ Damage in Children With Sickle Cell Anemia Completed NCT00006400 Phase 3 Hydroxyurea;Placebo
42 Penicillin Prophylaxis in Sickle Cell Disease (PROPS) Completed NCT00000585 Phase 3 penicillin
43 Effect of Inhaled Nitric Oxide in Acute Chest Syndrome (INOSTA Study) Completed NCT00748423 Phase 2, Phase 3 Nitric Oxide;Placebo
44 Choosing Opioid Management for Pain and Analyzing Acute Chest Syndrome (ACS) Rates Equally Completed NCT01380197 Phase 3 Morphine;Nubain
45 Silent Cerebral Infarct Transfusion Multi-Center Clinical Trial Completed NCT00072761 Phase 3
46 Novel Use Of Hydroxyurea in an African Region With Malaria Completed NCT01976416 Phase 3 Hydroxyurea;Placebo
47 Stroke Prevention in Sickle Cell Anemia (STOP 1) Completed NCT00000592 Phase 3
48 Stroke Prevention in Sickle Cell Anemia (STOP 2) Completed NCT00006182 Phase 3
49 MAST - Magnesium for Sickle Cell Acute Crisis in Children Completed NCT00313963 Phase 3 Magnesium Sulfate;Normal Saline
50 Mobilization and Handling of Stem Cells for Transplant From Healthy Volunteers With Sickle Cell Trait Completed NCT00005782 Phase 3 Granulocyte colony-stimulating factor

Search NIH Clinical Center for Sickle Cell Disease

Inferred drug relations via UMLS 73 / NDF-RT 51 :


Genetic Tests for Sickle Cell Disease

Anatomical Context for Sickle Cell Disease

MalaCards organs/tissues related to Sickle Cell Disease:

41
Bone, Bone Marrow, Lung, Heart, Kidney, Endothelial, Testes

Publications for Sickle Cell Disease

Articles related to Sickle Cell Disease:

(show top 50) (show all 1912)
# Title Authors Year
1
Bacteremia and Septic Arthritis due to a Nontoxigenic Strain of<i>Clostridium difficile</i>in a Patient With Sickle Cell Disease. ( 29410974 )
2018
2
Presence of pain on three or more days of the week is associated with worse patient reported outcomes in adults with sickle cell disease. ( 29445298 )
2018
3
Nonaneurysmal Subarachnoid Hemorrhage in Sickle Cell Disease: Description of a Case and a Review of the Literature. ( 29953035 )
2018
4
Ischemic Colitis in Sickle Cell Disease: A Case Report of a Diagnostic Challenge. ( 29623225 )
2018
5
Frequency of Red Blood Cell Alloimmunization in Patients with Sickle Cell Disease in Palestine. ( 29977298 )
2018
6
Clinical trial conduct in special populations and developing regions: An overview of the DOVE study in pediatric patients with sickle cell disease. ( 29104118 )
2018
7
Response to Long-term Vitamin D Therapy for Bone Disease in Children With Sickle Cell Disease. ( 29668535 )
2018
8
Airway inflammation in sickle cell disease-A translational perspective. ( 29314737 )
2018
9
When there is no match, the game is not over: Alternative donor options for hematopoietic stem cell transplantation in sickle cell disease. ( 29958565 )
2018
10
Early Noninvasive Ventilation and Nonroutine Transfusion for Acute Chest Syndrome in Sickle Cell Disease in Children: A Descriptive Study. ( 29356722 )
2018
11
Priapism in sickle cell disease: Beware of neuroleptics. ( 29885077 )
2018
12
Prevalence and Characteristics of Priapism in Sickle Cell Disease. ( 29745276 )
2018
13
Preconception risk assessment for thalassaemia, sickle cell disease, cystic fibrosis and Tay-Sachs disease. ( 29537064 )
2018
14
A common molecular signature of patients with sickle cell disease revealed by microarray meta-analysis and a genome-wide association study. ( 29979707 )
2018
15
Safety and efficacy of plerixafor dose escalation for the mobilization of CD34+ hematopoietic progenitor cells in patients with sickle cell disease: interim results. ( 29419425 )
2018
16
Prevalence and risk factors for venous thromboembolism in children with sickle cell disease: an administrative database study. ( 29431623 )
2018
17
Serum of sickle cell disease patients contains fetal hemoglobin silencing factors secreted from leukocytes. ( 29950916 )
2018
18
Early initiation of inhaled corticosteroids does not decrease acute chest syndrome morbidity in pediatric patients with sickle cell disease. ( 29550053 )
2018
19
Imaging for Pulmonary Embolism in Sickle Cell Disease: a 17-Year Experience. ( 29419477 )
2018
20
<i>g(HbF)</i> : a genetic model of fetal hemoglobin in sickle cell disease. ( 29437638 )
2018
21
High bone mineral density in sickle cell disease: Prevalence and characteristics. ( 29428552 )
2018
22
Selective Difficulties in Lexical Retrieval and Nonverbal Executive Functioning in Children With HbSS Sickle Cell Disease. ( 29432593 )
2018
23
Phytomedicines (medicines derived from plants) for sickle cell disease. ( 29446825 )
2018
24
More Than Meets the Eye: Cerebrovascular Disease in Sickle Cell Disease Is About More Than Sickling. ( 29695469 )
2018
25
Sickle cell disease is associated with higher mortality among patients hospitalized with ischemic bowel disease. ( 29957616 )
2018
26
From trust to skepticism: An in-depth analysis across age groups of adults with sickle cell disease on their perspectives regarding hydroxyurea. ( 29949647 )
2018
27
Lower than expected elevated tricuspid regurgitant jet velocity in adults with sickle cell disease in Nigeria. ( 29438485 )
2018
28
Variability of homozygous sickle cell disease: The role of alpha and beta globin chain variation and other factors. ( 28689691 )
2018
29
Adenotonsillectomy in children with sickle cell disease and obstructive sleep apnea. ( 29958601 )
2018
30
Comorbid obstructive sleep apnea and increased risk for sickle cell disease morbidity. ( 29450676 )
2018
31
Elevated Proangiogenic Markers are Associated with Vascular Complications within Ghanaian Sickle Cell Disease Patients. ( 29954157 )
2018
32
Genetic therapies for sickle cell disease. ( 29958563 )
2018
33
Malaria, sickle cell disease, HIV and co-trimoxazole prophylaxis: an observational study. ( 29408573 )
2018
34
Comprehensive management reduces incidence and mortality of acute chest syndrome in patients with sickle cell disease. ( 29205462 )
2018
35
Factors Associated With Mechanical Ventilation Use in Children With Sickle Cell Disease and Acute Chest Syndrome. ( 29923940 )
2018
36
Children with sickle cell disease migrating to the United States from sub-Saharan Africa. ( 29418070 )
2018
37
Improving disease knowledge in 6- to 10-year-olds with sickle cell disease: A quasi-experimental study. ( 29436011 )
2018
38
Sickle Cell Disease Complicated by Iron Overload: An Under-Recognized Risk Factor for Vibrio vulnificus Infection. ( 29734168 )
2018
39
Sickle cell disease-Unanswered questions and future directions in therapy. ( 29958561 )
2018
40
Specifying sickle cell disease interventions: a study protocol of the Sickle Cell Disease Implementation Consortium (SCDIC). ( 29945631 )
2018
41
Effective screening leads to better outcomes in sickle cell disease. ( 29444869 )
2018
42
No risk reduction for <i>Plasmodium vivax</i> malaria in sickle cell disease. ( 29881595 )
2018
43
The pain of children with sickle cell disease: the nursing approach. ( 29972538 )
2018
44
A phase 1 dose-finding study of intravenous L-citrulline in sickle cell disease: a potential novel therapy for sickle cell pain crisis. ( 29411862 )
2018
45
Intracranial 4D flow magnetic resonance imaging reveals altered haemodynamics in sickle cell disease. ( 29270975 )
2018
46
Sickle SCANa8c (BioMedomics) fulfills analytical conditions for neonatal screening of sickle cell disease. ( 29976532 )
2018
47
Sickle cell disease; An overview of the disease and its systemic effects. ( 29428339 )
2018
48
HO-1<sup>hi</sup>Patrolling Monocytes Protect against Vaso-occlusion in Sickle Cell Disease. ( 29437594 )
2018
49
Recurrent Acute Chest Syndrome in Pediatric Sickle Cell Disease: Clinical Features and Risk Factors. ( 29200151 )
2018
50
Clustering of end-organ disease and earlier mortality in adults with sickle cell disease: A prospective cohort study. ( 29981283 )
2018

Variations for Sickle Cell Disease

Expression for Sickle Cell Disease

Search GEO for disease gene expression data for Sickle Cell Disease.

Pathways for Sickle Cell Disease

Pathways related to Sickle Cell Disease according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1 11.65 HBB HBD HBG1 HBG2
2
Show member pathways
10.8 HBA2 HBB
3 10.68 HBA2 HBB VCAM1
4 10.61 HBA2 HBB SELP VCAM1

GO Terms for Sickle Cell Disease

Cellular components related to Sickle Cell Disease according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 extracellular space GO:0005615 9.87 EPO HBA2 HBB MIR144 PGF SELP
2 blood microparticle GO:0072562 9.46 HBA2 HBB HBD HBG2
3 endocytic vesicle lumen GO:0071682 9.26 HBA2 HBB
4 hemoglobin complex GO:0005833 9.02 HBA2 HBB HBD HBG1 HBG2
5 haptoglobin-hemoglobin complex GO:0031838 8.96 HBA2 HBB

Biological processes related to Sickle Cell Disease according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 response to lipopolysaccharide GO:0032496 9.65 EPO SELP VCAM1
2 response to hypoxia GO:0001666 9.63 EPO PGF VCAM1
3 leukocyte cell-cell adhesion GO:0007159 9.43 SELP VCAM1
4 calcium-mediated signaling using intracellular calcium source GO:0035584 9.4 SELP VCAM1
5 hydrogen peroxide catabolic process GO:0042744 9.37 HBA2 HBB
6 cell volume homeostasis GO:0006884 9.32 KCNN4 SLC12A4
7 leukocyte tethering or rolling GO:0050901 9.26 SELP VCAM1
8 blood coagulation GO:0007596 9.26 HBB HBD HBG1 HBG2
9 erythrocyte maturation GO:0043249 9.16 EPO G6PD
10 oxygen transport GO:0015671 9.02 HBA2 HBB HBD HBG1 HBG2

Molecular functions related to Sickle Cell Disease according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 iron ion binding GO:0005506 9.65 HBA2 HBB HBD HBG1 HBG2
2 heme binding GO:0020037 9.55 HBA2 HBB HBD HBG1 HBG2
3 peroxidase activity GO:0004601 9.37 HBA2 HBB
4 oxygen binding GO:0019825 9.35 HBA2 HBB HBD HBG1 HBG2
5 haptoglobin binding GO:0031720 9.32 HBA2 HBB
6 oxygen carrier activity GO:0005344 9.02 HBA2 HBB HBD HBG1 HBG2

Sources for Sickle Cell Disease

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 ExPASy
19 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
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