MCID: PLN006
MIFTS: 41

Poland Syndrome malady

Rare diseases, Reproductive diseases, Bone diseases, Fetal diseases, Oral diseases categories

Summaries for Poland Syndrome

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Sources:
22Genetics Home Reference, 44NIH Rare Diseases, 66Wikipedia, 48OMIM, 34MalaCards
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Genetics Home Reference:22 Poland syndrome is a disorder in which affected individuals are born with missing or abnormal muscles on one side of the chest wall. Most individuals with Poland syndrome also have abnormalities of the hand, which often involve shortened fingers, partial fusion of the fingers, or both. The hand abnormalities usually affect the same (ipsilateral) side of the body as those of the chest. Poland syndrome affects the right side of the body more often than the left side.

MalaCards: Poland Syndrome, also known as poland anomaly, is related to dextrocardia and adams oliver syndrome, and has symptoms including breast neoplasm/tumor/carcinoma/cancer, sternal/sternum anomalies and dextrocardia/abnormal heart position/cardiac heterotaxia/situs inversus. Affiliated tissues include breast, bone and liver.

NIH Rare Diseases:44 Poland syndrome is characterized by an underdeveloped or absent chest muscle on one side of the body, absence of the breastbone portion (sternal) of the chest muscle, and webbing of the fingers of the hand on the same side. the cause of poland syndrome is not known. this syndrome is nearly always sporadic. it tends to be right sided and is more common in boys than girls. last updated: 6/25/2011

Wikipedia:66 Poland syndrome (also Poland\'s syndrome, Poland\'s syndactyly, Poland sequence, and Poland\'s anomaly)... more...

Description from OMIM:48 173800

Aliases & Classifications for Poland Syndrome

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Sources:
9Disease Ontology, 44NIH Rare Diseases, 22Genetics Home Reference, 50Orphanet, 48OMIM, 63UMLS, 36MeSH, 41NCIt, 59SNOMED-CT, 37MESH via Orphanet, 27ICD10 via Orphanet, 60SNOMED-CT via Orphanet, 64UMLS via Orphanet
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Classifications:



Characteristics (Orphanet epidemiological data):

50
poland syndrome:
Inheritance: Autosomal dominant,Sporadic; Prevalence: 1-9/100000; Age of onset: Neonatal/infancy; Age of death: Normal


Aliases & Descriptions:

poland syndrome 9 44 22 50 48 63
poland anomaly 9 44 22 50
poland sequence 44 22 50
poland syndactyly 44 22
poland's syndrome 44 22
unilateral defect of pectoralis muscle and syndactyly of the hand 44
unilateral defect of pectoralis major and syndactyly of the hand 22
poland's syndactyly 9
poland's anomaly 22


External Ids:

Disease Ontology9 DOID:12961
MeSH36 D011045
OMIM48 173800
NCIt41 C85017
SNOMED-CT59 38371006, 205524002
MESH via Orphanet37 D011045
ICD10 via Orphanet27 Q79.8
SNOMED-CT via Orphanet60 38371006
UMLS via Orphanet64 C0032357

Related Diseases for Poland Syndrome

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18GeneCards, 19GeneDecks
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Graphical network of the top 20 diseases related to Poland Syndrome:



Diseases related to poland syndrome

Symptoms for Poland Syndrome

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48OMIM, 50Orphanet
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Symptoms by clinical synopsis from OMIM:

173800

Clinical features from OMIM:

173800

Symptoms:

50 (show all 33)
  • breast neoplasm/tumor/carcinoma/cancer
  • sternal/sternum anomalies
  • dextrocardia/abnormal heart position/cardiac heterotaxia/situs inversus
  • oligodactyly/ectrodactyly of fingers
  • hand agenesis/absence
  • radius anomaly/absence/agenesis/hypoplasia/abnormal radial ray
  • lower limb segmental anomalies
  • asymmetric rib cage/thorax
  • retinal hamartoma
  • absent pectoral muscles
  • breast tissue/mammary gland absence/aplasia
  • humerus anomaly/absence/agenesis/hypoplasia/congenital humerus varus
  • diaphragmatic hernia/defect/agenesis
  • thumb hypoplasia/aplasia/absence
  • anomalies of the ribs
  • microcephaly
  • ulnar/cubital anomaly/absence/agenesis/hypoplasia/abnormal ulnar/cubital ray
  • anomalies of skin, subcutaneous tissue and mucosae
  • osteoporosis/osteopenia/demineralisation/osteomalacia/rickets
  • autosomal dominant inheritance
  • scoliosis
  • syndactyly of fingers/interdigital palm
  • short hand/brachydactyly
  • cone epiphyses/epiphysis
  • anomalies of hands
  • anomalies of spine, vertebrae and pelvis
  • acute leukemia
  • agenesis/hypoplasia/aplasia of kidneys
  • structural anomalies of the liver and the biliary tract
  • abnormal dermatoglyphics
  • low hair line (back)
  • hypoplastic/absent nipples
  • short neck

Drugs & Therapeutics for Poland Syndrome

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Sources:
6CenterWatch, 43NIH Clinical Center, 7ClinicalTrials
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Approved drugs:

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Genetic Tests for Poland Syndrome

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Anatomical Context for Poland Syndrome

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Sources:
34MalaCards
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MalaCards organs/tissues related to Poland Syndrome:

34
Breast, Bone, Liver, Skin, Heart, Kidney, Retina

Animal Models for Poland Syndrome or affiliated genes

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Publications for Poland Syndrome

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Sources:
53PubMed
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Articles related to Poland Syndrome:

(show top 50)    (show all 107)
idTitleAuthorsYear
1
Chest wall reconstruction with methacrylate prosthesis in Poland syndrome. (23453291)
2013
2
Poland syndrome: from embryological basis to plastic surgery. (23420344)
2013
3
Poland syndrome a rare congenital anomaly. (24339551)
2013
4
A rare association of pulmonary hypertension and dextrocardia with Poland syndrome. (24054492)
2013
5
Autologous Reconstruction of a Complex Form of Poland Syndrome Using 2 Abdominal Perforator Free Flaps. (24322640)
2013
6
Poland syndrome with a rare association. (23547414)
2012
7
Poland syndrome. (23409420)
2012
8
Chest wall repair in Poland syndrome: complex single-stage surgery including Vertical Expandable Prosthetic Titanium Rib stabilization--a case report. (22424369)
2012
9
Poland syndrome with Mullerian agenesis: a rare occurrence. (22226538)
2012
10
Scapular winging in Poland syndrome. (21789405)
2011
11
Right-sided chest pain in Poland syndrome. (21959638)
2011
12
First case of Moebius-Poland syndrome in child prenatally exposed to misoprostol. (21420202)
2011
13
Poland syndrome associated with ipsilateral lipoma and dextrocardia. (22115239)
2011
14
Women's quality of life and surgical long-term outcome after breast reconstruction in Poland syndrome patients. (21501086)
2011
15
Poland syndrome. (20454789)
2010
16
Poland syndrome: evaluation and treatment of the chest wall in 63 patients. (20811224)
2010
17
Dextrocardia in patients with Poland syndrome: phenotypic characterization provides insight into the pathogenesis. (19909992)
2010
18
A case of Poland Syndrome associated with dextroposition. (20170539)
2010
19
Dextrocardia associated with left-sided Poland syndrome. (19169181)
2009
20
Unilateral hypoplastic breast in a male-to-female transsexual with Poland syndrome after gender reassignment--reconstructive considerations. (18639510)
2009
21
The carpal bones in Poland syndrome. (19183988)
2009
22
Poland syndrome with bilateral features: case description with review of the literature. (19533787)
2009
23
Titanium mesh prosthesis repair of symptomatic Poland syndrome in a premature infant. (18206489)
2008
24
Autologous rib transplantation and terylene patch for repair of chest wall defect in a girl with Poland syndrome: a case report. (18926229)
2008
25
Moebius-Poland syndrome and hypogonadotropic hypogonadism. (17401577)
2008
26
Complication after breast implantation in Poland syndrome. (19083584)
2008
27
Poland syndrome in a female patient reconstructed by endoscopically assisted technique. (17684839)
2007
28
Individualized implant-based reconstruction of Poland syndrome breast and soft tissue deformities. (17992143)
2007
29
Complete videoendoscopic harvest and transposition of latissimus dorsi muscle for the treatment of Poland syndrome: a first report. (17362186)
2007
30
Poland syndrome associated with an aberrant subclavian artery and vascular abnormalities of the retina in a child exposed to misoprostol during pregnancy. (17393483)
2007
31
Poland syndrome (anomaly) with congenital hemangioma: a new association. (16766839)
2006
32
The middle phalanx in Poland syndrome. (15655466)
2005
33
Poland syndrome: description of an atypical variant. (15759092)
2005
34
Acquired perforating dermatosis in a patient with Poland syndrome. (14657633)
2003
35
Unilateral absence of the external oblique muscle with hypoplasia of the rectus abdominis muscle in a patient with Poland syndrome. (12447072)
2002
36
Endoscopically assisted reconstruction of male and female poland syndrome. (11932594)
2002
37
A variant of Poland syndrome. (11832606)
2001
38
Anesthesia in Poland syndrome. (10349937)
1999
39
Lower extremity counterpart of the Poland syndrome. (10066030)
1999
40
Anaesthesia in Poland syndrome. (9579270)
1998
41
Neuroblastoma and Poland syndrome in a 15-year-old boy. (9447762)
1997
42
Peroneal muscular atrophy with Poland syndrome. (8042452)
1994
43
Poland syndrome with dextrocardia. (1286890)
1992
44
Poland syndrome and leukaemia. (2820736)
1987
45
Moebius-Poland syndrome. (2997111)
1985
46
Latissimus dorsi myocutaneous flap in Poland syndrome. (7247242)
1981
47
Vascular origin of Poland syndrome? A comparative rheographic study of the vascularisation of the arms in eight patients. (208850)
1978
48
Poland syndrome in British Columbia: incidence and reproductive experience of affected persons. (205132)
1977
49
The Poland syndrome. (209663)
1977
50
The familial occurrence of Poland syndrome. (195646)
1977

Variations for Poland Syndrome

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Expression for genes affiliated with Poland Syndrome

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16Gene Expression Omnibus DataSets
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Search GEO for disease gene expression data for Poland Syndrome.

Pathways for genes affiliated with Poland Syndrome

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Compounds for genes affiliated with Poland Syndrome

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GO Terms for genes affiliated with Poland Syndrome

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Products for genes affiliated with Poland Syndrome

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Sources for Poland Syndrome

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4CDC
14ExPASy
15FMA
23GTR
24HGMD
25HMDB
26ICD10
27ICD10 via Orphanet
28ICD9CM
30IUPHAR
31KEGG
36MeSH
37MESH via Orphanet
38MGI
41NCIt
42NDF-RT
45NINDS
46Novoseek
48OMIM
49OMIM via Orphanet
53PubMed
54QIAGEN
60SNOMED-CT via Orphanet
63UMLS
64UMLS via Orphanet