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Literature DB >> 7277426

Asplenia and polysplenia syndromes with abnormalities of lateralisation in a sibship.

Abstract

In the family presented here the first child had asplenia syndrome with cor biloculare' transposition of the great vessels, pulmonary stenosis, and anomalous pulmonary venous drainage. Another sib had situs inversus with polysplenia syndrome, including very similar cardiovascular defects and biliary atresia. The possibility that these two syndromes, namely asplenia and polysplenia, are different manifestations of a similar defect in the normal asymmetrical development of internal organs is discussed.

Entities: Disease Species

Mesh：

Year: 1981 PMID： 7277426 PMCID： PMC1048737 DOI： 10.1136/jmg.18.4.301

Source DB: PubMed Journal: J Med Genet ISSN： 0022-2593 Impact factor: 6.318

8 in total

1. SYNDROME OF CONGENITAL CARDIAC DISEASE WITH ASPLENIA. DISTINCTION FROM OTHER FORMS OF CONGENITAL CYANOTIC CARDIAC DISEASE.

Authors: H D RUTTENBERG; H N NEUFELD; R V LUCAS; L S CAREY; P ADAMS; R C ANDERSON; J E EDWARDS
Journal: Am J Cardiol Date: 1964-03 Impact factor: 2.778

2. Congenital absence of the spleen; syndrome with atrioventricularis and situs inversus; case reports and review of the literature.

Authors: D W POLHEMUS; W B SCHAFER
Journal: Pediatrics Date: 1952-06 Impact factor: 7.124

3. Familial splenic anomaly syndrome.

Authors: S C Chen; P L Monteleone
Journal: J Pediatr Date: 1977-07 Impact factor: 4.406

4. Biliary atresia and other structural anomalies in the congenital polysplenia syndrome.

Authors: R S Chandra
Journal: J Pediatr Date: 1974-11 Impact factor: 4.406

5. Syndromes of asplenia and polysplenia. A review of cardiac and non-cardiac malformations in 60 cases withspecial reference to diagnosis and prognosis.

Authors: V Rose; T Izukawa; C A Moës
Journal: Br Heart J Date: 1975-08

6. Renal dysplasia and asplenia in two sibs.

Authors: M D Crawfurd
Journal: Clin Genet Date: 1978-12 Impact factor: 4.438

7. Sepsis and congenital asplenia.

Authors: J D Waldman; A Rosenthal; A L Smith; S Shurin; A S Nadas
Journal: J Pediatr Date: 1977-04 Impact factor: 4.406

8. Familial occurrence of Ivemark syndrome with splenic hypoplasia and asplenia in sibs.

Authors: J Simpson; H Zellweger
Journal: J Med Genet Date: 1973-09 Impact factor: 6.318

8 in total

6 in total

Asplenia and polysplenia syndromes with abnormalities of lateralisation in a sibship.

1. SYNDROME OF CONGENITAL CARDIAC DISEASE WITH ASPLENIA. DISTINCTION FROM OTHER FORMS OF CONGENITAL CYANOTIC CARDIAC DISEASE.

2. Congenital absence of the spleen; syndrome with atrioventricularis and situs inversus; case reports and review of the literature.

3. Familial splenic anomaly syndrome.

4. Biliary atresia and other structural anomalies in the congenital polysplenia syndrome.

5. Syndromes of asplenia and polysplenia. A review of cardiac and non-cardiac malformations in 60 cases withspecial reference to diagnosis and prognosis.

6. Renal dysplasia and asplenia in two sibs.

7. Sepsis and congenital asplenia.

8. Familial occurrence of Ivemark syndrome with splenic hypoplasia and asplenia in sibs.

Review 1. Hepatobiliary anomalies associated with polysplenia syndrome.

Review 2. Defects in the determination of left-right asymmetry.

3. Heterotaxia syndrome: the role of screening for intestinal rotation abnormalities.

4. Consanguinity and complex cardiac anomalies with situs ambiguus.

5. Asplenia syndrome and isolated total anomalous pulmonary venous connection in siblings.

6. Prenatal diagnosis of atrial isomerism in the Korean population.