Syndrome of levocardia, multiple cardiac defects, situs inversus, and absent spleen A case report

Badr-el-din, M. K.

doi:10.1016/0002-8703(62)90229-6

Cited by 11 publications

(3 citation statements)

References 4 publications

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“…Two previous reports of consanguinity occurring in families with isolated cases of Ivemark syndrome complement this experience (Bader-El-Din 1962, Neiman et al 1966. Consanguinity was also observed in a family in which two males were affected (Schonfeld & Frischman 1958).…”

Section: Resultssupporting

confidence: 67%

Ivemark syndrome in siblings

Hurwitz

Caskey

1982

Clinical Genetics

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A family in which two brothers have the lemurs syndrome is reported, thus bringing to eight the total number of families reported with multiple affected siblings. Study of 4059 autopsies, performed over 21 years at a major pediatric referral hospital, identified 32 cases of Ivemark syndrome. All were isolated occurrences in the families. One of six families which provided complete pedigree information was found to be consanguineous. This brings to four the number of reported consanguineous families with Ivemark syndrome. The male excess of affected with Ivemark syndrome is found both for the families with multiple affected siblings and for autopsy‐identified cases. These data suggest a recessive inheritance of Ivemark syndrome with male predisposition.

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Section: Resultssupporting

confidence: 67%

Ivemark syndrome in siblings

Hurwitz

Caskey

1982

Clinical Genetics

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“…Since the morbidity of Ivemark syndrome in males and females is alike, Xlinked recessive inheritance can be excluded. The occurrence in sibs and parental consanguinity found in three instances (Schonfeld and Frischman, 1958;Badr-El-Din, 1962;Neimann et al, 1966) would support autosomal recessive inheritance. Putschar andManion (1956), Chaptal et al (1960), and Moss and Adams (1968) distinguished between cases with asplenia and cases where a spleen is present but abnormal (hypoplastic, lobulated, or polylocular) and postulated two different disease entities: Ivemark syndrome with asplenia and Ive-* Schonfeld and Frischman present a family in which two children have similar congenital abnormalities, including defects of heart and vessels and situs inversus.…”

Section: Discussionmentioning

confidence: 91%

Familial Occurrence of Ivemark Syndrome with Splenic Hypoplasia and Asplenia in Sibs

Simpson

Zellweger

1973

Journal of Medical Genetics

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“…Familial incidence has been described in six instances (Polhemus & Schafer 1952, Schonfeld & Frischman 1958, Ruttenberg et al 1964, Simpson & Zellweger 1973, Rose et al 1975. Ivemark syndrome in one child with Fallot's tetralogy in a sib has also been reported (Silver et al 1972) and parental consanguinity was noted by three groups of authors (Schonfeld & Frischman 1958, Badr-El-Din 1962, Neimann et al 1966. Table 1 Clinical findings in the two sibs Recent reviews include those of Randall et al (1973) and Rose et al (1975).…”

mentioning

confidence: 99%

Renal dysplasia and asplenia in two sibs

Crawfurd¹

1978

Clinical Genetics

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A family is reported in which two sibs, one male and the other female, both died within 24 hours of birth with enlarged plycystic kidneys. Postmortem histology in the second child showed gross renal dysplasia. In both children the pancreas was enlarged, nodular and cystic but the liver appeared macroscopically normal. In the second child, histological examination confirmed pancreatic fibrosis with cystic dilation of ducts, but showed portal fibrosis with bile duct proliferation in the liver.This combination of findings is very reminiscent of those in a girl and her brother reported by Ivemark et al. (1959). The children reported here also showed absence or hypoplasia of the spleen, cardiac anomalies and other features of the Ivemark syndrome (Ivemark 1955), a quite different, usually sporadic, congenital disorder. It is suggested that the children described here have a distinct lethal congenital disorder, probably inherited in an autosomal recessive manner.

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Syndrome of levocardia, multiple cardiac defects, situs inversus, and absent spleen A case report

Cited by 11 publications

References 4 publications

Ivemark syndrome in siblings

Ivemark syndrome in siblings

Familial Occurrence of Ivemark Syndrome with Splenic Hypoplasia and Asplenia in Sibs

Renal dysplasia and asplenia in two sibs

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