IVEMARKA SYNDROME

From Big Medical Encyclopedia

IVEMARKA SYNDROME (Century of I. I. Ivemark, sovr. Swede, pediatrician; syndrome:; synonym: agenesia of a spleen, inborn lack of a spleen) — combination of an agenesia of a spleen to inborn heart disease and anomalies of an arrangement of abdominal organs.

It is for the first time described by Ivemark in 1955. In domestic literature And. page it is for the first time described by I. K. Yesipova and A. D. Soboleva in 1958 Yu. E. Veltishchev and V. Ya. Lipets (1959) noted that 21 children from 50, having two-chamber heart, had a combination of this defect to an agenesia (aplasia) of a spleen and a partial return arrangement of abdominal organs, at 2 — the same with a full return arrangement of abdominal organs. It gave the grounds for allocation of full and incomplete forms I. page, and the last meet considerably more often. In 1959 Mr. N. Neimann and soavt, submitted the analysis of 104 observations of this complex of malformations and suggested to call it a syndrome to the Willow brand.

And. page — embryopathy (see), edges develops as genotypic anomaly, and also under the influence of the infectious and toxic factors damaging a differentiation of fabrics of an embryo in «critical period» (an interval between the 31st and 36th days of an embryogenesis). A role of hereditary factors in emergence And. the page is confirmed by the fact that it is observed at the children who were born from marriage between relatives and also can be combined with aberation chromosomes (structural changes of chromosomes), in particular with a syndrome of «cat's shout» (see. Le Jeune syndrome ).

Clinical picture And. the page is defined by degree of manifestation of inborn heart disease of «blue» type (see. Heart diseases inborn ), quite often combined with dextrocardia (see) or mesocardia. Typical for And. the page is total absence of an interventricular partition, is frequent also an interatrial partition with morfol. signs of two - or three-chambered heart. There can be a full transposition of the main vessels, a stenosis or an atresia of pulmonary arteries. For incomplete forms I. page Fallo's tetrad is most typical (see. Fallo tetrad ).

The full return arrangement of abdominal organs meets rather seldom (see. Transpozition of bodies ). Almost at a half of patients median position of a liver and only in isolated cases its inversion is defined (at the same time the stomach is located on the right, the return arrangement of a large intestine is noted). More than 90% of patients have no spleen; at incomplete forms I. page it is characterized by signs hypoplasias (see), existence of anomalies of a structure, lobation. An indirect symptom of an agenesia of a spleen is emergence in erythrocytes of inclusions (so-called little bodies of Heinz), increase in quantity of reticulocytes in blood, Eritroblastoz.

Diagnosis And. the page is put on the basis of anamnestic, a wedge., electrocardiographic, rentgenol. data and data of a gemogramma.

Forecast it is adverse. Children perish in the first months of life from a circulatory unefficiency or heavy septic complications.


Bibliography: Veltishchev Yu. E. and Lipets V. Ya. About a combination of two-chamber heart to an agenesia of a spleen and a partial return arrangement of interiors, Owls. medical, No. 8, page 107, 1959; Yesipova I. K. and With about about l of e in and A. D. Sluchay of rare inborn heart disease (a pra-vodeleniost of a ventricle with an atresia of an opening of the three-leaved valve) with lack of a spleen and a left-side arrangement of a liver, Arkh. patol., t. 20, No. 5, page 72, 1958; I v e m and of k B. Implications of agenesis of the spleen on the pathogenesis, of conot-runcus anomalies in childhood, Acta paediat. (Uppsala), v. 44, suppl. 104, p. 7, 1955; Neimann N. a. 0. Le «syndrome d’lvemark», Pediatrie, t. 21, p. 511, 1966.

BB. E. Veltishchev.

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