• The clinical and morphological characteristics of hepatic haemangiomatosis at Kasabach–Merritt’s syndrome at the newborn with congenital heart defect
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The clinical and morphological characteristics of hepatic haemangiomatosis at Kasabach–Merritt’s syndrome at the newborn with congenital heart defect

SOVREMENNAYA PEDIATRIYA. 2014. 5(61):140-144; doi 10.15574/SP.2014.61.140

The clinical and morphological characteristics of hepatic haemangiomatosis at Kasabach–Merritt's syndrome at the newborn with congenital heart defect

Abdullin R. F., Kondratenko E. G., Lepihov P. A., Turpakova G. N. 
Regional children's clinical hospital, Donetsk, Ukraine

Objective: to establish clinical and morphological characteristics multiple haemangiomas to a liver at Kazabakh-Merrittt's syndrome at the newborn with congenital heart defect.

Material and methods. Material of research were clinical and tool data, a section material of one case of death of the child with Kazabach-Merritt's syndrome at the multiple haemangiomas and congenital heart defect. Histologic preparations made by a standard technique, painted hematoxylin and eosin. Ultrasonic research was conducted by the linear sensor of 5–10 MHz of the device Medison Sonoace X–8.

Results. On the example of one case clinical data with results of laboratory and ultrasonic research which testified to violations of curtailing system of blood and disorders of blood circulation are submitted. These changes correspond to Kazabach–Merritt's syndrome. The morphological characteristic of structural components of multiple tumoral knots and a liver parenchyma is given.

Conclusions. It is established that multiple haemangiomas of the liver are clinically characterized by fast increase in its sizes, hepatic insufficiency, Kazabach–Merritt's syndrome and violations of activity of cardiovascular system. The morphological structure of a new growth which is presented by sites capillary, cavernous, branchy haemangioma, the areas of haemangioendothelioma with the phenomena of proliferative activity is defined. It is revealed that in tumoral tissue and on its periphery the expressed local disorders of blood circulation and violation of a structure of a parenchyma of a liver are noted. It is noted that the combination multiple haemangiomas of the liver and congenital heart defect brings to a heavy current and progressing of cardiovascular insufficiency.

Key words: haemangiomatosis, liver, morphological structure, Kazabach–Merritt syndrome, heart insufficiency.

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