[A case of hepatic and splenic peliosis occurring in the puerperium after normal pregnancy]. / Une observation de péliose hépatique et splénique survenue dans le post-partum au terme d'une grossesse normale.

The authors report a case of Peliosis of the liver and of the spleen that occurred at term after a normal pregnancy that lasted 38 weeks. The delivery was complicated by amniotic infection with hemorrhagic shock and uterine inertia that gave rise to the need for a sub-total hysterectomy in order to stop the bleeding. The progress of the case was made worse by the development of the disseminated intra-vascular coagulation syndrome with septicaemia and pulmonary oedema. When the operation was carried out to stop the bleeding it was noted that the liver was enlarged, hard, smooth and dark in colour and the histological specimen showed a major degree of Peliosis hepatis. The same lesions were found in the region of the spleen and a lymph node. The patient died 14 days after delivery of acute renal failure together with cerebral oedema and septic shock. The authors consider that the aetiology of the Peliosis could be due to hormone changes of pregnancy associated with septicaemia. The anatomical evolution of Peliosis hepatis to widespread necrosis of the parenchyma makes it possible to understand that the condition of diffuse intra-vascular coagulation will not improve in the presence of hepato-cellular insufficiency.

[Fetal ascites as a manifestation of infantile sialidosis. Significance of a study of oligosaccharides in amniotic fluid]. / Ascite foetale révélatrice d'une sialidose infantile. Intérêt de l'étude des oligosaccharides du liquide amniotique.

Non immune hydramnios and fetal ascite are demonstrated at 31 weeks gestation. There is no familial story. All etiologic investigations (repeated ultrasonographic examinations, amniocentesis) are negative. The delivered girl has a normal development. She presents a congenital ascite and edema. The diagnosis of sialidosis (mucolipidosis type I) is supported by the early finding of vacuolated lymphocytes, the excretion of oligosaccharides in the urine and, finally, the results of the study of alpha-D-neuraminidase fibroblasts and others lysosomal enzymes activities. Oligosaccharides and enzymic studies provide same results in amniotic fluid. Authors point the particular interest of amniotic fluid oligosaccharides study when the etiologic diagnosis of idiopathic fetal ascite or hydrops is to be done.