[Acute encephalopathy and recurrent hepatic steatosis with normal long and medium chain fatty acyl-CoA-dehydrogenase activity].

A boy suffering from recurrent episodes of acute encephalopathy and hepatic steatosis died at 40 months of age. The symptoms started when he was 13 months old and he appeared completely normal in the intervals. Pertinent biologic findings were as follows: slight labile hypoglycemia and hyperammonemia having no direct correlation with neurologic derangement, no elevation of ammonia levels in loading tests, complete failure to generate ketones and the absence of organic aciduria during a fast, normal plasma carnitine levels and normal activity of long and medium chain acyl-CoA-dehydrogenase in skin fibroblasts.

[Giant bronchogenic cyst in a newborn infant].

A 12 hour old newborn suffering from respiratory distress was found to have a well defined opaque mass in the right lung. The mass was well tolerated up the 33rd day of life when he developed acute respiratory distress, the upper respiratory tract being flooded with secretions. Chest X ray showed that the mass had disappeared and that there was a large cystic structure instead.

[Thromboangiitis obliterans and distal gangrene in a 5-year-old child].

Distal gangrene associated with arterial hypertension but without systemic manifestations or muco-cutaneous eruptions was noted in a 5 years old child. Gangrene first appeared in toes and then in fingers. Progression in a 7 months period led to bilateral midleg amputation and amputation of several fingers.

[Flaccid paraplegia caused by spinal cord compression disclosing Wilms' tumor in a 12-year-old child].

Flaccid paralysis with spinal cord compression led to discovery of Wilm's tumor with multiple subcutaneous and bone metastases in a 12 year-old child. Intraspinal seeding of Wilm's tumor by hematogenous route or direct extension is extremely rare and usually appears late in the course of therapy. Bone metastases are also rare and are frequently seen in the sarcomatous form of the tumor which involves the vertebral column and differential diagnosis with bone metastasizing renal tumor of childhood (BMRTC) should be considered.

[Diffuse cutaneous vasculitis disclosing acute lymphoblastic leukemia].

Biopsy of skin for etiologic evaluation of generalized erythematous eruptions in a 4 1/2 years old child, starting four months ago, led to diagnosis of acute lymphoblastic leukemia. Leukocyte counts five and eight weeks and four months after appearance of eruptions were normal and no blasts were found. A leukocyte count performed simultaneously with biopsy of skin also showed few (less than 10%) blasts.

[Coexistence of late spondyloepiphyseal dysplasia and congenital megaloblastic anemia with proteinuria in the same family].

A case of spondyloepiphyseal dysplasia tarda was noted in a dwarf (130 cm tall) 18 years old boy associated with congenital megaloblastic anemia and proteinuria. His two sisters and a cousin are also suffering from similar hematologic disorder. One of his brothers, 145 cm tall, is also involved by spondyloepiphyseal dysplasia, but there is no known hematologic abnormalities.

[Acute hemiplegia due to cerebral artery occlusion in children. Apropos of 9 cases].

The cerebral arterial occlusion was observed from nine patients aged from 2,5 to 12 years old, between 37 children with acute acquired hemiplegia. The thrombosis had an embolic origin in three cases of cardiopathy. Six patients had primary arterial occlusion, but all these patients were febrile and the onset of hemiplegia was accompanied by seizures in five cases.

[Pulmonary involvement in Schonlein-Henoch's purpura].

A case of vascular purpura was observed in a 8 year-old girl. The clinical symptoms and pathological findings in kidneys and skin were compatible with Henoch-Schonlein syndrome. She presented with dyspnea and frequent acute pulmonary failure and the chest X-ray showed changing pulmonary infiltration.

[Hemorrhagic hypertrophic gastropathy in a child. A 15 year follow-up].

A 7 year-old boy with hypertrophic gastropathy and anemia is described. He was given a substitutive therapy and was under regular control. Upper G.

[Bone scan in the diagnosis of infectious osteoarthritis (author's transl)].

Bone scan with Technetium 99m is harmless method of evaluation of skeletal lesions. It is safe in pediatrics age group and it can be used in early diagnosis of infectious osteoarthritis. Bone scan differentiate osteomyelitis from cellulitis, and also it may help in diagnosis of subclinical involvement of rheumatoid arthritis, benign and malignant bone tumors, stress fractures and periositis.