[Physicians' opinions and practices on the use of medical referrals at hospitals].

This work aims to take stock of the actual utilisation of medical standards (references), through a study of opinions and practices of the medical managers working in the clinical services of a university hospital centre. A survey through interviews was proposed to 103 medical managers, 101 responded to the questionnaire (38 Unit directors and 63 directors of "UF" units). Medical standards are used essentially as a teaching aid by 80% of doctors.

[Spinal cord injuries in infants in traffic accidents].

The authors report on three cases of spinal cord injury in 11, 14 and 21-month-old infants, involved in motor vehicle accidents. Neurologic lesions were caused by a forcible flexion of the head over a fixed trunk. Pathomechanics management problems and therapeutic considerations are discussed.

[A fatal respiratory form of type C Niemann-Pick disease].

An unusual case of Niemann-Pick disease type C is reported. The disease was first manifested in utero with hepatomegaly and ascitis. At the age of 3 months, respiratory signs were noted due to diffuse alveolar and interstitial pneumonia.

[Fusobacterium necrophorum septicemia in an infant].

In a 9 month-old infant admitted to hospital for a fever with chilles, anaerobic blood cultures isolated Fusobacterium necrophorum. On the 5th day of intravenous treatment with amoxicillin and metronidazole clinical signs of mastoiditis, the likely source of the sepsis, became apparent. Septicemias with Fusobacterium necrophorum are usually observed in teenagers and young adults during an acute bout of tonsilitis.

[Acute encephalopathy following acquired rubella. Apropos of 6 cases].

The authors report 6 cases of acute encephalopathy during the course of primary rubella infection. The mean age of the patients was 7 years and 6 months. Clinical features included, 3 days after an inconstant exanthema, seizures, coma and hyperthermia.

[Atheromatous lesions of the proximal aorta. Severe complications of homozygous type IIa hypercholesterolemia in children].

Coronary lesions with atheromatous deposits occurring in later childhood characterize homozygous type IIa hypercholesterolaemia and condition the somber prognosis of a disease which affects one subject in a million. However, aortic lesions are constantly found, as shown by routine ultrasonographic and angiographic studies in these children. The walls of the proximal aorta are cardboard-like and thick, the origin of the aorta is narrow and the semilunar aortic valves are thickened.

[Massive breast involvement in Burkitt's lymphoma].

Massive bilateral breast involvement in the course of a Burkitt's lymphoma is reported as an exceptional occurrence in a 13 year-old girl, in early puberty. It was an extensive form, stage IV, with massive organ involvement but without CNS involvement. Chemotherapy (LMB 84) induced total cure (18 months disease free survival).

[Severe hyperthermia syndrome in the infant. Apropos of 11 cases].

The authors report a retrospective study of 11 cases of malignant hyperthermia. The mean age of the patients was 5 months and 3 weeks. Clinical features included severe hyperthermia (greater than 41 degrees C), seizures, coma, collapse, rhabdomyolysis, acute renal failure and functional renal failure.

[Epidemiological and prognostic aspects of purpura fulminans in children. Regional survey].

A regional survey (Rhône-Alpes region) about fulminans purpura has been performed in the pediatric services and the pediatric intensive care units. Between January 1982 and July 1986, 101 cases have been observed. 93.

[Digitalis poisoning in children. Treatment with anti-digoxin Fab antibody fragments. Apropos of a case and a discussion of therapeutic indications].

We report one case of Digoxin intoxication in a child treated with Fab Fragments of Digoxin-Specific antibodies (Fabad), although there was no evidence of early life threatening complications. The efficacy of this treatment, which prevents further complications as well as its safety, represent strong arguments to treat children at the early stage of the intoxication in order to avoid temporary cardiac pacing.

[A case of thalamic cavernous hemangioma in an infant].

The case reports an asymmetric, slowly increasing hydrocephalus with left leg monoplegia. The vascular origin of the tumor was suspected by CT scan and confirmed by surgery and the pathology analysis. These tumors are infrequent in childhood especially in this region.

[A new case of aneurysm of Galen's vein. Use of echography. Treatment difficulties].

A new case of intracranial arteriovenous malformation was observed in a newborn infant presenting as severe congestive heart failure, massive cardiomegaly and intracranial murmur. Cerebral echotomography through the fontanelle showed a large echo-free structure behind the third ventricle. Cerebral arteriography confirmed the vein of Galen aneurysm.

The place of cryosurgery in the treatment of subglottic angiomas of the infant.

The treatment of subglottic angiomas of the infant is not clear since radiotherapy was rejected. Tracheotomy is very often indicated. Spontaneous recovery of angioma may be hoped for, but it is slow.

[Still another cause of hypermethioninemia in children: S-adenosylmethionine synthetase deficiency].

One case of hypermethioninaemia discovered on systematic neonatal screening examination is reported. This metabolic disorder was associated with growth retardation, anorexia, digestive disturbances, and a strong smell of "boiled cabbage" in urine and sweat. With a 6-year follow up, psychomotor and growth developments were excellent under a low methionine containing diet, in spite of a persistent pathological hypermethioninaemia.

[Fanconi's anemia: cytochemical and biochemical aspects (author's transl)].

Three cases of Fanconi's anemia are described in three siblings. Cytogenetic studies disclose increase of chromosomal structural anomalies (unclassifiable rearrangements, quadriradials, dicentrics) and suggest in the parents an increase in chromatid-type abnormalities. Further, the mother carries a 46,XX/47, XXX/48, XXXX mosaic and other kinds of aneuploidy.