[Physicians' opinions and practices on the use of medical referrals at hospitals]. / Opinions et pratiques des médecins sur l'utilisation des référentiels médicaux à l'hôpital.

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. Some of them (48%) make them available to prescribing doctors in the unit, and for 36% there exists an informational procedure for new residents. Evaluation studies concerning the implementation of medical standards remain rare (7 studies). Medical standards appear to be more useful for improving quality of care (90%) then for controlling health expenditures (72%). The majority of medical managers (72%) consider that certain standards should be opposable to hospitals. The medical managers of the university hospital centre are in favour of developing standards of clinical practice.

[Spinal cord injuries in infants in traffic accidents]. / Les traumatismes médullaires du nourrisson dans les accidents de la voie publique.

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]. / Forme respiratoire mortelle de maladie de Niemann-Pick type C.

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. Both bronchoalveolar lavage and blood serologic studies revealed respiratory infection by respiratory syncitial virus and Chlamydia trachomatis. These concomitant infections delayed the diagnosis of Niemann-Pick disease which was finally made by the electronic microscopic studies of liver biopsy and bone marrow specimens. Type C was identified by biochemical characterization of lipid accumulation in hepatocytes and by lipid enzyme profiles obtained from cutaneous fibroblast cultures. The child died at the age of 6 months from respiratory failure. Post mortem examination of the lung showed the presence of numerous overloaded alveolar macrophages in the alveolar spaces and walls. The severity of the lung issue disease is unusual in type C Niemann-Pick disease, in which neurologic involvement is usually the main prognosis factor.

[Fusobacterium necrophorum septicemia in an infant]. / Septicémie à Fusobacterium necrophorum chez un nourrisson.

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. This type of infection is exceptional in infants and requires a careful search for a primary focus in facial cavities and in the base of the skull.

[Acute encephalopathy following acquired rubella. Apropos of 6 cases]. / Encéphalopathie aiguë au décours d'une infection rubéolique acquise. A propos de six observations.

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. The diagnosis of rubella was confirmed by a high rate of antibodies in serum with presence of IgM fraction. Five children recovered fully, one patient remained epileptic. The authors discuss the problems of diagnosis, the nosological position and the treatment of non-congenital rubella encephalitis.

[Atheromatous lesions of the proximal aorta. Severe complications of homozygous type IIa hypercholesterolemia in children]. / Les lésions athéromateuses de l'aorte proximale. Complications graves de l'hypercholestérolémie type IIa homozygote chez l'enfant.

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. The valvular or supravalvular aortic gradient may be considerable; it is often progressive, but is sometimes stabilized or made regressive by medical treatments combined with plasmapheresis or porto-caval shunt. Aortoplasty or aortic valve replacement being difficult to perform in these patients, more aggressive therapeutic procedures, such as liver or heart transplantation, have been suggested. The last generation cholesterol-lowering drugs seem to offer some hope of success.

[Massive breast involvement in Burkitt's lymphoma]. / Infiltration mammaire massive au cours d'un lymphome de Burkitt.

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]. / Syndrome d'hyperthermie majeure du nourrisson. A propos de 11 observations.

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. Three infants died. Four patients presented neurological damages. Four recovered fully. The authors discuss the difficulties of diagnosis, the nosological position and the pathophysiology of this syndrome.

[Epidemiological and prognostic aspects of purpura fulminans in children. Regional survey]. / Aspects épidémiologiques et pronostiques des purpuras fulminans de l'enfant. Enquête régionale.

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. 93.5% (72/77) of the bacteriologically documented cases were due to Neisseria meningitidis. Fatality rate is 34.6%, 58.6% in infants younger than 1 year to 14.3% in children older than 2 years. Between 1982 and 1985 fatality rate was reduced from 54.5% to 27.6%. This study confirms the interest of the severity score established by the French Pediatric Intensive Care Club.

[Digitalis poisoning in children. Treatment with anti-digoxin Fab antibody fragments. Apropos of a case and a discussion of therapeutic indications]. / Intoxication digitalique chez l'enfant. Traitment par fragments Fab d'anticorps anti-digoxine. A propos d'un cas et discussion des indications thérapeutiques.

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]. / A propos d'un cas d'hémangiome caverneux thalamique chez un nourrisson.

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. The surgical treatment before hemorrhagic complication permits to hope good results in the absence of malignancy.

[A new case of aneurysm of Galen's vein. Use of echography. Treatment difficulties]. / Une nouvelle observation d'anévrysme de la veine de Galien. Apport de l'échographie. Difficultés thérapeutiques.

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. A neurosurgical therapy clipping the nutrient vessels had not allowed this child to survive. Diagnostic value of ultrasound examination is emphasized. Surgical therapy is discussed.