[Childhood visceral leishmaniasis in the Alpes-Maritimes from 1985 to 1992]. / La leishmaniose viscérale infantile dans les alpes-maritimes de 1985 a 1992.

Here, we present an analysis of clinical and biological data from 15 infantile visceral leishmaniasis recorded between 1985 and 1992 in the French department of Alpes-Maritimes. The association of fever, splenomegalia, tricytopenia remains the most constant and predictive of diagnosis (90% of cases). Bone marrow smear affirms diagnosis in 93% of cases. Leishmania infantum MON-1 is the causative agent. Except for one case of advanced stade with severe hepatic lesions and one case of clinical resistance to Glucantime, prognosis is favourable under treatment with antimony derivatives. The long term results for children considered cured after initial treatment is satisfactory. In the department, the number of cases of combined ages has increased regularly since 1985. The number of infantile forms has increased but now represents nevertheless no more than 28% of total cases. On the other hand, cases are increasing in adults and 40% of those adult cases are in HIV positive patients.

[Hermansky-Pudlak syndrome. Apropos of a case]. / Le syndrome d'Hermansky-Pudlak. A propos d'un cas.

A new case of Hermansky-Pudlak syndrome is reported. Clinical, ophthalmological, hematological and dermatological explorations are all described in detail, with emphasis placed on the risks of non-recognition of the syndrome. The ocular albinism is associated with a cutaneous syndrome of highly variable expression and a thrombopathy generally without frank clinical consequence. The quasi spontaneous meningeal hemorrhage noted in this case is unusual. Literature data on this syndrome is reviewed and its main features are described.

Effect of dose and repeat intravenous 24 hr infusions of methotrexate on cerebrospinal fluid availability in children with hematological malignancies.

This pharmacokinetic study examined the relationship between methotrexate (MTX) dose and drug concentrations in blood and cerebrospinal fluid (CSF) during repeated 24 hr infusions. Two regimens were used: an intermediate dose (ID) of 0.5 g/m2 (7 patients, 23 cycles) and a high dose (HD) of 2.5 g/m2 (8 patients, 39 cycles). Inter-patient variability in the drug concentration was apparent in serum and CSF for both doses. The dispersion was particularly wide in CSF for HD MTX. Considering median values, serum and CSF MTX were linked to dose escalation. Individual CSF/serum drug ratios were not modified by the dose (1.1% for ID MTX versus 1.4% for HD MTX). A potentially cytotoxic drug level in CSF (10(-6) M) was never obtained for ID MTX cycles, but was achieved in 44% of HD MTX cycles: for HD MTX, this corresponded to 88% of patients (7/8). Total body clearance did not modify the degree of CSF MTX passage. A positive, significant correlation (r = 0.62, P less than 0.05) was observed for ID MTX between individual serum and CSF MTX; no such relationship was seen with HD MTX. Individual cycle-to-cycle variations in the MTX concentration were particularly marked in CSF and for HD MTX, without strict concordance with blood levels.

[HIV-positive newborns via maternal-fetal transmission. Early ophthalmic lesions]. / Nourrissons HIV positifs par transmission materno-foetale. Lésions ophtalmologiques précoces.

Since January 1986, the authors have examined twenty infant HIV positives. In two cases specially, some serious and precocious ophthalmic lesions have been found : an ophthalmic zona appeared at the age of three months, a cytomegalovirus retinitis diagnosed at the age of six months. From this series, the particularities of the HIV infection by maternal-fetal transmission are discussed.

[Parents' view on the care of their children at pediatric emergency department in 1991]. / Opinion des parents sur la prise en charge de leur enfant par un service d'accueil des urgences pédiatriques en 1991.

A survey carried out by questionnaire was performed among parents accompanying their children to the emergency department of the Lenval's children hospital in order to determine their opinion on the quality of the reception and care. The questionnaire was distributed to 2,382 parents. A total of 719 questionnaires (30.2%) were returned. A large majority of the parents (99%) expressed their satisfaction with the care and reception in the emergency department. The necessary improvements following this survey concern the duration of waiting prior to the consultation and the quality of the reception in the radiological and admittance departments; it is hoped that the radiological and admittance areas will be included within the emergency department, in the future.

Abnormal red cell membrane phosphatase activity in an unidentified congenital hemolytic anemia.

The erythrocyte membrane contains a neutral phosphatase, which was studied with p-nitrophenyl-phosphate as the substrate. The enzyme was investigated in four members of the same family, suffering from a congenital spherocytic hemolytic anemia. Although the condition was transmitted as a dominant trait, it was not identified to the common hereditary spherocytosis. The phosphatase, instead of having a Michaelis-Menten kinetics, displayed a dramatically biphasic kinetics: substrate excess generated partial inhibition of the enzyme. We consider that such an abnormality, which was never encountered before, is a distinctive feature of a given type of congenital hemolytic anemia.

[Bloom's syndrome. Discussion of the diagnosis concerning two cases of terminal leukemia in a sibship (author's transl)]. / Le syndrome de Bloom. Discussion du diagnostic à propos de deux cas dans une fratrie avec leucémie terminale.

Two brothers developed acute leukemia, one at the age of 7 months and the other at the age of 14 months. Both suffered from a staturoponderal retardation and the same malformation syndrome. The karyotype carried out only on the second child revealed breaks and chromatid changes. A diagnosis of Fanconi's anaemia can be discarbed since no blood cytopenia preceded the leukemia. Finally, the diagnosis of Bloom's syndrome prevailed despite the absence of telangiectatic erythema and the atypical chromosomal anomalies.

[Coma and necrotizing enteritis in Schönlein-Henoch syndrome]. / Coma et entérite nécrosante au cours d'un syndrome de Schönlein-Henoch.

Authors report one case of HENOCH-SCHONLEIN syndrome particular by the association of two serious complications of good prognosis: neurological signs with coma and necrosing enteritis necessitating an intestinal resection of 60 cm of small bowel.