[Chediak- Higashi Syndrome. report of one case]

Chediak - Higashi syndrome is a rare hereditary disease. Most patients develop an accelerated phase of the disease. Once accelerated phases occurred, the disease is invariably fatal before the use of etoposide. We report the case of a 5 year-old girl with consanguineous parents, presenting a partial oculocutaneaous hypopigmentation admitted because of protracted fever, lethargy, paler and hepatosplenomegaly. Laboratory results confirmed the diagnosis of hemophagocytic syndrome. The finding of a large cytoplasm granules in born marrow leucocytes confirmed the diagnosis of Chediak Higashi syndrome. Treatment with immunoglobulin, steroids and cyclosporine induced a transitory remission and a relapse occured after 3 weeks. The use of etoposide associated with precedent treatment leads to a prolonged remission and bone marrow transplantation was succefuly performed after 5 months. This case reports the severity of accelerated phase of Chediak- Higashi syndrome and the efficiency of the treatment with etoposide

[ malignant adrenocortical carcinoma of the child to a comment]

Malignant corticossurrenaloma is very rare in childhood. It occurs more often before the age of 5 years. Its pathogeny remains unknown but a genetic predisposition was suggested. The tumor is usually of secreting type and revealed by virilisation and/or Cushing syndrome, seldom by feminization and exceptionally by Conn syndrome. We report the case of a 7 years old girl who presented with a hetero-sexual precocious pseudo-puberty associated to a Cushing syndrome. The clinical examination found an abdominal mass. Hormonal investigation confirmed androgen and gluco-corticoid hypersecretion. Malignancy of the tumor was evoked by MRI. Evolution was marked by a tumoral rupture which required a laparotomy in emergency. The anatomo-pathological examination confirmed the diagnosis. In spite of a favorable immediate post operative evolution, the child died eight days later. Through this observation, we discuss the clinical, biological, therapeutic and evolutive aspects of this affection

[Acute post-streptococcal glomerulonephritis and pneumonia. About 6 cases]

Acute post-streptococcal glomerulonephritis [GNA] typically occurs 7-14 days after streptococcal infection, however some children can develop GNA concomitantly with bacterial pneumonia. ONA and pneumonia is a rare association, its often underestimated, its etiopathogenie remains still not clear. The purpose of this study is to analyse the etiopathogeny, clinical course and the outcome of this association. It is about 6 children hospitalized for GNA and pneumonia among 417 cases of GNA admitted between 1982 and 2003. All patients had at the time of admission abnormal urianalysis associated with respiratory manifestations. The evolution was favorable at 5 patients while the last one presented an empyema, nephrotic syndrome and died by pulmonary emboly. In conclusion the presence of a renal symptomatology at a child with pneumonia should be evaluated for concomitant GNA. This glomerulonephrite has generally a good prognosis, but sometimes specific therapeutic interventions are necessary to avoid the risk of renal failure