Disseminated histoplasmosis in an adopted infant from El Salvador.

UNLABELLED: An HIV-negative 4-month-old infant recently adopted from El Salvador was admitted for high fever. Hepatosplenomegaly, anaemia, leucopenia, thrombocytopenia, increased transaminases, and diffuse interstitial pulmonary infiltration were present on admission. Granulomas were seen in bone marrow and liver biopsies without any organism. Disseminated histoplasmosis was diagnosed 2 weeks later when bone marrow and blood cultures taken on admission became positive for Histoplasma capsulatum and when histoplasmic antigen was detected in blood. The outcome was good after treatment with amphotericin B followed by itraconazole which was administered for a 6-month period without significant toxicity. CONCLUSION: Disseminated histoplasmosis is very rarely seen in Europe but should be suspected in case of unexplained fever in immigrants from the endemic areas in the world, particularly when hepatosplenomegaly and pancytopenia are present. Bone marrow examination and culture, blood cultures, and antigen testing are the mainstays of the diagnosis.

Prolonged remission induced by cyclosporine in a patient with familial thrombocytopenia and enteropathy.

A 21-year-old patient, since the age of 16, presented a familial type of chronic autoimmune thrombocytopenia that responded only partially to various types of immunosuppressive treatment. His prolonged survival, compared to his decreased siblings, was complicated by the appearance of a severe enteropathy. High doses of corticosteroids induced a type 1 diabetes as a major side effect. The introduction of cyclosporine resulted in both a continuous complete clinical remission and a partial hematological remission and allowed the discontinuation of all other medication for 18 months. In uncommon complex autoimmune disease, cyclosporine may represent a safe and effective alternate therapy when other immunosuppressive agents have failed.

[Exertion syncope disclosing supravalvular mitral stenosis in an infant]. / Syncope à l'effort révélatrice d'un obstacle supramitral chez un nourrisson.

An infant with frequent upper airways infections presented syncopes during meals and weeping since the age of eleven months. Cardiac examination was always normal. At 14 months of age, an echocardiogram with colour Doppler demonstrated a severely stenotic isolated supramitral membrane with severe pulmonary hypertension. The membrane was immediately excised curing the malformation and suppressing definitively the syncopes, probably due to decreased cerebral blood flow during exertion. An echocardiogram should always be performed when syncopes remain unexplained in small children. It allows early diagnosis and treatment of congenital heart defects which do not have auscultatory findings especially those resulting in severe pulmonary venous obstruction.

Chronic metabolic alkalosis in an infant with cystic fibrosis.

A 6-month-old infant suffering from cystic fibrosis is reported. In spite of an apparently appropriate treatment and in absence of respiratory infection, the patient showed progressive anorexia, intermittent vomiting and weight loss. These non-specific signs and symptoms could all be explained by metabolic alkalosis and disappeared immediately after oral supplementation with sodium and potassium chloride. This unusual metabolic complication should be searched for in every cystic fibrosis infant with unexplained anorexia and failure to thrive.

[Glucagon-induced neutrophil mobilization (author's transl)]. / Mobilisation des neutrophiles induite par le glucagon.

In 19 normal subjects an increase in the number of circulating neutrophils was observed after intramuscular injection of 1 mg glucagon. The response began at the end of the 1st hour following the injection and persisted beyond the 8th hour, with a peak between 2 and 5 hours. No response was obtained in patients with bone marrow aplasia, either primary or associated with acute leukaemia. In 20 patients with chronic primary neutropenia, the degree of response was proportional to the percentage of medullary polymorphonuclears. A comparison between the kinetics of the glucagon-induced granulocyte response and that of the response induced by other neutrophil mobilizing agents suggested that glucagon acts by releasing granulocytes from the bone marrow reserve compartment. The finding that an infusion of dibutyryl cyclic AMP results in granulocyte mobilization suggests that the effects of glucagon are mediated by cAMP at cell level. Since the glucagon response test is harmless and gives rapid and pronounced results, it may be useful in investigation of patients with neutropenia. In addition, the glucagon-induced granulocyte mobilization might improve leucocyte yield in blood donors used for transfusion in agranulocytosis.

[Acute lymphoblastic leukemia in childhood: importance of sex as a prognostic factor (author's transl)]. / Leucémie aiguë lymphoblastique de l'enfant. Importance du sexe parmi les facteurs de pronostic.

In order to appreciate their prognostic value in acute lymphoblastic leukemia (ALL) of childhood, the main initial clinical and biological features have been analysed in 63 patients. In all cases, follow-up was longer than 3 years; in 43 it was longer than 5 years. The survival rate in first remission was 52% after 3 years and 30% after 5 years. No relapse was observed after 5 years. In this series, the presence of very high leukocyte count (less than 30 x 10 9/l or 30,000/mm3) and sex were the only significant prognostic factors. A poorer prognosis of ALL was indeed present in boys. The sex-related difference is independent from other prognostic factors and may require special attention.

[Blood picture in the early diagnosis of neonatal septicemia]. / La formule sanguine dans le diagnostic précoce de la septicémie du nouveau-né

In the search of a fast and simple laboratory test for the diagnosis of neonatal septicemia the blood pictures of 29 cases of septicemia were compared with normal values derived from 390 healthy newborns. In each case the leucocyte count, the neutrophil count, the band count and its percentage, the ratio of band forms to segmented neutrophils and the platelet count were analysed in the blood smear taken on the day of clinical suspicion of septicemia. The leucocyte count, the neutrophil count and the band count are within the pathological range in 70% of the cases. The percentage of band forms and the ratio of bands forms to segmented neutrophils are, with one exception, always abnormally elevated. Thrombocytopenia is present in only 60% of the cases. Among the different criteria the percentage of band forms is evidently the most practical and most significant one. Considering our normal values, the pathological range for this percentage is above 20% on the first day of life, above 15% on the second day and above 8% from the third day on.