Experience in the evaluation of children with hepatosplenomegaly at a teaching ambulatory, São Paulo, Brazil.

OBJECTIVES: Describe cases of children with hepatosplenomegaly (HS) attended at the General Pediatric Teaching Ambulatory (AGER) of Instituto da Criança, São Paulo, identifying the main causes, evolution, necessity for hospitalization and/or referral to specialists. METHODOLOGY: Retrospective analysis of the records of children presenting HS on admission at AGER from September 1, 1993 to August 31, 1996. RESULTS: Of the 89 children included (age range, 1 to 148 months; 24 months), 64 (72%) were referred from other services for HS investigation. Most common presenting complaints were: fever--39 (44%); pallor--26 (29%); weight loss--21 (24%) and jaundice--14 (16%). Main alterations noticed on physical examination were: pallor--47 (53%) and short stature--17 (19%). Anemia was diagnosed in 70 children (79%); 35 children (39%) had infections; 7 (8%) metabolic disorders and 5 (6%) neoplastic disorders. The most frequent infections were of the urinary tract--9 (10%) and hepatitis A--6 (7%). Thirty six children (40%) were referred to specialists, 17 of which were already diagnosed. CONCLUSIONS: Most of the children with HS present deficiency anemia associated with infections which the general pediatrician is able to diagnose. Persistence of unexplained HS for more than 2 months, especially when there is substantial volume enlargement or alteration in the organs consistency, is an indication for referral to specialists.

Deficiencia seletiva de IgA: estudo de 10 casos. / Selective IgA deficiency: study of 10 cases

Foram estudadas 10 criancas de dois a 15 anos de idade, sete meninos e tres meninas, com diagnostico de deficiencia seletiva de IgA. O diagnostico foi baseado em IgA serica igual ou menor do que 5,0 mg%, IgA salivar ausente, IgG e IgM normais, imunidade celular normal e percentual e numero absoluto de linfocitos T perifericos normais. As manifestacoes infecciosas recorrentes mais comuns foram sinusites (100%), rinofaringites (70%), pneumonias (70%), amigdalites (40%) e otites (30%).Asma grave foi observada em 70% dos casos e rinite alergica em 30%. Dois pacientes apresentaram dores articulares recorrentes, sem achados laboratoriais sugestivos de processo reumatico. Comenta-se o tratamento da doenca em questao

Triagem da competencia imunitaria de criancas com infeccoes pulmonares de repeticao. / Screening tests of immunity competence in children with repetitive pulmonary infections

Os autores estudaram 19 criancas, 13 do sexo masculino e 6 do feminino, com idades entre 5 meses e 7 anos, com infeccoes pulmonares de repeticao, as quais apos afastadas outras causas, foram aplicados testes de triagem da competencia imunitaria, que constavam de leucograma, dosagens de imunoglobulinas sericas, porcentagem de linfocitos T e B, testes cutaneos de hipersensibilidade tardia e dosagem de C3 e C4. Foram encontrados 4 casos de deficiencia seletiva de IgA, sendo 2 casos transitorios; 3 casos de provavel hipogamaglobulinemia prolongada e 1 caso de deficiencia seletiva de IgM. Os testes de triagem aplicados serviram para diagnosticar as imunodeficiencias mais comuns na infancia que podem estar associadas a infeccoes pulmonares de repeticao