Kawasaki disease and juvenile systemic lupus erythematosus.

Kawasaki disease (KD) is a common vasculitis in childhood. To the authors' knowledge, only one case of juvenile systemic lupus erythematosus (JSLE)-like onset mimicking KD and another case of KD and JSLE association have previously been described. However, the prevalence of this association of the two diseases was not reported. Therefore, over 27 consecutive years, 5419 patients were followed at the Pediatric Rheumatology Unit and 271 (5%) of them met the ACR classification criteria for JSLE. Two (0.7%) of them were female. These also had KD according to European League against Rheumatism / Paediatric Rheumatology European Society (EULAR/PReS) consensus criteria and are described in this report. One case was a 13-year-old who presented all six KD criteria. Echocardiogram showed pericardial effusion, dilatation and tortuosity of right and left coronary, and her symptoms promptly improved after treatment with intravenous immunoglobulin (IVIG). Lupus diagnosis was established a few days later. Another case was a 4-year-old who had also met all six KD criteria, with improvement after IVIG, and lupus diagnosis was made 1 year later. In conclusion, the frequency of the association between these two autoimmune diseases was rare. The occurrence of a second autoimmune systemic disease in a patient with a history of KD should also be considered. Furthermore, the initial presentation of lupus may mimic KD.

[Measles seroprevalence among pediatricians in a teaching hospital]. / Soroprevalência de sarampo entre pediatras de um hospital-escola.

OBJECTIVE: A measles outbreak occurred in S. Paulo state, during 1996 and 1997, resulting in 20,921 cases. Forty seven percent of the cases occurred in people between 20 and 29 years of age, and one of the control strategies of the Department of Health was the vaccination of health care workers. The prevalence of antibodies against measles among the hospital pediatricians was investigated. METHODS: One hundred and fifty samples were taken from volunteer pediatricians to test for measles antibodies using ELISA. A questionnaire about their having had measles and the vaccine was filled out. RESULTS: Of the 150 doctors, 122 (81.4%) were female and 28 (18.6%) male, of between 23 and 46 years of age (mean and median 27 years). The majority (98%-147/150) had protective levels of antibodies against measles (>100 UI/ml); 118 (80.3%-118/147) without and 29 (19.7%-29/147) with a history of measles. Only 3 pediatricians (2%-3/150), had negative serology, 2 without and 1 with a history of measles. Out of the 118 without history of measles, 79 (67%-39/118) in spite of the protective level of antibodies against measles, did not know if they, had been vaccinated. Out of the 79 vaccinated pediatricians, 64 (81%-64/79), had been vaccinated 25 years before, and still maintained protective levels of antibodies. Of the 3 doctors with negative serologies only one declared that he had been vaccinated. CONCLUSIONS: Measles seroprevalence among pediatricians of this hospital is high, especially due to preceding vaccination. On the other hand, the 2% of pediatricians with negative serology, in an epidemic situation could constitute a significant population for the acquisition and dissemination of the disease.

[Positive conversion of AARB results in gastric lavage, in a child with AIDS and tuberculosis, after endoscopic endobronchial puncture]. / Positivação da pesquisa de baar em lavado gástrico, em criança com AIDS e tuberculose, após realizaçao de punção endobrônquica via endoscópica.

A case of tuberculosis in a pediatric AIDS patient, submitted to bronchoscopy and transbronchial biopsy as a part of chronic pulmonary disease evaluation, is presented. No AFB (acid-fast bacillus) detection was identified by gastric lavage before the bronchoscopy, nevertheless after the bronchoscopic procedure the patient became contagious, and persisted with positive baciloscopy until the 56th day of therapy.

Mycobacterium bovis dissemination (BCG strain) among immunodeficient Brazilian infants.

This study reports Mycobacterium bovis (BCG strain) dissemination in four primary immunodeficient (PID) patients. Their diagnoses were: chemotaxis defect, cellular immunodeficiency, combined immunodeficiency and chronic granulomatous disease (CGD). Patients were vaccinated with BCG (Moreau strain) between 17 days and 4 months of age. Identification of the BCG strain was performed in many sites, such as bone marrow, ganglionic mass, cerebrospinal fluid and in the site of vaccination. The first therapeutic schedule included rifampicin, isoniazid and ethambutol; no uniform treatment could be used for these patients. Therapy was adjusted as required on the basis of BCG sensitivity results. This study shows that PID patients are susceptible to BCG dissemination and demonstrates the importance of an early diagnosis for their prognosis.

[Congenital Malaria. Case reports and a brief review of literature]. / Malária congênita. Descrição de casos e breve revisão da literatura.

Two cases of congenital malaria due to Plasmodium vivax and Plasmodium falciparum respectively are reported. The children were born in São Paulo and Paraná to mothers from endemic area of malaria. The mothers had malaria during pregnancy. The children were admitted with severe anemia and hepatosplenomegaly. Despite congenital malaria is being considered a rare event it is a preventable disease and these cases should draw attention to the importance of adequate treatment during pregnancy. Clinical manifestations and management of congenital malaria are also discussed.

[Biliary tract dilatation and abnormal choledocho-pancreatic ductal junction in children]. / Dilatação da via biliar e anomalia da junção dos ductos colédoco e pancreático principal na criança.

Nine children aged between 2 months and 11 years, who had congenital dilatation of extrahepatic biliary tract, with or without intrahepatic dilatation, are presented. The radiological studies performed in eight patients demonstrated abnormal choledochopancreatico ductal junction. Thus, a long common channel is formed out of the duodenal wall, leading to a continuous reflux of pancreatic juice into the biliary system, chronic inflammation of biliary tract wall, fibrosis of muscular layer and finally different types of dilatations. The main clinical findings were upper abdominal pain, vomiting, or obstructive jaundice. In only one child the dilatation was cystic type and the clinical manifestation was palpable abdominal mass. In eight patients the initial diagnosis of biliary tract dilatation was made through abdominal ultrasound and in one case by direct visualization through emergent exploratory laparotomy. The final confirmation of the presence of long common channel was made by preoperative endoscopic retrograde cholangiopancreatography or intraoperative cholangiogram. The radiologic investigation was not performed in only one case due to bad clinical conditions. The surgical treatment indicated to all cases was Roux- en- Y hepatic- jejunostomy. The late follow up demonstrated that all patients are well and assymptomatic.

Vesícula biliar hidrópica na síndrome linfonodomucocutânea (Sindrome de Kawasaki): relato de caso / Hydropic blister gallbladder in mucocutaneous lymph node syndrome (Kawasaki sindrome): a case report

Uma possível manifestaçao da síndrome linfonodomucocutânea (SLMC), ou síndrome de Kawasaki, é a hidropsia da vesícula biliar. Pode apresentar-se com sintomas abdominais previamente ao estabelecimento clínico da síndrome ou aparecer já no curso dela. Existem poucos relatos desta associaçao, com variaçao de incidência estimada entre 3 por cento e 13,7 por cento. Neste relato descrevemos um caso de SLMC numa criança de cinco anos de idade, que na evoluçao apresentou sintomas e sinais de envolvimento abdominal, com ultra-sonografia e tomografia computadorizada de abdome demonstrando a presença de vesícula biliar hidrópica. Tal patologia deve ser lembrada principalmente no diagnóstico diferencial de colangite acalculosa em crianças, devido ao crescente número de casos de SLMC identificados em nosso meio.

[Common bile duct syndrome in children]. / "Síndrome do canal comum" na criança.

Two children bile duct dilatation due to anomalous junction of the pancreato-biliary ductal system are reported. A long common channel comes out of the duodenal wall and, thus, reflux of pancreatic juice into the biliary tree and dilatation of biliary ducts may occur. Clinical findings were upper abdominal pain, vomiting, or jaundice. Diagnosis was accomplished by abdominal ultrasonography and endoscopic or intra-operative colangiography. Both cases were treated by Roux-en-Y hepatico-jejunostomy, the best technique for these cases. Follow-up showed that the patients are well and free of symptoms.