Endocrine disorders in pediatric - onset Langerhans Cell Histiocytosis.

Langerhans Cell Histiocytosis (LCH) is a rare disorder with a great variety of clinical manifestations. The purpose of this retrospective study was to evaluate the pattern and the long-term course of clinical, laboratorial and radiological findings in pediatric-onset LCH. We reviewed 46 children with histological diagnosis of LCH. Ten children (22%) showed endocrine disorders. Central diabetes insipidus (DI) was observed in all ten patients; GH deficiency was confirmed in four and hypogonadism in two children. There were no adrenal, prolactin or thyroid axis abnormalities. Obesity was observed in three patients. Eight patients showed soft tissue infiltration and five bone involvement. The MRI showed a lack of posterior pituitary bright spot in all DI patients; infundibular infiltration (II) associated or not with sellar or supra-sellar mass was observed in 4 patients. We conclude that the investigation of LCH, a multi-systemic disease, should include central nervous system images. The presence of II and/or DI should raise the diagnosis of LCH. Complete endocrine evaluation, allowing an early hormone therapy, is required to obtain a better quality of life in children with LCH.

Midarm computerized tomography fat, muscle and total areas correlation with nutritional assessment data.

BACKGROUND AND OBJECTIVES: Correlated nutritional assessment data (anthropometric, bioimpedance and biochemical) with computerized tomography (CT) of total, muscle and fat midarm areas. Total body fat and fat-free mass were estimated using bioimpedance. Daily urinary urea and creatinine were also quantified. In all, 28 subjects (13 males, 15 females) were evaluated and, they were clinically divided in obese, malnourished and control subjects. DESIGN: Quantification of total, fat muscle midarm areas by tomography and anthropometry and total body fat and free-fat mass by bioimpedance. RESULTS: CT values were 29% higher for fat area and 4-5% lower for total and muscle midarm areas compared against anthropometric data. The midarm skinfold thickness highly correlated with CT fat midarm area. Total body fat and free-fat mass bioimpedance data showed significant correlation with CT midarm data. Urinary creatinine correlated with CT muscle midarm area. CONCLUSION: Utilization of anthropometry can lead to error estimation of fat and fat-free arm areas and that bioimpedance gives fair correlation between total body and CT midarm measurements.

Polycystic hydatid disease (Echinococcus vogeli). Treatment with albendazole.

Six patients with polycystic hydatid disease (PHD) were treated with 10 mg kg-1 day-1 albendazole. One patient was treated continuously for eight months and another for three months. In three other patients treatment was discontinuous, consisting of a series of at least three 30-day cycles separated by 15 days without treatment. The last patient was treated continuously with 12 mg kg-1 day-1 albendazole for 51 days and then with three 30-day cycles of treatment with 10 mg kg-1 day-1 separated by 15-day drug-free intervals. Follow-up ranged from 10-30 months. Considerable clinical improvement and cyst reduction or disappearance occurred in four patients. Clinical improvement, but no changes in the hepatic alterations detected by computerized tomography, occurred in the other two patients, although a pulmonary cyst disappeared in one of them. Adverse effects were proteinuria, alopecia, leucopenia, itching and discrete elevation in aspartate transaminase, all of them reversed after the end of treatment. These results indicate that albendazole is effective for the treatment of PHD.

Polycystic hydatid disease (Echinococcus vogeli). Clinical, laboratory and morphological findings in nine Brazilian patients.

Polycystic hydatid disease occurs in neotropical zones and is caused by Echinococcus vogeli. The paca, a wild rodent, is the intermediate host and the final host is the dog. Seven cases of polycystic hydatid disease autochthonous to the Brazilian Amazon region are described. The disease was polycystic in all cases and diagnosis was based on anatomopathological findings. E. vogeli was identified by the shape and dimensions of the rostellar hooks. The liver was the organ most often involved (6/7), followed by the lungs (2/7) and mesentery (2/7), spleen (1/7) and pancreas (1/7). The main clinical manifestations were abdominal pain, hepatomegaly, jaundice, weight loss, anemia, fever, hemoptysis, palpable abdominal masses and signs of portal hypertension. Hepatic calcifications were detected in four cases. Two cases from the hinterland of the State of São Paulo are also reported. Both had calcified round structures in the liver, highly suggestive of calcified polycystic hydatids. The aim of the present report was to report on this relatively unknown hydatid disorder of Tropical America and to disseminate its clinical, ultrasound and radiological features.

Polycystic hydatid disease (Echinococcus vogeli): clinical and radiological manifestations and treatment with albendazole of a patient from the Brazilian Amazon region.

We present a case of polycystic hydatid disease produced by Echinococcus vogeli in a tapper of rubber trees from the State of Acre, Brazil. The most relevant clinical data were pain, increased abdominal volume caused by palpable painful masses, fever and poor clinical condition. Laboratory tests showed anemia, eosinophilia, hypoalbuminemia, hypergammaglobulinemia and increased plasma levels of alkaline phosphatase. Computerized tomography revealed diffuse cysts throughout the peritoneal cavity up to the pelvis, and inside the liver, pancreas and spleen. Anatomopathological examinations of cysts obtained by laparotomy confirmed the etiological diagnosis. Treatment with 10 mg/kg Albendazole for 6 months caused complete regression of the disease.

Hiperdesenvolvimento do seio esfenoidal. / Hyperdevelopment of sphenoid sinus

Hiperdesenvolvimento do seio esfenoidal com recesso lateral amplo e simetrico, delineando as asas maiores do esfenoide, foi detectado em um paciente submetido a exame neuroradiologico de rotina.Trata-se de rara variacao anatomica que pode simular anormalidade radiologica