Importância da Prova de Synacthen no Diagnóstico Diferencial de Pubarca Precoce

Introdução: Nos doentes com pubarca precoce, o gold-standard para o diagnóstico diferencial entre pubarca precoce idiopática (PPI) e a forma não clássica da hiperplasia congénita da suprarrenal (HCSR--NC) é a prova de Synacthen. Esta permite também estimar a reserva adrenal de cortisol nos doentes com HCSR-NC.Objetivos: Comparar as características clínicas e perfil hormonal basal dos doentes com pubarca pre-coce; avaliar a importância da prova de Synacthen no diagnóstico diferencial entre PPI e HCSR-NC e na determinação da reserva adrenal de cortisol. Material e métodos: Estudo transversal de doentes com pubarca precoce que realizaram prova de Synacthen. Resultados: Foram incluídos 43 doentes, com idade mediana de 7,5 anos (3,5-9,4), sendo 37 (86,0%) do sexo feminino. Na prova de Synacthen, 37 (86,0%) foram classificados como PPI e 6 (14,0%) como HCSR-NC.Não houve diferencças significativas entre os 2 grupos quanto às características clínicas e doseamentos basais de ACTH, cortisol e androgénios da suprarrenal. A 17-OHP basal e estimulada foi mais elevada nos doentes com HCSR-NC (p = 0,001 e p < 0,001, respetivamente) (basal: 4,62 ± 3,70 ng/ml [0,80-10,50];estimulada: 35,41 ± 24,87 ng/ml [12,0-80,2]) do que nos doentes com PPI (basal: 1,04 ± 0,77 ng/ml [0,22-3,80]; estimulada: 4,18 ± 1,71 ng/ml [1,0-8,96]). O cut-off basal habitualmente proposto (< 2,0 ng/ml) paraa distinção entre estes grupos não o permitiu em 2 doentes, que apenas foram diagnosticados após realização da prova de Synacthen. Dois doentes com HCSR-NC (33,3%) tiveram cortisol após estimulação< 18 g/dl, revelando necessidade de tratamento com glucocorticoide em stress. Os doentes com HCSR--NC com valores mais elevados de 17-OHP basal tiveram valores de cortisol mais baixos após estimulação(p = 0,004; r = -0,43).Conclusão: A realização desta prova foi útil para distinguir os doentes com HCSR-NC e PPI, pois nenhum valor de 17-OHP basal permitia fazer o diagnóstico diferencial definitivo. Em alguns doentes com HCSR-NCa prova revelou secreção inapropriada de cortisol em stress, contribuindo para a decisão terapêutica.

Thoracic Duct Decompression for Protein-Losing Enteropathy in Failing Fontan Circulation

An infrequent but devastating late complication of Fontan circulation is protein-losing enteropathy (PLE), which results from unbalanced lymphatic homeostasis. Surgical decompression of the thoracic duct by redirecting its drainage to the pulmonary venous atrium has been introduced recently as a possible treatment. This report describes a single-institution experience with this innovative procedure in 2 patients with failing Fontan circulation with PLE refractory to optimized medical therapy.

Identificação do Trypanosoma cruzi nos tecidos extracardíacos de portadores de miocardite crônica chagásica

Em autópsias realizadas durante 20 anos em portadores de miocardite crônica chagásica parasitologicamente comprovados, foi realizada uma pesquisa exaustiva das formas tissulares do Trypanosoma cruzi nas secções histológicas de vários órgãos. Os parasitos intracelulares foram encontrados nos tecidos extracardíacos em 11 casos (55%), a saber: tubo digestivo (10 vezes), adrenal (6 vezes) e em vários outros órgãos (1 vez cada). A presença dos parasitos se associava com discreta infiltração mononuclear focal, mas, o mais das vezes, não havia qualquer alteração. O estudo mostra que as formas de multiplicação do T. cruzi tendem a se distribuir amplamente na infecção crônica, mas como são escassas, o seu encontro depende de pesquisa minuciosa. Um fato interessante é que somente no miocárdio os parasitos aparecem associados com inflamação crônica, difusa, progressiva e fibrosante.

Acometimento da supra-renal associado à paracoccidioidomicose

A supra-renal foi estudada em 60pacientes com paracoccidioidomicose. Dentre eles, 10(16,7%) apresentavam alterações anatômicas ou funcionais das supra-renais. As lesões glandulares associaram-se à paracoccidioidomicose disseminada, com evolução da doença de pelo menos cinco anos sem tratamento e com hipotensão arterial sistêmica. Não houve associação entre alterações anatomoclínicas supra-renais e alterações raáiolôgicaspulmonares. Esses dados revelam a importância da avaliação sistemática da função supra-renal em portadores de formas disseminadas da paracoccidioidomicose.

Doença de Chagas aguda pós-transfusional sem miocardite

Trata-se de paciente do sexo feminino, com 59 anos de idade, procedente de Itaporanga (SP), diabética e nefropata crônica, internada em virtude de surtos de pielonefnte e insuficiência renal aguda. Dentre outras medidas terapêuticas, recebeu transfusão de sangue. Cerca de dois dias após a última transfusão (sangue oriundo de doador, posteriormente identificado como chagásico) encontraram-se formas tripomastigotas de Trypanosoma cruzi em lâmina preparada para execução de hemograma. Iniciou-se tratamento com Benzonidazol. A paciente cursou para, pleuropneumonia e de secreção purulenta cirúrgica isolou-se Klebsiella spp. A septicemia conduziu a paciente ao êxito letal. Nenhuma lesão tecidual foi observada no miocárdio, no sistema nervoso central, adrenal ou nos demais órgãos examinados.

Real-time labview assessment on adsorption systems

Dissertação para obtenção do Grau de Mestre em Engenharia Química e Bioquímica

Histoplasmosis presenting as addisonian crisis in an immunocompetent host

A 71-year-old man with presumptively treated pulmonary tuberculosis ten years earlier and previous alcoholism presented with adrenal insufficiency. HIV serology was negative. A computerized tomography scan of the abdomen showed enlarged right adrenal. He recovered after emergency treatment with hydrocortisone IV. Right adrenalectomy was performed. Histoplasmosis was diagnosed and the patient was treated with itraconazole, corticosteroid replacement, and discharged with good health.

Burkitt's lymphoma of the duodenum in a patient with AIDS

Non-Hodgkin's lymphoma of B-cell type is the second most common neoplasm after Kaposi's sarcoma, among patients with human immunodeficiency virus infection. Most non-Hodgkin's lymphoma cases that are associated with acquired immunodeficiency syndrome involve extranodal sites, especially the digestive tract and the central nervous system. We report a case of primary lymphoma of the duodenum in a patient with AIDS. Upper gastrointestinal endoscopy revealed pseudopolypoid masses found in the second portion of the duodenum. A complete diagnostic study including histological, immunohistochemical and virological analyses showed high-grade B-cell Burkitt's lymphoma. The Epstein-Barr virus genome was detected in biopsies by immunohistochemical and in situ hybridization.

Oral cavity lymphoma as secondary AIDS-defining neoplasm in a patient on HAART with immune reconstitution

Lymphomas of the oral cavity are a rare complication of advanced HIV/AIDS disease. The clinical appearance of these neoplasms includes masses or ulcerative lesions that involve the oral soft tissue and the jaw as the predominant manifestation. We report the case of a patient with AIDS who developed diffuse large B-cell non-Hodgkin’s lymphoma of the oral cavity during highly active antiretroviral therapy, with undetectable plasma viral load and immune reconstitution.

Choque séptico puerperal por Streptococcus β-hemolítico e síndrome de Waterhouse-Friderichsen

É relatado caso excepcional de puérpera de 15 anos com choque séptico pelo Streptococcus beta-hemolítico do grupo A e síndrome de Waterhouse-Friderichsen, observado à necropsia. São revistos aspectos do diagnóstico, patogênese e evolução da infecção (sepse) puerperal associada à hemorragia e insuficiência das supra-renais.

Malária álgida: um diagnóstico sindrômico

São relatados dois casos de pacientes com malária por Plasmodium falciparum, evoluindo com síndrome do choque. Receberam suporte hemodinâmico em unidade de terapia intensiva, sem uso de antibióticos, evoluindo com melhora. Malária álgida deve ser um diagnóstico sindrômico, de etiologia diversa (desidratação, infecção bacteriana, sangramento e/ou insuficiência adrenal).

Hydatid cysts in muscles: clinical manifestations, diagnosis, and management of this atypical presentation

ABSTRACTINTRODUCTION: Hydatid cysts are rarely detected in muscle tissue (0.7-0.9%), even in endemic countries. The aim of this study was to present information regarding the clinical manifestations, diagnosis, and management of muscle echinococcosis.METHODS: Twenty-two patients with hydatid cysts in the muscle were followed from January 2006 through December 2014.RESULTS: Twenty-four sites of muscle involvement were observed in the 22 patients. Fifteen (68%) of our patients were women, while seven (32%) were men. The mean age was 28.1 ± 15.4 (6-61) years. The most frequent locations were the thigh (27.2%) and the paravertebral region (13.6%). Most patients reported a painless slow-growing mass with normal overlying skin. Most (90.2%) cases were treated by surgical excision and fine-needle aspiration.CONCLUSIONS: Primary muscle hydatid cyst should be considered in the differential diagnosis in cystic masses of the muscular system without pain and localized enlargement of soft tissue, especially in endemic areas. Hydatid cyst should be investigated using serological tests and imaging modalities. If possible, total surgical excision of hydatid cyst in the muscle should be performed.

Pheochromocytoma treated by laparoscopic surgery

OBJECTIVE: To evaluate the results of the laparoscopic technique in the treatment of adrenal pheochromocytoma. METHOD: Ten patients, 7 men and 3 women, between 10 and 67 years of age (mean 48) with pheochromocytoma underwent transperitoneal laparoscopic adrenalectomy and were evaluated retrospectively, based on clinical, laboratory, and pathological diagnosis. In all cases there was a solid unilateral adrenal tumor, 5 on the left side and 5 on the right side, whose greater diameter varied from 7 to 80 mm (mean 32). Nine of the 10 patients were chronically hypertensive or had already had hypertensive crises. One patient was normotensive, but presented metabolic alterations suggestive of adrenergic hyperfunction. RESULTS: No deaths occurred in this series. There were two (20%) conversions to open surgery, one due to venous bleeding and one due to the difficulty of dissection behind the vena cava in a patient presenting a partially retro-caval tumor. Surgical time in the 8 non-converted cases ranged from 70 to 215 minutes (mean 136). One patient (10%) received blood transfusion, and another (10%) presented two complications - acute renal failure and a subcutaneous infection. Both had been converted to open surgery. None of the non-converted cases was transfused or presented complications. Hospital discharge occurred between the 2nd and 11th post-operative day (mean 3). The pathological exam of the surgical specimens confirmed the diagnoses of pheochromocytoma in all 10 cases, one of them associated with an aldosterone-producing cortical tumor. CONCLUSIONS: Laparoscopic adrenalectomy for selected patients presenting pheochromocytoma is feasible and provides good results.

Adrenocortical tumors: results of treatment and study of Weiss's score as a prognostic factor

PURPOSE: The differential diagnosis between benign and malignant adrenal cortical tumors circumscribed to the gland is controversial. One hundred and seven patients with adrenal cortex tumors (excluding those with primary hyperaldosteronism) were studied to assess the 5-year survival rate of adults, children, patients stratified by pathological stage, and patients stratified according to Weiss's score of <3 or >3. METHODS: The patients were evaluated both clinically and biochemically. One hundred and five patients underwent surgery and were classified pathologically as stages I, II, III, or IV. The tumors were weighed, measured, and classified according to Weiss's criteria and divided into 2 groups: <3 and >3. RESULTS: After 5 years, the survival rate was 77.5% for the whole group, 74.61% for the adults, 84.3% for the children, 100% for stage I, 83.9% for stage II, 33% for stage III, and 11.7% for stage IV groups. Additionally, after 5 years, 100% of the patients with tumors with Weiss's score <3 were alive compared to 61.65% of those with Weiss's score >3. The average weights of the tumors of score <3 and >3 were 23.38 g ± 41.36 g and 376.3 ± 538.76 g, respectively, which is a statistically significant difference. The average sizes of tumors of Weiss's score <3 and >3 were 3.67 ± 2.2 cm and 9.64 ± 5.8 cm, respectively, which is also a statistically significant difference. CONCLUSIONS: Weiss's score may be a good prognostic factor for tumors of the adrenal cortex. Additionally, there was a statistically significant difference between the average weight and size of tumors with benign behavior (Weiss's score <3) and those with malignant behavior (Weiss's score >3).