RESUMO
A young emaciated male, known case of celiac disease came with complaints of diarrhea along with 5kgs of weight loss in 3 months' time. He had severe electrolyte abnormalities along with low albumin, low calcium and a high phosphate with deranged liver function test. Ultrasound abdomen had shown fatty liver. Nutrition consult was sought and he was found to have a BMI of 6.8kg/m2. He was started on nutrition support along with supportive therapy, which resulted in weight gain and improvement in his condition.
Assuntos
Doença Celíaca/fisiopatologia , Emaciação/etiologia , Hepatopatia Gordurosa não Alcoólica/diagnóstico , Terapia Nutricional/métodos , Diarreia/etiologia , Emaciação/terapia , Humanos , Masculino , Hepatopatia Gordurosa não Alcoólica/terapia , Redução de Peso , Adulto JovemRESUMO
The association of weight loss and pediatric brain tumors that affect the diencephalon or brain stem with weight loss is a recognized, but not fully understood phenomenon. Tumors located in the hypothalamic region may induce the diencephalic syndrome (DS), which is characterized by profound emaciation with almost complete loss of subcutaneous fatty tissue. Tumors that compress or infiltrate the brain stem rarely cause both psychological disturbance and emaciation. The clinical presentation may be different, depending on the location of the lesion and age of the patient. In this report we present an unusual case of severe emaciation in a 4(9)/(12)-year-old girl with a juvenile pilocytic astrocytoma of the hypothalamic region and brain stem with neuroaxis dissemination. This case illustrates the importance of considering intracranial mass-lesions in the differential diagnosis of weight loss, psychological disturbance and atypical eating disorder. We discuss the importance of tumor multifocality and the role of patient age in the clinical presentation with reference to the literature.
Assuntos
Astrocitoma/patologia , Neoplasias Encefálicas/patologia , Emaciação/etiologia , Transtornos da Alimentação e da Ingestão de Alimentos/etiologia , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Astrocitoma/complicações , Astrocitoma/tratamento farmacológico , Neoplasias Encefálicas/complicações , Neoplasias Encefálicas/tratamento farmacológico , Tronco Encefálico/patologia , Pré-Escolar , Diagnóstico Diferencial , Emaciação/patologia , Emaciação/cirurgia , Transtornos da Alimentação e da Ingestão de Alimentos/patologia , Transtornos da Alimentação e da Ingestão de Alimentos/cirurgia , Transtornos de Alimentação na Infância/diagnóstico , Feminino , Humanos , Hipotálamo/patologia , Neoplasias da Medula Espinal/complicações , Neoplasias da Medula Espinal/secundário , Resultado do TratamentoRESUMO
Experimentally emaciated male rats were produced by a bilateral electrical destruction of a part of hypothalamus. In a typical case, when the animals were fixed by perfusion, dissected, and organs weighted, the body weight became 1/2 of the control in 10 weeks. The weight of the viscera (including the subserous fat) was more decreased in comparison with the controls than the weight of the body wall (including extremities and the subcutaneous fat). The weight of the liver became 1/3, the adrenal 1/4, the testis 1/6 and the seminal vesicle 1/19 of the control. Light and electron microscopic examinations showed atrophy and fatty degeneration in the liver, atrophy of the zona reticularis in the adrenal, failure of spermatogenesis, especially at its spermiogenetic stage, in the testis, and an apoptosis in glandular epithelial cells of the seminal vesicle. Two weeks after partial hypothalamus destruction, the weight of the body wall was more decreased in comparison with the controls than the weight of the viscera. Possible pathophysiological mechanisms are discussed. An experimental model of electron microscopical research of apoptosis are presented.
Assuntos
Glândulas Suprarrenais/ultraestrutura , Emaciação/patologia , Fígado/ultraestrutura , Glândulas Seminais/ultraestrutura , Testículo/ultraestrutura , Glândulas Suprarrenais/patologia , Animais , Apoptose , Modelos Animais de Doenças , Emaciação/etiologia , Hipotálamo/lesões , Hipotálamo/fisiopatologia , Fígado/patologia , Masculino , Microscopia Eletrônica , Ratos , Ratos Sprague-Dawley , Glândulas Seminais/patologia , Testículo/patologiaRESUMO
A tumor was resected from the third ventricle of a four years and eleven month old girl with the diencephalic syndrome. By light microscopy, it was diagnosed as a polar spongioblastoma . Its ultrastructural study was undertaken and the features were found to be distinctive and previously unreported. The organization of the neoplasm was similar to that of the hypothalamic neuro-endocrine systems: Cellular perivascular arrangement with intra-cytoplasmic microtubules and membrane bound dense-core granules.