RESUMO
We are presenting the clinical features, diagnostic work up and treatment of acromegaly caused by Growth hormone releasing hormone (GHRH) secreting neuroendocrine tumor (NECT) in a case of multiple endocrine neoplasia type 1 (MEN-1). A 36 year old man, known case of MEN-1 presented with acromegalic features. He has high IGF-1, GH and very high GHRH levels with a pancreatic head tumor and pituitary mass. He had high GHRH arteriovenous gradient across pancreatic tumor and underwent tumor resection, Post operative GHRH level fell dramatically. Tumor had high GHRH m-RNA level. Acromegalic patients with MEN-1 should be screened for ectopic GHRH secretion. Measurement of GHRH arteriovenous gradient across NECT or mRNA for GHRH in resected tumor can confirm the ectopic source. Treatment of choice is surgical resection of the tumor. Somatostatin analogue is an alternative because of its dual action in the pituitary gland and the NECT. Life long surveillance is needed as recurrence chance is high.
Assuntos
Acromegalia/diagnóstico , Hormônio Liberador de Hormônio do Crescimento/metabolismo , Neoplasia Endócrina Múltipla/diagnóstico , Tumores Neuroendócrinos/diagnóstico , Neoplasias Pancreáticas/diagnóstico , Acromegalia/etiologia , Acromegalia/metabolismo , Adulto , Humanos , Fator de Crescimento Insulin-Like I , Masculino , Neoplasia Endócrina Múltipla/complicações , Neoplasia Endócrina Múltipla/metabolismo , Neoplasia Endócrina Múltipla/cirurgia , Tumores Neuroendócrinos/complicações , Tumores Neuroendócrinos/metabolismo , Tumores Neuroendócrinos/cirurgia , Neoplasias Pancreáticas/complicações , Neoplasias Pancreáticas/metabolismo , Neoplasias Pancreáticas/cirurgiaAssuntos
Adenoma/patologia , Neoplasias das Paratireoides/patologia , Adenoma/complicações , Humanos , Hipercalcemia/etiologia , Hiperparatireoidismo Primário/etiologia , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Neoplasias das Paratireoides/complicações , Neoplasias das Paratireoides/diagnóstico por imagem , Cintilografia , Compostos Radiofarmacêuticos , Tecnécio Tc 99m Sestamibi , Tomografia Computadorizada por Raios XAssuntos
Fraturas do Fêmur/diagnóstico por imagem , Dor/diagnóstico por imagem , Coxa da Perna/diagnóstico por imagem , Absorciometria de Fóton , Idoso , Diagnóstico Diferencial , Difosfonatos/efeitos adversos , Difosfonatos/uso terapêutico , Feminino , Fraturas do Fêmur/induzido quimicamente , Fraturas do Fêmur/complicações , Humanos , Osteoporose Pós-Menopausa/complicações , Osteoporose Pós-Menopausa/tratamento farmacológicoRESUMO
OBJECTIVE: To propose an approach for the diagnosis of secondary adrenal insufficiency (AI) by presentation of 2 clinical cases and review of the literature. METHODS: We describe 2 patients who were considered to have a normal hypothalamic-pituitary-adrenal axis on the basis of an appropriate response to the high-dose (250 microg) cosyntropin stimulation test (HST), with use of a cutoff value of 20 microg/dL. Our first patient had undergone resection of a 4-cm pituitary tumor a few months previously, and the second patient had hyponatremia with empty sella syndrome. Both patients, however, had strong clinical evidence suggestive of secondary AI. On testing by the insulin tolerance test (ITT) in the first patient and the overnight metyrapone test (OMT) in the second patient, secondary AI was diagnosed. We reviewed the literature to compare the utility of the different tests for the diagnosis of secondary AI. RESULTS: An 8 AM serum cortisol value less than 5 microg/dL or above 13 microg/dL and a stimulated cortisol level less than 16 microg/dL on both the low-dose cosyntropin stimulation test (LST) and the HST as well as above 22 microg/dL on the LST and above 30 microg/dL on the HST can reliably predict the functional status of the hypothalamic-pituitaryadrenal axis in chronic secondary AI. Values between these cutoff points may necessitate further assessment with the OMT or ITT based on clinical suspicion. CONCLUSION: We recommend a 3-step diagnostic approach, with the first 2 steps performed together-starting with the 8 AM basal cortisol determination in conjunction with either the LST (preferably) or the HST. In cases of an indeterminate response coupled with a strong clinical suspicion, the final step should be definitive testing with the OMT or ITT.
Assuntos
Insuficiência Adrenal/diagnóstico , Insuficiência Adrenal/etiologia , Doenças da Hipófise/fisiopatologia , Adenoma/complicações , Adenoma/cirurgia , Insuficiência Adrenal/sangue , Insuficiência Adrenal/fisiopatologia , Adulto , Idoso , Cosintropina/administração & dosagem , Síndrome da Sela Vazia/complicações , Síndrome da Sela Vazia/fisiopatologia , Feminino , Humanos , Hiponatremia/complicações , Sistema Hipotálamo-Hipofisário/efeitos dos fármacos , Sistema Hipotálamo-Hipofisário/fisiopatologia , Insulina/efeitos adversos , Neoplasias Hipofisárias/complicações , Neoplasias Hipofisárias/cirurgia , Sistema Hipófise-Suprarrenal/efeitos dos fármacos , Sistema Hipófise-Suprarrenal/fisiopatologiaRESUMO
Primary hyperparathyroidism (HPT) has catabolic effects on cortical bone and anabolic effects on cancellous bone with overall deleterious effects on skeleton. Primary HPT is associated with increased fracture risk both at the cancellous bone-enriched spine and the cortical bone-enriched distal one third of the radius. This risk is reversed by parathyroidectomy.