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OBJECTIVES: Adrenal insufficiency is often diagnosed by short synacthen test using intravenous Injection Synacthene, which is not marketed in India officially. To overcome this problem this study was planned to validate and use Acton Prolongatum (Ferring pharmaceuticals) as intramuscular ACTH stimulation test for evaluation of adrenal function. METHODS: This study was planned in two groups. First group called validation group, was studied for validation of intramuscular ACTH test and second group called study group, was evaluated for efficacy of intramuscular ACTH test to detect adrenal insufficiency. Twenty five units of ACTH (Acton Prolongatum) was injected intramuscularly and blood sample was collected after 60 minutes for estimation of cortisol. All subjects with one hour post ACTH cortisol < 18.0 microg/dl were diagnosed as having adrenal insufficiency. RESULTS: This study was carried out in 61 subjects in validation group and 89 patients in study group. Basal and post ACTH serum cortisol were within normal range in healthy adults, patients with diabetes mellitus and primary hypothyroidism in validation group. Basal cortisol ranged from 4.67-18.39 microg/dl and post ACTH serum cortisol ranged from 20.01-44.95 microg/dl in these groups. Patients with known adrenal insufficiency had significantly low basal cortisol level than controls (2.86 +/- 2.66 vs. 10.35 +/- 4.37 microg/dl, p < 0.001), and post ACTH serum cortisol was < 18.0 microg/dl in all. Among study group 37 patients (41.6%) were diagnosed as adrenal insufficiency using post ACTH cortisol levels. Basal cortisol (< 3.0 microg/dl) could detect only 60% of these patients. Basal cortisol level has sensitivity of 60% and specificity of 100% to detect AI when compared to ACTH stimulated cortisol levels. CONCLUSION: Intramuscular ACTH test using Acton Prolongatum is effective in evaluation of adrenal function in all suspected cases of primary or secondary adrenal insufficiency. Basal cortisol has poor sensitivity to diagnose AI.
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Testes de Função do Córtex Suprarrenal , Insuficiência Adrenal/diagnóstico , Hormônio Adrenocorticotrópico , Insuficiência Adrenal/sangue , Hormônio Adrenocorticotrópico/administração & dosagem , Adulto , Diabetes Mellitus Tipo 2/sangue , Feminino , Humanos , Hidrocortisona/sangue , Hipotireoidismo/sangue , Índia , Injeções Intramusculares , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Reprodutibilidade dos Testes , Adulto JovemRESUMO
Hyperthyroidism is rare in paediatric age group, the most common cause being Graves' disease. Very few cases of autonomous functioning thyroid nodule (AFTN) as a cause of paediatric hyperthyroidism have been reported. We herein report a case of AFTN in a 3 and a half-year-old boy who had become symptomatic with a swelling in the neck and hypermetabolic features at the age of 8 months. He was successfully treated with 131I radioablation, which rendered him euthyroid.
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Hipertireoidismo/etiologia , Hipertireoidismo/radioterapia , Radioisótopos do Iodo/uso terapêutico , Nódulo da Glândula Tireoide/complicações , Pré-Escolar , Humanos , MasculinoRESUMO
We report an association of sacral agenesis and hypopituitarism in a child born of a diabetic mother. The child presented with short stature and evaluation revealed sacral agenesis, growth hormone deficiency (GHD) and adrenal insufficiency. This association might be important as short stature in children with sacral agenesis is usually attributed to mechanical factors. Early detection of GHD and treatment might help the child in gaining height.
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Nanismo Hipofisário/diagnóstico , Gravidez em Diabéticas , Sacro/anormalidades , Insuficiência Adrenal/sangue , Insuficiência Adrenal/tratamento farmacológico , Adulto , Criança , Nanismo Hipofisário/sangue , Nanismo Hipofisário/tratamento farmacológico , Feminino , Hormônio do Crescimento Humano/deficiência , Hormônio do Crescimento Humano/uso terapêutico , Humanos , Hidrocortisona/uso terapêutico , Masculino , Gravidez , Resultado do TratamentoRESUMO
INTRODUCTION: Hematopoietic stem cell transplant (HSCT) is frequently complicated by endocrine abnormalities and loss of bone mass. This prospective study was conducted to evaluate the bone loss post-HSCT. MATERIALS AND METHODS: A total of 50 patients was evaluated pretransplantation, and 25 had HSCT (17 males, 8 females; 19 allogenic, 6 autologous). Bone mineral density (BMD) and bone markers were measured at baseline, 3-6 months and 12 months. RESULTS: The mean age and body mass index were 25.1 ± 16.3 years and 19.4 ± 4.5 kg/m(2), respectively. There were 15 adults (60%), and 10 adolescents (40%). There was a significant decline in BMD from the baseline at total femur (-8.7%; P < 0.0001), femoral neck (-5.0%; P = 0.003), femoral trochanter (-6.0%; P = 0.001), and Ward's triangle (-9.9%; P < 0.0001) at 6 months posttransplantation. From the 6 months to 12 months, there was a significant improvement in BMD at above sites except at Ward's triangle. The decline in BMD was nonsignificant at the whole body (-0.3%, P = 0.748) and the lumbar spine (-2.7%, P = 0.130) at 6 months posttransplant. Younger patients with allogenic graft and steroid use are more likely to have significant loss of BMD at hip posttransplant. Serum osteocalcin decreased, and N-telopeptide increased at 3-6 months, which return to baseline at 1-year posttransplant. CONCLUSIONS: A significant bone loss is observed at 6 months in patients with post-HSCT. The bone loss occurs predominantly at cortical bone. There is recovery of bone mass at 12 months posttransplant except at Ward's triangle. Bone loss after HSCT is multifactorial.
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A 27-year-old male was admitted with diabetic ketoacidosis and altered sensorium with slurring of speech and ataxia. He was managed with intravenous insulin and fluids and later shifted to basal bolus insulin regimen and during further evaluation was diagnosed to be suffering from primary hypothyroidism and adrenal insufficiency. He was started on thyroxin replacement and steroids only during stress. After three months of follow up he was clinically euthyroid. His glycemic control was adequate on oral anti-hyperglycemic drugs and adrenal insufficiency recovered. However, his thyrotropin levels were persistently elevated on adequate replacement doses of thyroxin. His repeat TSH was estimated after precipitating serum with polyethylene glycol which revealed normal TSH. Here we report reversible adrenal insufficiency with hypothyroidism with falsely raised TSH because of presence of heterophile antibodies in a case of poly glandular endocrinopathy syndrome.
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Association of Grave's ophthalmopathy with hyperthyroidism is well known, and it has also been reported in euthyroid or hypothyroid autoimmune thyroiditis, which rarely requires treatment. Here, we report a case of bilaterally symmetrical severe corticosteroid-resistant hypothyroid Grave's ophthalmopathy successfully treated with rituximab.
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Nelson's syndrome refers to a clinical spectrum arising from progressive enlargement of pituitary adenoma and elevated adrenocorticotrophic hormone after total bilateral adrenalectomy for Cushing's disease comprising of hyperpigmentation, visual field defects which can be life threatening. We report here a 50-year male presenting with rapid onset of Nelson's syndrome with an unusual finding of bilateral oculomotor palsy mistakenly treated as ocular myasthenia.
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Polyostotic fibrous dysplasia is a rare non-inheritable genetic disease due to mutation in GNAS gene. Here we present two adults who were accidentally detected lytic lesions in spine and after extensive evaluation for malignancies; was diagnosed on biopsy. Current concept of the disease and management is discussed.
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A 19-year-old girl presented with classical features of Cushing's syndrome. Endocrinal evaluation was consistent with pituitary source of ACTH; but imaging showed normal pituitary. Bilateral inferior petrosal sinus sampling confirmed the diagnosis. A successful remission was achieved after adenomectomy by transphenoidal route. Histopathological examination was consistent with pituitary oncocytoma and immunohistochemistry was positive for synaptophysin, chromogranin, neuron specific enolase, S-100, ACTH, prolactin, and GH.
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BACKGROUND: In recent times, there are reports of adrenal dysfunction in whole spectrum of liver disease. Adrenal insufficiency (AI) has been shown to correlate with progression of liver disease. Hence this study was conducted to assess adrenal function in subjects with acute liver disease (ALD), chronic liver disease (CLD) and post liver transplantation (LT). MATERIAL AND METHODS: This study included 25 healthy controls, 25 patients of ALD, 20 subjects of CLD with Child-Pugh stage A (CLD-1) and 30 with Child-Pugh stage B or C (CLD-2), and 10 subjects with LT. All subjects were assessed clinically, biochemically and for adrenal functions. RESULTS: AI was present in 9 (34.6%) patients with ALD, 20 (40%) patients with CLD and 4 (40%) in subjects with LT. AI was more common in CLD-2 (18 patients - 60%) than CLD-1 (2 patients - 10%). All patients with chronic liver disease had significantly lower basal cortisol (8.8±4.8, P=0.01), stimulated cortisol (18.2±6.3, P <0.00001) and incremental cortisol (9.4±4.6, P <0.00001) as compared to controls. There was increase in percentage of subjects with adrenal dysfunction with progression of liver disease as assessed by Child-Pugh staging. AI was predicted by lower levels of serum protein, serum albumin, total cholesterol and HDL cholesterol and higher levels of serum bilirubin and INR. Adrenal functions showed recovery following liver transplantation. CONCLUSIONS: AI forms important part of spectrum of acute and chronic liver disease. Deterioration of synthetic functions of liver disease predicts presence of AI, and these patients should be evaluated for adrenal dysfunction periodically.
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Multiple endocrine neoplasia-1 (MEN1) is an autosomal dominant syndrome with classic triad of parathyroid hyperplasia, pancreatic neuroendocrine tumors, and pituitary adenomas. Other recognized manifestations include carcinoid, cutaneous or adrenocortical tumors. It is commonly presented with clinical features related to parathyroid, pancreas or pituitary lesions. Here, we have presented a case that had virilization and biochemical Cushing's syndrome due to adrenocortical carcinoma as presenting feature of MEN1. Cushing's syndrome in MEN1 is an extremely rare and usually late manifestation and most cases are due to corticotropin-producing pituitary adenomas. Although Cushing's syndrome generally develops years after the more typical manifestations of MEN1 appear, it may be the primary manifestation of MEN1 syndrome particularly when related to adrenal adenoma or carcinoma.
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INTRODUCTION: Endocrine complications are common after hematopoietic stem cell transplant (HSCT). Although HSCT is performed at various centers in India, no study is available for endocrine dysfunctions among them. This study was carried out with the objective to evaluate endocrine dysfunction among patients undergone HSCT in the past. MATERIALS AND METHODS: We carried out a cross-sectional study in a 50 post-HSCT recipients (39 allogenic, 11 autologous). All relevant data were collected from patient's records. Samples for hormonal estimation were collected and stimulation tests for cortisol and growth hormone were interpreted based on peak values achieved during insulin tolerance test. RESULTS: The mean age of patients was 26.3 ± 16.9 years (range 4-74). Adrenal insufficiency (AI) was present in 60%, hypergonadotropic hypogonadism (HH) in 60%, growth hormone deficiency (GHD) in 54%, hypothyroidism in 4%, hyperprolactinemia in 4%, new onset diabetes after transplant in 4%, and impaired fasting glucose in 6%. Multiple endocrine complications were common. GHD was present in 77% of children (n = 22) although height standard deviation score was not statistically different compared to those who didn't have GHD. HH was present in 36% of children. In adults (n = 28), 36% had GHD, all females had HH, and 89% of males had HH. Germ cell dysfunction with compensated Leydig cell dysfunction was the most common pattern of HH in males. Fifteen patients had graft versus host disease (GVHD). GVHD had no bearing on development of endocrine deficiencies. AI was related to duration after and type of transplant, but was unrelated to steroid intake. CONCLUSIONS: Endocrine manifestations are common after HSCT; they can occur as early or late complications. All HSCT recipients should have endocrine evaluation as per prevailing guidelines.
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Graves' disease in childhood is a rare clinical entity. The authors report a case of Graves' disease in a 3-year-old child, who had opththalmopathy, accelerated growth, cervical lymphadenopathy, hepatosplenomegaly and congestive cardiac failure; and responded well to treatment.
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Doença de Graves/diagnóstico , Transtornos do Crescimento/etiologia , Insuficiência Cardíaca/etiologia , Doenças Linfáticas/etiologia , Pré-Escolar , Doença de Graves/complicações , Oftalmopatia de Graves/diagnóstico , Transtornos do Crescimento/diagnóstico , Insuficiência Cardíaca/diagnóstico , Hepatomegalia/diagnóstico , Hepatomegalia/etiologia , Humanos , Doenças Linfáticas/diagnóstico , Masculino , Esplenomegalia/diagnóstico , Esplenomegalia/etiologiaRESUMO
An 82-year-old male, a known case of severe osteoporosis with vertebral fracture and prostatic carcinoma, was treated with gonadotropin releasing hormone analogue, calcium carbonate, cholecalciferol sachet and injection teriparatide. His diet consisted of milk and curd. He developed altered behavior and generalized weakness, and on investigation, hypercalcemia, hypokalemia, and metabolic alkalosis with low parathyroid hormone levels were detected. Injection teriparatide was stopped and he was managed with forced saline diuresis and injection zoledronic acid. He was diagnosed as a case of milk alkali syndrome in whom teriparatide and prolonged immobilization played a permissive role in the development of hypercalcemic encephalopathy.
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OBJECTIVES: There is paucity of information regarding clinical profile of hypopituitarism from India. We report the clinical profile of hypopituitarism from a tertiary center in North India. MATERIALS AND METHODS: This study was carried out in patients attending our endocrine center between January 2010 and December 2011. All new patients were studied prospectively and those registered before January 2010 retrospectively. Relevant clinical, hormonal, and imaging data were collected. Dynamic testing for pituitary functions was carried out as necessary. Hormonal deficiencies were defined as per prevailing recommendations. RESULTS: This study included 113 subjects. The mean age was 38.6 ± 17.8 years (range, 4 - 76 years). There were 78 (69%) males and 35 females (31%). There were 22 subjects aged ≤18 years (childhood and adolescence) and 91 adults (>18 years). Visual disturbances were the most common presenting complaint (33%), though headache was the most common symptom (81%). Fifteen percent presented with pituitary apoplexy. Tumors comprised of 84% of cases. Hypogonadism (97%) was the most common abnormality seen followed by hypothyroidism (83.2%), hypoadrenalism (79.6%), growth hormone deficiency (88.1% of the 42 patients tested), and diabetes insipidus (13.3%). Panhypopituitarism was seen in 104 (92%) patients. There were no cases of hypopituitarism secondary to traumatic brain injury, subarachnoid hemorrhage, central nervous system infections, or cranial irradiation to extrasellar tumors. CONCLUSION: The most common cause of hypopituitarism at tertiary care center is pituitary tumors and the commonest presenting complaint is visual symptoms. Panhypopituitarism is present in 92% cases.
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We report a 27-year-old paramedical lady with no known comorbidities, who presented with rapid-onset coma with hypoglycemia (plasma glucose at admission was 35 mg/dL). Clinical alertness suspected and confirmed the diagnosis of exogenous insulin administration probably with suicidal intent. During the course of her ICU stay, she developed bradycardia and hypotension which required ionotropic support. She remained in coma for 90 hours. A total of 470 g of dextrose was infused until she regained consciousness. No other complications of insulin overdose were observed during her stay in the hospital. Recovery was complete without any residual neurological deficits. Insulin administration should be kept in differential diagnosis when any case presents with coma and hypoglycemia, especially in paramedical personnel.
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We report a case of 41-year-old lady who presented with chronic headache of 6-month duration and a sellar mass with a suprasellar extension on imaging, which was interpreted as pituitary macroadenoma. She had normal pituitary function and visual perimetry. On clinical examination and imaging it was provisionally diagnosed as pituitary incidentaloma due to hypophysitis and she was advised steroid therapy, but underwent transnasal resection of the tumor against suggestion. Histopathological examination revealed combined granulomatous and lymphocytic hypophysitis most likely of autoimmune in origin. Definitive diagnosis of hypophysitis can be made only on histopathological examination. As most cases of autoimmune hypophysitis are surgically treated, patients should be assessed on individual basis for requirement of steroids in postoperative period.