ECTOPIC CUSHING SYNDROME: A 10-YEAR EXPERIENCE FROM A TERTIARY CARE CENTER IN SOUTHERN INDIA.

OBJECTIVE: Ectopic adrenocorticotropic hormone (ACTH) secretion is a less common cause of Cushing syndrome and is seen in 5 to 10% of cases with endogenous hypercortisolemia. We hereby describe our experience of patients with ectopic ACTH syndrome, who have been managed over the past 10 years at a tertiary care center in Southern India. METHODS: The inpatient and outpatient records of patients from 2006 to 2015 were retrospectively reviewed. The clinical features, clinical history, biochemical values, imaging features, including radiologic findings and positron emission tomography scans, management, details of follow-up, and outcomes, were documented. We compared the biochemical findings in these patients with 20 consecutive patients with Cushing disease (Cushing syndrome of pituitary origin). RESULTS: A total of 21 patients were studied. The median age at presentation was 34 years (range, 19 to 55 years). Seven patients had thymic carcinoid, 7 had bronchial carcinoid, 3 had lung malignancies, 2 had medullary carcinoma thyroid, 1 patient had a pancreatic neuroendocrine tumor, and 1 patient had an occult source of ACTH. The most common clinical features at presentation were muscle weakness (95%), hyperpigmentation (90%), facial puffiness (76%), easy bruising (61%), edema (57%), and striae (52%). Extensive acne was seen in a large number of patients (43%). Only 3 patients (14%) had central obesity. The median 8 am cortisol was 55.5 µg/dL (range, 3.8 to 131 µg/dL), median 8 am ACTH was 207 pg/mL (range, 31.1 to 703 pg/mL), and the median 24-hour urinary free cortisol was 2,484 µg (range, 248 to 25,438 µg). Basal cortisol and ACTH, as well as midnight cortisol and ACTH level, were markedly higher in patients with ectopic Cushing syndrome as compared to patients with Cushing disease. Twelve of 21 patients had developed life-threatening infections by follow-up. Nine patients had undergone surgical intervention to address the primary tumor. However, only 1 patient exhibited a complete cure on follow-up. CONCLUSION: In our series, ectopic Cushing syndrome was most commonly seen in association with intrathoracic tumors such as bronchial or thymic carcinoid. Hyperpigmentation and proximal myopathy were frequent, while central obesity was uncommon. Early and rapid control of hypercortisolemia was important in order to prevent life-threatening infections and metabolic complications. ABBREVIATIONS: ACTH = adrenocorticotropic hormone CT = computed tomography DOTATATE = 68Ga-DOTA-Tyr3-octreotate ECS = ectopic Cushing syndrome FDG = fluorodeoxyglucose MTC = medullary thyroid cancer NET = neuroendocrine tumor PET = positron emission tomography.

Clinical characteristics, beta-cell dysfunction and treatment outcomes in patients with A-ß+ Ketosis-Prone Diabetes (KPD): The first identified cohort amongst Asian Indians.

OBJECTIVE: Ketosis-prone diabetes (KPD), an atypical form of diabetes, has emerged as a heterogeneous syndrome in multiple ethnic groups. The objectives of this study were to look into the clinical characteristics of adult Asian Indian patients with recently diagnosed, antibody negative diabetes presenting with unprovoked ketoacidosis (A-ß+ KPD) and to determine the natural course of recovery of beta-cell functions on serial follow-up over one year. RESEARCH DESIGN AND METHODS: Newly diagnosed adult diabetes patients (n=11) with suspected KPD (A-ß+) were prospectively studied over a period of 1-year with serial evaluations of clinical, biochemical and beta-cell secretion characteristics. These were compared with a control group (n=23) of KPD (A+ß-) (classical Type 1A diabetes) with similar presentation. Beta-cell secretion was assessed by fasting and stimulated C-peptide values after a standard mixed meal challenge. Glycaemic control and treatment outcomes were also documented. RESULTS: In comparison to the A+ß- KPD controls, the A-ß+ KPD patients had a significantly older age, higher BMI, stronger family history of type 2 diabetes, more severe ketoacidosis and higher fasting and stimulated C-peptide level at presentation. On serial follow-up, the patients with KPD achieved complete recovery of their beta-cell function with remission from insulin-dependence within 3-4months without further recurrences of DKA. CONCLUSIONS: This is the first reported series of A-ß+ KPD from India. The phenotype of Indian A-ß+ KPD patients differs from their Western counterparts in that they are relatively younger and leaner, though the male preponderance and natural history of recovery of beta-cell dysfunction bears similarity.

An uncommon cause for vomiting.

Thyrotoxicosis may present with a variety of non specific symptoms in elderly patients. Gastrointestinal symptoms such as nausea, vomiting and abdominal discomfort can occasionally be the presenting feature of thyrotoxicosis in this age group. We describe an elderly patient in whom thyrotoxicosis was diagnosed after extensive evaluation for nausea, vomiting and anorexia. This patient was also found to have hypercalcemia. This case highlights the importance of recognizing thyrotoxicosis in older patients presenting with GI symptoms and mild hypercalcemia.

An uncommon cause for hip pain and limping.

Slipped capital femoral epiphysis (SCFE) is characterized by displacement of the capital femoral epiphysis from the femoral neck. An 18-year-old male presented with left hip pain and a limping gait, following a trivial trauma. Radiological examination revealed bilateral SCFE. Clinically and biochemically, he had features of hypopituitarism. His prolactin levels were high and magnetic resonance imaging (MRI) scan showed a pituitary macroadenoma, suggesting a diagnosis of macroprolactinoma causing hypopituitarism and presenting as SCFE. He was started on dopamine agonist cabergoline as well as thyroxine and glucocorticoid replacement treatment. He was also scheduled for an orthopedic surgical procedure for his SCFE.