Ectopic Cushing's syndrome: experience from a tertiary care centre.

BACKGROUND & OBJECTIVE: Ectopic secretion of adrenocorticotropic hormone (ACTH) is rare, contributing to 10 per cent cases of endogenous Cushing's syndrome. We describe our experience of about two decades of patients with ectopic Cushing's syndrome (ECS) seen at a tertiary care centre from north India. METHODS: Records of patients with ECS from 1985 to 2006 were retrospectively reviewed that included the presenting manifestations, clinical symptoms and signs, biochemical investigations including plasma cortisol, ACTH and high dose dexamethasone suppression test (HDDST), imaging modalities to localize the non pituitary source of ACTH production, management and follow up of these patients. RESULTS: The study group included 12 patients (7 men) with mean (+/-SD) age at presentation 27.6 +/- 9.5 yr (range 13 to 48 yr) and the mean lag period between onset of symptoms and the diagnosis was 18.3 +/- 12.9 months with a range of 3 to 48 months. The weight loss (41.7%) followed by hyperpigmentation (25%) and infections (16.7%) were the common presenting manifestations. Cuticular atrophy (100%), hypertension (100%), bruise (92%) and proximal myopathy (83%) were the commonest signs. Plasma cortisol at 0800 h was 1267.3 +/- 483.3 nmol/l and at 2200 h was 1214.9 +/- 442.6 nmol/l indicating loss of circadian rhythm. The mean plasma ACTH was 221.1 +/- 55.9 (range 21.7 to 950 pg/ml). All but 2 patients had non-suppressibility of 0800 h plasma cortisol with HDDST. Five patients had thymic carcinoid, 3 had bronchial and one each had islet, hepatic and gut carcinoid and one had medullary thyroid carcinoma as a cause of ectopic ACTH secretion. The mean duration of follow up of these patients was 42.6 months and only two could sustain cure while remaining had either residual or recurrence. INTERPRETATION & CONCLUSION: Ectopic Cushing's syndrome is a rare disease with varied manifestations and associated with increased morbidity and mortality. It presents with clinical features quite similar to classical Cushing's. Surgery with removal of primary tumour was found to be treatment of choice.

Unusual malignant tracheal tumour presenting with acute airway obstruction.

A 25-year-old male presented with features of acute airways obstruction. He was diagnosed to have a lower tracheal mass with near total tracheal obstruction and complete obstruction of the left main bronchus. The tumour was resected successfully using a two-step method of ventilation. Histopathology of the mass revealed it to be a spindle cell sarcoma. Subsequently, local irradiation and systemic chemotherapy was given.

Retained intracardiac spring: an unusual foreign body.

A rare case of a penetrating intracardiac injury due to a ball-point pen in an adult male is reported. The patient presented with a retained metal spring embedded in the interventricular septum without any cardiac defects. A small metal tip of the pen was also present in the left lung. The patient was asymptomatic and without any cardiovascular compromise. The cardiac injury was managed conservatively. At two years after the injury the patient is still asymptomatic.

Severe acute bleeding from portal colopathy controlled by somatostatin: a case report.

Portal hypertensive colopathy (PHC) is a recently described entity in patients with portal hypertension which can cause even life-threatening lower gastrointestinal bleeding. In contrast to variceal bleed, there is no standardized treatment for the control of bleeding from these lesions. We report a case of alcoholic cirrhosis with portal hypertension, in whom bleeding from colonic angiodysplasia-like lesions was effectively controlled by somatostatin infusion.

Hemobilia as presenting manifestation of polyarteritis nodosa.

Polyarteritis nodosa (PAN) is a multisystem necrotizing inflammatory vasculitis of medium-sized muscular arteries. Hepatobiliary complications secondary to thrombosis or aneurysm formation in PAN are rare and are associated with poor outcome. We describe an 18-year-old man with PAN who presented with hemobilia secondary to rupture of a hepatic artery aneurysm, which was successfully managed with coil embolization followed by immunosuppressive therapy.

Serological study of hepatitis A virus infection amongst the students of a medical college in Delhi & evaluation of the need of vaccination.

BACKGROUND & OBJECTIVES: Recent seroepidemiological studies have demonstrated a decrease in the seroprevalence of hepatitis A virus (HAV) infection, thereby increasing the pool of susceptible adult population. Health care workers, especially those working in paediatric wards and nurseries, are at an increased risk of developing HAV infection. The present study was undertaken to determine the prevalence of antibody against HAV (IgG anti-HAV) among a batch of medical students of the Maulana Azad Medical College, New Delhi, so as to identify the proportion of students who would be susceptible to HAV infection and hence would benefit from a vaccination programme against HAV. METHODS: A total of 91 medical students were enrolled and divided into groups on the basis of sex. The serum samples were evaluated for the presence of immunoglobulin (IgG) against HAV (IgG anti-HAV) using a commercially available enzyme linked immunosorbent assay (ELISA) kit. RESULTS: The mean age of all the subjects was 19.9 +/- 1.5 yr and the male: female ratio was 1.2: 1. Fifty seven (62.6 +/- 5.1%) students were positive for IgG anti-HAV while 34 (37.4%) were seronegative and hence susceptible to HAV infection. No statistically significant difference was observed in the seroprevalence of HAV between males and females [64 +/- 6.7% (32/50) versus 60.9 +/- 7.6% (25/41)]. INTERPRETATION & CONCLUSION: our results indicate that more than one-third of the medical students were seronegative for IgG anti-HAV and hence at an increased risk of developing HAV infection as a result of occupational exposure. Therefore, we suggest that students in a health care set up should undergo vaccination against HAV after pre-vaccination immunity screening.

Hepatopulmonary syndrome: a case report.

The Hepatopulmonary Syndrome (HPS) is one of many extrahepatic manifestations of liver failure. It consists of a triad of liver dysfunction, intrapulmonary vascular dilatation and hypoxemia. We present a case of post necrotic cirrhosis (HBV related) who developed exertional dyspnoea subsequent to the development of hepatopulmonary syndrome.

AIDS cholangiopathy as initial presentation of HIV infection.

AIDS related cholangiopathy is a known clinical entity, but most cases described in literature are known cases of HIV infection who have presented with pain abdomen. AIDS cholangiopathy as the initial presentation of HIV infection is very rare. Here we report such a case.