Cushing's syndrome due to primary pigmented nodular adrenal disease in two brothers with Carney complex.

Two brothers (19 and 18 years old, respectively) presented with weight gain and stunted growth since the age of 10 years. They had spotty skin pigmentation over the face along with florid features of Cushing's syndrome with low bone density, renal calculi, dyslipidaemia, hypertension, and dilated cardiomyopathy. They underwent evaluation of the hypothalamic-pituitary-adrenals axis, which suggested ACTH-independent Cushing's syndrome, and the abdominal CT imaging revealed normal adrenals. The diagnosis of Familial Cushing's syndrome with primary pigmented nodular adrenal disease and Carney complex was made. Bilateral adrenalectomy was carried out in both of them with resolution of the features of hypercortisolaemia post-operatively.

Markers of Oxidative Stress and Clinical Outcome in Critically ill Septic Patients: A Preliminary Study from North India.

INTRODUCTION: Sepsis is the leading cause of mortality in non-coronary Intensive Care Units (ICUs). Oxidative stress is one of the important features in pathogenesis of sepsis. AIM: This study was undertaken to evaluate levels of oxidants and antioxidants in patients with sepsis admitted to ICU. STUDY DESIGN: This was a non-interventional clinical case-control study undertaken at a tertiary level teaching hospital in New Delhi, India. MATERIALS AND METHODS: Forty-six consecutive non-pediatric patients admitted to ICU with sepsis were included and subjected to detailed history, physical examination and investigations. Blood samples were drawn to evaluate oxidant Malondialdehyde (MDA) and antioxidant (alpha-tocopherol) levels. Acute Physiology and Chronic Health Evaluation II (APACHE II) and Organ Dysfunction and/or Infection (ODIN) scores were calculated and patients followed up for outcomes. Twenty age and sex matched healthy subjects served as controls. RESULTS: Mean levels of malondialdehyde were higher in patients than controls (17.2±3.8nm/ml versus 4.6±1.6nm/ml, p<0.001) while levels of alpha-tocopherol were lower (3.2±1.3µg/ml versus 9.9±2.0µg/ml, p<0.001). The mean APACHE II and ODIN scores were 18.1±9.3 and 1.7±1.3 respectively in patients. These scores were two to three fold higher in non survivor patients (n=22) in comparison with survivors (n=18) (p<0.001). There was no significant difference between the two groups in oxidants and antioxidants levels (p>0.05). However, a significant and positive correlation was observed between oxidant -antioxidant levels and APACHE II, ODIN and International Normalized Ratio (INR) scores in septic patients overall. CONCLUSION: The oxidants in septic patients were significantly higher while antioxidants were significantly lower than healthy controls. There was also a significant correlation with APACHE II and ODIN scores. A large patient population based study may draw more specific conclusions.