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Introduction: Lipodystrophies are rare disorders characterized by loss of body fat resulting in leptin deficiency. Patients are predisposed to metabolic complications such as severe insulin resistance, hypertriglyceridemia, and hepatic steatosis. Werner syndrome (WS) is among the progeroid syndromes in the classification of lipodystrophy. Due to its extremely rare occurrence, it can often be overlooked by the clinician. Clinical Case: A 19 years old male patient hospitalized for non-biliary acute pancreatitis was consulted for hypertriglyceridemia and hyperglycemia. He had no history of any disease or medication use. His sister was diagnosed with type 2 diabetes mellitus at age 29. She was using linagliptin and gliclazide for 3 years. She had short stature (147 cm) and low body weight (40 kg) and had scleroderma-like skin findings. The patient also had short stature (157 cm) and low body weight (41 kg). He had scleroderma-like skin findings and generalized loss of subcutaneous adipose tissue. The laboratory results were; glucose, 261 mg/dl; creatinine, 0.46 mg/dl; ALT, 24 U/l; lipase, 161 U/l; HbA1c, 9.6%; triglyceride, 799 mg/dl; HDL, 9.6 mg/dl; LDL, 75 mg/dl; c-peptide, 4.3 ng/dl. The thyroid function test results (TSH: 29.7 µIU/ml, fT4: 6.81 pmol/L, Anti-TPO: negative) were consistent with primary hypothyroidism. Hepatosteatosis was observed in abdominal ultrasonography. We initiated intensive insulin, metformin as well as fenofibrate and levothyroxine treatment. The patient needed high insulin doses (60 IU/day). Measured serum leptin levels were found to be low (2.11 ng/ml) in suspicion of lipodystrophy. Genetic analysis of the patient and his sister showed homozygous mutation in the WRN gene (c.1105C>T p.Arg369Ter, Homozygous) which was compatible with WS. The patient was started on leptin analog therapy in a clinical trial. Conclusion: WS is an exceptionally rare autosomal recessive genetic disorder characterized by premature aging and multisystemic complications. The clinical features encompass scleroderma-like skin changes, short stature, low body weight, alopecia, cataracts, osteoporosis, and a propensity for atherosclerotic complications and malignancies. Impaired glucose tolerance is reported in 15%–20% of WS subjects, diabetes mellitus in 55%–70%, and dyslipidemia in 60%–85%. The presence of hypertriglyceridemia, diabetes mellıtus and generalized lack of subcutaneous adipose tissue led us to suspect from lipodystrophy. Although acute pancreatitis due to hypertriglyceridemia can be seen in lipodystrophy (15-20%), first presentation of WS presenting with acute pancreatitis is very rare in the literature. The most common mutation in non-Japanese patients in WRN gene is c.1105 C>T (18.6%). Metreleptin, an analog of human leptin, is shown to ameliorate the metabolic derangements. WS remains a diagnostic challenge due to its rarity, atypical presentation, and should be kept in mind in patients with severe dyslipidemia and insulin resistance. [ABSTRACT FROM AUTHOR]

Introduction: Ectopic Cushing's syndrome (ECS) is a rare endocrine disorder caused by the autonomous secretion of ACTH from a tumor apart from the hypophysis. ECS is the second most common paraneoplastic syndrome associated with small cell lung cancer (SCLC). Due to the aggressive nature of the syndrome, ECS is difficult to diagnose. Most patients present electrolyte disturbances and muscle weakness rather than the typical clinical features of CS. Clinical Case: A 70-year-old female patient hospitalized due to pleural effusion was referred for worsening hyperglycemia. She had no previous history of diabetes mellitus. She had a history of 90 pack-years of smoking and chronic obstructive pulmonary disease. The laboratory results were: fasting plasma glucose 216 mg/dl; creatinine, 0.81 mg/dl; sodium, 138 mmol/l; potassium, 3.1 mmol/l; HbA1c, 8.3%; Arterial blood gas analysis revealed metabolic alkalosis (pH 7.63, HCO3 53.6 meq/L). Due to resistant hypokalemia, the patient was evaluated for CS. The patient's ACTH level was 415.5 pg/ml and cortisol level was 63.44 μg/dl. Table 1 shows the tests perfomed for the diagnosis of CS. Pituitary MRG revealed no pathology. IPSS could not be performed as it is not available at our center. A suspicious mass in the right lung was observed on the thorax CT. The pathology of the cytology sample from the pleural effusion revealed SCLC. A chemotherapy regimen containing cisplatin was initiated. Intravenous potassium and acetazolamide treatment were administered for resistant hypokalemia. However, the patient died one month after diagnosis due to type 2 respiratory failure. Conclusion: Ectopic secretion of ACTH accounts for 10–15% of CS. ECS is usually caused by mainly SCLC (45%), thymic carcinoma (15%), bronchial carcinoid (10%) and pancreas islet-cell carcinoma (10%). ECS is reported to occur in 1.6%–4.5% of patients with SCLC. IPSS is the most reliable examination for ECS. For ECS, surgery remains the optimal treatment. Some reports showed that metyrapone, ketoconazole and octreotide are effective but not widely used. In our case, the presence of resistant hypokalemia, metabolic alkalosis, and worsening hyperglycemia with the lack of classic clinical features of CS led us to suspect ECS. ECS has a higher incidence of hypokalemic alkalosis than other causes of Cushing's syndrome (>90% vs. 10%). Excessive amounts of adrenal steroids with the inhibition of the enzyme 11β-hydroxysteroid dehydrogenase may cause this pathology. This enzyme normally converts cortisol to cortisone avoiding the mineralocorticoid action of cortisol. In conclusion, although rare, in cases where conditions such as metabolic alkalosis, resistant hypokalemia, with the absence of classic clinical features of CS, the possibility of ectopic CS should be kept in mind. Table 1. Laboratory results of the patient [ABSTRACT FROM AUTHOR]