Assessment of aortomesenteric distance and mesenteric and retroperitoneal adipose tissue thickness in genetic forms of lipodystrophy.

INTRODUCTION: Lipodystrophy is a rare disease characterized by the loss of adipose tissue. Visceral adipose tissue loss in certain forms of lipodystrophy may affect the amount of mesenteric fat. METHOD: We studied visceral adipose tissue by measuring the thickness of mesenteric and retroperitoneal adipose tissue and the aortomesenteric (AOM) distance in patients with genetic forms of lipodystrophy (n = 48; 7 males; 41 females; mean age 39.1 ± 11.9 years; 19 with congenital generalized lipodystrophy [CGL], and 29 with familial partial lipodystrophy [FPLD]). An age- and gender-matched control group with a ratio of 1:2 was generated. RESULTS: Patients with CGL had severely depleted mesenteric adipose tissue (2.0 [IQR: 1.5-3.5] mm vs. 18.8 [IQR: 4.4-42.2] mm in FPLD, P < .001; 30.3 [IQR: 13.9-46.6] mm in controls, P < .001) and retroperitoneal adipose tissue (1.3 [IQR: 0.0-5.3] mm vs. 33.7 [IQR: 21.6-42.1] mm in FPLD, P < .001; 29.7 [IQR: 23.1-36.7] mm in controls, P < .001). The AOM distance was shorter in patients with CGL (8.1 [IQR: 6.0-10.8] mm) compared to patients with FPLD (vs. 13.0 [IQR: 8.8-18.1] mm; P = .023) and controls (vs. 11.3 [IQR: 8.4-15.5] mm, P = .016). Leptin levels were positively correlated with AOM distance in lipodystrophy (r = .513, P < .001). Multivariate linear regression analysis identified body mass index as a significant predictor of AOM distance (data controlled for age and sex; beta = 0.537, 95% CI: 0.277-0.798, P < .001). Twelve of 19 patients (63%) with CGL had an AOM distance of < 10 mm, a risk factor that may predispose patients to developing superior mesenteric artery syndrome. CONCLUSION: CGL is associated with a severe loss of mesenteric adipose tissue, which leads to a narrowing of the space between the superior mesenteric artery and the aorta.

Clinical features of generalized lipodystrophy in Turkey: A cohort analysis.

AIM: To describe the Turkish generalized lipodystrophy (GL) cohort with the frequency of each complication and the death rate during the period of the follow-up. METHODS: This study reports on 72 patients with GL (47 families) registered at different centres in Turkey that cover all regions of the country. The mean ± SD follow-up was 86 ± 78 months. RESULTS: The Kaplan-Meier estimate of the median time to diagnosis of diabetes and/or prediabetes was 16 years. Hyperglycaemia was not controlled in 37 of 45 patients (82.2%) with diabetes. Hypertriglyceridaemia developed in 65 patients (90.3%). The Kaplan-Meier estimate of the median time to diagnosis of hypertriglyceridaemia was 14 years. Hypertriglyceridaemia was severe (≥ 500 mg/dl) in 38 patients (52.8%). Seven (9.7%) patients suffered from pancreatitis. The Kaplan-Meier estimate of the median time to diagnosis of hepatic steatosis was 15 years. Liver disease progressed to cirrhosis in nine patients (12.5%). Liver disease was more severe in congenital lipodystrophy type 2 (CGL2). Proteinuric chronic kidney disease (CKD) developed in 32 patients (44.4%) and cardiac disease in 23 patients (31.9%). Kaplan-Meier estimates of the median time to diagnosis of CKD and cardiac disease were 25 and 45 years, respectively. Females appeared to have a more severe metabolic disease, with an earlier onset of metabolic abnormalities. Ten patients died during the follow-up period. Causes of death were end-stage renal disease, sepsis (because of recurrent intestinal perforations, coronavirus disease, diabetic foot infection and following coronary artery bypass graft surgery), myocardial infarction, heart failure because of dilated cardiomyopathy, stroke, liver complications and angiosarcoma. CONCLUSIONS: Standard treatment approaches have only a limited impact and do not prevent the development of severe metabolic abnormalities and early onset of organ complications in GL.

Menstrual Cycle Characteristics in Women With and Without Thyroid Disease.

INTRODUCTION: This study was designed to evaluate the frequency and type of menstrual disorders in thyroid dysfunction. The relationship between thyroid dysfunction and menstrual disorders has been known for a long time. The menstrual cycle should be checked in women with thyroid dysfunction. On the contrary, women with menstrual irregularities should be investigated for thyroid dysfunction. METHODS: Women who presented to our hospital's internal medicine and endocrinology clinics that recently diagnosed thyroid dysfunction were included. The patients were divided into five groups (subclinical hypothyroidism, overt hypothyroidism, subclinical hyperthyroidism, overt hyperthyroidism, and euthyroid) according to thyroid functions. They were questioned regarding the amount, frequency, and duration of menstrual bleeding. The prevalence of menstrual disturbances, including secondary amenorrhea, hypomenorrhea, oligomenorrhea, hypermenorrhea, polymenorrhea, menorrhagia, metrorrhagia, and menometrorrhagia, was examined in 485 patients and 108 healthy controls. RESULTS: Hypermenorrhea was significantly more common in patients with overt hypothyroidism (33%) than in controls (6%) (p<0.05). The types and frequencies of menstrual disorders in patients with hyperthyroidism and those with normal thyroid function were not significantly different from those in controls. CONCLUSION: Menstrual abnormalities frequently occur in women with thyroid dysfunction. Therefore, menstrual dysfunction should be considered when treating patients with thyroid abnormalities.