Autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED) is an inherited disease which may comprise many endocrine and non-endocrine components. GH insufficiency has not been recognised as a classical manifestation of this syndrome. We describe the case of a girl with APECED, who presented with four endocrine (hypoparathyroidism, Addison's disease, hypothyroidism, gonadal failure) and three non-endocrine components (candidiasis, ectodermal dystrophy and lichen ruber planus). In addition, growth failure was documented beginning at approximately 8 years; bone age was delayed and stimulated GH peaks after clonidine and arginine were 2.2 and 9.2 microg/l, respectively. A partial empty sella was found on a computed tomography scan of the hypothalamic-pituitary region. At 10.5 years rhGH therapy was started and height gain of 26 cm was observed after 2.7 years of treatment. Puberty started at 11.2 years and menarche occurred at 12.7 years. At 13.25 years rhGH therapy was discontinued owing to frequent hypocalcemic crises; serum IGF-1 levels were persistently low in the following years (between 160 and 180 microg/l, normal range for age 250-600 microg/l). The patient attained a final height of 160.8 cm, which was appropriate for her target height. The presence of lichen ruber planus and GH insufficiency probably secondary to empty sella are two unusual findings in patients with APECED.

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Objective To prepare a high fat diet rich in satured fatty acids and supplemented with omega 3 for experimental studies in rodents. Methods Purified industrial ingredients and flaxseed oil as a source of omega 3 at a concentration of 3.5% (v/w) were used in the elaboration of the diets. Centesimal and nutritional compositions, fatty acids profile and dietary intake were evaluated. Serum levels of total protein, albumin, cholesterol and glucose in pregnant rats were verified. The offspring were assessed with regard to body mass and waist circumference. Statistical analysis was performed using the Kolmogorov-Smirnov, Anova One-Way test and Bonferroni post-test. Results High fat and high fat with omega 3 diets presented, respectively, 37% and 36% saturated fat, and the lipid amount was 80% higher than the American Institute of Nutrition 93G control diet. The omega 3 content was 50% higher in the high fat with omega 3 diet. There was no difference in consumption of diet types in weight (grams). The dams that received the High fat diet developed hypercholesterolemia and their High fat offspring exhibited higher body mass on the 1st day of life and increased abdominal circumference on the 30th day of life compared to the control and the high fat with omega 3 offspring. Conclusion The formulated diets with a higher amount of saturated fatty acids meet the nutritional requirements of the gestation and lactation period. The high fat diet with omega 3 was able to attenuate the changes observed in dams and their offspring.

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Interactions between mitochondria and the endoplasmic reticulum, known as MAMs, are altered in the liver in obesity, which contributes to disruption of the insulin signaling pathway. In addition, the plasma level of glycine is decreased in obesity, and the decrease is strongly correlated with the severity of insulin resistance. Certain nutrients have been shown to regulate MAMs; therefore, we tested whether glycine supplementation could reduce insulin resistance in the liver by promoting MAM integrity. Glycine (5 mM) supported MAM integrity and insulin response in primary rat hepatocytes cultured under control and lipotoxic (palmitate 500 µM) conditions for 18 h. In contrast, in C57 BL/6 JOlaHsd mice (male, 6 weeks old) fed a high-fat, high-sucrose diet (HFHS) for 16 weeks, glycine supplementation (300 mg/kg) in drinking water during the last 6 weeks (HFHS-Gly) did not reverse the deleterious impact of HFHS-feeding on liver MAM integrity. In addition, glycine supplementation worsened fasting glycemia and glycemic response to intraperitoneal pyruvate injection compared to HFHS. The adverse impact of glycine supplementation on hepatic gluconeogenesis was further supported by the higher oxaloacetate/acetyl-CoA ratio in the liver in HFHS-Gly compared to HFHS. Although glycine improves MAM integrity and insulin signaling in the hepatocyte in vitro, no beneficial effect was found on the overall metabolic profile of HFHS-Gly-fed mice.