Antoni Dyduch

RANKL/RANK/OPG system and bone status in females with anorexia nervosa

Minimal data exist concerning the relationship between osteokines of the RANKL/RANK/OPG system, especially RANKL, and bone status in females with anorexia nervosa (AN). For this reason we investigated the relationship between bone metabolism (as assessed based on serum levels of OC and CTx), and OPG and sRANKL concentrations in females with AN. Ninety-one female patients with AN and 29 healthy female subjects aged 13 to 18 years of age participated in the study. Serum OC, CTx, OPG and sRANKL were measured by ELISA. The female patients with AN demonstrated an essential suppression of OC and CTx, increased OPG and sRANKL levels, and a reduced OPG/sRANKL ratio. OC, CTx and the OPG/sRANKL ratio correlated positively with body mass and BMI in these patients, whereas in the case of OPG and sRANKL the relationship was negative. A significant positive correlation was observed between OPG and sRANKL and also between bone markers and the OPG/sRANKL ratio, and negative between CTx and sRANKL. In female patients with AN, the OPG/RANKL ratio was a significant and independent predictor of osteocalcin, a bone formation marker - OC (R(2)=0.065, p=0.012) whereas the OPG/sRANKL ratio and BMI were significant and independent predictors of a bone resorption marker - CTx (R(2)=0.095; p=0.012). In conclusion, the body mass, BMI values, and bone markers suppression observed in female patients with AN might be associated with an increase in OPG and sRANKL levels and a significant decrease of the OPG/sRANKL ratio. Although higher OPG levels may compensate for excessive bone resorption in female patients with AN, the lower OPG/sRANKL ratio seems to indicate that some inadequacies exist regarding this compensation effect, which might contribute to low bone density in these patients. The OPG/sRANKL ratio might prove a more relevant marker to predict bone metabolism in female patients with AN than sRANKL and/or OPG alone.

Assessment of Serum Apelin Levels in Girls with Anorexia Nervosa

CONTEXT Pilot studies in rats have suggested that apelin (APE) is involved in the control of appetite and food intake. APE is secreted in the organs involved in the control of hunger and satiety: the stomach, hypothalamus, and fat tissue. Anorexia nervosa (AN) is an eating disorder that represents a good biological model of chronic fat tissue atrophy in humans. To date, there are no reports of APE expression in the fat tissue and its circulating concentrations in patients with AN. OBJECTIVE Our objective was to assess serum APE concentrations in girls with AN. DESIGN, PARTICIPANTS, AND SETTING APE-36 and APE-12 serum concentrations were evaluated in 87 Polish girls with restrictive AN, in 61 healthy (H) controls, 17 girls with no otherwise specified eating disorders (NOS), and 30 girls with simple obesity (OB). RESULTS Mean serum APE-36 and APE-12 concentrations in patients with AN and NOS were significantly lower than in the H and OB groups. However, no differences between AN, H, and NOS groups were observed when APE concentrations were calculated per body mass index (BMI). In participants with normal BMI, serum APE-36 (r = 0.35) and APE-12 (r = 0.37) concentrations correlated positively with BMI. CONCLUSIONS We conclude that compared with H controls, serum APE-36 and APE-12 concentrations decreased as a result of fat tissue depletion in patients with AN. Conversely, obese adolescents had elevated APE-36 and APE-12 due to excessive fat mass as well as increased APE production in adipose tissue.

Acquired partial lipodystrophy in an 11-year-old girl

© 2008 Japan Pediatric Society Acquired partial lipodystrophy (APL), also known as Barraquer – Simons syndrome, is a rare disorder characterized by selective loss of adipose tissue, which usually starts in the face, extends downward to the waist, but spares the legs. Two hundred and fi fty cases of this syndrome have already been reported in the literature. 1 Female patients are affected fourfold more often than male patients. Approximately 83% of the patients have low C3 component levels and the presence of polyclonal immunoglobulin C3 nephritic factor (C3NeF). 1,2

Hipernatremia neurogenna u dzieci z holoprosencefalią – opis dwóch przypadków

Holoprosencefalia jest rzadko wystepującą wadą rozwojową przodomozgowia (1:13 000–1:16 000 urodzen), w 25–45% spowodowaną aberracjami chromosomow. Znane są trzy formy holoprosencefalii: alobarna, semilobarna i lobarna, identyfikowane za pomocą MR mozgu. Postaci alobarna i semilobarna przebiegają klinicznie z ciezkimi zaburzeniami neurologicznymi i glebokim upośledzeniem rozwoju somatycznego i psychicznego. Postac lobarna bywa na ogol rozpoznawana przypadkowo u dziecka z padaczką. Hipernatremia neurogenna jest niezwykle rzadkim powiklaniem holoprosencefalii. Cechuje ją przewlekla hipernatremia z brakiem wzmozonego pragnienia (adypsja), bez wielomoczu i klinicznych oznak odwodnienia. Tlumaczy sie to mozliwością wspolistnienia w holoprosencefalii uszkodzenia osmoreceptorow w podwzgorzu, regulujących wydzielanie wazopresyny. Hipernatremia w tych przypadkach wymaga nie tylko roznicowania z moczowką neurogenną (diabetes insipidus), ale i odmiennego postepowania. Przedstawiono opis dwoch przypadkow hipernatremii ośrodkowej: u 2-miesiecznego chlopca z alobarną postacią i u 3-letniego chlopca z lobarną postacią holoprosencefalii. Dzieci te byly skierowane do kliniki z podejrzeniem diabetes insipidus.