[The relationship between chemerin, bone metabolism, the RANKL/RANK/OPG system, and bone mineral density in girls with anorexia nervosa].

INTRODUCTION: Based on recent studies in humans, chemerin has been classified as an adipokine that might be associated with osteoporosis and BMD. Bone loss is common in adolescents with anorexia nervosa (AN). Moreover, dysfunction in the production of chemerin has also been shown. Therefore, we carried out a comparative analysis between chemerin, bone metabolism, the RANKL/RANK/OPG system, and BMD in girls with AN. MATERIAL AND METHODS: Plasma chemerin, OC, CTx, OPG, and sRANKL were determined by ELISA in 75 girls with AN aged 12.6-17.8 years. BMD was assessed by DXA and expressed as Z-score according to the lumbar spine (s) and total body (TB) sites. According to the s-BMD- and TB-BMD Z-score, girls with AN were divided into two subgroups with parallel analyses used: normal (Z-score > -2.0) and low (Z-score ≤ -2.0) s-BMD, and normal (Z-score > -2.0) and low (Z-score ≤ -2.0) TB-BMD. RESULTS: Mean OC and the OPG/sRANKL ratio were markedly lower in the low s-BMD subgroup compared to the normal s-BMD subgroup. The s-Z-score values (both low and normal) correlated significantly and positively with the OPG/sRANKL ratio. Only in the low s-BMD subgroup did chemerin correlate significantly and positively with all nutritional indices and the OPG/sRANKL ratio. In the low TB-BMD subgroup the mean OC and the OPG/sRANKL ratio were lower than in the normal TB-BMD subgroup. The TB-Z-score values (both normal and low) correlated significantly and positively with all nutritional indices and the OPG/sRANKL ratio. The low TB-Z-score values correlated significantly and positively also with chemerin. In the low TB-BMD subgroup chemerin correlated significantly and positively with weight and BMI (expressed as absolute values), Cole index, the duration of the disease, and OPG/sRANKL ratio while its correlation with age was negative. CONCLUSIONS: Undernutrition and associated deficit of adipose tissue may result in inadequate chemerin production and skeletal disorders in girls with AN. Chemerin acts as a coordinator of the dynamic balance between bone metabolism and the OPG/RANK/RANKL system and, in turn, may contribute to the loss of bone mass in girls with AN. The cortical bone site seems to be more severely responsive to chemerin actions than the trabecular bone site.

A rare case of primary amenorrhoea and breast development in a 46,XY 15-year-old girl.

A disorder of sex development (DSD) is defined as a congenital condition in which development of chromosomal, gonadal, or anatomical sex is atypical. Swyer syndrome is an example of 46,XY DSD with a female phenotype. It usually becomes apparent in adolescence with delayed puberty and amenorrhoea. Spontaneous breast development is very rare. A 15-year-old girl was presented due to primary amenorrhoea with breast development compatible with Tanner stage V. Hormonal tests revealed hypergonadotropic hypogonadism with low level of oestradiol. Pelvic ultrasound and magnetic resonance imaging revealed a small uterus, and no ovaries were found. In the right lower abdomen, a structure of unknown origin was visible. The chromosome analysis revealed a 46,XY karyotype. The patient was qualified for a laparoscopic bilateral gonadectomy. Postoperative histopathological examination revealed gonadoblastoma. We underline the need to consider DSD 46,XY in the presence of primary amenorrhoea, even when pubertal development is present. Germ cell tumors have a tendency to grow and metastasize rapidly. Delayed diagnosis may increase the risk of malignant transformation and cause a poor diagnosis.

A rare complication of systemic lupus erythematosus in a 9-year-old girl: Questions.

Serious renal involvement in systemic diseases is common and generally constitutes a pivotal prognostic factor, making those pathology frequently seen in nephrology departments. Authors describe the case of a nine-year-old girl with lupus nephritis. After admission the patient's state deteriorated over a period of a few days, with an unremitting high-grade fever, significant weakness and drowsiness, generalized erythema, and decrease of the kidney function to eGFR nadir of 56 ml/min/1,73m 2. Treatment with pulsed methylprednisolone was started. After the first pulse the general state of the patient improved slightly, although laboratory tests showed an alarming evolution, with the exacerbation of anemia, leukopenia, neutropenia, increase of serum CRP concentration, extremely high D-dimer concentration and increase in activity of lactate dehydrogenase. The concentration of ferritin rose reaching the level of 540 µg/l, triglicerydes level was also high. Intravenous cyclophosphamide pulse therapy was added to the ongoing steroid treatment, and resulted in a radical patient improvement. Authors underline that it seems important to be aware of rare, non-renal, but potentially devastating complications of systemic diseases, like in this clinical case: the secondary hemophagocytic lymphohistiocytosis (HLH). When HLH complicates a rheumatic disease, it is also referred to as macrophage activation syndrome (MAS). Unfortunately treatment of MAS is still based on reports provided by individual centres and gathered own experiences so drawing up unambiguous diagnostic criteria will be valuable in future. The treatment should be individually tailored, and more specific evidence-based recommendations are needed.

[The new adipokine zinc-α2-glycoprotein (ZAG) as a link between adipose tissue and kidney? [Czy nowa adipocytokina cynkowa α2-glikoproteina (ZAG) stanowi ogniwo miedzy tkanka tluszczowa a nerkami?]].

Adipose tissue is currently considered not only as an energy store but also as an organ of internal secretion. Numerous adipocytokines regulating a number of human body processes are important in many disease processes, including chronic kidney disease (CKD). Nowadays, the role of zinc α2-glycoprotein (ZAG) is being sought as a potential link between these two organs. ZAG, through its lipolytic effect, contributes to progressive malnutrition in patients undergoing dialysis, and this significantly increases their mortality. It seems that ZAG may be a new potential biomarker of kidney damage, and the specific pharmacotherapy will significantly reduce the progressive process of cachexia.

Vaspin and selected indices of bone status in girls with anorexia nervosa.

INTRODUCTION: In vitro studies indicate that vaspin may act as a regulator of bone metabolism. The aim of the study was to evaluate the relationship between vaspin and bone metabolism in girls with anorexia nervosa (AN), as well as the potential involvement of OPG and RANKL in this relationship. MATERIAL AND METHODS: Serum vaspin, OC, CTx, OPG, and sRANKL were determined by ELISA in 50 girls with AN and in 30 healthy controls aged 13 to 17 years. RESULTS: Girls with AN exhibited significant reduction in body weight, BMI, and Cole index as well as a significant increase in serum level of vaspin compared to healthy participants. These changes were associated with a significant decrease in serum OC and CTx levels and a significant increase in OPG and sRANKL, while the OPG/sRANKL ratio was significantly decreased. BMI and Cole index correlated negatively and significantly with CTx levels in the control group (C), girls with AN, and all study participants (C+AN). Girls with AN showed a significant negative correlation between BMI, the Cole index, and OPG levels. The combination group (C+AN) showed a significant positive correlation between BMI, Cole index, and the OPG/sRANKL ratio. In this group of girls vaspin levels correlated positively and significantly with sRANKL and negatively with body weight, BMI, Cole index, and OPG/sRANKL ratio. Girls with AN showed a significant negative correlation between vaspin levels and the OPG/sRANKL ratio. CONCLUSIONS: Undernourishment and associated deficit of adipose tissue may result in inadequate vaspin production and bone metabolism disorders in girls with AN. Vaspin acts as a coordinator of the dynamic balance between bone formation and resorption processes; its action is affected by the cytokines of the RANKL/RANK/OPG system. Changes in the relationships between vaspin, bone markers, OPG, and RANKL might contribute to the development of osteoporosis in girls with AN. (Endokrynol Pol 2016; 67 (6): 599-606).