Background Fibroblast growth factor 23 (FGF23) is a recently discovered bone-derived regulator of vitamin D metabolism and phosphate homeostasis. It inhibits phosphate reabsorption and calcitriol production by the kidney. Myelomeningocele (MMC) remains the most severe form of neural tube defects involving serious locomotor disability, osteoporosis and pathologic fractures. We aimed to investigate the influence of vitamin D replacement therapy on serum FGF23 concentration in children with MMC and compare the results with healthy participants. Methods This prospective analysis was conducted on 16 children with MMC and 20 healthy children. Serum FGF23 levels were measured; for the studied group, before and after vitamin D replacement therapy with cholecalciferol (vitamin D3). The children’s medical charts were analyzed to determine age, sex, anthropometric measurements, calcium and phosphate, cholecalciferol and renal function parameters. Results There were significant differences in vitamin D and FGF23 serum concentrations between the studied groups. The median vitamin D levels in the MMC group increased during replacement therapy (7 vs. 18.5 ng/mL, p = 0.29) in comparison to the median of 25.5 ng/mL in the control group. In MMC children we found a significant decrease in median serum FGF23 after vitamin D replacement therapy (from 42.1 to 0 RU/mL, p < 0.001). FGF23 correlated positively with albumin, serum and urine phosphate levels and negatively with alkaline phosphatase. Conclusions 1. Serum concentration of FGF-23 is increased in MMC children in comparison to a healthy control group. 2. Vitamin D replacement therapy decreases FGF23 concentrations in MMC children, although further studies are still warranted to gain detailed insight on the FGF23 in the MMC population. 3. Children with MMC present vitamin D deficiency. Nutrition supplemented with low doses of cholecalciferol (vitamin D3) (intakes reaching recommended daily allowances) was insufficient to correct 25(OH)-D level in that population of patients.

Introduction: A disturbed calcium-phosphate balance is an important issue for kidney stone formation in nephrolithiasis. Hypercalciuria (HC) has been proposed as an essential etiology of monosymptomatic nocturnal enuresis (MNE). Objectives: We may suspect that patients with MNE may be at risk of stone formation hence the objective of this paper was to assess the risk in MNE children using Bonn Risk Index (BRI). Patients and Methods: The urinary work-up of 204 children (83 with MNE and 121 controls) included urinary calcium (Ca), magnesium (Mg) and sodium (Na) excretion, Ca/creatinine ratio, BRI, ionized calcium (Ca2+), Mg/creatinine and Ca/citrate ratios, urinary citrates and oxalates (Ox). Results: Ca/creatinine and Mg/creat ratios were higher in the MNE group. There were no differences in Mg and Ca amount in urine and Mg/Ca ratio between MNE and the reference group. Both groups differed in Mg and Ca excretion per kg of body mass. MNE children differed from controls regarding BRI, Ox and urinary Ca2+. No differences in urinary citrate excretion nor Ca/citrate ratio between MNE and the controls were found. Correlations between factors important in the crystallization process in MNE children were recorded. Conclusion: MNE patients may be at risk of oxalate nephrolithiasis. Further studies to assess the role of the BRI and Ca/citrate ratio in predicting stone formation in MNE children are needed.

Aim: We wanted to investigate serum levels of ghrelin and leptinappetite-regulating hormonesand their correlation with the nutritional status of children with neurogenic bladder (NB) due to myelomeningocele (MMC) in comparison to healthy individuals. Methods: This prospective analysis was conducted on 67 children with NB after MMC and 20 healthy children. Children's medical charts were analysed to determine age, gender, anthropometric measurements, body mass index (BMI), activity assessment using Hoffer's scale and renal function parameters. Serum total ghrelin and leptin levels were measured using the enzyme-linked immunosorbent assay. Results: There were no differences in the age, gender, weight and BMI between the studied groups. Median serum levels of ghrelin and leptin were higher compared with the reference group. A significant negative correlation between serum leptin concentration and Hoffer's scale was found in children with NB. Conclusions: Elevated levels of leptin and ghrelin could be considered factors influencing nutritional status in children with NB due to MMC. Children with NB after MMC may have disturbed endocrine regulation of energy homeostasis. Physical activity may be the factor that affects serum leptin concentration.

Introduction: Myelomeningocele (MMC) is the first cause of neurogenic bladder in children. Adequate nephrourological management is crucial to prevent renal damage and bladder dysfunction. In the last decade, the prognosis of MMC children has dramatically improved, but can we do better? We wanted to detect risk factors involved in renal deterioration in MMC patients. Material and methods: This retrospective analysis was based on 178 children with MMC (99 girls and 79 boys) from two Polish hospitals, in Bialystok and Cracow. The children's medical records were analysed to determine sex, age, and kidney and urinary tract ultrasound, voiding cystourethrogram, and urodynamic findings. The number of clean intermittent catheterisations performed per day, cystatin C, serum creatinine, urea, and glomerular filtration rate (GFR) Schwartz were also recorded. The Hoffer scale was used to assess MMC children's motor function. Results: Of the 111 from Bialystok and 67 children from Cracow, with a median age of nine years, 45 (25%) had vesicoureteral reflux. The most common urodynamic finding was overactive bladder with a prevalence of 59%. There were statistically significant differences between girls and boys, including the presence of reflux, increased echogenicity of the kidneys' parenchyma in ultrasound examination and cystometric capacity in urodynamic study. There were statistically significant differences between the Hoffer scale groups, including GFR Schwartz. The number of performed urodynamics correlated with GFR Schwartz, serum concentrations of cystatin C, and creatinine. Conclusions: Potential risk factors of renal deterioration in children with MMC are abnormalities in ultrasound, the presence of high-grade vesicoureteral reflux in cystogram, increased bladder pressure on urodynamics, hyperfiltration, and increased levels of cystatin C. Urodynamic evaluation may play a role in the management of children with MMC by identifying those with increased bladder pressures. Frequent urodynamic assessment may contribute to prevention of renal function in children with MMC.

A -study Design, B -Data Collection, C -statistical analysis, D -Data interpretation, E -Manuscript Preparation, F -Literature search, G -Funds Collection Background. Myelomenigocele (MMC) is the most severe form of spina bifida, caused by genetic and environmental components; it has many serious complications in a patient's future life, where dysfunctional mobility is one of the most troublesome. Objectives. The aim of our study was to explore the role of selected factors: parental and perinatal in children with myelomenigocele and correlate them with their future motor function. Material and methods. This retrospective analysis was based on 95 births that occurred from 1985 to 2013. Clinical data were collected using medical records and supplemented by a questionnaire for parents. standard deviation of WHo scores (Z-scores) for weight, length, and body mass index (BMi) at birth were calculated. Hoffer's scale was used to assess MMC children's motor function.Results. There were no statistically significant differences between 53 girls and 42 boys who were enrolled in the study, excluding the month of conception. The tested parameters did not vary substantially according to maternal age. Maternal age correlated positively with paternal age at conception, birth order, and stillbirth rate. Mode of delivery was strongly associated with apgar scale and birth weight. apgar score, birth length, and thus WHo Z-score for length-for-age had positive correlations with future mobility of MMC children. Conclusions. 1. Perinatal factors have a significant effect on children's physical activity. 2. There is no clinical evidence that cesarean section improves early neurological outcome of MMC infants. 3. Parental factors, particularly maternal age, do not determine the future mobility of children with myelomeningocele. Key words: myelomeningocele, parental factors, perinatal factors, motor function.Wstęp. Przepuklina oponowo-rdzeniowa jest najcięższą postacią rozszczepu kręgosłupa, której przyczyny mogą być genetyczne i środowiskowe; daje wiele ciężkich powikłań w przyszłym życiu pacjentów, wśród których trudności w poruszaniu się są jednymi z najbardziej uciążliwych. Cel pracy. ocena wybranych czynników: rodzicielskich i okołoporodowych i ich korelacja z czynnością motoryczną dzieci z przepukliną oponowo-rdzeniową. Materiał i metody. Retrospektywnej analizie poddano 95 historii pacjentów objętych opieką w latach od 1985 do 2013. Dane zostały uzupełnione wywiadem od rodziców dzieci. Dla takich parametrów jak: ciężar ciała, długość oraz wskaźnik masy ciała (BMi) w momencie urodzenia wyliczono odchylenia standardowe według WHo. Do oceny czynności motorycznej uży-to skali Hoffera. Wyniki. nie wykazano istotnych statystycznie różnic między 53 dziewczynkami i 42 chłopcami, z wyjątkiem miesiąca urodzenia. Wartości badanych parametrów nie różniły się istotnie w zależności od wieku matki. Wiek matki korelował pozytywnie z wiekiem ojca w momencie poczęcia dziecka, rodzajem porodu i częstością poronień. Rodzaj porodu korelował ze skalą ...