Quality of medical follow-up of young women with Turner syndrome treated in one clinical center.

For Turner syndrome (TS) patients, smooth transition from pediatric to adult health care is a critical point. The study objective was to evaluate the medical follow-up of young women with TS in one clinical center 3 years after the latest guidelines had been introduced by the TS Study Group. A questionnaire study was performed in 59 TS adults selected from a database of 117 patients. Twenty-two of them, aged 23.0 ± 2.8 years, consented to participate. Nineteen responders (86.4%) were followed up by general practitioners who were not aware of the TS diagnosis in 14 (63.6%) cases. Eight (36.4%) were seen regularly by the relevant specialists. Adequate medical assessment varied from 5% (celiac serology) to 74% (gynecology assessment) and 82% (ear-nose-throat) of participants. None of the patients had undergone all of the recommended investigations according to recommendation. Height deficiency, body mass index, age at TS diagnosis and level of education did not correlate with the number of assessments performed (p = 0.687, p = 0.810, p = 0.641, and p = 0.568, respectively). Three years after the introduction of the current guidelines, medical follow-up in the transition phase is still inadequate. Improvement in transitional health care is warranted through better patient education, referring to physicians caring for adults with TS and better cooperation with general practitioners with wider popularization of the TS recommendations among them.

Blood pressure regulation and resting heart rate abnormalities in adolescent girls with polycystic ovary syndrome.

OBJECTIVE: To assess the risk of cardiovascular disease on the basis of biochemical, echocardiographic, and 24-hour blood pressure (BP) monitoring parameters in adolescent girls with polycystic ovary syndrome (PCOS). DESIGN: Cross-sectional study. SETTING: Academic and research institution. PATIENT(S): Thirty-four obese and nonobese girls with PCOS were evaluated and compared with body mass index-matched girls with regular menses. INTERVENTION(S): None. MAIN OUTCOME MEASURE(S): Androgens, gonadotropins, lipids, and fasting and oral glucose tolerance test-stimulated glucose and insulin concentrations were measured. Echocardiographic assessment and 24-hour BP monitoring were done. RESULT(S): Compared with obese controls, obese girls with PCOS had significantly higher 24-hour mean BP, day mean BP, day diastolic BP, and diastolic BP nighttime dip (75.5 ± 4.5 mm Hg vs. 71.7 ± 3.7 mm Hg; 78.2 ± 5.0 mm Hg vs. 73.6 ± 4.0 mm Hg; 67.6 ± 4.9 mm Hg vs. 63.7 ± 3.7 mm Hg; and 20.2% ± 5.2% vs. 15.0% ± 6.6%, respectively). Obese girls with PCOS had significantly higher night heart rate than obese controls (60.4 ± 5.6 beats per minute vs. 61.7 ± 4.8 beats per minute). Left ventricle end-diastolic (4.6 ± 0.3 cm vs. 4.2 ± 0.2 cm) and end-systolic diameter (3.0 ± 0.3 cm vs. 2.7 ± 0.2 cm) were also significantly higher in nonobese girls with PCOS than in nonobese controls; however, all values were still within the accepted range of normal limits. CONCLUSION(S): Higher night heart rate in obese girls with PCOS and higher day BP but preserved diastolic nocturnal dip in nonobese girls with PCOS may be regarded as early cardiovascular disease risk factors.

Are metabolic syndrome and its components in obese children influenced by the overweight status or the insulin resistance?

INTRODUCTION: The aim of this study was to determine which factors increase the risk of metabolic syndrome (MS) and its components in obese children and adolescents. MATERIAL AND METHODS: In 78 obese children (42 girls, 36 boys), mean age 14.6 ± 3.5 years, blood pressure, total cholesterol, triglycerides, HDL-cholesterol (HDL), insulin and glucose at fasting state as well as in OGTT were measured. Body mass index (BMI) Z-score, LDLcholesterol, and insulin resistance indices (FIGR, R-HOMA) were calculated. RESULTS: Metabolic syndrome was diagnosed in ten (12.8%) children. Hyperinsulinaemia was present in 42 (53.8%) subjects, increased FIGR in eight (10.3%) and increased R-HOMA in 49 (62.3%). Significant correlations between BMI Z-score ≥ 2.5 and MS occurrence and its components (hypertriglyceridaemia, isolated systolic and diastolic hypertension) were found. Hypertriglyceridaemia, low HDL and hypertension, as well as MS occurrence, correlated significantly with stimulated hyperinsulinaemia and increased FIGR. Risk of hypertension was increased 5.6 times by fasting hyperinsulinaemia. Stimulated hyperinsulinaemia increased the risk of hypertriglyceridaemia 3.7 times, risk of low HDL 14.4 times and risk of MS 10.3 times. These risks did not change significantly when adjusted for BMI Z-score. CONCLUSIONS: Our study results show that both BMI Z-score and OGTT stimulated hyperinsulinaemia are good predictors of MS occurrence in obese children and adolescents. The risk of dyslipidaemia and hypertension increase significantly with hyperinsulinaemia and insulin resistance, with low HDL cholesterol being the most affected.

[Causes of precocious puberty in children referred for evaluation in hospital conditions]. / Przyczyny przedwczesnego dojrzewania dzieci kierowanych do dianostyki w warunkach szpitalnych.

INTRODUCTION: Symptoms of precocious puberty (PP) in children always arouse anxiety in their parents. Many children with PP are being hospitalized for the detailed diagnostic work-up. The aim of our study was to analyze the frequency of the variants of PP in children referred to our department. MATERIAL: Retrospective analysis of 119 children (103 girls and 16 boys) referred for hospitalization in the years 2003-2005 due to signs of precocious puberty was performed. RESULTS: Premature thelarche, benign variant of puberty, was diagnosed in 62 (53%) girls, in the mean age of 3.39 (+/- 2.33) years. Their mean height was within 0.7 +/- 1.1 SD. Premature pubarche was diagnosed 30 (25%) children--22 girls and 8 boys in the mean age was 7.24 (+/- 0.81) years. Their mean height was 1.3 +/- 1.0 SD and was significantly higher than normal (p < 0.0001). Premature menarche was diagnosed in 8 (7%) girls in the mean age 4.81 +/-2.26 years. Mean height in this group was normal for age (0.9+/-0.8 SD). PP was diagnosed in 19 (16%) children (11 girls and 8 boys) in the mean age 5.91 +/- 1.63 years. Mean height in this group was 1.6 +/- 0.7 SD, and was significantly higher than the mean for age (p<0.0005). GnRH-dependent type was present in 15 children, diagnosed as idiopathic in 9 girls and 1 boy. In 5 children (4 boys and 1 girl) pathology of central nervous system was found. In 4 children GnRH-independent precocious puberty was diagnosed--in 3 caused by congenital adrenal hyperplasia and in 1 boy by tumour of testis (leydigioma). CONCLUSIONS: Girls with precocious thelarche without growth acceleration present the benign variant of puberty and need clinical follow up only. Boys with clinical signs of precocious puberty should be carefully evaluated to rule out the organic cause.

[Influence of fat tissue distribution on metabolic complications in children and adolescents with simple obesity]. / Wplyw rozmieszczenia tkanki tluszczowej na zaburzenia metaboliczne u dzieci i mlodziezy z otyloscia prosta.

BACKGROUND: Increased prevalence of obesity in children and adolescents results in more common metabolic complications characteristic for adults, particularly those with abdominal obesity. The objective of the study was to determine the relation between the fat tissue distribution and metabolic complications and to estimate the prevalence of the metabolic syndrome in obese children and adolescents. MATERIAL AND METHODS: We studied 64 children (42 girls and 22 boys) with simple obesity (BMI =97 pc) in the mean age 12.4+/-3.4 years. The fat tissue distribution was assessed on the basis of waist circumference, hip circumference, waist to hip ratio (WHR) and skinfold thickness (suprailiac, subscapular, biceps and triceps). In all children plasma concentrations of total cholesterol, HDL and LDL cholesterol as well as triglycerides were estimated. Plasma glucose and insulin levels were measured in fasting state and during the oral glucose tolerance test (OGTT). Fasting insulin to glucose ratio (FIGR) was calculated. Blood pressure was measured in triplicate. RESULTS: In 33 (51.6%) of children dyslipidemia, in 10 (15.6%) hyperinsulinemia or impaired glucose tolerance and in 12 (18%) hypertension was diagnosed. The Metabolic syndrome was present in 9 (14%) children. The anthropometric predictor for the risk of metabolic complications was a greater waist circumference, while greater hip circumference decreased the risk. CONCLUSIONS: The metabolic complications characteristic of metabolic syndrome, previously diagnosed exclusively in adults, may occur also in obese children and adolescents. As in adults, abdominal obesity is the most relevant risk factor of the metabolic syndrome.