Volume CXIV, Number 4, November 7, 1996

Objective: Turner syndrome (TS) is a chromosomal disorder caused by complete or partial X chromosome monosomy that manifests various clinical features depending on the karyotype and on the genetic background of affected girls. This study aimed to systematically investigate the key clinical features of TS in relationship to karyotype in a large pediatric Turkish patient population.Methods: Our retrospective study included 842 karyotype-proven TS patients aged 0-18 years who were evaluated in 35 different centers in Turkey in the years 2013-2014.Results: The most common karyotype was 45,X (50.7%), followed by 45,X/46,XX (10.8%), 46,X,i(Xq) (10.1%) and 45,X/46,X,i(Xq) (9.5%). Mean age at diagnosis was 10.2±4.4 years. The most common presenting complaints were short stature and delayed puberty. Among patients diagnosed before age one year, the ratio of karyotype 45,X was significantly higher than that of other karyotype groups. Cardiac defects (bicuspid aortic valve, coarctation of the aorta and aortic stenosis) were the most common congenital anomalies, occurring in 25% of the TS cases. This was followed by urinary system anomalies (horseshoe kidney, double collector duct system and renal rotation) detected in 16.3%. Hashimoto's thyroiditis was found in 11.1% of patients, gastrointestinal abnormalities in 8.9%, ear nose and throat problems in 22.6%, dermatologic problems in 21.8% and osteoporosis in 15.3%. Learning difficulties and/or psychosocial problems were encountered in 39.1%. Insulin resistance and impaired fasting glucose were detected in 3.4% and 2.2%, respectively. Dyslipidemia prevalence was 11.4%.Conclusion: This comprehensive study systematically evaluated the largest group of karyotype-proven TS girls to date. The karyotype distribution, congenital anomaly and comorbidity profile closely parallel that from other countries and support the need for close medical surveillance of these complex patients throughout their lifespa

Is presence of hypertension in obese children correlate with the criteria of metabolic syndrome?

WOS: 000251988800011PubMed ID: 17596290In this article, we will describe the presence of metabolic syndrome and its components in a group of hypertensive and obese adolescents. The study presented here was conducted on 20 patients (10 boys) presented with complaints of obesity and hypertension who were diagnosed as metabolic syndrome. In 20 patients who were all obese and hypertensive, existence of a third metabolic syndrome component such as glucose intolerance or dyslipidaemia, was 47% and 35% respectively, whereas existence of both was 55%. Only three of the patients carry all of the five criteria of metabolic syndrome. Mean body mass index and mean blood pressure (P=0.021), uric acid (P=0.046) and fasting blood glucose levels (P=0.023) were found statistically significant. Mean blood pressure levels were related with increase at LDL-cholesterol (P=0.029) and increase in fasting blood glucose levels (P=0.04). Prevention of complications by effective measures in the metabolic syndrome patients is also mentioned. We believe that obese and hypertensive children should be screened for the other components of metabolic syndrome

Agricultural Pesticides and Precocious Puberty

WOS: 000331015200002PubMed ID: 24388186The onset and course of puberty is under the control of the neuroendocrine system. Factors affecting the regulation of timing and order of this system's functions may alter the onset and course of puberty. Several environmental endocrine disruptors (EDs) with significant influences on the normal course of puberty have been identified. Despite the numerous animal and human studies on EDs that may extensively affect human health, there are still several issues that need to be clarified. This chapter discusses the effects of pesticides, which constitute a significant portion of disruptors and have been increasingly used in agriculture, on precocious puberty

Is anti-Mullerian hormone an indicator of potential polycystic ovary syndrome in prepubertal girls with simple obesity?

WOS: 000398205800010PubMed ID: 28276214The aim of this anti-Mullerian hormone (AMH) levels in prepubertal obese girls are a predictive marker for polycystic ovary syndrome (PCOS) and to investigate the relationship between insulin resistance and AMH. Sixty girls with premature pubarche or obesity and 20 healthy controls between the ages of 6-9 were enrolled. Of the patients, 22 (36.7%) were in the obese group (Group 1), 28 (46.7%) in the early pubarche group (Group 2) and 10 (16.6%) in the early pubarche + obese group (Group 3). Comparison of the subjects' fasting insulin and homeostatic model assessment insulin resistance (HOMA-IR) demonstrated significantly higher values for group 1 compared to group 2 (p=0.001 and 0.001) and, likewise, significantly higher values for group 3 compared to group 2. There was no significant differences between all groups for AMH levels. AMH levels were not significantly different in the obese girls compared to the other groups. There was also no relationship between AMH and insulin resistance in any of the groups. Further studies, however, are needed due the limited number of subjects in this study and in the absence of adequate relevant data.Department of Research Project of Ege UniversityEge UniversityThis study was supported by the Department of Research Project of Ege University

Possible problem with optipen pro-1 - Should diabetic patients continue to use this product?

WOS: 000238854000046PubMed ID: 1680160

Acute guttate psoriasis associated with streptococcal perianal dermatitis

WOS: 000243200600011PubMed ID: 1716451

Splenogonadal fusion-limb deformity syndrome: a rare but important cause of undescended testis

WOS: 000372302000020PubMed ID: 26582293Splenogonadal fusion is a rare congenital anomaly which is characterized by fusion formation between the spleen and gonad. We report a case of a 14-month boy with spleongonadal fusion-limb deformity syndrome focusing on the importance of awareness of this syndrome. The patient was admitted to our clinic because of a left undescended testis, and preoperative diagnosis was not made. During the operation, "spleen-like" tissue attached to the gonad induced splenogonadal fusion, which was confirmed by laparoscopy. The patient also had a short right femur, hip dysplasia and a syndromic face. Splenogonadal fusion anomaly should be considered in the evaluation of undescended testis, especially in patients with facial and limb deformities

Are there any risks of hyperglycaemia during acute febrile illness at pediatric emergency department?

WOS: 00027048990097