Determinants of Increased Aortic Diameters in Young Normotensive Patients With Turner Syndrome Without Structural Heart Disease.

Factors associated with aortic dilation and dissection in patients with Turner syndrome (TS) remain unclear. We assessed magnetic resonance imaging-based aortic diameters at nine predefined anatomic positions and examined associations of increased aortic diameters with B-type natriuretic peptide (BNP), A-type NP (ANP), growth hormone treatment, insulin-like growth factor 1 (IGF1), and estrogen status. Forty-seven patients with TS aged 7.3-21 years and 34 healthy peers were enrolled in this study. Aortic diameters were higher in patients with TS at three positions than in controls (p < 0.05). History of GH treatment, pubertal status, and serum estradiol levels were not associated with increased aortic diameters. Patients with TS had higher plasma BNP and ANP levels than controls. BNP and IGF1 were independently associated with the increase in aortic diameters in TS at three positions of the ascending aorta (R2 = 0.361-0.458, p < 0.05 for all). At two positions of the descending aorta, only BNP emerged as an independent variable (R2 = 0.130-0.139, p < 0.05). We conclude that young, normotensive patients with TS had greater aortic diameters at several positions than healthy controls. BNP and IGF1 were independently associated with increased aortic diameters in TS.

Sonography and Magnetic Resonance Imaging Characteristics of Testicular Adrenal Rest Tumors.

BACKGROUND: The aim of this study was to describe the gray-scale and color Doppler ultrasonography (US) and magnetic resonance (MR) imaging features of testicular adrenal rest tumors (TART) in patients with congenital adrenal hyperplasia. MATERIAL/METHODS: Forty-one patients with congenital adrenal hyperplasia were evaluated by gray-scale and color Doppler ultrasonography. Totally eighteen adrenal rest tumors in 9 patients were diagnosed TART on US and MR imaging. Gray-scale and color Doppler US and MR findings of the patients were documented. RESULTS: A total of eighteen masses were evaluated in nine patients. The mean age of these patients was 14.3±4.5 (range 10.1-23.3) years. US revealed hypoechoic lesions around the mediastinum testis with hypervascularity dispersing in ten patients and hypovascularity in two patients. In six patients, the lesions were hyperechoic with poor vascularity. Lesions exhibited homogeneous (n=8) and heterogeneous (n=10). Testicular microlithiasis was present in 4 of 9 patients with TART. Doppler ultrasound showed normal testicular vessels passing through the mass which were undisturbed, not displaced and not change in caliber. MRI features were the following: all lesions were hypointense on T2- and hyperintense (n=12) and isointense (n=6) on T1-weighted images. All masses revealed homogeneous contrast enhancement on postcontrast T1-weighted images. CONCLUSIONS: Ultrasonography and MRI are good methods for detecting and monitoring TART. US is the first preferable modality because it is quick and cheap than MRI. Bilateral mostly hypoechoic lesions depicted around the mediastinum testis with no mass effect is highly suggestive for the diagnosis of testicular adrenal rest tissues on ultrasonography. Normal testicular vessels coursing through the lesions undisturbed and not change in caliber is described specific for this kind of tumors.

Increased arterial stiffness in young normotensive patients with Turner syndrome: associations with vascular biomarkers.

OBJECTIVES: Factors contributing to arteriopathy in patients with Turner syndrome (TS) remain unclear. We assessed arterial stiffness in young, normotensive patients with TS and correlated arterial stiffness with vascular biomarkers, GH treatment and oestrogen exposure. Sixty-one patients with TS (mean age, 12·6 years; range 6·6-21·3 years) were matched for age and sex with 61 healthy peers. Associations between arterial stiffness and high-sensitivity C-reactive protein (hsCRP), B-type natriuretic peptide (BNP), atrial NP (ANP), plasma aldosterone/plasma renin activity (PRA), IGF1 and IGFBP3 were examined after adjusting for well-established confounders of vascular disease. RESULTS: Carotid intima media thickness standard deviation score (SDS), arterial stiffness index SDS and incremental modulus of elasticity SDS were higher, and distensibility coefficient SDS was lower in patients with TS. The duration of GH treatment and oestrogen exposure was not associated with indices of arterial stiffness. TS patients had higher hsCRP, BNP and ANP. Plasma aldosterone/PRA, IGF1 and IGFBP3 were similar in patients and controls. Multivariable regression analyses (R(2) = 0·200-0·668, P < 0·01) showed that BNP was associated with all indices of arterial stiffness. We found that hsCRP was associated with distensibility coefficient SDS (ß = -0·16, P < 0·01). TS was independently associated with increased arterial stiffness (ß = 0·420-3·424, P < 0·001 for all, R(2) = 0·06-0·31). CONCLUSIONS: Young, normotensive TS patients had increased arterial stiffness than that of healthy peers. BNP, and possibly hsCRP, was independently associated with arterial stiffness in TS. Further research will determine any causal inference of these relationships.

Pelvic ultrasound findings in prepubertal girls with precocious adrenarche born appropriate for gestational age.

BACKGROUND: Precocious adrenarche (PA) refers to the clinical onset of excess androgen in girls before the age of 8. It is associated with an increased risk of functional ovarian hyperandrogenism after puberty. PA may be associated with polycystic ovary syndrome (PCOS). We compared pelvic ultrasound (US) findings of girls with PA born appropriate for gestational age (AGA) to healthy body mass index (BMI)-matched peers to determine whether US findings in AGA-born PA girls are associated with PCOS antecedents. SUBJECTS AND METHODS: We conducted a cross-sectional study on 56 AGA-born girls with PA (6·9 ± 0·6 years) and 33 BMI-matched prepubertal AGA-born peers (7·1 ± 1·0 years). Hormonal data, homeostasis model assessment of insulin resistance (HOMA-IR), insulin sensitivity index (ISIcomp ) and pelvic US findings were compared. Associations of pelvic US findings with clinical and metabolic data were investigated. RESULTS: Precocious adrenarche girls had greater height and bone age-adjusted uterine length (UL; P = 0·01) and UL standard deviation score (SDS) (P = 0·02) than BMI-matched peers. Mean ovarian volume (MOV), MOV SDS, uterine volume, uterine cross-sectional area and ovarian morphology were similar between the groups (P > 0·05). MOV and MOV SDS correlated with ISIcomp (r = -0·683, P < 0·001; r = -0·760, P < 0·001; respectively). Correlations of pelvic US findings with other biochemical data did not reach significance (P > 0·05). Multivariate regression analysis revealed that in girls with PA, ISIcomp had the most significant effect on MOV SDS (R(2) = 0·731, ß = -4·784, P = 0·001). CONCLUSIONS: Appropriate for gestational age-born PA girls have greater UL measurements than AGA-born BMI-matched peers. In AGA-born girls with PA, decreasing insulin sensitivity is strongly and independently associated with an increase in MOV. Longitudinal follow-up of our cohort after menarche will allow us to establish how many AGA-born girls with PA will ultimately develop PCOS.

Sitting height and sitting height/height ratio references for Turkish children.

UNLABELLED: Sitting height (SHt) measurements and sitting height/height (SHt/Ht) ratio are important criteria in the diagnosis of growth problems and particularly in the diagnosis of dysproportionate growth. It is known that body proportions are related to genetic influences and show variations among different populations. This study aimed to provide reference data on SHt and SHt/Ht ratios for Turkish children of ages 6-18 years. SHt measurements were performed on a sample of 1,100 boys and 1,020 girls between 6 and 18 years of age attending primary and secondary schools located in six different districts of Istanbul city. Criteria advanced by WHO for establishing reference standards for growth were observed in the study design. The sample consisted of a mixture of children measured only once and those measured at follow-up over different periods of time. Parallel to increase in Ht, SHt increased with age. Mean value for SHt/Ht ratio was 55-56% at ages 6 to 8.5 years in both sexes. In girls, this value started to decrease at age 11.5 years and remained between 53% and 54% thereafter. In the boys, a decrease to 52-53% was noted in the SHt/Ht ratio after age 12 years. In both sexes, SHt/Ht ratio decreased with puberty, demonstrating that growth in trunk length exceeded growth in limb length in midpubertal ages. These changes occurred at an earlier age in the girls. Values obtained for SHt/Ht ratios in Turkish children were high as compared to Dutch children and low as compared to Chinese children. CONCLUSION: This study, by providing reference data on sitting height and sitting height/height ratios in Turkish children of ages between 6 and 18 years, will be useful in the diagnosis and follow-up of children with growth problems. This study also supports the view that body proportions are influenced by genetic makeup.

Netherton syndrome associated with growth hormone deficiency.

Netherton syndrome (NS) is a rare autosomal recessive disorder characterized by ichthyosiform scaling, hair abnormalities, and variable atopic features. Mutations in the serine protease inhibitor Kazal type 5 (SPINK5) gene leading to lymphoepithelial Kazal-type-related inhibitor (LEKTI) deficiency cause NS. Growth retardation is a classic feature of NS, but growth hormone (GH) deficiency with subsequent response to GH therapy is not documented in the literature. It is proposed that a lack of inhibition of proteases due to a deficiency of LEKTI in the pituitary gland leads to the overprocessing of human GH in NS. Herein we report three patients with NS who had growth retardation associated with GH deficiency and responded well to GH therapy.

Are metabolic syndrome antecedents in prepubertal children associated with being born idiopathic large for gestational age?

INTRODUCTION: Being born large for gestational age (LGA) is a risk factor for development of metabolic syndrome (MS) in adolescents and adults. OBJECTIVE: To evaluate prepubertal children born idiopathic LGA to non-obese mothers without gestational diabetes or glucosuria with respect to the presence of MS antecedents. PATIENTS AND METHODS: We conducted a cross-sectional study to compare 40 (19 F) LGA-born prepubertal children of a mean age of 6.1 ± 2.5 yr and 49 (25 F) appropriate for gestational age (AGA)-born body mass index (BMI)-matched peers of a mean age of 5.4 ± 1.8 yr with respect to their anthropometric data, blood pressure measurements, fasting serum glucose and insulin levels, homeostasis model assessment-insulin resistance (HOMA-IR), and lipids and atherogenic index (AI) [triglyceride (TG)/high-density lipoprotein cholesterol (HDL-C)]. HOMA-IR > 2.5 was used to define IR. HDL-C ≤ 40 mg/dL and TG ≥ 110 mg/dL were used to define dyslipidemia. Both groups were further divided into subgroups as obese and non-obese according to their BMI percentiles and the analyses were repeated. RESULTS: Non-obese LGA children had higher waist circumference (WC) standard deviation scores (SDSs) than BMI-matched AGA-born peers (p = 0.024). There were no significant differences between pooled, obese and non-obese subgroups of LGA-born children and their AGA counterparts with respect to dyslipidemia and IR. AI was higher in non-obese LGA children than in AGA counterparts (p = 0.028). CONCLUSIONS: Non-obese idiopathic LGA-born children have higher AIs than AGA-born counterparts in the absence of IR. WC seems to be a good clinical screening tool in identifying at risk of non-obese LGA children. Further studies are needed to evaluate MS antecedents in idiopathic LGA-born children.

Precocious adrenarche in children born appropriate for gestational age: is there a difference between genders?

We aimed to determine whether precocious adrenarche (PA) has a different impact on screening tests for metabolic issues and pubertal timing in boys and girls born appropriate for gestational age (AGA). Puberty and initial metabolic screening results of 47 girls and 23 boys with PA born AGA followed up from our outpatient endocrinology clinic between May 2000 and October 2009 were reviewed. Initial anthropometric measurements except for body mass index standard deviation score (SDS) being higher in boys than girls (p = 0.01), bone age (BA) SDS, homeostasis model assessment of insulin resistance, and plasma lipids were similar between sexes. Hormone levels except for significantly higher dehydroepiandrosterone sulfate levels in boys than girls (p = 0.0006) were also similar between the sexes. BA SDS and BA/chronological age were significantly advanced (p < 0.05) with respect to initial evaluation in 28 girls at onset of gonadarche unlike the case in 13 boys with PA (p > 0.05). In conclusions, PA in children born AGA does not herald any significant differences with respect to adverse metabolic screening results between sexes, and it appears to be a discrete process from onset of puberty in girls unlike boys, in whom it is likely a variant of normal puberty.

Evaluation of adrenomedullary function in patients with congenital adrenal hyperplasia.

BACKGROUND/AIMS: Congenital adrenal hyperplasia (CAH) is characterized by adrenal insufficiency with or without salt wasting. It is also accompanied by adrenomedullary hypofunction. The aim of the present study was to investigate adrenomedullary function in patients with CAH due to 21-hydroxylase and 11beta-hydroxylase deficiencies and in age-matched normal subjects. METHODS: We measured plasma catecholamines (epinephrine and norepinephrine) and urine metanephrine in 44 patients with CAH, 32 due to 21-hydroxylase deficiency (17 patients with the salt-wasting form and 15 patients with the simple virilizing form), and 12 due to 11beta-hydroxylase deficiency, and in 25 healthy controls. RESULTS: Plasma epinephrine and urine metanephrine levels were significantly higher in the controls than in patients with CAH (p = 0.02 and p < 0.001, respectively). Plasma norepinephrine levels were significantly lower in the controls than in patients with CAH (p < 0.001). Interestingly, patients with the salt-wasting form had lower norepinephrine levels in comparison to the other subgroups of CAH. CONCLUSION: Despite the fact that CAH patients have insufficient epinephrine secretion, these patients have the ability to increase compensatory norepinephrine. However, this increase is much lower in patients with the salt-wasting form. These findings need to be confirmed by other studies.

Target height estimation in children with idiopathic short stature who are referred to the growth clinic.

AIM: It was the aim of this study to evaluate adult height (AH) and different methods used for estimation of target height (TH) in children with idiopathic short stature (ISS). METHODS: Eighty-five ISS children (36 female, 49 male) were followed until AH was evaluated retrospectively. TH was calculated according to the following 4 methods: (1) as +/-6.5 cm to the mean parental heights for boys or girls, respectively, (2) as the mean standard deviation score (SDS) of the parents' heights, (3) as the sum of the SDS of the parents' heights divided by 1.61, and (4) as the mean SDS of the parents' heights multiplied by 0.72. ISS was classified as familial short stature (FSS) if the height was within the TH range and as nonfamilial short stature (NFSS) if it was below the TH range. RESULTS: The number of FSS and NFSS children differed by the method chosen. The mean AH SDS was lower than the TH SDS in FSS in all methods, except in method 3. NFSS children did not attain their TH in either of the methods. CONCLUSIONS: Classification of ISS depends on the method of the TH range chosen. ISS children reach a mean AH SDS lower than the mean TH SDS. Only FSS children classified by method 3 reached a mean AH SDS close to the mean TH SDS.

The role of leptin, soluble leptin receptor, resistin, and insulin secretory dynamics in the pathogenesis of hypothalamic obesity in children.

INTRODUCTION: In this study, we have investigated the role of leptin, soluble leptin receptor(sOb-R), resistin, and insulin secretory dynamics in the development of hypothalamic obesity. MATERIALS AND METHODS: Children who had hypothalamo-pituitary tumor were divided into two groups. First group included obese-overweight (hypothalamic obese = HOB group, n = 23) and second group included non-obese children (hypothalamic non-obese = HNOB group, n = 16). Exogenously obese-overweight children (OB group, n = 22) were included as controls. Basal and second-hour serum glucose and insulin in oral glucose tolerance test (OGTT), basal serum leptin, sOb-R, resistin levels, and homeostasis model assessment (HOMA) indexes were compared between the groups. RESULTS: Age, sex, and pubertal status were similar in study groups. Median and interquartile ranges of body mass index (BMI) z scores were similar in HOB and OB groups (2.0 (1.5-2.1) and 2.1 (1.8-2.3), respectively). Serum leptin levels corrected for BMI were highest and total leptin/sOb-R ratios (free leptin index (FLI)) tended to be higher in HOB than HNOB and OB groups, indicating leptin resistance (leptin/BMI, 4.0 (1.6-5.2), 1.5 (0.8-3.1), and 2.5 (1.8-3.5); FLI, 2.0 (0.8-3.5), 0.6 (0.3-1.2), and 1.5 (1-2.3) in HOB, HNOB, and OB groups; respectively). Serum resistin levels were similar in groups (2.6 (1.9-3.1), 2.8 (1.7-3.4), and 3.0 (2.2-3.5) ng/ml in HOB, HNOB, and OB groups, respectively). Basal serum glucose, basal and second-hour insulin levels in OGTT, and HOMA index were higher in OB group than the HOB and HNOB groups, indicating insulin resistance in simple obesity; however, increment of insulin to same glycemic load in OGTT was highest in the HOB group indicating insulin dysregulation (p < 0.05). CONCLUSION: Hypothalamic obesity seems to be related to both dysregulated afferent (leptin) and efferent (insulin) neural outputs through the autonomic nervous system resulting in energy storage as fat.

Type 1 diabetes mellitus associated with autoimmune thyroid disease, celiac disease and familial Mediterranean fever: case report.

It is known that type 1 diabetes mellitus (type 1 DM) may be associated with other autoimmune diseases. Recently, a patient with an association of type 1 DM and familial Mediterranean fever (FMF) was reported in the medical literature. A 10.5-year-old boy was brought to our clinic with complaints of polydipsia, polyuria and weight loss and was diagnosed as diabetic ketoacidosis due to autoimmune type 1 DM. Insulin therapy was started. Elevated thyroid antibodies associated with diffuse goiter and hypothyroidism led to the diagnosis of autoimmune thyroid disease (ATD), and elevated antiendomysial antibodies and abnormal intestinal biopsy findings led to the diagnosis of celiac disease (CD). L-thyroxine therapy and gluten-free diet were initiated accordingly. At the third-year of follow-up, acute attacks of fever, abdominal pain and chest pain developed. Laboratory investigations, which were normal between the attacks, revealed elevated erythrocyte sedimentation rate, fibrinogen, white blood cell count and pleural effusion on chest X-ray during the attacks. Molecular analysis for FMF revealed compound heterozygous M694I and V726A. The patient responded well to colchicine therapy started at a dose of 1.5 mg/day. We present the second patient with type 1 DM associated with FMF who also had ATD and CD.

Frequency of Ambiguous Genitalia in 14,177 Newborns in Turkey.

CONTEXT: Limited data are available on the exact incidence of disorders of sex development (DSD) with genital ambiguity at birth. OBJECTIVE: To determine frequency of ambiguous genitalia in newborns. DESIGN: Prospective multicenter study. SETTING: Three tertiary care hospitals. PATIENTS OR OTHER PARTICIPANTS: All 14,177 babies born during the study period were included. MAIN OUTCOME MEASURES: All newborns were examined at birth; data on weeks of gestation, birth weight, and length were collected. A structured questionnaire was used for data collection. Quigley and Prader scales were used for phenotypic grading. Clinical and genetic investigations were performed. RESULTS: Eighteen babies with ambiguous genitalia were found among 14,177 newborns (1.3/1000). Fifteen newborns had 46,XY DSD, one had 46,XX congenital adrenal hyperplasia, and one had 45,X/46,XY mixed gonadal dysgenesis. Karyotype analysis was not done in one baby who died in the neonatal period. The ratio of prematurity was higher in the DSD group (44% vs 11%; P < 0.001) and the ratio of small for gestational age was also higher in the DSD group (22% vs 5%; P = 0.007). Eight babies with DSD had mothers who had additional medical conditions, such as preeclampsia, depression, insulin resistance, and gestational diabetes mellitus. CONCLUSION: The frequency of ambiguous genitalia was higher than in previous studies, but, as with any experiment, the finding should be met with caution because this study was conducted in tertiary care hospitals. In addition, lower birth weight in the DSD group supports the hypothesis that early placental dysfunction might be important in the etiology of male genital anomalies.

Evaluation of diagnosis and treatment results in children with Graves' disease with emphasis on the pubertal status of patients.

This retrospective study evaluated the clinical and laboratory characteristics at presentation and treatment results of patients with Graves' disease (GD) with respect to pubertal status. Records of 143 patients (108 F, 35 M) were reviewed in a multicenter study. At diagnosis, 38% of patients were prepubertal. Anti-thyroid drugs (ATD) were used as initial therapy. There was no significant difference in clinical and laboratory characteristics at diagnosis, during treatment and adverse reaction to ATD with respect to pubertal status. Twenty patients (7 prepubertal, 13 pubertal) reached remission on ATD. Surgery was performed in seven and radioiodine (RAI) in four patients. Duration of treatment needed to achieve remission was longer in prepubertal (4.2 +/- 1.0 yr) than in pubertal patients (3.1 +/- 1.3 yr) (p = 0.02). The rate of remission was not different between prepubertal (25.9%) and pubertal patients (33.3%) (p = 0.59). ATD were associated with low remission rate in pediatric GD and required longer duration of therapy in prepubertal patients. For definitive treatment in older children, RAI could be evaluated as the initial therapy.

The effect of growth hormone treatment on head circumference in growth hormone-deficient children.

The aim of this study was to analyze head circumference (HC) growth retrospectively in longitudinally followed growth hormone (GH)-deficient children on GH therapy. Data of 54 (25 F, 29 M) children with GH deficiency were analyzed by dividing the children into two groups: Group 1 with height age (HA) < or =5 years (yrs) (n:18) and Group 2 with HA >5 yrs (n:36). Anthropometric measurements were expressed as standard deviation score (SDS) for chronological age (CA), and HC was also expressed as SDS for CA and HA. Group 1, with CA 6.6 (2.9) yrs at onset of therapy, showed an increase in height SDS from -3.8 (1.4) to -2.4 (1.7) (p < 0.001) and in HC SDS for CA from -1.9 (1.5) to -1.3 (1.6) (p < 0.05) on 4.8 (3.5) yrs of therapy. Group 2, with CA 12.6(2.2) yrs, increased height SDS from -3.4 (1.3) to -2.5 (1.4) (p < 0.001) and HC SDS for CA from -1.2 (1.3) to -1.4(1.2) (NS). HC SDS for HA was -0.4(1.3) in Group 1 and -0.2 (1.1) in Group 2 and showed no significant change. When analyzed by quartiles for cumulative dose of GH, HC SDS for HA became 0.08(1.2) in the fourth dosage quartile (p = 0.043), not significantly different from the mean. HC is disproportionately small for age but normal for the height. GH treatment results in an increase in HC of the children towards normalization in younger children. An increase in cumulative GH dose is associated with an increase in HC, but this is not inappropriate.

Adult height in Turkish patients with Turner syndrome without growth hormone treatment.

Spontaneous adult height (AH) in Turner syndrome (TS) varies among populations. Population-specific AH data is essential to assess the efficacy of growth-promoting therapies in TS. A multicenter study was performed to establish AH of nongrowth hormone (GH)-treated Turkish patients with TS. One hundred ten patients with TS (diagnosed by karyotype) who reached AH (no growth in the previous year, or bone age > 15 years) without receiving GH treatment were included in the study. The average AH was found to be 141.6 +/- 7.0 cm at the age of 22.9 +/- 6.2 years, which is 18.4 cm below the population average and 16.4 cm below the patients' mid-parental heights. Bone age at start of estrogen replacement was 12.3 +/- 1.3 year. Karyotype distribution of the patients was 45X (43%), 45X/46XX (16%), 45X/46Xi (12%), 45XiXq (10%) and others (19%). When the patients were evaluated according to their karyotype as 45X and non-45X, no significant difference in AH was observed (142.4 +/- 6.9 cm vs 140.9 +/- 7.1 cm, respectively). Adult height of non-GH-treated Turkish TS patients obtained in this study was comparable to that of other Mediterranean populations, but shorter than that of Northern European patients. Karyotype does not seem to affect AH in TS.

Evaluation and Treatment Results of Ovarian Cysts in Childhood and Adolescence: A Multicenter, Retrospective Study of 100 Patients.

STUDY OBJECTIVE: To investigate the characteristics of children with ovarian cysts and evaluate treatment strategies. DESIGN: Retrospective study. SETTING: Eight pediatric endocrinology clinics, Turkey. PARTICIPANTS: A total of 100 children and adolescents with ovarian cysts. INTERVENTIONS: Patient data collected via retrospective chart review. Patients were stratified according to age into 4 groups (newborns, 1-12 months, 1-8 years, and 8-18 years). MAIN OUTCOME MEASURES: Special emphasis was given to torsion and tumor cases, concomitant diseases, treatment modalities, and problems during follow-up. RESULTS: Most newborns and infants were asymptomatic with the cysts being discovered incidentally; in girls ages 1-8, symptoms were common, including breast budding (47.1%, 16 of 34) and vaginal bleeding (29.4%, 10 of 34). Girls older than 8 years mostly presented with abdominal pain (31.6%, 12 of 38) and menstrual irregularity (21.1%, 8 of 38). Most of our patients were diagnosed with a simple ovarian cyst, but 9 patients were found to have ovarian tumors. Ovarian torsion was detected in 7 patients; 5 with large and 2 with small cysts (<20 mm). Two patients had central precocious puberty (CPP) at presentation and 5 patients developed CPP during follow-up. The surgical intervention rate was high (38%, 38 of 100), but was associated with earlier treatment year, and this association remained significant after adjusting for confounders (P = .035). CONCLUSION: Most girls have simple cysts, which have a favorable prognosis without intervention; however, there might be coexisting pathologies or complications such as tumors, torsion, and CPP; hence these patients should be evaluated accordingly and treated with a multidisciplinary approach.

Worster-Drought syndrome (congenital bilateral perisylvian syndrome) with posterior pituitary ectopia, pituitary hypoplasia, empty sella and panhypopituitarism: a patient report.

Worster-Drought syndrome (WDS) (congenital bilateral perisylvian syndrome, congenital pseudobulbar paresia) is characterized by neuronal migration defect, pseudobulbar paralysis, epilepsy, neuromotor retardation and perisylvian dysplasia. We report a patient with WDS associated with posterior pituitary ectopia, pituitary hypoplasia, partial empty sella and panhypopituitarism, not previously reported in the literature. The 16.4 year-old female patient had severe growth retardation with height SDS -4.5, delayed puberty, microcephaly, pes equinovarus deformity, developmental delay, speech disorder and epilepsy. Laboratory findings, which revealed abnormal electroencephalography and bilateral perisylvian cortical dysplasia on cranial magnetic resonance imaging (MRI) were consistent with WDS. Endocrinological evaluation revealed secondary hypothyroidism and combined deficiency of adrenocorticotropin, gonadotropin and growth hormone (GH). Sella MRI showed congenital empty sella, anterior pituitary hypoplasia, ectopic neurohypophysis, and stalk agenesis. Appropriate replacement therapy was started. GH treatment resulted in a final height of 150.3 cm, appropriate for her target height. This is the first reported patient with WDS associated with congenital structural hypothalamic-pituitary abnormalities, including empty sella, pituitary hypoplasia, posterior pituitary ectopia, stalk agenesis and panhypopituitarism. GH has been successful in the treatment of her short stature.

Evaluation of glucose intolerance in adolescents relative to adults with type 2 diabetes mellitus.

AIM: There is an increasing trend in the prevalence of type 2 diabetes mellitus (DM2) in childhood and adolescence, while positive family history of DM2 and obesity are the most important risk factors. To study the influence of family history and obesity on glucose intolerance in our country was the aim of this study. STUDY DESIGN AND METHODS: A total of 105 children and adolescents aged 10-18 years (mean 13.3 +/- 2.5 years) were included in the study. All children and adolescents were divided into three groups according to positive family history of DM2 and obesity, and an oral glucose tolerance test (OGTT) was performed for all. Prediabetes was defined as impaired glucose tolerance (IGT) and/or impaired fasting glucose (IFG). Insulin secretion and insulin resistance were estimated using the insulinogenic index; and the homeostatic model assessment for insulin resistance (HOMA-IR) and Matsuda index, respectively. RESULTS: The prevalence of prediabetes was 15.2% in the whole group, while it was 25.5% in obese children who also had a positive family history of DM2. The frequency of hyperinsulinism was 57.1% in all groups. Prediabetic children had significant insulin resistance (HOMA-IR 11.5 +/- 7.1 and 4.1 +/- 6.4, respectively, p = 0.034). CONCLUSIONS: Obesity and glucose intolerance are also a problem in developing countries. The risk of prediabetes in children is highest in obese children who also have a positive family history of DM2. There is a need for a lifelong preventive program starting in childhood to avoid DM2 and decrease cardiovascular risk factors