Comprehensive Insights Into Pediatric Craniopharyngioma: Endocrine and Metabolic Profiles, Treatment Challenges, and Long-term Outcomes from a Multicenter Study

Objective: Craniopharyngiomas (CPG) have complex treatment challenges due to their proximity to vital structures, surgical and radiotherapeutic complexities, and the tendency for recurrence. The aim of this study was to identify the prevalence of endocrine and metabolic comorbidities observed during initial diagnosis and long-term follow-up in a nationwide cohort of pediatric CPG patients. A further aim was to highlight the difficulties associated with CPG management. Methods: Sixteen centers entered CPG patients into the ÇEDD NET data system. The clinical and laboratory characteristics at presentation, administered treatments, accompanying endocrine, metabolic, and other system involvements, and the patient's follow-up features were evaluated. Results: Of the 152 evaluated patients, 64 (42.1%) were female. At presentation, the mean age was 9.1±3.67, ranging from 1.46 to 16.92, years. The most common complaints at presentation were headache (68.4%), vision problems (42%), short stature (15%), and nausea and vomiting (7%). The surgical procedures were gross total resection (GTR) in 97 (63.8%) and subtotal resection in 55 (36.2%). Radiotherapy (RT) was initiated in 11.8% of the patients. Histopathological examination reported 92% were adamantinamatous type and 8% were papillary type. Postoperatively, hormone abnormalities consisted of thyroid-stimulating hormone (92.1%), adrenocorticotropic hormone (81%), antidiuretic hormone (79%), growth hormone (65.1%), and gonadotropin (43.4%) deficiencies. Recombinant growth hormone treatment (rhGH) was initiated in 27 (17.8%). The study showed hesitancy among physicians regarding rhGH. The median survival without relapse was 2.2 years. Median (range) time of relapse was 1.82 (0.13-10.35) years. Relapse was related to longer followups and reduced GTR rates. The median follow-up time was 3.13 years. Among the last follow-up visits, the prevalence of obesity was 38%, but of these, 46.5% were already obese at diagnosis. However, 20% who were not obese at baseline became obese on follow-up. Permanent visual impairment was observed in 26 (17.1%), neurological deficits in 13 (8.5%) and diabetes mellitus in 5 (3.3%) patients. Conclusion: Recurrence was predominantly due to incomplete resection and the low rate of postoperative RT. Challenges emerged for multidisciplinary regular follow ups. It is suggested that early interventions, such as dietary restrictions and increased exercise to prevent obesity, be implemented.

Serum Irisin Levels in Central Precocious Puberty and Its Variants.

AIM: The exact mechanisms that trigger the onset of puberty are not well known. Adipomyokines are postulated to stimulate the central neural network. In the present study, we investigated irisin levels in girls with central precocious puberty (CPP), slowly progressing precocious puberty (SPPP), or premature thelarche (PT); we also studied prepubertal girls and to determine if this adipomyokine could be used as a marker in this context. METHODS: A total of 94 girls including 33 with CPP, 31 with precocious puberty (PP) variants (SPPP or PT), and 30 healthy controls were enrolled to the study. The mean irisin levels were compared between groups. The bivariate correlations of irisin levels with clinical and laboratory parameters were assessed. Multivariate linear regression analysis was performed to determine independent predictive factors of irisin levels. RESULTS: Irisin levels were higher in the CPP group compared with the other groups (CPP group: 723.25 ±â€…62.35 ng/mL; PP variants group: 529.60 ±â€…39.66 ng/mL; and control group: 325.03 ±â€…27.53 ng/mL) (P < 0.001). Irisin levels were positively correlated with body mass index standard deviation scores (BMI-SDS), height-SDS, weight-SDS, bone age, uterus long axis, ovary size, baseline FSH and LH, and peak LH levels. Multivariate linear regression analysis revealed that irisin levels had the strongest correlation with peak LH. The other independent predictive factor of irisin levels was BMI-SDS. CONCLUSIONS: The mean irisin levels were higher in patients with CPP compared with other groups. The results of this study imply that increased irisin levels may be used as a marker of CPP provided that these findings are confirmed in larger prospective studies.

Omentin-1 level in adolescents with polycystic ovarian syndrome.

BACKGROUND: Adipokines have been suggested to play an important role in the pathogenesis of polycystic ovarian syndrome (PCOS). Omentin is an adipokine secreted essentially by visceral adipose tissue with an insulin-sensitizing effect. Insulin resistance (IR) is a common feature of PCOS, therefore the aim of this study was to investigate omentin-1 level in adolescent girls with PCOS and its relationship with IR and androgens. METHOD: A total of 41 obese girls with PCOS, and 30 age- and body mass index (BMI)-matched obese girls without PCOS were enrolled in the study. The demographic, clinic and laboratory characteristics of the groups were compared. Additionally, bivariate correlation analysis of omentin-1 with BMI standard deviation score (BMI-SDS), insulin, glucose, homeostatic model assessment of IR (HOMA-IR), total and free testosterone was performed. RESULTS: In the PCOS group HOMA-IR, free and total testosterone were higher than in the control group. Omentin-1 was lower in the PCOS group compared with the controls (55.01 ± 7.99 ng/mL vs 59.10 ± 7.02 ng/mL, respectively; P = 0.027). Omentin-1 was inversely correlated with free testosterone (r = -0.527, P = 0.030) and BMI-SDS (r = -0.241, P = 0.046) but it was not correlated with total testosterone, HOMA-IR, glucose, insulin or serum lipids. CONCLUSION: Omentin-1 was lower in obese girls with PCOS and hyperandrogenism was associated with this condition.

The relation of urinary bisphenol A with kisspeptin in girls diagnosed with central precocious puberty and premature thelarche.

BACKGROUND: Bisphenol A (BPA) is known as an endocrine disruptor and it is supposed to have a role on the development of central precocious puberty (CPP). Kisspeptin, a hypothalamic peptide, is a neuromodulator of gonadotropin releasing hormone and it has an important role on regulation of the onset of puberty. The BPA levels in girls with CPP and premature thelarche (PT) and its relation with kisspeptin levels were investigated. METHODS: Twenty-eight girls with CPP, 28 girls with PT and 22 prepubertal girls as a control group were enrolled to the study. Urinary BPA and serum kisspeptin levels were compared in the groups. Bivariate correlations were performed to evaluate the relations of BPA with kisspeptin and estradiol. RESULTS: There was no statistical difference between groups regarding BPA levels. Serum kisspeptin levels were higher in CPP group than controls [306.56 (interquartile range (IQR), 175.63-504.66) vs. 157.62 (IQR, 55.61-285.00) p: 0.008]. There were no correlations between BPA and kisspeptin levels (r: 0.088, p: 0.391) and between BPA and estradiol (r: -0.171, p: 0.144). CONCLUSIONS: The BPA levels did not differentiate between groups and it seems that the exposed amount of BPA in daily life did not affect kisspeptin levels in girls with CPP and PT.

Response to Disodium Etidronate Treatment in Three Siblings with Pulmonary Alveolar Microlithiasis.

Pulmonary alveolar microlithiasis (PAM) is a rare chronic genetic lung disease in childhood with no proven therapy. It is characterized by the deposition of calcium phosphate microliths within the alveolar air spaces. The effect of disodium etidronate (DE) treatment on PAM is controversial. We report 3 siblings (an 11-year-old boy and 4-year-old twin girls) with PAM diagnosed by chest X-ray, thoracic high-resolution computed tomography, technetium-99m bone scan and bronchoalveolar lavage fluid findings. After the administration of DE (200 mg/day) for a 1-year period, 2 siblings showed radiological improvement, while 1 sibling did not. No drug side effects were observed within the treatment period.

Determination of early urinary renal injury markers in obese children.

BACKGROUND: Obesity is an important health issue, the prevalence of which is increasing in childhood. The aim of this study was to examine urinary renal injury markers in order to determine the renal effect of obesity and its comorbidities in a pediatric population. METHODS: Eighty-four obese children and 64 healthy control subjects were enrolled in the study. We checked their urine using N-acetyl-beta-D-glucosaminidase (NAG), neutrophil gelatinase-associated lipocalin (NGAL), kidney injury molecule-1 (KIM-1), and microalbumin as renal injury markers. Associations of renal damage markers with hypertension, an impaired glucose tolerance test, and insulin resistance were assessed. RESULTS: Obese individuals had higher urinary NAG and KIM-1 values compared to those of healthy controls (p = 0.027, p = 0.026). There was no difference in urinary NGAL between obese and lean subjects (p = 0.885). Urinary renal injury markers were not statistically different in the obese group when checked for impaired glucose tolerance, insulin resistance, and hypertension (p > 0.05). CONCLUSIONS: This study shows that urinary NAG and KIM-1 could be used as a screening method for detection of early renal damage in obese children.

Congenital agenesis of scrotum and labia majora in siblings.

Congenital agenesis of the scrotum and labia majora is very exceptional. To date, only 6 cases of scrotal agenesis have been reported. To our knowledge, the anomalies of the labioscrotal folds in siblings have not yet been reported. We report the complete agenesis of the scrotum and labia majora within the 3 members of the same family. Additionally, successful reconstruction of the neoscrotum was performed for the first time for congenital scrotal agenesis.

The clinical and biochemical presentation of vitamin D deficiency and insufficiency in children and adolescents.

AIM: To evaluate the clinical and biochemical findings of the children and adolescents with vitamin D deficiency and insufficiency in order to determine the clinical and biochemical presentation differences between age groups. METHODS: This retrospective study included a review of medical reports of 543 patients (aged between 1-17 years) who were referred to our hospital between October 2011 and May 2012 with symptoms related to vitamin D deficiency or insufficiency. The patients were divided into four groups by age: 1-3 years (Group 1), 4-6 years (Group 2), 7-11 years (Group 3) and 12-17 years (Group 4). Patients diagnosed with vitamin D deficiency or insufficiency were evaluated as to their clinical and biochemical findings. RESULTS: Gender distribution were not statistically different between the four groups. The mean ages of Groups 1-4 were 1.9±0.7, 5.1±0.9, 8.9±1.3, 13.1±1.1, respectively. Major complaints on admission were muscle weakness (91%), low weight gain (failure to thrive) (89%), head deformity (frontal bossing) (35.6%), bone deformity (enlargement of wrist and ankles) (29.7%) for Group 1. Muscle weakness (76%) and low weight gain (failure to thrive) (68%) for Group 2. Leg and chest pain were the major symptoms in Group 3 (57% and 28%, respectively) and in Group 4 (26% and 55%, respectively) as well as high rates of obesity (31% and 63%). The biochemical findings of vitamin D deficiency mostly appeared in the first group who developed vitamin D deficiency due to the lack of vitamin D supplementation. However, in older children, the majority of the patients had low 25 hydroxyvitamin D (25 OHD) values without evidence of biochemical findings of osteomalacia. CONCLUSION: Depending on the degree of deficiency and insufficiency, and the age of the patients, the clinical and biochemical findings varied widely. Children under the age of 3 who either never received vitamin D supplementation or who had been receiving supplementation that was stopped too early were at a greater risk for developing clinically and biochemically proved vitamin D deficiency. In older children, low vitamin D levels mostly resulted in subtle complaints without abnormal biochemical findings.

Effects of individual factors on adolescent obesity: study in Turkey.

BACKGROUND: The aim of the present study was to investigate the effects of sociodemographic and lifestyle variables, eating habits, adolescent weight perception and weight changes on obesity in Turkish adolescents. METHODS: A total of 87 normal weight and 78 obese participants were included in the study. They were selected from adolescents who had no other health problems beside obesity. Underweight and overweight adolescents were excluded. Estimation of prevalence of overweight and obesity were based on cut-off points of the International Obesity Task Force (excess of the 85th and 95th percentiles, respectively). The prevalence of underweight was defined as the percentage of adolescents below the fifth percentiles of the US adolescents' age- and gender-specific body mass index. A structured questionnaire that included sociodemographic and lifestyle variables, eating habits, weight questions about adolescents' weight perception and weight changes was administered to the participants. RESULTS: In the obese group, the level of the mothers' and fathers' education was lower than in the normal group (P = 0.006; P = 0.001, respectively). Obese adolescents had fewer obese people in their family (P = 0.001). There were statistically significant correlations between groups for fathers' occupation, joining in cultural activities, physical exercise, feeling about body shape and food preference (P = 0.014; P = 0.001; P = 0.003; P = 0.001; P = 0.001, respectively). Increase in weight was larger in the obese group (P= 0.001) and those who wanted to loose weight but failed (P = 0.001). CONCLUSIONS: There are associations between obesity and level of mothers' and fathers' education, number of obese people in family, fathers' occupation, joining in cultural activities, physical exercise and food preference in Turkish adolescents.

Hair selenium status in children with leukemia and lymphoma.

Selenium (Se) is a trace element contributing to the structure of antioxidant system that saves cells from reactive oxygen species. Low serum Se levels have been reported in pediatric and adult patients with cancers. On the other hand, hair Se levels, predicting the long-term body Se status, have been reported in only adult patients with cancer. The aim of the study was to investigate the hair Se status in children with newly diagnosed lymphoid malignancies and the relation between malnutrition and Se deficiency. Thirty patients with leukemia (n=17) and lymphoma (n=13), and 25 healthy controls were enrolled to the study. Se was determined with atomic absorption spectrophotometrical method. Hair Se levels of the patients were significantly lower than those of control group [666.96+/-341.46 ng/g vs. 1019.22+/-371.83 ng/g (P<0.001)]. Children with lymphoma had lower Se than the children with acute lymphoblastic leukemia but not statistically significant [547.03+/-283.67 ng/g vs. 758.67+/-361.05 ng/g (P>0.05)]. Malnourished patients (11/30) had lower hair Se levels (483.51+/-235.55 ng/g) than those of the controls (P=0.036), whereas the Se levels of the patients who had no malnutrition (773.17+/-352.92 ng/g) were also lower than those of the controls but not statistically significant (P=0.053). There was no correlation between age, sex, and the hair Se levels. In this study, we found that hair Se levels of the children with leukemia and lymphoma, especially those of malnourished patients, were lower than those of controls. Additional studies are needed to determinate whether low levels of hair Se may play a role in carcinogenesis.