|1.||Aromatase Inhibitors to Augment Height: Continued Caution and Study Required|
Mitchell E. Geffner
doi: 10.4274/jcrpe.v1i6.256 Pages 256 - 261
Aromatase inhibitors (AIs) are a class of drugs that prevent conversion of androgens to estrogens, and that are approved in the United States as adjunctive treatment of estrogen receptor-positive breast cancer. Because ultimate fusion of the growth plates is estrogen-dependent in both boys and girls, AI administration may help to slow down epiphysial maturation and allow for greater height potential. Research trials in children with short stature have predominantly been done in Finland and Florida. Despite the apparent efficacy described by these groups, only ~110 children worldwide have been treated with AIs in research protocols (and usually concomitant with other growth-promoting agents) as of the end of 2008 (and none to final height). That said, many children are being treated with AIs in the United States outside of research protocols. Furthermore, little is known about the short- and long-term safety of AIs in children. Thus, it is imperative that there be well-designed, long-term studies of efficacy and safety of AI use in pediatric populations.
|2.||The Effect of Vitamin D Treatment on Serum Adiponectin Levels in Children with Vitamin D Deficiency Rickets|
Behzat Özkan, Hakan Döneray, Halil Keskin
doi: 10.4274/jcrpe.v1i6.262 Pages 262 - 265
Objective: Adiponectin and its receptors are known to be expressed in osteoblasts and may have important functions in normal bone cells. The aim of this study was to investigate the effect of vitamin D therapy on serum adiponectin levels in children with vitamin D deficiency rickets (VDDR).
Methods: 21 patients with VDDR were included in the study. Patients were treated with 300,000 U D3 (IM) and calcium lactate (50mg/kg/ day, PO, for 10 days). Anthropometric parameters and serum biochemical markers including calcium (Ca), phosphorus (P), alkaline phosphatase (ALP), intact parathormone (iPTH), 25-hydroxyvitamin D (25(OH)D), and adiponectin levels were measured before and after one month of therapy.
Results: Weight and length, but not BMI, increased significantly after treatment. Serum 25(OH)D level increased significantly after treatment, while serum adiponectin level decreased (4.21±1.84 vs 52.73±17.63 ng/ml, p<0.000; 150.1±66.14 vs 84.29±9.06 mg/ml, p<0.000, respectively). No significant correlations were found between serum adiponectin and 25(OH)D levels before and after treatment or between delta adiponectin concentrations and delta 25(OH)D levels.
Conclusion: Serum adiponectin levels are increased in patients with VDDR, a finding which is probably related to increased osteoblastic activity.
|3.||Vitamin D Deficiency in Turkish Mothers and Their Neonates and in Women of Reproductive Age|
Ayça Törel Ergür, Merih Berberoğlu, Begüm Atasay, Zeynep Şıklar, Pelin Bilir, Saadet Arsan, Feride Söylemez, Gönül Öcal
doi: 10.4274/jcrpe.v1i6.266 Pages 266 - 269
Objective: Materno-fetal vitamin D deficiency (VDD) may occur in the early neonatal period. We aimed to evaluate the vitamin D (vitD) status and risk factors for VDD in healthy newborns and their mothers, and also in fertile women.
Methods: Serum 25 hydroxyvitamin D3 (25(OH)D), calcium (Ca), phosphorus (P) and alkaline phosphatase (ALP) levels were measured in 70 mothers (study group) and their newborns, and in umbilical cord samples. 104 nonpregnant fertile women comprised the control group. Demographic factors such as education and clothing habits of the mother, number of pregnancies and month of delivery were recorded. A serum 25(OH)D level below 11 ng/ml was accepted as severe, 11-25 ng/ml as moderate VDD, and a value over 25ng/ml as normal.
Results: Severe VDD was found in 27% of the mothers, and moderate deficiency in 54.3%. Severe VDD was detected in 64.3% of the neonates, and moderate deficiency in 32.9%. Only 18.6% of the mothers and 2.9 % of the neonates had normal vitD levels. In thecontrol group, severe VDD was observed in 26.9%, and moderatedeficiency in 45.2 %. Only 27.8 % of the controls had normal vitD levels. In the control group, the 25(OH)D levels of the women dressed in modern clothes were significantly higher than those of the women wearing traditional clothes. This difference was not observed in the study group because 75% of these 70 mothers wore modern clothes. Mothers giving birth during the summer months and their neonates had significantly higher serum 25(OH)D levels than those of the mothers giving birth during the winter months and their neonates.
Conclusion: The study has shown that in Turkey VDD is an important problem in women of reproductive age, in mothers and their neonates. The 25(OH)D levels obtained from the cord may serve as a guide in the determination of the high risk groups.
|4.||Diagnostic Use of Skeletal Survey in Suspected Skeletal Dysplasia|
Amith Kumar Iynapillai Veeramani, Paul Higgins, Sandra Butler, Malcolm Donaldson, Elizabeth Dougan, Roderick Duncan, Victoria Murday, Syed Fasial Ahmed
doi: 10.4274/jcrpe.v1i6.270 Pages 270 - 274
Objective: To review the practice of skeletal surveys in cases of suspected skeletal dysplasia.
Methods: Retrospective review of records of patients with suspected skeletal dysplasia between December 1997 and December 2005.
Results: A diagnosis of a specific skeletal dysplasia was reached in 155 out of a total of 285 suspected cases (54%). In 260 (91%), a record of radiological examination was available and out of these cases, 91 (35%) had a full skeletal survey. A diagnosis was reached in 79% of cases that had a full skeletal survey and in 44% of cases that had a limited survey. A possible skeletal dysplasia was excluded in 44 out of 260 (17%) cases. In 79 out of 260 (30%) cases, skeletal abnormalities were present but a clear diagnosis could not be reached. Over the period of study, there was no clear change in the practice of performing x-rays and the rate of reaching a diagnosis.
Conclusion: A clear diagnosis of skeletal dysplasia is not possible in a third of cases and there is a need for greater access to multidisciplinary input.
|5.||Iodine Overload and Severe Hypothyroidism in Two Neonates|
Selim Kurtoğlu, Leyla Akın, Mustafa Ali Akın, Dilek Çoban
doi: 10.4274/jcrpe.v1i6.275 Pages 275 - 277
Iodine overload frequently leads to transient hyperthyrotropinemia or hypothyroidism, and rarely to hyperthyroidism in neonates. Iodine exposure can be prenatal, perinatal or postnatal. Herein we report two newborn infants who developed severe hypothyroidism due to iodine overload. The overloading was caused by excessive use of an iodinated antiseptic for umbilical care in the first case, and as a result of maternal exposure and through breast milk with a high iodine level in the second case. Presenting the two cases, we wanted to draw attention to these preventable causes of hypothyroidism in infants.
|6.||Metabolic Syndrome Features Presenting in Early Childhood in Alström Syndrome: A Case Report|
Özgür Pirgon, Mehmet Emre Atabek, İlhan Asya Tanju
doi: 10.4274/jcrpe.v1i6.278 Pages 278 - 280
Alström syndrome is a rare autosomal recessive disorder characterized by retinal degeneration, sensorineural hearing loss, early-onset obesity, and non-insulin-dependent diabetes mellitus. Affected individuals have normal birth weight, but growth deceleration starts at about 8-10 years of age. In patients with the disorder linked to chromosome 2, the increase in body mass index is very high in childhood and continues high thereafter. In this paper, we report a patient who had the proposed diagnostic criteria for Alström syndrome associated with metabolic syndrome starting at age 7, a relatively early age.
|7.||A Rare Cause of Precocious Puberty: Hepatoblastoma|
Erdal Eren, Metin Demirkaya, Esra D. Papatya Çakır, Betül Sevinir, Halil Sağlam, Ömer Tarım
doi: 10.4274/jcrpe.v1i6.281 Pages 281 - 283
Hepatoblastoma, an embryonal tumor, is one of the most common primary liver tumors in childhood. It secretes human chorionic gonadotropin (hCG), which can cause precocious puberty (PP). Herein, we present a case with PP who had enlarged penile size noticed during a diagnosis of hepatoblastoma. Laboratory examination revealed increased testosterone, alpha-fetoprotein (AFP), and hCG levels. Serum follicle-stimulating hormone (FSH) and luteinizing hormone (LH) levels were within prepubertal ranges. The diagnosis of hepatoblastoma was made by liver biopsy. Chemotherapy was administered, and the patient was referred to surgery. Ten months later, testis volumes were below 4 ml bilaterally, and penile length was 5.5 cm. Serum testosterone, AFP, and hCG levels decreased. Resection of the tumor and chemotherapy are essential for the treatment of hepatoblastoma and they can eliminate the symptoms of PP.
|8.||2009 Referee Index|
|9.||2009 Author Index|
|10.||2009 Subject Index|