Alev Oguz Kutlu

Sertoli Cell Tumor Causing Prepubertal Gynecomastia in a Boy with Peutz-Jeghers Syndrome: The Outcome of 1-Year Treatment with the Aromatase Inhibitor Testolactone

Peutz-Jeghers syndrome (PJS) is a rare disorder characterized by benign intestinal hamartomatous polyps and mucocutaneous pigmentation, and with an increased risk for intestinal and extra-intestinal neoplasms. Sertoli cell tumors in boys with PJS have been increasingly recognized as a cause of prepubertal gynecomastia. However, an association between nephrocalcinosis and PJS has not been reported before. We report on a 7.25-year-old boy with PJS, bilateral gynecomastia, Sertoli cell tumor and nephrocalcinosis, and present the outcome of 1-year treatment with the aromatase inhibitor testolactone. The patient presented with bilateral breast and testis enlargement, and mucocutaneous pigmentation. Testicular ultrasound revealed parenchymal multiple microcalcifications. Histopathological examination was consistent with Sertoli cell tumors. Nephrocalcinosis due to idiopathic renal hypercalciuria was also detected. The aromatase inhibitor testolactone was begun in an attempt to prevent acceleration in skeletal maturation. One-year treatment with testolactone reduced the breast base diameter from 7 to 3 cm, and bone age advanced 1.2 years during this period. Our case demonstrates that waiting for the effect of aromatase inhibitors on gynecomastia before making a decision for mastectomy may be a reasonable option. We also consider that the association between PJS and nephrocalcinosis may be a coincidence.

Iodine deficiency in pregnant women in the apparently iodine‐sufficient capital city of Turkey

Previous studies of the current iodine status in Turkey have yielded contradictory results. Although urinary iodine concentration (UIC) in school age children (SAC) suggests sufficient iodine status, studies on neonatal thyroid‐stimulating hormone (TSH) indicate that iodine deficiency is a continuing problem. We aimed to assess the iodine nutritional status of pregnant women living in Ankara, an area that has appeared to be iodine sufficient in earlier studies.

External Genital Proportions in Prepubertal Girls: A Morphometric Reference for Female Genitoplasty

PURPOSE An understanding of normal genital anatomy is essential for a successful surgical approach and outcome in feminizing genitoplasty. We sought to establish genital standards in female children through external genital measurements taken from the end of the neonatal period until the beginning of adolescence. MATERIALS AND METHODS This prospective study included 205 females who were anesthetized for surgery for various diagnoses between January 2007 and March 2008. Patient age ranged from 1 month to 10 years. Patients were divided into 4 age groups-1 to 12, 13 to 24, 25 to 60 and 61 to 120 months. Information on patient age, height, weight and, for patients younger than 1 year, head circumference was retrieved from patient charts. Measurements of clitoris length, clitoris width, labia majora length, left and right labia minora length and width, and perineal distance were recorded. RESULTS Specific equations were generated using these values to estimate the expected external genital structure dimensions in girls. Length of labia majora vs age, length of labia majora vs body weight, perineal distance vs body weight, clitoral width vs body weight and clitoral length vs age reference percentile curves were prepared. CONCLUSIONS The equations and percentile curves generated can be used as a guide in prospective feminizing genitoplasty. Furthermore, patients and their families can be informed regarding the variability of external genitalia dimensions. This information should ensure a healthier appreciation of the postoperative genitalia by patients and their families.

Rhabdomyolysis without detectable myoglobulinuria due to severe hypophosphatemia in diabetic ketoacidosis.

Clinical signs of hypophosphatemia, even when severe, are rare in diabetic ketoacidosis despite their high frequency in this condition. This article presents a patient with rhabdomyolysis due to severe hypophosphatemia, where the level of serum phosphorus was observed to be as low as 0.42 mg/dL on the 16th hour of ketoacidosis treatment. The patient developed acute tubular necrosis due to rhabdomyolysis, but there was no blood reaction in the urine, and the creatine kinase increased to 1200 U/L. The patient was treated without dialysis and was cured after a polyuria period of 2 months after the oliguric period.

Single dose povidone-iodine on thyroid functions and urinary iodine excretion

The effect of single dose povidone-iodine on serum thyrotropin and thyroxine levels and urinary iodine excretion in 30 preterm, 40 full-term newborns and 50 infants at Dr. Sami Ulus Children’s Hospital was studied. There was no significant change of thyroid function in any of the groups (p>0.05). Urinary iodine excretion in preterm and full-term groups elevated significantly (p<0.05). The authors conclude that patients who receive single dose povidone-iodine for skin disinfection are not at risk for thyroid disorders.

Incidence of iodine deficiency in Turkish patients with congenital hypothyroidism.

Background: Turkey is located in an area of mild to moderate iodine deficiency. The aim of the present study was to investigate the incidence of iodine deficiency in patients with congenital hypothyroidism.

Yardımcı üreme teknikleri ile doğan beş olguda görülen erken puberte

Yardimci ureme teknikleri ile dogan cocuklar intrauterin donemde yuksek doz hormona maruz kalmaktadir Ozellikle erken dogum oncesi gelisim sirasinda yuksek doz hormona maruz kalan cocuklarin ileri donem saglik durumlari tam olarak bilinmemektedir Ancak bu cocuklarda dusuk dogum agirligi erken dogum yuksek kan basinci ve kan sekeri erken ergenlik riski olabilecegi tartisilmaktadir Burada yardimci ureme teknikleri kullanilarak dusuk dogum agirligi ile dogan bes erken ergenlik tanisi konulmus olgu dogum oncesi hormon maruziyeti ve ergenlik gelisimi arasindaki iliskiye dikkat cekmek bakimindan sunuldu Turk Ped Ars 2012; 47: 213 5

Postnatal testicular regression mircopenis and microcephaly: Conformation of a new syndrome?†

Testicular regression may develop at various phases of the intrauterine period and the clinical findings vary depending on the timing of the intrauterine phase. On the other hand, postnatal regression of the testicles is rare and few cases have been reported in the literature. Our patient presented with undescended testicles, micropenis, flat scrotum and microcephaly. The patients levels of basal luteinizing hormone (LH) were low/normal and follicle stimulating hormone (FSH) was high. No response was obtained in the human chorionic gonadotropin (HCG) stimulation test. The levels of inhibin B and anti Mullerian hormone (AMH) were found to be low. Penile growth response to intramuscular testosterone injections was 2.5 cm. Testicles were visualized bilaterally in inguinal canal by ultrasound examination initially but by 2 years of age no testicular tissue was observed during inguinal exploration. In conclusion we recognized postnatal testicular regression in our patient that had started in the intrauterine period and persisted into infancy. The genital system anomalies, microcephaly and motor retardation in our patient confirm the hypothesis of Parisi et al. of a novel condition of postnatal regression and micropenis.