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

OBJECTIVE: 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. DESIGN: Hospital-based, noninterventional, prospective, cross-sectional study. METHODS: A total of 162 pregnant women in their second trimester were examined regarding iodized salt use, UIC, presence or absence of goitre and thyroid function. Goitre status was determined by palpation. UIC was measured using colorimetric method based on Sandell-Kolthoff reaction. Thyroid hormones and TSH were measured by chemiluminescence immunoassays. RESULTS: While the proportion of iodized salt use was 80·2%, UIC was below 150 µg/l in 72·8% of the women. The median UIC was 80·5 (8·9-340·3) µg/l, indicating insufficient iodine intake. Total goitre rate was 15·4%. Preferential T3 secretion reflected by elevated molar ratios of FT3/FT4 was present in 89·5% of the women. 12·4% had subclinical hypothyroidism or isolated hypothyroxinaemia based on serum TSH and FT4 levels. CONCLUSIONS: Our study shows that iodine deficiency is a serious problem among pregnant women in Ankara. These data confirm that iodine nutritional status among SAC does not reflect the iodine supply for pregnant women. We propose that nationwide surveillance studies should urgently be performed to directly assess and monitor the iodine status of pregnant women. We also consider that pregnant women in Turkey should be supplemented by iodine-containing preparations in addition to iodized salt.

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.

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.

Normative data for penile length in Turkish newborns.

BACKGROUND: Local normative data for penile size will aid physicians in clinical practice. There are no studies on stretched penile length (SPL) in newborn Turkish boys. OBJECTIVE: To establish normative data for SPL of newborn Turkish boys and compare these with data from different countries. METHODS: 514 newborn Turkish boys, who were not small for gestational age (SGA) or premature, were included in this cross-sectional study. SPLs of the newborns were measured by the same investigator. The correlations between SPL and gestational age, weight, length, and head circumference were evaluated. RESULTS: The 3(rd) percentile value for SPL was found as 3.00 cm in these Turkish newborns. Positive correlations were shown between SPL and height (r=0.240, p<0.001), weight (r=0.251, p<0.001), and head circumference (r=0.235, p<0.001). Multiple linear regression analysis showed that SPL positively correlated with height and head circumference (p=0.021 and p= 0.042, respectively). CONCLUSIONS: This is the largest study on SPL in newborns from our country. This normative data can be used in clinical practice for defining micropenis.

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 patient's 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.

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. METHODS: Twenty five patients with a median age of 12 days (6 days-6 months) at diagnosis and followed for a median time of 7 months (1-60 months) were enrolled in the study. Thyroid function tests, thyroid scintigraphy, ultrasonography and urine iodine measurements of the patients and mothers were performed. RESULTS AND CONCLUSION: Congenital hypothyroidism was diagnosed within postnatal day 13, between days 13 and 30, and after 30 days of age in 68%, 20% and 12% of the patients, respectively. At the time of diagnosis mean serum thyroid-stimulating hormone and total T4 were 85.3 +/- 27.6 mIU/L and 3.9 +/- 2.8 microg/dL, respectively. Incidence of iodine deficiency was 36% in the patients (median, 110 microg/L) and 88% in the mothers (median, 40 microg/L). Thyroid scintigraphy and ultrasound were normal in all of the patients with iodine deficiency. At scintigraphic evaluation, thyroid gland was not visualized in 28% of patients; in the patients whose thyroid glands were not visualized scintigraphically thyroid ultrasonography indicated agenesis in 57%, and hypoplasia in 43%. In all the patients with thyroid agenesis or hypoplasia iodine levels were normal. In 36% of the patients imaging studies of thyroid gland and urine iodine measurements were normal. Despite salt iodization program, incidence of iodine deficiency is still high in patients with congenital hypothyroidism and mothers. National measures are urgently required for correction of iodine deficiency in Turkey.

Paternal X could relate to arithmetic function; study of cognitive function and parental origin of X chromosome in Turner syndrome.

BACKGROUND: 45,X Turner syndrome (TS) female subjects have visuospatial skill and social cognition deficits that may arise from X-linked imprinting. The aim of the present study was to compare phenotypic characteristics and neurocognitive pattern of 12 monosomic TS girls, according to X-linked imprinting. METHODS: Microsatellite markers were used to determine the parental origin of the missing chromosome X. Wechsler Intelligence Scale for Children-Revised (WISC-R) was administered as measures of general intellectual functioning. The results were compared in TS patients with maternally derived X chromosome (Xm) and paternally derived X chromosome (Xp). RESULTS: Six out of 12 patients (50%) had Xm, and the other six (50%) had Xp chromosome. There was no difference in the total, verbal and performance IQ score between the TS subgroups with Xm and Xp. When the WISC-R subtest score patterns were compared, the mean arithmetic scores were significantly poorer in the Xm TS than in the Xp TS. CONCLUSION: In monosomic TS cases, paternal imprinting may predict arithmetic ability, on the other hand, reductionist consideration defined by genetic imprinting is not sufficient to confirm this. Further studies should be undertaken to clarify this situation.

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.

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.