Ileus and Intra-Abdominal Hypertension due to Phosphate- Containing Enema.

The most well known complications of fleet enema solution are cardiac insufficiency, renal failure, water-electrolyte imbalance, and ileus. A 7-y-old girl with phenylketonuria and long-term constipation was admitted to the emergency department with symptoms of seizure, vomiting and abdominal distention. Laboratory results revealed hypocalcemia and hyperphosphatemia. ECG findings showed normal sinus rhythm and prolonged QT interval. At the follow-up, the patient's abdominal distention was markedly increased. She was evaluated for a surgical pathology and, this was considered unlikely. Intra-abdominal pressure (IAP) was 19.5 mmHg. Gastric and colonic decompression, intravenous 10 % calcium gluconate were applied. After 2 d of treatment, the patient's condition became stable, and serum calcium and phosporus normalized to 8.8 mg/dl and 4.0 mg/dl, respectively. Abdominal distention regressed and the last IAP measurement was 3.5 mmHg. Thus, IAP measurements are a useful adjunct in clinical follow-up of patients with progressive abdominal distention due to phosphate enema use.

Effects of L-carnitine supplementation on respiratory distress syndrome development and prognosis in premature infants: A single blind randomized controlled trial.

The aim of the present study was to investigate the efficacy of L-carnitine therapy on the occurrence and prognosis of respiratory distress syndrome (RDS). A single blind, randomized controlled trial study was conducted on 130 infants with gestational ages of 28-36 weeks. Infants were assigned to experimental groups (groups 1 and 2) and control groups (groups 3 and 4). Groups 1 and 3 consisted of infants with RDS, and groups 2 and 4 groups were composed of infants without RDS. The experimental groups were treated with carnitine. No statistically significant differences in serum carnitine levels were detected between the study and the control groups on day 1 of treatment (P=0.06). However, on day 7 of treatment, serum carnitine levels in the experimental groups were significantly increased (P=0.02), as compared with the control groups. The surfactant requirement value, which is how many rounds of surfactant therapy were required, was 1.56±0.97 in group 1, and 2.12±0.99 in group 3 (P<0.001). The mean duration of mechanical ventilation required was 3.04±3.60 days in group 1, and 4.73±5.63 days in group 3 (P<0.001). The present results indicate that carnitine supplementation in premature infants with RDS may help to increase carnitine levels, thus decreasing the duration of mechanical ventilation and surfactant requirement.

Effect of parenterally L-arginine supplementation on the respiratory distress syndrome in preterm newborns.

L-Arginine (L-Arg) is the precursor of nitric oxide which plays an important role on pulmonary circulation and pulmonary vascular tone. Earlier studies suggested that L-Arg levels in preterm newborns with respiratory distress syndrome (RDS) were low due to its consumption and L-Arg supplementation may reduce the severity of RDS. Our aim was detect the effect of the parenterally L-Arg supplementation on RDS severity. The subjects were chosen between preterm newborns (gestational age <34 weeks) (n = 30). Twenty of the subjects were diagnosed with permaturity and RDS, and 10 of the subjects were healthy preterm newborns. Ten of the subjects was taken L-Arg (1.5 mmol/kg/d) in addition to routine RDS treatment and assumed as "Group 1". In this group, daily L-Arg supplementation was started end of the first day, and continued at end of fifth day. The others of the subjects diagnosed with RDS was take routine RDS treatment and assumed as "Group 2". Healthy preterm newborns assumed as "Group 3". Blood collections for L-Arg levels via tandem mass spectrometry were made in first day and repeated on the seventh days. Oxygenation index was used to determine severity of RDS. L-Arg consentrations in Group 1 were 8.7 ± 4.1 µM/L and 11.9 ± 5.0 µM/L in first and seventh day, respectively. L-Arg consentrations were 12.6 ± 4.5 µM/Land 10.9 ± 5.4 µM/L in Group 2 and 8.6 ± 5.1 µM/L and 9.4 ± 4.1 µM/L in Group 3. There is no correlation between L-Arg concentrations and OI also duration of the mechanical ventilation of the subjects in patient groups (Group 1 and 2).

Investigation on malondialdehyde, S100B, and advanced oxidation protein product levels in significant hyperbilirubinemia and the effect of intensive phototherapy on these parameters.

BACKGROUND: The parameters of oxidative stress [advanced oxidation protein products (AOPPs), malondialdehyde (MDA), and S100B] and the effect of intensive phototherapy (PT) on these parameters have not been studied extensively in newborns with significant hyperbilirubinemia (SH). We aimed to measure the levels of MDA, S100B, and AOPPs in newborns with SH, and to compare newborns with healthy control newborns without hyperbilirubinemia on the basis of these parameters of oxidative stress. In addition, we investigated the effect of intensive PT on these parameters during the treatment of SH and report our findings for the first time in the literature. METHODS: The study was performed in newborns (n = 62) who underwent intensive PT because of SH. Newborns without jaundice constituted the control group (n = 30). Both groups were compared with respect to demographic characteristics and biochemical (laboratory) parameters including MDA, AOPPs, and S100B. MDA, AOPPs, and S100B were also compared before and after intensive PT in the PT group. In the study group, a correlation analysis of demographic characteristics; MDA, AOPP, and S100B values; and changes occurring in MDA, AOPPs, and S100B values due to the effect of intensive PT was performed. RESULTS: Serum total bilirubin, S100B, and MDA levels in the PT group before performing PT were significantly higher than those in the control group. In newborns receiving PT serum total bilirubin, MDA and AOPP levels decreased significantly after intensive PT. In correlation analysis, a statistically significant negative correlation was found only between the amount of bilirubin decrease with PT and AOPP levels after PT in the study group. CONCLUSION: Whether the significant decrease in MDA levels, which was higher prior to PT, is due to the decrease in serum bilirubin levels or due to the effect of intensive PT itself remains to be determined in further studies. The decrease in AOPP levels after PT implies that intensive PT has protective effects on oxidative stress.

The comparison of thyroxine versus thyroxine plus oral iodine in the treatment of congenital hypothyroidism due to iodine deficiency.

AIM: Iodine deficiency is one of the most important causes of congenital hypothyroidism. In addition to thyroid hormone replacement, iodine supplementation is also given to newborns with congenital hypothyroidism due to iodine deficiency. We aimed to determine whether it is beneficial to administer iodine supplementation in addition to the L-thyroxine (L-T4) treatment of newborns with congenital hypothyroidism due to iodine deficiency. MATERIALS AND METHODS: Of 51 newborns, 26 who were diagnosed with congenital hypothyroidism due to iodine deficiency were treated with L-T4. The remaining 25 cases were given L-T4 plus 100 µg/day of oral iodine. Free triiodothyronine (fT3), free thyroxine (fT4), thyroid-stimulating hormone (TSH), thyroglobulin (TG), thyroid volume, urine iodine and breast milk iodine levels were measured in the first and third months of treatment, and the data were compared between the two groups. RESULTS: First- and third-month values of fT3, fT4, TSH, TG and thyroid volume for both groups were statistically similar. There was no significant difference between the two groups in respect to falling levels of fT3 and TSH, the rate of increase of fT4 levels or the shrinkage rate of thyroid volume. CONCLUSION: In this study, the addition of oral iodine to L-T4 treatment provided no benefit compared to treatment with L-T4 alone.

Iodine deficiency: a probable cause of neural tube defect.

INTRODUCTION: Iodine deficiency is the most devastating event in developing brain in the fetus and neonate. Iodine is absolutely necessary on the myelination, neuronal differentiation, and formation of neural processes, synaptogenesis, and neuronal migration by thyroidal hormones throughout pregnancy and shortly after birth. Neural tube defects (NTD) form after third and fourth gestational weeks and their etiologies are multifactorial. CASE REPORT: We herein present a male newborn with iodine deficiency and thoracic neuroenteric cyst bound to a myelomeningocele via a pedinculi. We hypothesize that iodine deficiency may be a cause of NTD, and iodine supplementation in preconception and pregnancy may prevent NTD.

Guaiazulene: a new treatment option for recalcitrant diaper dermatitis in NICU patients.

AIM: Based on a maternal observation, we aimed to evaluate the treatment effectiveness of guaiazulene (GA) containing local pomade in the high-risk neonates with recalcitrant diaper dermatitis (RDD). METHODS: We included 30 NICU patients of RDD, with level II-III aged between 22 and 67 days. Study group patients (n = 20) were treated with GA containing local pomade (0.05 g/100 g). Control group consisted of patients who had extended antifungal treatment. A visual scale was used to assess the response to treatment at the end of a week. Scoring was done at the beginning of the treatment, on the first, third and seventh days. RESULTS: Statistically significant differences in visual scores were determined between the two groups at the initial and following days of the treatment. In study group, improvements at the first and third days of the treatment were better than those of control group. Additionally, complete recovery rate in study group was better than that in controls. CONCLUSION: Having beneficial but no adverse effects, GA containing local pomade provided rapid recovery in risky neonates with RDD, who required rapid improvement.

Case series of mercury toxicity among children in a hot, closed environment.

Mercury poisoning is much more prevalent in the general population than possibly many physicians realize. We present data on 26 pediatric cases with mercury intoxication from exposure to mercury by inhalation or skin contact as a result of a broken thermometer in a school laboratory. This is the largest pediatric series in Turkey. During a 3-month period, the study team observed the children for clinical symptoms, physical findings, and blood and mercury levels. Of all patients, 21 inhaled, 3 inhaled and touched the element, and 2 took the mercury home. Sixteen children were symptomatic at admission, although blood mercury levels in the symptomatic children were higher than those in asymptomatic children (P = 0.003). The urine mercury levels were not statistically different between the groups at the admission (P > 0.05). The exposure times were 3.5 and 2 hours for symptomatic and asymptomatic children, respectively (P = 0.003). The 2 children who took the mercury home had the highest blood mercury levels and the most prolonged exposure time. N-acetylcysteine and chelation treatments were started in 21 children who had symptoms of mercury intoxication and high mercury levels in their blood or urine. No adverse effects were observed during chelation therapy. Prompt removal of children from contaminated environments and proper decontamination or elimination of devices containing large amounts of mercury from schools are necessary to prevent serious complications caused by exposure to mercury.

A novel mutation in the MC2R gene causing familial glucocorticoid deficiency type 1.

Familial glucocorticoid deficiency (FGD) or hereditary unresponsiveness to adrenocorticotropin (ACTH) is an autosomal recessive disorder characterized by isolated glucocorticoid deficiency associated with normal mineralocorticoid secretion. Mutations in genes encoding either ACTH receptor or melanocortin 2 receptor accessory protein are responsible for the disease in about 50% of cases, named FGD type 1 and type 2, respectively. Patients may present with hyperpigmentation, recurrent infections, failure to thrive, hypoglycemic seizures, and coma in infancy or early childhood. Here we report the case of a 17-day-old newborn diagnosed with FGD type 1 who presented with hyperbilirubinemia and hyperpigmentation, a sign which was erroneously assumed to be due to prolonged phototherapy by the referring physician. Hormone analysis showed low cortisol and high ACTH levels with normal serum electrolytes and renin-aldosterone axis. Genetic analysis revealed a novel homozygous melanocortin 2 receptor mutation p.Leu225Arg in the patient. The healthy parents were heterozygous for the mutation.

Does early treatment prevent deafness in thiamine-responsive megaloblastic anaemia syndrome?

Thiamine-responsive megaloblastic anaemia (TRMA; OMIM 249270) syndrome is an autosomal recessive disorder characterized by diabetes mellitus, megaloblastic anaemia, and sensorineural deafness. Progressive hearing loss is one of the cardinal findings of the syndrome and is known to be irreversible. Whether the deafness in TRMA syndrome can be prevented is not yet known. Here, we report a four-month-old female infant diagnosed with TRMA syndrome at an early age. There was no hearing loss at the time of diagnosis. The patient's initial auditory evoked brainstem response measurements were normal. Although she was given thiamine supplementation regularly following the diagnosis, the patient developed moderate sensorineural hearing loss at 20 months of age, indicating that early diagnosis and treatment with oral thiamine (100 mg/day) could not prevent deafness in TRMA syndrome. It would be premature to draw general conclusions from one case, but we believe that further patient-based observations can shed light on the pathophysiology of this rare syndrome as well as prediction of its prognosis.

Thiamine withdrawal can lead to diabetic ketoacidosis in thiamine responsive megaloblastic anemia: report of two siblings.

Thiamine responsive megaloblastic anemia syndrome (TRMA), an autosomal recessive disorder caused by the deficiency of thiamine transporter protein, is the association of diabetes mellitus, anemia and deafness. Pharmacological dose thiamine normalizes hematological abnormalities and their effects on the course of diabetes mellitus. We report on 8 years follow up of two siblings with TRMA. They presented in the prepubertal period with diabetic ketoacidosis due to lack of thiamine supplementation for 2 months. Their insulin requirements fell rapidly and disappeared with thiamine therapy. Hematological parameters normalized within 30 days. The diabetic picture is responsive to thiamine treatment in patients with TRMA. Insulin dependent diabetes may occur throughout the pubertal period. If thiamine supplementation is not sufficient, ketoacidosis may develop in patients during the prepubertal period.

Congenital pulmonary lymphangiectasia in a newborn: a response to autologous blood therapy.

Congenital pulmonary lymphangiectasia is a rare condition that may present antenatally with pleural effusions and hydrops, and the prognosis is reported to be very poor. Treatments for lymphangiectasia have included corticosteroids for patients with primary inflammatory conditions, dietary modifications, surgical resection for isolated lesions, octreotide, antiplasmin therapy and fibrin glue pleurodesis. However, there is no experience with pleurodesis by autologous blood therapy in the literature. We present a newborn with primary pulmonary lymphangiectasis who developed progressively profuse chylous pleural effusions after enteral full feeding from the 8th day of life and improved with pleurodesis by autologous blood therapy.

Influence of maternal nicotine exposure on neonatal rat bone: protective effect of pentoxifylline.

Limited research in young adults and immature animals suggests a detrimental effect of tobacco on bone during growth. The aim of this study was to determine the adverse effects of maternal nicotine exposure during pregnancy and lactation on neonatal rat bone development, and to determine a protective effect of pentoxifylline (PTX). Gravid rats were assigned into four groups, one control (group I) and three experimental (groups II, III, and IV). In group II, pregnant rats received 3 mg/kg/day nicotine alone, subcutaneously, until 21 days postnatal. In group III, pregnant rats received nicotine (3 mg/kg/day) and PTX (60 mg/kg/day). In group IV, pregnant rats received PTX alone (60 mg/kg/day). Whole body mineral density (BMD), content (BMC), area (BA), and histopathologic and morphologic findings of the femur were determined at 21 days of age. The study revealed that nicotine exposure (group II) decreased birth weight, pregnancy weight gain, and length of femur compared with other groups (P < 0.01). Birth weight was higher in groups III (PTX + nicotine) and IV (PTX) than in group II (nicotine). Body weight at 21 days of age was higher (P = 0.009) in the PTX alone group (group IV) compared with the other groups. BMD was higher (P < 0.001) in the PTX-treated groups (group III and IV) compared with other groups. In addition, there were more apoptotic chondrocytes in the hypertrophic zone of rats exposed to nicotine alone (group II) compared with the other groups (P < 0.001). In conclusion, maternal nicotine exposure resulted in decreased birth weight, pregnancy weight gain, and bone lengthening, and increased apoptosis. Pentoxifylline supplementation was found to prevent the adverse effects of maternal nicotine exposure on BMD and birth weight.

Pubertal arrest due to Zn deficiency: the effect of zinc supplementation.

The Prasad Syndrome is characterized by iron deficiency anemia, hepatosplenomegaly, skin changes, hypogonadism, dwarfism and geophagia. Hypogonadism is a major manifestation of zinc (Zn) deficiency in both humans and animals. T he mechanism of hypogonadism caused by Zn deficiency has not been clarified. We present a 19 year-old boy with short stature, pubertal arrest, iron deficiency anemia and Zn deficiency. Based on the dynamic tests, the hypogonadism seems to be due to hypothalamic dysfunction. T he growth retardation was associated with low IGF-I and normal growth hormone (GH) secretion, indicating GH receptor or post receptor defect. Growth acceleration and testicular development was observed after Zn supplementation. Zn deficiency, although very rare, should be considered in patients with poor growth and hypogonadism associated with skin changes and anemia.

The relationship between birthweight, 25-hydroxyvitamin D concentrations and bone mineral status in neonates.

BACKGROUND: Calcium (Ca), phosphorus (P) and 25-hydroxyvitamin D (25-OHD) are the major micronutrients for fetal skeletal development. AIMS: To compare whole body bone mineral density (WB BMD) and bone mineral content (WB BMC) in different birthweights of term neonates and to determine correlations of biological criteria of bone health between neonates and their mothers. SUBJECTS AND METHODS: Serum Ca, P, alkaline phosphatase (ALP) and 25-OHD levels were measured in 30 small-for-gestational-age (SGA, group 1), 40 appropriate-for-gestational-age (AGA, group 2) and 30 large-for-gestational-age (LGA, group 3) neonates and their mothers in winter. WB BMD and WB BMC of neonates were estimated by dual-energy X-ray absorptiometry (DEXA) in the 1st 24 hrs after delivery. RESULTS: Mean (SD) serum 25-OHD levels in the mothers [8.7 (3.0), 8.6 (3.0) and 7.7 (2.8) microg/L, respectively] and their infants [6.3 (2.5), 6.0 (2.2) and 5.7 (1.8) microg/L, respectively] in groups 1, 2 and 3 were similar. Compared with the mothers, the mean 25-OHD levels of the neonates in all groups were significantly lower (p<0.05), and they were highly correlated (r=0.755, p<0.05). Ninety-three per cent of the neonates and 82% of their mothers had 25-OHD levels <10 microg/L, the lowest limit of normal. Mean (SD) WB BMD and WB BMC were higher in LGA infants [0.442 (0.025) g/cm(2), 71.6 (9.0) g, p<0.01, p<0.001, respectively] but lower in SGA [0.381 (0.027) g/cm(2), 29.1 (9.1) g, p<0.001, p<0.001, respectively] than in AGA infants [0.426 (0.022) g/cm(2), 53.7 (9.6) g, respectively]. The percentage of WB BMC was lower in SGA than in AGA and LGA infants. WB BMC and WB BMD were positively correlated with birthweight (r=0.910, p<0.05) and gestational age (r=0.707, p<0.05) but not with serum 25-OHD. CONCLUSIONS: The neonates' bone indices increased significantly with gestational age and birthweight but this was not related to serum 25-OHD levels in the infants and their mothers.

Influence of maternal nicotine exposure on neonatal rat bone: protective effect of ascorbic acid.

Limited research in young adults and immature animals suggests a detrimental effect of tobacco on bone during growth. We aimed to determine the adverse effects of maternal nicotine exposure during pregnancy and lactation on neonatal rat bone development, and to determine a protective effect of ascorbic acid. Gravid rats were assigned into three groups: two experimental and one control (group I). In the first experimental group (group II), pregnant rats received 3 mg/kg/d nicotine subcutaneously during pregnancy from 1 to 21 days of gestation and lactation (until postnatal day 21). The second experimental group (group III) received nicotine and ascorbic acid (1 mg/kg body mass/d). Whole body mineral density (BMD), content (BMC), and area (BA) were measured on postnatal day 21. Histopathologic and morphologic findings of the femur were obtained. Maternal nicotine exposure decreased the body weight of the rat at the birth and postnatal day 21. The values of BMD, BA, and BMC of the groups were similar to each other. Width of the epiphyseal plate and the hypertrophic zone were higher in group III but lower in group II than in group I. Number of apoptotic chondrocytes was significantly increased in group II. The length of femur was higher in group I but lower in group II than in group III. Maternal nicotine exposure during gestation and lactation resulted in decreased body weight and bone lengthening. Ascorbic acid supplementation was found to prevent the adverse effects of maternal nicotine exposure on the growth plate.

Effects of phototherapy on the growth plate in newborn rats.

The aim of the present study was to evaluate the effect of phototherapy and oxidative stress on the growth plate of newborn rats. Forty newborn Sprague-Dawley rats were randomized into a phototherapy group and a control group. Twenty of the rats received phototherapy for 7 days. All zones of the growth plate were assessed with quantitative histomorphometric analysis. Individual zonal lengths were measured for the reserve zone (RZ), the proliferative zone (PZ), the hypertrophic zone (HZ), ossifying cartilage (OC), and total zone (TZ) of the growth plate. Levels of plasma malondialdehyde (MDA), an index of oxidative stress, were also evaluated. Compared with zonal lengths on day 7 after phototherapy between the two groups, the phototherapy group had significantly lower values than those of controls for RZ (5.13 +/- 0.36 vs. 6.4 +/- 0.85 mm x 10(-2); P < 0.001), PZ (20.6 +/- 3.0 vs. 29.25 +/- 1.68 mm x 10(-2); P < 0.001), HZ (15.4 +/- 1.44 vs. 20.87 +/- 1.12 mm x 10(-2); P < 0.001), OC (47.08 +/- 4.25 vs. 62.06 +/- 3.7 mm x 10(-2); P < 0.001), and TZ (88.15 +/- 6.56 vs. 118.48 +/- 4.50 mm x 10(-2); P < 0.001). Plasma MDA levels were correlated with the size of the PZ in the phototherapy group (r = -0.53, P = 0.01). In a multivariate regression model for all rats, being in the phototherapy group was the best predictor of the size of the TZ (beta = -0.94, P < 0.001), with the total variance explained being 88%. These results suggest that in newborn rats, receiving phototherapy is associated with early impairment of growth plate structure, and oxidative stress may be the main risk factor for growth plate injury.

Severe hyperinsulinaemic hypoglycaemia in a baby born to a mother taking oral ritodrine therapy for preterm labour.

Hyperinsulinism of infancy is a major cause of persistent hypoglycaemia in the newborn period. Transient mild self-limiting hyperinsulinaemia and hypoglycaemia have been described in neonates born to mothers taking ritodrine therapy for premature labour. Ritodrine crosses the placental barrier and enters the fetal circulation readily but the mechanism of how it causes hyperinsulinaemia and hypoglycaemia is unclear. We report the case of severe prolonged hyperinsulinaemic hypoglycamia in a neonate born to a mother taking ritodrine therapy from 16 weeks' gestation for preterm labour. The hyperinsulinaemic hypoglycaemia was managed with oral nifedipine as diazoxide was contraindicated due to fluid overload. Possible mechanisms of ritodrine-induced hypoglycaemia and insulin secretion are discussed.

Iodine status remains critical in mother and infant in Central Anatolia (Kayseri) of Turkey.

BACKGROUND: Severe iodine deficiency disorders have been eradicated in many parts of the world, but milder forms still exist and may escape detection. Turkey has long been known to be a mild to moderate iodine deficiency area. AIM OF THE STUDY: The aim of this study was to assess the iodine nutritional status and the thyroid function of pregnant women and their neonates in the region of Kayseri (central Anatolia of Turkey) that appeared to be iodine deficient in previous studies performed before the introduction of mandatory salt iodization. METHODS: A cross-sectional voluntary screening study was performed in the Maternity Unit of a university hospital. A total of 70 mothers and their healthy full-term neonates were included in this study. Urinary iodine concentration was estimated in spot urine samples obtained from mothers and their neonates on day 5. All the neonates were breastfed. The iodine content was determined in the breast milk of all mothers on day 5. Serum concentrations of TSH, thyroglobulin (Tg), free triiodothyronine (FT3) and free thyroxine (FT4) were investigated in the cord serum of neonates and compared to those of mothers immediately after parturition RESULTS: The median urinary iodine on day 5 in mothers and their babies were 30.20 and 23.80 microg/l, respectively. These figures are much lower than normal for these age groups (150-200 microg/l). The median iodine content of breast-milk was 73 microg/l. It is again much lower than in iodine sufficient areas, indicating that the status of iodine nutrition of pregnant and lactating women is clearly insufficient. The median concentrations (and ranges) of neonatal TSH, Tg, FT3 and FT4 were 7.44 mU/l, 71.62 ng/ml, 1.30 pg/ml and 1.34 ng/dl respectively. The corresponding levels for the mothers during labor were 2.19 mU/l, 25.65 ng/ml, 1.31 pg/ml and 1.23 ng/dl respectively. The median neonatal serum concentrations of TSH and Tg were significantly higher than the corresponding maternal levels (P < 0.0001, P < 0.0001, respectively) and 27.1% of the neonates had serum TSH concentrations above 10 mU/l and 57.1 % had cord blood serum Tg concentrations above 54 ng/ml. None of the mothers showed TSH concentrations above 5 mU/l and 41.4% had serum Tg concentrations above 30 ng/ml. CONCLUSION: Iodine deficiency with low urinary iodine excretion and high serum Tg and TSH concentrations were recognized among pregnant women and their babies in Kayseri in spite of the program of salt iodization. National measures are urgently required for improving the correction of iodine deficiency in Turkey. This includes regular supplementation with iodine, starting at preconception or in early pregnancy and continuing during the period of nursing in this region.

Adrenocortical adenoma associated with inadequately treated congenital adrenal hyperplasia.

We report a 6 year-old boy with the simple virilizing form of 21-hydroxylase deficiency in whom an adrenal adenoma developed following 5 years of steroid treatment. Extremely high levels of basal serum 17alpha-hydroxyprogesterone as well as an exaggerated response of 17alpha-hydroxyprogesterone to adrenocorticotropic hormone confirmed congenital adrenal hyperplasia at 7 years of age. Initially elevated serum steroid levels were restrained by high dose hydrocortisone therapy, but he chronically tended to take inadequate doses of glucocorticoid. At 12 years of age an adenoma was found in the cortex of the hyperplastic right adrenal gland. The importance of early diagnosis and compliance with medication in the simple virilizing form of 21-hydroxylase deficiency is stressed.