Ultrasonic evaluation of thelarche grading in 2-8-year-old obese girls suspected of precocious pubert.

INTRODUCTION: The prevalence of obesity in children is increasing. In obese children, clinical examination alone is not sufficient to differentiate lipomastia from thelarche. The aim of this study was to investigate the frequency of true thelarche in obese girls suspected of precocious puberty. MATERIAL AND METHODS: In a cross-sectional study, 100 obese girls between the ages of 2 and 8 years were screened. Maturity stages were determined based on the Tanner scale in the questionnaire. Breast ultrasound was performed for all cases. Other uterine and ovarian laboratory tests, including blood tests to determine plasma levels of LH, FSH, oestradiol, and bone age, were also performed. RESULTS: In ultrasonography examination of 80 children (80%), one breast was pubertal and in 72 people both breasts were pubertal in which ultrasonography of 78 children showed puberty of right breast and 74 children showed puberty of left breast. Twenty children showed bilateral lipomastia. In clinical examination, breasts of 18.9% children seemed lipoid, 35.8% children were seriously suspicious, and 45.3% children seemed pubescent. In pelvic ultrasound, the relationship between ovarian volume and breast ultrasound grade was significant, but uterine volume was not significantly related to breast ultrasound grade. Also, uterine volume with age at ultrasonography, bone age, birth height, left ovarian volume, right ovarian volume, right breast bud diameter, and left breast bud diameter were correlated. CONCLUSIONS: Ultrasound can help obese or overweight children to differentiate between true thelarche and lipomastia. The study also found that most suspected children had true thelarche.

Gonadotropin-Releasing Hormone Agonist Therapy and Obesity in Girls.

BACKGROUND: Depot preparations of gonadotropin-releasing hormone agonists (GnRHa) are the gold standard drugs for the treatment of central precocious puberty. A concern about these drugs is obesity. OBJECTIVES: This study aimed to investigate the effect of gonadotropin-releasing hormone agonists (GnRHa) therapy on body mass index (BMI) in girls with central precocious puberty (CPP). PATIENTS AND METHODS: The girls with onset of puberty before eight years of age or menarche before nine years of age were studied. The weight, height, BMI, and pubertal stage were determined before and at sixth and 12th months of treatment. The GnRHa (Triptorelin) was administered intramuscularly for patients with rapidly progressive forms of CPP. Patients with slowly progressive forms of CPP were considered as control group. RESULTS: From 110 subjects with CPP, 46 girls (41.8%) were considered as intervention and 64 (58.2%) as control groups. The mean age at initial visit was 7.46 ± 1.03 years. The BMI standard deviation scores in both groups was not significantly different at sixth and 12th months of treatment compared with baseline (P = 0.257 and P = 0.839, respectively). The prevalence of obesity was not significantly different between study groups at baseline and at and sixth and 12th months of therapy (P = 0.11, P = 0.068, and P = 0.052, respectively). CONCLUSIONS: The GnRHa therapy has no effect on BMI and the prevalence of obesity.

First report of 3-oxothiolase deficiency in iran.

INTRODUCTION: Mitochondrial acetoacetyl-CoA thiolase (3-oxothiolase) deficiency is a rare metabolic disorder involving ketone body metabolism characterized by acute attacks of vomiting, acidosis, ketosis, and lethargy along with some laboratory criteria including excessive excretion of 2-methyl-3-hydroxybutyric acid in urine. CASE PRESENTATION: This is a case report of 3-oxothiolase deficiency in a young Iranian boy with presentation of intractable vomiting and severe metabolic acidosis following a common cold in six months of age with abundant urinary 2-methyl-3- hydroxybutyric acid. DISCUSSION: This is the first Iranian 3-oxothiolase deficiency case report as searched in the literature. Because of the high rate of consanguineous marriages in Iran, physicians should consider the 3-oxothiolase deficiency in the differential diagnosis of any patient with intractable vomiting and severe metabolic acidosis.

Effect of Vitamin E and Metformin on Fatty Liver Disease in Obese Children- Randomized Clinical Trial.

BACKGROUND: We evaluated the effect of vitamin E and metformin on fatty liver disease in obese children. METHODS: This interventional study has been done on 119 children with Non-alcoholic fatty liver disease (based on sonography results). Patients were divided into four treatment groups; they received metformin 1gr daily (age< 12 years), metformin 1.5 gr daily (age> 12 years), vitamin E 800 U daily and vitamin E 400 U daily. Liver sonography was performed for patients for two periods of two months. This trial was registered in Iranian Registry of Clinical Trials (IRCT), No.IRCT2013021012421N1. RESULTS: The study group comprised 119 individuals (62 females, 57 males). The mean age was 10± 3.19 yr. There was no significant difference in terms of sex and BMI between the groups. Overall liver sonography showed normal liver in 66 patients (55.46%), 66.63% after two months and 33.37% after four months. After two months, the most therapeutic response observed in the group which received vitamin E 800 u daily (48.1%) and the least therapeutic response was in the group which received vitamin E 400 u daily (14.3%). After four months, the greater response was seen in vitamin E 400 u daily group (45.8%) and the least response in the metformin 1 gram daily group (19%). CONCLUSION: In comparison with metformin, vitamin E is more influential in remission; however both are efficient in treatment of fatty liver. Vitamin E 400 u daily responses better in four-month treatment.

Cartilage Hair Hypoplasia: First report from Iran.

Cartilage hair hypoplasia (CHH), is a rare cause of metaphyseal chondrodysplasia and short stature. Other featuresincluded hair abnormality, immunodeficiency, anemia, gastrointestinal disorders (Hirschsprung disease,celiac, …) and increased risk of cancer. The disease is an autosomal recessive disorder and previously has notbeen reported in Iran. We report a 9-year-old boy diagnosed as cartilage hair hypoplasia, with severe short stature,metaphyseal chondrodysplasia, hair hypoplasia, Hirschsprung disease, hypothyroidism, vesicouretral refluxand renal stone. Renal stone and hypothyroidism have been reported in cartilage hair hypoplasia with lower frequencies.This is the first report of cartilage hair hypoplasia in Iran.