Evaluation of frequency of autoimmune hepatitis autoantibodies in children with type 1 diabetes / Frecuencia de anticuerpos de hepatitis autoinmune en niños con diabetes tipo I

Background: Diabetes mellitus type 1 (T1DM) is one of the childhood diseases with growing prevalence. Various accompanying autoimmune diseases were seen with type 1 diabetes. The most common autoimmune diseases with T1DM are autoimmune thyroiditis and celiac disease. In some reports, autoimmune hepatitis has been reported in association with DM-1. Objectives: The aim of this study was to evaluate autoimmune hepatitis autoantibodies in children with T1DM. Materials and methods: In this crosssectional study, 202 children with T1DM were evaluated (47.5% were males and 52.5% were girls). Liver enzymes, autoimmune hepatitis related autoantibodies such as anti-nuclear antibodies (ANA), anti-smooth muscle (ASMA) and anti liver and kidney microsomal antibodies (LKM-1) were measured. Liver ultrasound was done for participants and biopsy of liver was taken for children with increased echogenicity of the liver, hepatomegaly or elevated liver enzymes. Results analyzed by statistical software spss-16, Descriptive statistics and chi-square test, paired T-TEST. Level of less than 5% was considered statistically significant. Results: In 6 patients ANA and in 4 patients (2%) ASMA was positive,1 patient was ASMA positive but ANA negative. None of the patients were Anti LKM-1 positive. 3 patients had positive ANA and ASMA, and increased liver echogenicity on ultrasound simultaneously. Histological evaluation was showed that 2 patients had findings in favor of autoimmune hepatitis. Conclusion: Auto antibodies were positive in 10 cases. ANA was positive in 6 (2.97%) of all cases. ASMA was positive in 4 (1.98%) cases. Increased echogenicity was found in 3 cases. Histological evaluation showed 2 patients had biopsy confirmed autoimmune hepatitis. AIH-2 was not seen among our cases.

Antecedentes: La diabetes mellitus tipo 1 (DM1) es una de las enfermedades infantiles con mayor prevalencia. Se observaron varias enfermedades autoinmunes acompañantes con diabetes tipo 1. Las enfermedades autoinmunes más comunes con DM1 son la tiroiditis autoinmune y la enfermedad celíaca. En algunos reportes, se ha encontrado hepatitis autoinmune en asociación con DM-1. Objetivos: El objetivo de este estudio fue evaluar los autoanticuerpos de hepatitis autoinmunes en niños con DM1. Materiales y métodos: En este estudio transversal, se evaluaron 202 niños con DM1 (47,5% eran hombres y 52,5% eran niñas). Se midieron las enzimas hepáticas, los autoanticuerpos autoinmunes relacionados con la hepatitis, como los anticuerpos antinucleares (ANA), el músculo liso (ASMA) y los anticuerpos microsomales hepáticos y renales (LKM-1). Se realizó una ecografía hepática para los participantes y se tomó una biopsia del hígado para niños con mayor ecogenicidad del hígado, hepatomegalia o enzimas hepáticas elevadas. Los resultados fueron analizados por el software estadístico spss-16 usando estadística descriptiva y prueba de chi-cuadrado, T-TEST pareado. Se consideró estadísticamente significativo un nivel menor del 5%. Resultados: En 6 pacientes con ANA y en 4 pacientes (2%) ASMA fue positiva, 1 paciente fue ASMA positiva pero ANA negativa. Ninguno de los pacientes fue anti LKM-1 positivo. 3 pacientes tuvieron ANA y ASMA positivas, y aumentaron la ecogenicidad hepática en la ecografía simultáneamente. La evaluación histológica mostró que 2 pacientes tenían hallazgos a favor de la hepatitis autoinmune. Conclusión: Los autoanticuerpos fueron positivos en 10 casos. ANA fue positivo en 6 (2,97%) de todos los casos. La ASMA fue positiva en 4 (1,98%) casos. Se encontró mayor ecogenicidad en 3 casos. La evaluación histológica mostró que 2 pacientes tenían biopsia confirmada de hepatitis autoinmune. AIH-2 no fue visto entre nuestros casos.

Evaluation of frequency of autoimmune hepatitis autoantibodies in children with type 1 diabetes.

BACKGROUND: Diabetes mellitus type 1 (T1DM) is one of the childhood diseases with growing prevalence. Various accompanying autoimmune diseases were seen with type 1 diabetes. The most common autoimmune diseases with T1DM are autoimmune thyroiditis and celiac disease. In some reports, autoimmune hepatitis has been reported in association with DM-1. OBJECTIVES: The aim of this study was to evaluate autoimmune hepatitis autoantibodies in children with T1DM. MATERIALS AND METHODS: In this crosssectional study, 202 children with T1DM were evaluated (47.5% were males and 52.5% were girls). Liver enzymes, autoimmune hepatitis related autoantibodies such as anti-nuclear antibodies (ANA), anti-smooth muscle (ASMA) and anti liver and kidney microsomal antibodies (LKM-1) were measured. Liver ultrasound was done for participants and biopsy of liver was taken for children with increased echogenicity of the liver, hepatomegaly or elevated liver enzymes. Results analyzed by statistical software spss-16, Descriptive statistics and chi-square test, paired T-TEST. Level of less than 5% was considered statistically significant. RESULTS: In 6 patients ANA and in 4 patients (2%) ASMA was positive,1 patient was ASMA positive but ANA negative. None of the patients were Anti LKM-1 positive. 3 patients had positive ANA and ASMA, and increased liver echogenicity on ultrasound simultaneously. Histological evaluation was showed that 2 patients had findings in favor of autoimmune hepatitis. CONCLUSION: Auto antibodies were positive in 10 cases. ANA was positive in 6 (2.97%) of all cases. ASMA was positive in 4 (1.98%) cases. Increased echogenicity was found in 3 cases. Histological evaluation showed 2 patients had biopsy confirmed autoimmune hepatitis. AIH-2 was not seen among our cases.

Wolcott-Rallison Syndrome With Different Clinical Presentations and Genetic Patterns in 2 Infants.

Wolcott-Rallison syndrome is a rare disease presenting with insulin-dependent diabetes mellitus (DM) before 6 months old, skeletal dysplasia after 6 months old, and liver failure. Other manifestations are renal failure, microcephaly, epilepsy, central hypothyroidism, neutropenia, and dental and dermal problems. The cases were 2 patients from 2 different states of Iran (Khoozestan and Fars) who had developed DM before 6 months old. The first one was a 7-month-old infant who was healthy; in the genetic study (screening), autosomal recessive pattern and novel deletion in EIF2AK3 were reported; her sister had died at 5.5 years old due to diabetic ketoacidosis (DKA) that was associated with liver and renal failure. The second patient had developed DKA at 45 days old, which was associated with mild acute tubular necrosis and abnormal coagulation tests at onset clinical presentation, which were then resolved. He was treated with insulin, and at follow-up, the laboratory data are normal; in the genetic study, EIF2AK3 nonsense homozygous mutation was diagnosed. Genetic study of patients with insulin-dependent DM before 6 months old, especially those with DKA and associated with or without other disorders; attention to novel deletion of in EIF2AK3 gene; screening for skeletal dysplasia after 1 year old; and renal, liver, pancreatic, and thyroid function tests are recommended.

Evaluation of Metabolic Syndrome and Related Factors in Children Affected by Acute Lymphoblastic Leukemia.

INTRODUCTION: Obesity is among the medical problems in survivors of childhood acute lymphoblastic leukemia (ALL). These patients are at risk of metabolic syndrome (MS). The present study aimed to follow the patients with ALL regarding the incidence of MS. PATIENTS AND METHODS: This study was conducted on all patients who referred to the oncology clinic from July 2012 to July 2013. The exclusion criteria of the study were ALL relapse, secondary malignancy, hypothyroidism, Down syndrome, and below 2 years of age. ALL had to be diagnosed at least 12 months before enrollment into this study. MS was assessed by serum triglyceride, cholesterol, fasting blood sugar, leptin, and insulin levels. Besides, body mass index (BMI) and blood pressure (BP) were measured at diagnosis and at the last visit. RESULTS: This study was conducted on 53 patients (male = 35, female = 18). At the end of the study, 13 patients (24.53%) were overweight compared to 3 patients at diagnosis (P = 0.04). The mean blood leptin level was higher in overweight patients compared to the others (P = 0.001). MS was detected in 21 patients (39.6%), including 12 males and 9 females. In addition, the BMI Z-score significantly increased over the study period (P = 0.001). CONCLUSIONS: Being overweight is a complication of ALL treatment, which is associated with elevated blood leptin level and BMI Z-score. Therefore, MS criteria, such as BP, weight, and serum triglyceride level, should be taken into account in each visit.

Bruck syndrome - a rare syndrome of bone fragility and joint contracture and novel homozygous FKBP10 mutation.

Bruck syndrome is an autosomal recessive syndrome consisting of bone fragility and congenital joint contractures. According to the genotype, it has been classified into types 1 and 2. Recently, mutations in FKBP10, localised to chromosome 17q21, have been identified in some patients of Bruck syndrome. Twenty-seven patients of this syndrome have been reported so far. We present a new patient of this syndrome, with frequent fractures, congenital joint contractures, kyphoscoliosis, bilateral clubfoot, and pectus carinatum. The clinical and genetic features of all previously reported cases are also reviewed.

Effect of Growth Hormone treatment on Height Velocity of Children with Pycnodysotosis.

OBJECTIVE: Pycnodysostosis is a rare autosomal recessive osteochondrodysplasia resulting from osteoclast dysfunction. Growth hormone (GH) secretion impairment and low insulin growth factor 1 (IGF-I) concentrations have been reported in these patients. The present study aims to describe GH effect on linear growth of eight children with pycnodysostosis. METHODS: This study was conducted on 8 children suffering from pycnodysostosis. After evaluating systemic diseases, adrenal insufficiency, and hypothyroidism, bone age, height standard deviation score (HtSDS), body mass index (BMI), and some demographical characteristics were measured. To measure the serum GH, we performed two clonidine tests in two different days with an interval of 24 hours. With initiation of the trial, human GH was injected subcutaneously once a day 6 days a week for a period of 1.5 years. The patients were followed up every 3 months to document their height and BMI until 6 months after the end of the treatment. FINDINGS: All of the patients had growth hormone deficiency. HtSDS at the first visit continued to decrease during the 6 months before starting the treatment; however, HtSDS started to increase after beginning of GH administration. This value again declined after discontinuing the GH. Overall, the mean of linear growth was improved after GH administration in the patients. CONCLUSION: The present clinical study revealed that GH administration had a positive impact on the linear growth of the children suffering from pycnodysostosis.

Metabolic syndrome, insulin resistance and Fatty liver in obese Iranian children.

BACKGROUND: Obesity is a global epidemic and its morbidities such as metabolic syndrome, insulin resistance, and fatty liver leads to a spectrum of psycho-social and medical consequences. OBJECTIVES: The objective of this study was to investigate the prevalence of fatty liver in obese Iranian children and its' association with metabolic syndrome and insulin resistance. PATIENTS AND METHODS: 102 obese Iranian children, referred to pediatric clinics from March 2011 to March 2012, were enrolled in this cross-sectional study. All the patients were visited by a pediatric endocrinologist, a pediatric gastroenterologist and an expert radiologist in the evaluation of fatty liver grading. RESULTS: The grade of fatty liver was higher in older children (P = 0.001). It was also more in taller and heavier children (P = 0.000). The more the BMI was, the more the fatty liver grade was (P = 0.002). Severity of fatty liver according to liver sonography in patient had a positive relationship with waist circumference, hip circumference, serum TG, serum FBS, serum fasting insulin, serum ALT, systolic blood pressure and HOMA index and had a negative correlation with the level of alkaline phosphatase. Severity of fatty liver also had a close relationship with the presence of acanthosis nigricans and HOMA index. CONCLUSIONS: Prevalence of fatty liver is high in our obese children. It was associated with criteria of metabolic syndrome and insulin resistance, so visceral fat may participate in the pathogenesis of the metabolic syndrome or merely serve as a marker of increased risk for the metabolic complications of obesity.

Hypothyroidism in ß-Thalassemia Intermedia Patients with and without Hydroxyurea.

Hydroxyurea (HU) has been successfully used in patients with ß-thalassemia intermedia (ß-TI). We aimed to evaluate the effect of the long-term use of HU on thyroid function in patients with ß-TI. Seventy-five patients with ß-TI aged≥11 years and taking HU were randomly selected during 2010 in southern Iran. Thirty-one patients with ß-TI without HU were considered as a control group. Serum levels of thyroid stimulating hormone (TSH) and T4 were measured. The mean age of the participants was 22.7±5.1 years (age range=12-41 years). Serum ferritin level had no significant correlation with HU consumption (P>0.05). Overall, we detected 10 (9.4%) patients with hypothyroidism. We found that the use of HU at a dose of 8-15 mg/kg/day has no significant association with thyroid function in ß-TI patients. However, due to the small sample size in our study, documentation of this finding needs further studies with higher numbers of patients.

Effects of hypo- and hyperthyroid states on herpes simplex virus infectivity in the rat.

OBJECTIVE: Available data from in vitro studies show that thyroid hormones (THs) regulate herpes simplex virus (HSV) gene expression and may modulate latency/reactivation of the virus. Whether infectivity of the virus is also affected by THs is not known. Using animal models (in vivo study) and Vero cell culture (in vitro study), we examined the effects of alterations in THs level on HSV-1 infectivity. METHODS: Rats were rendered hypo- and hyperthyroid by daily addition of methimazole and l-thyroxine into their drinking water, respectively. Euthyroid animals served as control. All animals were given a single dose of HSV-1 (10(7)TCID50, ip) and sacrificed 3 d later. The spleen of the animals was then removed and viral particles were recovered from the tissue extract through aseptic procedures. Serial dilution of the extracts was prepared and added to Vero cell culture. For the in vitro study, the cultures were pretreated with l-thyroxine and the viral particles were then added. Virus titration was determined by Reed-Muench quantal assay. RESULTS: The viral load of spleen in hyperthyroid rats was significantly lower (1000-fold) than that of the euthyroid rats. Similarly, in vitro presence of supraphysiologic levels of l-thyroxine in the culture media of Vero cells decreased virus infectivity. Interestingly, hypothyroid animals showed a significant increase (10-fold) in spleen viral load as compared to that of their euthyroid counterparts. CONCLUSIONS: These data clearly show that the HSV-1 infectivity is affected by THs, and suggest that THs or their analogs may have a potential application in prevention and/or treatment of viral infections.

Effect of pubertal suppression on linear growth and body mass index; a two-year follow-up in girls with genetic short stature and rapidly progressive puberty.

OBJECTIVE: Gonadotropin-Releasing Hormone agonists (GnRHa) are used to improve the final adult height in short stature children. There are limited studies which address the potential side effect of these agents: excessive weight gain. We have followed girls with rapidly progressive puberty receiving GnRHa and results were focused on the effect of treatment on final height, weight and body mass index Methods: Thirty girls between 8.5 and 12 years with short stature and predicted adult height of less than 155 cm were enrolled in the study. All had rapidly progressive puberty. Weight and height measurements were done at the beginning of treatment, 6 and 12 months after starting and 6 and 12 months after the cessation of treatment. Bone age and stages of puberty were estimated at the beginning of treatment, after 12 months of starting and 12 months after the treatment was stopped. Findings : Predicted adult height (PAH) changes during treatment were not significant. There was no significant difference between final height and weight according to the body mass index (BMI), PAH or bone age. CONCLUSION: We conclude that girls with genetic short stature and rapidly progressive puberty will not benefit receiving a one-year course of GnRHa and there is no significant difference between the final height and final weigh among children according to BMI.

Beneficial effect of cyproheptadine on body mass index in undernourished children: a randomized controlled trial.

OBJECTIVE: Cyproheptadine hydrochloride (CH) is a first-generation antihistamine which is used as an appetite stimulant. This study was designed to identify the role of CH therapy on weight gain, linear growth and body mass index in children with mild to moderate undernutrition. METHODS: Eighty-nine patients were enrolled. The present randomized, double-blinded controlled trial included 77 evaluable patients, aged 24-64 months with undernutrition. The patients were randomized to receive cyproheptadine with multivitamin, or multivitamin over a period of four weeks. The weight, height and body mass index were measured at the baseline, four weeks after intervention and four weeks after discontinuation. FINDINGS: A significant higher body mass index was observed among CH-treated patients after 8 weeks intervention with cyproheptadine compared with the control group (P<0.041). Mean weight gain after eight weeks was 0.11 kg in the control group and 0.60 kg in the CH group. There were no significant differences in changes of weight and height velocity across the study between CH-treated and control group at the end of study. CONCLUSION: In our study, cyproheptadine promotes increase in body mass index in children with mild to moderate undernutrition after four weeks treatment.

Changing the Treatment of Permanent Neonatal Diabetes Mellitus from Insulin to Glibenclamide in a 4-Month-Old Infant with KCNJ11 Activating Mutation.

Permanent neonatal diabetes mellitus (PNDM) is a rare type of diabetes and KCNJ11 gene activating mutation is one of its prevalent causes. We introduced a 4-month-old male infant with poor feeding, restlessness, tachypnea, hyperglycemia, metabolic acidosis, and ketonemia. He was discharged with insulin and after 2 months, KCNJ11 gene mutation was found and treatment was switched from subcutaneous insulin to oral glibenclamide. Now, he is 1 year old with desirable glycemic control; therefore, genetic study is recommended for KCNJ11 gene mutation in such patients because if the mutation is found, treatment can be switched from insulin to sulfonylurea.

Prevalence of celiac disease in patients with type 1 diabetes mellitus in the south of Iran.

BACKGROUND/AIMS: Celiac disease is a chronic enteropathy caused by hypersensitivity to gluten. An increased prevalence of celiac disease has been found in children with diabetes mellitus type 1. The large differences in the frequency of celiac disease in different countries show a great regional variability. Our aim was to detect the prevalence of celiac disease in diabetes mellitus type 1 children among southern Iranians. MATERIALS AND METHODS: A prospective study was conducted on 83 diabetes mellitus type 1 children from the south of Iran. They were tested for the presence of anti-tissue transglutaminase immunoglobulin A antibody and total immunoglobulin A level. The patients testing immunoglobulin A anti-tissue transglutaminase-positive were offered small bowel biopsy. RESULTS: Eighty-three children with diabetes mellitus type 1 (49 females, 34 males) aged 10.38±4.7 years were enrolled. None of the patients was immunoglobulin A deficient. Twelve diabetic children had a high titer of anti-tissue transglutaminase immunoglobulin A (14.4%). In four patients, biopsy was in favor of celiac disease (4.8%). CONCLUSIONS: Our study showed that the prevalence of celiac disease in diabetes mellitus type 1 children in Iran is more than in America and Europe but similar to that observed in Turkey. The age of the patient and duration of disease had an effect on this prevalence, and more studies should be done to determine the effects of ethnic, genetic and environmental factors such as diet to identify the reasons for these differences.

The side effects of gonadotropin releasing hormone analog (diphereline) in treatment of idiopathic central precocious puberty.

Treatment of central precocious puberty (CPP) is the administration of GnRH analogs. Metabolic syndrome comprised metabolic disturbances that confer increased risk of (CVD) diabetes mellitus (DM) and cardiovascular disease. This study is a longitudinal prospective study in pediatric endocrinology clinic. 30 non-obese children with idiopathic CPP were involved. Total body weight, height, blood pressure, BMI and waist circumference of the patients along with their triglyceride (TG), total cholesterol (TC), low density lipoprotein (LDL), high density lipoprotein (HDL), fasting plasma sugar (FPS) were evaluated at the beginning and during 3 and 6 months GnRH analog therapy. All of the patients involved in this study were female with age 9.5±1.02 years. Waist circumference, weight and BMI were 69.3 cm, 37.21 kg, and 19.13 kg/cm(2) before therapy and 72.25 cm, 40.11 kg, and 19.54 kg/m(2) 6 months after therapy respectively. Mean systolic and diastolic blood pressure of the patients before therapy was 96.83 mmHg, 66mmHg and after 6 months therapy was 98.66 mmHg, 89.63 mmHg respectively. Mean TG, LDL, HDL and FPS were 90.06 mg/dl, 91.6 mg/dl, 43.7 mg/dl and 89.6 mg/dl before therapy and 96.4 mg/dl, 93.1 mg/dl, 44.7 mg/dl and 91.36 after 6 months therapy respectively. GnRH analog therapy doesn't cause metabolic syndrome after 3 and 6 month therapy but it may cause hyperlipidemia and central obesity.

Juvenile paget's disease in an Iranian kindred with vitamin D deficiency and novel homozygous TNFRSF11B mutation.

Juvenile Paget's disease (JPD) is a rare heritable osteopathy characterized biochemically by markedly increased serum alkaline phosphatase (ALP) activity emanating from generalized acceleration of skeletal turnover. Affected infants and children typically suffer bone pain and fractures and deformities, become deaf, and have macrocranium. Some who survive to young adult life develop blindness from retinopathy engendered by vascular microcalcification. Most cases of JPD are caused by osteoprotegerin (OPG) deficiency due to homozygous loss-of-function mutations within the TNFRSF11B gene that encodes OPG. We report a 3-year-old Iranian girl with JPD and craniosynostosis who had vitamin D deficiency in infancy. She presented with fractures during the first year-of-life followed by bone deformities, delayed development, failure-to-thrive, and pneumonias. At 1 year-of-age, biochemical studies of serum revealed marked hyperphosphatasemia together with low-normal calcium and low inorganic phosphate and 25-hydroxyvitamin D levels. Several family members in previous generations of this consanguineous kindred may also have had JPD and vitamin D deficiency. Mutation analysis showed homozygosity for a unique missense change (c.130T>C, p.Cys44Arg) in TNFRSF11B that would compromise the cysteine-rich domain of OPG that binds receptor activator of NF-κB ligand (RANKL). Both parents were heterozygous for this mutation. The patient's serum OPG level was extremely low and RANKL level markedly elevated. She responded well to rapid oral vitamin D repletion followed by pamidronate treatment given intravenously. Our patient is the first Iranian reported with JPD. Her novel mutation in TNFRSF11B plus vitamin D deficiency in infancy was associated with severe JPD uniquely complicated by craniosynostosis. Pamidronate treatment with vitamin D sufficiency can be effective therapy for the skeletal disease caused by the OPG deficiency form of JPD.

Cardiovascular involvement in children with osteogenesis imperfecta.

OBJECTIVE: Osteogenesis imperfecta is a hereditary disease resulting from mutation in type I procollagen genes. One of the extra skeletal manifestations of this disease is cardiac involvement. The prevalence of cardiac involvement is still unknown in the children with osteogenesis imperfecta. The present study aimed to investigate the prevalence of cardiovascular abnormalities in these patients. METHODS: 24 children with osteogenesis imperfecta and 24 normal children who were matched with the patients regarding sex and age were studied. In both groups, standard echocardiography was performed, and heart valves were investigated. Dimensions of left ventricle, aorta annulus, sinotubular junction, ascending and descending aorta were measured and compared between the two groups. FINDINGS: The results revealed no significant difference between the two groups regarding age, sex, ejection fraction, shortening fraction, mean of aorta annulus, sinotubular junction, ascending and descending aorta, but after correction based on the body surface area, dimensions of aorta annulus, sinotubular junction, ascending and descending aorta in the patients were significantly higher than those in the control group (P<0.05). Two (8.3%) patients had aortic insufficiency and five (20%) patients had tricuspid regurgitation, three of whom had gradient >25 mmHg and one patient had pulmonary insufficiency with indirect evidence of pulmonary hypertension. According to Z scores of aorta annulus, sinotubular junction and ascending aorta, 5, 3, and 1 out of 24 patients had Z scores >2 respectively. CONCLUSION: The prevalence of valvular heart diseases and aortic root dilation was higher in children with osteogenesis imperfecta. In conclusion, cardiovascular investigation is recommended in these children.

Investigation of the relationship between retinol binding protein 4, metabolic syndrome and insulin resistance in Iranian obese 5-17 year old children.

OBJECTIVE: Retinol-binding protein 4 (RBP4) has recently been reported to be associated with insulin resistance (IR) and the metabolic syndrome by a number of researchers in various populations. However, controversies are present among different studies, which might be due to the differences between various ethnic, age, and sex groups. This study aimed to determine whether RBP4 can be assumed as a marker of IR and the metabolic syndrome in the Iranian obese children. METHODS: In the present longitudinal cross-sectional study, 100 5-17 years old obese children were recruited from January 1, 2011 to February 1, 2012. The patients' information including the demographic variables, health status and behavior, and daily physical activity were collected. Moreover, serum RBP4 was measured and correlated with the homeostasis model assessment of IR index (HOMA-IR), components of the metabolic syndrome, and lipoprotein metabolism. FINDINGS: The results revealed a positively significant correlation between RBP4 and the HOMA-IR index (P=0.02). Partial Spearman test also revealed a significant correlation between RBP4 plasma concentrations and the components of the metabolic syndrome, including waist circumference, systolic (but not diastolic) blood-pressure, and fasting blood sugar (P<0.05). However, no significant correlation was observed between RBP4 and HDL (P=0.3) as well as triglycerides concentration (P=0.1). Moreover, plasma RBP4 level gradually increased with the increasing number of the metabolic syndrome components. CONCLUSION: Regarding the results of the present study and previous investigations, RBP4 seems to be a suggestible predictive marker for both insulin resistance and metabolic syndrome in Iranian obese children; however, further studies are needed to be conducted among different ethnicities and age groups in order to determine the predictive value of this correlation.