Endokrynologia, diabetologia i choroby przemiany materii wieku rozwojowego : organ Polskiego Towarzystwa Endokrynologow Dzieciecych最新文献

The most frequent cause of goiter in children is a deficit of iodine, leading to endemia of goiter in the regions with insufficient supplementation of this element. Goiter occurs also in the course of autoimmunological diseases of the thyroid gland (Hashimoto disease, Graves' disease), genetically-related disorders of thyroid hormones, biosynthesis/impaired biosynthesis of thyroid hormones. According to the theory of goiter pathogenesis, excessive enlargement of the thyroid gland is due to adaptation of follicle cells of the gland aiming at neutralizing the impaired synthesis of the thyroid hormones caused by various intrathyroid, environmental and genetic factors/agents. The mechanisms stimulating thyrocytes to hyperplasia or hypertrophy are very complex and still unknown in spite of having identified many physiological and pathogenetic factors connected with goiter.

The treatment of diabetes mellitus with a continuous subcutaneous insulin infusion (CSII) has become very popular and is well accepted by diabetic patients. Pump therapy mimics the physiological insulin secretion and has been shown to be a safe and effective method of insulin administration alternative to the method of multiple injections. Continuous insulin infusion provides greater flexibility in the timing of meals and snacks with higher treatment satisfaction. Programmed basal rates improve nocturnal glycemic control and help to minimize a pre-breakfast increase of blood glucose level (the dawn phenomenon). Moreover, CSII can reduce exercise-induced and nocturnal hypoglycemia. Insulin pump therapy is effective in lowering glycated hemoglobin levels without higher risk of severe hypoglycaemia and ketoacidosis. To achieve a proper metabolic control with this method of treatment, the patient and his family requires appropriate education including knowledge of diet management, insulin therapy and manual competence of pump device. Here we present general guidelines for patients education concerning insulin dosage, programming of basal insulin rates and meal boluses based on carbohydrates and protein-fat exchanges.

Background: Increased prevalence of obesity in children and adolescents results in more common metabolic complications characteristic for adults, particularly those with abdominal obesity. The objective of the study was to determine the relation between the fat tissue distribution and metabolic complications and to estimate the prevalence of the metabolic syndrome in obese children and adolescents.

Material and methods: We studied 64 children (42 girls and 22 boys) with simple obesity (BMI =97 pc) in the mean age 12.4+/-3.4 years. The fat tissue distribution was assessed on the basis of waist circumference, hip circumference, waist to hip ratio (WHR) and skinfold thickness (suprailiac, subscapular, biceps and triceps). In all children plasma concentrations of total cholesterol, HDL and LDL cholesterol as well as triglycerides were estimated. Plasma glucose and insulin levels were measured in fasting state and during the oral glucose tolerance test (OGTT). Fasting insulin to glucose ratio (FIGR) was calculated. Blood pressure was measured in triplicate.

Results: In 33 (51.6%) of children dyslipidemia, in 10 (15.6%) hyperinsulinemia or impaired glucose tolerance and in 12 (18%) hypertension was diagnosed. The Metabolic syndrome was present in 9 (14%) children. The anthropometric predictor for the risk of metabolic complications was a greater waist circumference, while greater hip circumference decreased the risk.

Conclusions: The metabolic complications characteristic of metabolic syndrome, previously diagnosed exclusively in adults, may occur also in obese children and adolescents. As in adults, abdominal obesity is the most relevant risk factor of the metabolic syndrome.

Unlabelled: We analyzed the relationship between serum leptin levels and body mass index in children treated for acute lymphoblastic leukemia during and after the maintenance therapy.

Material and methods: We studied 99 survivors (62 boys) in mean age 12.65 years +/- who have been treated for ALL according to the Polish Paediatric Leukemia and Lymphoma Study Group. During the maintenance therapy there were n=34 patients (23 boys) and after treatment n=75 (39 boys). 16 patients received cranial irradiation (12 Gy). We calculated body mass index (BMI) using the formula weight/(height)2 (kg/m2). Leptin levels were measured with the RIA method. The results were expressed as SDS.

Results: 1. No difference was found in leptin SDS and BMI SDS from zero -- in the whole study group, similar in boys and girls. The leptin/BMI ratio was significantly higher in girls than in boys. 2. No differences were observed in the group during and after therapy in values of BMI SDS and leptin SDS. We found higher leptin SDS in boys during (4.86+/-1.01) and after puberty (1.53+/-0.75). We found higher leptin SDS in boys than in girls during (1.6+/-1.1 vs. 0.6+/-0.6) and after therapy. 3. In boys and girls diagnosed before puberty leptin SDS were higher in boys (1.7+/-1.3 vs. 0.2+/-1.7). The leptin/BMI ratio was significantly higher in girls than in boys diagnosed before puberty. 4. We found correlations between leptin levels and BMI in the whole group as well as in boys and girls analysed according to puberty and time of treatment. 5. No differences were observed between patients with and without cranial irradiation.

Conclusions: Anti-neoplastic treatment for acute lymphoblastic leukemia and age of disease did not influence body mass index and leptin level in the examined population.

Background: Insulin resistance--a key element of the metabolic syndrome--is observed in children with simple obesity. Adipose tissue is producing bioactive substances called adipocytokines. Some of them may play a role in the development of insulin resistance.

Aim of the study: Estimation of the frequency of insulin resistance and its correlation with leptin, adiponectin and resistin levels in children with simple obesity.

Material and methods: The 53 children (BMI>97 centile), mean age 13.57 years. Mean BMI was +4.04 SDS. Oral glucose tolerance test (OGTT) was performed. Insulin levels at 0' < or = 15 microIU/mL and/or insulin peak during OGTT < or = 150 microIU/mL and/or peak at 120' < or =75 microIU/mL were established as normal values. Homa ratio was calculated. Patients were divided into groups depending on the presence or absence of hyperinsulinemia / insulin resistance. Concentrations of adiponectin, leptin, resistin were measured.

Results: In 13.23 % children various types of hyperglycemia were diagnosed and hyperinsulinemia in OGTT was noted in 83.02 %. Severe insulin resistance (HOMA>3) was diagnosed in 71.82 %. In the hyperinsulinemia group higher glucose levels in OGTT were stated comparing to the non hyperinsulinemia group. In children with insulin resistance, higher BMI and SD BMI were observed. In this paper results of correlations of adipocytokines levels and anthropometric parameters or carbohydrates metabolism in children with / without insulin resistance are presented.

Conclusions: In children with severe insulin resistance adiponectin concentrations correlate negatively with glucose levels, there is a positive correlation of adiponectin and glucose and insulin in a group without severe insulin resistance. In patients with insulin resistance leptin concentrations correlate positively with the degree of obesity and insulin levels in OGTT.

Introductions: High levels of tumor necrosis factor (TNF-alpha)-cytokine produced by adipocytes, are involved in the development of obesity-related insulin resistance and metabolic syndrome. Therefore, we investigated whether the polymorphism of TNF-alpha-308 gene can predict the conversion from insulin resistance and obesity parameters in children with obesity. THE STUDY comprised 72 children with obesity simplex (9-18 y.o). The following anthropometric parameters: weight, height, BMI, SDS for BMI, WHR, sum of 3, 10 skinfolds and percentage of body fat by Slaughter's equation were calculated. In each child, after 12 hour overnight fast, glucose, insulin, leptin and lipids: triglycerides (Tg), cholesterol total (Chol-T), cholesterol HDL (Chol-HDL), cholesterol LDL (Chol-LDL) were measured. The oral glucose tolerance test was performed and HOMA-IR was calculated.

Results: Using the technique of PCR-RELP and SSCP, three variants of genotype TNF-alpha were obtained: G/G-68%, A/G-29%, A/A-3%. Statistical analysis of anthropometric, biochemical and leptin variables in groups G/G, vs. A/G +A/A, in boys and girls was performed. We did not find any significant differences between groups (G/G vs. A/G +A/A in all group, and between G/G girls, boys vs A/G +A/A girls, boys) Our data indicate that in smaller children studies, TNF-alpha polymorphism does not seem not to be associated with the degree of obesity and insulin resistance.

Introduction: Turner syndrome is a common chromosomal aberration. Among other features of that syndrome susceptibility to autoimmune diseases is well known. Most often there is Hashimoto's thyroiditis. Because of short stature the evaluation of thyroid volume according to chronological age seems to be not efficient. THE AIM of the study was to find an adequate method of thyroid volume evaluation in girls with Turner syndrome using standards for healthy children.

Material and methods: 54 girls with Turner syndrome were examined, mean age was 11 years and 9 months. Physical and ultrasonographic examination of thyroid gland was performed. The hormonal state and presence of antithyroid autoantibodies were evaluated. The thyroid volume was evaluated according to normal range (by Delange) for chronological and height age of each girl.

Results: According to chronological age the thyroid volume was in normal range, but according to height age in 3 patients the thyroid volume was over 97 percentile. They were the same girls in whom we detected goiter according to physical examination.

Conclusions: We suggest that proposed method gives the possibility of an accurate evaluation of thyroid volume in patients with Turner syndrome and in other patients with growth deficiency.

Unlabelled: Etiology and the course of diabetes mellitus type 1 diagnosed in infancy remains the subject of intensive investigations, nonetheless the diagnosis of hyperglycemia in this period obliges prompt start of the insulin therapy. The treatment of newborns and infants is challenging because of the necessity to use very low doses of insulin.

Case report: A boy was transferred from the neonatal ward on the 8th day of life with the diagnosis of diabetes mellitus. He was born of young healthy parents (mother: 27, father: 30 years old), with no family history of diabetes or other autoimmune disorders. The course of the pregnancy remained normal until 37th week of gestation when due to alterations in the fetal heart rate the cesarean section was done. In spite of precise i.v. insulin therapy fluctuations in glucose levels persisted. Additionally the achievement of the metabolic compliance was complicated by generalized infections caused by hospital pathogens that required broad-spectrum i. v. antibiotic therapy. At the age of 7 weeks personal insulin pump Pradigm 712 for the delivery of NovoRapid insulin was introduced. Already on the 3rd day of such treatment, the daily insulin dose could be decreased from 0.7 unit/kg/24 hrs down to 0.5 unit/kg/24 hrs, and glucose levels normalized. In the course of this treatment the patient weighed 6300 g at the age of 6 months, and his psychomotoric development reached the milestones adequate for the age. The biochemical examinations revealed C-peptide levels both fasting and after glucagon stimulation <0.5 ng/ml. Additionally, the analyzed immunologic markers of type 1 diabetes are negative. The genetic testing for Kir6.2 mutation gave negative results.

Conclusions: The presented case shows that the insulin therapy carried out via personal insulin pump is the possible and effective treatment in children diagnosed with diabetes mellitus during infancy.

Background: Because of many disadvantages of growth hormone (GH) stimulation tests the diagnosis of growth hormone deficiency (GHD) is still problematic for the clinician. THE AIM of the study was the estimation of diagnostic usefulness of the GH provocation tests and basal IGF-I concentration measurement in the diagnosis of GHD.

Material and methods: The study group consisted of 180 children with short stature diagnosed in the Department of Pediatric Endocrinology and Diabetes in the years 1998-2003. Pharmacological stimulation test with insulin, clonidine, glucagon and L-dopa were used. GH concentration in physiological test after the onset of sleep was also measured. In 60 patients IGF-I concentration was estimated. For statistical analysis Spearman test was used.

Results: The highest mean GH concentrations (19.18 microl U/ml) and GH peaks (26.39 microl U/ml) were observed in sleep test. IGF-I concentration was correlated with GH concentration levels in physiological test after onset of sleep (r=0.4; p<0.05).

Conclusions: Sleep is the strongest stimulatory agent for GH secretion. The estimation of GH secretion after the onset of sleep can be used as a screening test in the diagnosis of GHD. Because of many diagnostic problems in estimation of pituitary function the auxologic parameters should be considered as the most important part in the diagnostic work-up of children with short stature due to GHD.

Objective: Recent studies have shown a correlation between advanced diabetic retinopathy and late stages of atherosclerosis. There are no findings on a possible relation between diabetic retinopathy and diseases of the cardiovascular system at their earliest stage in young people with diabetes type 1. The purpose of the study was to analyze a correlation between diabetic retinopathy and early atherosclerotic changes in adolescents with type 1 diabetes.

Research design and methods: The study included 28 adolescents aged 17.6+/-1.4 years suffering from type 1 diabetes mellitus for 7.9+/-3.1 years, the mean age of the disease onset - 9.5+/-3.7 years, a mean level of HbA1c - 8.6+/-1.9%. Eight patients with developing simple retinopathy, were separated from the whole group of young people. First control group consisted of the remaining patients with type 1 diabetes chosen with regard to age and sex, without disease complications. Second control group consisted of 11 healthy young people. The function of endothelium by measuring the brachial artery dilatation -- FMD and the intima-media complex thickness of the common carotid arteries were evaluated ultrasonographically.

Results: Young people with retinopathy had higher systolic pressure: 133+/-19 mmHg in comparison with patients without complications: 117+/-14 mmHg (p<0.05) and healthy people: 115+/-8 mmHg (p<0.05). All patients with diabetes showed significantly lower FMD (7.6+/-5.1%, p<0.05). In the group with retinopathy, FMD equaled 7.8+/-4.1% (p=0.04) and in the group without retinopathy - 7.6+/-5.5% (p<0.05) in comparison with 12.1+/-5.1% in healthy volunteers. Significantly higher IMT was found in all patients with diabetes in comparison with healthy young people: 0.49+/-0.06 vs. 0.42+/-0.03 mm (p<0.001). Patients with retinopathy had a significantly higher value of IMT in comparison not only with controls but also with patients without complications: 0.56+/-0.06 vs. 0.47+/-0.03 mm (p<0.001).

Conclusions: 1. Young people with type 1 diabetes had a significantly impaired function of endothelium and higher IMT in comparison with healthy young people. 2. Adolescents with retinopathy were characterized by significantly higher values of systolic arterial blood pressure when compared to patients without complications 3. Higher IMT was found in patients with diabetic retinopathy in comparison with patients without complications, which may suggest that macrovascular changes are more advanced in case of complications than in patients without retinopathy.