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Pediatric Endocrinology, Diabetes, and Metabolism

Joanna Zarębska, Katarzyna Musioł, Paulina Brożek, Adriana Zybała, Patrycja Szerląg, Grażyna Sobol-Milejska, Przemysława Jarosz-Chobot
Idiopathic thrombocytopenic purpura (ITP) is an acquired autoimmune disease, caused by antibodies against platelet glycoproteins, which provoke platelet destruction and inhibit platelet production in bone marrow. Type 1 Diabetes Mellitus (T1DM) is an acquired autoimmune disease in witch beta cells are destroyed by autoantibodies. Patient with T1DM since the age of 6, was treated by intensive functional insulin therapy by insulin pump. At the age of 14,5 he was also diagnosed with ITP. Due to the short effect of immunoglobulin therapy, glucocorticoids were introduced...
2017: Pediatric Endocrinology, Diabetes, and Metabolism
Karolina Klenczar, Grażyna Deja, Barbara Kalina-Faska, Przemysława Jarosz-Chobot
Diabetes type 1(T1D) is the most frequent form of diabetes in children and young people, which essence is autoimmune destruction of pancreatic B cells islet. Co-occurrence of other autoimmune diseases is observed in children with T1D, the most often are: Hashimoto disease or coeliac disease. We report the case of the patient, who presents coincidence of T1D with other rare autoimmune diseases such as: Graves - Basedow disease, myasthenia gravis, vitiligo and IgA deficiency. All mentioned diseases significantly complicated both endocrine and diabetic treatment of our patient and they negatively contributed her quality of life...
2017: Pediatric Endocrinology, Diabetes, and Metabolism
Aleksandra Drewa, Katarzyna Zorena
In the 21st century the occurrence of overweight and obesity in children and adolescents reaches the size of a global epidemic. It is recommended to promote a healthy lifestyle and, above all, to educate children, adolescents and adults about the dangerous consequences of being overweight or obese. It is crucial for prevention programs to comprise educational activities so that the participants are encouraged to change their lifestyle for better. However, it must be borne in mind that the overall effectiveness of such activities is determined by the commitment of both the obese child and the environment - the school or kindergarten and the media...
2017: Pediatric Endocrinology, Diabetes, and Metabolism
Anna Dittfeld, Katarzyna Gwizdek, Anna Brończyk-Puzoń
Endocrinology is a branch of medicine dealing with the functioning of endocrine glands. Theain role of the endocrine glands is to control various functions and to adopt internal environment to changes in the external environment. Glands manage to do this by regulating the metabolism of target cells by hormones. One of the earliest connections with endocrinology is found in theHippocratic humoral hypothesis dating about 400 years BC. The author of the first endocrinological observations was Aristotle, who noticed changes in behavior and appearance in castrated roosters...
2017: Pediatric Endocrinology, Diabetes, and Metabolism
Arkadiusz Michalak, Marta Koptas, Aleksandra Świercz, Krystyna Wyka, Anna Hogendorf, Agnieszka Szadkowska, Wojciech Młynarski, Wojciech Fendler
INTRODUCTION: Literature reports link psoriasis with insulin resistance characteristic for type 2 diabetes. However, this condition may also affect the clinical course of type 1 diabetes (T1D). AIM: To investigate whether children with type 1 diabetes mellitus (T1D) and psoriasis have a different course of diabetes. METHODS: We evaluated patients diagnosed with T1D in the years 2002-2011 for the presence of psoriasis and matched them 1:10 with T1D-only patients by sex and duration of diabetes using propensity score...
2017: Pediatric Endocrinology, Diabetes, and Metabolism
Agnieszka Kalinowska, Barbara Orlińska, Mateusz Panasiuk, Milena Jamiołkowska, Aneta Zasim, Bożena Florys, Katarzyna Wojtkielewicz, Włodzimierz Łuczyński, Barbara Głowińska-Olszewska, Artur Bossowski
INTRODUCTION: Type 1 diabetes mellitus is a disease caused by the autoimmune destruction of pancreatic beta-cells. It was previously believed that the loss of the endocrine function of the pancreas is total and inevitable. With the rise of new knowledge and new methods allowing to reliably measure c-peptide in the low plasma concentration range, we have learned otherwise. Some residual function of the beta-cells can be present even after decades of the course of the disease. The aim of the study was to evaluate the c-peptide level with routine laboratory and ultrasensitive methods in children with long-standing type 1 diabetes in relation to clinical characteristics...
2017: Pediatric Endocrinology, Diabetes, and Metabolism
Christine Frithioff-Bøjsøe, Cæcilie Trier, Cilius Esmann Fonvig, Anne Nissen, Julie Tonsgaard Kloppenborg, Pernille Maria Mollerup, Poul Jannik Bjerrum, Oluf Pedersen, Torben Hansen, Jens-Christian Holm
INTRODUCTION: The accumulation of components of the metabolic syndrome (MetS) is associated with a disturbed glucose metabolism in obese children. AIM OF STUDY: The aim of the present study was to evaluate the association between MetS and estimates of insulin sensitivity and β-cell function obtained from oral glucose tolerance test (OGTT)-derived indices in lean and obese children. MATERIAL AND METHODS: A 2-hour OGTT was administered in 83 children aged 7-17 years...
2017: Pediatric Endocrinology, Diabetes, and Metabolism
Milena Jamiołkowska, Artur Bossowski
Polyglandular autoimmune syndromes (PAS) is a group of heterogenous conditions characterized by the association of at least two organ-specific autoimmune disorders, concerning both endocrine and non-endocrine organs. Type III is defined as the combination of autoimmune thyroid disease and other autoimmune condition (other than Addison's disease) and is divided into four subtypes. We describe a teenage female patient - with the family history of autoimmune diseases, who has simultaneously developed the symptoms of autoimmune thyroid disease with the clinical picture of hyperthyroidism and myasthenia gravis at the age of fifteen...
2017: Pediatric Endocrinology, Diabetes, and Metabolism
Adrianna Zajkowska, Marta Rydzewska, Katarzyna Wojtkielewicz, Janusz Pomaski, Tomasz Romer, Artur Bossowski
INTRODUCTION: Inactivating mutations in the enzyme hexose-6-phosphate dehydrogenase (H6PDH), the enzyme responsible for NADPH generation playing critical role in 11-hydroxysteroid dehydrogenase type 1 (11b-HSD1) activity, cause apparent cortisone reductase deficiency (ACRD). It leads to increased metabolic clearance rate of cortisol due to a defect in cortisone to cortisol conversion by 11b-HSD1. We want to analyse the process of the disease, efficacy of long-lasting treatment with glucocorticoids throughout childhood and adolescence in only male patient with ACRD...
2017: Pediatric Endocrinology, Diabetes, and Metabolism
Aleksandra Rojek, Karolina Kwasiuk, Monika Obara-Moszyńska, Zofia Kolesińska, Marek Niedziela
Turner syndrome (TS) is an inherited genetic disorder caused by numerical and/or structural chromosome X aberrations occurring at a frequency of 1:1200-1:2500 live-born girls. The most common karyotype is X chromosome monosomy (45,X) (approximately 50-60% of cases). Approximately 5-6% of patients may have abnormal Y chromosome or mosaicism characterized by the coexistence of 45,X cell line with cell line in which all or part of chromosome Y is present. In patients with TS who have all or fragmented genetic material from chromosome Y there is a substantial risk of cancerous lesions in these dysgenetic gonads...
2017: Pediatric Endocrinology, Diabetes, and Metabolism
Michalina Horochowska, Elżbieta Kołeczek, Zygmunt Zdrojewicz, Jacek Jagiełło, Karolina Pawlus
Topinambour (Helianthus tuberosus L.), also known as the Jerusalem artichoke is a plant, which origins from North America. In XVII century it was brought to the Europe. For ages it was cultivated due to edible tuber and its healing properties.The aim of the article is to present medical properties and application of topinmbour in patient's diet. Many studies were performed on animals to specify medical properties of topinambour. Results show, that topinambour lowers plasma glucose, total cholesterol and triglyceride levels...
2017: Pediatric Endocrinology, Diabetes, and Metabolism
Katarzyna Piechowiak, Beata Zduńczyk, Agnieszka Szypowska
INTRODUCTION: The way parents manage diabetes of their small children and environmental influence are crucial for maintaining glycemic control. The aim of the study was to assess environmental factors affecting metabolic control of young children with T1D treated with insulin pumps. MATERIAL AND METHODS: Parents of children with T1D under the age of 10 years completed: General Self-Efficacy Scale, Parental Diabetes Quality of Life Questionnaire, Beck Depression Inventory and a questionnaire on socioeconomic factors...
2017: Pediatric Endocrinology, Diabetes, and Metabolism
Anna Gryko, Barbara Głowińska-Olszewska, Katarzyna Płudowska, W Henry Smithson, Anna Owłasiuk, Beata Żelazowska-Rutkowska, Katarzyna Wojtkielewicz, Robert Milewski, Sławomir Chlabicz
INTRODUCTION: In the recent years, alterations in the carbohydrate metabolism, including insulin resistance, are considered as risk factors in the development of hypertension and its complications in young age. Hypertension is associated with significant cardiovascular morbidity and mortality. The onset of pathology responsible for the development of hypertension, as well as levels of biomarkers specific for early stages of atherosclerosis are poorly understood. AIM: To compare a group of children whose parents have a history of hypertension (study group) with a group of children with normotensive parents (reference group), with consideration of typical risk factors for atherosclerosis, parameters of lipid and carbohydrate metabolism, anthropometric data and new biomarkers of early cardiovascular disease (hsCRP, adiponectin, sICAM-1)...
2017: Pediatric Endocrinology, Diabetes, and Metabolism
Jacqueline P Velasco Md, Joshua Fogel PhD, Robert L Levine Md PhD, Peter Ciminera Md, David Fagan Md, Renee Bargman Md
INTRODUCTION: Diabetic ketoacidosis (DKA) is a life-threatening complication of diabetes mellitus (DM) that requires appropriate treatment with insulin and intravenous fluids. Both one-bag and two-bag systems of fluid management are used to treat pediatric diabetic ketoacidosis. AIM OF THE STUDY: We compare the one-bag and two-bag systems of fluid managementwith regard to incidence of hypoglycemia, serum bicarbonate correction, pH correction and discharge from the pediatric intensive care unit (PICU)...
2017: Pediatric Endocrinology, Diabetes, and Metabolism
Karolina Orchel-Szastak, Katarzyna Ptak, Katarzyna Hrnciar, Bożena Pilch, Urszula Kania, Mirosław Bik-Multanowski
We present an unexpected outcome of 10 years of enzyme replacement therapy of a boy with mucopolysaccharidosis type II. Due to a positive familiar history (severe disease course in a sibling) the diagnosis was established in the first month of life. Treatment with Elaprase was introduced two months later. Since then normal physical and mental development is observed. The patient presents only relatively large head circumference (+2.1 SD) and slight decrease of joints mobility. In our opinion, early introduction of enzyme replecement therapy could attenuate the disease course...
2017: Pediatric Endocrinology, Diabetes, and Metabolism
Halis Kaan Akturk Md, Seda Yasa MSc
Wolfram syndrome (WFS) is a rare autosomal recessive disease with non-autoimmune childhood onset insulin dependent diabetes and optic atrophy. WFS type 2 (WFS2) differs from WFS type 1 (WFS1) with upper intestinal ulcers, bleeding tendency and the lack ofdiabetes insipidus. Li-fespan is short due to related comorbidities. Only a few familieshave been reported with this syndrome with the CISD2 mutation. Here we report two siblings with a clinical diagnosis of WFS2, previously misdiagnosed with type 1 diabetes mellitus and diabetic retinopathy-related blindness...
2017: Pediatric Endocrinology, Diabetes, and Metabolism
Agata Krasińska, Bogda Skowrońska
Prader-Willi Syndrome is a genetic condition caused by an abnormality of chromosome 15, mostly resulting from a deletion.The prevalence of syndrome in Europe has been reported between 1 in 8,000 to 1 in 45,000 births. Characteristic features of the syndrome include hypotonia, short stature, psychomotor development delay, hypogonadism and progressive, life-threatening obesity. Treatment of Prader-Willi Syndrome consists of intensive rehabilitation, psychologicalcare, speech therapy and also, if patient is fulfilling appropriate criteria, growth hormone treatment...
2017: Pediatric Endocrinology, Diabetes, and Metabolism
Zygmunt Zdrojewicz, Ewa Popowicz, Marta Szyca, Tomasz Michalik, Bartłomiej Śmieszniak
Recent studies have reported a significant increase in the incidence of type 2 diabetes in the past 30 years. They also predict that this trend will continue. This is related to a change in lifestyle, which results in a parallel increase in the incidence of overweight and obesity. However, symptoms of the metabolic syndrome, so far closely related to abdominal obesity, are relatively common among people with normal body mass index (BMI) and waist circumference. The aim of the study is to present the TOFI phenotype (thin outside, fat inside) as an important factor in pathogenesis of type 2 diabetes...
2017: Pediatric Endocrinology, Diabetes, and Metabolism
Honorata Kołodziejczyk, Małgorzata Wajda-Cuszlag, Anna Świercz, Mieczysław Szalecki
INTRODUCTION: Disorders of somatic development in children and adolescents with type 1 diabetes can lead to unstable course of the disease and the difficulties in obtaining good metabolic control. AIM: Evaluation of somatic development in children and adolescents with type 1 diabetes in different age categories. MATERIAL AND METHODS: Agroup of 97 girls and 90 boys with type 1 diabetes was examined. Children were divided into three age groups: girls with mean age of 9...
2017: Pediatric Endocrinology, Diabetes, and Metabolism
Joanna Połubok, Anna Malczewska, Małgorzata Rąpała, Jerzy Szymocha, Marta Kozicka, Katarzyna Dubieńska, Monika Duczek, Bernarda Kazanowska, Ewa Barg
INTRODUCTION: Children with a neoplastic disease are highly susceptible to malnutrition. The main objective of the study was to assess the frequency of undernourishment and obesity at the time of the diagnosis of the neoplastic disease at children. MATERIALS AND METHODS: The study included 734 patients (58% males) at the age 1-20,25, with the diagnosis of neoplasm in the years 1986-2014. The patients were divided into groups depending on the type of the diagnosis: 1) ALL, 2) ANLL, 3) HL, 4) NHL, 5) NBL, 6) Wilms tumor, 7) mesenchymal malignant tumor...
2017: Pediatric Endocrinology, Diabetes, and Metabolism
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