hrp0092p3-54 | Diabetes and Insulin | ESPE2019

Relationship Between Chloride Infusion and Base Excess in Initial Treatment of Pediatric Diabetic Ketoacidosis

Sawano Kentaro , Nagamatsu Fusa , Shimura Kazuhiro , Abe Yuki , Izumita Yukie , Ogawa Yohei , Komatsu Nagisa , Takishima Shigeru , Nakamura Akie , Nyuzuki Hiromi , Yamaguchi Takeshi , Muroya Koji , Watanabe-Yamamoto Sayaka , Nagasaki Keisuke , Saitoh Akihiko , Hasegawa Yukihiro

Background: In initial treatment of DKA, volume expansion should begin with 0.9% saline to restore the peripheral circulation. The use of large amounts of chloride-rich/bicarbonate-free fluids may cause the rapid development of hyperchloremic metabolic acidosis, which is described in ISPAD Clinical Practice Consensus Guidelines 2018.The severity of DKA, defined by pH, HCO3- Base Excess (BE), is one of the factor...

hrp0092p3-65 | Diabetes and Insulin | ESPE2019

Insulin-Induced Oedema in a Child with Newly Diagnosed Diabetes Mellitus

Cakir Meltem Didem , Baysal Ozlem

Oedema is a care complication of insulin therapy. It affects mainly patients with newly diagnosed type 1 diabetes mellitus, poorly controlled diabetes mellitus or patients on large doses of insulin. There are only a few case report showing that it is an uncommon and probably an under-reported complication. Two mecanisms are known: the sodium and water retention and vasoactive mediators release. The oedema tends to develop shortly after initiation of insulin therapy and resolve...

hrp0089p2-p064 | Diabetes & Insulin P2 | ESPE2018

Early Diagnosis of Diabetes Type 2 in Children with Progeria Syndromes

Bald Martin , Timmermann Kirsten , Wadien Tanja , Krahling Eva , Holder Martin

Progeria syndromes are rare in children and include several diseases which lead to premature ageing already in children. Therefore, the pediatrician may be confronted with diseases which are normally seen only in persons with advanced age. We report about two children with progeria, in whom diabetes type 2 become manifest early and with a fulminant course in one patient. The first boy was known with Cockayne syndrome, which belongs to the progeria syndromes. He suffered from l...

hrp0089p2-p066 | Diabetes & Insulin P2 | ESPE2018

Prothrombin Gene 20210A Mutation Heterozygosity and MTHFR Gene C677T Mutation Homozygosity Detected in a Male Toddler Experiencing Femoral Venous Thrombosis During Diabetic Ketoacidosis

Kleisarchaki Angeliki N , Giza Styliani , Nikolaidou Olga , Mouzaki Konstantina , Kotanidou Eleni P , Litou Eleni , Rengina Tsinopoulou Vasiliki , Papadakis Emmanouil , Galli-Tsinopoulou Assimina

Introduction: Diabetic ketoacidosis (DKA) as an inflammatory state combined with the disruption of the normal coagulation cascade can lead patients to an increased risk of thrombosis. Especially, patients that are genetically susceptible to thrombosis could develop deep venous thrombosis (DVT) due to inflammation, dehydration, and hyperviscosity secondarily to DKA. It is noteworthy that children with DKA who underwent central venous catheter placement could develop DVT, especi...

hrp0089p2-p093 | Diabetes & Insulin P2 | ESPE2018

Acute Mononeuropathy in an 8-Year-Old-Girl with Newly Diagnosed Type 1 Diabetes

Giza Styliani , Litou Eleni , Kotanidou Eleni P , Koliatos Panagiotis , Kleisarchaki Angeliki N , Tzirtzipis Tasos , Tsinopoulou Vasiliki Rengina , Tihalas Athanassios , Evangeliou Athanassios , Galli-Tsinopoulou Assimina

Introduction: Neuropathy, as a complication of type 1 diabetes (T1D), is a heterogeneous group with chronic polyneuropathy being the most frequent form. Acute mononeuropathy is rare and its pathophysiology has not been elucidated.Purpose: To describe acute mononeuropathy during the course of severe ketoacidosis in an 8-year-old girl diagnosed with T1D.Case report: An 8-year old girl was admitted to the Emergency Department because ...

hrp0089p3-p104 | Diabetes & Insulin P3 | ESPE2018

The Frequency of Diabetic Ketoacidosis Hospitalization in Siberian Children and Adolescent

Osokina Irina

Introduction: Diabetic ketoacidosis is a frequent reason for hospital admission of children with newly diagnosed diabetes and the most frequent cause for hospitalization of children with poorly controlled diabetes.Aim: To evaluate the frequency of diabetic ketoacidosis (DKA) hospitalization for pediatric patients and resources for its decrease.Methods: Subjects included children <19 years who hospitalized with DKA in the pediat...

hrp0089p3-p116 | Diabetes &amp; Insulin P3 | ESPE2018

Monogenic Diabetes in 2 Years and 4 Months Old Girl: Is it DEND?

Rochmah Nur , Faizi Muhammad , Purwana Arie

Background: Monogenic forms of diabetes are still rare and not well understood. Their prevalence accounting for 1–4% of pediatric diabetes cases. Several genes encoding proteins important to β-cell function or regulation have been identified that lead to monogenic diabetes. However, awareness of these conditions may be lacking, and screening for them genetically is not routinely undertaken due to lack facility.Objective: To report a case diagno...

hrp0089p2-p368 | Sex Differentiation, Gonads and Gynaecology or Sex Endocrinology P2 | ESPE2018

A Case of Gonadal Dysgenesis Due to a Novel Homozygous Mutation in NR5A2 Gene

Denzer Friederike , Denzer Christian , Hornig Nadine , Holterhus Paul-Martin , Hiort Olaf , Wabitsch Martin

Background: Steroidogenic factor (SF1, NR5A2) regulates multiple genes known to be involved in gonadal development, adrenal development, steroidogenesis, and gonadotroph development. Heterozygous mutations in the NR5A1 gene have been described in association with mild to severe gonadal dysgenesis with or without adrenal failure. Homozygous mutations are rare and have also been described in association with gonadal dysgenesis with or without adrenal failure.<p class="abstex...

hrp0086rfc9.6 | Pathophysiology of Disorders of Insulin Secretion | ESPE2016

Emerging Pitfalls of Etiological Diagnosis of Diabetes in Children and Adolescents? Analysis of a French Cohort of 310 Recent-Onset Cases

Bismuth Elise , Plat Helene , Chevenne Didier , Chantelot Christine Bellane , Carel Jean-Claude , Tubiana-Rufi Nadia

Background: A diagnosis of diabetes in children used to mean type 1 diabetes (T1D) and lifelong insulin therapy. However, over the last decades the spectrum of diabetes has widened and even if auto immunity remains the most prevalent etiology, confounding factors and overlap with other causes of diabetes types sometimes make a revision of the initial diagnosis necessary.Objective and hypotheses: To classify diabetes etiologies in youth with diabetes usin...

hrp0086p2-p80 | Adrenal P2 | ESPE2016

Addisonian Crisis Due to Autoimmune Adrenalitis in a 14 Year Old Boy with a History of Stem cell Transplantation (HSCT)

Penger Theresa , Albrecht Andrea , Marx Michaela , Voelkl Thomas , Stachel Daniel , Metzler Markus , Doerr Helmuth G.

Background: Various endocrine complications are common after HSCT but primary adrenal insufficiency (Addison’s disease, AD) is absolutely rare. To the best of our knowledge, there is only one case of AD reported in a 9-year-old girl after HSCT and busulfan and cyclophosaphamide-based conditioning for myelodysplastic syndrome.Objective: We report on a 14-year-old boy from Albania who developed an Addisonian crisis 12.7 years after HSCT.<p class="...