Techniques in pediatric surgery: congenital hyperinsulinism.

abstract：BACKGROUND/AIMS:To evaluate effects of growth hormone (GH) treatment on behaviour and psychosocial characteristics in short-stature children. METHODS:99 referred prepubertal non-familiar short-stature children (32 GH deficiency; 67 idiopathic short stature) aged 3-11 years, randomized to fixed or individual GH doses a...

journal_title：Hormone research in paediatrics

authors： Chaplin JE,Kriström B,Jonsson B,Hägglöf B,Tuvemo T,Aronson AS,Dahlgren J,Albertsson-Wikland K

更新日期：2011-01-01 00:00:00

abstract：:The preproghrelin gene is responsible for generating ghrelin and obestatin, two gastric peptides with opposite effects on food intake. Obestatin suppresses food intake and digestive motility through interaction with GPR39 (GPCR). Ghrelin is supposed to be a link connecting metabolism and energy homeostasis with growth...

journal_title：Hormone research in paediatrics

authors： Bossowski A,Czarnocka B,Harasymczuk J,Moniuszko A,Bardadin K,Lyczkowska A,Hanusek K,Bossowska A

更新日期：2013-01-01 00:00:00

abstract：:The hypothalamic-pituitary-adrenal axis is susceptible to programming during fetal development and may be linked to risk of disease later in life. In a former prospective study the cohort was divided into those born appropriate for gestational age (AGA) or small for gestational age (SGA; birth weight <10 percentile). ...

journal_title：Hormone research in paediatrics

authors： Beck Jensen R,Vielwerth S,Larsen T,Hilsted L,Cohen A,Hougaard DM,Jensen LT,Greisen G,Juul A

更新日期：2011-01-01 00:00:00

abstract：BACKGROUND/AIMS:Temple syndrome is an imprinting disorder caused by maternal uniparental disomy of chromosome 14 (mat UPD14), paternal deletion of 14q32 or paternal hypomethylation of the intergenic differentially methylated region (MEG3/DLK1 IG-DMR). Patients with Temple syndrome have pre- and postnatal growth restric...

journal_title：Hormone research in paediatrics

authors： Brightman DS,Lokulo-Sodipe O,Searle BA,Mackay DJG,Davies JH,Temple IK,Dauber A

更新日期：2018-01-01 00:00:00

abstract：BACKGROUND/AIMS:Genetic variation in several candidate genes has been associated with short stature. Recently, a high-mobility group A2 (HMGA2) gene SNP has been robustly associated with height in the general population. Only few have attempted to study these genes in extremely tall stature. We therefore studied common...

journal_title：Hormone research in paediatrics

authors： Hendriks AE,Brown MR,Boot AM,Oostra BA,Drop SL,Parks JS

更新日期：2011-01-01 00:00:00

abstract：:Following the introduction of life-saving glucocorticoid replacement 60 years ago, congenital adrenal hyperplasia (CAH) has evolved from being perceived as a paediatric disorder to being recognized as a lifelong, chronic condition affecting patients of all age groups. Increasing evidence suggests that patients with CA...

journal_title：Hormone research in paediatrics

authors： Reisch N,Arlt W,Krone N

更新日期：2011-01-01 00:00:00

abstract：BACKGROUND:Diazoxide is the first-line treatment for pediatric hyperinsulinemic hypoglycemia (HI). This study aimed to elucidate the pharmacokinetics of diazoxide in children with HI. METHODS:We obtained 81 blood samples from 22 children with HI. Measured serum diazoxide concentrations were used for population pharmac...

journal_title：Hormone research in paediatrics

authors： Kizu R,Nishimura K,Sato R,Kosaki K,Tanaka T,Tanigawara Y,Hasegawa T

更新日期：2017-01-01 00:00:00

abstract：:The pathophysiology of type 1 diabetes mellitus (DM) involves the selective autoimmune destruction of the pancreatic beta-cells [Pihoker et al.: Diabetes 2005;54(suppl 2):S52-S61]. The onset of type 1 DM is characterised by hyperglycaemia. Islet cell antibody (ICA), anti-insulin, anti-glutamic acid decarboxylase and t...

journal_title：Hormone research in paediatrics

authors： Rafeullah N,Cackett N,Hussain K

更新日期：2012-01-01 00:00:00

abstract：BACKGROUND:Glycogen storage disease type 1 (GSD1) is a rare and genetically heterogeneous metabolic defect of gluconeogenesis due to mutations of either the G6PC gene (GSD1a) or the SLC37A4 gene (GSD1b). Osteopenia is a known complication of GSD1. OBJECTIVES:The aim of this study was to investigate the effects of poor...

journal_title：Hormone research in paediatrics

authors： Melis D,Pivonello R,Cozzolino M,Della Casa R,Balivo F,Del Puente A,Dionisi-Vici C,Cotugno G,Zuppaldi C,Rigoldi M,Parini R,Colao A,Andria G,Parenti G

更新日期：2014-01-01 00:00:00

abstract：BACKGROUND:Significant idiopathic bone age (BA) advancement is defined as BA >2 SD above the mean chronological age (CA) with no underlying etiology. BA advancement due to endocrinopathies is associated with early puberty and compromised adult height (AHt), necessitating treatment. The natural history of idiopathic BA ...

journal_title：Hormone research in paediatrics

authors： Lazar L,Lebenthal Y,Shalitin S,Phillip M

更新日期：2011-01-01 00:00:00

abstract：BACKGROUND/AIMS:To study serum testosterone and estradiol in healthy boys in relation to growth during puberty up to peak height velocity (PHV). METHODS:Growth velocity was analyzed through testosterone (n = 41) and 17β-estradiol (n = 37) 24-hour profiles in a dose-response model. Participants were 26 healthy boys adm...

journal_title：Hormone research in paediatrics

authors： Albin AK,Norjavaara E

更新日期：2013-01-01 00:00:00

abstract：BACKGROUND/AIMS:Laron syndrome (LS) is an autosomal recessive disease characterized by marked short stature and very low serum IGF-1 and IGFBP-3 levels. This study assessed the clinical and endocrine features alongside determining the growth hormone receptor gene (GHR) mutation in Saudi Arabian patients with LS in orde...

journal_title：Hormone research in paediatrics

authors： Al-Ashwal AA,Al-Sagheir A,Ramzan K,Al-Owain M,Allam R,Qari A,Al-Numair NS,Imtiaz F

更新日期：2017-01-01 00:00:00

abstract：AIM/BACKGROUND:Vitamin D supplementation during pregnancy is a well-accepted recommendation worldwide; however, the debate about the correct dose is ongoing. We aimed to compare daily doses of 600, 1,200, and 2,000 IU in this randomized, controlled study. METHODS:The study group consisted of 91 pregnant women aged 16-...

journal_title：Hormone research in paediatrics

authors： Yesiltepe Mutlu G,Ozsu E,Kalaca S,Yuksel A,Pehlevan Y,Cizmecioglu F,Hatun S

更新日期：2014-01-01 00:00:00

abstract：BACKGROUND/AIMS:Because the criteria for genetic screening of short children are unknown, we performed genetic analysis of 199 short children born small for gestational age (SGA) or with normal birth size (idiopathic short stature, ISS). METHODS:After selection with a modified scoring system for SHOX and a novel score...

journal_title：Hormone research in paediatrics

authors： Caliebe J,Broekman S,Boogaard M,Bosch CA,Ruivenkamp CA,Oostdijk W,Kant SG,Binder G,Ranke MB,Wit JM,Losekoot M

更新日期：2012-01-01 00:00:00

abstract：BACKGROUND:Aromatase inhibition has been proposed as a potential approach for growth enhancement in children with short stature, but detailed animal studies are lacking. AIM:To assess the effect and potential adverse effects of aromatase inhibition on growth in female rats. METHODS:Prepubertal Wistar rats received in...

journal_title：Hormone research in paediatrics

authors： van Gool SA,Wit JM,De Schutter T,De Clerck N,Postnov AA,Kremer Hovinga S,van Doorn J,Veiga SJ,Garcia-Segura LM,Karperien M

更新日期：2010-01-01 00:00:00

abstract：BACKGROUND:ACP5 deficiency is known to cause spondyloenchondrodysplasia (SPENCD), which is characterized by various autoimmune and neurological symptoms in addition to short stature. METHODS:Two siblings from a consanguineous Turkish family, a girl aged 13 years (P1) and a boy aged 8 years (P2), presented to their end...

journal_title：Hormone research in paediatrics

authors： de Bruin C,Orbak Z,Andrew M,Hwa V,Dauber A

更新日期：2016-01-01 00:00:00

abstract：BACKGROUND:Congenital hypothyroidism is a common congenital endocrine disorder prevailing all over the world. No nationwide screening exists for any sub-Saharan country. We present normative cord and capillary thyroid-stimulating hormone (TSH) values for healthy Nigerian newborns. SUBJECTS AND METHODS:A cross-sectiona...

journal_title：Hormone research in paediatrics

authors： Yarhere IE,Jaja T,Oduwole A,Ibekwe MU,Suwaid S,Alkali Y,Adeniran K,Fetuga B,Jarrett OO,Elusiyan JB,Adesiyun O,Idris HW,Blankenstein O,Akani NA

更新日期：2016-01-01 00:00:00

abstract：BACKGROUND:The steroidogenic acute regulatory protein (StAR) is essential for steroidogenesis by mediating cholesterol transfer into mitochondria. Inactivating StAR mutations cause lipoid congenital adrenal hyperplasia. OBJECTIVE AND METHODS:To identify causative mutations in a patient presenting with adrenal failure ...

journal_title：Hormone research in paediatrics

authors： Yüksel B,Kulle AE,Gürbüz F,Welzel M,Kotan D,Mengen E,Holterhus PM,Topaloğlu AK,Grötzinger J,Riepe FG

更新日期：2013-01-01 00:00:00

abstract：:3-M syndrome is an autosomal recessive primordial growth disorder characterised by severe postnatal growth restriction caused by mutations in CUL7, OBSL1 or CCDC8. Clinical characteristics include dysmorphic facial features and fleshy prominent heels with a variable degree of radiological abnormalities. CUL7 is a stru...

journal_title：Hormone research in paediatrics

authors： Hanson D,Murray PG,Black GC,Clayton PE

更新日期：2011-01-01 00:00:00

abstract：BACKGROUND:Over the past 15 years, the incidence of type 1 diabetes mellitus (T1DM) in Central and Eastern Europe has grown rapidly from an initially low rate. This growth cannot be attributed to genetic factors: it appears that changing environmental exposures are responsible, though no single environmental factor can...

journal_title：Hormone research in paediatrics

authors： Cinek O

更新日期：2011-01-01 00:00:00

abstract：AIMS:To compare adult heights of GH-treated and GH-untreated patients with Silver-Russell syndrome (SRS) who were epigenotyped. METHODS:This was a nonrandomized retrospective study with matched controls at a single center. Molecular analysis of 32 out of 37 GH-treated patients (16 females) revealed IGF2-H19 epimutatio...

journal_title：Hormone research in paediatrics

authors： Binder G,Liebl M,Woelfle J,Eggermann T,Blumenstock G,Schweizer R

更新日期：2013-01-01 00:00:00

abstract：BACKGROUND:Altered adrenocortical activity is one suggested mechanism relating small birth size with the metabolic syndrome in adulthood. Adrenal androgen concentrations are higher in children born small (SGA) than appropriate for gestational age (AGA). AIM:To compare adrenocortical hormonal activity between 20-year-o...

journal_title：Hormone research in paediatrics

authors： Todorova B,Salonen M,Jääskeläinen J,Tapio A,Jääskeläinen T,Palvimo J,Turpeinen U,Hämäläinen E,Räsänen M,Tenhola S,Voutilainen R

更新日期：2012-01-01 00:00:00

abstract：:Minipuberty describes the transient sex-specific activation of the hypothalamic-pituitary-gonadal (HPG) axis during the first 6 months of life in boys and during the first 2 years in girls. It leads to a rise of luteinizing hormone, follicle-stimulating hormone, estradiol, and testosterone. The existence of minipubert...

journal_title：Hormone research in paediatrics

authors： Becker M,Hesse V

更新日期：2020-01-01 00:00:00

abstract：BACKGROUND:The paradox of pituitary tumours is that persistent growth is so atypical. By definition, all pituitary microadenomas regain complete trophic stability after an initial period of deregulated growth. Unlike tumours in many other organ systems, concern about significant growth of macroadenoma remnants after de...

journal_title：Hormone research in paediatrics

authors： Levy A

更新日期：2011-01-01 00:00:00

abstract：:Disorders of adrenal steroidogenesis comprise autosomal recessive conditions affecting steroidogenic enzymes of the adrenal cortex. Those are located within the 3 major branches of the steroidogenic machinery involved in the production of mineralocorticoids, glucocorticoids, and androgens. This mini review describes t...

journal_title：Hormone research in paediatrics

authors： Baranowski ES,Arlt W,Idkowiak J

更新日期：2018-01-01 00:00:00

abstract：BACKGROUND:A UK study showed final height in Turner syndrome (TS) girls receiving growth hormone is affected by age at pubertal induction and oxandrolone (Ox). Using data from that study, we analysed the effect of timing of oral ethinylestradiol (EE2) and Ox on height velocity (HV), bone maturation and pubertal progres...

journal_title：Hormone research in paediatrics

authors： Perry RJ,Gault EJ,Paterson WF,Dunger DB,Donaldson MD

更新日期：2014-01-01 00:00:00

abstract：BACKGROUND:Treatment with radioiodine for Graves' disease regularly increases the level of antithyroid antibodies, and transplacental passage of stimulating thyrotropin receptor antibodies (TRAb) may cause fetal hyperthyroidism. CASE PRESENTATION:A 21-year-old woman with Graves' disease received radioiodine treatment ...

journal_title：Hormone research in paediatrics

authors： Bjørgaas MR,Farstad H,Christiansen SC,Blaas HG

更新日期：2013-01-01 00:00:00

abstract：:Feeding problems are frequent in infants with congenital hyperinsulinism (HI) and may be exacerbated by continuous enteral nutrition (EN) used to maintain euglycemia. Our center's HI team uses dextrose solution given continuously via gastric tube (intrasgastric dextrose, IGD) for infants not fully responsive to conven...

journal_title：Hormone research in paediatrics

authors： Vajravelu ME,Congdon M,Mitteer L,Koh J,Givler S,Shults J,De León DD

更新日期：2019-01-01 00:00:00

abstract：:Prenatal treatment of congenital adrenal hyperplasia with dexamethasone (DEX) has been in use since the mid-1980s and has proven effective at reducing virilization of external genitalia in affected girls. However, multiple experimental studies on animals and clinical studies on humans show that prenatal administration...

journal_title：Hormone research in paediatrics

authors： Lajic S,Karlsson L,Nordenström A

更新日期：2018-01-01 00:00:00

abstract：:The diagnosis of childhood-onset growth hormone (GH) deficiency (GHD) is not straightforward, requiring a comprehensive clinical, anthropometric, biochemical, endocrine, and neuroradiological assessment. Although pharmacological GH stimulation tests are still considered the gold standard for GHD diagnosis, they are bu...

journal_title：Hormone research in paediatrics

authors： Federico G,Cianfarani S

更新日期：2010-01-01 00:00:00