Volume 82 | ESPE2014 | Next issue

ESPE 2014

Dublin, Ireland
18 Sep 2014 - 20 Sep 2014

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Dublin, Ireland; 18-20 September 2014. Further information

Poster Category 3

Growth (2)

hrp0082p3-d3-836 | Growth (2) | ESPE2014

What is the Profile of Gigantism: Seven Observations

Achir Samia , Semrouni Mourad

Background: Gigantism is a condition characterized by excessive growth and height significantly above average; it is usually caused by a tumor on the pituitary gland. In some cases the condition can be passed on genetically through a mutated gene.Objective and hypotheses: Describe clinical, hormonal, and morphological profile of seven children with gigantism. Mean age=14 years (9–16), age of onset of symptoms 12 years. Tall stature (50%), and brain ...

hrp0082p3-d3-837 | Growth (2) | ESPE2014

GH Treatment in Dent’s Disease: a Case Report

Falcone Simona , Guidoni Elisa , Municchi Giovanna , Cioni Maddalena

Background: Dent’s disease is an X-linked recessive proximal tubulopathy characterized by low molecular weight proteinuria, hypercalciuria, nephrocalcinosis, nephrolithiasis, and progressive renal failure. Growth retardation, due to resistance to GH action is a feature commonly associated with Dent’s disease.Case report: We describe a boy diagnosed with Dent disease, treated with recombinant human GH. He was initially referred at 6 years of age...

hrp0082p3-d3-838 | Growth (2) | ESPE2014

Analysis of the Effectiveness of Treatment with GH in a Tertiary Hospital in the Last 30 Years

Rojo Jaime Cruz , Lorenzo Lucia Garzon , Gomez Ma Elena Gallego , Pozo Jaime Sanchez del

Objective: To analyze the efficacy of GH treatment in pediatric patients in a hospital from 1982 to 2013.Material and methods: Retrospective study whose population are patients who have been or are being treated with GH in a Tertiary Hospital. The following data were collected: indication of treatment, years of treatment, genetic target height, height at start and the end of treatment, with their corresponding S.D.. A simple linear regress...

hrp0082p3-d3-839 | Growth (2) | ESPE2014

A Rare Syndrome Benefits from GH Therapy: Hypotonia–Cystinuria Syndrome

Akin Onur , Hacihamdio[gbreve]lu Bulent , Ye[scedil]ilkaya Ediz

Background: Hypotonia–Cystinuria syndrome (HCS), characterized by hypotonia at birth, poor feeding and growth retardation is an autosomal recessive disorder caused by homozygous microdeletions of PREPL and SCL3A genes. Increase in the urinary amino acids including cystine, lysine, arginine, and ornithine is the main laboratory finding.Objective and hypotheses: HCS was described in 17 patients so far and previous reports with favorable responses to G...

hrp0082p3-d3-840 | Growth (2) | ESPE2014

Changes in BMI in GHD and SGA Children in the First Year of Treatment

Llano Juan-Pablo , Picon Teresa Ortiz , Llano Mauricio

Background: The effect of GH has been classically described as anabolic which should lead to changes in body composition in children during treatment. Likewise, GHD typically occurs in children with short stature and increased BMI.Objective and hypotheses: The aim of this study was to assess changes in BMI in children before and after initiation of treatment with GH in patients with GH deficit in small for gestational age and year. Starting variables as ...

hrp0082p3-d3-841 | Growth (2) | ESPE2014

Study of the Effect of GH Treatment on Growth in Patients Affected by the Inherited Metabolic Disease Methylmalonic Acidemia

Eyskens Francois

Background: Methylmalonic acidemia (MMA) is an inborn error of metabolism affecting the catabolism of essential amino acids: valine, isoleucine, methionine, and threonine. A great problem in these children is that of a growth abnormality with progressive slowing of the statural growth rate, in contrast with a normal or even accelerated weight gain. These abnormalities are accompanied by bone demineralization and muscle hypotonia.Objective and hypotheses:...

hrp0082p3-d3-842 | Growth (2) | ESPE2014

Pubertal Development of Isolated GH Deficient Patients

Fedala Soumeya , Haddam Mahdi el Mahdi , Chentli Farida

Background: Hormone deficiency no or late treated causes delayed puberty and reduced final height.Objective and hypotheses: Assess the progress of puberty in isolated GH deficient (GHD) patients.Method: 34 patients with GHD in puberty were followed in endocrinology. The average age at diagnosis of GH deficiency was 8±2.4 (7–19) in girls and 9±1.2 (8–18) in boys. The majority of patients received an irregular GH ...

hrp0082p3-d3-843 | Growth (2) | ESPE2014

Two Years of GH Therapy in Children with Growth Deficiency

Bessahraoui Mimouna , Niar Sakina , Naceur Malika , Bouziane-Nedjadi Karim

Background: GH therapy improves height outcome in children with GH deficiency (GHD). Height velocity (HV) is maximum in the first year of treatment. Early diagnosis and therapy initiation optimize growth outcomes.Objective and hypotheses: Of this study was to evaluate growth during the first 2 years of GH treatment in 33 GHD children and evaluated the height velocity.Method: The study enrolled 54 children (29 boys, and 25 girls) wi...

hrp0082p3-d3-844 | Growth (2) | ESPE2014

WHO Growth Charts Replacing National Reference Data: Their Influence on Screening for Over- or Underweight and of Growth Disorders

Eisenegger Celine , Allenspach-Moser Sarina , Lallemand Dagmar

Background: In 2011, nationwide growth charts were introduced, replacing different regional references (ZLS, Prader 1989; Sempé 1979 and, for BMI only, Kromeyer 2001).Objective and hypotheses: The aim was to examine the influence of new growth charts (WHO 2010) on the prevalence of nutrition- and growth-related disorders compared the ‘old’ references.Method: 6007 anonymised weight and height datasets of children meas...

hrp0082p3-d3-845 | Growth (2) | ESPE2014

Skeletal Maturity of Radius, Ulna, and Short Bones in TW3 Method for Children in Korea

Lee Jieun , Kim Jaesuk , Cho Jurae

Purpose: The Tanner-Whitehouse (TW) system is the method of choice for skeletal maturity assessment in both clinical practice and auxological research (Tanner et al. 1983), and it has been used to estimate skeletal maturity in groups of children from all over the world. This study aimed on analyzing the characteristic of the RUS maturity of Korea children by evaluating RUS maturity of Korea children using the TW3 method, compared with children in China and Japan<stron...

hrp0082p3-d3-846 | Growth (2) | ESPE2014

Design and Recruitment of a Longitudinal Cohort Study of Growth and Puberty in Russian Boys

Sergeyev Oleg , Lam Thuy , Williams Paige L , Burns Jane S , Korrick Susan A , Hauser Russ , Revich Boris , Dikov Yury , Sergeyeva Lyubov , Lee Mary M

Introduction: There are few longitudinal male cohort studies with serial assessments of growth and puberty.Objective: To describe the design and implementation of a longitudinal cohort study of Russian boys evaluated annually for growth, development and puberty.Design/methods: We assembled a multi-disciplinary team of U.S. and Russian researchers to design and conduct a longitudinal boys’ cohort study in Chapaevsk, Russia with...

hrp0082p3-d3-847 | Growth (2) | ESPE2014

Multiple Endocrinopathies in a Case with H Syndrome due to a Novel slc29a3 Mutation

Mutlu Gul Yesiltepe , Kirmizibekmez Heves , Ozsu Elif , Zlotogorski Abraham , Hatun Sukru

Background: H syndrome (OMIM #602783) is an autosomal recessive syndrome resulted from mutations in the SLC29A3 gene, encoding hENT3 protein. Characteristic findings are cutaneous hyperpigmentation, hypertrichosis, hepatosplenomegaly, hypogonadism, hyperglycemia/diabetes mellitus, cardiac anomalies, hallux valgus and short stature. Herein we report a girl with multiple endocrinopathies due to H syndrome.Case: Ten year and 5 month old girl was referred be...

hrp0082p3-d3-848 | Growth (2) | ESPE2014

Severe Short Stature with Features of Achondroplasia, Later Diagnosed as Panhypopituitarism: a Case Report

Matei Cristina , Karam Maria , Peters Catherine

Background: Severe short stature is considered when height falls below −4 SDS. When infants with extreme short stature are evaluated, often multidisciplinary input is required, before a formal diagnosis is met.Objective and hypotheses: We want to learn from delayed diagnosis of child with panhypopituitarism and review subsequent difficulties in starting GH treatment.Method: Retrospective review of notes, investigations and pa...