Previous issue | Volume 86 | ESPE2016 | Next issue

55th Annual ESPE

Paris, France
10 Sep 2016 - 12 Sep 2016

Card image cap
Paris, France: 10-12 September 2016 Further information

hrp0086p2-p641 | Growth P2 | ESPE2016

Hypochondroplasia (HC) Treatment with rGH: Actualization of Pilot Observations

Bougneres Pierre , Linglart Agnes

Background: In patients with HC due to N540K FGFR3 mutations, adult height ranges 138–155 cm (men) and 128–145 cm in women. We have previously reported that a mean 0.075 μg/k.d rGH dose could allow a gain of 1.9 S.D. of height over 6.1 year and could reduce body disproportion in 6 young patients (Journal of Pediatrics 2012).Objectives: To confirm these results and extend observation of rGH effects.<p class="abst...

hrp0086p2-p642 | Growth P2 | ESPE2016

The Influence of Growth Hormone Treatment on the Basal Metabolism in Prepubertal Children with Kabuki Syndrome

Remmel Robin , Schott Dina , Gerver Willem-Jan , Stumpel Constance

Background: The influence of growth hormone (GH) on the metabolism of prepubertal children with Kabuki syndrome (KS) was never investigated before. Kabuki syndrome (KS) is a rare syndrome, which is mainly characterized by mental retardation, short stature, specific facial features, obesity and hypotonia. This syndrome caused by a mutation in the KMT2D or KDM6A gene.Objective and hypotheses: In this prospective study we investigated the ...

hrp0086p2-p643 | Growth P2 | ESPE2016

An Analysis of the Safety of Childhood Growth Hormone (GH) Therapy: Data from the NordiNet® International Outcome Study (IOS)

Savendahl Lars , Rohrer Tilman R , Pournara Effie , Pedersen Birgitte Tonnes , Blankenstein Oliver

Background: NordiNet® IOS (NCT00960128), a non-interventional study, collects long-term effectiveness and safety data of GH (Norditropin®, Novo Nordisk) treatment in everyday clinical practice.Objective and hypotheses: Identify paediatric patients more likely to experience a second adverse event (AE).Method: Based on diagnosis at GH treatment start and associated risk for mortality, patients were cla...

hrp0086p2-p644 | Growth P2 | ESPE2016

Adverse Effects after Priming with Testosterone in Short Statured Boys before Growth Hormone Stimulation Test

Albrecht Andrea , Penger Theresa , Marx Michaela , Voelkl Thomas , Hirsch Karin , Doerr Helmuth G

Background: Current guidelines recommend the priming with low-dose testosterone in prepubertal boys prior to growth hormone stimulation tests. To our knowledge, only few adverse events after low-dose testosterone have been described so far.Objective and hypotheses: To assess possible side effects of testosterone priming.Patients: We studied 188 prepubertal boys aged between 10 and 15 years (mean ± S.D.: 11.4&#17...

hrp0086p2-p645 | Growth P2 | ESPE2016

Effect of One-Year Growth Hormone Therapy on Serum Levels of Ghrelin and Leptin in Children with Growth Hormone Deficiency and their Correlations with Cardiac Functions and Dimensions

Khalaf Randa , ElKholy Mohamed , Elsedfy Heba , Kotby Alyaa , Hamza Rasha , Youssef Omneya , Mahmoud Nermine

Background: Controversial data on ghrelin and leptin concentrations in patients with growth hormone deficiency (GHD) have been published. Little has addressed the correlation between Ghrelin and leptin with cardiac functions in patients with GHD.Aim: To investigate the effect of one year Growth hormone (GH) therapy on serum levels of ghrelin and leptin in children with GHD and to study their correlations with cardiac functions and dimensions in patients ...

hrp0086p2-p646 | Growth P2 | ESPE2016

Metabolic Parameters and Glucose Homeostasis in Childhood Onset Growth Hormone Deficiency at Time of Initial Evaluation and Retesting

Ahmid M , McMillan M , Ahmed S F , Shaikh M G

Background: It is well known that growth hormone (GH) brings about several effects, involving bone, body composition, lipids and glucose homeostasis. However, the complex interplay between these parameters is rather poorly studied in children with childhood-onset-GH deficiency (CO-GHD).Objective and hypotheses: To investigate lipids, adipokines (leptin- adiponectin- resistin) and glucose homeostasis and their relationship with bone and body composition i...

hrp0086p2-p647 | Growth P2 | ESPE2016

Final Adult Height (FAH) in Patients with PROR-1 Gene Mutations during GH Long-Term Therapy

Anna Gavrilova , Elena Nagaeva , Tatiana Shiryaeva , Valentina Petekova , Ivan Dedov

Background: PROP-1 gene mutations are responsible for most of the cases of multiple pituitary hormone deficiencies (MPHD).Objective and hypotheses: We performed to evaluate the final adult height (FAH) in a group of patients with a PROP-1 gene mutations. Twenty-five patients (11 males) with a PROP-1 gene mutation, not treated before, were recruited. All the patients had been treated with a fixed rhGH dose (0.033 mg/kg per day) for 10.5 years (7.0–11...

hrp0086p2-p648 | Growth P2 | ESPE2016

Congenital Hypopituitarism: Genotypic–Phenotypic–Neuroradiological Correlation

Pozzobon Gabriella Cinzia , Partenope Cristina , Gallo Dario , Damia Chiara , Lezzi Marilea , Pajno Roberta , Marinella Gemma , Osimani Sara , Weber Giovanna

Background: Congenital hypopituitarism is a rare cause of pituitary insufficiency (incidence: 12–42 new cases/million per year; prevalence: 300–455 cases/million). The aetiology remains largely unclear: the most frequently correlated genetic abnormalities are those involving transcription factors implicated in pituitary organogenesis. The phenotype and neuroradiological findings associated with the underlying genotype may be highly variable (from an isolated hypopitu...

hrp0086p2-p649 | Growth P2 | ESPE2016

A Case of Hypopituitarism in a Patient with Cantù Syndrome

Azzali Annachiara , La Spina Luisa , Gioe Daniela , Scalini Perla , Sandini Elena , Farri Martina , de Martino Maurizio , Stagi Stefano

Background: Cantù syndrome is a rare disorder characterized by congenital hypertrichosis, neonatal macrosomia, a distinct osteochondrodysplasia, and cardiomegaly. Other findings described are vascular abnormalities, pulmonary hypertension, generalized edema, mild learning disability and behavioral problems. Cantù syndrome is related to an heterozygous pathogenic variant in ABCC9 or KCNJ8, which can be inherited in an autosominal dominant manner or d...

hrp0086p2-p650 | Growth P2 | ESPE2016

Delaying Puberty with GnRHa does not Promote Adult Height in GH Treated Children Who Enters Puberty at Average Age

Hansson Felicia , Bang Peter

Background: Delaying puberty with GnRHa in an attempt to improve final height in GH treated children is relatively common in clinical practice. Such treatment is only supported by one retrospective study in which pubertal start was relatively early.Objective and hypotheses: To retrospectively assess adult height in children with short stature treated with GH and receiving or not receiving GnRHa. We hypothesized that delaying puberty with GnRHa improves a...

hrp0086p2-p651 | Growth P2 | ESPE2016

Estimation of FGF21 Concentration in Prepubertal Children with Growth Hormone Deficiency before and after 6 Months of Growth Hormone Treatment

Rudzka-Kocjan Agnieszka , Szalecki Mieczyslaw , Malinowska Anna

Background: Fibroblast growth factor 21 (FGF21) is a metabolic and growth regulator. The growth-promoting effect of GH in children with GH deficiency (GHD) depends on many factors. FGF21 concentration in and its interaction with growth deficiency and growth response to GH therapy in GHD was not examined.Objective and hypotheses: To estimate the FGF21 concentration and its correlation with degree of growth deficiency and growth response in non-obese, prep...

hrp0086p2-p652 | Growth P2 | ESPE2016

Abstract withdrawn...

hrp0086p2-p653 | Growth P2 | ESPE2016

Improving the ‘Gold Standard’: The Insulin Tolerance Test Revisited

Daskas Nikolaos , Barton John , Burren Christine , Crowne Elizabeth

Background: The optimal method to assess GH status remains controversial. GH provocation tests are used and the Insulin Tolerance Test (ITT) is regarded as the ‘gold standard’ to diagnose GH deficiency (GHD). The original selection of 0, 20, 30, 60, 90 and 120 min time points is still used in many protocols worldwide, but variations have evolved.Objective and hypotheses: Comparing standard ITT (StdITT) to a revised (RevITT) protocol.<p clas...

hrp0086p2-p654 | Growth P2 | ESPE2016

Adherence to Growth Hormone Therapy: Comparison of Electronic Auto-Injection to Non-Electronic Injection Devices

Trendafilow Mia , Hartmann Klaus

Background: Mean adherence (AD) rates in patients treated with recombinant human GH (r-hGH) using either the easypod™ or a non-electronic (NEL) device have recently been reported.Objective and hypotheses: To evaluate AD rates of r-hGH treatment under everyday conditions and to calculate the amount of r-hGH administered using the easypod™ or a NEL device.Method: Retrospective, observational, open-label, non-controlled stud...

hrp0086p2-p655 | Growth P2 | ESPE2016

Factors Influencing Peak GH Response During Insulin and Clonidine Stimulation Tests

Pascanu Ionela , Armean Iulia , Gherlan Iuliana , Procopiuc Camelia , Raluca Pop

Background: Several factors (bone age, BMI, target height, age) have been previously demonstrated to impact on GH response during stimulation tests, none of them proving to be of crucial importance.Objective and hypotheses: To analyze the influence of several anthropometric and laboratory parameters on peak GH response during insulin and clonidine stimulation tests.Method: Retrospective review of 265 patients who underwent GH stimu...

hrp0086p2-p656 | Growth P2 | ESPE2016

Improved Growth Outcomes with Jet Delivery of Growth Hormone in Children are Maintained Over Long-Term Treatment

Michaelidou Maria , Knight Alastair D , Whitten Sue , Bajaj Priti , Spoudeas Helen A

Background: We previously reported in a national cohort, that adherence to subcutaneous Growth Hormone (GH) treatment in children is better with jet delivery compared with needle devices (Spoudeas et al. Patient Prefer Adherence. 2014;8:1255–1263). We also separately reported that adherent children showed significantly improved height outcomes at one year.Objective and hypotheses: To examine the potential influence of adherence and demograp...

hrp0086p2-p657 | Growth P2 | ESPE2016

Septo-Optic Dysplasia and Excellent Growth with Low Growth Hormone Dose: Our Experience

Pozzobon Gabriella Cinzia , Marinella Gemma , Damia Chiara , Partenope Cristina , Gallo Dario , Pajno Roberta , Osimani Sara , Weber Giovanna

Background: Septo-optic dysplasia (SOD) is a congenital, rare anomaly (1:10 000) associated with optic nerve hypoplasia, midline brain abnormalities and pituitary hormone abnormalities. Genetic alterations associated are: HESX1, SOX2 and SOX3. SOD’s pattern of growth even without GH treatment has been reported to be often good.Objective and hypotheses: Evaluate SOD’s growth in patients followed in our Centre.Method: See F...

hrp0086p2-p658 | Growth P2 | ESPE2016

Early Diagnosis and Treatment of a Newborn with POU1F1 Mutation

Bouthors Therese , Antoniou Marie-Christina , Dwyer Andrew , Stoppa-Vaucher Sophie , Elowe-Gruau Eglantine , Phan-Hug Franziska , Pitteloud Nelly , Hauschild Michael

Background: POU1F1 encodes a pituitary-specific homeodomain transcription factor that is crucial for development and differentiation of anterior pituitary cell types. Mutations in this gene result in GH, TSH and prolactin (PRL) deficiencies.Objective and hypotheses: To describe a male newborn of a mother with known dominant p.R271W mutation in the POU1F1 gene.Methods: Case report with clinical follow up, endocrine investigations, n...

hrp0086p2-p659 | Growth P2 | ESPE2016

Influences of GHR-Exon 3 and -202 A/C IGFBP3 Polymorphisms on 1 Year Follow-Up Outcome of Growth Hormone Treatment in Korean Children with Growth Hormone Deficiency

Baek Joon Woo , Oh Yeon Joung , Kang Min Jae , Shim Young Suk , Hwang Il Tae , Yang Seung

Background: The GHR-exon3 and the -202 A/C IGFBP3 polymorphisms have been suggested to affect responses to recombinant human GH (rhGH) therapy in some individuals with short stature. This study aimed to assess the influences of the two polymorphisms on treatment outcomes in patients with GH deficiency (GHD).Method: In 72 (32 girls and 40 boys) children with confirmed diagnosis of GHD, genotyping and serial measurements of auxological and endocrinological...

hrp0086p2-p660 | Growth P2 | ESPE2016

Comparison between Effects of Oral Iron and Vitamin A with Oxandrolone upon Height and Puberty of Children with Constitutional Delay of Growth and Puberty

Pournasiri Zahra , Salehpour Shadab

Background: Constitutional delay of growth and puberty (CDGP) is one of the most common problems of pediatrics. It causes a lot of psychological and social disorders in families. Currently there are a variety of therapies including administration of testosterone enantate, oxandrolone and growth hormone.Objective: The aim of this study, which has been performed as the first in Iran and the second in the world, is the comparison between effects of oral iro...

hrp0086p2-p661 | Growth P2 | ESPE2016

‘First Do No Harm’: Growth Hormone (hGH) Treatment in a Case of Recurrent Craniopharyngioma

Zmau George-Sebastian , Armasu Ioana , Beleceanu Alina , Bursuc Anamaria , Puiu Mirela , Poeata Ion , Preda Cristina , Vulpoi Carmen

Introduction: Craniopharyngiomas (CP) are benign, dysontogenic supra or intra-sellar tumours. They are locally aggressive with severe endocrine, neurological and ophthalmological implications. Somatotroph deficiencies rise therapeutic management problems due to increased risk of tumour growth and recurrence.Case report: Male patient, aged 18 years 4 months, born naturally, late-term (42 weeks, 3200 g, Apgar 7), third child in a brotherhood of four (appar...

hrp0086p2-p662 | Growth P2 | ESPE2016

High Efficacy Growth Hormone Therapy in Patient with Homozygous Mutation in Growth Hormone Gene (GH-1) During 3 Years

Anna Gavrilova , Elena Nagaeva , Tatyana Shiryaeva

Background: Mutations in GH-1 gene is a rare cause of isolated growth hormone deficiency. Main features of this condition include markedly reduced secretion of GH combined with low concentrations of IGF-I leading to short stature.Objective and hypotheses: 1.56 years old girl was admitted to our hospital because of short stature. She was born at term from closely related healthy parents. Her birth length and weight were 48 cm (SDS: −1.07) a...

hrp0086p2-p663 | Growth P2 | ESPE2016

A Case of GH Deficiency in a Female with 3M Syndrome

Bortone Barbara , Scalini Perla , de Martino Maurizio , Giglio Sabrina , Lapi Elisabetta , della Monica Matteo , Stagi Stefano

Background: 3-M syndrome is an autosomal recessive primordial growth disorder characterized by severe prenatal and postnatal growth retardation, normal mental development, unusual facial features and skeletal abnormalities. Mutations in the CUL7, OBSL1 and CCDC8 genes are responsible for this syndrome. In literature a modest response to GH treatment in 3M children is reported without a significant improvement in the final height, suggesting a picture...

hrp0086p2-p664 | Growth P2 | ESPE2016

Case Report of SHOX Gene Haploinsufficiency Diagnosed in Early Infancy

Cinzia Pozzobon Gabriella , Gallo Dario , Damia Chiara , Partenope Cristina , Marinella Gemma , Osimani Sara , Pajno Roberta , Weber Giovanna

Background: SHOX-D is rarely diagnosed in early infancy as cause of short stature.Objective and hypotheses: Describe clinical characteristics of two girls with an early diagnosis of Leri-Weill dyschondrosteosis, admitted to our hospital because of severe short stature.Method: Mutation screening of SHOX and its regulatory regions was performed by MLPA. Family analysis was undertaken.Results: The first 1.3 year...

hrp0086p2-p665 | Growth P2 | ESPE2016

Association between IGF-1 (Insulin-Like Growth Factor) SD Levels and Children with Growth Hormone Deficiency with and without Pituitary Morphological Abnormalities

Claudia Schmitt-Lobe Maria , Frare Dionara , Greco Rafael

Background: The diagnostic use of IGF-1 in children suspected of GHD is based on the assumption that a single determination of this parameter reflects the integrated 24-h GH secretion. IGF-1 levels are low in GHD, but a study found a significantly lower sensitivity in children with organic lesions in the brain.Objective: The aim of this study was to check if there is a statistically significant relation between IGF-1 values in children with GHD with and ...

hrp0086p2-p666 | Growth P2 | ESPE2016

Usefulness of Growth Hormone Transient Treatment Suspension in Prepubertal Children Treated with Growth Hormone

Martinez Barahona Maria , Cecenarro Laura , Murillo Valles Marta , Luisa Granada Ybern M. , Ropero Ramos Esther , Bel Comos Joan

Background: The primary goal of treatment is to achieve a final height within the normal range and avoid the physical and psychological consequences of short stature (SS). If after a time, treatment efficacy criteria are not achieved, it is necessary to revalue its usefulness. There is no consensus about which conduct should be adopted in these cases. An alternative, is the suspension of rhGH treatment and evaluate the clinical and biochemical results to decide to continue or ...

hrp0086p2-p667 | Growth P2 | ESPE2016

Birth Length, Weight and Head Circumference of Neonates with IGF-I Receptor Mutations

Essakow Jenna , Lauterpacht Aaron , Laron Zvi

Background: In recent years more and more genetic defects along the GHRH–GH–IGF-I axis have been reported. Those mutations of the IGF-I receptor (R) are a rare abnormality of whom only the heterozygotes progenies survive.Objective and hypotheses: To determine the birth length, weight and brain size using head circumference and find out whether these correlate with the type of mutation.Method: Collection of data of 65 neon...

hrp0086p2-p668 | Growth P2 | ESPE2016

A Rare Cause of Growth Delay: Jacobsen Syndrome

Constantinescu Georgiana , Belceanu Alina , Bursuc Anamaria , Armasu Ioana , Braha Elena , Rusu Cristina , Mogos Voichita , Vulpoi Carmen

Background: Jacobsen syndrome is a rare genetic condition caused by partial deletion of the long arm of chromosome 11 associated with delayed development, distinctive facial features, bleeding disorder, skeletal abnormalities and endocrine disorders.Case report: We report a rare case of Jacobsen syndrome in 4 year old boy addressed for short stature. Born at term (36 W) with low birth weight (1780 g) and delayed development, his height at presentation wa...

hrp0086p2-p669 | Growth P2 | ESPE2016

A GH-1 Mutation Diagnosed in a Preadolescent Obese Girl with Only Mild Reduced Height

Bereau Agnes , Coutant Regis , Bouhours-Nouet Natacha

Background: Mutations in GH-1 are classically associated with autosomal dominant familial isolated GH deficiency (IGHD type II).Objective and hypotheses: Here, we report a new case of GH-1 mutation identified in a preadolescent girl consulting for a mild reduced stature contrasting with obesity.Method: The patient was born from non consanguineous French parents. She consulted at the age of 9 for short stature. Her...

hrp0086p2-p670 | Growth P2 | ESPE2016

A New Reusable Manual Pen Device for Injection of Human Growth Hormone: Results of a Convenience and Functionality Evaluation Study

Sauer Maritta , Castel Marie-Nathalie

Background: This multi-country study, conducted in the USA, is also ongoing in France, Germany, Brazil and South Korea (52 healthcare professionals (HCPs) and 30 patients/caregivers in total). The new device consists of a reusable aluminium body and cap, with a multi-use cartridge system, viewing window, dose-display window, dose-selection knob and injection button.Method: Semi-structured 60-min qualitative interviews were conducted in six major US citie...

hrp0086p2-p671 | Growth P2 | ESPE2016

Assessing Disease and Treatment Burden for Young Children with Growth Hormone Deficiency (GHD)

Brod Meryl , Wilkinson Lars , Alolga Suzanne Lessard , Hojbjerre Lise , Beck Jane , Rasmussen Michael Hojby

Background: Children with GHD, in addition to short stature, may experience physiological symptoms as well as social and emotional problems. Assessing these impacts is critical for understanding the extent of GHD burden and assessing treatment benefit. Since many children initiating treatment are too young to self-report information, we must rely on adult reporters. However, according to FDA guidelines and established measure development principles, adult reporter information ...

hrp0086p2-p672 | Growth P2 | ESPE2016

Long-Term Results of GH Therapy in GH-Deficient Children Treated in Albania

Gjikopulli Agim , Grimci Lindita , Kollcaku Laurent , Tomori Sonila , Ylli Zamira

Background: GH treatments aim to normalize growth, correct health problems associated with GH deficiency, and help patients achieve an adult height in the normal range for the general population and for familial genetic potential.Objective and hypotheses: To evaluate the efficiency of recombinant GH (rhGH) for improving adult height in children with GH deficiency (GHD).Method:: This is an observational follow up study which enrolle...

hrp0086p2-p673 | Growth P2 | ESPE2016

Abstract unavailable....

hrp0086p2-p674 | Growth P2 | ESPE2016

Did Growth Hormone Treatment Associated with Psychological Status in Children with Short Stature?

Chen Shoukang

Background: Short stature is clearly not a disease, but is commonly perceived to be associated with social and psychological disadvantage.Objective and hypotheses: To evaluate the psychological changes in children with short stature after growth hormone therapy.Method: One hundred and thirty children aged 6–14 years old were diagnosed as short stature (ISS and GHD). And they were divided into intervention group (55 children) a...

hrp0086p2-p675 | Growth P2 | ESPE2016

The Effects and Safety of Recombinant Human GH (rhGH) Treatment on Growth Hormone Deficiency in Children with Rathke’s Cleft Cyst

Liyang Liang , Zhe Meng , Lina Zhang , Hui OU , Lele Hou , Zulin Liu

Background: Rathke’s cleft cyst (RCC) has been reported rarely in children and adolescents. The occurrence of RCC with hypoplastic anterior pituitary can be explained by their common embryologic origin. However, the effects and sfety of recombinant human GH (rhGH) in GH deficient (GHD) children with RCC has not been previously reported.Objective and hypotheses: To assess the effects and safety of recombinant human GH (rhGH) in GH deficient (GHD) chi...

hrp0086p2-p676 | Growth P2 | ESPE2016

Is the Growth Hormone Deficiency the Cause of Short Stature in Floating Harbor Syndrome?

Albu Alice , Nicolaescu Irina , Petre Oana , Gheorghe-Fronea Ivona

Background: Floating Harbor syndrome (FGS) is a dominant autosomal genetic disorder characterized by facial dysmorphism, delay in language development and short stature associated with delayed bone age. Currently there are about 100 cases reported worldwide. Although the short stature is one of the main features of the FHS, its etiology is poorly understood. A limited number of cases reported growth hormone deficiency as a cause of short stature in FHS and the evolution during...

hrp0086p2-p677 | Growth P2 | ESPE2016

LHX-4 Gene Mutation in a Boy with Hypopituitarism and Severe Congenital Myopathy

Gucev Zoran , Tasic Velibor , Plasevska-Karanfilska Dijana , Stamatova Ana , Laban Nevenka , Polenakovic Momir

Background: LHX4 mutations are rare in combined pituitary hormone deficiency, and even rarer in isolated GHD.Objective and hypotheses: We describe a 14 years old boy who was referred for investigation of short stature.Methods and Results: Short stature, convergent strabismus, nystagmus was present. At the age of 5 years his gait was unstable. A progressive myopathy ensued. Tests of pituitary reserve showed partial IGHD (8....

hrp0086p2-p678 | Growth P2 | ESPE2016

Prevalence and Causes of Short Stature and Impact of GH Therapy among Preschool Children at King Abdul-Aziz Medical City for National Guard in Riyadh, Saudi Arabia

Aboulfotouh Mostafa

Background: It is widely assumed that short stature can result in psychological, social, and physical problems. The validity and efficacy of GH in management of short stature is still debated.Aims: This study aimed to i) estimate the prevalence of short stature in preschool children (4–6 years), and determine the final diagnosis of abnormality based on different investigations, and ii) assess the impact of intervention for short stature among presch...

hrp0086p2-p679 | Growth P2 | ESPE2016

Association between Growth Hormone Peak at a Stimulation Test and Pituitary Morphological Findings in Children with Growth Hormone Deficiency

Schmitt-Lobe Maria Claudia , Perini Lais Dadan , Salm Leticia

Background: GH deficiency (GHD) diagnosis includes clinical manifestations, laboratory tests and imaging. There are controversies about the validity of the GH stimulation test. A variety of stimulation tests are used in clinical practice. The biochemical definition of GHD has generally been considered to be a peak stimulated GH concentration <10 ng/ml.Objective: The aim of this study was to check if there is a statistically significant relation betwe...

hrp0086p2-p680 | Growth P2 | ESPE2016

A Case of Growth Hormone Deficiency with Combined Encephalocraniocutaneous Lipomatosis and Jaffe–Campanacci Syndrome

Choi Eun Mi , Shim Ye Jee , Kim Jun Sik , Kim Heung Sik

Introduction: Encephalocraniocutaneous lipomatosis (ECCL) is a rare neurocutaneous syndrome characterized by unilateral lipomatosis and ipsilateral neurologic/ophthalmic malformation. Jaffe–Campanacci syndrome (JCS) is characterized by the association of café-au-lait spots, axillary freckles, multiple non-ossifying fibromas of the long bones and jaw. Both of ECCL and JCS was associated neurofibromatatosis type 1. We had a case of GH deficiency with combined ECCL and ...

hrp0086p2-p681 | Growth P2 | ESPE2016

Evaluation of GH Deficient Pre Pubertal Children Treated with Omnitrope® Using the AuxoLog Computer Program

del Campo Maria Ruiz , Gonzalez Jose Revorio , Lechuga Alfonso , Gila Ana Lucia Gomez , Cancio Monica Fernandez , Carrascosa Antonio , Garcia Margarida Palla

Background: AuxoLog is a validated computer program that evaluates auxologic parameters comparing them with the Spanish growth charts. It also allocates subjects to the corresponding pubertal development group.Objective: To assess the evolution of auxological parameters in GH deficient (GHD) pre pubertal children treated with Omnitrope for a minimum of 2 years prior to puberty.Method: This study is non-interventional, retrospective...

hrp0086p2-p682 | Growth P2 | ESPE2016

Calcaneal Apophysitis (Sever’s Disease) Development in a Case Using Growth Hormone

Kurnaz Erdal , Erdeve Senay Savas , Aycan Zehra , Cetinkaya Semra

Background: Orthopedic complications related to the GH are rare and there is no clear pathological association between the use of GH and these complications. Calcaneal apophysitis is an inflammation of the apophysis and is caused by the constant pull of the Achilles tendon. A literature search did not reveal a similar case of calcaneal apophysitis during GH use.Case presentation: A 13-year-old male receiving GH treatment for isolated GH deficiency presen...

hrp0086p2-p683 | Growth P2 | ESPE2016

Bone Health Index: A Potential Discriminator between Growth Hormone Deficiency and Constitutional Delay in Growth and Puberty in Adolescent Children

Patil Prashant , Dharmaraj Poonam , Povall Ann , Abernethy Laurence , Das Urmi , Didi Mohommed , Ramkrishnan Renuka , Senniappan Senthil , Blair Jo

Background: Constitutional delay in growth and puberty (CDGP), the most common cause of short stature in children, is a transient state of delayed growth, skeletal maturation and attenuated pubertal growth spurt. It is not always easy to differentiate from GH deficiency (GHD) even with robust clinical and auxological assessment, measurement of IGF1 and bone age evaluation Bone health index (BHI) is a quantitative measure of bone health calculated from a hand and wrist X-ray us...

hrp0086p2-p684 | Growth P2 | ESPE2016

Growth Effects of Somatropin during the Treatment Congenital Hypopituitarism in Children after the Start of Puberty

Berseneva Olga , Bashnina Elena , Turkunova Maria

Background: Today the features of GH therapy in children after reaching the beginning of puberty and the necessity of therapy in such age group are discussed.Objective and hypotheses: To identify the relationship between the growth increase after the start of puberty (patient’s bone age achieved 12–13 years, according to the atlas Greulich) and the individual characteristics of the patients.Method: Thirty-four patients wi...

hrp0086p2-p685 | Growth P2 | ESPE2016

Congenital Hypopituitarism and Giant Cell Hepatitis in a Two-Months-Old Boy

Skorodok Yulia , Arestova Anzhelika , Kazachenko Natalia , Mullachmetova Zuhra , Ivanov Dmitriy

Background: Congenital hypopituitarism (CH) in the neonate which manifests as the deficiency of one or more pituitary hormones can be presented by a highly variable phenotype. Either as isolated hypopituitarism or with associated developmental defects such as ocular, midline, and genital abnormalities. Mutations in genes encoding for a number of transcription factors have been described in a minority of patients with CH. This indicats that further genes remain to be identified...

hrp0086p2-p686 | Growth P2 | ESPE2016

Neonatal Characteristics of GH Deficiency in 107 Children

Fedala Nora Soumeya , Haddam Ali El Mahdi , Meskine Djamila

Background: GH deficiency (GHD) rarely reveals at birth. Pregnancy is proceeding normally. The size and weight are generally normal and the birth occurs at terms. In some cases, neonatal markers and other pituitary deficits are present and allow early diagnosis.Objective and hypotheses: Report neonatal characteristics of GHD.Method: 107 children GHD were followed. The interrogation noted the progress of pregnancy, childbirth, weigh...

hrp0086p2-p687 | Growth P2 | ESPE2016

Diagnostic Value of Growth Hormone Stimulation Test for Growth Hormone Deficiency in Short Children

Jeong Seung Yeon , Lee Seung Ho , Yu Jeesuk

Background: It is important to find and manage the cause of short stature in children. GH stimulation test is considered as a ‘gold standard’ for the diagnosis of GH deficiency (GHD), and several pharmacologic agents including insulin, glucagon, L-dopa, or clonidine are used for GH stimulation test (GHST). However, diagnostic value, sensitivity or specificity of each GHST is not clear.Objective and hypotheses: This study was desi...