A Critical Appraisal of Growth Hormone Therapy in Growth Hormone Deficiency and Turner Syndrome Patients in Turkey

Z, Yavaş Abalı; Darendelıler, Feyza; Neyzi, Olcay

doi:10.4274/jcrpe.3209

Cited by 6 publications

(4 citation statements)

References 37 publications

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“…ITT, considered a gold standard GH stimulation test for GH deficiency, is rarely used nowadays, which was used previously by paediatricians considering the risk of hypoglycemia in patients. This practice is similar to data collected by Yavaş Abalı et al 12 Body mass index and short fasting also raise GH response. 13 No patients were sex primed in our setup, as against many studies where sex priming before the GH stimulation test is advised.…”

Section: Discussionsupporting

confidence: 87%

“…There was a lack of consensus on it as it falsely failed the GH stimulation test as observed by Martínez et al 14 The cut-off value utilised in most studies was 7ng/ml (21mIU/l) as in our setup. 15 Some mutations have been identified in the routine workup, though not utilised in idiopathic GHD. Genetic causes can be considered in a specific group of children whose phenotypic assessment suggest increased chances of genetic causes.…”

Section: Discussionmentioning

confidence: 99%

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A Survey of Growth Hormone Stimulation Test Efficacy for Workup of Short Stature in a Referral Laboratory

Munir

Aamir

Haroon

et al. 2023

PAFMJ

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Objective: To assess growth hormone (GH) stimulation test efficacy in short stature workup for establishing GH deficiency. Study Design: Cross-sectional survey. Place and Duration of Study: Diagnostic Endocrine Section, Department of Chemical Pathology & Endocrinology, Armed Forces Institute of Pathology (AFIP), Rawalpindi Pakistan, from Aug 2020 to Jan 2021. Methodology: The study was conducted on 129 individuals aged 2-16 years. History, axiological data and biochemical parameters were assessed to establish GH deficiency for short stature workup. Results: Out of 129 individuals, 76(59%) boys and 53(41%) girls reported GH stimulation tests. 81(62.3%) children were in <3rd percentile, 29(22.3%) in <5th percentile, 9(6.9%) in <10th percentile and 5(3.8%) in <25th percentile. Among the group with bone age difference >2 years, 34(82.9%) fell in <3rd percentile. GH Stimulation test post-Levo Dopa was performed in 102(78.46%) patients. 49(37.4%) patients responded inadequately to the GH stimulation test. The adequate response to the GH Stimulation test improved as the percentile declined from the 50th to the 3rd percentile. Conclusion: GH stimulation test results can only partially rely upon though their importance remains in combination with other short-stature workup parameters for ruling out growth hormone deficiency.

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Section: Discussionsupporting

confidence: 87%

Section: Discussionmentioning

confidence: 99%

A Survey of Growth Hormone Stimulation Test Efficacy for Workup of Short Stature in a Referral Laboratory

Munir

Aamir

Haroon

et al. 2023

PAFMJ

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“…TS and GH deficiency are important differential diagnoses in females with short stature and are the two most frequently approved conditions for GH treatment [ 11 ]. TS can be differentiated from GH deficiency by delayed bone age, hypogonadism, characteristic phenotypic features, and peak GH levels after GH provocation tests [ 12 ].…”

Section: Discussionmentioning

confidence: 99%

Coexistence of Growth Hormone Deficiency and Pituitary Microadenoma in a Child with Unique Mosaic Turner Syndrome: A Case Report and Literature Review

Park

Kim

et al. 2020

Diagnostics

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Turner syndrome (TS) is a genetic disorder with phenotypic heterogeneity caused by the monosomy or structural abnormalities of the X chromosome, and it has a prevalence of about 1/2500 females live birth. The variable clinical features of TS include short stature, gonadal failure, and skeletal dysplasia. The association with growth hormone (GH) deficiency or other hypopituitarism in TS is extremely rare, with only a few case reports published in the literature. Here, we report the first case of a patient with mosaic TS with complete GH deficiency and pituitary microadenoma, and we include the literature review. During the work-up of the patient for severe short stature, three GH provocation tests revealed peak GH levels of less than 5 ng/mL, which was compatible with complete GH deficiency. Sella magnetic resonance imaging showed an 8 mm non-enhancing pituitary adenoma with mild superior displacement of the optic chiasm. Karyotyping revealed the presence of ring chromosome X and monosomy X (46,X,r(X)/45,X/46,X,psu dic r(X;X)), which indicated a mosaic TS. It is important to consider not only chromosome analyses in females with short stature, but also the possibility of the coexistence of complete GH deficiency accompanying pituitary lesions in TS. In conclusion, the present study reports the first case of GH deficiency and pituitary adenoma in a patient with rare mosaic TS, which extends the genotype–phenotype spectrum for TS.

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“…GH is administered subcutaneously with a dose of 0.025-0.1 mg/kg/day ( 3 , 5 ) for six or seven days per week until the final adult height (FAH) is achieved. Factors affecting response to GH therapy have been reported to be frequency, dose, duration of treatment, adherence to treatment, age at onset of treatment, birth length, height standard deviation score (SDS) at the beginning of treatment, parental height, and the first-year response to GH treatment ( 6 , 7 , 8 ).…”

Section: Introductionmentioning

confidence: 99%

Evaluation of the Final Adult Height and Its Determinants in Patients with Growth Hormone Deficiency: A Single-centre Experience from the South-Eastern Region of Turkey

Demiral¹,

Ünal²,

Baysal³

et al. 2020

Jcrpe

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The aim was to determine the final adult height (FAH) achieved by recombinant human growth hormone (rhGH) treatment, the factors affecting FAH and the success of attaining the genetic potential. Methods: Data of 133 patients treated with rhGH therapy were reviewed retrospectively. Patients were grouped according to diagnosis, either isolated GH deficiency (IGHD) or multiple pituitary hormone deficiency (MPHD), and by sex, and pubertal status at the beginning of treatment. Results: The mean age of initiation of treatment was 12.3±2.18 years, and the mean duration of rhGH treatment was 3.65±1.5 years. The mean height standard deviation score (SDS) at diagnosis was-3.11±0.75 SD. All patients received a standardized GH dose of 0.033 mg/kg/day. Mean FAH-SDS was-1.8±0.77 and delta height-SDS (the change in height SDS between the beginning and end of treatment) was 1.28±0.94 SD. FAH-SDS was-1.79±0.86 SD in males;-1.82±0.64 in females (p=0.857);-1.94±0.71 at the beginning of treatment in pubertal patients and-1.68±0.81 in prepubertal patients (p=0.056);-1.84±0.89 in patients with IGHD and-0.47±0.2 in patients with MPHD (p˃0.05). In multiple regression analysis, First year delta height-SDS was the most predictive factor for both FAH-SDS and delta height-SDS. Conclusion: The majority of our patients achieved a final height compatible with their genetic potential as well as population standards when treated with rhGH even having started at a relatively late age. First year delta height-SDS was a predictive factor for FAH.

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A Critical Appraisal of Growth Hormone Therapy in Growth Hormone Deficiency and Turner Syndrome Patients in Turkey

Cited by 6 publications

References 37 publications

A Survey of Growth Hormone Stimulation Test Efficacy for Workup of Short Stature in a Referral Laboratory

A Survey of Growth Hormone Stimulation Test Efficacy for Workup of Short Stature in a Referral Laboratory

Coexistence of Growth Hormone Deficiency and Pituitary Microadenoma in a Child with Unique Mosaic Turner Syndrome: A Case Report and Literature Review

Evaluation of the Final Adult Height and Its Determinants in Patients with Growth Hormone Deficiency: A Single-centre Experience from the South-Eastern Region of Turkey

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