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جستجوی مقالات مرتبط با کلیدواژه « Growth Hormone Deficiency » در نشریات گروه « پزشکی »

  • Yaping Ma *, Zhuangjian Xu
    Introduction

    Gitelman syndrome (GS) is an inherited kidney disease that causes an imbalance of charged ions in the body. SLC12A3 mutation is the predominant cause of GS. There are different known and unknown pathogenic mutations in SLC12A3.

    Case Presentation

    In the present case report, an 8-year-old girl was referred to our pediatric endocrinology clinic for evaluation of short stature. Her height was 113 cm (-2.94 SD). Her growth hormone peak was 5.81 ng/mL. IGF-1 was lower than -2SD. Thyroid stimulating hormone was high. The blood potassium was 3.37 mmol/L. After 3 months of GH treatment, her blood potassium dropped further (3.01 mmol/L). The gene test results showed that there were two heterozygous mutations of the SLC12A3 gene: C.1456G>A (p.D486N) and c.1065_1072 delGCAGGG (p.A356Qfs*5), which her parents verified.

    Conclusions

    Gitelman syndrome can be associated with growth hormone deficiency and hypothyroidism in addition to short stature, hypokalemia, and hypomagnesemia, and the underlying molecular mechanisms need to be explored in the coexistence of these three diseases. The experience, in this case, is that blood electrolytes should be checked monthly for the first three months after growth hormone treatment for short stature. Once the blood potassium level is low, much attention should be paid to further diagnosis to avoid misdiagnosis.

    Keywords: Gitelman Syndrome, SLC12A3 Gene Mutation, Growth Hormone Deficiency, Hypothyroidism}
  • دنیل زمانفر*، جمشید یزدانی چراتی، هادی مجیدی، مریم دومهری، سحر فلاح
    مقدمه

    کمبود هورمون رشد ایدیوپاتیک ممکن است به صورت منفرد یا در ارتباط با کمبود سایر هورمون های هیپوفیزی باشد. استفاده از یافته های MRI مغزی برای افتراق میان دو گروه می تواند مفید باشد.

    روش کار

    این مطالعه توصیفی گذشته نگر تمامی کوکان 16-2 ساله ای که بین سال های 1390-96 با شکایت کوتاهی قد به کلینیک باغبان ساری مراجعه کرده اند و تشخیص کمبود هورمون رشد منفرد یا کمبود متعدد هورمون های هیپوفیزی داده شد را مورد بررسی قرار می دهد.

    نتایج

    در این مطالعه 150 بیمار که 42 درصد آنها دختر بودند مورد ارزیابی قرار گرفتند. . میانگین سن بیماران  71/34±63/113 ماه بوده است. 7/68  درصد کمبود خفیف هورمون رشد و 3/31 درصدکمبود شدید هورمون رشد را نشان دادند . در مجموع 67/6 درصد کمبود متعدد هورمون های هیپوفیزی و  33/3 درصد کمبود ایزوله هورمون رشد داشتند. 33/79 درصد بیماران Brain MRI نرمال 67/20 درصد بیماران  MRI غیرطبیعی داشتند. یافته ها شامل هایپوپلازی و فقدان آدنوهیپوفیز 41 درصد، نوروهیپوفیز اکتوپیک56/2 درصد، میکروآدنوم هیپوفیز 82/12 درصد، Partial empty sella  64/25 درصد،  Complete empty sella  69/7 درصد و سایر یافته های گزارش شده 25/10 درصد بوده است.

    نتیجه گیری

    در این مطالعه آنومالی های هیپوفیز در MRI مغزی بیماران با کمبود متعدد هورمون های هیپوفیزی بیشتر از بیماران مبتلا به کمبود ایزوله هورمون رشد گزارش شده اما همچنان مشخص نشده است که یافته های پاتولوژیک در MRI به طور ثابتی با کمبود هورمون رشد دایمی در ارتباط باشد.

    کلید واژگان: کمبود هورمون رشد, تصویربرداری مغزی, کوتاهی قد, کمبود متعدد هورمون های هیپوفیزی, کودک}
    Daniel Zamanfar *, Jamshid Yazdani Charati, Hadi Majidi, Maryam Domehri, Sahar Fallah
    Introduction

    Idiopathic growth hormone deficiency may be isolated or associated with deficiency of other pituitary hormones. the use of brain MRI findings to differentiate between the two groups can be useful.

    Methods

    This descriptive study of the retrospective method, investigates all 2–6-year-old children, who referred to Sari Gardener Clinic between 1390 and 1396 with a short stature complaint, and diagnosed them with a single growth hormone deficiency or multiple hypophysis hormone deficiency. Patient file information that includes demographic information, history and clinical examination information, laboratory information, and paraclinical information were extracted.

    Results

    In this study, 150 patients, 63 of whom (42%) were girls, were evaluated. The mean age of the patients was 34.71 ± 113.63 months. 68.7% showed a slight deficiency of growth hormone and 31.3% showed a severe deficiency of growth hormone. A total of 6.67% had a significant deficiency of pituitary hormones and 93.33% had an isolated deficiency of growth hormone. 79.33% of patients had normal Brain MRI and 20.67% had abnormal MRI. The findings included hypoplasia and 41% loss of adenohypophysis, 2.56% ectopic neurohypophysis, 12.82% pituitary microadenoma, 25.64% partial empty sella, 7.69% Complete empty sella and other reported findings were 10.25%.

    Conclusion

    In this study Pituitary abnormalities in brain MRI have been reported in patients with multiple pituitary hormone deficiency more than in patients with isolated growth hormone deficiency, However, it has not been established that the pathological findings detected on MRI are consistently associated with permanent growth hormone deficiency.

    Keywords: growth hormone deficiency, brain imaging, Short stature, multiple pituitary hormone deficiency, Child}
  • Héla Ayadi, Leila Cherif, Imen Hadjkacem, Yousr Moalla, Thouraya Kammoun, Mongia Hachicha, Farhat Ghribi, Wiem Kammoun *, Khaoula Khemakhem, Souhel Khemekhem
    Background
    The aim of this study was to assess self-esteem in children and adolescents with growth hormone deficiency and to establish the factors influencing self-esteem in children and adolescents with growth hormone deficiency.
    Methods
    This cross-sectional study was carried out on 26 children and adolescents, aged 8 to 18, with a total growth hormone deficiency. The Cooper Smith self-esteem inventory was used in this study.
    Results
    Patients with growth hormone deficiency had low self-esteem in more than 50% of the cases. Among the studied factors influencing the self-esteem, such as school performance, disturbance in parent-child and peers-child relationships, perceived by parents, and parents’ acceptance of the illness, there was a correlation in most of the subscales.
    Conclusions
    Growth hormone impaired children and adolescents require psychological counseling and comprehensive care.
    Keywords: Growth Hormone Deficiency, Self-Esteem, Children, Adolescents}
  • Şule Yildirim, Naci TopaloĞlu, Mustafa Tekin, Fatma Silan
    We report a case of 11-year-old girl with growth retardation and 46, XX, Xq27-qter deletion. The endocrinologic evaluation revealed growth hormone deficiency. In karyotype analysis 46, XX, Xq27-qter deletion was determined. The deletion of terminal region of chromosome 27 is most commonly being detected during the evaluation of infertility, premature ovarian insufficiency or in screening for fragile X carrier status. To our knowledge, this is the first reported case with 46, XX, Xq27-qter deletion and growth hormone deficiency. Furthermore, this case might facilitate future search for candidate genes involved in growth hormone deficiency.
    Keywords: Short stature, Growth hormone deficiency, X chromosome, Deletion, Gene}
  • Shahin Abdollahifakhim, Gholamreza Bayazian*, Siamak Shiva, Mojtaba Sohrabpour, Younes Ebrahimzade
    Introduction
    Failure to thrive (FTT) is relatively common among cleft patients, most commonly attributed to feeding problems during the first months of life. Close association between midline clefts and pituitary gland abnormalities prompted us to determine the frequency of growth hormone deficiency in cleft patients, which is easily treated.
    Methods
    Any cleft patient with FTT was studied and when the patient’s height was under the 3rd percentile of normal, growth hormone was checked after clonidine administration. Growth hormone was checked before and 30, 60 and 90 minutes after clonidine use.
    Results
    Of 670 patients with cleft lip or palate, 31 patients (4%) had some kind of growth retardation according to weight, height or head circumstance. Eighteen patients were under the 3rd percentile of normal height. Growth hormone deficiency was detected in 8 patients out of 18 patients and overall frequency of growth hormone deficiency among cleft patients with growth retardation was 25.8% (8 out of 31). Seven patients of 8 were male whereas one was female and half of the patients were syndromic.
    Conclusion
    Cleft patients have many problems with normal feeding and all kind of support should be provided to achieve near-normal feeding and they should be monitored for normal growth. Any patient with growth retardation, especially height decrease, should be assessed for growth hormone deficiency.
    Keywords: Cleft lip, Cleft palate, Growth hormone deficiency, Failure to thrive}
  • Cheng Guo, Li Chen
    Objective
    To evaluate the diagnostic value of provocative test by insulin combined with clonidine for growth hormone deficiency (GHD) during childhood
    Methods
    Eighty children underwent a provocative test with insulin(0.075U/Kg, intravenous) combined with clonidine (4μg/kg, orally). Among them, 40 children underwent clonidine provocative test, 40 children underwent insulin tolerance test (ITT) in another day.
    Findings
    The specificity of ITT+clonidine test (74%, 88%) was remarkably higher than that of ITT (48%) or clonidine test (65%). ITT+clonidine test had a better accuracy (75%, 85%) than that of ITT (63%) or clonidine test (73%)
    Conclusion
    We conclude that the combined clonidine+insulin test is a feasible, reliable, convenient, time saving, and safe tool for evaluation of the growth hormone (GH) axes than the clonidine test or ITT.
    Keywords: Clonidine, Insulin, Growth Hormone, Growth Hormone Deficiency, Short Stature}
  • Fahri Bayram, Gulden Baskol, Fatih Tanri, Verdi, Mevlut Baskol, Derya Kocer, AyŞ, En CaniklioĞ, Lu
    Background
    Growth hormone deficiency (GHD) is associated with an increased cardiovascular mortality.Increased oxidative stress has been associated with development of cardiovascular and cerebrovascular diseases. In the present study, we aimed to evaluate oxidant and antioxidant status in patients with GHD by analyzing serum paraoxonase1 (PON1) activity, and malondialdehyde (MDA) and thiol levels.
    Materials And Methods
    This study was a case–control study. Thirty patients with GHD were included in the study and compared with 20 healthy controls. Serum PON1 activity, and MDA and thiol levels were measured according to an enzymatic spectrophotometric method.
    Results
    Serum MDA levels (2.8 } 1.3 nmol/mL) were higher in GHD group than the controls (1.7 } 0.5 nmol/mL) (P = 0.001). PON1 activity (149.9 } 77.9 U/L) was lower in GHD group than the controls (286.3 } 126.7 U/L) (P = 0.001). Thiol and high‑density lipoprotein cholesterol (HDL‑cholesterol) levels were lower in GHD group (218.6 } 103.9 μmol/L and 32.6 } 13.4 mg/dL, respectively) than the controls (289.6 } 101.1 μmol/L and 54.3 } 14.9 mg/dL, respectively) (P = 0.021 and P = 0.001, respectively). In GHD patients, serum MDA level was negatively correlated with serum HDL‑cholesterol (r = −0.499, P = 0.001), and serum PON1 activity was positively correlated with serum thiol and HDL‑cholesterol levels (r = 0.306, P = 0.032 and r = 0.303, P = 0.033, respectively).
    Conclusion
    These data support that GHD is characterized by an imbalance between oxidant and antioxidant factors. This abnormality may contribute to the increased atherogenic risk in patients with GHD.
    Keywords: Growth hormone deficiency, malondialdehyde, oxidative stress, paraoxonase}
  • مهرنوش کوثریان، شیرین جوان پرست، ناصر ولایی
    سابقه و هدف
    کمبود هورمون رشد از دلایل اختلال رشد کودکان می باشد. پاسخ به درمان، تابع عواملی مانند سن، دوز دارو، مقدار دفعات تجویز، و مدت درمان می باشد. مطالعاتی راجع به رابطه پاسخ درمانی با حداکثر ترشح هورمون رشد (GH) در تست های تحریکی انجام شده است. به منظور بررسی این رابطه، مطالعه ای روی مراجعه کنندگان به درمانگاه غدد کودکان بیمارستان بوعلی سینا ساری در سال های 1375 تا 1378 انجام گرفت.
    مواد و روش ها
    یک کارآزمایی بالینی باز روی دو گروه کودک که دچار کمبود هورمون رشد بوده اند، انجام شد. بیماران بر اساس حداکثر مقدار GH در تست های تحریکی به دو گروه تقسیم شدند. گروه الف شامل بیماران با حداکثرترشح کمتر ازng/ml 5 و گروه ب شامل بیماران با حداکثر ترشح بیشتر از ng/ml 5 بود. بیماران از نظر سن تقویمی و استخوانی، دوز درمان، و دفعات تزریق درهفته یکسان بودند. متغیرهای Standard Deviation Score (SDS) وزن، قد، و سرعت رشد مورد مقایسه قرارگرفتند. نتایج در داخل گروه ها با Paired t-test و بین گروه ها با t-test مقایسه شدند.
    نتایج
    11 نفر از گروه الف و 5 نفر از گروه ب تحت بررسی قرار گرفتند. تغییرات رشد قدی و وزنی و سرعت رشد در داخل گروه ها به لحاظ آماری معنی داربود. تغییرات SDS وزن بین دو گروه معنی دار بوده است. کودکانی که حداکثر ترشح هورمون رشد بیشتری داشتند، بهتر وزن اضافه کردند، ولی تغییرات SDS قد و سرعت رشد بین دو گروه به لحاظ آماری معنی دارنبود.
    استنتاج
    به نظر نمی رسد پاسخ به درمان با هورمون رشد به حداکثر ترشح هورمون رشد در تست تحریکی وابسته باشد. مطالعه طولانی تر و با تعداد بیشتر بیمار توصیه می شود.
    کلید واژگان: هورمون رشد, کمبود, درمان}
    M. Kosarian, Sh Javan Prast, N. Valayee
    Background and
    Purpose
    Growth hormone deficiency is the reason of growth disorder in children. Therapeutic response is related to the factors such as, age, drug dosage, numbers of administrations and duration of therapy. Researches have been done to clarify the relationship between therapeutic response with the maximum amount of GH secretion in stimulatory tests. Ïn order to study this relationship, a research was conducted in Boali endocrine clinic on referred patients. in 1997-1990.
    Materials And Methods
    This study was an open clinical trial done on two groups of children with growth hormone deficienes. The patients were divided in two groups of  and B on the basis of maximum amount of growth hormone secretion on stimulatory tests. Group  included 11 patients, with maximum secretion of not more than 5 ng/ml. Group B, included 5 patients with maximum secretion of more than 5 ng/ml. Patients were matched for numerical and skeletal age, dosago and frequency of injection per week. Standard Deviation Score(SDS) for weight, height and the rate of growth were compared. The results were compared by paired T-test in the groups and T-test between the groups.
    Results
    The height growth, weight changes and also the growth rate in the groups were significant statistically. Weight SDS change was significant between two groups, but height and growth rate SDS changes between two groups was nor significant. Çonclusion: No significant changes was observed in group Â, which could be due to the less number of samples and much height growth SDS changes. Hence, further studies with more number of samples is recommended.
    Keywords: Growth hormone deficiency, Growth hormone, Therapy}
نکته
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