scholarly journals A SOX3 (Xq26.3-27.3) duplication in a boy with growth hormone deficiency, ocular dyspraxia, and intellectual disability: A long-term follow-up and literature review

HORMONES ◽  
2014 ◽  
Author(s):  
Stefano Stagi ◽  
Elisabetta Lapi ◽  
Marilena Pantaleo ◽  
Giovanna Traficante ◽  
Sabrina Giglio ◽  
...  
Keyword(s):  
Growth Hormone ◽  
Intellectual Disability ◽  
Literature Review ◽  
Growth Hormone Deficiency ◽  
Hormone Deficiency ◽  
Long Term Follow Up

Prevalence of childhood growth hormone deficiency in survivors of pediatric intracranial germ cell tumors.

2020 ◽  
Vol 38 (15_suppl) ◽  
pp. e22526-e22526
Author(s):  
Diana Lone ◽  
Karim Thomas Sadak ◽  
Bradley S Miller ◽  
Michelle Roesler ◽  
Jenny N Poynter
Keyword(s):  
Growth Hormone ◽  
Germ Cell ◽  
Growth Hormone Deficiency ◽  
Late Effects ◽  
Germ Cell Tumors ◽  
Hormone Deficiency ◽  
Endocrine Disorders ◽  
Intracranial Germ Cell Tumors

e22526 Background: Survival rates for childhood cancer continue to rise, and there are now greater than 420,000 survivors in the United States. However, high cure rates come at the cost of short and long-term treatment-related toxicities. Endocrine disorders are among the most common late effects and are associated with poor health outcomes and lower quality of life. Survivors of pediatric intracranial germ cell tumors (iGCTs) are at high risk for endocrine disorders, particularly for growth hormone deficiency (GHD), due to their exposures to cranial radiation, chemotherapy, and brain surgery. To date, no long-term follow-up studies have explored the late effects experienced by survivors of iGCTs. Methods: Study participants were enrolled in the Germ Cell Tumor Epidemiology Study, which is a case-parent triad study conducted using the resources of the Children’s Oncology Group’s Childhood Cancer Research Network. Eligibility criteria included diagnosis with a germ cell tumor in any location at age 0-19 years in the years 2008-2015. The study population included 233 cases with a diagnosis of iGCT. We are currently following the cohort to evaluate outcomes and late effects of treatment, including medical record review to extract data on treatment characteristics and hormone deficiencies. This interim analysis includes chart review for 57 iGCT cases. Results: Of the 57 cases reviewed, there was a male predominance (73.7%) with the highest prevalence in non-Hispanic whites (80.4%). Cases of iGCTs can be subdivided into two main histologic subtypes, germinomas (36 cases) and non-germinomatous GCTs (NGGCT, 21 cases). The median age at diagnosis was 14.6 years for the germinomas and 10.5 years for NGGCTs. Data on growth hormone deficiency (GHD) was available for 42 of the 57 cases with a median follow-up of 7.4 years. Twenty-eight of the 42 cases (66.7%) had GHD; 19 in the germinoma group and 9 in the NGGCT group (p = 0.47). 17 of those with GHD were males (p = 0.10). There was no significant difference in prevalence of GHD by age of tumor diagnosis (p = 0.20). Conclusions: Survivors of iGCTs are at high risk for growth hormone deficiency. Identifying specific risk factors for developing GHD amongst these survivors can enhance the current guidelines for screening and management.

scholarly journals LONG TERM FOLLOW-UP OF ONE OF THE FIRST PATIENTS TO RECEIVE HUMAN GROWTH HORMONE THERAPY

2020 ◽  
Vol 6 (5) ◽  
pp. e262-e264
Author(s):  
Cassandra Hawco ◽  
Robyn L. Houlden
Keyword(s):  
Growth Hormone ◽  
Medical Center ◽  
Final Height ◽  
Sella Turcica ◽  
Hormone Deficiency ◽  
Pituitary Hormone ◽  
Long Term Follow Up ◽  
To Receive

Objective: Treatment of growth hormone (GH) deficiency with GH extracts from human pituitary glands was introduced by Dr. Maurice Raben at Tufts New England Medical Center in 1956. We report long term follow-up of one of the first patients treated with GH. Methods: Clinical, radiographic, and genetic data are presented. Results: A 78-year-old man presented for follow-up. He was one of the first patients to receive GH therapy from Raben in 1958. Growth was reported as normal until age 3 and then decelerated. At age 17 years, he was 129.5 cm with absent sexual development and bone age of 7 years. Treatment was initiated with desiccated thyroid and cortisone acetate for 8 months. Human GH extract was then initiated with 2 mg 3 times/week for 2 years, then 3 mg 3 times/week for 6 months, resulting in a final height of 168.9 cm. Testosterone intramuscularly every 2 weeks was added with sexual maturation over 2 years. He remained on testosterone injections until the age of 40 years, and on transdermal testosterone until the age of 50 years. At age 27 years, he was treated by Raben with human chorionic gonadotropin and menotropins for spermatogenesis restoration with successful conception by his wife. At age 78 years, pituitary MRI revealed a tiny amount of pituitary tissue within normal-sized sella turcica with absent pituitary infundibulum. A combined pituitary hormone deficiency genetic panel did not reveal any mutations. Conclusion: Sixty-two years later, the patient remains in good health and grateful to a pioneer in Endocrinology for groundbreaking therapy of short stature. The cause of his hypopituitarism remains unknown.

New Treatments for Growth Hormone Deficiency

2013 ◽  
Vol 09 (01) ◽  
pp. 71
Author(s):  
Stephen F Kemp ◽  
Keyword(s):  
Growth Hormone ◽  
Growth Hormone Deficiency ◽  
Serum Insulin ◽  
Hormone Deficiency ◽  
Long Term Follow Up ◽  
Igf Binding ◽  
Long Acting ◽  
New Treatments ◽  
Igf Binding Protein

First recognized in the early 20th century, growth hormone deficiency (GHD) has been treated with growth hormone (GH) replacement since 1958. Initial replacement was with cadarevic GH. In 1985, GH therapy with recombinant human GH (rhGH) replaced cadaveric GH, which increased not only safety, but also efficacy (because of increased supply). Improvements in GH dosing and frequency of injection has resulted in adult heights now usually in the normal range. GHD is diagnosed from the clinical picture, along with measurement of serum insulin-like growth factor 1 (IGF-I), IGF binding protein-3, and GH response to provocative stimuli. Several long-acting GH preparations are now under development. There has been a great deal of data from databases that confirms safety of GH administration while patients are taking GH. A recent report of increased mortality risk in adults who were treated with GH as children has not been confirmed by a second similar retrospective evaluation. Additional long-term follow-up studies of adults who took GH as children are needed.

Effects of long-term growth hormone replacemente therapy on body composition in adult patients with growth hormone deficiency

2020 ◽  
Author(s):  
García Moreno Rosa María ◽  
Ramírez Paola Parra ◽  
Saiz Gonzalo Baonza ◽  
Rojas-Marcos Patricia Martin ◽  
Beatriz Lecumberri ◽  
...  
Keyword(s):  
Growth Hormone ◽  
Body Composition ◽  
Growth Hormone Deficiency ◽  
Adult Patients ◽  
Hormone Deficiency
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