Pituitary Dwarfism

A form of dwarfism caused by complete or partial GROWTH HORMONE deficiency, resulting from either the lack of GROWTH HORMONE-RELEASING FACTOR from the HYPOTHALAMUS or from the mutations in the growth hormone gene (GH1) in the PITUITARY GLAND. It is also known as Type I pituitary dwarfism. Human hypophysial dwarf is caused by a deficiency of HUMAN GROWTH HORMONE during development.

Also Known As:

Dwarfism, Pituitary; Dwarfism, Growth Hormone Deficiency; Isolated GH Deficiency; Isolated Growth Hormone Deficiency; Isolated HGH Deficiency; Isolated Human Growth Hormone Deficiency; Isolated Somatotropin Deficiency; Isolated Somatotropin Deficiency Disorder; Nanism, Pituitary; Pituitary Nanism; Growth Hormone Deficiency Dwarfism; Hypophysial Dwarf; Hyposomatotrophic Dwarfism; Pituitary Dwarf

Networked: 676 relevant articles (9 outcomes, 49 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Hypopituitarism (Sheehan's Syndrome)
2.	Diabetes Insipidus
3.	Dwarfism
4.	Combined Pituitary Hormone Deficiency
5.	Acromegaly

Experts

1.	Salvatori, Roberto: 40 articles (04/2022 - 03/2002)
2.	Aguiar-Oliveira, Manuel H: 32 articles (04/2022 - 07/2002)
3.	Oliveira, Carla R P: 21 articles (01/2021 - 04/2003)
4.	Pereira, Rossana M C: 14 articles (09/2014 - 07/2002)
5.	Souza, Anita H O: 11 articles (04/2022 - 07/2002)
6.	Campos, Viviane C: 10 articles (01/2021 - 06/2007)
7.	Valença, Eugênia H O: 9 articles (01/2022 - 06/2012)
8.	Laron, Zvi: 9 articles (01/2018 - 01/2002)
9.	Dattani, Mehul T: 9 articles (06/2015 - 01/2004)
10.	Melo, Enaldo V: 8 articles (04/2022 - 09/2012)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Pituitary Dwarfism:

1.	Hormones (Hormone)IBA 01/26/2018 - "In the multiple pituitary hormone deficiency (MPHD) group (n=50), first year GH response was significantly greater than in the isolated GH deficiency (IGHD) group (n=17) (p=0.030). " 01/01/1991 - "Previous studies have indicated that the duration of puberty is shortened by GH treatment in patients with idiopathic multiple pituitary hormone deficiency or isolated GH deficiency (GHD). " 11/22/2023 - "PROKR2 Mutations in Patients with Short Stature Who Have Isolated Growth Hormone Deficiency and Multiple Pituitary Hormone Deficiency" 10/01/2023 - "The male to female ratio was 2.5:1. Nine (64%) children had multiple pituitary hormone deficiency (MPHD) and 5 (36%) had isolated growth hormone deficiency (IGHD). " 06/14/2023 - "Phenotype-genotype correlations of GH1 gene variants in patients with isolated growth hormone deficiency (IGHD) or multiple pituitary hormone deficiency (MPHD)."
2.	Growth Hormone (Somatotropin)IBA 07/13/2002 - "To evaluate the efficacy of recombinant growth hormone for increasing adult height in children treated for idiopathic isolated growth hormone deficiency. " 05/17/2022 - "Spontaneous dwarf rat (SDR) is a primary experimental animal model for the study of pituitary dwarfism with a point mutation in the Gh gene encoding growth hormone (GH). " 01/01/2018 - "PurposeThe aim of this study was to determine the choroidal thickness measurement values in cases with isolated growth hormone deficiency (IGHD), to compare them with the healthy control group by using enhanced depth imaging optical coherence tomography (EDI-OCT), and to evaluate the effect of growth hormone (GH) treatment on choroid.Patients" 02/01/2012 - "Long-term mortality after recombinant growth hormone treatment for isolated growth hormone deficiency or childhood short stature: preliminary report of the French SAGhE study." 07/01/1996 - "Regulation of postnatal intestinal maturation by growth hormone: studies in rats with isolated growth hormone deficiency."
3.	Human Growth Hormone (Saizen)FDA LinkGeneric 01/01/2010 - "The study comprised 30 children and young people aged 4.2-18 years with isolated growth hormone deficiency both prior to and 6 months after rhGH (recombinant human growth hormone) treatment with a dose of 0.093 IU/kg every 24 hr. The control group comprised 25 healthy children with normal height in the respective age bracket. " 09/01/1994 - "Molecular study of human growth hormone gene cluster in three families with isolated growth hormone deficiency and similar phenotype." 03/01/1976 - "A trial of the relative effect on growth of 20 IU/week and 10 IU/week of human growth hormone has been made in 38 patients with "isolated" growth hormone deficiency over 1 year of treatment, 18 patients over 2 years and 10 over 3 years, and in 17 patients with surgically treated craniopharyngiomata over 1 year. " 01/01/1975 - "The study has aimed at investigating the blastic transformation of peripheral blood lymphocytes in patients with pituitary dwarfism before, during and after the treatment with human growth hormone. " 07/01/1965 - "[Study of the effects of human growth hormone in a patient with pituitary dwarfism]."
4.	Gonadotropin-Releasing Hormone (GnRH)FDA Link 10/01/1992 - "Our preliminary data showed that the addition of GnRH-analogues to GH in subjects with isolated GH deficiency reduces the effect of GH on height velocity, but determines an improvement in statural prognosis, although a proper answer will not be obtained until final height has been achieved." 05/01/2004 - "The use of gonadotropin-releasing hormone (GnRH) analogues has been advocated as a tool to delay puberty in patients with isolated GH deficiency (IGHD), allowing longer time for the beneficial effect of exogenous human GH (hGH) treatment on growth. " 10/01/1992 - "In subjects with an isolated GH deficiency the inhibition of puberty by GnRH-analogue administration may be attempted to delay the onset, or to prolong the duration, of pubertal maturation in order to improve final height. " 01/01/1992 - "A long acting analogue of gonadoliberin, Trp-6-GnRH, has been given to 17 patients with isolated growth hormone deficiency in order to delay pubertal progression. " 06/01/1989 - "A long-acting analogue of gonadoliberin, Trp6-GnRH, has been given to GH-treated patients with isolated growth hormone deficiency at the time they reached pubertal stage 2, in combination with unchanged doses of GH, for one year in 11 and for two years in 7 of them. "
5.	Glucagon (Glukagon)FDA Link 06/01/1974 - "Normal responsivity of serum IRI following subcutaneous administration of glucagon in 25 cases of pituitary dwarfism in children and adolescents. " 12/01/1978 - "Patients with isolated growth hormone deficiency were characterized by high plasma glucagon response and low plasma insulin response, whereas acromegalic patients showed exaggerated plasma glucagon response and almost normal insulin response. " 09/01/1977 - "Six children and adolescents (aged from 2 6/12 to 16 years) with isolated hGH deficiency were subjected to a standard oral glucose tolerance test (OGTT) followed by the administration of IV glucagon at 180 mins. " 05/10/1974 - "[Propranolol-glucagon tolerance test--with special reference to pituitary dwarfism]." 02/20/1974 - "[Proceedings: Propranolol-glucagon loading test--with special reference to pituitary dwarfism]."
6.	GonadotropinsIBA 04/01/1992 - "The effects of hCG-hMG treatment in 13 boys with pituitary dwarfism associated with gonadotropin deficiency, were assessed. " 05/01/2000 - ": We found isolated growth hormone deficiency as the only identifiable cause for primary amenorrhea in three patients with sufficient gonadotropins secretion. " 06/01/1981 - "Females with pituitary dwarfism of the multiple pituitary hormone deficiency form have ovarian failure due to hypogonadotropism which would be expected to respond favorably to human gonadotropin treatment. " 12/01/1981 - "Subnormal gonadotropin responses to gonadotropin-releasing hormone persist into puberty in children with isolated growth hormone deficiency." 06/01/1981 - "This is a description of a pituitary dwarf in whom pregnancy was achieved with gonadotropin therapy; its progress was followed regularly by hormone assays."
7.	N 30IBA 05/01/2001 - "Three groups with comparable age, sex and pubertal stage were studied: group I (n=42)--patients with isolated growth hormone deficiency (IGHD); group II (n=22)-- patients with multiple pituitary hormone deficiency (MPHD); and group III (n=30)--healthy controls. " 02/01/1997 - "The aim of the study was to assess the efficacy of GH therapy in GH-deficient children treated before the age of 3 yr. A noncomparative multicenter prospective study included 49 children (22 girls and 27 boys) with isolated GH deficiency (n = 19) or multiple pituitary hormone deficiency (n = 30) treated with daily s.c. "
8.	AndrogensIBA 06/01/1979 - "The results indicate that brief androgen administration can be helpful in delineating the cause of growth retardation in boys with short stature and delayed sexual maturation, particularly when the diagnosis of isolated growth hormone deficiency is suspected. " 07/20/1999 - "In hypophysectomized or pituitary dwarf mice the reduced androgen stimulation of GUS can be partially restored with GH treatment. " 06/01/1984 - "[Treatment of pituitary nanism: is it necessary to treat adrenal androgen deficiency?]." 01/01/1982 - "Mean plasma androgen levels are normal in isolated GH deficiency. " 01/01/1982 - "Longitudinal analysis of the data, however, shows that patients with either isolated GH deficiency or multiple pituitary deficiencies without ACTH deficiency constitute a heterogeneous population, with either normal or low to very low plasma androgen levels. "
9.	Insulin-Like Growth Factor I (IGF-1)IBA 09/01/2018 - "non-syndromic CPHD), 2. Isolated GH deficiency (IGHD), 3. GH insensitivity, and 4. IGF-I insensitivity. " 05/01/2018 - "We conclude that IGF-1 test can be a useful tool in the diagnosis of pituitary dwarfism in children suspected of this condition, but due to relatively poor sensitivity the testing cannot be performed alone, but in combination with other tests. " 05/01/2012 - "In the previous report, the -278G and - 57T allele are associated with low serum IGF-I levels in patients with isolated GH deficiency and the haplotype of the - 278T, - 57G, +1169 A, and +2103T allele exhibited an impaired GH secretion in vitro.It" 01/01/2010 - "Genetic polymorphisms in the locus control region and promoter of GH1 are related to serum IGF-I levels and height in patients with isolated growth hormone deficiency and healthy controls." 03/01/2008 - "The growth velocity obtained with IGF-I administration is smaller than that observed with hGH in children with congenital isolated GH deficiency; C. "
10.	somatotropin releasing hormone receptorIBA 08/01/2013 - "To screen Isolated Growth Hormone Deficiency (IGHD) patients with congenital Familial Isolated (FIGHD) and Nonfamilial Isolated Growth Hormone Deficiency (NFIGHD) for GH1gene deletions (6.7 kb,7.6 kb,7 kb) and Growth hormone releasing hormone receptor GHRHR(E72X) gene mutation and study genotype/phenotype correlation in this multicentre study. " 10/01/2005 - "Growth hormone releasing hormone receptor (GHRH-r) gene mutation in Indian children with familial isolated growth hormone deficiency: a study from western India." 01/01/2023 - "In humans and mice, loss-of-function mutations in growth hormone-releasing hormone receptor (GHRHR) cause isolated GH deficiency. " 04/28/2022 - "We have described large kindred with isolated GH deficiency (IGHD) due to a GHRH receptor mutation, resulting in severe short stature and high-pitched voice. " 01/01/2021 - "Adult subjects with isolated growth hormone deficiency (IGHD) due to a mutation in the growth hormone releasing hormone receptor gene exhibit higher values formant frequencies. "

Therapies and Procedures

1.	Therapeutics 03/01/2008 - "This study describes the orthodontic treatment of 2 patients (a boy and a girl) diagnosed with pituitary dwarfism and treated with rhGH therapy. " 10/30/2021 - "We aimed to evaluate thyroid function in initially euthyroid children with idiopathic isolated GH deficiency during long-term rhGH therapy and to determine who is at a higher risk of thyroid function alterations during the therapy. " 07/01/2016 - "The aim of our prospective case-control study was to evaluate long-term effects of GH replacement therapy on erythrocytes parameters, leukocytes, and platelets numbers in a large cohort of children with isolated GH deficiency (GHD). " 01/01/2015 - "A number of investigations have proven that GF has a direct lipolytic effect: adequate replacement therapy for pituitary nanism gives rise to a reduction in fat depots. " 01/01/2012 - "Isolated growth hormone deficiency type 2: from gene to therapy."
2.	Hormone Replacement Therapy (Therapy, Hormone Replacement) 05/01/2019 - "To evaluate efficacy of hormone replacement therapy in children with isolated GH deficiency. "
3.	Cesarean Section (Caesarean Section) 04/01/2017 - "We describe the anaesthetic management for caesarean section in a 32-year-old patient with pituitary dwarfism. " 04/01/2017 - "Epidural anaesthesia for caesarean section in pituitary dwarfism." 01/01/2010 - "Sub arachnoid block for emergency cesarean section in a pituitary dwarf." 07/01/1998 - "Anesthesia for cesarean section in a pituitary dwarf."
4.	Cranial Irradiation 01/01/2012 - "Isolated growth hormone deficiency (IGHD) may be congenital, often due to genetic mutations, or acquired as a result of other factors such as cranial irradiation. " 02/01/2009 - "The growth hormone (GH) axis is the most vulnerable to radiation damage, and isolated GH deficiency can occur after doses as low as 18 Gy. Furthermore, the frequency of GH deficiency can reach 50-100% within 3-5 years of cranial irradiation with doses of 30-50 Gy. TSH and adrenocorticotropic hormone deficiency occur in 3-6% of patients after conventional irradiation (30-50 Gy). "
5.	Prostatectomy (Retropubic Prostatectomy) 06/01/2021 - "Erratum to prostate biopsy free system for laparoscopic radical prostatectomy in a pituitary dwarfism: a case report." 12/01/2020 - "Prostate biopsy free system for laparoscopic radical prostatectomy in a pituitary dwarfism: a case report." 12/01/2020 - "This is the first report of a laparoscopic radical prostatectomy (LRP) in a pituitary dwarfism who didn't have a prostate biopsy before LRP due to anal stenosis. "