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Philip Galkin
Philip Galkin

Download __EXCLUSIVE__ Treatment Growth Hormone Deficiency Pdf


The study investigated cardiovascular changes in adult-onset growth hormone deficiency (GHD) and showed that patients with adult-onset GHD have a left ventricular mass index (LVMi) at or below the lower limit of normal, which improves with one year of growth hormone replacement.




Download Treatment Growth Hormone Deficiency pdf



Patients with GHD on GH treatment for 1 year showed an increase in median insulin-like growth factor I (IGF-I) SDS from -1.83 to +0.40 (p=0.0068). There was no correlation between LVMi and IGF-I SDS (r=0.164, p=0.657). At one year the median LVMi moved into the previously published normal range (55.0 to 63.0 g/m2, normal range 55.4 - 74.0) achieving statistical significance compared to pre-treatment values (p=0.0156).


Growth hormone deficiency (GHD), which may be congenital or acquired, can afflict any age and manifests itself similarly across age ranges. Children with GHD suffer from short stature, increased and disproportionately distributed body fat, lipid abnormalities and decreased bone mineral density while adults with GHD suffer similar consequences except short stature (6, 7).


Studies of GH infusion have demonstrated that continuous exposure to unmodified GH is a safe and effective alternative to daily injections (14, 15, 16). Thus, to ameliorate injection fatigue and improve outcomes, development of long-acting formulations has garnered considerable interest. As early as 1979, Lippe and coworkers evaluated the efficacy of a depot GH preparation in GHD children (17). Since then, multiple companies have attempted to develop a long-acting growth hormone (LAGH).


GH has direct in vivo effects (independent of circulating IGF-1) exerted, in part, through local IGF-1 production (5). As much as 20% of linear growth is estimated to be the result of the direct effects of GH on growing bone (5, 58, 59), a finding described in animal studies and supported by studies of children with GH receptor insensitivity/deficiency. Patients with Laron syndrome treated with IGF-1 demonstrated lower growth rates compared to patients with GHD treated with daily GH, supporting the contribution to growth that both GH and IGF-1 provide (59, 60).


Recombinant human growth hormone (r-hGH) is used to treat: growth hormone deficiency in children and adults; children born small for gestational age; Turner's syndrome; and chronic renal failure. r-hGH is administered by daily subcutaneous injection and may be given using a number of different administration devices. The aim of this survey was, firstly, to identify which attributes of an r-hGH administration device are considered most important to physicians, teenage patients, parents of young children requiring GH and nurses who have experience of r-hGH administration, and, secondly, to determine how they rate existing devices in each of these key attributes.


Approximately 1 in 4000 children are born every year with growth hormone deficiency (GHD) [1]. GHD causes short stature, low growth velocity, excess subcutaneous fat and delayed skeletal maturation [1], which have a considerable impact on physical and psychological functioning [2]. Adults with untreated GHD also have an increased cardiovascular risk [3]. Replacement therapy using exogenous GH has been used successfully since the 1950s to treat children (and more recently adults) with GHD [4, 5].


Most important device attributes. Mean scores for the five attributes of a recombinant human growth hormone administration device considered most important by participants in the survey. Device attributes (19 in total) were assessed in the questionnaire completed by physicians, nurses and parents.


Comparative ratings for devices used previously. Comparative ratings for recombinant human growth hormone injection devices used previously by participants in the survey. Device attributes were assessed in the questionnaire completed by physicians, nurses and parents. Ratings are shown as mean scores for the five device attributes considered most desirable by survey participants.


A case of isolated growth hormone deficiency type IA (IGHD IA) caused by novel compound heterozygous mutation in the GH1 gene was reported in this study, which aimed to provide insights that will benefit future diagnosis and treatment.


No discernible abnormalities were found in the blood gas analysis, liver and kidney function assays, and serum electrolytes. The thyroid function indicators, including total triiodothyronine (TT3) (2.06 nmol/l), total thyroxine (TT4) (107.00 nmol/l), and free thyroxine (FT4) (1.15 ng/dl), were in the normal range. However, the TSH (6.97 mIU/l) level was elevated. The serum ACTH (29.80 pg/ml) and cortisol (414.16 nmol/l) levels were normal, but the IGF-1 (16.99 ng/ml) level was low, and the level of growth hormone was exceedingly low (


IGHD Type IA is an autosomal recessive hereditary disease. It is clinically characterized by short stature accompanied by a decreased growth rate and severe growth retardation in the first six months of life (with a height SDS score less than -4.50 SD). The GH levels of patients with IGHD Type IA are undetectable, and anti-GH antibodies occur after exposure to rhGH [6]. Although the initial reaction to rhGH treatment might be favorable, there is a tendency to produce antibodies that adversely affect treatment [7]. Although children with IGHD exhibit normal body length and weight at birth, GHD caused by GH1 gene mutations inhibits growth in body length after birth. Some patients may have small penises or exhibit fasting hypoglycemia. Other common characteristics reported in the literature include truncal obesity, a raised forehead, and a low and flat nasal bridge [8, 9]. Currently, most cases of IGHD reported in the literature are familial, and sporadic cases are rare. Therefore, IGHD should be considered as a differential diagnosis for children with severe growth retardation. Differential diagnosis was made on the basis of normal birth length and weight, growth retardation after birth, severe deficiency of growth hormone, combined with the results of gene mutation and pedigree analysis. For example, the children with Silver-Russel syndrome (RSS) (OMIM:180860) also showed short stature, but their growth hormone level were normal. Moreover, their birth length and weight were lower than those of children of the same gestational age due to intrauterine growth restriction, and they also had clinical characteristics such as postnatal feeding difficulties, special facial features, and limb asymmetry, which could be distinguished from IGHD IA. The need to improve early biochemical detection and genetic analysis is critical to provide a prompt etiological diagnosis, prevention, and treatment.


The facial and clinical features of the patient reported in this case were consistent with those reported in the literature for the most part. In this case, the patient exhibited severe growth retardation after birth until the initiation of treatment. Her growth hormone level (T) and a missing fragment inherited from her father, which resulted in a GH1 gene deletion. Based on the analysis of the family diagram, we speculated that both parents carried mutations. Therefore, two alleles, each with a mutation, were passed on to the child resulting in a compound heterozygous mutation of the GH1 gene, which produced the phenotypic characteristics of IGHD Type IA (Fig. 1c). The gene mutations reported in the literature that cause IGHD Type IA are summarized and compared with this case, as seen in Fig. 3.


IGHD can be divided into four types, including autosomal recessive (IA and IB), autosomal dominant (II), and X-linked (III). The phenotypic characteristics of IGHD IA have been described above. IGHD IB is primarily caused by GH1 or GHRHR mutations. Compared with IGHD IA, patients with IGHD IB exhibit more variable phenotypes. Children exhibit short stature, a slow growth rate, delayed bone age, low but detectable growth hormone concentrations, and respond favorably to exogenous growth hormone treatment [12, 13]. IGHD II is the most common genetic form of IGHD, and children show considerable variability in the time of onset and severity of GHD. Over time, they might develop deficiencies in other pituitary hormones [14, 15], requiring lifelong follow-up assessments. IGHD III can be caused by mutations in SOX3 or BTK genes [16], and patients often exhibit mental retardation, craniofacial malformations, hypoglycemia, and agammaglobulinemia [17,18,19]. The phenotypic characteristics of each genetic form of IGHD are similar, but they also exhibit specific phenotypic characteristics, which should be distinguished through clinical assessments. In addition, GH1 gene mutations can cause IGHD IB, IGHD II, and Kowarski syndrome. Patients with these conditions exhibit exceedingly short stature, although their specific clinical phenotypes are not identical because of the type and site of the mutations. Overall, the GH1 genotype is closely associated with the phenotype of short stature caused by the GH1 mutations.


Despite the increasing number of genes known to be involved in IGHD etiology, mutations in known genes account for only a small number of cases. Therefore, understanding emerging, newly discovered mutations can guide clinical diagnosis and facilitate patient treatment. Early application of rhGH to treat IGHD IA has demonstrated favorable results. However, antibodies to rhGH eventually are produced during treatment. Therefore, long-term follow-up assessments must be conducted. Also, the presence of circulating growth hormone antibodies will substantially influence the ongoing treatment of these children, making it difficult for them to reach their expected height. Thus, further research is needed to improve the prognosis for the affected children.


Most patients with childhood non-organic growth hormone (GH) deficiency (GHD) produce a normal GH peak as young adults. Our objectives were to better define this transient GHD and evaluate the factors influencing the growth response of patients with pituitary stalk interruption syndrome (PSIS). 041b061a72


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