Managing the child with severe primary insulin-like growth factor-1 deficiency (IGFD): IGFD diagnosis and management.

Growth failure associated with severe primary insulin-like growth factor 1 (IGF-1) deficiency (SPIGFD), a condition defined as basal IGF-1 standard deviation score (SDS) less than or equal to -3 and height SDS less than or equal to -3 in a child with normal or elevated levels of growth hormone, can be successfully treated with the recombinant human IGF-1 mecasermin. In this review, we describe the most safe and effective way to use mecasermin in the treatment of patients with SPIGFD, including how to initiate dosing, key side effects, and how to monitor treatment. Finally, mention of how to reinitiate therapy is made, given the recent drug shortage with mecasermin.

Age of thelarche and menarche in contemporary US females: a cross-sectional analysis.

Aim: A recent secular trend towards earlier thelarche has been suggested. The aim of this study is to examine normative ages of thelarche and menarche in contemporary US females.

Methods: Trained physicians documented Tanner breast stage by observation in a cross-sectional cohort.

Effectiveness of self- or partner-administration of an extended-release aqueous-gel formulation of lanreotide in lanreotide-naïve patients with acromegaly.

Surgical resection is often not curative in patients with acromegaly and long-acting somatostatin analogues (lanreotide or octreotide) are often needed. This study assessed the efficacy and safety of self- or partner-administration of lanreotide in patients with acromegaly. This was a six-month, single-arm, open-label study conducted at 13 endocrinology clinics.

Leukemia in children treated with growth hormone.

An association between treatment with growth hormone (GH) and the development of leukemia was described in 1988. This perceived association is best explained by the fact that there are more children with GH deficiency (GHD) with risk factors predisposing them to leukemia than in the general population. These factors include previous cancers and their treatment, as well as co-existing conditions such as Down, Bloom and Fanconi syndromes.

Indications and recommendations for the use of recombinant human growth hormone in adult short bowel syndrome patients dependent on parenteral nutrition.

Given the lack of published guidelines regarding the use of trophic factors to treat patients with short bowel syndrome (SBS), a group of experts in the field convened to discuss best-practice strategies. Trophic factors, such as recombinant human growth hormone (r-hGH) and glucagon-like peptide-2 (GLP-2), may enhance intestinal adaptation and decrease parenteral nutrition (PN) requirements; therefore, their utility in treating SBS patients was evaluated. Available clinical data on use of r-hGH therapy in SBS patients were discussed, as were the utility of r-hGH in the PN weaning process, the optimal timing of r-hGH therapy, and how to select appropriate patients for r-hGH therapy.

Treatment of adult short bowel syndrome with recombinant human growth hormone: a review of clinical studies.

Extensive resection of the intestinal tract frequently results in inadequate digestion and/or absorption of nutrients, a condition known as short bowel syndrome (SBS). Several therapies, including parenteral nutrition (PN), bowel rehabilitation, and surgical procedures to reconstruct the bowel, have been used for patients with SBS. However, these treatments only partially correct the underlying problem of reduced bowel function in some patients.

Patient outcomes in the GHMonitor: the effect of delivery device on compliance and growth.

To determine the effect of method of growth hormone (GH) administration on patient outcomes, we studied data from the GHMonitor, an electronic database registry of North American children treated with Saizen GH (somatropin [rDNA origin] for injection). Data from 631 children, 305 treated with needle and syringe and 326 treated with cool.click needle-free device, were analyzed.

An overview of the GHMonitor, a registry of children treated with Saizen somatropin [recombinant hGH for injection].

The GHMonitor, introduced in 1998, monitors demographics and outcomes in children treated with Saizen (somatropin [recombinant hGH for injection]). Follow-up data are available on 697 patients. The proportion of male to female patients receiving growth hormone (GH) treatment was 67:33.

Pharmacokinetic and pharmacodynamic characteristics of a long-acting growth hormone (GH) preparation (nutropin depot) in GH-deficient children.

Long-term GH replacement therapy is indicated for children with growth failure due to GH deficiency (GHD). We evaluated the feasibility of administering a long-acting GH preparation [Nutropin Depot (somatropin, rDNA origin) for injectable suspension] to prepubertal children with GHD by examining pharmacokinetic and pharmacodynamic response parameters after single or multiple doses. Data were collected from three studies involving 138 children treated with Nutropin Depot 0.

Neonatal hypoglycemia in a growth hormone registry: incidence and pathogenesis.

Objective: To examine the characteristics of infants with neonatal hypoglycemia treated with growth hormone (GH) in order to gain insights into factors aiding in the identification of and timely treatment of hypopituitary neonates.

Study Design: The National Cooperative Growth Study (NCGS) database was examined to identify infants with neonatal hypoglycemia started on GH by 6 months of age. 169 infants (100 males, 69 females) were found and their data analyzed for physical characteristics, the presence of other hormone deficits, and the diagnostic methods used.

Screening girls with Turner syndrome: the National Cooperative Growth Study experience.

The National Cooperative Growth Study (NCGS) database was examined to determine whether the availability of expert guidelines affected the clinical management of 955 patients with Turner syndrome (TS). Although cardiac and renal evaluations increased in frequency after guideline publication, hearing screenings declined. Although girls with TS show significant cardiac, renal, and hearing problems, screening for these disorders remains inadequate.

Adverse events with rhGH treatment of patients with chronic renal insufficiency and end-stage renal disease.

Objective: Recombinant human growth hormone (rhGH) has been used to improve the growth retardation associated with chronic renal insufficiency (CRI) and end-stage renal disease. We determined the incidence of one of four targeted adverse events (AEs): malignancy, slipped capital femoral epiphysis (SCFE), avascular necrosis (AN), and intracranial hypertension (ICH).

Study Design: During a 6.

Do growth hormone (GH) serial sampling, insulin-like growth factor-I (IGF-I) or auxological measurements have an advantage over GH stimulation testing in predicting the linear growth response to GH therapy?

Objective: To compare the relative utility of GH secretion via pharmacological stimulation, overnight serial sampling, IGF-I levels and auxological variables as predictors of change in height standard deviation score (deltaHt SDS) during GH treatment.

Design: A multicentre observational study.

Patients: Prepubertal children (n = 825) with idiopathic growth failure who were subsequently treated with GH were divided into two groups, based on their maximum GH response to pharmacological stimulation testing: (1) idiopathic GH deficiency (IGHD), defined by a maximum GH response < 10 microg/l (n = 300); and (2) idiopathic short stature (ISS), with a maximum GH response > or = 10 microg/l (n = 525) (GH conversion factor: 3 IU = 1 mg).

The pharmacokinetic and pharmacodynamic characteristics of a long-acting growth hormone (GH) preparation (nutropin depot) in GH-deficient adults.

A pharmacokinetic-pharmacodynamic study of a long-acting GH [Nutropin Depot; somatropin (rDNA origin) for injectable suspension] was performed in 25 patients with adult GH deficiency. Single doses of 0.25 mg/kg and 0.

A long-acting human growth hormone (Nutropin Depot): efficacy and safety following two years of treatment in children with growth hormone deficiency.

Background: Nutropin Depots [somatropin (rDNA origin) for injectable suspension] is a long-acting form of human growth hormone (GH) to be administered by subcutaneous (s.c.) injection.

Septo-optic dysplasia/optic nerve hypoplasia: data from the National Cooperative Growth Study (NCGS).

We analyzed data from 65 children with septo-optic dysplasia (SOD) referred for evaluation and followed in the National Cooperative Growth Study (NCGS) Substudy 8 and from 758 children treated with growth hormone (GH) and followed in the NCGS core study. Compared to other children referred for evaluation of short stature, children with SOD were younger (mean age 3.7 +/- 3.

Use of magnetic resonance imaging in short stature: data from National Cooperative Growth Study (NCGS) Substudy 8.

The primary use of magnetic resonance imaging (MRI) in the evaluation of children with short stature (SS) is to discover lesions in the central nervous system (CNS), particularly tumors that may require intervention. MRI has a secondary role in identifying structural abnormalities responsible for growth hormone deficiency (GHD). We examined data from the National Cooperative Growth Study (NCGS) Substudy 8 to determine how American physicians are using MRI in evaluating children with SS.

Early initiation of growth hormone treatment allows age-appropriate estrogen use in Turner's syndrome.

Because estrogen (E) accelerates skeletal maturation it can decrease final height attainable with GH therapy in girls with Turner's syndrome (TS). Nonetheless, as age-appropriate E administration does have psychobehavioral benefits for such patients, we asked whether E treatment in TS could occur without adverse impact on final adult height if GH therapy were started at an earlier age. Near adult height (NAH) was assessed in 344 girls with TS, who had received both GH and E and were followed in the National Cooperative Growth Study database.

Growth hormone safety update from the National Cooperative Growth Study.

We reviewed adverse event (AE) data in the National Cooperative Growth Study from start-up (1985) until January 1, 1999. Enrollment was 33,161. A total of 2,632 AE reports were received; 863 were serious events, with 156 deaths.

Body mass index (BMI) in Turner Syndrome before and during growth hormone (GH) therapy.

Objective: To study whether body mass index (BMI) is different in girls with Turner syndrome (TS) compared to normal girls, and whether BMI in TS is affected by growth hormone (GH) treatment.

Design: A retrospective cross-sectional study.

Subjects: 2468 girls with TS enrolled in the National Cooperative Group Study (NCGS), a collaborative surveillance study for assessing GH-treated children.

The impact of recombinant human growth hormone treatment during chronic renal insufficiency on renal transplant recipients.

Objective: To evaluate post-transplant outcomes for patients treated with human growth hormone (rhGH) during the course of chronic renal insufficiency (CRI).

Study Design: Patients (the "cohort" group) were identified who had been enrolled in 2 controlled studies to determine the efficacy and safety of rhGH in growth-retarded children with CRI and were subsequently enrolled in the North American Pediatric Renal Transplant Cooperative Study (NAPRTCS) and received a renal transplant. Patient survival, graft survival, time to first acute rejection episode, causes of graft failure, adverse events, and serial growth data from transplant to 60 months were evaluated.

Growth hormone stimulation test results as predictors of recombinant human growth hormone treatment outcomes: preliminary analysis of the national cooperative growth study database.

Growth hormone (GH) stimulation tests are considered a prerequisite to clinical trials of recombinant human GH (rhGH) therapy, but the test results may not be predictive of the treatment outcomes with rhGH. We examined the GH stimulation test results as a predictor of the treatment outcome in a cohort of prepubertal subjects in the National Cooperative Growth Study. A standard is proposed in which a diagnosis of GH deficiency is considered appropriate when a patient has significant first-year catch-up growth and that a positive stimulation test result predicts this outcome.

Combined use of growth hormone and gonadotropin-releasing hormone analogues: the national cooperative growth study experience.

Gonadotropin-releasing hormone ana-logues (GnRHa) are used to treat central precocious puberty. They also are used to delay puberty in short children with a prognosis for impaired adult height. In both cases, growth hormone (GH) treatment is sometimes added.

The effect of growth hormone treatment on stature in Aarskog syndrome.

We describe 19 males with Aarskog syndrome who were treated with growth hormone (GH) and enrolled in the National Cooperative Growth Study (NCGS). There was a significant increase in both growth rate (3.9 +/- 1.