Administration of Growth Hormone to Patients with Familial Dwarfism with High Plasma Immunoreactive Growth Hormone: Measurement of Sulfation Factor, Metabolic and Linear Growth Responses

Laron, Zvi; Pertzelan, A; Karp, M; Kowadlo-Silbergeld, A.; Daughaday, William H.

doi:10.1210/jcem-33-2-332

Cited by 205 publications

(69 citation statements)

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“…The oldest girl (VI-2), in fact, is now entering the seventh year of treatment with r-hGH and has maintained a low anti-hGH antibody titer and a satisfactory height velocity, whereas the other two children had the typical strong anabolic response to an initial course of r-hGH rapidly followed by the appearance of high-titer anti-hGH antibodies with growth arrest. Birth size in patients with IGHD type IA is extremely heterogeneous and does not seem to correlate with the deletion size, ruling out, together with other clinical and experimental evidence (20)(21)(22)(23), a role for GH in fetal growth. The low birth weights and lengths of the patients that we studied cannot be related to the absence of the other genes of the cluster either, because patients with the same deletion previously reported (11) had normal birth weights.…”

Section: Discussionmentioning

confidence: 96%

Isolated Growth Hormone Deficiency Type IA Associated with a 45-Kilobase Gene Deletion within the Human Growth Hormone Gene Cluster in an Italian Family

et al. 1994

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Section: Discussionmentioning

confidence: 96%

Isolated Growth Hormone Deficiency Type IA Associated with a 45-Kilobase Gene Deletion within the Human Growth Hormone Gene Cluster in an Italian Family

et al. 1994

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“…Laron syndrome is a hereditary form of dwarfism characterized by extremely low serum concentrations of insulin-like growth factor (IGF)-I, despite high concentrations of biologically active growth hormone (GH), and by resistance to exogenous GH (1)(2)(3)(4). In most of the patients, the disease is caused by defects in the GH receptor gene, leading to absence of a functional GH receptor (3,4), or by defects in post-receptor mechanisms (5).…”

Section: Introductionmentioning

confidence: 99%

Long-term effects of insulin-like growth factor (IGF)-I on serum IGF-I, IGF-binding protein-3 and acid labile subunit in Laron syndrome patients with normal growth hormone binding protein

Kanety

Silbergeld²,

Klinger³

et al. 1997

European Journal of Endocrinology

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A minority of patients with Laron syndrome have normal serum GH binding protein (GHBP), indicating that the defect is elsewhere than in the extracellular domain of the GH receptor. We have evaluated the effect of long-term IGF-I treatment on serum IGF-binding protein (IGFBP)-3 and the acid-labile subunit (ALS) in three siblings with Laron syndrome caused by a GH post-receptor defect and with normal GHBP. The children (a boy aged 3 years, a girl aged 4 years and a boy aged 10 years) were treated by daily s.c. injection of IGF-I in a dose of 150 mg/kg. IGFBP-3 was measured by RIA and Western ligand blotting, ALS by RIA. Basal values of IGFBP-3 and ALS were low. During IGF-I treatment, the IGFBP-3 concentrations in the girl gradually increased, whereas in the boys there was a 60% decrease during the first week, followed by gradual increase towards baseline. The ALS concentrations followed a similar pattern. We conclude that IGF-I treatment induces an initial suppression and then an increase in the IGFBP-3 and ALS concentrations, confirming data from animal experiments that IGFBP-3 synthesis is not solely under GH control. The differences in responsiveness between the female and male siblings may reflect genetic differences, or lower circulating concentrations of IGF-I in the boys compared with the girl. European Journal of Endocrinology 137 626-630

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“…In addition to having been observed in children with GH deficiency, hypercalcuria has been noted in normal children and adults, and in patients with such diverse conditions as Turner's syndrome [10], acromegaly [22], primordial dwarfism [39], dwarfism with elevated plasma GH levels (Laron dwarfs) [21], resistant rickets, renal osteodystrophy [16], and osteoporosis [13,30]. However, Vest et al [36] found hypercalcuria in only half of the normal infants whom they studied; Villee et al [37] failed to find it in one child with progeria, as did Root and Oski [27] in two of three senile adults.…”

Section: Introductionmentioning

confidence: 99%

Metabolic Response to Growth Hormone Administration, with Particular Reference to the Occurrence of Hypercalcuria

et al. 1972

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ExtractThe effect of human growth hormone (GH) administration (5 mg daily) was studied in 12 children with short stature, in an attempt to elucidate the cause of the hypercalcuria which so commonly occurs at the initiation of GH therapy. Complete metabolic balances were determined for Ca, P, N, Na, K, and Cl; and urine was analyzed for hydroxyproline, stable Sr, Pb, Mg, 137 Cs, F, and creatinine. Some subjects had determinations of basal metabolic rate (BMR) and of ultrafiltrable serum Ca. In keeping with the reports of others, levels of N, P, K, Na, and Cl in urine all declined, as did that of 187 Cs; Mg excretion fell slightly. Ten of the 12 children with short stature (6 of whom had subnormal plasma GH responses to a variety of stimuli) had hypercalcuria; 3 of these had an increase in urine Ca in the face of a low Ca diet (170 mg/24 hr). Fecal Ca declined in some subjects and rose in others. Ultrafiltrable serum Ca did not change.A majority of the subjects showed an increase in the urinary excretion of F, Sr, Pb, and hydroxyproline.The effect of the hormone was also studied in three patients with idiopathic hypoparathyroidism; two of these responded with an increase in urine Ca.The magnitude of the rise in urine Ca could not be correlated with changes in BMR, changes in creatinine clearance, or changes in Na excretion; it was positively correlated with the increases in Sr and Pb in urine. SpeculationWe have evidence that the hypercalcuria accompanying GH administration is not due to increased gastrointestinal absorption, alterations in serum Ca, or changes in metabolic rate or renal function; nor is it mediated via parathyroid activity. These considations, plus the fact that the hypercalcuria is accompanied by hydroxyprolinuria and an increase in the urinary excretion of three "bone seekers" (Sr, Pb, and F), lead us to postulate that the hormone enhances bone resorption, either per se or via "sulfation factor."

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Administration of Growth Hormone to Patients with Familial Dwarfism with High Plasma Immunoreactive Growth Hormone: Measurement of Sulfation Factor, Metabolic and Linear Growth Responses

Cited by 205 publications

References 1 publication

Isolated Growth Hormone Deficiency Type IA Associated with a 45-Kilobase Gene Deletion within the Human Growth Hormone Gene Cluster in an Italian Family

Isolated Growth Hormone Deficiency Type IA Associated with a 45-Kilobase Gene Deletion within the Human Growth Hormone Gene Cluster in an Italian Family

Long-term effects of insulin-like growth factor (IGF)-I on serum IGF-I, IGF-binding protein-3 and acid labile subunit in Laron syndrome patients with normal growth hormone binding protein

Metabolic Response to Growth Hormone Administration, with Particular Reference to the Occurrence of Hypercalcuria

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