Laron dwarfism: Growth and immunoreactive insulin following treatment with human growth hormone

Growth Hormone Secretion in Protein Energy Malnutrition

“…In fact, in 4 of the 5 subjects presented in Table 2, insulin levels were found to be low, an unexpected finding in the presence of high hGH levels. Similar findings in cases of Laron type of dwarfism have been interpreted to be due to a possible lack of insulinotropic effect in the hGH molecules secreted by these patients (24). The absence of an insulin response to IV glucose in cases of PEM may be considered as a compensatory mechanism protecting the organism and particularly the nervous system from glycopenia.…”

Growth Hormone Receptor Deficiency (Laron Syndrome): Clinical and Genetic Characteristics

“…None of the patients experienced delays in learning to talk. 10,13 4,8,9,II,12 9,16 The intelligence of patients reported in previous studies is..summarized in Table 2. In contrast to the Israeli patients and others reported, the· Ecuadorean patients were highly intelligent, with few exceptions.…”

“…Laron was aware of 43 other patients in non-Jewish populations (3), and some 30 of these have been reported. They include patients from Lebanon (4), Pakistan (5), Spain (6), Tunisia (7), Italy (8,9), Mexico (10), France (9), Denmark (I 1), the Netherlands (12), Saudi Arabia (13), Sardinia (14), Canada (15), Brazil (16), and the USA (17,18). A group of 20 patients with a distorted sex distribution (19 female: I male) from Loja province in southern Ecuador was reported recently (19).…”

Some Properties of the Plasma hGH Activity in Patients with Laron-Type Dwarfism Determined by a Radioreceptor Assay Using Human Liver Tissue

“…In 1966, investigators from our Institute described a new syndrome of familial dwarf ism [16] which subsequently came to be termed Laron-type dwarfism [LTD] in the literature [6], In its clinical picture and in many of the laboratory findings, it is indistin guishable from isolated growth hormone de ficiency (1GHD) [18], but it is characterized by high levels of plasma immunoreactive hu man growth hormone (IR-hGH) and low lev els of serum somatomedin [19], Since then additional patients have been reported from our Institute [20] and by others [4,15,24,26,29], This syndrome was first suspected to be caused by an abnormal hGH molecule [16], but later evidence pointed towards resistance to exogenous hGH [5,6,10,19,21] and to an abnormality in the hGH receptors [8]. In the present investigation we compared binding conditions of plasma hGH of normal, acro megalic and LTD patients to hGH receptors in human liver microsomes.…”