Effects of the infusion of insulin-like growth factor I in a child with growth hormone insensitivity syndrome (Laron dwarfism)

Article Abstract:

Many patients with growth retardation have a relative lack of the pituitary hormone called growth hormone. These patients respond to the administration of growth hormone by increasing growth, and generally achieve a taller height than would have been achieved without the treatment. However, not all patients with growth retardation have abnormally low growth hormone. The growth disorder characterized by high levels of growth hormone and no response to the administration of growth hormone is referred to as Laron dwarfism. (Some medical researchers prefer to call the syndrome growth hormone insensitivity syndrome, which is not only more descriptive, but avoids the potentially objectionable word 'dwarf'.) Data obtained from research on laboratory animals has indicated that many of the effects of growth hormone are made possible through the action of another hormone called insulin-like growth factor I (IGF-I). It was conjectured that some patients with growth hormone insensitivity syndrome may be able to respond to IGF-I, even if their response to growth hormone was abnormal. This notion was tested in the treatment of a nine-year-old boy with growth hormone insensitivity syndrome. The patient was about 3.5 feet tall, which is shorter than most 5-year-old children. Repeated measurements were made of urea nitrogen in the blood and urine, as well as calcium, phosphate, and other substances which reflect the metabolism of the body. These same measurements were continued while the patient was treated for one week with IGF-I. Changes observed in blood and urine analyses were consistent with an anabolic shift. (Anabolic refers to the aspects of metabolism involved with building the body.) When the treatment was discontinued, the laboratory values reverted to their pretreatment amounts. The results indicate that an anabolic response can be achieved in patients with growth hormone insensitivity syndrome using insulin-like growth factor I. More research needs to be conducted to determine optimal dosages and to evaluate possible disruptions of the hormone system which might complicate the long-term use of this treatment for affected individuals. (Consumer Summary produced by Reliance Medical Information, Inc.)

Health aspects, Drug therapy, Growth disorders, Insulin-like growth factor 1, Insulin-like growth factor I, Dwarfism

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The molecular biology of Laron dwarfism and medullary thyroid cancer

Article Abstract:

Two articles in the October 12, 1989 issue of the New England Journal of Medicine demonstrate the value of using DNA analysis techniques to understand disease at the molecular level. The genetic studies of families with Laron dwarfism and multiple endocrine neoplasia type 2a (MEN2a) required accurate clinical diagnosis and illustrate many molecular biology techniques that, in the former report, revealed a genetic mutation responsible for defective growth hormone-receptor, and, in the latter, a new method of screening carriers of a familial disease. The Laron dwarfism study will enhance understanding of other conditions that involve abnormalities of growth hormone-receptors. Identification of the chromosomal abnormality in family members of MEN2a patients will increase the effectiveness of genetic counselling, preventive medical screening and early treatment of patients and family members. For example, the type of tumors occurring in families with MEN2a were found to be similar, and thus easier to identify in susceptible family members. The sensitivity of the genetic screening techniques described in this study will lead to preventive, diagnostic, and therapeutic interventions for other diseases. These research studies are strong examples of the essential partnerships that must be developed between medical clinicians and molecular biologists. (Consumer Summary produced by Reliance Medical Information, Inc.)

Research, Diagnosis, Genetic aspects, Human genetics, Molecular biology, Familial diseases, Endocrine gland diseases, Endocrine diseases, editorial

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