Acta paediatrica Scandinavica. Supplement最新文献

The effect of a 6-month period of substitution therapy with recombinant human growth hormone (rhGH) on muscle fibre size and muscle fibre type proportions has been investigated in a group of 13 adults with growth hormone deficiency. All had a peak growth hormone (GH) response to insulin-induced hypoglycaemia of less than 7 mU/l. There was no statistically significant change in the lesser fibre diameter or fibre proportions of either type 1 or type 2 muscle fibres in the rhGH group, as compared with placebo.

A multicentre trial with recombinant somatropin was initiated in West Germany in early 1986. Acceptance of patients to the study was determined according to criteria outlined in a detailed study protocol. A total of 62 patients with GH deficiency has now been treated with recombinant somatropin for a minimum of 12 months. Of these, 34 were previously untreated and 28 had previously received pituitary GH. Recombinant somatropin, 12 IU/m2/week, was administered subcutaneously, divided into six doses. Height velocities increased from 3.4 cm/year (pretreatment) to 10.4 cm/year in the previously untreated group, and from 6.0 cm/year during the last year on pituitary GH to 8.3 cm/year for the previously treated patients. Tolerance of recombinant somatropin was good, and no anti-GH antibodies were detected in any of the patients.

The three commonly used methods of height prediction employ various combinations of anthropometric data and bone age. In normal children, the regression type methods are preferable, though they do not perform well in the more severe disorders of growth. They are extremely dependent upon the limitations of the method of bone age determination that is used. A major source of error is the inability to predict the timing or the intensity of the adolescent growth spurt. Until this proves possible it is unlikely that significant improvements can be made.

The aim of surgical orthopaedics in short stature is to increase overall height and improve body proportions. It is particularly applicable to lower limb lengthening in achondroplasia, hypochondroplasia, Turner's syndrome and Ellis-van Creveld syndrome. Two methods are routinely used in Verona: chondrodiatasis and callotasis. Although simultaneous lengthening of tibia and femur of the same limb have generally been employed, recent efforts have concentrated on simultaneous lengthening of one femur and the contralateral tibia. Lengthening of up to 20-25 cm can now be achieved, with a complication rate below 20%.

With the presently available RIAs, serum levels of IGF-I, IGF-II and somatomedin binding protein can be determined specifically. Basal IGF-I and binding protein levels are low in GH deficiency, and normality of these parameters virtually excludes the condition. If, in a given clinical situation, auxological criteria and IGF-I (binding protein) levels suggest GH deficiency but the diagnosis is rejected by conventional stimulation tests, a further diagnostic work-up is needed, including measurements of spontaneously secreted GH. IGF measurements may serve as tools to disclose the unknown pathogenesis in growth disorders. Short-term responses of IGFs to exogenous GH may assist in defining the GH doses required to induce long-term growth.

The evaluation of episodic GH release is made difficult by the apparently random nature of GH secretory bursts, the frequent occurrence of minimally detectable plasma GH concentrations, the relatively rapid plasma disappearance rate of endogenous GH, and the large number of metabolic and environmental cues that alter GH dynamics. Nonetheless, the development of objective, statistically based, and reproducible computerized algorithms to quantify episodic GH release has offered new insights into the pathophysiological regulation of GH secretion in health and disease. Moreover, the recent formulation of algebraically explicit biophysical models of GH secretion and clearance has made possible a complete quantitative description of GH secretory and clearance dynamics over a full 24 hours of observation. Such analytical tools allow investigators to enumerate with statistically bounded confidence limits the number, amplitude, durations, and temporal locations of all significant underlying secretory bursts and simultaneously calculate the half-life of endogenous GH disappearance from all GH concentrations and their variances considered together. Accordingly, in conjunction with contemporary refinements in GH assay techniques, such novel approaches to dissecting the temporal structure of GH secretion and clearance in vivo should result in significantly enhanced understanding of GH dynamics in health and disease.

Immunoreactive and receptor-reactive IGF-I was found to be present in human urine; 30% of the IGF-I immunoreactivity in urine was in its free form and the remainder was a high molecular weight form (approximately 40,000 MW). Urinary IGF-I was quantified by radioimmunoassay after extraction by Sep-Pak C18 cartridge, a method that measures only the free form of IGF-I. Daily (24-hour) urinary IGF-I excretion was measured in 3 hypopituitary children and 16 short normal children. The IGF-I level in the 24-hour urine samples correlated with the plasma IGF-I level and the mean 24-hour plasma GH concentration. The mean 24-hour plasma GH concentration, however, correlated better with the GH level in the 24-hour urine samples and the plasma IGF-I level than with the urinary IGF-I value. The mean IGF-I levels in single urine samples from normal subjects lay between those from patients with acromegaly (which were high) and those from patients with hypopituitarism (which were low). There were overlaps, however, in individual values between the normal and hypopituitary patients. These data indicate that urinary IGF-I values are altered by the GH secretion state, though the clinical application of urinary IGF-I measurement may be limited.

The diagnosis of Cow's Milk Protein Allergy was considered in 303 infants aged less than 1 year, who presented with one or more of the following symptoms: acute reaction related to cow's milk proteins (CMP) ingestion, severe colics, persisting vomiting, protracted diarrhea with or without blood and mucus, failure to thrive, eczema, respiratory symptoms, such as chronic rhinitis and wheezing. A diagnosis of CMPA was confirmed in 148 cases (60%): 125 relapsed on milk challenge, 23 were not challenged because of acute reactions at onset, presence of specific IgE (RAST and prick), and improvement on milk free diet. Familial atopy, familial history of CMPA and previous acute gastroenteritis were significantly more frequent in cases than in 191 age matched controls. Breast feeding was not more common or of longer duration in controls, compared to cases. Mean IgE serum levels were higher (46.3 U/ml) in cases than in controls (17 U/ml), while specific Cow's Milk Protein IgE were found in 71/148 cases (48%). 15 infants entered the study while on breast milk, because of the confirmed relation between their symptoms and CMP on the maternal diet. These infants had a higher prevalence of IgE mediated problems. All cases improved on a milk free diet but in 26 (17.8%) a further modification of the diet was required after the first prescription. Milk challenge was monitored by simple laboratory tests: all cases who had symptoms on challenge showed at least one test modification. Six infants, with no history of acute reaction, showed severe self-limited clinical symptoms at challenge. Key words: cow's milk allergy, milk, allergy, prick test, eczema, diarrhea.