Renal salt-wasting increases vasopressin excretion in preterm infants.
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Biomedical subjects
Publications and source records attributed to V Némethová.
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A retrospective analysis of the course of histiocytosis X in 16 children treated at our hospital over the past 15 years is presented. Nearly all of the patients were at generalized stages of the disease. In this study the staging according to Greenberg was used. The disease had a favorable course at Stages I and II, 1 patient at Stage III and 2 at Stage IV died. All the children were treated by chemotherapy, in some of them combined with radiotherapy. Individual patients differed from one another with respect to chemotherapeutic regimens. The actuarial survival rate was 78% for the whole group. Better results were obtained after a less intense but long-term treatment than after an intense but interrupted chemotherapy.
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Ten cases of monosomy X and caryotype 45,X, and 24 cases of the disorder with various forms of mosaicism are reported. The diagnostic significance of particular stigmata is underlined, and the possibility stressed to demonstrate a hidden pterygium colli by declining the patient's head laterally. Moreover, several peculiarities of individual cases are shown: In three girls with mosaicism, spontaneous puberty was observed, however, in two of them, it was followed by precocious menopause at the age of 14 and 18 years, respectively. The necessity of caryotyping in growth-retarded girls with secondary amenorrhoea or with a ren arcuatus or unilateral solitary kidney is emphasized. High values of urinary gonadotropins in girls without signs of puberty or with precocious menopause, indicate the need for substitution. This therapy leads to the appearence of secondary sex phenomena and menstruation, and at the same time prevents osteoporotic changes.
A short-term test to determine renal concentrating ability without any water deprivation has been described. It consists of osmolatity determinations in three onehour urine portions from spontaneous voidings following an intranasal application of 7 microng of the synthetic analogue of vasopressin-DDAVP per 0,5 m2 body surface. The values of maximal urine osmalatity over 900 mosm/Kg H2O following DDAVP are considered normal whereas values under 600 mosm are consistent with impaired renal function. If, however, the maximal urine osmolality is in the so called "uncertain range" i.e. from 600 to 900 mosm, it is advisable to perform in the same patients also the classical concentration test based on a prolonged dehydration (26 h).
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