Biomedical subjects
Publications and source records attributed to R Stanhope.
Eight patients from M.D. Anderson Hospital underwent Williams' vulvovaginoplasty for sexual rehabilitation following pelvic exenteration. In 7 of the 8 patients the functional results were excellent, and none of the patients experienced morbidity. This surgical procedure is less extensive than other reconstructive methods and skin grafting or mechanical dilatation are rarely required.
When unlimited supplies of biosynthetic human growth hormone (hGH) became available in the late 1980s, the envisaged potential of its therapeutic applications was enormous in that it was hoped that perhaps most short children would be able to be made taller. However, this seems to have been far too simplistic a view and certainly in those children with various growth disorders treated with pharmacological doses of growth hormone (GH), the potential for increased final height does not appear to be fulfilled, with the exception of girls with Turner's syndrome. Further challenges in the growth field involve the manipulation of pubertal development during concomitant GH therapy.
We describe 65 children (32F, 33M) with psychosocial short stature from 51 families. Average age was 6.6 years (range 0.9-16.5) and all but five were prepubertal. 67% of the patients lived in families with three or more children, but in 73% of cases the patient was the first or the second born child. 45% of the parents were divorced and in 31% of the families the father was unemployed. In 56 children, the birth weight was known and in only 29% was it above 3000 g; 21% were premature, 29% had features of low birth-weight syndrome (including four with Russell-Silver syndrome). Average birth weight was 2786 g (range 1650-4676). In all patients, the predominant reason for referral was growth failure. In 28% an environmental aetiology was suspected and in a further 29%, social or emotional problems were known to the referring physician but not suspected as the aetiology of the growth failure, despite social services involvement in 60% at the referral to our unit. At initial presentation in our clinic, we found additional features leading to the suspicion of psychosocial short stature; 54% abnormal eating pattern, 42% behaviour problems, 26% encopresis, 18% nocturnal enuresis and 12% inappropriate urination. During the observation period of a mean of 3.7 years, 27 (41%) of our patients were found to have been sexually or physically abused. In these 27 children hyperphagia, bizarre eating habits, behaviour problems, soiling and nocturnal enuresis were more common.
OBJECTIVE: To assess the incidence and associated risk factors of adverse reactions of DDAVP treatment of children with diabetes insipidus, comparing different routes of administration. DESIGN: We retrospectively studied 103 children (44 females, 59 males) with cranial diabetes insipidus (mean age 6.9 years at diagnosis) treated with intramuscular (59), intranasal (84) and/or oral (64) DDAVP, over a mean follow-up period of 5.2 years. RESULTS: Eight patients died. For at least two children death was related to water intoxication. Major complications (symptomatic water overload with or without seizures) or asymptomatic hyponatraemia were observed in 33 patients. The incidence of total complications was significantly higher in cortisol deficient patients than in those with normal cortisol reserve (36% vs 6%). In patients on concomitant carbamazapine treatment major complications were more frequent in comparison to the remaining patients (33% vs 10%). Although not achieving significance, there were fewer complications using the oral route. CONCLUSIONS: Caution is needed in managing patients with DI, especially if risk factors such as cortisol deficiency or concomitant carbamazepine treatment are present. The oral route of administration seems to be preferred for both convenience and safety. Major changes in dose and formulation should be undertaken in hospital.
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One of the most important criteria for good health in childhood is normal growth. Taking regular accurate measurements of length and plotting them on a centile chart is essential to spot early signs of growth disorders. Be alert for a "zig-zag" pattern on the chart: it could indicate psychosocial dwarfism (see opposite). Length is more important than weight for identifying growth disorders. Lack of love, or an adverse emotional or social environment, can cause growth failure even in a child who is eating enough. Such children have a condition called psychosocial dwarfism, which is due to hypopituitarism (too little growth hormone secretion from the pituitary gland). This condition does not respond to growth hormone treatment. Once the child is placed in an alternative environment, eg a good foster home, the hypopituitarism is reversed and rapid "catch-up" growth takes place. It often emerges that such children have been physically, emotionally or sexually abused.
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