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• W1969090239 abstract "Summary One hundred cases of children, below the age of 15 years, have been reported in the literature before June, 1945, with the diagnosis of Addison's disease. Sixty-two have been classified as proved , twenty-three as probable , andfifteen as doubtful . The following summary has been made of the proved cases: Age Incidence .—The onset of symptoms was first noticed in children: Under 2 yr. of age in three, or 4.9 per cent From 2 to 5 yr. of age in three, or 4.9 per cent From 5 to 10 yr. of age in seven, or 11.5 per cent From 10 to 15 yr. of age in forty-eight, or 78.7 per cent The age was not reported in one case Sex and Race Incidence .—Thirty-eight, or 61.3 per cent, were males and twenty-four or 38.6 per cent were females, giving an almost 2 to 1 incidence in favor of the males. This ratio is in agreement with that usually quoted in adults with tuberculosis of the adrenals. It is frequently stated, however, that the ratio is reversed in primary atrophy in adult patients. It is, therefore, of interest to note that the children with atrophy of the adrenals were all males. To my knowledge, no cases have been reported in the Negro race. Symptoms and Signs .—Fifty-three, or 85.5 per cent, of the cases manifested gastrointestinal symptoms some time during the course of the illness. No statement was made concerning the presence or absence of intestinal complaints in the remaining nine, or 14.5 per cent. The incidence of gastrointestinal symptoms, therefore, is probably much higher than that stated. Attacks of vomiting, diarrhea, and generalized abdominal discomfort were the most common complaints. Asthenia, anorexia, weight loss, dehydration, and general debility were present in practically all of the cases. In eight, or 12.9 per cent, there were repeated convulsions which might be interpreted as hypoglycemic in origin. A definite statement was made concerning the color of the skin in sixtypatients. Of these, fifty-six, or 93.3 per cent, had a generalized pigmentation. The duration of symptoms before death ranged from three days to four years. No definite statement was made in seventeen, or 27.4 per cent, of the cases. Of the remaining forty-five, in twenty-five, or 55.6 per cent, death occurred seven months or less after the onset of symptoms. Twenty, or 44.4 per cent, of the patients were ill for one year or longer. Mode of Termination .—By far the majority of these children presented the picture of a progressive debilitating illness, resulting in the usual slow death. However, twenty, or 32.2 per cent, of them exhibited dramatic episodes of convulsions, profuse diarrhea or vomiting, or sudden coma just prior to death. Etiology .—In one case the nature of the pathologic involvement of theadrenals was not given. Of the remaining sixty-one, fifty-three, or 86.9 per cent, were of tuberculous origin, fifty, or 94.3 per cent, showed bilateral involvement of the adrenals. Pulmonary tuberculosis was present in twenty-three, or 43.4 per cent, and tuberculosis of the mesenteric glands was present in seven, or 15.0 per cent, of the cases showing tuberculous adrenals. In five, or 8.0 per cent, of the cases there was atrophy of both adrenal glands without clinical or pathologic evidence of tuberculosis. In three, or 4.8 per cent, macrogenitosomia was an associated finding. Of the six cases, in which the patients were alive at the time of the report, two were diagnosed atrophy; two, hyperplasia; and two, tuberculosis of the adrenals. In adults, pyogenic abscesses, vascular lesions, amyloid degeneration, mycosis fungoide and echinococcus cysts have been responsible for destruction of the adrenal glands resulting in the development of Addison's disease. To my knowledge, these etiological agents have not been responsible for this disease in children. Treatment .—Although in the older literature there was an occasional referenceto the use of crude adrenal extracts, it was not until 1927 that Hartman and associates 104 demonstrated that Interrenalin prolonged the lives of adrenalectomized dogs. The period between 1936 and 1941 105–110 produced important contributions in the isolation of various fractions of the whole gland which put potent extracts at the disposal of the clinician. For these reasons, and the lack of knowledge until 1932 4 concerning the importance of electrolyte metabolism, these patients could not have received adequate therapy until after this time. In Butler, Ross, and Talbot's case, 59 associated with macrogenitosomia,there was sudden collapse on restriction of fluids or sodium chloride but the patient was apparently well-regulated on the addition of 2 to 3 Gm. of sodium chloride to the diet, without the use of extracts. Ginandes' patient, 62 although probably having tuberculous adrenals, waswell-adjusted on daily injections of whole cortical extract. In addition, a diet high in sodium-chloride and low in potassium was prescribed. During the acute phase of the illness a dramatic response was obtained with normal saline and glucose intravenously. In the other case associated with macrogenitosomia the patient was regulated by substituting physiologic sodium chloride solution for water in the formula and the addition of 0.2 c.c. desoxycorticosterone acetate by injection daily. At 9 months of age the therapy was changed to 3 drops of desoxycorticosterone in propylene glycol, five to six times daily sublingually. Anderson's 64 11-year-old patient with probable atrophy of the adrenals was given 5.0 mg. desoxycorticosterone acetate and 6.0 c.c. whole gland extract daily, by injection. The therapy was later changed to 5.0 mg. desoxycorticosterone in propylene glycol twice daily sublingually. Rosin and Friedman's patient, 65 a case of adrenal atrophy, was improving on injections of desoxycorticosterone, but he was lost sight of. Jaudon's patient, 66 a probable case of tuberculosis of the adrenals, has been well controlled for over two years by injections of 1.5 to 2.0 mg. desoxycorticosterone acetate daily. Recently, four 125 mg. desoxycorticosterone acetate pellets were implanted and daily injections are no longer necessary. The child has remained in good health." @default.
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• W1969090239 date "1946-06-01" @default.
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• W1969090239 title "Addison's disease in children" @default.
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• W1969090239 doi "https://doi.org/10.1016/s0022-3476(46)80072-6" @default.
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