Vincent C. Kelley

Blood Adrenocorticotrophin in Children with Congenital Adrenal Hyperplasia.

Summary The oxycellulose technic has been applied to the analysis of blood ACTH in children. ACTH was not present in detectable quantities in the blood of afebrile children without endocrine disease. ACTH was detected in the blood of untreated, but not of cortisone-treated, children with adrenogenital syndrome.

Acute phase reactants

Summary 1. Serum nonglucosamine polysaccharides were determined in fifty-seven patients with rheumatic fever or related disease in an active phase and in thirty patients with inactive rheumatic fever. 2. Elevated serum levels of these substances were observed consistently in every individual who was studied during the active phase of any of these diseases with the exceptions of one patient suffering with active Sydenhams chorea and one patient suffering with active lipoid nephrosis. 3. Many, but not all, patients with inactive rheumatic fever exhibited normal serum levels of nonglucosamine polysaccharides. Statistical analysis reveals a significant difference between the serum nonglucosamine polysaccharide levels of the inactive rheumatic fever patients studied and the control group.

Studies of 17-hydroxycorticosteroids

Summary Plasma 17-hydroxycorticosteroidconcentrations were found to be elevated markedly in seven patients with salicylate intoxication and in guinea pigs given large doses of sodium salicylate intraperitoneally. Guinea pigs given sodium lactate or smaller amounts of sodium salicylate, intraperitoneally, were found to have levels of these steroids which were not significantly different from those of untreated animals.

Symptomatology resulting from withdrawal of steroid hormone therapy.

Several different types of clinical phenomena produced by suddenly withdrawing or decreasing the dose of steroid in patients receiving hormone therapy are discussed and illustrated by case reports. These include reoccurrence of symptomatology of the patients disease, the occurrence of symptomatology of “steroid pseudo-rheumatism” and the occurrence of toxic reactions associated with adrenal cortical insufficiency. The steroid dose should be increased in severe reactions of these types and later gradually decreased to levels satisfactory for maintenance therapy. Data are presented illustrating that steroid dependency may be induced in normal individuals by a single large steroid load and that during the ensuing hours transient rheumatic symptoms may appear. Es discutite e illustrate per reportos de casos, varie typos de phenomeno clinic que es producite per le subite discontinuation o reduction del dosage de steroide in patientes sub tractamento hormonal. Iste phenomenos include le recurrentia del symptomatologia del morbo del patiente, le occurrentia del symptomatologia de “pseudo-rheumatismo steroide,” e le occurrentia de reactiones toxic del genere associate con insufficientia adreno-cortical. In sever reactiones de iste genere, le dose de steroide deberea esser augmentate e reducite plus tarde gradualmente usque a nivellos satisfactori pro le therapia de mantenentia. Es presentate datos que illustra le facto que dependentia de steroides pote esser inducite in individuos normal per un sol massive carga de steroide e que durante le horas que seque, transiente symptomas rheumatic pote manifestar se.

Studies of 17-hydroxycorticosteroids in children

Summary 1. The blood levels of circulating 17-hydroxycorticosteroids in children with and without evident disease have been studied and are reported. 2. The data obtained do not justify an interpretation that the diseased groups differ significantly from the “well-control” group, with the exception of the group of children with congenital adrenal hyperplasia. 3. Serial values for 17-hydroxycorticosteroids in patients with rheumatic fever receiving hormone therapy are reported.

Acute phase reactants: II. Serum hexosamines in patients with rheumatic fever and related diseases*

Summary 1. Serum hexosamines were determined in 147 patients with rheumatic fever and related diseases. 2. Elevated serum levels of these substances were observed at some time during the illness of all but three of forty patients suffering with acute rheumatic fever but in none of forty patients with convalescent rheumatic fever and in only three of forty patients with inactive rheumatic fever. 3. Only one of ten patients suffering with Sydenhams chorea exhibited an elevated serum level of hexosamines. 4. The serum hexosamine levels were elevated in most but not all patients with other related diseases included in this study.

Species Differences in Circulating 17-Hydroxycorticosteroid Concentrations.∗

Summary 1. Quantitative determinations of 17-hydroxycorticosteroid concentrations in the peripheral blood of rabbits, rats, guinea pigs and human subjects were made. 2. Considerable species differences in these concentrations were noted. These differences appear to be quantitative as well as qualitative. 3. Of the species observed, the guinea pig appears to be the best experimental animal for studies of 17-hydroxycorticosteroids.

Comparison of eosinophil and circulating 17-hydroxycorticosteroid responses to epinephrine and ACTH.

Summary 1. A comparison of the responses of eosinophils and of circulating 17-hydroxycorticosteroid plasma concentrations; is presented. 2. In general, responses of eosinophils and 17-hydroxycorticosteroids to the injection of ACTH are adequate, with a reduction of the former and an elevation of the latter. 3. Certain exceptions to this generalization are noted. On the basis of these exceptions the question is raised whether the eosinopenic response to ACTH need be mediated by the mechanism previously accepted. 4. There is a strong suggestion that the eosinopenia in response to epinephrine is not mediated by an increased circulating concentration of 17-hydroxycorticosteroids secreted by the adrenal cortex.

Paper Electrophoresis of Duodenal Fluid from Patients with Cystic Fibrosis of Pancreas

Summary Duodenal fluids from patients with cystic fibrosis of the pancreas and from control subjects were studied by paper strip electrophoresis. Components with greater electrophoretic mobility than any in normal duodenal fluid occurred consistently in cystic fibrosis duodenal fluids. The most rapidly migrating of these fractions was identical in electrophoretic mobility to the albumin fraction of normal human serum.

Rationale for hormone therapy in rheumatic fever.

The dramatic response of acute manifestations of rheumatic fever to hormone therapy, which has been observed by many,’ has been attributed solely to pharmacologic action of the hormones.2 This conclusion has seemed justified since several studies in patients with rheumatic disease3-I0 have failed to detect evidence of any consistent abnormality in pituitary-adrenal function. These studies, however, have employed indirect techniques for evaluation of this function and cannot be considered conclusive. The data to be presented here concerning circulating concentrations of pituitary and adrenal hormones, adrenal responsiveness, and the half life of hydrocortisone in patients with rheumatic fever indicate that abnormalities of pituitary-adrenal-cortical function do occur consistently in these patients and provide rationale for hormone therapy in this disease on a physiological, as well a s on a pharmacological basis.