William W. Cleveland

Pathophysiology and management of acute corrosive burns of the esophagus: Results of treatment in 285 children

Abstract Two hundred eighty-five children with possible caustic burns of the esophagus have been managed at two university hospitals using similar protocols. Of these, 235 (82%) had immediate esophagoscopy and 69 (29%) had demonstrated esophageal burns. They were treated with steroids and antibiotics. Eight (12%) with proven burns developed strictures that responded to prolonged dilatations and none have required esophageal replacement. The remainder are free of swallowing symptoms. By contrast, eight patients from other hospitals who were not treated by this protocol were referred for esophageal replacement 6 and prolonged dilatation. 2 Our strong impression is that immediate steroid-antibiotic therapy greatly decreases the incidence of esophageal stricture but does not completely eliminate it. Those children who develop strictures on this treatment regimen seem to have milder esophageal scarring, which usually responds to dilatation rather than requiring esophageal replacement.

THE LONG TERM NEUROLOGICAL SEQUELAE OF ANEMIA IN INFANCY

To determine if hypoxemia from anemia in infancy causes brain damage, 61 full term infants from comparable socioeconomic groups were studied developmentally from birth to 7 years Thirty-two of the infants developed iron deficiency anemia between 6-18 months of age (Hgb range 6.1-9.5 G%) in the absence of protein calorie malnutrition. Twenty-nine infants received neonatal iron dextran injections and were not anemic (Hgb range 11.5-12.96%).Neurological evaluation was done at 6-7 years of age without knowledge of the presence or absence of prior anemia. Each test was rated on a 0-3 scale or by stanines. The group formerly anemic had a higher incidence of “soft signs” e.g. clumsiness with balancing on one foot, in tandem walking, and repetitive hand or foot movements, etc. and were more inattentive and hyperactive than the non anemic group. Binet IQ scores averaged 98 in the non anemic and 92 in the anemic group. Anemia in infancy appears to be one cause of minimal brain dysfunction which may be permanent.

Oral rehydration of infants in a large urban U.S. medical center

A prospective randomized study of 100 well-nourished infants with acute gastroenteritis resulting in dehydration and acidosis was carried out at the Jackson Memorial Hospital, Miami from 1981 to 1983. Patients were randomly assigned to receive either standard intravenous therapy or oral rehydration. Infants in the latter group first received solution A containing 75 mEq/L sodium, 30 mEq/L potassium, 75 mEq/L chloride [corrected], 30 mEq/L bicarbonate, and 2 gm/dL glucose [corrected]. After ad libitum feeding for six hours, solution B containing 50 mEq/L sodium, 30 mEq/L potassium, 50 mEq/L chlorine, 30 mEq/L bicarbonate, and 3 gm/dL [corrected] glucose was given. With three exceptions (6%), oral rehydration was comparable to the intravenous regimen in clinical estimates of improvement, although the oral group had more stools in the first day. The oral group had faster correction of acidosis and a sustained rise in serum potassium concentration, whereas in the intravenous group the potassium concentration showed first a drop with a later increase, but levels were at all times below those in the oral group. Although potassium was given from the beginning of oral rehydration, and at a higher concentration than recommended by the World Health Organization, no hyperkalemia occurred. We concluded that oral therapy is safe, less expensive for patients, and more convenient for the medical and nursing staffs.

Effect of Intravenous Insulin-like Growth Factor I in Two Patients with Leprechaunism

ABSTRACT: Leprechaunism (Donohue syndrome) is an autosomal recessive disorder characterized by hyperglycemia, extreme insulin resistance, dysmorphic features, failure to thrive, and early death. In this study, recombinant IGF-I, which has both insulin-like and anabolic effects, was administered to two infants with leprechaunism in an attempt to reduce hyperglycemia and improve nutritional status. IGF-I was infused for 66 h in patient FL-1 and 62 h in patient NC-2, with maximal infusion rates of 110 and 40 μg/kg/h, respectively. Although supraphysiologic concentrations of IGF-I were achieved (459 and 1583 μg/L in FL-1 and NC-2, respectively), there were no apparent glucoselowering or nitrogen-sparing effects. Insulin concentrations decreased from extremely high values (16804 and 10224 μmol/L) but remained elevated (611 pmol/L in FL-1 and 5869 pmol/L in NC-2). No changes in serum and urinary urea nitrogen or electrolytes occurred. IGF binding protein-2, which was the predominant IGF binding protein in serum by ligand blot and immunoblot, did not change with IGF-I infusion. IGF binding protein-3 levels were low at baseline and increased slightly during the infusion. We hypothesize that the lack of significant glucose-lowering and anabolic responses to IGF-I could be secondary to a postreceptor defect in IGF-I signaling resulting from the absence of functional insulin receptors.

Measurement of urinary testosterone by gas-liquid chromatography☆

Abstract A method has been developed which employs gas-liquid chromatography for the assay of testosterone from extracts of human urine prepared according to an earlier method described by Camacho & Migeon in 1963. The trimethyl silyl ethers of testosterone and an internal standard, cistestosterone, were separated on the nitrile silicone rubber stationary phase XE-60. This method has given results which correspond satisfactorily with those obtained with UV spectrophotometry or measurement of sulfuric acid chromogens after paper chromatography.

Acquired Fanconi syndromefollowing degraded tetracycline

Three patients have been found to manifest a Fanconi syndrome followingingestion of outdated tetracycline. Marked hypokalemia, changes in sensorium, and photosensitivity were prominent additional features. The generalized aminoaciduria was quantitated, and the pattern of its return to normal documented. The disorder appears to be a toxic effect of a degradation product of tetracycline.

Measurement of plasma prednisolone by a competitive protein-binding assay☆

Abstract Although recently procedures have been developed for measurement of prednisolone (11β,17α,21-trihydroxypregna-1,2-diene-3,20-dione) in blood, they lack sensitivity needed for plasma concentrations found in patients on usual therapeutic dosages (1, 2). We have applied the competitive protein-binding radioassay technique developed by Murphy to measurement of plasma prednisolone (3). This method, while very sensitive, also contributes considerable specificity due to the high affinity of the protein used for binding (4). Preliminary purification is provided by extraction with methylene chloride and paper chromatographic separation of prednisolone from cortisol (11β,17α,21-trihydroxypregna-4-ene-3,20-dione) using a Bush descending chromatographic system (5). The assay is performed by displacement of tritiated corticosterone (11,21-dihydroxypregna-4-ene-3,20-dione) from corticosterone-binding globin (CBG) present in dog plasma. The specific technique used is a modification of one for measurement of progesterone published by Neill and his co-workers (4).

Function of the human corpus luteum during the puerperium: Its maintenance by exogenous human chorionic gonadotropin

Abstract The human corpus luteum is maintained and continues to function throughout pregnancy. Human chorionic gonadotropin (HCG) plays a major role in the maintenance of the corpus luteum until term. After delivery, the source of HCG disappears and the corpus luteum ceases to function. Daily administration of exogenous human chorionic gonadotropin in large doses for as long as 5 days post partum appeared to maintain the function of the corpus luteum and prevented the decline usually observed after delivery in untreated subjects. Corpus luteum function was assessed by means of two parameters: the measurement of progesterone concentrations in peripheral and ovarian venous plasma and synthesis of progesterone in vitro by surviving slices of corpus luteum tissue removed at various times post partum.

Radioulnar synostosis, behavioral disturbance, and XYY chromosomes

Two prepubertal boys with radioulnar synostosis, aggressive behavior, mild retardation, and tall stature were found to have an XYY chromosomal constitution. The chromosomal abnormality in one of them was found by testing 6 patients, 4 of whom were boys with radioulnar synostosis. This suggested a close relationship between the extra Y chromosome and this skeletal abnormality. Violent, criminal tendencies have been reported in male adults with extra Y chromosomes. Disturbance in childhood may be an expression of this pattern or an indication of a much broader relationship between chromosomal composition and behavior.

Hematogenous brain metastasis in children

Hematogenous brain metastases are uncommon in childhood. Three patients and a literature review that includes centers reporting up to 36 years of experience are presented in this study. The total of 2,040 patients includes our three examples of one neuroblastoma, one hepatoblastoma, and one adrenal carcinoma. Cerebral hematogenous metastases were reported in 4.4% of 429 patients with neuroblastoma, 1.9% of 574 rhabdomyosarcoma patients, 6.5% of 386 patients with osteosarcoma, 3.3% of 487 Ewing sarcoma patients, 3.6% of 44 melanoma patients, 13.5% of 37 patients with germ cell tumors, and 1.3% of the 78 patients with Wilms tumor. Five miscellaneous patients included three with a hepatoblastoma and one each with adrenal carcinoma and nephroma. All of the large series reports have been published in oncology journals.