Showing papers by "Children's Memorial Hospital published in 1978"

Hyperkinesis and food additives: testing the Feingold hypothesis

Abstract: Teacher ratings, objective classroom and laboratory observational data, attention-concentration, and other psychological measures obtained on 36 school-age, hyperactive boys under experimental and control diet conditions yielded no support for the Feingold hypothesis. Parental ratings revealed positive behavioral changes for the experimental diet; however, they seemed primarily attributable to one diet sequence. Parents' behavioral ratings on ten hyperactive, preschool boys indicated a positive response to the experimental diet; again, laboratory observations showed no diet effect.

Undifferentiated sarcoma of the kidney. A tumor of childhood with histopathologic and clinical characteristics distinct from Wilms' tumor

Abstract: The pathological and clinical features of 123 patients with Wilms' tumor diagnosed between 1961 and 1977 were analyzed. A subgroup of nine patients with distinctive histology characterized by the presence of exclusively sarcomatous elements was identified. These patients were indistinguishable clinically from the other patients with Wilms' tumor and were treated as Wilms' tumor. The rate of recurrence in this group of sarcomatous Wilms' tumor was very high (7/9) and long-term disease-free survival correspondingly low )3/9 free of disease from 15 months to nine years) when compared to the patients with classical Wilms' tumor (50% recurrence rate and 71% currently free of disease). In addition, patients with metastases (seven of seven patients with recurrence had skeletal involvement as contrasted to one of 50 patients with metastatic classical Wilms' tumor). We conclude that the "sarcomatous Wilms' tumor" represents a distinct clinicopathological entity identifiable morphologically at diagnosis, best described as renal sarcoma and that in the future optimal treatment of this disease may vary somewhat from the treatment of classical Wilms' tumor.

Radiation myelopathy in children.

Abstract: Three children, 1 with medulloblastoma, 1 with anaplastic cerebellar tumor, and 1 with acute lymphocytic leukemia, received craniospinal irradiation with an estimated cord dose between 1,100 and 1,200 rets. All 3 patients after a latent period of seven to eight months developed subacute transverse myelopathy. The myelopathy was cervical in 2 patients and thoracic in 1. Spinal fluid examination showed high protein levels in 2 patients. Myelography demonstrated a swollen cord in 2 patients and normal appearance in 1. Autopsy in 1 patient disclosed a necrotizing myelopathy. The findings suggest that children tolerate less well than adults what previously have been considered safe doses of radiation to the spinal cord.

Activity level: A comparison between actometer scores and observer ratings

Abstract: Activity level of 13 boys (aged 9–13) from a day hospital program was measured using actometers in classroom, gym, woodshop, and group therapy settings. Ratings of Ss' activity were obtained from mothers using the Werry scale, and from six clinical staff familiar with the Ss using the Davids scale. It was predicted that activity ratings would have situationally specific relationships with actometermeasured activity level according to the rater's opportunities for observation. Comparisons between measures indicated that all clinical staff ratings correlated significantly with actometer activity in the classroom (r=.49 to r=.73), while mothers' ratings correlated significantly with actometer activity in gym (r=.67), and woodshop (r=.77), and with overall activity (r=.65), a combined measure derived from actometer scores in the four conditions tested. Five of six clinical staff raters showed significant interrater reliability (r=.58 to r=.83). Results are discussed in terms of their implications for solution of current problems in assessment of activity level and hyperactivity.

Hodgkin disease and red cell aplasia.

Abstract: A teenage boy with mixed-cellularity Hodgkin disease presented with severe anemia secondary to pure red cell aplasia of marrow without evidence of lymphomatous infiltration or hemolysis. In vitro studies of the patient's serum demonstrated an inhibitor of erythropoietin activity which appeared to be an IgG but which did not directly bind erythropoietin. The patient's anemia resolved and the inhibitor disappeared following chemotherapy for Hodgkin disease. Presumably, the inhibitor was directed at a very early stage of red blood cell production. This phenomenon may be related to other autoimmune manifestations occasionally seen in patients with lymphomas. The case is presented to bring attention to the unusual occurrence of red cell aplasia in Hodgkin disease. Several hypotheses concerning significance and etiology of the anemia are detailed.

Clinical diagnosis of the inborn errors of metabolism in the neonatal period.

Abstract: The first step in the evaluation of any sick neonate, and clearly the most important one, is a thorough clinical assessment including history and physical examination. In the case of metabolic disorders, the family history is critical, particularly if it reveals previously poorly explained neonatal deaths. The physical examination may alert the clinician to the possibility of a specific diagnosis and then appropriate laboratory tests may be performed in an attempt to verify that diagnosis. Such is the case in the neonate who has jaundice, hepatomegaly, vomiting, diarrhea, failure to thrive, and cataracts. Most pediatricians would immediately think of galactosemia, test the urine for reducing substances, and institute a galactose-free diet pending the results of definitive enzyme assays. The same is true of Pompe9s disease where the findings of macroglossia, hypotonia, hepatomegaly, cardiomegaly, and congestive heart failure without evidence of structural heart disease are rarely associated with any other diagnosis. When the clinical findings are not so distinctive, the diagnosis may, on rare occasions, be suggested by the observation of an unusual feature such as the odor of maple syrup in the urine of infants with maple syrup urine disease. In the vast majority of instances, the clinician is not so fortunate. Although the same may not be true for older children, the majority of inborn errors of metabolism that occur in the neonatal period are characterized by nonspecific signs and symptoms which in themselves are not useful in making a diagnosis but when observed in combination, without a known cause, are suggestive of such a diagnosis. It is hoped that the guidelines presented here will assist the physician in the evaluation of such infants. It has not been our purpose to discuss the definite diagnostic studies leading to a precise diagnosis or to outline specific therapeutic measures. These are best left within the province of those experienced in the management of these disorders. Astute clinical diagnosis and some simple laboratory studies may provide the assurance that affected infants will receive the benefits of further evaluation and treatment. If death appears imminent in a newborn suspected of having a metabolic disorder, we would recommend that the following samples be collected and stored: urine, frozen; plasma, separated from whole blood and frozen; and a small snip of skin obtained by sterile technique and stored at room temperature or 37 C in tissue culture medium, if available, or in 5% dextrose in normal saline solution. If an autopsy is performed, a sample of unfixed liver tissue should also be frozen at -20 C. These samples subsequent can be transported to a diagnostic center for studies that appear to be indicated.

Erythromycin Estolate Hepatotoxicity

Abstract: A recent review of erythromycin 1 stated that of 506 cases of cholestatic jaundice following erythromycin estolate administration reported between 1961-1973, only 12 (2%) occurred in children younger than 12 years of age. During the same period an estimated 30 million courses of therapy with a liquid form of estolate were prescribed. We report the case of a child in whom cholestatic jaundice developed and who was seen as an acute surgical emergency after erythromycin estolate administration. A percutaneous liver biopsy specimen demonstrated changes consistent with a hypersensitivity drug reaction. Report of a Case .—A 7-year-old boy was hospitalized with abdominal pain, rash, and fever of two days' duration. Four weeks prior to admission to the hospital his mother administered two 250-mg tablets of erythromycin estolate for treatment of a sore throat. Three days prior to admission an upper respiratory infection again developed and the patient was given erythromycin estolate

Cerebral angiography in meningocerbral inflammatory diseases in infancy and childhood: a study of thiryt-five cases.

Abstract: The value of cerebral angiography in the diagnosis of intracranial infections is presented, and the differential diagnosis of the various angiographic appearances in meningocerebral infections is discussed. Specific angiographic characteristics permitting the diagnosis of vasculitis, cerebritis, meningitis, brain abscess, and subdural and epidural fluid collections are correlated with known concepts of the pathological anatomy of these entities. Angiography is recommended in those infants known to have a meningocerebral infection who show signs of an increase in intracranial pressure or focal neurological dysfunction.

Enterobacter aerogenes primary bacteremia in pediatric patients.

Abstract: Enterobacter aerogenes bacteremia associated with the infusion of contaminated admixed intravenous (IV) fluid occurred in seven patients in a pediatric hospital over a five-day period. Clinical illness was characterized by spiking fever in all patients. The temporal clustering of cases allowed for rapid recognition of the problem. The primary control measure was the prompt replacement of the IV fluids, although IV antibiotics were also administered. Hospital pharmacy practices for admixing IV solutions should follow published recommendations to minimize this source of potential contamination of fluids.

Chemical diabetes in childhood. Integrated concentrations of glucose, insulin, and growth hormone.

Abstract: Children and adolescents with varying degrees of glucose intolerance were studied with constant, blood withdrawal methods, and concentrations of glucose, insulin, and growth hormone were evaluated during a normal twenty-four-hour routine. Integrated concentrations of glucose and insulin in children with chemical diabetes were normal despite abnormal oral glucose–tolerance tests. All but two insulin–dependent diabetics had elevated integrated concentrations of growth hormone, as did some but not all chemical diabetics. Three of four mildly ketoacidotic individuals with newly diagnosed diabetes, who were studied before insulin therapy, had normal growth hormone–integrated concentrations. These data differentiate pharmacologic and physiologic assessments of carbohydrate homeostasis, and they support the concept that elevated growth hormone concentrations may not be a direct result of poor diabetic control.

Reactive neuroaxonal dystrophy in children. Clinical pathological correlation.

Abstract: The aim of the present study was to determine the frequency and etiological factors of neuroaxonal dystrophy (N.D.) in brainstem and spinal cord of children with various non-neurological diseases. The material used in this study consisted of 266 consecutive autopsies from 1974–1976 and an additional 13 cases from previous years. By far, the most common cause of N.D. in children was chemotherapeutic drugs, particularly vincristine, used for the treatment of malignant tumors. Approximately 90% of these children developed N.D. and the changes ranged from mild to severe. Approximately two-thirds of children with cystic fibrosis, congestive heart failure and chronic renal failure developed N.D. and the changes were mostly mild. Only one-third of patients with congenital biliary atresia and malnutrition developed N.D. and the changes were always mild. The frequency and severity of N.D. increased in patients who had prolonged clinical courses; no N.D. was seen in patients who died from acute causes such as trauma, acute infection, intoxication, acute renal failure or prematurity. This type of N.D. may be considered as reactive to a wide variety of injurious factors such as drug toxicity, malnutrition, chronic hypoxia and chronic renal failure, either alone or in combination. In contrast to previous reports of a low incidence of N.D. in children, there has been a sharp increase in recent years due to the advent of chemotherapy.

Effects of erotic stimulation and masturbatory training upon situational orgasmic dysfunction

Abstract: Six single women, aged 22 to 29 years, were treated in a laboratory situation through erotic stimulation with masturbatory training for the disorder of situational orgasmic dysfunction. With single subject designs, three conditions of treatment were counterbalanced to estimate component effects. Intervention conditions included exposure to selected erotic stimuli, self-masturbation, and the preceding simultaneous combination. Frequency of orgasm was monitored via heart rate and verbal confirmation. Erotic stimulation with masturbatory training proved adequate to establish and maintain orgasmic responsiveness. Follow-up measures, conducted 6 to 12 months thereafter, partially supported generalization of treatment effects across environments and into existing heterosexual patterns of behavior.

Effects in vitro of the proposed antisickling agent DBA.

Abstract: A VARIETY of antisickling substances, both synthetic and naturally occurring, have been studied for their potential value as agents for the treatment of sickle cell disease. 3,4-dihydro-2, 2-dimethyl-2H-l-benzopyran-6-butyric acid (DBA), a chemically modified derivative of xanthoxylol isolated from Fagara zanthoxyloides, has been reported to prevent as well as reverse sickling of sickle cell anaemia erythrocytes1. 10 mM DBA was described as being able virtually to abolish sickling within a period of 30 min1. We have examined the effect of DBA on washed erythrocytes prepared from blood of sickle cell anaemia patients, and report here that we have been unable to demonstrate any antisickling effect of DBA.

Serum bile acid levels in protracted diarrhea of infancy.

Abstract: • Significant elevations in two glycineconjugated serum bile acid levels (cholic and chenodeoxycholic) were detected in a majority of infants with intractable diarrhea of infancy. In contrast, children with chronic inflammatory bowel disease had values of serum bile acids within the normal range. Although intravenous alimentation and constant-infusion elemental diet may alter hepatic function, serum bile acid levels were also elevated in other infants with intractable diarrhea not treated by these methods. We hypothesize that endotoxemia or other unknown mechanisms together with therapy are exerting a detrimental effect on hepatic function. ( Am J Dis Child 132:1001-1003, 1978)