PubMed Health⌕ Search

Biomedical subjects

A R Rausen

Publications and source records attributed to A R Rausen.

At least 19 recordsLinked to original sources

Association of Gaucher's disease and lymphoid malignancy in 2 children.

2 cases of acute lymphoblastic leukaemia (ALL) of childhood in association with Gaucher's Disease (GD) are presented, which are the first to be described. One of them is a common non-B non-T cell ALL and the other is at T-cell ALL. The association of GD with benign and malignant B-cell proliferation has recently been described. It is suggested that chronic stimulation of the immune system by the accumulated glucocerebroside may predispose to B cell proliferation.

B-Lymphocytes↗

Breast-vs. bottle-feeding. A study of morbidity in upper middle class infants.

This study compares the morbidity of two groups of healthy, full-term infants (25 in each group) who were exclusively either bottle-fed or breast-fed for 5 months. There were no statistically significant differences in morbidity between the two groups except for a borderline greater frequency of upper respiratory infections in the bottle-fed group. Although the study groups are limited in size, the results suggest that, when appropriate hygienic measures are taken and statistical biases eliminated, differences in morbidity between bottle-fed and breast-fed babies are relatively minor. The data also show that modern infant formulas seem to be nutritionally complete in that there was no difference in the rate of growth or in hematological parameters measured in the bottle-fed and breast-fed group.

Bottle Feeding↗

Progressive esophageal dysfunction in chronic granulomatous disease.

Chronic granulomatous disease of childhood (CGD), a hereditary disorder of neutrophil function, affects the gastrointestinal tract in a variety of ways. Esophageal involvement has only rarely been reported. An 11-year-old boy with CGD and progressive esophageal dysmotility is described. Repeated radiographic, endoscopic, and motility studies revealed a markedly atonic esophagus with varying function of the lower esophageal sphincter. Pharmacologic therapy and esophageal dilatations were unsuccessful in establishing adequate esophageal function. A feeding gastrostomy was required for nutritional support.

Child↗

Thrombocytosis and increased circulating platelet aggregates in newborn infants of polydrug users.

Thirty-three prospectively studied neonates born to mothers using methadone plus other drugs developed significant thrombocytosis by the second week of life compared to platelet counts performed during the first week. This increase persisted for over 16 weeks, with a further short-lived significant peak at 10 weeks of age. Platelet counts exceeding 1,000,000/mm3 were found in seven infants. Thrombocytosis was not related to withdrawal symptoms or treatment (phenobarbital or paregoric). No thrombocytosis was found in 36 normal control infants up to eight weeks of life. Twenty-eight of the study group infants were evaluated for circulating platelet aggregates. Thirteen patients had a normal aggregate index and a mean platelet count of 468,000/mm3; 15 patients had increased aggregates and mean platelet count of 754,000/mm3. The risk for increased circulating platelet aggregates correlated directly with an increase in platelet count. Thrombocytosis and increased circulating platelet aggregates may be factors in the pathogenesis of the focal infarcts, and subarachnoid and germinal plate hemorrhages, described at autopsy in infants of addicted mothers.

Amphetamine↗

Immunochemotherapy in advanced neuroblastoma.

Twenty-two children with advanced (Stage III and IV) neuroblastoma have been treated in a nonrandomized fashion, half with a three-drug regimen consisting of vincristine, adriamycin, and cyclophosphamide, and half with this same drug combination plus the nonspecific immunostimulatory agent, MER/BCG. The addition of MER to the three-drug combination appeared to improve the duration of survival in this pilot study. The median duration of response was less than one year in the combination chemotherapy alone arm. The median duration of complete remission in children treated with the addition of MER has yet to be reached at 24 months.

Antineoplastic Agents↗

Cellular immune responses to methylcholanthrene-induced fibrosarcoma in BALB/c mice.

Several in vitro parameters of cellular immunity were examined in BALB/c mice with an experimentally induced fibrosarcoma tumor. The results of capillary migration of spleen cells in high tumor cell dose inoculated mice show appearance of cellular immune response in the early stages of the tumor growth. As the tumor progresses, the cellular response declines and rapidly disappears, culminating in stimulation values near the time of the death of these mice. The blastogenic studies also show early cellular recognition of tumor antigen by mouse spleen cells and whole blood (Z24 h). After the 2nd day following tumor injection, no blast transformation is noted. However, the results obtained with a lower inoculating tumor cell dose demonstrate an initial cellular recognition on the 7th day. This response gradually disappears by the 19th day and remains negative up to the time of the death of these mice. This cellular immunity was confirmed by the cytotoxic experiments showing that the primary cells responsible for this cellular reactivity were the immune cells. An interesting finding was the presence of a factor(s) capable of blocking the cytotoxic effect. The nature and mechanism of this blocking factor(s) is now under investigation.

Animals↗

Association of Pseudomonas cepacia with chronic granulomatous disease.

Pseudomonas cepacia was recovered from a number of infected sites in three patients with chronic granulomatous disease of childhood. The organisms were identified on the basis of their oxidative utilization of a variety of carbohydrates and their positive beta-galactosidase and oxidase activities. They were resistant to most antimicrobial agents and moderately susceptible to chloramphenicol. Peripheral blood leukocytes isolated from two siblings with chronic granulomatous disease, including one of the patients in this series, failed to kill P. cepacia in vitro. Prolonged prophylactic and antimicrobial therapy may well have played a significant role in the colonization and infection of these patients with P. cepacia.

Blood Bactericidal Activity↗

Compatible transfusion therapy for paroxysmal cold hemoglobinuria.

Two children with paroxysmal cold hemoglobinuria (PCH) are described. Both cases were associated with cold-warm serum lysins having anti-Tja activity. Transfusion with compatible frozen-thawed red cells tided these children over the critical acute hemolytic phase of the syndrome. These are the first recorded examples of successful compatible transfusion therapy in PCH.

Blood Transfusion↗