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Biomedical subjects

R W Wilmott

Publications and source records attributed to R W Wilmott.

51 records · Page 3Linked to original sources

A chronic lung disorder following abdominal pregnancy.

Abdominal pregnancy is a rare condition that is associated with a high infant mortality, as well as orthopedic and pulmonary deformations. This article describes the clinical and radiological courses of two infants born after abdominal pregnancies. Evidence of pulmonary hypoplasia secondary to fetal compression was present. The morbidity and mortality for infants born of abdominal pregnancies may depend on the extent of pulmonary involvement, which may relate to the length of fetal compression.

Chronic Disease↗

Interleukin-1 activity from human cord blood monocytes.

Cord blood monocyte synthesis of IL-1 was investigated by using a thymocyte proliferation assay. Monocytes from 27 infants ranging in gestation from 31 to 41 weeks (mean 38.9, SE 0.54) with birthweights from 1.20 to 4.31 kg (mean 3.24, SE 0.13) were isolated from cord blood; 2 x 10(5) cells/ml were plated in 15 mm wells and stimulated with 10 micrograms/ml LPS (E. coli). Control cultures contained medium alone. Supernatants were harvested after 24 hr and tested in a C3H/HeJ mouse thymocyte proliferation assay. The mean response for 27 cord monocyte samples at 24 hr was 14,142 cpm (SE 1,499), not significantly different than that for cells obtained from eight normal adult volunteers (15,137 cpm, SE 3,535). Vaginally delivered infants with perinatal complications such as amnionitis, fetal distress, or early sepsis had significantly increased unstimulated activity (5,139 vs 1,331 cpm) compared to samples from normal infants, whereas stimulated activity was not significantly different (16,219 vs 12,261 cpm). Thus, the IL-1 response to lipopolysaccharide is intact in newborn human monocytes and there is evidence of an increased unstimulated activity following neonatal complications.

Cells, Cultured↗

Large-airway collapse due to acquired tracheobronchomalacia in infancy.

Seven infants with wheezing and cyanotic spells were diagnosed as having tracheobronchomalacia by bronchoscopy or fluoroscopy. These studies demonstrated narrowing of the central airways by 75% or more on exhalation. Five patients were premature infants who had been ventilated for hyaline membrane disease. The mean time from onset of symptoms to diagnosis was six months. Determination of the optimal positive end-expiratory pressure (PEEP) during fluoroscopy facilitated subsequent management. Six of the seven patients required PEEP of 8 to 18 cm H2O for at least three months, and five of the seven still require mechanical ventilation. All patients improved, with decreased cyanotic spells and a reduced requirement for ventilatory support. Tracheostomy without PEEP did not appear to be helpful. Tracheobronchomalacia may be more frequent than usually appreciated; the treatment of choice appears to be long-term PEEP.

Airway Obstruction↗

Pulmonary function abnormalities in homozygous beta-thalassemia.

Pulmonary function tests were performed on 19 patients with homozygous beta-thalassemia ranging in age from 10 to 29 years. These included patients who had and had not received transfusions. None of the 19 subjects had completely normal pulmonary function. Residual volume (in 16 of 19 patients), ratio of residual volume to total lung capacity (12 of 19), and airway resistance (16 of 19) were abnormally increased; maximum expiratory flow (15 of 19) and peak flow (10 of 19) were abnormally reduced. Single-breath carbon monoxide diffusion was normal in 16 of 19. These results indicate that mild to moderate small airway obstruction and hyperinflation are common in thalassemia and that patient age, transfusion history, and iron accumulation are not important factors in the genesis of these pulmonary abnormalities.

Adolescent↗

Serial evaluation of lymphocyte function in bone marrow-grafted patients.

Deficiencies in both cellular and humoral immunity follow human bone marrow transplantation, predisposing recipients to life-threatening infections. Peripheral blood mononuclear cells (cells of donor marrow origin) from nine patients were collected serially at 3-month intervals during the first year post transplant and evaluated for proliferation and lymphokine (gamma interferon and interleukin-2) production in vitro. Cultures of patient cells or those of normal adult volunteers were stimulated by phytohemagglutinin (PHA) in vitro, and lymphocyte blastogenesis was assayed by tritiated thymidine uptake on day 2. PHA blastogenesis for peripheral blood mononuclear cell cultures from patients post bone marrow transplant achieved normal levels 3-6 months post transplant. Supernatants produced by cells from marrow recipients (less than 12 months post transplant) had lower-than-normal IFN-gamma activity and decreased IL-2 activity. Two patients with acute graft-vs-host disease (GVHD) had persistently depressed PHA blastogenesis, IFN-gamma production, and IL-2 production at 12 months post transplant.

Bone Marrow Transplantation↗

Cystic fibrosis survival rates. The influences of allergy and Pseudomonas aeruginosa.

Allergy and chronic Pseudomonas aeruginosa (PA) infection are two factors that possibly affect the clinical severity of cystic fibrosis pulmonary disease, although the role of allergy is controversial. We have examined the effects of these factors on actuarial survival rates in 117 children with cystic fibrosis who were skin tested in 1974 and classified as allergic (A+) or nonallergic (A-) by their reactions to 12 prick tests with common environmental allergens. Patients were also classified according to whether or not they had chronic pulmonary infection with PA in 1974 (PA-positive or PA-negative). Survival rates in A+ patients were not significantly different from those in the A- group (percent survival to age 16 years, 67% vs 80%), whereas the PA+ group had significantly worse survival rates than the PA- group (percent survival to age 16 years, 53% vs 84%). There was no significant interaction between allergic skin reactions and either age at onset of chronic PA infection or subsequent duration of survival.

Adolescent↗

Survival rates in cystic fibrosis.

Life tables were calculated for 273 British children with cystic fibrosis for the period 1974-9. There was a marked improvement in survival rates in the meconium ileus group compared with the 1969-73 data, but there was little improvement in patients presenting later with other symptoms.

Adolescent↗

Desquamative interstitial pneumonia and antigen-antibody complexes in two infants with congenital rubella.

Desquamative interstitial pneumonia was observed in two infants with the late-onset congenital rubella syndrome. In both infants this unusual lung disease was associated with circulating immunoglobulin M complexes and interstitial pulmonary deposits of IgM by immunofluorescence. Both infants had IgG deficiency. The first child recovered with a reduction in IgM complex levels and synthesis of rubella-specific IgG. The second infant died during the acute phase of his illness at which time there were high serum concentrations of IgM complexes and slightly increased levels of IgG complexes. Delayed maturation of the immune response in congenital rubella may predispose to persistent antigenemia and pulmonary deposition of rubella antigen-containing IgM complexes resulting in an acute form of interstitial pneumonia.

Antigen-Antibody Complex↗

Pulmonary hemosiderosis.

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Diagnosis, Differential↗

The relationship between atopy and cystic fibrosis.

The incidence and clinical significance of allergy in cystic fibrosis have been discussed. There is little evidence that the high prevalence of positive allergy skin tests in CF is a clinical manifestation of a hypersensitivity lung disease complicating the primary pulmonary disorder, except in the special case of allergic bronchopulmonary aspergillosis. The lung disease of CF appears to be caused by excessive bronchial secretions and recurrent infection that are the result of abnormal ion transport across the apical membrane of the respiratory epithelial cell. There are two important questions concerning the allergic reactions: Are they clinically significant, and, why do they occur? The former question is partly resolved by the data presented, although well-controlled studies of intervention would help to resolve it further. The cause of these reactions, which occur particularly in relationship to the mold Aspergillus fumigatus, is unknown but the author is inclined to the view that they are the result of recurrent infection that induces heightened immunologic reactivity to inhaled allergens. The significance of AF may be simply that this mold has a "predilection for diseased airways" or may be more complex; for example, owing to abnormal ion composition in respiratory secretions, abnormal lectin expression by CF respiratory epithelial cells, or selective filtration by the airways based on particle size.

Cystic Fibrosis↗