Biomedical subjects
Publications and source records attributed to P Small.
Ninety-seven patients presenting with isolated Raynaud's phenomenon were evaluated clinically and immunologically. Three patients developed progressive systemic sclerosis in a 3-5-year follow-up. Nineteen patients had positive antinuclear antibody, 32 depressed serum complement, and 31 elevated serum immune complexes. None of the immunologic abnormalities had obvious diagnostic significance.
The presence of immunoglobulins in nasal polyps and surrounding mucosa was studied by direct immunofluorescence in 14 patients. Specific and total IgE were determined in polyps and in the sera of this group. Significant immunofluorescence to IgE was demonstrated in 12 polyps but in only four samples of adjacent mucosa. Eight polyps showed significant immunofluorescence to IgM. Six patients had elevated total IgE levels within polyp sac fluid in the absence of elevated serum values. In seven cases, specific IgE was found in polyp sac fluid which could not be detected on skin or serum testing. These results may suggest local production of IgE and support the concept of nasal polyps being a manifestation of a local allergic phenomenon.
Echo planar imaging (EPI) is that form of magnetic resonance imaging which provides very short image acquisition times. EPI also provides very rapid sequential imaging. The EPI method is ideal for imaging the heart and thoracic content because images are devoid of cardio-respiratory motion artefact. Previously an analysis of transectional images has been presented. This paper is concerned with the study of the heart by the use of EPI constructions in the sagittal and coronal planes. Defining connections between ventricle and great artery is of cardinal importance in paediatric cardiology. EPI constructions in the normal heart, transposition, truncus arteriosus and right heart hypoplasia are presented and discussed.
Echo planar imaging is that form of magnetic resonance imaging which gives very short image acquisition times. The method has been used to produce images of the infant heart which are free of cardiorespiratory motion artefact, despite tachypnoea and tachycardia. EPI transections of the normal heart are compared with transections in truncus arteriosus, tetralogy of Fallot, right heart hypoplasia and transposition of the great arteries. The diagnosis of the cause of cyanosis in these infants was established by the noninvasive EPI method and validation of the findings may be found in transectional postmortem analyses reported in the literature.
Femoral head shape has been assessed in 47 children with unilateral Perthes' disease and 41 control children. Measurements were taken from radiographs and arthrographs by a computerized method. In controls the femoral head was round, particularly in lateral outline, and characteristically asymmetric in anteroposterior views at all ages studied. In children with Perthes' disease the bony femoral head was less round than in controls and flattened anteriorly. Mediolateral symmetry differed from that of controls, reflecting lateral flattening of the cross section of the femoral epiphysis. Age-related changes correlated with disease duration but not with treatment. The "unaffected" hip showed some evidence of anterior and lateral flattening from the time of diagnosis of disease in the other hip. No subject developed bilateral disease during the follow-up period. This early involvement of the "unaffected" hip could indicate a constitutional abnormality. The cartilaginous femoral head was rounder than the underlying bony epiphysis, particularly in anteroposterior views. The cartilage of the hip with Perthes' disease was less round than that on the "unaffected" side. There was no evidence of anterior or lateral flattening of either cartilaginous femoral head.
Sixteen of 53 patients with rhinitis had positive skin tests corresponding with serum-specific IgE (RAST). Concomitant positive nasal secretion (NS) RAST was found in 10/16. Four patients with both negative skin tests and serum RAST had positive NS RAST. In a subset of patients with perennial rhinitis, specific IgE may be detected only in NS. Further studies are necessary to document the clinical relevance of such NS IgE antibodies.
Twenty-two adult patients with polymyositis/dermatomyositis (PM/DM) have been seen at the Sir Mortimer B. Davis-Jewish General Hospital since 1970. DM accounted for 50% of the patients. Malignancy was found within 3 years of presentation in 32% and was present in 46% of patients over age 50. Forty-six percent of patients with DM versus 18% of those with PM had a malignancy. Seven patients died. Fourteen patients were seen at a mean followup of 5.6 years. By life-table analysis, 42% of patients terminated corticosteroids within 3 years from diagnosis. The mean dose of prednisone in 8 patients being treated at followup was 11.7 mg daily. Functional disability, however, was common at followup. Thirty-six percent had substantial disability related to the disease or its treatment.
Forty-six patients were studied to assess the role of immunological factors in the formation of nasal polyps. Total IgE levels did not correlate with positive skin testing and were generally higher in polyp sac fluid than in corresponding sera. Asthmatics had significantly higher IgE levels in both serum and polyp fluid than nonasthmatics. Specific IgE (RAST) found in polyp specimens could not be detected by conventional skin or serum testing in nine of 15 patients. By direct immunofluorescence IgE was present in 81% of polyp specimens compared with 13% in mucosal controls. These data indicate that nasal polyp formation may be an IgE mediated disease process and conventional skin or serum testing may not detect this local allergic process.
Eighteen of 24 patients undergoing nasal polypectomies demonstrated polyp fluid total IgE higher than corresponding serum values. Allergen-specific IgE was detected in polyp fluid of 19/24 patients. Since only 9/19 had positive skin tests, ten patients had specific IgE locally in polyp tissue which could not be detected systemically.
A group of 143 patients with angioedema was reviewed. Women in their third and fourth decades were most commonly affected. Head and neck involvement was found in 94% of cases and this was the only anatomical site in 24%. Twenty-one patients had swelling of the oropharynx while four presented with laryngeal edema. A diagnosis of hereditary angioedema (HAE) was confirmed in one case. Asthma, allergic rhinitis, and drug sensitivity were significantly associated, while smaller numbers of patients had underlying vasculitis or active ongoing infection. The pathophysiology and management of angioedema are discussed.
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A 70-year-old female presented with a generalized allergic reaction to heterologous insulin. Subsequent epicutaneous testing to human insulin (Humulin) evoked a large wheal and flare reaction and generalized pruritus. The institution of human insulin therapy in patients allergic to heterologous insulin may cause further allergic reactions.
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Echo-planar imaging (EPI), a distinctive variant of nuclear magnetic resonance, needs only a fraction of a second for an image to be acquired and so is free from movement artifacts caused by respiration or heart beat. Clinical findings in the lungs, heart, and mediastinum of three children with high respiratory and heart rates who were examined by EPI are described.
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Two half-brothers with tuberous sclerosis (TS) presented with polycystic kidneys in early childhood, before the classical stigmata became apparent. Their father shows no evidence of the disease. The older boys subsequently developed adenoma sebaceum at nine years and the younger boy developed infantile spasms. Hypertension occurred in both cases but neither showed evidence of renal failure. Extensive renal cyst formation in TS is rare, but when it does occur it differs from both infantile and adult-type polycystic disease. TS should be considered in the differential diagnosis of renal enlargement, haematuria and hypertension in childhood.
An immunological evaluation of 62 Hymenoptera sensitive patients, including 13 treated with venom, was undertaken. Skin testing was more sensitive than serum specific IgE and total IgE was not helpful. Specific IgE did not decrease on therapy. Changes in specific IgG occurred in bee but not in mixed vespid treated patients.
The efficacy and safety of beclomethasone dipropionate (BD), 0.05 mg three times daily, sprayed in each nostril was studied in 39 adult patients with perennial rhinitis in a 12 wk, double-blind, vehicle controlled trial. Parameters of IgE-mediated reactivity, including epicutaneous skin testing, total serum IgE, specific serum IgE, and nasal eosinophilia, were assessed. All adverse reactions, including changes in serum cortisol and nasal and pharyngeal Candida infections, were monitored. Sixty-three percent of BD patients achieved total or substantial control of nasal symptoms compared with 25% of controls (p = 0.04). Eighty-three percent of BD-treated, skin test-positive patients improved, while only 14% of BD nonatopics improved (p less than 0.05). All BD patients with nasal eosinophilia improved compared with 38% without eosinophilia. Adverse reactions were frequent, minor, and equal in both groups. Serum cortisols were stable and no nasal Candida infections were documented. This study demonstrates the efficacy and safety of BD in treatment of perennial rhinitis, particularly in atopic patients with nasal eosinophilia.