Biomedical subjects
V Rose
Publications and source records attributed to V Rose.
Computed tomography scanning of the liver to determine efficacy of iron chelation therapy in thalassemia major.
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Faecal incontinence in childhood: a multidisciplinary approach including biofeedback.
One hundred and seven children with faecal incontinence were evaluated and managed over a 3 year period by a multidisciplinary team. After initial clinical assessment, evaluation of defaecatory mechanisms (using a balloon model) and assessment of personal-social development and self-concept were undertaken. Management was based on initial bowel evacuation, short-term laxatives, and habit training involving systematic use of positive reinforcement; 69 children received biofeedback conditioning. Idiopathic megacolon with constipation and soiling was the most common finding (98 cases). Other diagnoses included previously undiagnosed neurogenic bowel (three cases), post-surgical and anomalies (four cases), and psychogenic encopresis (two cases). Idiopathic megacolon was characterized by decreased rectal sensation, increased threshold for external sphincter relaxation and an inability to evacuate. Faecal incontinence was associated with an undesirably low social self-concept (70% of the 40 evaluated), but was not related to a delay in development (mean general developmental quotient = 105 +/- 8, for the 35 tested). Family psychopathology warranting referral for family therapy was found in 14 children (13%). The management programme yielded a short-term (3 months) cure rate of 68% and a long-term (12 months) cure rate of 90%, with 10% having continued soiling which varied from occasional to several incidents/week. No significant improvement in self-concept was observed overall, although marked improvements were observed in some children. We conclude that disordered defaecatory dynamics are a major determinant of faecal incontinence in children. Undesirably low social self-concepts but normal developmental ability accompany this condition. Management is facilitated by a multidisciplinary approach, acknowledging the role of both behavioural and physiological components of the problem.(ABSTRACT TRUNCATED AT 250 WORDS)
Dilated cardiomyopathy with neutropenia, short stature, and abnormal carnitine metabolism.
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Obstructive sleep apnea: therapeutic compliance.
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Kawasaki disease: a measles cover-up?
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Exercise radionuclide right ventriculography in children.
Supine exercise equilibrium radionuclide right ventriculography was performed in 13 children (8-18 years) with hypercholesterolemia. Phase analysis was used to construct right ventricular regions of interest, and a peri-right ventricular region was used for background correction. Right ventricular ejection fraction at rest and exercise was 50.5 +/- 9.2% and 61.5 +/- 8.1%, respectively, with a mean increase of 11.0 +/- 7.5 percentage units (range 1-27 percentage units). During exercise, end-diastolic volume remained unchanged while end-systolic volume decreased by 19.4%, producing a 21.7% increase in stroke volume. Stroke volume ratios (left ventricular stroke volume counts/right ventricular stroke volume counts) approach unity (1.00 +/- 0.27). However, interobserver and intraobserver correlations are just fair, implying only a modest degree of accuracy and reliability of the procedure. This imprecision needs to be considered when evaluating the results of nuclear equilibrium right ventriculography.
Tricuspid valve endocarditis in two children with normal hearts: diagnosis and therapy of an unusual clinical entity.
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Severity and recurrence risk of congenital heart defects exemplified by atrial septal defect secundum.
It has been reported that the frequency of congenital heart defects (CHD) in children of an affected parent is now three to five times what it was 20 years ago. One conceivable cause of this is that patients with CHD of a severity that would have precluded parenthood before the advent of treatment are now able to (and consequently do) have children once the defect has been corrected. The mean severity of CHD among parents who have it would have thereby increased and this might, in turn, have increased the frequency of CHD in their children. But the latter would occur only if severely affected parents confer a greater risk on their children than parents who are mildly affected. To determine whether this is the case, we investigated 83 probands, each of whom had had an atrial septal defect secundum whose dimensions had been recorded at the time of operation. Of their 166 children, 17 had CHD. The defects of probands with affected children were not found to be significantly different in size from the defects in probands whose children were all normal. Analysis of the hemodynamic data available yielded a similar result. Our findings decisively exclude surgical correction as a significant explanation for the increased recurrence of atrial septal defect.
The descriptive epidemiology of Kawasaki syndrome in Canada, 1979-1985.
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Kawasaki disease: review of risk factors for coronary aneurysms.
Between June 1, 1979, and May 31, 1984, at The Hospital for Sick Children in Toronto, Kawasaki disease was diagnosed in 163 patients (112 boys, 51 girls, P less than 0.001). Fifteen percent of the children had coronary artery aneurysms. Prior to diagnosis, 24% had been given low doses of aspirin, and 50% acetaminophen. Children with coronary aneurysms had significantly higher temperature during days 10 to 13 of the disease. The febrile phase of the disease was also significantly longer in these children. Coronary artery involvement occurred with equal frequency in boys and girls. There was no significantly greater incidence of coronary artery involvement in infants younger than 1 year of age than in older children. Duration of fever (greater than or equal to 14 days vs less than 14 days) was equally as predictive of the eventual occurrence of coronary aneurysms as the modified Asai score.
High persistence rate of established coronary artery lesions secondary to Kawasaki disease among a panethnic Canadian population.
The incidence of resolution of proximal coronary artery lesions subsequent to Kawasaki disease over an 11-year period was studied retrospectively. In 28 patients proximal coronary artery lesions were identified by two-dimensional echocardiography or angiography at 1 to 3 months after disease onset. Follow-up data were adequate in 27 patients, 17 of whom were boys; 18 were white, two black, four Oriental (non-Japanese), and three of East Indian origin. Age at onset of Kawasaki disease ranged from 11 weeks to 9 years (mean 2.5 years). Coronary artery lesions were categorized as ectatic (internal diameter greater than 3 mm in those less than 5 years of age) or aneurysmal (internal lumen of a segment one and one-half times larger than an adjacent segment). Resolution was assessed by two-dimensional echocardiography or angiography during a follow-up period of 3 months to 11 years (mean 2.7 years). Apparent resolution (vessel diameter less than 3 mm) of coronary artery lesions occurred in four (15%) patients--one with aneurysmal lesions, three with ectatic lesions--all within 1 year of disease onset. Eight patients developed myocardial infarction; one of these patients died. The resolution rate was less than previously reported. This high rate would warrant closer attention to the state and sequelae of coronary artery lesions, including obstructive coronary artery disease, in follow-up assessments.
Probable efficacy of high-dose salicylates in reducing coronary involvement in Kawasaki disease.
The efficacy of high-dose salicylates in reducing the coronary artery involvement of Kawasaki disease was compared in 36 children who received acetylsalicylic acid, 80 to 180 mg/kg/day, and in 18 who did not receive high-dose salicylates during the febrile phase of the disease and whose fever was controlled mainly with acetaminophen. The two groups were comparable with respect to age and body weight. In the acetylsalicylic acid-treated group, the dose was adjusted to meet the therapeutic serum concentration range (greater than or equal to 20 mg/dL). There were significantly more cases of coronary involvement in the nontreated group (50%) than in the salicylate-treated group (16.6%) and of coronary aneurysms (39% vs 3%). During the febrile phase of the disease, salicylate serum concentrations achieved with a given dose were on the average twofold lower than during the nonfebrile phase, owing to impaired absorption of acetylsalicylic acid. It is suggested that despite the difficulty in achieving therapeutic serum concentrations of salicylate during the febrile phase of Kawasaki disease with a dose as high as 100 mg/kg/day, this dose is potentially capable of preventing the associated coronary disease.
Prevention of cardiac disease by subcutaneous deferoxamine in patients with thalassemia major.
We examined the efficacy of long-term subcutaneous deferoxamine therapy in the prevention of iron-related cardiac disease in patients with thalassemia major who began treatment after the age of 10 years. Of 36 such patients without preexisting cardiac disease, 19 did not comply with the program of chelation therapy. Over the course of treatment (1977 to 1983) serum ferritin and aspartate aminotransferase levels fell in the compliant group, from mean values (+/- S.D.) of 4765 +/- 2610 to 2950 +/- 1850 ng per milliliter and 58.1 +/- 22 IU to 30 +/- 20 IU per liter, respectively (P less than 0.05), but rose in the noncompliant group, from 5000 +/- 2316 to 6040 +/- 2550 ng per milliliter and 56.6 +/- 20 to 90 +/- 35 IU per liter, respectively. Only one patient in the compliant group acquired cardiac disease and died of fulminant congestive heart failure. In contrast, 12 noncompliant patients acquired cardiac disease, and 7 died. In addition, the mean age of the compliant population (18.9 +/- 4.5 years) now approaches the mean age of acquisition of cardiac disease in the noncompliant group (19 +/- 4.3). These data demonstrate that compliance with treatment with deferoxamine may protect patients from cardiac disease induced by iron overload.
Kawasaki disease: Canadian update.
Kawasaki disease, or mucocutaneous lymph node syndrome, is a multisystem disorder that affects young children. Between 1979 and 1982, 357 patients from 15 university pediatric centres in Canada were reported to have the disease. The diagnosis of Kawasaki disease is based on six clinical features, including fever, conjunctivitis, cracked lips, reddening and swelling of the hands and feet, rash and cervical lymphadenopathy. A scoring system is described that may help predict the development of cardiovascular complications. Coronary artery involvement can be recognized early by two-dimensional echocardiography. Anti-inflammatory therapy, principally with acetylsalicylic acid, is indicated in the acute phase and antithrombotic treatment in the subacute and chronic phases of the disease if coronary artery aneurysms have developed. Prolonged follow-up for patients with aneurysms is necessary. The length of follow-up for patients without aneurysms will depend on the results of studies on patients with Kawasaki disease after they reach adulthood.
A possible increase in the incidence of congenital heart defects among the offspring of affected parents.
The incidence of congenital heart disease in the children of 219 probands was determined. Each of these probands had one of four selected defects: atrial septal defect, coarctation of the aorta, aortic valve stenosis or complex dextrocardia. Of their children, 8.8% had substantial congenital cardiac defects. This is a much higher incidence than that reported in most comparable studies. The difference is highly significant statistically and therefore is likely to be genuine. The cause of the high recurrence is probably environmental.