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Biomedical subjects

S Cullen

Publications and source records attributed to S Cullen.

At least 19 recordsLinked to original sources

Illness perception and symptom components in chronic fatigue syndrome.

Two-hundred and eight patients with chronic fatigue syndrome (post-viral fatigue syndrome) completed a questionnaire which dealt both with their illness in general and with the extent to which they experienced specific symptoms. A factor analysis of the symptom data yielded four components: emotional distress; fatigue; somatic symptoms; and cognitive difficulty. Emotional disturbance is a common feature of the disorder and its role has been widely debated. When the symptom components were considered independently, fatigue, somatic symptoms and cognitive difficulty were associated with questionnaire items relating to general illness severity, but emotional distress was not. Thus negative emotions did not contribute directly to patients' perception of illness severity. They were, however, correlated with the other symptom components. It is argued that this correlation reflects a reciprocal influence, with negative emotions exacerbating fatigue and other key symptoms and the debilitating nature of these symptoms enhancing emotional vulnerability.

Activities of Daily Living

Outcome in neonates with Ebstein's anomaly.

The presentation and outcome of 50 patients with neonatal Ebstein's anomaly seen from 1961 to 1990 were reviewed. The majority (88%) presented in the 1st 3 days of life; cyanosis (80%) was the most common presenting feature. Associated defects, present in 27 infants (54%), included pulmonary stenosis in 11 and atresia in 7. Nine patients (18%) died in the neonatal period; there were 15 late deaths (due to hemodynamic deterioration in 9, sudden death in 5 and a noncardiac cause in 1) at a mean age of 4.5 years (range 4 months to 19 years). Actuarial survival at 10 years was 61%. A new echocardiographic grade (1 to 4 in order of increasing severity of the defect) was devised with use of the ratio of the area of the right atrium and atrialized right ventricle to the area of the functional right ventricle and left heart chambers. Cardiac death occurred in 0 of 4 infants with grade 1, 1 (10%) of 10 with grade 2, 4 (44%) of 9 with grade 3 and 5 (100%) of 5 with grade 4. In a multivariate analysis of clinical and investigational features at presentation, echocardiographic grade of severity was the best independent predictor of death. Neonates with Ebstein's anomaly have a high early mortality rate and those surviving the 1st month of life remain at high risk of late hemodynamic deterioration or sudden death. Echocardiographic grading of severity of the defect permits prognostic stratification.

Abnormalities, Multiple

Potential impact of population screening for prenatal diagnosis of congenital heart disease.

The potential impact of prenatal screening for the detection of congenital heart disease (CHD) was assessed by prospective analysis of 428 consecutive infant admissions to a supraregional centre; 28 (6.5%) did not have CHD and were excluded from analysis. Of the 400 cases with CDH, 396 (99%) underwent fetal ultrasonography but scanning was performed only before 18 weeks' gestation in 200 (50%). One hundred and forty nine (37%) of all cardiac abnormalities and 149/283 (53%) of severe abnormalities were considered to be detectable prenatally in a screening echocardiographic four chamber view had this technique been used. Prenatal diagnosis of severe CHD actually occurred in only eight (2%) cases and was after 30 weeks' gestation in all. There were 181/347 (52%) of all mothers and 177/253 (70%) of the subgroup with severe abnormalities who expressed an opinion volunteered their preference for termination of pregnancy if mid-trimester diagnosis had been available. Mid-trimester detection of congenital heart disease rarely occurs at present despite fetal ultrasound scanning in almost all pregnancies. More than half of all severe congenital heart defects seen in infancy are potentially detectable by screening. Major training at primary scan level and modification of the timing of existing fetal anomaly scanning would be required for a screening programme to be effective.

Female

Growth hormone treatment in idiopathic short stature: a preliminary analysis of cardiovascular effects.

Growth hormone (GH) hypersecretion is associated with an increased incidence of hypertension and cardiac hypertrophy, resulting in excess cardiovascular morbidity and mortality. Abnormalities in the renin-angiotensin-aldosterone (RAA) system have been reported in acromegaly and in normal adults treated with recombinant human GH. The RAA system was investigated in prepubertal children with idiopathic short stature during treatment with recombinant human GH in doses up to 40 IU/m2/week. In addition, left ventricular size and function were assessed by serial echocardiography over an initial 12-month period. Modest and transient increases in blood pressure and body weight were observed during the first 7 days of GH treatment, but this was not accompanied by activation of the RAA system. Echocardiographic parameters of left ventricular size and function remained within the normal range for age and body size. Short-term GH treatment of idiopathic short stature was thus not associated with an increase in risk factors known to be associated with later cardiovascular morbidity. Longer follow-up studies will be required to confirm the safety of high-dose GH in this respect.

Blood Pressure

Pituitary adenylate cyclase-activating polypeptide: a novel, long-lasting, endothelium-independent vasorelaxant.

The vasoactivity of the 27- and 38-amino acid forms of the novel peptide pituitary adenylate cyclase-activating polypeptide (PACAP) was tested in vitro. Both forms of PACAP caused endothelium-independent vasodilation (assayed by their vasodilator action on rabbit aorta). When superfused for 1 min the relaxation EC50 of PACAP27 was 23 +/- 8 nM and of PACAP38 was 152 +/- 66 nM. PACAP was 100-fold more potent than vasoactive intestinal polypeptide (VIP) (PACAP27 shows 68% amino acid sequence homology with VIP), and had a prolonged duration of action, a 1 min exposure to 1 microM PACAP27 lasting 135 +/- 7 min and to 1 microM PACAP38 108 +/- 3 min. Adenylate cyclase activity in homogenates of rabbit aortic smooth muscle cells was increased by PACAP27 and PACAP38 with EC50s of 4.4 and 0.73 nM, respectively. PACAP27 and PACAP38 are potent, long-lasting, endothelium-independent vasodilators.

Adenylyl Cyclases

Congenitally corrected transposition and Ebstein's anomaly of the systemic atrioventricular valve: association with aortic arch obstruction.

Aortic arch abnormalities are uncommon in patients with congenitally corrected transposition of the great arteries. Over a 20-year period, 10 patients with congenitally corrected transposition and Ebstein's anomaly of the systemic atrioventricular (AV) valve were identified. Five neonates had severe systemic AV valve regurgitation with severe coarctation of the aorta (n = 4) or aortic atresia (n = 1) and presented with heart failure. Four died in the neonatal period and one who had coarctation repair died 7 months postoperatively. The remaining five patients with congenitally corrected transposition and Ebstein's anomaly had mild left AV valve regurgitation; none of these had aortic arch obstruction. In neonates who have coexisting Ebstein's anomaly of the systemic AV valve and congenitally corrected transposition, obstruction to aortic arch flow is common. Severe systemic AV valve regurgitation with a morphologic systemic right ventricle may have contributed to low anterograde flow in the ascending aorta prenatally and thereby to the aortic arch abnormality.

Aorta, Thoracic

The miscarriage clinic: an audit of the first year.

We preview the results of the first year in a Miscarriage Clinic set up in 1989 in an effort to improve the support and counselling of women who have a miscarriage. Of 381 patients referred, 79% attended. The only statistically significant difference between the women who attended and those who did not attend was in the proportion of women who had planned their pregnancies (65% versus 33%, P less than 0.01). Of the 300 patients who attended, 4% reported no grief reaction; 75% experienced a reaction which had resolved within one month and 21% experienced a reaction which had not resolved. No factor was identified which could predict the duration of the grief reaction. This audit demonstrates that there is a strong demand and need for this service for couples who experience a miscarriage.

Abortion, Spontaneous

Risk factors for arrhythmia and death after Mustard operation for simple transposition of the great arteries.

From 1965 to 1980, 249 consecutive patients underwent a Mustard procedure for simple transposition of the great arteries. Hospital mortality was 23 of 249 (9.2%); follow-up for 24 years (mean, 11.7 +/- 6.1 yr) revealed 50 of 249 (20%) late deaths, of which seven were noncardiac, six were in the hospital, and 37 were sudden (27 patients with poor hemodynamics and 10 unexplained). Actuarial survival after 1, 10, and 20 years was 85, 75, and 67%. The instantaneous risk of death decreased rapidly after surgery but showed a second peak from 8 to 15 years postoperation. When last seen 87% were in New York Heart Association (NYHA) class I; 69% had sinus rhythm, 20% nodal rhythm, 10% atrial flutter, and 1% paced. Survival in uninterrupted sinus rhythm after 5, 10, and 20 years was 75, 62, and 47%. Nodal rhythm detected by annual ECG occurred in 69 patients. Excluding the early experience, the risk of loss of sinus rhythm was constant in time and constant throughout the experience (2.4%/yr). Atrial flutter was observed in 36 patients. Occurrence of atrial flutter was limited to two discrete surgical periods: the first operations (26 with atrial flutter of the first 50) and an ill-defined period in the middle of the experience. Patients not operated on during these two periods were very unlikely to develop atrial flutter (one of 104, p less than 0.001). As for death, the hazard function showed a bimodal curve, the second peak appearing earlier from 5 to 11 years. If nodal rhythm was noted on the yearly routine ECG, the risk for developing subsequent atrial flutter increased by a factor of 2.1 (p less than 0.05).(ABSTRACT TRUNCATED AT 250 WORDS)

Actuarial Analysis

Isolated pulmonary vein atresia.

Two patients with isolated atresia of the pulmonary veins are described. One patient presented with haemoptysis and the other with recurrent unilateral chest infections. The diagnosis was confirmed in both instances by cardiac catheterisation and pulmonary angiography. One patient died one year after the diagnosis was made and the second child has been stable during a follow up period of three years.

Blood Pressure

Calcitropic hormone levels in polynesians: evidence against their role in interracial differences in bone mass.

Blacks are known to have a higher bone mass than whites and have recently been found to have significantly different levels of calcitropic hormones and other biochemical indices of calcium metabolism. To assess the possible significance of these biochemical differences to interracial differences in bone mass, we have undertaken an assessment of indices of calcium metabolism in Polynesian subjects, since they also have a higher bone mass than whites. Serum concentrations of 25-hydroxyvitamin D were slightly lower in Polynesians than in whites (65 +/- 5 vs. 95 +/- 10 nmol/L; P less than 0.02), but there were no differences between the groups in serum levels of calcium (total and ionized), phosphate, magnesium, 1,25-dihydroxyvitamin D, PTH, calcitonin, alkaline phosphatase activity, and bone gla-protein. Furthermore, urinary excretion of hydroxyproline, calcium, phosphate, magnesium, sodium, and potassium and the tubular maximum for the reabsorption of phosphate were not different between whites and Polynesians. Intestinal strontium absorption was similar in the two groups. In contrast, distal forearm bone mineral content was higher in Polynesians (P less than 0.01) and midupper arm muscle area was also increased in this group (P less than 0.005). It is concluded that the higher bone mass of Polynesians cannot be attributed to alterations in the basal levels of calcitropic hormones, but may be related to their greater muscle mass. It is probable that the previously observed black-white differences in the vitamin D endocrine system are secondary to the effects of skin color on vitamin D synthesis and are not contributory to the lower bone mass of whites.

Adult

Cardiovascular manifestations in Kawasaki disease.

Thirteen patients with Kawasaki disease were reviewed. The average age at presentation was 31 months (range: 6 weeks to 8 years). Mean follow up was 36 months, with a range of 5 to 53 months. Cardiovascular manifestations were present in 9 patients. These comprised pericarditis with myocarditis (2), pericardial effusion (4), coronary artery ectasia (3), coronary artery aneurysms (3), peripheral artery aneurysm (1) and peripheral vasculitis (1). Treatment consisted of aspirin alone in 11 and gammaglobulin with aspirin in 2 patients. One patient with coronary artery involvement died following a myocardial infarction. A large coronary artery aneurysm persists, 31 months after initial detection in another patient. Spontaneous resolution of the cardiovascular complications occurred in the remaining seven patients. The importance of clinical awareness and detailed evaluation and follow up of the cardiovascular system in Kawasaki disease is emphasised. There is also a need, however, to exclude other diagnoses which may have a similar clinical presentation.

Cardiovascular Diseases

Paracetamol suppositories: a comparative study.

Paracetamol suppositories in two different bases were given to children who had fever after operations. Plasma concentrations and the effect on temperature were compared. There was a significant correlation between peak plasma concentrations and maximum drop in temperature. A lipophilic base produced better results than a hydrophilic base.

Acetaminophen

Atlantoaxial instability in Down's syndrome: clinical and radiological screening.

One hundred and thirty children with Down's syndrome were screened for the presence of atlantoaxial instability, using both clinical examination and radiographs of the cervical spine taken in flexion and hyperextension views. Seven children were found to have radiological evidence of atlantoaxial instability, with an atlanto-dens interval greater than 5.0 mm in one or all positions. Although a full clinical history was obtained from the attending parent and each child underwent a complete neurological examination, there were no factors detected which differentiated between those with radiological evidence of atlantoaxial instability and those with a normal atlantodens distance. It is recommended that children with Down's syndrome be screened twice, at the ages of 5-10 years and at 15 years.

Adolescent

Bacterial endocarditis and lymphadenopathy mimicking Kawasaki disease.

Two children whose illness initially fulfilled the clinical criteria for the diagnosis of Kawasaki disease were both subsequently found to have acute bacterial endocarditis and one child had a disseminated non-Hodgkins lymphoma. We describe their clinical course and emphasize the importance of the exclusion of other diagnosis as well as strict adherence to the accepted diagnostic criteria of Kawasaki disease.

Child