Cholesterol and carotid artery wall in children and adolescents with familial hypercholesterolaemia: a controlled study by ultrasound.

The carotid artery wall was studied with ultrasound in 23 children and adolescents with familial hypercholesterolaemia and in 23 age-matched healthy controls. The study revealed changes in the carotid artery wall related both to familial hypercholesterolaemia and to age. In the control subjects, the carotid artery wall became stiffer with age. In the patients with hypercholesterolaemia, no clear age-dependence was found, but wall stiffness correlated with total and low-density lipoprotein cholesterol. The intimal-medial wall thickness was associated with serum total cholesterol, low-density lipoprotein and triglyceride concentrations, and correlated inversely with the ratio of high-density lipoprotein to total cholesterol. Carotid artery wall properties seem to be associated with the degree of hypercholesterolaemia and the high-density lipoprotein-to-total cholesterol ratio even in children. In childhood and adolescence it is already possible, with ultrasound, to detect changes in the arterial wall related both to familial hypercholesterolaemia and to age.

Segmental colonic motility in patients with anorectal malformations.

BACKGROUND: Constipation is one of the most important functional sequelae in patients with anorectal malformations. The cause of this motility disorder is unknown. AIM: The purpose of this study was to assess total colonic transit time (TCT) and segmental colonic transit time (SCT) in patients with anorectal malformations. METHOD: Ninety patients with anorectal malformations (40 low and 50 high; median age, 7 years; range, 3 to 13) and twenty-five healthy children (median age, 8 years; range, 3 to 14 years) underwent measurement of TCT and SCT by the saturation technique. Ten radiopaque markers were ingested daily for 6 days followed by administration of a single abdominal x-ray on day 7. TCT in days was calculated by dividing the number of retained markers in the whole colon by the daily intake. SCT in four colonic segments (right, transverse, left, rectosigmoid) was described as a percentage of TCT (markers in one segment versus total number of retained markers). In high anomalies the degree of rectosigmoid dilatation was assessed by contrast enemas taken before closure of the stoma and later during follow-up. RESULTS: TCT was significantly (P < .03) prolonged in patients with anorectal anomalies (median high, 2.1 days; low, 1.9 days versus 1.3 in healthy subjects). In patients with high anomalies right SCT was prolonged when compared with low anomalies and healthy subjects (median high, 24% versus low, 10% and normal subjects, 10%; P < .01). The impairment was more severe in patients with very high anomalies (P < .005). Patients with a low anomaly had prolonged rectosigmoid SCT (median low, 65% versus high, 43% and normal subjects, 49%; P < .05). Prolonged right colonic SCT and TCT correlated with symptomatic constipation in patients with high anomalies (P < .05) but not with those who had low anomalies. Impaired overall functional outcome correlated with prolonged right colonic SCT in patients with high anomalies and with prolonged rectosigmoid SCT in patients with low anomalies. There was no correlation between the degree of rectosigmoid dilatation and SCT or TCT. CONCLUSION: Patients with anorectal malformations have abnormal colonic motility. The type of motility disorder in low anomalies is rectosigmoid hypomotility. In patients with high anomalies the motility disturbance is more generalized. The overall functional outcome was strongly related to the degree of these motility disorders.

Radiological signs in newborns exposed to primary Toxoplasma infection in utero.

A total of 44 181 serum samples from 16 733 pregnant women were analyzed for findings suggesting primary Toxoplasma infection. Thirty-seven newborns exposed to maternal primary Toxoplasma infection in utero were studied prospectively with ultrasound, CT, and MRI for signs of intrauterine infection. Their mothers had been treated during pregnancy, and all infants were treated. The children were assigned to three groups according to their mothers' serological status, and the radiological results were compared with the clinical outcome. Although radiological signs were scarce, ultrasound findings combined with maternal serology were found to be significantly related to clinical outcome.

Respiratory health of workers exposed to low levels of chromium in stainless steel production.

OBJECTIVES: To determine whether occupational exposure to chromite, trivalent chromium, or hexavalent chromium causes respiratory diseases, an excess of respiratory symptoms, a decrease in pulmonary function, or signs of pneumoconiosis among workers in an integrated chain of stainless steel production. METHODS: This cross sectional study was carried out in 1993 and the inclusion criterion was a minimum of eight years of employment in the same production department. A self administered questionnaire was collected, and spirometry, measurement of diffusing capacity, chest radiography, and laboratory tests were carried out by a mobile research unit. RESULTS: There were 221 workers in the exposure groups and 95 in the control group. The average duration of employment was 18 years. No significant differences in the odds ratios (ORs) of the symptoms were found between the exposure and the control groups. In a logistic regression analysis age and smoking significantly explained the occurrence of most of the respiratory symptoms. The smokers in the chromite group had significantly lower forced vital capacity (FVC), forced expiratory volume in one second (FEV1), and diffusing capacity than the corresponding values of the control group. The analysis of variance between study groups, smoking, and exposure time, without modelling for interactions, showed that the chromite group had lower values for FVC, FEV1, and diffusing capacity than the other groups. The occurrence of small opacities was more frequent on the chest radiographs of the workers in the chromite group. CONCLUSIONS: An average exposure time of 18 years in ferrochromium and stainless steel production and exposure to dusts containing low concentrations of hexavalent or trivalent chromium do not lead to any respiratory changes detectable by lung function tests or radiography nor to any increase in symptoms of respiratory diseases. The lung function values were lower and the occurrence of radiological findings was more frequent among the workers from the chromite mine than among the controls. The difference was partly caused by differences in age and smoking habits, but evidently also partly by higher exposures more than two decades ago or by the fibrous components of the dust.

Myocardial function in children and adolescents after therapy with anthracyclines and chest irradiation.

Cardiotoxicity is a potential adverse effect of anthracycline (A) therapy. Radiotherapy (XRT) may also cause a variety of cardiac complications. The purpose of the present study was to evaluate these cardiac side-effects in children and adolescents treated for cancer. We assessed the cardiac status of 91 patients, divided into three groups: Group A (n = 53) had anthracyclines at a mean cumulative dose of 410 mg/m2, group A+XRT (n = 26) had both chest irradiation (XRT) and A (mean 360 mg/m2), and group XRT (n = 12) had XRT alone. The patients differed from the controls in both systolic and diastolic indices of myocardial function. In echocardiography, the left ventricular (LV) contractility was abnormal in 32% in group A, in 50% in group A+XRT, and in 8% in group XRT. In radionuclide cineangiography, the LV ejection fraction was subnormal in 19% in group A, in 24% in group A+XRT, and in 1 patient in group XRT. A higher cumulative dose of A predicted decreased contractility. Treatment with A and/or XRT often leads to cardiotoxicity. Although in most cases this cardiotoxicity seems to be mild and subclinical, the long-term clinical sequelae merit further evaluation.

Effects of bone marrow transplantation on myocardial function in children.

Of 41 pediatric patients currently alive after total body irradiation (TBI) and bone marrow transplantation (BMT), 30 (allogeneic 20, autologous 10) participated in the study. Pre-transplant therapy included high-dose cyclophosphamide (CY) and TBI (n = 12), high-dose CY alone (n = 4), high-dose Ara C and TBI (n = 5), cisplatinum, high-dose melphalan, VP-16 and TBI (n = 9). Acute cardiotoxicity was suggested by a > 15% decrease in the QRS voltage sum of the limb leads in all patients. Late cardiotoxicity was evaluated 0.5-10 years (median 5 years) post-transplant by ECG, chest radiograph, radionuclide cineangiography (RNCA) and echocardiography (ECHO). Six patients had a persistent decrease in the QRS amplitudes. They were all asymptomatic but had abnormal systolic function at the time of the study. BMT patients differed from their controls in the mean values of both the systolic and diastolic indices of myocardial function shown by RNCA and ECHO. Treatment was associated with decreased myocardial contractility. Isovolumic relaxation time and deceleration time were longer in BMT patients than in controls. Myocardial damage seemed to be worst after CY while high-dose Ara C was tolerated best. We conclude that both acute and late cardiotoxicity may occur after BMT, calling for long-term cardiac follow-up.

Transient ischemic cerebral lesions during induction chemotherapy for acute lymphoblastic leukemia.

Ninety children were treated for acute lymphoblastic leukemia or non-Hodgkin lymphoma during 1986 through 1992 in the Children's Hospital, University of Helsinki, in Finland. During induction chemotherapy, nine of the children had visual hallucinations progressing to confusion and seizure. The symptoms were often preceded by severe constipation and significantly elevated blood pressure. Neuroradiologic examinations showed bilateral cortical or subcortical white matter lesions. Despite the stroke like manifestations, the lesions were reversible. The triangular shape and location of the lesions in the watershed areas between the major cerebral arteries suggest vascular ischemia as the cause.

Early high-dose phenobarbital treatment for prevention of hypoxic-ischemic brain damage in very low birth weight infants.

In a randomized prospective trial, we studied the effect of early high-dose phenobarbital treatment on the early (intraventricular hemorrhage) and late (neurodevelopmental abnormalities) manifestations of hypoxic-ischemic encephalopathy in preterm infants weighing 1500 g or less at birth. The first intravenous dose of 15 mg/kg was given at a mean age of 110 minutes, followed by 15 mg/kg after 4 hours and then by 5 mg/kg at 24-hour intervals for 5 days. The overall incidence of intraventricular hemorrhage was 32% in treated and 46% in control infants, a nonsignificant difference. An ultrasound brain scan at 9 months old revealed no significant difference in the incidence of ventricular dilatation between treated (19%) and control (29%) infants. At 27 months, a similar incidence of mild (10%) and severe (10%) neurodevelopmental handicaps was found in both treated and control groups. Since beneficial effects could not be documented by any of the criteria used, we conclude that routine administration of phenobarbital to low birth weight infants is not justified.

30-year follow-up of the original Sulamaa (end-to-side) operation for oesophageal atresia.

Between 1949 and 1955 end-to-side anastomosis was performed on 26 patients with oesophageal atresia at Helsinki Children's Hospital. During the same period, 28 patients with oesophageal atresia were operated by the end-to-end method. The re-fistula rate and rate of anastomotic leaks did not differ significantly. After 30 years, four patients operated with the end-to-side method were alive and were re-examined. All four are well and lead a normal life. Three have no symptoms and one patient has minor swallowing symptoms. In oesophagograms two patients had grossly pathological peristalsis and two had slightly disturbed oesophageal motility. For comparison five patients operated with the end-to-end technique in 1951-1956 were also re-examined. One was symptom-free, the other four had minor symptoms on swallowing, but all were satisfied with their oesophagus. Two oesophagograms showed slight dysfunction, two were moderately pathologic and one was grossly pathologic. The oesophagograms correlated poorly with the subjective symptoms. The long-term prognosis of both end-to-side and end-to-end operated patients with oesophageal atresia seems equally good, all the re-examined patients leading a normal life.

Colon interposition or gastric tube? Follow-up study of colon-esophagus and gastric tube-esophagus patients.

Between 1963 and 1980, 34 elective esophageal reconstructions were performed on 29 esophageal atresia patients. Five patients needed two reconstructions. Among 20 colon replacement procedures there were 2 early deaths and three transplants failed. In 14 gastric tube reconstructions there was no mortality, but two tubes failed. The mean follow-up age of the 15 colon esophagus patients was 11.8 yrs, while for the 12 gastric tube patients it was 5.6 yrs. One death occurred during the follow-up period in each group. Late complications occurred in 7/15 of the colon and 3/12 of the gastric tube groups. Most of the serious complications occurred within 3 yr after surgery. The previously reported malabsorption following colon interposition seemed to be transient. At follow-up all but 3 patients with no significant other anomalies were within two standard deviations of the mean of height and weight. All were satisfied with their new esophagus. Nine out of 14 of the colon and 7/11 of the gastric tube groups were without symptoms, the others having only minor complaints. It is concluded that both colon replacement and gastric tube are satisfactory methods for esophageal reconstruction, and the long-term function seems equally good. However, the gastric tube procedure is easier to perform, has less mortality and fewer complications than colon replacement.