Regulatory effects of cytokines and cyclosporine A on peripheral blood mononuclear cells from stable multiple sclerosis patients.

The proliferative response (PR) of peripheral blood mononuclear cells (PBMC) to lectins such as phytohemaglutinin (PHA), anti-CD3 monoclonal antibodies such as OKT-3 or phorbol esters such as tetradecanoyl-phorbol 13-acetate (TPA) was investigated in 18 stable multiple sclerosis (MS) patients (9 untreated and 9 treated patients) and 10 healthy controls. PBMC from untreated MS patients showed a significantly higher PR to PHA than healthy controls. The PR of PHA, anti-CD3 or TPA stimulated PBMC from treated patients was lower than that from untreated MS patients. Mitogen stimulated PBMC from untreated patients shown both increased sensitivity to the stimulatory effect of IL-2 and increased resistance to the inhibitory effect of IL-10 and IFN-alpha. The addition of IL-2 increased the PR in PHA-stimulated PBMC from untreated MS patients, but not in those from treated MS patients and healthy controls. Mitogen stimulated cells from untreated patients were more resistant to the inhibitory effect of IL-10 and IFN-alpha than PBMC from either treated MS patients or healthy controls. Cyclosporine A (CsA) inhibited the PR and the expression of activation antigens induced by PHA in PBMC from the three groups of subjects. This inhibitory effect of CsA have was enhanced by the addition of IFN-alpha.     

Ethnic differences in plantar pressures in diabetic patients with peripheral neuropathy

Aims To compare plantar foot pressures between Caucasian and Hispanic diabetic patients with peripheral neuropathy (PN) without a history of foot ulceration and between Caucasian and Hispanic non-diabetic individuals. Methods Forty-four Hispanic diabetic patients with PN (HDPN), 35 Caucasian diabetic patients with PN (CDPN), 41 non-diabetic Hispanic subjects and 33 non-diabetic Caucasian subjects participated. Total and regional peak plantar pressures (PPs) and pressure time integrals (PTIs) were assessed using the EMED-SF-4 plantar pressure system. Results Hispanic diabetic patients with PN had significantly lower peak PP than Caucasian diabetic patients with PN in the entire foot (552.4 ± 227.9 vs. 810.1 ± 274.6 kPa; P < 0.001), forefoot (464.1 ± 222.6 vs. 699.6 ± 323.1 kPa; P < 0.001), hindfoot (296.3.4 + 101.8 vs. 398.1 + 178.3 kPa; P < 0.01) and at the fifth metatarsal head (MTH5; 204.3 ± 143.2 vs. 388.2 ± 273.9 kPa; P < 0.001). The PTI in the entire foot, forefoot and MTH5 were also lower in HDPN than in CDPN. The ethnic differences between the diabetic groups with PN for the entire foot, forefoot and MTH5 remained significant after adjusting for the effect of age, gender, weight and duration of diabetes. There were no significant differences in peak PP and PTI among non-diabetic individuals, except for a lower peak PP at the MTH5 in Hispanic compared with Caucasian subjects. Conclusions Despite a well-known higher incidence of foot complications in diabetic Hispanic subjects, dynamic plantar pressures are lower in Hispanic diabetic patients with PN when compared with their Caucasian counterparts, suggesting that differences in other risk factors exist between these two ethnic groups.     

Acute-phase reactants after paediatric cardiac arrest. Procalcitonin as marker of immediate outcome

Objective  

Procalcitonin (PCT) and C reactive protein (CRP) have been used as infection parameters. PCT increase correlates with the infection's severity, course, and mortality. Post-cardiocirculatory arrest syndrome may be related to an early systemic inflammatory response, and may possibly be associated with an endotoxin tolerance. Our objective was to report the time profile of PCT and CRP levels after paediatric cardiac arrest and to assess if they could be use as markers of immediate survival.     

A new measure for assessing the health-related quality of life of patients with vertigo, dizziness or imbalance: the VDI questionnaire

Vertigo, dizziness and imbalance are frequent symptoms among patients in clinical practice. We present the development of a new multipurpose measure specific to patients with these symptoms: the VDI questionnaire. Items of the VDI questionnaire were generated through an international panel of clinicians, in-depth interviews with 25 patients and an extensive literature search. Items were reduced by qualitative and quantitative methods. Psychometric properties were tested in 130 patients recruited in primary care general practices, neurologists and ENT out-patient clinics. Patients were clinically evaluated and administered several specific and generic health status instruments: the Balance Scale, the SF-12 Health Survey and the General Health Questionnaire 12 items. The VDI symptoms and the VDI health related quality of life (HRQoL) scales were administered during the visit and some weeks after. Correlation and confirmatory factor analysis were used. Reliability of the VDI was high (0.92 for VDI HRQoL scale and 0.86 for VDI symptoms). VDI scores were more correlated with Balance Scale scores than with mental health indicators. All factor loadings were significantly different from zero and confirmed the expected relations. In the second administration, VDI scores showed a deterioration among those reporting a worsening in their symptoms and an improvement among those reporting feeling better. The data presented suggest that the VDI questionnaire is a reliable, valid and responsive instrument for patients with vertigo, dizziness or imbalance. The VDI questionnaire could be a useful complement of the clinical evaluation of these patients at both group and individual level.     

Presentation, acute illness, and learning difficulties in salt wasting 21-hydroxylase deficiency

The presentation, pattern of acute illness, and incidence of learning difficulties are described in 63 (33 boys, 30 girls) children with salt wasting 21-hydroxylase deficiency, drawn from a cohort study of congenital adrenal hyperplasia in the South West Region of England between 1968 and 1988. Thirty boys presented with a salt losing crisis from birth whereas the other three boys presented between 2 and 14 months of age with failure to thrive and hyponatraemia. Diagnostic uncertainty led to 13 (43%) of 30 girls developing a salt losing crisis. Five girls were misassigned as boys at birth. There were four deaths in the group, two due to salt losing crisis, one to complications of prematurity possibly compounded by 21-hydroxylase deficiency, and one from heart failure probably related to an excess of steroids. Acute admissions were common, especially during the first year of life, with convulsions in 7% of admissions. The 9% incidence of hypoglycaemia was considered to be an underestimate as blood glucose was measured in only 56 (22%) of 254 admissions. No convulsions occurred in the 38 (15%) admissions where the parents had given intramuscular hydrocortisone before bringing the child to hospital. A high incidence of learning difficulties was found among the 59 surviving children (9/30 (30%) boys and 6/29 (21%) girls), and in only two children could any factor other than 21-hydroxylase deficiency be invoked. Analysis of the subgroup with learning difficulties indicated that they were more ill at presentation with a significantly higher incidence of hypoglycaemia, and that growth in the first year was significantly worse. It is concluded that congenital adrenal hyperplasia remains a formidable disorder with an appreciable mortality and morbidity. The high incidence of learning difficulties seen in salt wasting 21-hydroxylase deficiency needs further attention. A prospective study is indicated to examine the effect of neonatal screening on morbidity from congenital adrenal hyperplasia, particularly the intellectual impairment seen in this study.     

Clinical predictors of acute radiological pneumonia and hypoxaemia at high altitude

Fast breathing has been recommended as a predictor of childhood pneumonia. Children living at high altitude, however, may breathe faster in response to the lower oxygen partial pressure, which may change the accuracy of prediction of a high respiratory rate. To assess the usefulness of clinical manifestations in the diagnosis of radiological pneumonia or hypoxaemia, or both, at high altitude (2640 m above sea level), 200 children aged 7 days to 36 months presenting to an urban emergency room with cough lasting less than seven days were studied. Parents were interviewed and the children evaluated using standard forms. The results of chest radiographs and pulse oximetry obtained after clinical examination were interpreted blind. Radiological pneumonia and haemoglobin oxygen saturation < 88% were used as 'gold standards'. One hundred and thirty (65%) and 125 (63%) children had radiological pneumonia and hypoxaemia respectively. Crepitations and decreased breath sounds were statistically associated with pneumonia, and rapid breathing as perceived by the child's mother, chest retractions, nasal flaring, and crepitations with hypoxaemia. The best single predictor of the presence of pneumonia is a high respiratory rate, although the results are not as good as those reported by other studies. A respiratory rate > or = 50/minute had good sensitivity (76%) and specificity (71%) for hypoxaemia in infants. Hypoxaemia had a good sensitivity and specificity for pneumonia mainly in infants (83% and 73%, respectively). Logistic regression analysis showed that decreased or increased respiratory sounds and crepitations were associated with pneumonia, and that hypoxaemia is the best predictor when auscultatory findings are excluded. These results suggest that some clinical predictors appear to be less accurate in Bogota than in places at lower altitude, and that pulse oximetry can be used for predicting pneumonia.     

Long-term results of surgical treatment for pulmonary valve stenosis

109 children who survived surgical treatment for isolated pulmonary valve stenosis were followed for up to 17 years. In all the postoperative status was assessed as satisfactory. Cardiac catheterization repeated in 43 gave a resting valve gradient below 40 mmHg. The 22 children whose pulmonary valves had been excised were as healthy as the 87 who had undergone pulmonary valvotomy. Consideration was given to the desirable length of postoperative review. Except for the few children with symptoms before operation, a postoperative increase in exercise tolerance was not a feature.     

Diagnosis and therapeutic options in chronic idiopathic intestinal pseudo-obstruction: review of 16 cases

Chronic idiopathic intestinal pseudo-obstruction (CIPS) is a rare condition in which there is a defective motility of the gastrointestinal tract of unknown cause leading to repeated bouts of intestinal obstruction without organic explanation. This syndrome groups several ill-defined varieties of motor disorders that can sometimes be classified according to the presence of familial incidence and to the presence of muscular or nervous lesions. Nevertheless, a considerable proportion of cases cannot be ascribed to either type. CIPS is a very difficult challenge for pediatric surgeons because our role is never curative and because when we are involved in it is usually as a result of a false diagnosis. We present herein the experience of 2 Pediatric Surgery Departments in this entity. In the last 30 years we have been involved in the management of 16 children with CIPS. Male-to-female ratio was 5:11 and all but 3 patients had symptoms before 6 months of life. Thirteen had abdominal distension, 10 maldevelopment, 9 recurrent bouts of intestinal obstruction, 8 chronic diarrhea, 7 vomiting, 2 dysphagia and 2 constipation. Seven out of the 16 had urinary tract involvement and in three prenatal diagnosis of megacysts was made. The mean delayed time for diagnosis was 3.08 years. Esophageal or antroduodenal manometry was performed in 8 patients and it was abnormal in 7. Histologic and histochemical samples were available in 8 patients, but only in 4 was enough to make a diagnosis of myopathy. Twelve patients underwent 41 surgical procedures. Three are currently included in a program of home parenteral nutrition. Only three have died, and the mean age of the survivors is 13.9 years. In most of the patients with CIPS surgery is only useful for nutritional purposes, for diversion procedures or for intestinal transplantation in extreme cases. Every effort should be made to avoid unnecessary explorations, misdiagnosis and delay in the identification of the syndrome.     

Bilharziasis and bladder cancer. A case report

In our setting, the association between bilharziasis and bladder cancer is uncommon. However, the presence of recurrent haematuria in a patient from an endemic area of bilharziasis (east of Africa and Middle East), is highly suspicious of this disease. Lack of an adequate treatment by using anthelminthic drugs during the acute phase of disease, might lead to the onset of chronic bladder lesions and, on occasion, bladder cancer, of the epidermoid type in the majority of cases. Because of the high mortality and aggressivity of this type of cancer, an early diagnosis and appropriate treatment of acute lesions is mandatory. We report a 40-year-old black male from Senegal, presenting chronic bladder schistosomiasis and infiltrative epidermoid carcinoma of the bladder, diagnosed and treated in our department by palliative cystectomy.