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Biomedical subjects

J Wilson

Publications and source records attributed to J Wilson.

At least 577 records · Page 32Linked to original sources
PubMed1987

Diagnostic difficulties in infantile neuroaxonal dystrophy. A clinicopathological study of eight cases.

The clinical features of eight children with infantile neuroaxonal dystrophy are presented. Diagnosis was established by brain biopsy (4 cases), conjunctival biopsy (1 case), and the family history (2 cases), while in one case a presumptive diagnosis was made on the combination of clinical and neurophysiological findings without histopathological confirmation. The pleomorphic clinical picture and variable neurophysiological findings make a firm diagnosis difficult without histopathological confirmation. However, in the appropriate clinical context, serial neurophysiological investigations (ERG, VEP, EEG, ENMG) may suggest the diagnosis after the age of 2 years. Conjunctival biopsy is not invariably helpful, and neuroaxonal spheroïds are not always demonstrated in brain biopsies by conventional techniques. However, they were consistently identified using a non-specific esterase stain and by electron microscopy. This technique is described, and the significance of ultrastructural and neuropathological findings in infantile neuroaxonal dystrophy is discussed.

Axons↗
PubMed1987

The use of a computerised database for the diagnosis of a rare neurological syndrome.

A database which runs on an office microcomputer is being developed for the diagnosis of genetically determined neurological disorders. At present about 1100 conditions with their clinical features and 3000 references are stored in the database. We discuss a family with 3 sibs affected by a unique neurological disorder and show how the database is used. The 3 sibs, 4, 5 and 10 years old, show the same clinical course characterized by congenital cataracts, microcephaly, hypotonia, mental retardation, pyramidal signs and choreoathetoid movements starting in early childhood. The parents are first cousins of Bangladeshi origin. This condition does not appear in published report and is not listed in the database. It can therefore be concluded that the sibs have a unique autosomal recessive disorder.

Athetosis↗
PubMed1987

Spatial frequency and selective attention to local and global information.

Probe methods were used to investigate whether the distribution of attention to the local or the global structure of a stimulus affects the detectability of different spatial frequencies. Four experiments are reported in which the detectability of threshold probe gratings of different spatial frequencies was measured while subjects analyzed either the local or the global information from a display. A relative shift in the detectability of low and high frequencies was observed. Low frequencies were facilitated during global processing and/or high frequencies were facilitated during local processing.

Adult↗
PubMed1987

Spatial frequency and selective attention to spatial location.

The effect of spatial attention on the detectability of gratings of different spatial frequency was measured using a probe technique. Three experiments are reported in which the detectability of full-field probe gratings was measured while subjects analyzed stimuli presented in either the central or the peripheral visual field. Selective attention to peripheral stimuli produced a facilitation at low frequencies and a decrement at high frequencies. These effects disappeared under forced-choice presentation.

Attention↗
PubMed1987

Endoscopic diagnosis of gastritis: causative factors in 100 patients.

The frequency of gastritis in relation to its various predisposing conditions is unclear, as is the respective distribution of damage caused by its predisposing conditions. We studied 100 patients with the endoscopic diagnosis of gastritis. The incidence of gastritis in our university gastroenterology endoscopy service was 23%. A history of aspirin (ASA) or nonsteroidal anti-inflammatory drug (NSAID) use was present in 42%. No predisposing factors were found in 28% of cases. Stress gastritis was present in 10% of patients, all of whom were in the intensive care unit. Alcoholism, gastric resection, and portal hypertension were considered causative in 19%. Multiple predisposing factors were uncommon (3%). Coincident duodenal ulcer(s) and erosive duodenitis were common and were more frequent in the patients having idiopathic gastritis (46%) than in those who used ASA or NSAIDs (29%). The antrum was the portion of the stomach most frequently involved. This antral distribution of damage was predominant in both the ASA/NSAID-associated cases and in the idiopathic group. In contrast, patients with stress gastritis were more likely to have involvement of the gastric fundus and body.

Adult↗
PubMed1987

The effects of propranolol and metoprolol on skin blood flow in diabetic patients.

The effect of 2 weeks treatment with propranolol or metoprolol on skin blood flow (SBF) at rest was examined in 12 diabetic patients with essential hypertension in whom gross large vessel disease had been excluded. Neither drug significantly altered resting skin blood flow. However we cannot exclude an important difference between the two beta-adrenoceptor blockers because of the great variability of SBF within subjects. A larger study and/or more accurate methods of measuring SBF are needed to determine if beta 1-selective adrenoceptor antagonists differ from non-selective beta-adrenoceptor blockers with respect to skin blood flow.

Adult↗
PubMed1987

Evaluation of the efficacy of elastic compression stockings in prevention of hypotension during epidural anaesthesia for elective caesarean section.

The ability of graduated compression elastic stockings to prevent hypotension during elective epidural caesarean section was evaluated. Twenty women were randomly assigned to two groups of ten, one group being fitted with the stockings. The incidence and degree of hypotension were the same in both groups. Graduated compression elastic stockings are of no benefit in reducing the incidence of maternal hypotension during caesarean section.

Anesthesia, Epidural↗
PubMed1987

A 30-day forearm work protocol increases maximal forearm blood flow.

To evaluate the local circulatory changes that accompany chronic localized work, we studied the effects of a 4-wk handgrip work protocol on maximal forearm work-related blood flow (ml X min-1 X 100 ml-1) in the nondominant forearms of six normal subjects. The reactive hyperemic blood flow response (RHBF) was also evaluated pre- and posttraining in both forearms of each subject to determine whether maximal vasodilatory capacity would be enhanced. In addition, maximal O2 consumption (VO2max) was measured. We found that chronic handgrip work led to an increase in work-related blood flow (before, 22.4; after, 32.1; P less than 0.05); a drop in work-related minimal resistance (R) (before, 6.4; after, 4.1; P less than 0.05). RHBF rose in the chronically exercised extremity by 30% (before, 33.5; after, 43.7; P less than 0.05) as minimal R fell (before, 3.2; after, 2.2; P less than 0.05). RHBF and R in the unstimulated dominant forearm remained unchanged (blood flow: before, 33.5; after, 31.0; NS; R before, 3.2; after, 3.2; NS). VO2max (ml X kg-1 X min-1) did not change (before, 35.7; after, 34.0). These findings show that localized skeletal muscle forearm work is associated with a localized increase in vasodilation (RHBF). Thus the vascular system appears to be an independent integral partner in the training process.

Adult↗
PubMed1987

Progressive neuronal degeneration of childhood (PNDC) with liver disease.

Thirteen children with progressive neuronal degeneration and liver disease are reported. Clinical features included developmental delay after a normal initial period with later onset of intractable epilepsy. The EEG showed an unusual but characteristic pattern, and visual evoked responses (VER) were abnormal. Rapidly progressive cerebral atrophy was seen on computerized axial tomography (CAT). Inheritance was consistent with an autosomal recessive trait. Pathological findings were neuronal degeneration and spongy change of the cerebral cortex. The calcarine cortex was more severely affected than other areas. Hepatic lesions included severe fatty change and cirrhosis. In six patients liver disease was detected before the onset of epilepsy and exposure to anticonvulsants. Two others were reported to have died from sodium valproate (SV) toxicity, but both had abnormal liver enzymes before treatment with SV, and in both the neuropathological findings were indicative of PNDC. During life, PNDC may be indicated by the characteristic clinical course, abnormal liver function tests, and abnormalities of EEG, VER, and CAT.

Brain↗
PubMed1987

Family interactions surrounding feedings of infants with nonorganic failure to thrive.

Family interactional processes surrounding infant feedings in 34 consecutive cases of nonorganic failure to thrive (NFT) admitted to a university teaching hospital are reported. Observations of family interactions for 90 minutes, including one feeding, yielded clinically useful information not readily available from other sources. Behaviors that supported inadequate feedings were reliably identified in 79 percent of the families. Failure of mothers to appropriately respond to their infants' cues, coupled with few signs of emotional attachment, were present in only 26 percent of cases, and inadequate child-care knowledge and skills in only 6 percent. Thus, widely held conceptual models explained perpetuation of inadequate feedings in only 32 percent of cases. Instead, sibling rivalry, displaced maternal anger, and undermined mother were among the problems found. Assessments of family members' interactions surrounding feedings have the potential to enhance the current management of NFT.

Depression↗
PubMed1987

Factitious illness in children: the social worker's role in identification and management.

Factitious illness in children is a phenomenon that results in adverse consequences for the child. Munchausen by proxy is a factitious disorder, first described by Meadow (1977), in which a parent induces or creates the appearance of illness in the child. This article highlights the importance of social work skills in the identification and management of this syndrome, and proposes a format for decision making at various points in the process.

Adolescent↗
PubMed1987

Abnormal kinetic behavior of cytochrome oxidase in a case of Leigh disease.

Cultured skin fibroblasts from a child with fatal lacticacidemia displayed an abnormally high lactate:pyruvate ratio of 77:1, compared with control values of 22:1-27:1. When protease-treated isolated mitochondria were used, activity of the respiratory-chain enzymes was found to be approximately 60% of normal, and adenosine triphosphate synthesis was found to be normal with all substrates tested. In mitochondria prepared by means of digitonin treatment, adenosine triphosphate synthesis was depressed with all substrates tested, suggesting a defect in the operation of the cytochrome oxidase complex. In disrupted whole cells from the patient, cytochrome oxidase activity was 56% of the activity in the control cell line with the lowest activity. In the presence of a twofold excess of oxidized cytochrome c, patient cells showed 31% of the activity in controls. Cytochrome oxidase activity in both sonicated whole-cell preparations and in sonicated mitochondria displayed abnormal kinetics with regard to the substrate-reduced cytochrome c, which was particularly evident in the presence of excess oxidized cytochrome c. We believe that kinetically abnormal cytochrome oxidase complex is responsible for the biochemical and clinical abnormalities present in this patient.

Acidosis, Lactic↗
PubMed1987

Erythropoietin titers in anemic, nonuremic patients.

Erythropoietin titers when related to the hematocrit percentage and measured by bioassay in 33 normal volunteers and in 61 patients with anemias not complicated by renal or chronic disease were found to overlap with titers measured by radioimmunoassay in 20 normals and 28 patients with similar anemias. Erythropoietin titers measured by radioimmunoassay in 34 patients with rheumatoid arthritis, 25 patients with sickle cell anemia (58 separate samples), and 28 patients with erythroid hypoplasia caused by hematologic malignancies were compared with those in the control group of patients with uncomplicated anemias and found not to differ significantly from titers in this group. Erythropoietin titers measured by bioassay in 12 patients with aplastic anemia also fell within the range of those in the control group. Consequently, erythropoietin titers in these anemias appear to be determined primarily by the degree of anemia and not by any specific effect of these illnesses on the production of erythropoietin.

Anemia↗
PubMed1987

Intestinal obstruction: still a lethal clinical entity.

A retrospective analysis of 70 consecutive patients with a clinical diagnosis of intestinal obstruction from January 1983 to September 1985 was reviewed. Mean age was 62 years. Etiological factors included adhesions 50 percent, malignancy 24 percent, volvulus 12 percent, diverticulitis 7 percent, hernias 4 percent, and radiation enteritis, mesenteric infarction, and perforation of the cecum in the remaining 3 percent. Complications included wound infection 9 percent (n = 6), intra-abdominal sepsis 7 percent (n = 5), and recurrent small bowel obstruction 4 percent (n = 3). Overall mortality was 24 percent (n = 7).Results of the univariant analysis showed no association between the clinical signs of intestinal obstruction, that is, fever, tachycardia, leukocytosis, and local tenderness, and gangrenous bowel. A multiple regression analysis showed, however, that only 14 percent of the variance was able to predict the gangrenous bowel based on clinical signs. In conclusion, the classical signs of intestinal obstruction are poor indicators for compromised bowel, and early surgical intervention will reduce the incidence of ischemic bowel and mortality.

Adolescent↗