PubMed Health⌕ Search

Biomedical subjects

M L Wagner

Publications and source records attributed to M L Wagner.

81 records · Page 5Linked to original sources

Sclerosing cholangitis and ulcerative colitis in a mother and her son.

We present two cases, a 32-year-old mother and her 10-year-old son, both of whom had ulcerative colitis and sclerosing cholangitis. The mother had radiologic as well as pathologic evidence of both diseases, while the child's disease was demonstrated pathologically. A review of the literature indicates the rarity of sclerosing cholangitis in childhood, with no reported instances of a parent-child affliction. The hypothesis that there is a genetic component to the development of sclerosing cholangitis may be supported by this family.

Adult↗

Graft-versus-host disease: gastrointestinal involvement with a rectovaginal fistula.

Bone marrow transplantation is an accepted form of treatment for aplastic anemia and end-stage hematological malignancies. Despite a satisfactory outcome in selected patients, more than one-half develop acute graft-versus-host disease (GVHD), characterized typically by involvement of the skin, liver, and gastrointestinal tract. We describe an unusual case in which a 10-year-old girl developed gastrointestinal symptoms as the only manifestation of GVHD. The patient presented with a rectovaginal fistula and had clinical, radiological, and histological features of gastrointestinal GVHD without evidence of liver or skin involvement.

Anemia, Aplastic↗

Discontinuation of phenytoin and carbamazepine in patients receiving felbamate.

Five patients participated in a controlled discontinuation of phenytoin (PHT) and carbamazepine (CBZ) after a study in which all subjects had felbamate (FBM) added to both PHT and CBZ. Four subjects (three women and 1 man aged 23-36 years) completed the protocol. Mean total seizure frequency per day with PHT and CBZ was 1.33 +/- 0.93 (mean +/- SEM), decreasing to 0.87 +/- 0.71 with addition of FBM, and 0.82 +/- 0.78 after discontinuation of PHT. Only one subject tolerated discontinuation of CBZ; the other three had dosage reductions of 33, 54, and 63%. Toxicity attributable to FBM was not observed, and patients often described less severe seizures. Results from four refractory patients indicated that FBM was able to replace PHT and reduce the need for CBZ. In addition, as PHT dosages were reduced, FBM clearance decreased 21%. As the CBZ dosages were reduced. FBM clearance decreased an additional 16.5%.

Adult↗

High-dose clonidine in a case of restless legs syndrome.

OBJECTIVE: To describe a patient with idiopathic restless legs syndrome (RLS). CASE SUMMARY: A 37-year-old man developed severe symptoms of RLS. Treatment using combinations of levodopa, opioids, benzodiazepines, and baclofen provided only partial benefit or resulted in intolerable adverse effects. Higher dosages of clonidine than previously reported in the literature (0.9 mg/d in divided doses) were needed to completely relieve his RLS symptoms. The only prominent adverse effect was dry mouth. The RLS symptoms returned after subsequent reductions in the dosage. After the dosage of clonidine was again increased, complete relief of the symptoms was achieved again. After several months, clonidine was tapered to zero and the patient entered a period of spontaneous remission. When his symptoms returned four months after clonidine had been discontinued, clonidine therapy was restarted. DISCUSSION: Clonidine alleviated RLS symptoms in 30 of 41 patients reported in the literature, indicating that the adrenergic nervous system may play a role in RLS. CONCLUSIONS: High-dose clonidine appears to be useful in treating RLS when other therapies fail. However, well-controlled, polysomnographic studies are needed.

Adult↗

Allele frequency and likely impact of the glycogen branching enzyme deficiency gene in Quarter Horse and Paint Horse populations.

Glycogen Branching Enzyme Deficiency (GBED), a fatal condition recently identified in fetuses and neonatal foals of the Quarter Horse and Paint Horse lineages, is caused by a nonsense mutation in codon 34 of the GBE1 gene, which prevents the synthesis of a functional GBE protein and severely disrupts glycogen metabolism. The aims of this project were to determine the mutant GBE1 allele frequency in random samples from the major relevant horse breeds, as well as the frequency with which GBED is associated with abortion and early neonatal death using the tissue archives from veterinary diagnostic laboratories. The mutant GBE1 allele frequency in registered Quarter Horse, Paint Horse, and Thoroughbred populations was 0.041, 0.036, and 0.000, respectively. Approximately 2.5% of fetal and early neonatal deaths in Quarter Horse-related breeds submitted to 2 different US diagnostic laboratories were homozygous for the mutant GBE1 allele, with the majority of these being abortions. Retrospective histopathology of the homozygotes detected periodic acid Schiff's (PAS)-positive inclusions in the cardiac or skeletal muscle, which is characteristic of GBED, in 8 out of the 9 cases. Pedigree and genotype analyses supported the hypothesis that GBED is inherited as a simple recessive trait from a single founder. The frequency with which GBED is associated with abortion and neonatal mortality in Quarter Horse-related breeds makes the DNA-based test valuable in determining specific diagnoses and designing matings that avoid conception of a GBED foal.

1,4-alpha-Glucan Branching Enzyme↗

The prevalence of dental erosion in a United States and a United Kingdom sample of adolescents.

PURPOSE: A high prevalence of tooth surface loss due to erosion is well recognized in the United Kingdom (UK), but not in the United States (US). This could be due to prevalence or perception or a combination of both. The aim of this study was to measure the prevalence of erosion of the upper permanent incisors in US and UK samples of 11-13 year old children. METHODS: Convenience samples of 129 subjects were examined in the US and 125 in the UK by two trained examiners. The palatal and buccal surfaces of the upper permanent incisors were assessed for the presence of erosion. Subjects also completed a questionnaire investigating any association between the presence of erosion and possible etiological factors. RESULTS: The prevalence of erosion was 41% in the US and 37% in the UK samples, this difference was not statistically significant. Similarly no statistically significant difference was found between the sexes. The erosion present was confined to enamel in the vast majority of subjects. The questionnaire did not detect any link between the presence of erosion and possible etiological factors. CONCLUSION: The results of this study suggest that dental erosion is common in both US and UK adolescent populations. There is a need for a larger study to investigate this issue further.

Adolescent↗

Faculty have few incentives for becoming board certified in pharmacotherapy.

A telephone survey of all colleges of pharmacy in the United States (including Puerto Rico) and Canada was conducted to assess the incentives offered for pharmacy practice faculty to become board-certified pharmacotherapy specialists (BCPS). Board certification is not a requirement for employment at any college of pharmacy; however, it plays a part in promotion, tenure, and merit salary increases at several schools. Fewer than half of the schools provide at least partial reimbursement of examination-related expenses. Sixteen have initiated BCPS study groups. We perceive that faculty must have more incentives to take the examination. It may be difficult to change institutional policies regarding merit increases or promotion and tenure, but colleges of pharmacy can do more to decrease the cost burden and to promote study groups within the department.

Canada↗

Complications of disease and therapy: a comparison of younger and older patients with Parkinson's disease.

The incidence of complications associated with disease and treatment was compared in younger versus elderly patients with Parkinson's disease (PD). One hundred sixty-five patient records were divided according to patient age into two groups ("younger," 41 to 64, and "elderly," > or = 65 years) and reviewed for the incidence of dyskinesias, fluctuations, freezing, psychosis, dementia, depression, and insomnia. Younger patients had a greater incidence of chorea (75.8 percent vs 49.5 percent), dystonia (82.3 percent vs 49.0 percent), fluctuations (90.1 percent vs 68.1 percent), depression (73.2 percent vs 36.8 percent), and insomnia (57.9 percent vs 18.1 percent). There were no significant differences in the incidence of freezing, dementia, or psychosis. At the time of the first adverse event, there was no difference in patient characteristics such as gender, lag time from disease diagnosis to levodopa initiation, disease symptoms at the time of diagnosis, levodopa dose, or concomitant drug use despite the fact that the older group had a longer duration of disease, higher Hoehn and Yahr stage, an older age at onset of PD, and longer duration of levodopa use. Younger patients with PD experience a greater incidence of adverse effects than do elderly PD patients. The spectrum of adverse effects is comparable to those of young-onset (< or = 40 years) patients.

Aged↗