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Biomedical subjects

M C Walter

Publications and source records attributed to M C Walter.

At least 19 recordsLinked to original sources
PubMed2003

Successful treatment of muscle sarcoidosis with thalidomide.

A 36-year-old male patient suffered from therapy resistant sarcoidosis with long-standing contractures, myopathy, skin lesions and pulmonary changes. Low-dose therapy with thalidomide (50 mg/day) was well tolerated, and the patient rapidly improved. Thalidomide was effective for muscular, cutaneous, and pulmonary involvement in our patient. This is the first report on the efficacy of thalidomide in muscle sarcoidosis. Therefore, thalidomide may become a second-line agent in patients with severe muscle and skin involvement, but further studies are warranted.

Adult↗
PubMed2002

Genetic identity of Marinesco-Sjögren/myoglobinuria and CCFDN syndromes.

OBJECTIVE AND BACKGROUND: To describe three Gypsy families with Marinesco-Sjögren syndrome (MSS), demyelinating neuropathy, and recurrent episodes of myoglobinuria in five of the six affected subjects. Because these families originated from the same genetically isolated founder population as did patients with congenital cataracts facial dysmorphism neuropathy (CCFDN) syndrome, and because the two syndromes have clinical manifestations in common, we hypothesized that the two related, albeit distinct, syndromes may represent clinical variants of a single genetic disorder. METHODS: Clinical studies were conducted and linkage and haplotype analyses were performed for the three families. A total of 16 individuals, including the 6 with MSS and 10 unaffected relatives, were genotyped for six polymorphic microsatellite markers from the CCFDN region on 18qter. RESULTS: Linkage analysis of markers in the 18qter region, where we previously had located the CCFDN gene, produced a lod score of 3.55, demonstrating colocalization of the gene responsible for MSS with demyelinating neuropathy and myoglobinuria with the CCFDN gene. Moreover, the patients with MSS shared the conserved marker haplotype found in CCFDN chromosomes. CONCLUSIONS: These data suggest that Marinesco-Sjögren syndrome with peripheral neuropathy and myoglobinuria, and congenital cataracts facial dysmorphism neuropathy syndrome are genetically identical and are caused by a single founder mutation.

Adolescent↗
PubMed2001

[New insights in pathogenesis and therapy of sporadic inclusion body myositis (s-IBM)].

Sporadic inclusion body myositis (s-IBM) is a chronic progressive inflammatory myopathy which occurs preferentially in older patients. Histologic hallmarks are rimmed vacuoles and eosinophilic cytoplasmatic inclusions. The etiology is still unknown, but different pathogenetic mechanisms such as slow virus infection, autoimmunopathogenesis, myonuclear alterations, and mitochondrial defects have been implicated. A relation to neurodegenerative disorders and prion diseases has also been suggested. There is a poor response if any to immunosuppressive therapy. Stabilization of disease progression was shown only by intravenous immunoglobulin (IVIG) therapy. Future findings in the field of s-IBM pathogenesis may result in better therapeutic options.

Aged↗
PubMed2001

Novel approaches to treat muscular dystrophies.

Muscular dystrophies (MD) are a clinically and genetically heterogeneous group of skeletal muscle-wasting diseases. Mutations in the dystrophin gene result in dystrophin deficiency, which constitutes the pathogenic basis of Duchenne and Becker MD (DMD and BMD). Several MD are caused by mutations in other recently identified genes coding for proteins linked to the sarcolemma, the nuclear envelope or the contractile apparatus. In addition, several MD have been mapped to different chromosomal loci and for most of them, the identification of the molecular defect is underway. The immediate result is an ongoing reclassification of the MD into disorders defined not by clinical characteristics but specific genetic mutations. At present, therapy of MD is based on symptomatic treatment and supportive care. Convincing evidence for clinical efficacy is only available for corticosteroids that also suffer from frequent and severe side effects. Up to now, curative therapy is not available, although promising new molecular therapies are under investigation in animal models of MD. Current treatment strategies are discussed and a perspective for effective molecular therapy is given.

Adrenal Cortex Hormones↗
PubMed2000

Creatine monohydrate in muscular dystrophies: A double-blind, placebo-controlled clinical study.

The authors assessed the safety and efficacy of creatine monohydrate (Cr) in various types of muscular dystrophies in a double-blind, crossover trial. Thirty-six patients (12 patients with facioscapulohumeral dystrophy, 10 patients with Becker dystrophy, 8 patients with Duchenne dystrophy, and 6 patients with sarcoglycan-deficient limb girdle muscular dystrophy) were randomized to receive Cr or placebo for 8 weeks. There was mild but significant improvement in muscle strength and daily-life activities by Medical Research Council scales and the Neuromuscular Symptom Score. Cr was well tolerated throughout the study period.

Activities of Daily Living↗
PubMed2000

High-dose immunoglobulin therapy in sporadic inclusion body myositis: a double-blind, placebo-controlled study.

Sporadic inclusion body myositis (s-IBM) is an acquired inflammatory muscle disease of unknown cause. In general, s-IBM presents with slowly progressive, asymmetric weakness, and atrophy of skeletal muscle. There is a mild transitory or nil responsiveness to standard immunosuppressive treatment. A controlled cross-over study of 22 s-IBM patients over 3 months showed a partial improvement in those treated with high-dose intravenous immunoglobulin therapy (IVIG) versus placebo. The present study included 22 patients aged 32-75 years and with a mean duration of disease of 5.2+/-3.6 years. They were randomized by a double-blind, placebo-controlled, cross-over design to monthly infusions of 2 g/kg bodyweight IVIG or to placebo for 6 months each, followed by the alternative treatment. After 6 and 12 months the response to treatment was evaluated, using a modified Medical Research Council scale, Neuromuscular Symptom Score (NSS), the patient's own assessment of improvement, arm outstretched time, and electromyography. No serious side effects were seen, in particular no viral infection and no major cardiac or neurological complications. Overall there was no progression of the disease in 90% of patients, unlike that which might have been expected in untreated patients. A mild and significant improvement (11%) in clinical symptoms was found using NSS, but not with other test procedures. There was a trend to mild improvement in treated patients when using other tests. Individual responses to treatment was heterogeneous. The validity of this study may be reduced by mismatch of groups with regard to age at onset and variability in disease expression. The findings of this study largely confirm those of a previous IVIG trial. Treatment with IVIG may be mildly effective in s-IBM by preventing disease progression or inducing mild improvement. Long-term studies are needed to evaluate further the benefit of IVIG therapy in s-IBM.

Adult↗
PubMed1993

Acquired antral pyloric hypertrophy in the dog.

Acquired antral pyloric hypertrophy is one of the most common causes of pyloric obstruction in the small (10 kg) mature or old dog. Clinical signs include chronic intermittent vomiting after meals and occasional weight loss or abdominal distension. Definitive diagnosis is made by exploratory laparotomy and histologic examination of excised tissues. Most dogs with acquired antral pyloric hypertrophy become clinically normal after surgical correction of the gastric outlet obstruction.

Animals↗
PubMed1989

Furosemide overdose and maximal allowable weight standards.

Adherence to the Maximal Allowable Weight (MAW) standards established by regulation can be difficult for many active duty personnel. We have discovered some members of this group utilizing the potentially dangerous rapid weight loss methods that are commonly seen in patients with bulimia and anorexia nervosa. Two cases of furosemide (Lasix) overdose in active duty members are presented in an effort to enlighten military clinicians concerning this hazardous practice in our patient population. Furosemide overdose related to a scheduled or mandatory weight measurement has not been previously reported.

Adult↗
PubMed1988

Spinal decompressive procedures and dorsal compartment injuries: comparative biomechanical study in canine cadavers.

Effects of decompressive procedures or dorsal injuries on flexion-extension, 4-point bending properties of the L-3 and L-4 motion segment in dogs were quantitated and compared. Hemilaminectomy did not significantly (P less than 0.05) affect mechanical properties of the spine. Bilateral facetectomy caused a small increase in the range of motion and a 56% decrease in ultimate bending strength. Excision of the supraspinous and interspinous ligaments decreased the flexural stiffness of the spine, increased the range of motion of the interspace, and decreased ultimate flexion bending strength 62%. Dorsal laminectomy caused a marked decrease in spine stiffness in all phases of flexion and extension, increased the range of motion, and decreased ultimate flexion bending strength 75%. Seemingly, a hemilaminectomy was preferable to the dorsal laminectomy if adequate decompression and exploration were achieved with the hemilaminectomy.

Animals↗
PubMed1988

Ureterocolonic anastomosis in clinically normal dogs.

Ureterocolonic anastomosis was evaluated in 13 clinically normal dogs. Urinary continence was maintained after surgery, and the procedure was completed without technique errors in all but 2 dogs. Three dogs died within 5 weeks (2 of undetermined causes and 1 of aspiration pneumonia and neurologic disease), and 1 dog was euthanatized 4 months after surgery because of neurologic signs. Two healthy dogs were euthanatized 3 months after surgery for light microscopic evaluation of their kidneys. Five dogs were euthanatized 6 months after surgery for light microscopic evaluation of their kidneys. Gastrointestinal and neurologic disturbances developed in 4 dogs at various postoperative intervals. Plasma ammonia concentration measured in 2 dogs with neurologic signs was increased. Plasma ammonia concentration measured in 5 dogs without neurologic signs was within normal limits. All 5 dogs, in which metabolic acidosis was diagnosed, had high normal or above normal serum chloride concentration. Serum urea nitrogen values were increased after surgery because of colonic absorption of urea. Serum creatinine concentration was increased in 1 dog 6 months after surgery. Individual kidney glomerular filtration rate was reduced in 38% (3/8) of the kidneys from 4 other dogs at 6 months after surgery. Of 5 dogs euthanatized at 3 to 4 months after surgery, 4 had bilateral pyelitis, and 1 had unilateral pyelonephritis. Six months after surgery, pyelonephritis was diagnosed in 40% (4/10) of the kidneys from 5 dogs. The ureterocolonic anastomosis procedure is a salvage procedure that should allow complete cystectomy. However, variable degrees of metabolic acidosis, hyperammonemia, and neurologic disease may result.

Anastomosis, Surgical↗
PubMed1987

Vitamin B-6 metabolism as affected by exercise in trained and untrained women fed diets differing in carbohydrate and vitamin B-6 content.

To assess the effect of exercise on vitamin B-6 metabolism in women, five young-trained, five young-untrained, and five postmenopausal-untrained women were alternately fed four diets and then exercised (80% VO2 max; 20 min) after each diet. The following diets were fed: 2 wk moderate carbohydrate (CHO) (49%) (2.3 mg B-6); 1 wk high CHO (64%) (2.4 mg B-6); 2 wk moderate CHO + B-6 (10.3 mg B-6); 1 wk high CHO + B-6); 1 wk high CHO + B-6 (10.4 mg B-6). Blood was drawn pre- and postexercise and post-30 min and post-60 min of exercise and analyzed for plasma pyridoxal 5'-phosphate (PLP) and total B-6. Twenty-four-hour urines were analyzed for 4-pyridoxic acid and urinary B-6. PLP and total B-6 increased significantly with exercise and decreased significantly from post- to post-60 min for all diets. Four-pyridoxic acid increased significantly from the preexercise day to the day of exercise for all diets. Although there was a significant change in B-6 metabolism with exercise, neither training, increased CHO, nor age had an effect on this change.

Adult↗
PubMed1986

Treatment of severely comminuted diaphyseal fractures in the dog, using standard bone plates and autogenous cancellous bone graft to span fracture gaps: 11 cases (1979-1983).

Severely comminuted diaphyseal fractures in 11 dogs were repaired with standard bone plates that spanned a fracture gap filled with autogenous cancellous bone graft. Five dogs had closed injuries, 4 dogs had open fractures, and 2 dogs had infected nonunion fractures for which previous attempts at internal pin fixation had failed. A second autogenous cancellous bone graft was performed in 3 of the dogs during the healing period. The technique was successful in all dogs. The technique was considered a versatile and relatively simple alternative, compared with meticulous small fragment reconstruction and cortical bone allografts.

Animals↗
PubMed1985

Pylorectomy and gastroduodenostomy in the dog: technique and clinical results in 28 cases.

Pylorectomy and end-to-end gastroduodenostomy are surgical procedures that allow excision of abnormal pyloric tissue and provide improved gastric outflow. These techniques were used for the treatment of benign, malignant, and ulcerative conditions that were judged to be not adequately treatable with pyloromyotomies or pyloroplasties. End-to-end gastroduodenostomy was not much more difficult than a standard intestinal anastomosis; however, a thorough knowledge of the pyloric area anatomy was required to avoid serious surgical errors. In addition, gentle tissue manipulation and precise suture placement reduced the chance of iatrogenic pancreatitis, biliary obstruction, tissue ischemia, and/or suture line leakage. The results of surgery depended on the underlying disease process. Dogs with benign lesions such as chronic hypertrophic pyloric gastropathy responded favorably to treatment. Dogs with malignant disease and perforated ulcers had low long-term survival rate. Pyloric adenocarcinoma was not adequately treated with this method alone.

Animals↗
PubMed1985

Chronic hypertrophic pyloric gastropathy as a cause of pyloric obstruction in the dog.

Six small-breed, middle-age dogs with a history of chronic intermittent vomiting had benign pyloric lesions causing gastric outlet obstruction. Marked similarities were found in clinical signs, pathologic changes, and treatment results. The condition was classified as a syndrome and was named chronic hypertrophic pyloric gastropathy. The appearance of the obstructive lesions at surgery were various forms of mucosal hypertrophy. Microscopically, the syndrome was characterized by mucosal foveolar and glandular hyperplasia, cystic glandular dilatation, superficial mucosal ulcerations, and various cellular infiltrates. The affected dogs were successfully treated by surgical correction of the gastric outlet obstruction.

Animals↗