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Biomedical subjects

K Mathews

Publications and source records attributed to K Mathews.

13 recordsLinked to original sources
PubMed2005

Practice parameter: corticosteroid treatment of Duchenne dystrophy [RETIRED]: report of the Quality Standards Subcommittee of the American Academy of Neurology and the Practice Committee of the Child Neurology Society.

BACKGROUND: The Quality Standards Subcommittee of the American Academy of Neurology and the Practice Committee of the Child Neurology Society develop practice parameters as strategies for patient management based on analysis of evidence. OBJECTIVE: To review available evidence on corticosteroid treatment of boys with Duchenne dystrophy. METHODS: Relevant literature was reviewed, abstracted, and classified. Recommendations were based on a four-tiered scheme of evidence classification, and areas for future research are defined. RESULTS: Seven class I studies and numerous less rigorous trials all demonstrated that corticosteroid treatment for 6 months with prednisone (0.75 or 1.5 mg/kg/day) increased muscle strength, performance, and pulmonary function and significantly slowed the progression of weakness. Two class I trials examined the effect of lower dosage of prednisone (0.30 and 0.35 mg/kg/day), demonstrated lesser but similar benefits, and showed a lower frequency of side effects (e.g., weight gain). The only significant side effects in all class I trials were weight gain and development of a cushingoid facial appearance. One longer-term trial of daily prednisone (0.3 to 0.7 mg/kg/day), a class III study, showed prolongation of functional ability and slower progression of weakness in patients during 3 years of treatment. One class IV, open trial of alternate-day prednisone (2 mg/kg for 2 months, then two-thirds dose every other day) extended ambulation by approximately 2 years in treated compared with untreated patients. Deflazacort, a corticosteroid similar in structure to prednisone, produced similar improvement in muscle strength and function with a similar side effect profile. CONCLUSIONS: Prednisone has been demonstrated to have a beneficial effect on muscle strength and function in boys with Duchenne dystrophy and should be offered (at a dose of 0.75 mg/kg/day) as treatment. If side effects require a decrease in prednisone, tapering to dosages as low as 0.3 mg/kg/day gives less robust but significant improvement. Deflazacort (0.9 mg/kg/day) can also be used for the treatment of Duchenne dystrophy in countries in which it is available. Benefits and side effects of corticosteroid therapy need to be monitored. The offer of treatment with corticosteroids should include a balanced discussion of potential risks.

Adolescent↗
PubMed2001

The doctor on the patient's turf: assessing patient satisfaction with physician home visit programs.

We evaluated a physician home visit program (n = 23 patients) focusing on program implementation and quality. Quality was measured by evaluating patient satisfaction with services using a patient satisfaction scale and interviews with patients, caregivers, and providers. Scale results showed patients expressed the highest satisfaction with access to routine care and physician consideration. Patients expressed less satisfaction with access to emergency care and continuity of care. Physician communication and integration with home- and community-based service providers were other areas of concern. Recommendations include enhancing physician communication skills in the home, providing care for urgent medical conditions, improving chart documentation, and incorporating community-based chronic care experts into the program.

Aged↗
PubMed1998

Presentation, management and follow-up of Schilder's disease.

Schilder's diffuse myelinoclastic sclerosis is a rare demyelinating disease which often mimics intracranial neoplasm or abscess. We have treated 3 patients with this disorder in the past 5 years and followed their postoperative course. Certain distinct features of this disease will allow neurosurgeons to preoperatively entertain this diagnosis. We discuss postoperative treatment and propose a new hypothesis regarding the variable prognoses of this disorder. Schilder's disease constitutes an important diagnosis for any neurosurgeon to be aware of (especially those treating the pediatric age group) which has not received adequate coverage in the neurosurgical literature.

Adolescent↗
PubMed1992

Regional mapping of facioscapulohumeral muscular dystrophy gene on 4q35: combined analysis of an international consortium.

Members of an international consortium for linkage analysis of the facioscapulohumeral muscular dystrophy (FSHD) gene have pooled data for joint analyses, in an attempt to determine the precise location of the FSHD gene and the order of four DNA markers on 4q35 region. Six laboratories determined a total of 3,078 genotypes in 65 families, consisting of a total of 504 affected subjects and 559 unaffected subjects. For each marker, a mean of 648 meioses were informative. D4S139 and D4S163 were identified as the closest linked markers to the FSHD locus, with 99% upper confidence intervals of recombination fractions of .08 and .10, respectively. We have used the CRI-MAP program to construct the most likely order of cen-D4S171-F11-D4S163-D4S139-FSHD-tel, with favorable odds of 10(8)-10(114) over all other orders except that in which F11 and D4S171 are reversed, for which the odds ratio was 191:1. With this order, the genetic map of this region extends 25.5 cM in males and 13.8 cM in females (averaging 19.5 cM for sexes combined); the sex difference was statistically significant (P = .0013). Comparison between families for the two-point and multipoint lod scores involving FSHD showed no evidence for heterogeneity of this disorder. However, after the completion of this analysis, one large family which might show heterogeneity was identified. In view of this and the fact that all of the linked markers reside on the same side of the FSHD locus, the clinical application of these markers is not recommended at this time.

Chromosome Mapping↗
PubMed1990

Generalized motor seizures after portosystemic shunt ligation in dogs: five cases (1981-1988).

Severe generalized motor seizures developed in 5 dogs between 13 hours and 3 days after surgical attenuation of a single extrahepatic portosystemic shunt. Three dogs died, and a fourth was euthanatized because of poor prognosis for recovery. The fifth dog, although improved, was still not clinically normal 15 months after surgery. An intra-or extracranial cause for the seizures could not be identified in any of these dogs. Postmortem examination results in 3 dogs were similar to findings previously reported in dogs with portosystemic shunts, but which did not undergo surgical shunt ligation. Of the 5 dogs, 4 were greater than 18 months old, which may indicate that older dogs are more likely to develop seizures after shunt ligation than are younger dogs.

Animals↗
PubMed1989

Replication blocks and sequence interaction specificities in the codon 12 region of the c-Ha-ras proto-oncogene induced by four carcinogens in vitro.

We have examined the region surrounding codon 12 in the human c-Ha-ras proto-oncogene in vitro to determine the reaction intensities at the guanine nucleotides after exposure to the mycotoxin aflatoxin B1, to one of its human metabolites aflatoxin M1, to dimethyl sulfate, and to the major ultimate carcinogen of benzo(a)pyrene, benzo(a)pyrene diol-epoxide. Among the adducts produced, those at N-7-guanyl sites are alkali-labile and can be identified using a variation of the Maxam-Gilbert sequencing procedure. Data indicate that the guanine nucleotides of codon 12 have above average potential for adduct formation by the genotoxins when compared to other guanine sites, but were not the strongest sites. This codon 12 region has been inserted into single-stranded M13 phage, exposed to several of the genotoxins, and used as a template for DNA synthesis in vitro. There is a sequence-specific variation in polymerase inhibition at various adducted nucleotide sites, but replication blocks are not preferentially seen at the carcinogen-adducted guanines of codon 12. These results indicate that the predominance of point mutations which are detected in vivo at codon 12 do not reflect sequence-mediated preferential susceptibility of these sites to initial DNA adduction or to replication errors. The mechanisms controlling these sequence effects are not currently understood, and attempts to predict relative alkylation or termination frequencies based solely on local DNA sequence are not reliable.

Aflatoxin B1↗
PubMed1986

Cerebral infarction complicating Fontan surgery for cyanotic congenital heart disease.

We report on four children who had cerebral vascular events in the first three months after the Fontan procedure for complex cyanotic congenital heart disease. Potential risk factors in these children included congestive heart failure, postoperative thrombocytosis, and cardiac arrhythmias. These cases suggest that children who undergo Fontan surgery may be at increased risk for cerebral infarction.

Adolescent↗
PubMed1983

The Birmingham blood pressure school study.

Four-hundred and twenty-eight school leavers of 3 ethnic groups (white, black and Asian) were screened for blood pressure, resting pulse rate and general anthropometric characteristics. Asian pupils were both shorter and lighter than the other two groups whilst black males were heavier and taller. There was no significant difference in the mean systolic or diastolic blood pressure between the 3 groups, although the black pupils had a stronger family history of hypertension, particularly on the mother's side. These observations differ in some respects from other ethnic blood pressure studies and establish values for the local population.

Adolescent↗