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Biomedical subjects

R R Howell

Publications and source records attributed to R R Howell.

At least 19 recordsLinked to original sources
PubMed1993

Prescription-writing errors and markers: the value of knowing the diagnosis.

BACKGROUND AND OBJECTIVES: Duplicate prescription pads are used in many family practice residency programs to monitor prescribing patterns and detect errors in the actual prescription-writing process. This study looked at whether the review of duplicate prescriptions could be enhanced by adding the patient's diagnosis to the prescription. METHODS: The prescription-writing errors of Shaughnessy and Nickel were revised to include prescription-writing markers. These markers were defined as either an indication of use or a duration of therapy that differed from current medical literature or manufacturers' recommendations. Duplicate prescriptions of first-year family practice residents were reviewed for prescription-writing errors and markers before and after an in-service training regarding prescription writing. RESULTS: Following the in-service training, the number of prescriptions containing the patient's diagnosis increased significantly (20% to 61%). Nineteen percent of all prescriptions contained prescription-writing errors. Eight percent of the prescriptions with the patient's diagnosis contained prescription-writing markers. CONCLUSIONS: The addition of the patient's diagnosis to the prescription enhanced the review of duplicate prescriptions. It permitted the evaluation of not only the prescription-writing process but also the decision-making process that led to writing the prescription. It also permitted a more thorough evaluation of appropriate drug use.

Diagnosis↗
PubMed1988

Echocardiographic abnormalities in the mucopolysaccharide storage diseases.

The mucopolysaccharide storage diseases express themselves clinically with a wide variety of abnormalities, including growth and mental retardation, skeletal abnormalities, clouded corneas, nerve compression syndromes, upper airway obstruction and cardiovascular involvement, to name the most common. In most cases the cause of early death is cardiorespiratory failure secondary to cardiovascular involvement and upper airway obstruction. The findings of cardiac ultrasound examination in 29 children, adolescents and young adults are presented. In addition to the previously well-described abnormalities of the mitral and aortic valves in several types of mucopolysaccharide storage disease, we report patchy involvement in some cases, 3 instances of asymmetric septal hypertrophy not previously reported in mucopolysaccharide storage diseases, cardiac involvement in half of our patients with Sanfilippo syndrome and a lack of age-related severity of cardiac involvement even within the specific syndromes.

Adolescent↗
PubMed1988

Hypertension in a child with type IA glycogen storage disease.

Hypertension and proteinuria were observed in a 2-year-old child with type IA (von Gierke's) glycogen storage disease (GSD). She had evidence of hyperfiltration and had elevated selective renal vein renins. On renal biopsy, increased mesangial cell matrix and cellularity were observed with focal thickening and irregularity of the basement membrane. This case may be representative of the early renal findings in type IA GSD.

Female↗
PubMed1987

Magnetic resonance imaging in the diagnosis of the cranio-cervical manifestations of the mucopolysaccharidoses.

Sixteen magnetic resonance (MR) studies were performed in eight patients with mucopolysaccharidosis (MPS). In patients with Hunter, Hurler, and Scheie syndromes, multiple areas of increased signal intensity were noted in the periventricular white matter. Computerized tomography (CT) frequently failed to demonstrate these white matter lesions. Other findings included spinal cord compression, hydrocephalus and airway obstruction due to soft tissue thickening around pharynx. In patients with Morquio syndrome, cervical spine dislocation, spinal cord compression and hydrocephalus were diagnosed by MR. MR was superior compared to CT, plain films and plain tomography, as the narrowing caused by bone and soft tissue changes were better seen with MR. Our experience suggests that MR should be the primary imaging modality for the detection of cranial abnormalities in patients with MPS. High resolution surface coil imaging may be preferable to invasive procedures such as myelography and CT with intrathecal contrast agents for the evaluation of cervical spine disease.

Adolescent↗
PubMed1985

Detection of human acid alpha-glucosidase in fibroblasts using monoclonal antibodies in a biotin-avidin amplified ELISA.

A sensitive and specific immunological assay for detection of human lysosomal alpha-glucosidase was developed using a mouse monoclonal antibody incorporated into a biotin-avidin amplified ELISA. The immunoassay was more than 60 times more sensitive than the currently used enzymatic assay for alpha-glucosidase activity using a fluorimetric substrate. This methodology provides an alternative approach with increased sensitivity for screening individuals for alpha-glucosidase deficiency.

Antibodies, Monoclonal↗
PubMed1985

Ectodermal manifestations in Menkes disease.

Hair and skin pigmentation changes are described in males with Menkes disease from birth to 12 years of age and in 28 obligate carrier or at-risk females. Pili torti were observed in all affected males and in 43% of the females studied. The presence of pili torti may be considered a reliable diagnostic feature of the carrier state. Suggestions are given for evaluation of the hair in individuals in Menkes pedigrees.

Brain Diseases, Metabolic↗
PubMed1983

Ruptured aortic valve cusp: a complication of the Heimlich maneuver.

A case of traumatic rupture of the aortic valve as a complication of the Heimlich maneuver is presented. Conformation was made by comparative echocardiographic studies available from three months before and immediately following the incident. The patient refused surgical intervention and died one month later with severe congestive heart failure despite vigorous medical therapy.

Aged↗
PubMed1983

X-ray fluorescence spectrometry in the analysis of trace elements in human milk.

XRF has broad applications in the quantitative analysis of trace elements in biological materials. Evaluation of its potential for the analysis of trace elements in human milk was performed. Precision of the method was demonstrated by the low (less than 3%) coefficient of variation in reproducibility of analyses performed within a given day, on different days, and by different operators. Standard curves were constructed for human milk and correlation was high (r greater than 0.98) for each of nine metals analyzed. The lower limits of determination and minimum detection limits were calculated for nine elements. Excellent correlation (r greater than 0.99) was achieved in comparison of XRF TO AA in analysis of zinc in human milk. Preliminary results of the analysis of milk zinc, copper, and iron throughout the initial 8 months of lactation disclose a decrease in trace metal concentration, most notable for zinc, during the first 60 days.

Copper↗
PubMed1981

Synthesis of a metallothionein-like protein in cultured human skin fibroblasts: relation to abnormal copper distribution in Menkes' disease.

A metallothionein-like protein (MTP) is synthesized in normal diploid human skin fibroblasts cultured in Zn- or Cu-supplemented medium. Synthesis of MTP is not detected in cells cultured without metal supplementation of complete tissue-culture medium. Cultured fibroblasts from patients with Menkes' disease accumulate excess Cu which chromatographs both with high-molecular-weight protein(s) and with a Cu-MTP. Under normal culture conditions, the Menkes' MTP incorporates [35S]-cystine, but not appreciable amounts of 65Zn. However, Menkes fibroblasts retain the ability to incorporate 65Zn into MTP in response to Zn supplementation of the medium. The results do not support the idea that Menkes' disease results from a failure of Cu to bind to MTP, but rather that an elevated intracellular Cu concentration in Menkes' disease fibroblasts leads to association of excess Cu with high-molecular-weight protein, stimulating synthesis of a Cu-binding MTP.

Brain Diseases, Metabolic↗