Complete reversal of moderate/severe brain MRI abnormalities in a patient with classical phenylketonuria.
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Biomedical subjects
Publications and source records attributed to B P Wilson.
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Currarino et al. in 1981 described an association between an anterior sacral defect, congenital anorectal stenosis or another type of low anorectal malformation and a presacral mass. We are only aware of one previous report in the English literature of the association of a rectovaginal fistula with this triad and to our knowledge the association has not been described in the radiological literature.
The intra- and interobserver variability of the measurement of renal length in an unselected series of children attending for renal tract ultrasound were estimated using a standard technique and test-retest protocol. The standard errors of measurement were calculated and the limits of agreement derived. The results indicate that for normal kidneys there is a 95% probability that a second measurement by one observer will be within 4.6 mm of his first reading. Similarly, a single measurement will be within 7.8 mm of a reading by a second examiner. For abnormal kidneys in this series the intraobserver variability was similar to normal kidneys but interobserver variability was greater. Observer error in the measurement of renal length is equivalent to between 2 and 3 years normal growth for children older than 1 year.
The hydrostatic pressures and flow rates of barium sulphate and water soluble contrast in concentrations representative of those used for intussusception reduction were measured. The change of height with discharge of fluid from the filled kit was also assessed. A group of experienced paediatric radiologists and radiographers significantly underestimated the height to which contrast should be placed for intussusception reduction. The results indicate that baseline hydrostatic reduction pressures tend to be less and maximum pressures significantly less than those presently advocated for pneumatic reduction. This disparity may account for the apparent improvement in intussusception reduction rates reported for air enema when compared with barium enema. Intraluminal pressure monitoring during contrast enema would aid control of intussusception reduction but hydrostatic reduction would still be at a disadvantage because of lower flow rates. Where hydrostatic reduction is performed, the contrast density and height used should be set to give known pressure, according to local guidelines.
To find out whether diabetic children may inject their insulin intramuscularly rather than subcutaneously, a random sample of 32 patients aged 4.3-17.9 (median 11.3) years was studied. Distance from skin to muscle fascia was measured by ultrasonography at standard injection sites on the outer arm, anterior and lateral thigh, abdomen, buttock, and calf. Distances were greater in girls (n = 15) than in boys (n = 17). Whereas in most boys the distances were less than the length of the needle (12.5 mm) at all sites except the buttock, in most girls, the distances were greater than 12.5 mm except over the calf. Over the fascial plane just lateral to the rectus muscle the distance from skin to peritoneum was less than 12.5 mm in 14 of the 17 boys and one of the 15 girls. Twenty five of the 32 children injected at an angle of 90 degrees, and 24 children raised a skinfold before injecting. By raising a skinfold over the anterior thigh, the distance from skin to muscle fascia was increased by 19% (range 0-38%). We conclude that most boys and some girls who use the perpendicular injection technique may often inject insulin into muscle, and perhaps on occasions into the peritoneal cavity.
We have reviewed the appearances of 35 patients with 51 fractures of the thoracic and lumbar spine who were seen over the past 3 years; all received plain film radiography and computerized tomography (CT). The types and distribution of fractures are similar to those in series already reported. The severity of each fracture can be described using a three-column model for the functional anatomy of the spine. The liability to orthopaedic instability or late neurological damage can be accurately assessed by computerized tomography. CT demonstrates many fractures of parts of vertebrae not seen on plain films. We propose a classification which is clinically relevant and simple.
A community approach to the provision of hospice care utilizing existing community institutions in the metropolitan Washington, DC, area was started as a pilot project among three entities: the Washington Home, a licensed, skilled nursing facility, which served as the hospice inpatient unit; Georgetown University Hospital and its home care program, which were to serve as the sole referral sources; and Group Hospitalization, Inc., the local Blue Cross Plan. Insufficient referrals resulted in expansion of referral sources to include other hospice home care providers and acute care hospitals in the general community. As other community hospices developed, the Blue Cross Plan expanded the pilot program and fostered the communitywide coordination of hospice services.
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The effect of increasing doses of fenfluramine on dream patterns was studied in 20 patients receiving a reducing diet with or without a controlled dietary intake of vitamin C daily. The dream pattern was unchanged in six patients and dreams disappeared in another who normally dreamed often. In 13 patients dreams increased in frequency and intensity, and in five the dreams assumed frightening proportions. There was a significant straight-line relation between response and the size of the dose. When placebo tablets were given to four patients their dreams disappeared or assumed their pretreatment normal pattern. Absence of vitamin C from the diet did not significantly affect the dream pattern. That fenfluramine has dose-related cerebral effects should be remembered in patients with a history of mental illness.
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An interlaboratory investigation of technical chlordane residues in food crops was carried out to determine the most practical and consistent method of reporting results. Using a technical chlordane reference standard, 8 gas chromatographic stationary phases were studied for their resolution capabilities. The best separations were obtained with SE-30 and its OV-1 equivalent. Using these columns and electron capture detection, potatoes and carrots from supervised field experiments were analyzed in duplicate and quantitated by using 4 methods of calculation. The data were statistically treated to determine the precision and bias for each method. Also, 1 sample was analyzed in duplicate on 2 different occasions by 6 laboratories to substantiate the initial conclusions. Based on the criterion of high precision it is suggested that a comparison of total area under the chromatogram of the sample with total area of standard technical chlordane be the method of quantitation. Only peaks which are common to both standard and sample have any significance in this type of calculation.
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