Patricia W. Hayden

Myelodysplasia: Decision for Death or Disability

Abstract Treatment of myelodysplastic patients must avoid neglecting a potentially functional child on the one hand and the perpetuation of rejected, brain-damaged cripples on the other. Immediate, complete assessment for brain mass and function and other system anomalies were documented before treatment of 371 patients, with fully informed consent. Of these, 283 had maximum treatment because their brain mass was estimated to be 60 per cent of normal, or greater, and there were no noteworthy complications. The others were treated symptomatically. Seventy-five per cent of patients of normal intellect but paralyzed at high and 90 to 95 per cent of those paralyzed at low levels survived with maximum treatment. Of the patients treated only symptomatically because of criteria excluding maximum treatment, 10 per cent survived to the age of 2 1/2 years. There was a slightly better survival in a group treated symptomatically (42 per cent survival to teen age) before establishment of selection criteria. Children s...

A LONGITUDINAL STUDY OF SHUNT FUNCTION IN 360 PATIENTS WITH HYDROCEPHALUS

Data from an eight‐year clinical study of shunt‐treated hydrocephalus indicated continued shunt dependency in 289 of 360 patients. 919 combined pressure‐radionuclide clearance studies were performed. In 307 studies in which the shunt was identified as nonfunctional, 208 patients were symptomatic for increased intracranial pressure and had the shunt revised. Of the other 99 patients, 71 have remained asymptomatic for three to 60 months (mean 27 months) and 28 subsequently required shunt revision after one to 67 months (mean 24 months). Five other clinical patterns are described. Various methods of monitoring shunt function are recommended.

Psychological adjustment of adolescents with myelodysplasia.

The psychosocial development of 20 adolescents with congenital paralysis due to myelodysplasia is compared to 20 age- and gender-matched subjects with no physical handicap. On many of the measures the myelodysplasia group showed poorer adjustment and lower self-esteem than the controls. Consistent with hypotheses regarding adjustment during adolescence the paralyzed males did show greater concern on Offers scales of Body and Self-Image and External Mastery and showed poorer adjustment on his Sexual Attitudes Scale. The paralyzed girls, particularly those 13 years or younger, showed the poorest emotional adjustment with a significant variability in responses on many of the tests. This degree of variability may indicate a lack of integration of self-concepts and is consistent with an emotional immaturity discordant with advanced physiologic maturation of these girls.

Diuretic renography in evaluating dilated upper urinary tract in children

The diuretic renogram provides a previously unavailable noninvasive method for assessing and following urinary tract dilation of diverse cause, and defining true obstruction in children. Technetium-99m-DTPA (diethylenetriaminepentaacetic acid) is injected intravenously and a posteriorly placed gamma scintillation camera used to image the kidneys, ureters, and bladder. Furosemide is subsequently injected to stimulate a diuresis, and the washout pattern of isotope is monitored (time-activity histogram). Nonobstructive, obstructive, and poor renal function patterns were seen. Forty patients with varying degrees of hydroureteronephrosis were evaluated. Tracings were performed serially and compared with the clinical course in time, as well as with pressure flow studies and operative findings. All patients with a nonobstructed diuretic renogram did well, except for 1 boy in whom ureteropelvic obstruction developed later. Difficulty in evaluating the obstructive renogram pattern occurred only in some children with severe (grade V) hydronephrosis. Further standardization of the diuretic renogram with regard to time of diuretic injection, state of patient hydration, and regions of imaging may improve diagnostic accuracy.

Adolescents with myelodysplasia: impact of physical disability on emotional maturation

Certain physical, psychological and social characteristics of 20 adolescents with myelodysplasia are compared to those of age and gender-matched controls. In addition to the obvious physical differences the areas of greatest concern are self-esteem and social-sexual adjustment. Family relations, feelings, and modes of expression were not different in the two groups. Lack of appropriate chores, decreased opportunities to interact and compete with peers, plus uncertainties to interact and compete with peers, plus uncertainties about bowel and bladder continence appear to be the greatest impediments to emotional growth in this physically handicapped group of teenagers. Early recognition of such problems and finding strategies to overcome them are important aspects of the comprehensive care of any person with a chronic disability.

A Pulsed Transilluminator for the Infant Cranium

This work was underwritten in part by the National Foundation-March of Dimes and The National Institutes of Health Grants #16436 and #5 P01 HD 02774. * Associate Professor, Division of Congenital Defects, Department of Pediatrics, University of Washington School of Medicine, Seattle, Wash. 98195. † Professor of Bioengineering, University of Utah, Salt Lake City, Utah 84112. ** Center for Bioengineering, Univerity of Utah. Correspondence to Patricia W. Hayden, M.D., Department of Pediatrics, University of Washington School of Medicine, Seattle, Wash. 98195. THE PATTERNS of transillumination of

95 Continuous Recording of Cerebral Ventricular Fluid Pressure in Hydrocephalic Children

To quantitate physiologic variations in and the effect of chemical agents on cerebral intraventricular pressure a technique for continuous pressure recording via an indwelling ventricular catheter and strain gauge transducer has been developed. Pressure studies for periods up to 10 days have been made on 12 hydrocephalic children ranging in age from 3 weeks to 5 years. Baseline recordings extending over 24 to 96-hour periods revealed multiple patterns of rhythmic variation with mean pressures ranging from 280 to 50 mm of water. Other physiologic parameters studied included respiratory rate and pattern, electrocardiogram and scalp venous pressure. Serum and ventricular fluid electrolyte and osmolar concentrations have been monitored. Ten of 11 children given oral isosorbide showed an immediate decrease in ventricular pressure ranging from 53 to 100 % of baseline similar to the effect of mannitol and urea. Ventricular wave form flattened during periods of pressure reduction. Five patients studied were also given acetazolamide, urea and mannitol. Acetazolamide increased ventricular pressure 2 to 3 fold with a concommitant exaggeration of pressure wave form. This hypertensive effect was immediate and persisted 2 to 4 hours before returning to baseline levels. Pressure studies were done under relatively isometric, not open manometric, conditions. Studies done with the ventricular fluid system open to flow at various levels of pressure show an early increase and a delayed decrease in cerebrospinal fluid formation with acetazolamide compared to a decrease in flow following isosorbide. (APS)