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Biomedical subjects

B J Reilly

Publications and source records attributed to B J Reilly.

At least 19 recordsLinked to original sources

Prognostic indicators of joint destruction in systemic-onset juvenile rheumatoid arthritis.

We retrospectively reviewed the charts and radiographs of 38 patients with systemic-onset juvenile rheumatoid arthritis, attempting to identify early in the disease course the clinical and laboratory observations most predictive of the later development of destructive arthritis. In 12 of the patients, destructive arthritis developed within 2 years of disease onset. When first examined, these patients could not readily be differentiated from those in whom joint destruction did not develop, but they more commonly had hepatosplenomegaly (p less than 0.04), serositis (p less than 0.01), and a lower mean serum albumin concentration (26.7 vs 31.3 gm/L; p less than 0.02). However, by 6 months after onset, patients with destructive arthritis more frequently had persistent systemic symptoms (92% vs 12%; p less than 0.0001), polyarthritis (67% vs 19%; p less than 0.0005), a lower mean hemoglobin level (95 vs 114 gm/L; p less than 0.001), a higher mean leukocyte count (21.2 vs 10 x 10(9)/L; p less than 0.0003), a higher mean platelet count (794 vs 400 x 10(9)/L; p less than 0.0001), and a higher mean erythrocyte sedimentation rate (43 vs 24 mm/hr; p less than 0.05). Multivariate analysis of the results at 6 months revealed that persistent systemic symptoms and a platelet count greater than or equal to 600 x 10(9)/L were the variables most highly predictive of the later development of joint destruction. We conclude that patients at high risk for the development of destructive arthritis may be identified within 6 months of disease onset, thereby indicating the need for more aggressive early therapy.

Adolescent

Growth failure and bony changes induced by deferoxamine.

We reviewed the linear growth and growth plate morphology in all children with homozygous beta thalassemia followed in Toronto, for whom monthly height percentiles were available before, and for a 36-month period after, the initiation of nightly subcutaneous deferoxamine therapy. All patients were less than 7 years of age when begun on deferoxamine, and had received nightly deferoxamine for a minimum of 36 months. Marked abnormalities of the metaphyseal growth plate were readily observed in the distal ulnar, radial, and tibial metaphyses in 11 of 37 patients in whom a significant decline in mean height percentile was also noted. (In 10 of these 11 patients, height was less than the 15th percentile after 36 months.) These 11 patients had received a significantly greater (p less than 0.025) initial and average daily dose of deferoxamine, and had maintained a significantly lower (p less than 0.025) mean serum ferritin concentration over the 36 months, than the remainder of the cohort. To determine whether deferoxamine played a causative role in growth failure, growth in patients who began deferoxamine before the age 2 years was compared to that of patients who began after age 5 years, for the period between 2 and 5 years of age. Only patients begun on deferoxamine prior to age 2 years demonstrated a significant (p less than 0.01) decline in height percentile by the third year, implicating deferoxamine therapy as the cause of growth failure. We conclude that both the decline in height percentile and the bony changes observed in well-chelated patients are directly related to deferoxamine therapy.(ABSTRACT TRUNCATED AT 250 WORDS)

Alkaline Phosphatase

Necrotizing enterocolitis and volvulus in the premature neonate.

Premature infants presenting to the neonatal intensive care unit at the Hospital for Sick Children with a surgical abdomen over a 5-year period were studied retrospectively to determine the factors leading to the diagnosis of malrotation with volvulus and necrotizing enterocolitis (NEC). Fifteen preterm infants (less than 37 weeks) were diagnosed as having volvulus, and 54 had surgically treated NEC. Those with NEC were more likely to be systematically ill with grossly bloody stools, abdominal tenderness, and thrombocytopenia (P less than .005). Bilious vomiting and bilious gastric residuals were the only hallmarks of volvulus (P less than .005). Although the radiographic findings of thickened bowel walls and intramural air were significantly related to NEC, the accuracy and interobserver reliability in diagnosing these features was variable as was the ability to distinguish NEC from volvulus or normal on plain abdominal radiographs. Volvulus is an important cause of surgical abdomen in the preterm infant and can be misdiagnosed as NEC. An unusual course or the presence of bilious vomiting in any patient thought to have NEC should alert the clinician to the possibility of this diagnosis.

Birth Weight

Ultraviolet irradiation for hepatic rickets.

An infant with chronic cytomegalovirus hepatitis and a child with atypical Alagille's syndrome had vitamin D deficiency rickets due to malabsorption. Both received ultraviolet irradiation. This treatment corrected biochemical abnormalities and healed the rickets. In the infant use of a sunlamp at home maintained normal 25 hydroxy-vitamin D for over a year. Our study shows that ultraviolet irradiation is an effective treatment of hepatobiliary rickets.

Child

John Caffey Award. Intussusception reduction in children by rectal insufflation of air.

Air was used as the contrast medium for colonic studies in 282 patients with suspected intussusception. Intussusception was documented in 118 of these patients (42%), and reduction was achieved in 89 (75%) of those 118. Twenty-nine patients, in whom the intussusception could not be reduced with air, underwent surgery. Seven of these patients required surgical resection of devitalized bowel. In seven others, the intussusception was reduced by the surgeon with difficulty; in 12, surgical reduction was achieved easily. Three perforations occurred with the use of air. These patients were treated surgically without sequelae. This pilot study of 282 patients suggests that air is a safe substitute for hydrostatic reduction of intussusception.

Administration, Rectal

Cavitation of anterior mediastinal masses in children with histiocytosis-X. Report of four cases with radiographic, pathologic findings and clinical follow up.

Four children with histiocytosis-X had large solid mediastinal masses at diagnosis. Follow-up studies in three showed large air cysts occupying the sites of the original masses; the fourth patient had small air cysts at the periphery of the mass. All patients survived. One patient was explored and the large air cysts were in the mediastinum with adherence to the adjacent lung. Lesser pulmonary involvement was noted in the lungs of three of the four patients. Cavitation within mediastinal masses in histiocytosis-X appears to reflect two processes. The first is necrosis within the masses themselves. The second is involvement of adjacent lung with adherence of lung and pleura to the mediastinum allowing air to fill the mediastinal cavities.

Child, Preschool

Bowel perforation with nonoperative treatment of meconium ileus.

Over the last 11 years, 22 neonates were treated with water-soluble contrast enemas to relieve the obstruction of meconium ileus. Fifteen babies had a gestational age of at least 36 weeks, and 16 weighed more than 2,500 g. All presented with clinical findings of a bowel obstruction, confirmed by roentgenograms, and each eventually had high sweat chloride levels. Each neonate had from 1 to 4 water-soluble contrast enemas administered slowly by syringe over 15 to 30 minutes. Eight enemas were successful in relieving the obstruction, four newborns requiring only one enema. Fourteen were unsuccessful, three having more than one enema. Seven of these 14 had intraabdominal pathology that would have required surgery. In five babies the bowel was perforated by the enema, the colon in three, and terminal ileum in two. These perforations were all immediately recognized during the course of the enema and operated on forthwith; a stoma was made in four cases. Only one of these five babies would have required an operation because of a volvulus. There were no fluid or electrolyte disturbances caused by the contrast material, and none of the babies with perforations died. Although this enema technique was successful in one third of cases, and despite the fact that perforations ensued in one quarter of cases, the procedure still seems warranted if the following precautions are taken: establishment of proper temperature, fluid, and electrolyte balance; the radiologist is not rushed, is extremely gentle, willing to repeat the study until no further progress is evident; the surgeon is available for an immediate laparotomy should a perforation occur.(ABSTRACT TRUNCATED AT 250 WORDS)

Diatrizoate Meglumine

Hydrostatic reduction of intussusceptions caused by lead points.

It is generally accepted that an intussusception caused by a lead point will not be reduced by hydrostatic barium enema. This was reported several years ago, and has continued to be a consistent finding, prompting us to attempt hydrostatic barium enema reductions of recurrent intussusceptions in infants and children and also in older children with a first intussusception. However, in the last 9 years we have treated five children whose ileocolic intussusceptions were caused by lead points but which were reduced by hydrostatic barium enema. The histories and physical examinations were not any different than those of the average pediatric patient with an intussusception. The ileocolic intussusceptions diagnosed by barium enema were reduced with adequate reflux of barium into the terminal ileum. However, a residual and persistent filling defect in the colon or ileocecal area made laparotomy mandatory. In all five cases, a lead point was found and resected. This experience has suggested to us that a residual intraluminal filling defect in the barium column following what appears to be adequate flooding of the terminal ileum should be interpreted as a lead point, and an indication for a laparotomy.

Adolescent

The disappearance of free air after pediatric laparotomy.

The disappearance of intraperitoneal free air after pediatric laparotomy has been the subject of much talk and speculation, but little scientific publication. Moreover, there is no unanimous opinion as to the "correct" time of disappearance of such postoperative free air. Eighty-eight pediatric surgical patients ranging in age from newborn to 18 years were studied after each had a laparotomy to determine when the postoperative free intraabdominal air disappeared. Upright abdominal x-rays were taken in each patient starting on the second day after operation and these x-rays were repeated every few days until the free air was no longer visible. No more than eight such single upright abdominal x-rays were taken in any one patient, the majority having only two or three. Free air under the diaphragm(s) was the specific criterion followed until disappearance of the free air. At the time of the x-ray, the temperature was noted, as well as the presence or absence of the following: nasogastric suction, bowel sounds, flatus, stools, drains, and wound infection. The patients in this series fit into three distinct groups: all newborns and small infants had no free air seen on upright x-rays by the second postoperative day. The free air found in older infants and children operated on through small abdominal incisions (eg, McBurney) was gone in most by the third postoperative day, with the longest time being eight days.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent

Long-term follow up of children with the Wilson-Mikity syndrome.

Nine children who survived the development of Wilson-Mikity (W-M) syndrome were studied at a mean age of 7.6 +/- 0.3 years. Persistent respiratory symptoms during the year preceding the study were present in one child. Pulmonary function studies demonstrated a significant degree of airway obstruction in 3 children. Positive methacholine challenge was observed in 6 children indicating bronchial hyperreactivity. Abnormal chest radiographs were present in 3 children. One showed the characteristic appearances of asthma with air trapping, bronchial wall thickening and redistribution of pulmonary vessels. The other child showed minimal residual peribronchial thickening and the third child had normal lungs, but a rather prominent pulmonary artery segment. Echocardiographic studies revealed enlarged right ventricular cavity in 2 children. However, the pre-ejection period and the systolic time intervals were all within normal limits. It appears that unlike patients with bronchopulmonary dysplasia survivors of W-M syndrome have a good prognosis.

Adult

National health insurance: a new imperative.

When viewed from the perspective of the policy analyst, observed inequities in the access to health services and the rising costs of physician and hospital care are among the most important issues confronting the American health delivery system. Recognizing that publicly financed health insurance programs result in a more equitable distribution of medical services, this paper focuses on the components of a national health insurance scheme that not only offers a comprehensive range of benefits but also employs prospective payment and a set of financial incentives to control the costs of care provided by physicians, hospitals, and other health facilities. The national health insurance program proposed in this paper is designed to eliminate or reduce unwarranted expenditures on plant and equipment; the responsibility for approving and funding capital acquisitions is also regarded as an integral component of the program.

Capital Expenditures

Pediatric urography: comparison of metrizamide and methylglucamine diatrizoate.

A non-ionic contrast medium (metrizamide) was compared to an ionic agent (methylglucamine diatrizoate) for pediatric urography. Fifty children were divided into two age groups: under 5, and 5 to 10. In younger children, metrizamide gave more excellent images (19% vs. 0%) and fewer inadequate images (0% vs. 18%) than methylglucamine diatrizoate (p = 0.06). In older children, metrizamide likewise gave more excellent images (44% vs. 14%) and fewer inadequate images (0% vs. 7%). The same pattern was seen when the two groups were combined (excellent, 28% vs. 8%; inadequate, 0% vs. 12%) (p = 0.05). Differences with respect to changes in hematocrit, serum osmolality, serum sodium, and SGOT were statistically significant, but not adverse reactions. In terms of both efficacy and safety, the authors conclude that metrizamide is preferred for pediatric urography.

Child

Renal osteodystrophy in children undergoing continuous ambulatory peritoneal dialysis.

Fifteen children undergoing continuous ambulatory peritoneal dialysis for 0.3 to 2.4 years were evaluated longitudinally for renal osteodystrophy. Immunoreactive parathyroid hormone, 25-OHD, total and ionized calcium, inorganic phosphate, and alkaline phosphatase levels were measured regularly. Skeletal radiographic studies were performed at the onset and conclusion of CAPD and at six-month intervals during therapy. All children received 1,25(OH)2D3 and aluminum hydroxide, and nine received supplemental calcium. Plasma 25-OHD concentrations were normal to elevated, and calcium increased steadily to high normal levels despite a trend to persistent hyperphosphatemia. The increased calcium levels suppressed parathyroid hormone overactivity in only one patient. At the onset of CAPD, nine patients had hyperparathyroid bone disease seen radiographically, three of whom also had rachitic lesions. At the end of CAPD, the hyperparathyroid lesions had improved in four patients, completely resolved in three, and deteriorated in two. Rachitic lesions had completely healed in two patients and improved in the third. However, among the six children without radiographically evident lesions at onset of CAPD, hyperparathyroid bone lesions developed in two and rachitic lesions in two others during CAPD. Although CAPD and appropriate therapy benefited most patients with renal osteodystrophy, the benefits were not uniform, and bone lesions deteriorated in some.

Adolescent

Neonatal ascites due to lysosomal storage disease.

The clinical and radiographic features of four newborns with lysosomal storage disease (LSD) in whom the dominant presenting clinical feature was ascites are presented. The diseases included infantile Gaucher disease, GM I gangliosidosis, infantile sialidosis, and Salla disease. Abdominal distention due to ascites and hepatosplenomegaly, and hypoplastic lungs were seen in all four infants. In the infant with Gaucher disease, the ribs and long bones were markedly thinned. Varying degrees of coarsening of the trabecular pattern of the bones and thinning of the cortex, and a lack of modeling were seen in all patients. Metaphyseal irregularity was noted in the patients with sialidosis and Salla disease. These skeletal radiographic findings may alert the radiologist to the cause for ascites in these patients, which is obscure. In all four patients, there was a history of perinatal death due to the same disease in a sibling; ascites was present in three of the siblings. The diagnosis was missed at autopsy in each of these siblings, underlining the lack of awareness of LSD as a cause for neonatal ascites.

Ascites

Osteomyelitis in the neonate. Clinical aspects and the role of radiography and scintigraphy in diagnosis and management.

Based on experience with 22 cases of neonatal osteomyelitis in 10 years, the authors suggest these patients can be divided into two groups depending on severity of disease. Premature infants requiring umbilical catheterization and severely ill full-term infants constitute a high-risk group; signs are more overt, multifocal infection and joint involvement more frequent, and severe skeletal deformities more common. The patients in the low-risk group had much milder disease but also presented more difficulty in diagnosis because of the vagueness of the presenting signs. Radiographic examination is essential for diagnosis and follow-up of osteomyelitis, particularly limb deformities. Bone scans should be reserved for situations in which the clinical and radiographic findings are equivocal.

Diphosphonates

The embattled hospital: cost control measures versus imperatives for expansion.

As the costs of the American health care system escalate, there is a tendency to identify the biggest cost item and attempt to reduce it to a manageable size. However, since that biggest cost item, the hospital, is a creation of uncontrolled forces within the system as a whole, attempts to manage costs will be limited in effectiveness. The hospital is the end product of an uncontrolled system, a product that displays little understanding of the economic principles of trade-off, efficiency, and productivity. To limit spending in the hospital care system, controls must be rooted in an analysis of the forces that have shaped the hospital's present form and structure. Factors to be considered are those that have defined what a "good" hospital is, who its real consumers are, and what economic and quality control factors must be part of the entire health system. This article suggests modification of public policies relative to medical manpower an insurance, and recommends adoption of reimbursement by Diagnostic Related Groups.

Cost Control