Search PubMed⌕ Search

Biomedical subjects

D F Geary

Publications and source records attributed to D F Geary.

42 records · Page 3Linked to original sources
PubMedDate not supplied

Manifestations and treatment of Schimke immuno-osseous dysplasia: 14 new cases and a review of the literature.

UNLABELLED: Schimke immuno-osseous dysplasia (SIOD) is a rare autosomal recessive spondylo-epiphyseal dysplasia. The characteristic features of SIOD include 1) short stature with hyperpigmented macules and an unusual facies, 2) proteinuria with progressive renal failure, 3) lymphopenia with recurrent infections, and 4) cerebral ischaemia. Although 25 patients have been reported with this disorder, the clinical course and phenotype of SIOD are not well characterized. This report summarizes the clinical findings, course and treatment of reported patients and includes 14 additional patients with SIOD. We emphasize the high incidence of cerebral ischaemia and ocular abnormalities, define the high incidence of thyroid dysfunction and blood cytopenia, and confirm the absence of effective and durable medical therapies. CONCLUSION: Schimke immuno-osseous dysplasia is a multi-system autosomal recessive disorder with variable expression that affects the skeletal, renal, immune, vascular, and haematopoietic systems. Medical therapy is limited especially for more severely affected individuals.

Adolescent↗
PubMedDate not supplied

Efficacy of gastrostomy feeding in infants and older children receiving chronic peritoneal dialysis.

OBJECTIVE: To assess the efficacy of supplemental gastrostomy tube (g-tube) feeding in infants and children receiving chronic peritoneal dialysis (CPD). DESIGN: Retrospective observational study. SETTING: Pediatric nephrology division of tertiary care center. PATIENTS: Fifteen patients undergoing g-tube insertion while receiving CPD were included in the study, and were subdivided, on the basis of age, into the following groups: infants (< or = 2.5 years, n = 8); older children (> 2.5 years, n = 7). MAIN OUTCOME MEASURES: Assessment of the effect of CPD and g-tube feeding on statural growth assessed by height standard deviation score (SDS) and percentage weight-for-height, and measured anthropometric variables including triceps skinfold thickness (TSF), midarm muscle circumference (MAMC), and midarm mean circumference (MAC). Assessment of the effects of CPD and g-tube feeding on measured biochemical variables including total protein, albumin, cholesterol, triglycerides, and high density lipoprotein. RESULTS: No significant change in height SDS was observed; however, the reported continuing decline in height SDS in infants was arrested. Percentage weight-for-height increased significantly in infants at 6 months (p = 0.008) and 12 months (p = 0.006) following initiation of g-tube feeding. An increase was also observed in the older child group, being significant at 12 months (p = 0.031) following g-tube insertion. Increases in all anthropometric variables occurred in the infant group during CPD and post g-tube insertion, significant only for MAMC at 12 months (p = 0.037) post g-tube insertion. In older children little change occurred during CPD, with all variables increasing post g-tube insertion, significant only for MAMC at 6 months (p = 0.02) and 12 months (p = 0.02). An increase in total protein and albumin was noted; however, no significant changes in any biochemical parameters were observed. CONCLUSIONS: Supplemental g-tube feeding facilitates weight gain in infants and older children receiving CPD and arrests the decline in height SDS traditionally observed in infants with end-stage renal disease. No significant alteration was observed in measured biochemical variables, although an increase in total protein and albumin was noted.

Anthropometry↗
PubMedDate not supplied

Peritonitis in children undergoing dialysis. 10 years experience.

The clinical aspects of peritonitis were reviewed in 83 patients treated with continuous ambulatory or continuous cyclic peritoneal dialysis between May 1978 and April 1988. Peritonitis occurred in 50 patients whose mean duration of dialysis was 17.8 months, but not in 33 patients with a mean duration of dialysis of 10.4 months. The mean time from starting dialysis to the first episode of peritonitis was 7.1 months. The peritonitis rate was lower for continuous cyclic than for continuous ambulatory peritoneal dialysis (1 episode per 12.9 vs. 1 episode per 8.1 patient months, respectively). In 39% of the episodes, gram stain of the dialysate was positive. The dialysate leukocyte count was higher in gram-negative than in gram-positive peritonitis. Seventy percent of the peritonitis episodes were gram positive, and Staphylococcus aureus was predominant. Only 1 of the 7 diapered infants had gram-negative organisms associated with peritonitis. Catheters were replaced in 48 cases, 26 because of infection. Sixty-nine percent of the patients were cured with antibiotic therapy alone. Although peritonitis was associated with a mortality rate of 1.2%, peritoneal dialysis remains the favored dialytic mode for children.

Catheters, Indwelling↗