Academic missions of division chiefs in pediatric departments. III. The division chief as a director of fellowship training.
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Biomedical subjects
Publications and source records attributed to F B Stapleton.
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Hyperuricemia is an unusual presenting feature of acute lymphoblastic leukemia (ALL) and is generally associated with a large leukemic cell burden. We describe three children with T-cell ALL who presented with acute renal failure and very high serum uric acid concentrations, despite a relatively small leukemic cell burden. Two of the three patients had normal complete blood counts without circulating blasts or other physical evidence of leukemia. An isolated renal relapse in one case was associated with hyperuricemia, increased renal excretion of uric acid, and renal dysfunction. An unusually high rate of purine catabolism of the lymphoblasts may cause hyperuricemia in these cases. Unexplained hyperuricemia should prompt a search for occult malignancy.
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Changing perspectives in 95 children with poststreptococcal acute glomerulonephritis (PSAGN) in our hospital between 1979 and 1988 are reported. Between 1961 and 1970 an average of 31 +/- 6.3 patients/year with PSAGN were treated and 70% had antecedent pyoderma. In the present study antecedent pharyngitis was observed in 59 children and pyoderma in 36. In comparison to the decade ending in 1970 our data show: (1) a marked decline in the prevalence of PSAGN (P = less than 0.0005), (2) a predominance of antecedent pharyngeal infection (P = 0.044), (3) a decline in urban and an increase in rural patients with PSAGN (P = 0.0483); and in the last decade: (1) a predominance of antecedent pharyngeal infection in children over 6 years of age (P = 0.0009) and (2) a predominance of antecedent pyoderma in black children (P = 0.0004).
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The renal excretion of uric acid in children differs quantitatively, and perhaps qualitatively, from that in adult humans. The younger the child, the greater the renal clearance of uric acid and the greater the excretion of uric acid expressed as mg per kg body weight. During infancy, the reduced ability to maximally concentrate the urine may protect against precipitation of uric acid crystals within the kidney. Conversely, the extremely high urinary uric concentrations places the very small infant at jeopardy during sudden increases in the filtered load of uric acid. Understanding the pharmacologic and physiologic modulators of renal uric acid clearance will allow the pediatrician to minimize the risk of uric acid nephropathy, and to understand the implications of uric acid in the serum or urine in children with fluid and electrolyte disorders. Certainly evaluation of serum and urinary uric acid concentrations is essential in any child with acute renal failure.
A prospective multicenter study was designed to determine the frequency and prognostic importance of hypercalciuria in children with hematuria. Urinary calcium excretion was examined in 215 patients with unexplained isolated hematuria (no proteinuria, urolithiasis, infection or systemic disorder). Hypercalciuria (urinary calcium excretion greater than 4 mg/kg/day) was identified in 76 patients (35%). Compared to patients with normal urinary calcium excretion, children with hematuria and hypercalciuria were characterized by male preponderance, white race, family history of urolithiasis, gross hematuria and calcium oxalate crystals. Renal biopsies were performed in 10 patients with urinary calcium excretion 0.4 to 2.5 mg/kg/day; three had IgA glomerulonephritis, three had glomerular basement membrane thinning, one had proliferative glomerulonephritis and three were normal. Renal biopsies in three patients with hypercalciuria showed focal segmental glomerulosclerosis, hereditary nephritis or no abnormalities. Oral calcium loading tests showed renal hypercalciuria in 26 patients, absorptive hypercalciuria in 15 patients and were not diagnostic in 35 patients. Serum parathyroid hormone, bicarbonate and phosphorus and urinary cyclic adenosine monophosphate concentrations were similar in the three groups of hypercalciuric patients. Urinary calcium excretion after one week of dietary calcium restriction was higher (5.8 mg/kg/day) in renal hypercalciuria than in other hypercalciuric patients (3.4 mg/kg/day), P less than 0.01. One to four years follow-up was available for 184 patients. Eight of 60 hypercalciuric patients developed urolithiasis or renal colic compared to 2 of 124 patients with normal urinary calcium excretion (P less than 0.001). Hypercalciuria is commonly associated with isolated hematuria and represents a risk factor for future urolithiasis in children with hematuria.(ABSTRACT TRUNCATED AT 250 WORDS)
The urinalysis has long been considered an important screening test for hospitalized children. The value of the admission urinalyses, required by hospital bylaws, was assessed for detecting urinary tract disease in children admitted to a day-care unit or impatient medical or surgical services during the months of June and November 1987. Of 2695 admissions, urinalyses were considered essential for diagnosis or therapy in 543 patients (excluded from analysis). In the remaining 2152 patients eligible for screening, urinalyses were obtained in 145 (30%) of day-care unit and 587 (35%) of inpatient admissions. The results of 149 (20%) of screening urinalyses were abnormal (23% abnormal in inpatient and 10% abnormal in day-care unit admissions). The numbers of urinalyses with abnormal results were similar in surgical and medical admissions and between the months of June and November. Pyuria (90 patients) and hematuria (66 patients) were the most common abnormalities in initial urinalyses. Follow-up urinalyses and other diagnostic studies were obtained in 38% of patients with abnormal admission urinalysis results. In subsequent urinalyses, 58% showed persistent abnormalities; however, most subsequent abnormal results were not pursued with additional tests. Urinary tract infections were diagnosed in 6 patients. No other additional diagnoses were determined. The cost of initial urinalyses and subsequent diagnostic studies in these 732 patients during the 2 months was $23,465. The cost per diagnosis of urinary infection was $3911. It is concluded that when hospital bylaws require routine urinalyses at the time of admission, this requirement is frequently ignored and, in addition, abnormal results are often unappreciated or not pursued.(ABSTRACT TRUNCATED AT 250 WORDS)
Group A streptococcus can cause both acute glomerulonephritis and acute rheumatic fever. The occurrence of characteristic acute poststreptococcal glomerulonephritis and acute rheumatic fever in the same patient is rare. We describe a 10-year-old girl with acute rheumatic fever who presented with the typical clinical and biopsy findings of acute poststreptococcal glomerulonephritis.
Our data indicated that self-reports of consumption of salty foods by children and adolescents were associated with 24-hour urinary sodium excretion. Specifically, youths 10 to 18 years of age who selected a poster depicting high-sodium foods excreted significantly more sodium than youths who selected a poster depicting low-sodium foods. Future research is needed to refine simplified self-report measures, to corroborate the validity of the measures, and to extend the studies to other samples, e.g., younger children.
Persistent gross hematuria associated with sickle hemoglobinopathy that fails to respond to conventional supportive therapy represents a difficult management dilemma. Two such patients with protracted, often painful, sickle trait macrohematuria are described. Both patients had normal renal anatomy and vasculature and had failed to respond to bed rest, intravenous hydration, and a trial of oral epsilon-aminocaproic acid. Patient 1 had normal coagulation function. Patient 2 had von Willebrand disease (decreased factor VIII antigen and quantitative ristocetin cofactor activity). Patient 1 responded to intravenous desmopressin acetate at a dose of 0.3 microgram/kg with a 155% increase in factor VIII clotting activity and a 135% increase in ristocetin cofactor and cessation of her macrohematuria within 18 hours after completion of the desmopressin infusion. She remained free of gross hematuria for 5 months with the exception of short-lived trauma-induced hematuria (in three voids) 6 weeks after desmopressin therapy. Patient 2 did not respond to intravenous desmopressin infusion despite a 234% and a 360% increase in factor VIII clotting activity and ristocetin cofactor, respectively. Intravenous desmopressin acetate may be helpful in halting protracted significant macrohematuria associated with sickle trait hemoglobinopathy in some patients when conventional management fails.
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Urinary uric acid excretion was assessed in 38 children to determine whether hyperuricuria was a risk factor in children with urolithiasis. Uric acid excretion (measured per deciliter glomerular filtration rate), and fractional excretion of uric acid were similar in 27 children with hypercalciuria and calcium oxalate urinary stones, in six children with idiopathic calcium oxalate urolithiasis, and in five with uric acid urolithiasis, of whom four were white boys and one was an Asian girl. One boy with a urate stone had cystinosis. Serum uric acid concentrations exceeded 6.0 mg/dl (360 mumol/L) in two children with hypercalciuria and in two patients with idiopathic calcium oxalate urolithiasis. None of the children with calcium urolithiasis had excessive urinary excretion of uric acid. In children with hypercalciuria, uric acid excretion did not change significantly when dietary sodium was increased from 1.0 to 5.0 gm/1.73 m2. We conclude that excessive urinary uric acid excretion is seldom an additional risk factor in children with calcium urolithiasis and that dietary sodium chloride does not have a strong influence on urinary excretion of uric acid in children with hypercalciuria.
Urinary volume in 24-hour urine collections was examined in 50 children with hypercalciuria and urolithiasis or hematuria, 12 with idiopathic calcium oxalate urolithiasis and 36 healthy children. Urinary volume was 22.2 +/- 2.0 ml. per kg. per day in healthy children and 25.4 +/- 2.0 ml. per kg. per day in children with hypercalciuria, and it was similar in children with absorptive and renal hypercalciuria, and significantly lower in children with idiopathic calcium oxalate urolithiasis (12.2 +/- 1.4 ml. per kg. per day, p less than 0.001 from controls and children with hypercalciuria). Volume was not statistically different in hypercalciuric children with and without urolithiasis. Urinary sodium excretion in children with idiopathic calculi was not statistically different from controls. Urine osmolality was similar among the groups. Urinary volume represents a risk factor in children with idiopathic calcium oxalate urolithiasis, and increased fluid intake should be emphasized in such patients.
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