Biomedical subjects
C M Edelmann
Publications and source records attributed to C M Edelmann.
Treatment of mesangiocapillary glomerulonephritis with alternate-day prednisone--a report of the International Study of Kidney Disease in Children.
It has been claimed that long-term prednisone treatment ameliorates the course of children with mesangiocapillary glomerulonephritis (MCGN). The International Study of Kidney Disease in Children conducted a randomized, double-blinded, placebo-controlled clinical trial in 80 children with idiopathic MCGN, including 42 patients with type I disease, 14 with type II disease, 17 with type III disease, and 7 with nontypable disease. Criteria for admission included heavy proteinuria and a glomerular filtration rate of greater than or equal to 70 ml/min per 1.73 m2. Prednisone or lactose, 40 mg/m2, was given every other day as a single morning dose. The mean duration of treatment was 41 months, renal failure being the most common reason for termination of therapy. Treatment failure was defined as an increase from baseline of 30% or more in serum creatinine, or more than 35 mumol/l. Overall, treatment failure occurred in 55% of patients treated with lactose, compared with 40% in the prednisone group. Life-table analysis showed a renal survival rate (i.e., stable renal function) at 130 months of 61% among patients receiving prednisone and 12% among patients receiving lactose (P = 0.07). Of patients with type I or III MCGN, 33% treated with prednisone were treatment failures, compared with 58% in the lactose group. Long-term treatment with prednisone appears to improve the outcome of children with MCGN.
Development of pediatric nephrology.
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Clinical quiz. Renal tubular acidosis (RTA).
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Urinary tract infection and vesicoureteral reflux.
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Minimal change nephrotic syndrome. Histopathology and steroid-responsiveness.
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The renin angiotensin system in newborn dogs: developmental patterns and response to acute saline loading.
Plasma renin (PRC) and aldosterone concentrations are known to be high during early postnatal life. Whether this is related to the low rates of renal blood flow or to sodium homeostasis remains unknown. Measurement of PRC, renal blood flow, and its intrarenal distribution were performed in 1- to 3-wk-old puppies subjected to maneuvers known to stimulate or inhibit renin release. In the awake state, PRC was observed to be higher in 2-wk-old puppies than in older or younger dogs, (P less than 0.0001). Significant differences in PRC were also found between litters (P less than 0.0001), but they did not account for the age-related changes. Anesthesia resulted in a 3- to 5-fold rise in PRC, whereas saline expansion suppressed PRC at all ages, the fall tending to become progressively greater with age (P less than 0.09). There was no significant correlation between the age-related changes in PRC and those in renal blood flow or its intrarenal distribution. The results of these experiments demonstrate that in the newborn from a qualitative point of view, PRC changes appropriately in response to various stimuli. However, quantitative age-related differences exist in this regard, reflecting an initial immaturity of the feedback system.
Late metabolic acidosis: a reassessment of the definition.
The term "late metabolic acidosis" is generally used to define a population of apparently healthy LBW infants who fail to grow and have a base deficit in excess of 5 mEq/l (CO2TOT less than 21 mM). A relationship between hypobasemia and the lack of appropriate growth was postulated. This conclusion was reached, however, in the absence of adequate information regarding the distribution of acid-base variables in healthy LBW infants. The results of this study demonstrate that the CO2TOT of LBW infants (n = 114) rises between birth and three weeks of life from a mean of 18.6 to 20.3 mM. The frequency distribution of CO2TOT values did not show any significant deviations from normality, and 2 SD included values as low as 14.5 mM. No difference in the rate of growth was detected between "hypobasemic" infants given a solution of bicarbonate calculated to bring their blood CO2TOT to greater than 21 mM and those given similar amounts of isotonic saline solution. The ability of the LBW infants to excrete an ammonium chloride load was not related to their acid-base status and was comparable to that of term infants. It is apparent that the definition of late metabolic acidosis needs to be reconsidered.
Hemodynamic and excretory response of the neonatal canine kidney to acute volume expansion.
It is generally recognized that developing animals retain sodium due to an enhanced reabsorption in distal tubule segements, even when the amount administered is in excess of their needs. This study was designed to test the relationship between this relative inability to dispose of a saline load and the functional characteristics of the kidney during postnatal maturation. In addition, we explored the role played by some of the factors known to affect natriuresis in the adult subject. Measurements of sodium excretion, glomerular filtration rate (GFR), and renal blood flow (RBF) and its intrarenal distribution were made in three age groups of puppies and in adult dogs. During expansion the GFR rose rapidly and to a similar extent at all ages, but it fell thereafter, the rate of decline being much slower in adult than in developing animals (P less than 0.001). RBF and its intrarenal distribution were not altered by volume expansion. The degree of natriuresis did not reflect either the age-related or the expansion-induced changes in GFR. Fractional and absolute sodium excretion were substantially higher in 2-wk-old puppies than in either 1- or 3-wk-old animals (P less than 0.002). These findings demonstrate that the blunted renal response of the maturing animal to saline loading is due to the persistence of an enhanced tubular reabsorption rather than to a limitation in glomerular filtration.
Physiologic adaptations required of a newborn's kidney.
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Proposed selective approach to radiographic evaluation of children with urinary tract infections.
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A simple estimate of glomerular filtration rate in children derived from body length and plasma creatinine.
Based on statistical analysis of data in 186 children, a formula was derived which allows accurate estimation of glomerular filtration rate (GFR) from plasma creatinine and body lenght (GFR(ml/min/1.73 sq m) = 0.55 length (cm)/Per (mg/dl). Its application to clearance data in a separate group of 223 children reveals excellent agreement with GFR estimated by the Ccr (r = .935) or Cin (r = .905). This formula should be useful for adjusting dosages of drugs excreted by the kidney and detecting significant changes in renal function.
Effect of chronic salt and water loading on the tubular defects of a child with Fanconi syndrome (cystinosis).
A male child discovered to have cystinosis and Fanconi syndrome at the age of 2 years 8 months was hospitalized repeatedly for episodes of hyponatremic, hypokalemic dehydration and acidosis. Attempts to correct the fluid and electrolyte abnormalities by increasing progressively the supplements of sodium chloride, sodium bicarbonate, and potassium chloride resulted only in an exacerbation of the symptoms. Tests of discrete renal functions and metabolic balance studies disclosed that, although all disturbances were expressions of the primary disease, their severity was enhanced considerably by the extracellular volume expansion induced by the vigorous therapy. Restriction in water intake resulted in an improvement in the renal performance and in the clinical condition.
Late nonresponsiveness to steroids in children with the nephrotic syndrome.
Among 195 nephrotic children, ten developed resistance to prednisone therapy after responding to this drug on one or more occasions (late nonresponders). All were found to have "minimal lesions" on renal biopsy. Nine of these patients went into remission: one responded to further prednisone therapy, one went into remission while receiving azathioprine, and the remaining seven children responded to cyclophosphamide. Five of these seven patients subsequently relapsed; three of them have continued to respond to prednisone. The other two eventually became steroid resistant a second time, but in both instances a second course of cyclophosphamide again induced a remission. These nine patients have been followed for periods ranging from 6 months to 9.5 years (median = 53 months); all are doing well and have normal renal function. The tenth patient died from sepsis four months after the onset of steroid resistance.
Neonatal changes in renal blood flow distribution in puppies.
The intrarenal distribution of blood flow was studied in 31 newborn mongrel puppies from 18 h to 70 days using xenon washout and krypton autoradiography. Mean renal blood flow increased from 0.39 plus or minus 0.05 ml/g per min (SE) the 1st wk to 2.06 plus or minus 0.12 ml/g per min at 6 wk. During the 1st wk of life renal cortex was perfused homo-geneously at 0.88 plus or minus 0.19 ml/g per min (SE) and accounted for 35 plus or minus 4% of the renal blood flow. During the 2nd wk a narrow, rapidly perfused zone of outer cortex was identified which was perfused at 3.35 plus or minus 0.26 ml/g per min, received 19.53 plus or minus 5.05% of the total renal blood flow, and represented 15 plus or minus 4% of the mass of the total cortex. The inner cortex and outer medulla at this time received 53.40 plus or minus 4.12% of the flow at 1.07 plus or minus 0.08 ml/g per min. Outer cortical flow increased with age reaching adult values by about 6-10 wk when the rapidly perfused area represented 40 plus or minus 8% of the cortex. These changes are parallel to the results of previously reported studies with microspheres in newborn puppies and are compatible with the well established maturational changes noted in neonates of several species. They represent the first gas-washout studies in animals during the first 6 wk of life.
The renal reabsorption of glucose in the developing canine kidney: a study of glomerulotubular balance.
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Renal tubular acidosis.
Renal tubular acidosis (RTA) is recognized to occur in several forms. Primary proximal RTA presents in infancy, almost exclusively in males, and is the only form of primary RTA that is transient. Distal RTA is encountered in familial and sporadic forms, the latter predominantly in females. A severe form of distal RTA in infancy, recently recognized, may represent still another type of the disease.