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Biomedical subjects

C F Piel

Publications and source records attributed to C F Piel.

At least 19 recordsLinked to original sources
PubMed1989

Familial hypophosphatemic rickets: bone mass measurements in children following therapy with calcitriol and supplemental phosphate.

Familial hypophosphatemic rickets is characterized by defective skeletal mineralization resulting in abnormal growth and development. The pathologic and radiologic correlates of this syndrome have been given some investigation, but the effect of this mineralization defect on bone mineral density has not been adequately assessed. We measured axial and appendicular bone mineral in 17 children (mean age 5.59 +/- 4.87) with familial hypophosphatemia at baseline and at 6-month intervals after initiation of therapy with vitamin D3 (calcitriol) and phosphate supplementation. Noninvasive quantitative techniques included single photon absorptiometry (SPA) of the radius, combined cortical thickness (CCT) of the second metacarpal, and quantitative computed tomography (QCT) of vertebral trabecular bone. Thoraco-lumbar and hand/wrist radiographs were qualitatively assessed for the prevalence and severity of osteosclerosis, rickets, and other parameters indicative of metabolic bone disease as well as skeletal age. Quantitative determinations of bone mineral by each technique were compared with normal values for age and sex, and individual standardized scores (z-scores) were calculated at each measurement interval. Standard scores were also calculated for bone age-adjusted mineral values. At baseline, spinal trabecular bone by QCT was not significantly different from normal values; however, measurements of peripheral cortical bone by either SPA or CCT were significantly lower than values for normal children of the same age and sex (P = 0.05 and P = 0.01, respectively). Following therapy with calcitriol and phosphate, peripheral bone mass was not shown to improve significantly when contiguous standard scores were compared even when values were adjusted for bone age.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1988

Naproxen nephrotoxicity in a 2-year-old child.

The development of acute renal failure and interstitial nephritis due to therapeutic doses of nonsteroidal anti-inflammatory drugs has been documented repeatedly in adult patients but is rare in children. We report the occurrence of this complication in a child. Acute renal failure and hyperkalemia developed in a 2-year-old boy with juvenile rheumatoid arthritis after one month of naproxen sodium therapy. The evidence of renal toxic effects became manifest after an episode of dehydration. A percutaneous renal biopsy specimen revealed interstitial nephritis. The patient recovered promptly after withdrawal of the drug.

Acute Kidney Injury↗
PubMed1986

Analysis of selected plasma constituents in continuous ambulatory peritoneal dialysis effluent.

Patients maintained on continuous ambulatory peritoneal dialysis (CAPD) lose plasma constituents into the dialysis effluent. We have analyzed 24-hour CAPD effluents for selected components--total protein, a typical glycoprotein (alpha 1-acid glycoprotein), a typical lipoprotein (high density lipoprotein), and glycosaminoglycans. Our findings suggest that the plasma constituents found in CAPD effluent are similar to those found in urine from nephrotic patients. The loss of one or more of these plasma constituents into the dialysis solution may be linked to the hypertriglyceridemia observed in these patients.

Adolescent↗
PubMed1986

Antibiotic-associated interstitial nephritis and nephrotic syndrome.

The combination of interstitial nephritis and minimal-change nephrotic syndrome has been well described in adults receiving nonsteroidal anti-inflammatory agents. The association of interstitial nephritis and minimal-change nephrotic syndrome has recently been described in 1 adult patient receiving ampicillin and in 1 patient receiving rifampin. We describe an 8-month-old child who developed reversible interstitial nephritis and minimal-change nephrotic syndrome while taking antibiotics.

Amoxicillin↗
PubMed1984

How to cheat in morphology: the renal ultrafilter.

It is easy to distort the "truth" in morphological reports in order to confirm a previously established dogma by selecting from a field of view only such details which are in accordance with the dogma and by ignoring parts contradictory to it. The renal corpuscle is used in this paper as an example and as a guide to the readers for future fraud.

Animals↗
PubMed1983

Disparate effects of vitamin D treatment upon mitochondrial granulation in proximal and distal renal tubule.

The distribution of calcium phosphate granules in mitochondria of proximal and distal renal tubules of nonuremic and uremic children was analyzed by electron microscopy of material obtained by percutaneous kidney biopsy. Although distal tubule had fewer granules/mitochondrion than proximal tubule, uremia induced a significant drop (50%) in both, related to an increase in mitochondria containing O granules and a decrease in mitochondria with 2+ granules. The decrease observed in uremic children was reproduced experimentally by partial nephrectomy in rats. Uremia resulted in a 58% decrease of calcium phosphate granules in rat proximal tubule while a smaller but significant decrease (36%) occurred in distal tubule. Vitamin D deficiency in rats was associated with greatly decreased granulation in proximal tubule (80%) whereas distal tubule was less severely affected (36%). Supplementation of vitamin D to uremic rats restored mitochondrial granulation to normal in proximal tubule in 24 hr, but had no effect in distal tubule since the number of granules/mitochondrion, 0.5 +/- 0.1, remained statistically similar to that of untreated animals. Granulation in both proximal and distal tubule of uremic rats was unaffected by parathyroid hormone administration. Since restoration of granulation occurred only in proximal tubule, the defect in uremia which can be overcome by vitamin D treatment appears localized at the level of the proximal tubular cell membrane, indicating an action of vitamin D on calcium and/or phosphorus translocation into the proximal tubule.

Adolescent↗
PubMed1982

Glomerular basement membrane attenuation in familial nephritis and "benign" hematuria.

Lamination of the basement membrane has been considered to be the lesion characteristic of familial nephritis and attenuation to be the lesion of "Benign" familial hematuria. Electron micrographs were reviewed of 57 children who had renal biopsies for persistent hematuria. Attenuation or lamination of the glomerular capillary basement membrane was found in each. Twenty of the 57 children had familial nephritis; 20 had familial hematuria; and 17 had no involved relatives. Follow-up data were available for 14 of 20 children with familial nephritis, 12 of 20 with familial hematuria, and 12 of 17 with sporadic hematuria for 13.6 +/- 6.3, 6.7 +/- 4.6, and 7.0 +/- 4.8 years, respectively, after discovery of hematuria. Five children developed end-stage renal disease: three with familial nephritis, one with familial hematuria, and one with sporadic hematuria. Only two no longer had hematuria. Attenuation of the glomerular capillary basement membrane was seen in every biopsy, whereas lamination was not. Because hematuria and ultrastructural abnormalities were findings shared by all the children, we suggest the possibility that familial nephritis, and familial or sporadic hematuria as defined in this study, may be variations in a spectrum of inherited abnormality or abnormalities in the formation of the glomerular capillary basement membrane.

Adolescent↗
PubMed1980

Treatment of end-stage renal disease in children: a 15-year experience.

From 1964-1979, 154 children 1 to 16 years of age with end-stage renal disease (ESRD) were treated in a regional pediatric dialysis and transplant program. The incidence of ESRD was 1.6 per million total population per year. The survival rate of children undergoing dialysis for an average of 10 months was 93%. After living donor kidney transplantation (LD), patients survival rates in 60 children were 89% at 5 years, 83% at 10 years, and 74% at 15 years. After cadaver donor kidney transplantation (CD), patient survival rates in 85 children were 70% at 5 and 10 years. LD kidney survival was 71% at 5 years, 55% at 10 years, and 40% at 15 years, whereas CD kidney survival was 43% at 5 years and 31% at 10 years. The survival of first and second transplants was similar. Patient and kidney survival have improved since 1972. The survival rate of 26 children 1 to 5 years of age was 46%, but patients with Wilms' tumor accounted for most of the deaths. We attribute these favorable long-term results in children to an integrated program of dialysis and transplantation with special pediatric facilities.

Adolescent↗
PubMed1979

Mitochondrial granulation in the proximal renal tubule in uremia.

Mitochondria contain electron-dense particles, partly composed of an amorphous form of calcium phosphate. We have used electron microscopy from percutaneous renal biopsy material to analyze mitochondrial granulation in the proximal renal tubule of nonuremic and uremic children. Based on a technique of cutting mitochondria from ten electron micrographs per biopsy, counting the granules in each mitochondrion and weighing the paper, we found that mitochondria of nonuremic children averaged 23.7 +/- 1.2 granules/g paper while uremic children had only 11.8 +/- 1.1 granules/g. The number of granules per gram was unrelated to the serum calcium phosphate solubility product. A significant decrease in calcium granulation in uremia can also be produced experimentally in rats. Control rats averaged 14.7 +/- 1.5 granules/g, while rats made uremic by partial nephrectomy had 6.0 +/- 0.7 granules/g. Treatment of uremic rats with a pharmacological dose of vitamin D restored granulation to normal within 24 h. The significant decrease in calcium phosphate granulation in the renal proximal tubule in uremic children and in experimental animals is probably related to the documented loss of 1 alpha-hydroxylation of vitamin D in uremia.

Adolescent↗