Scholars@Duke
John William Foreman

John William Foreman

Professor Emeritus of Pediatrics
Pediatrics, Nephrology
forem001@mc.duke.edu
2301 Erwin Rd, Durham, NC 27710
2301 Erwin Rd, Durham, NC 27710

Selected Publications

Fanconi Syndrome and Other Proximal Tubule Disorders

Chapter · January 1, 2023 Fanconi syndrome is a global dysfunction of the proximal tubule characterized by glycosuria, phosphaturia, generalized aminoaciduria, and type 2 renal tubular acidosis. Often there is hypokalemia, sodium wasting, hypouricemia, and dehydration. In children ... Full text Cite

Tailored use of belatacept in adolescent kidney transplantation.

Journal Article Am J Transplant · March 2020 Adolescent transplant recipients are at risk for nonadherence, development of de novo donor-specific antibody (dnDSA), and allograft loss. Belatacept, a selective T cell costimulatory blocker, is associated with reduced dnDSA, improved renal function, and ... Full text Link to item Cite

Correction to: Recurrence of nephrotic syndrome following kidney transplantation is associated with initial native kidney biopsy findings : A Midwest Pediatric Nephrology Consortium (MWPNC) study.

Journal Article Pediatr Nephrol · March 2019 The original version of this article unfortunately contained a mistake. The subtitle "A Midwest Pediatric Nephrology Consortium (MWPNC) study" was missing. The correct title including subtitle is given above. ... Full text Link to item Cite

Fanconi Syndrome.

Journal Article Pediatr Clin North Am · February 2019 Fanconi syndrome, also known as the DeToni, Debré, Fanconi syndrome is a global dysfunction of the proximal tubule characterized by glucosuria, phosphaturia, generalized aminoaciduria, and type II renal tubular acidosis. Often there is hypokalemia, sodium ... Full text Link to item Cite

Essentials of Nephrology

Chapter · January 1, 2019 The basic functions of the kidney are to maintain fluid and electrolyte homeostasis and metabolism. Renal disease requires the practitioner to be vigilant about fluid homeostasis, acid-base balance, electrolyte management, choice of anesthetics, and potent ... Full text Cite

Psychosocial Assessment of Candidates for Transplantation (PACT) Score Identifies High Risk Patients in Pediatric Renal Transplantation.

Journal Article Front Pediatr · 2019 Background: Currently, there is no standardized approach for determining psychosocial readiness in pediatric transplantation. We examined the utility of the Psychosocial Assessment of Candidates for Transplantation (PACT) to identify pediatric kidney trans ... Full text Open Access Link to item Cite

Recurrence of nephrotic syndrome following kidney transplantation is associated with initial native kidney biopsy findings.

Journal Article Pediatr Nephrol · October 2018 BACKGROUND AND OBJECTIVES: Steroid-resistant nephrotic syndrome (SRNS) due to focal segmental glomerulosclerosis (FSGS) and minimal change disease (MCD) is a leading cause of end-stage kidney disease in children. Recurrence of primary disease following tra ... Full text Link to item Cite

Essentials of Nephrology

Chapter · January 1, 2018 The basic functions of the kidney are to maintain fluid and electrolyte homeostasis and metabolism. Renal disease requires the practitioner to be vigilant about fluid homeostasis, acid-base balance, electrolyte management, choice of anesthetics, and potent ... Full text Cite

Neutropenic enterocolitis (typhlitis) in a pediatric renal transplant patient. A case report and review of the literature.

Journal Article Pediatr Transplant · September 2017 NE (typhlitis) is a potentially life-threatening disease process characterized by bowel wall edema, ulceration, and hemorrhage in an immunosuppressed patient. We report a 15-year-old boy status post deceased donor renal transplantation who presented with f ... Full text Link to item Cite

Renal tubular acidosis

Chapter · November 25, 2016 Full text Cite

Rare variants in tenascin genes in a cohort of children with primary vesicoureteric reflux.

Journal Article Pediatr Nephrol · February 2016 BACKGROUND: Primary vesicoureteral reflux (PVUR) is the most common malformation of the kidney and urinary tract, and reflux nephropathy is a major cause of chronic kidney disease in children. Recently, we reported mutations in the tenascin XB gene (TNXB) ... Full text Link to item Cite

Genetic Diseases of the Kidney

Journal Article The Open Journal of urology and Nephrology · November 11, 2015 Full text Cite

Variability in phenotype induced by the podocin variant R229Q plus a single pathogenic mutation.

Journal Article Clin Kidney J · October 2015 BACKGROUND: Mutations in podocin (NPHS2) are the most common cause of childhood onset autosomal recessive steroid-resistant nephrotic syndrome (SRNS). The disease is characterized by early-onset proteinuria, resistance to immunosuppressive therapy and rapi ... Full text Open Access Link to item Cite

HLA-DQA1 and PLCG2 Are Candidate Risk Loci for Childhood-Onset Steroid-Sensitive Nephrotic Syndrome.

Journal Article J Am Soc Nephrol · July 2015 Steroid-sensitive nephrotic syndrome (SSNS) accounts for >80% of cases of nephrotic syndrome in childhood. However, the etiology and pathogenesis of SSNS remain obscure. Hypothesizing that coding variation may underlie SSNS risk, we conducted an exome arra ... Full text Link to item Cite

Efficacy of antibiotic prophylaxis in children with vesicoureteral reflux: systematic review and meta-analysis.

Journal Article J Urol · March 2015 PURPOSE: Controversy exists regarding the use of continuous antibiotic prophylaxis vs observation in the management of children with vesicoureteral reflux. The reported effectiveness of continuous antibiotic prophylaxis in children with reflux varies widel ... Full text Link to item Cite

Effects of low-dose dopamine on urine output in normotensive very low birth weight neonates.

Journal Article J Perinatol · August 2013 OBJECTIVE: To determine the effects of low-dose dopamine on urine output (UOP) in very low birth weight premature neonates. STUDY DESIGN: Retrospective cohort study of all low-dose (3-5 μg kg(-1) per min) dopamine infusions >24-h duration in neonates 1500 ... Full text Link to item Cite

TNXB mutations as a cause of vesicoureteral reflux (VUR)

Journal Article PEDIATRIC NEPHROLOGY · August 1, 2013 Link to item Cite

TNXB mutations can cause vesicoureteral reflux.

Journal Article J Am Soc Nephrol · July 2013 Primary vesicoureteral reflux (VUR) is the most common congenital anomaly of the kidney and the urinary tract, and it is a major risk factor for pyelonephritic scarring and CKD in children. Although twin studies support the heritability of VUR, specific ge ... Full text Link to item Cite

Hypercalcemia, hypercalciuria, and elevated calcitriol concentrations with autosomal dominant transmission due to CYP24A1 mutations: effects of ketoconazole therapy.

Journal Article J Clin Endocrinol Metab · March 2012 BACKGROUND: Mutations of the CYP24A1 gene, which encodes the 1,25-dihydroxyvitamin D-24-hydroxylase cytochrome P450, Cyp24A1, are predicted to result in elevated 1,25-dihydroxyvitamin D concentrations, hypercalcemia, hypercalciuria, nephrolithiasis, and bo ... Full text Link to item Cite

Mutations in kelch-like 3 and cullin 3 cause hypertension and electrolyte abnormalities.

Journal Article Nature · January 22, 2012 Hypertension affects one billion people and is a principal reversible risk factor for cardiovascular disease. Pseudohypoaldosteronism type II (PHAII), a rare Mendelian syndrome featuring hypertension, hyperkalaemia and metabolic acidosis, has revealed prev ... Full text Link to item Cite

Pathogenesis and therapy of focal segmental glomerulosclerosis: an update.

Journal Article Pediatr Nephrol · July 2011 Focal and segmental glomerulosclerosis (FSGS) is an important cause of steroid-resistant nephrotic syndrome in adults and children. It is responsible for 5-20% of all cases of end-stage kidney disease (ESKD) in the United States. The pathogenesis of FSGS h ... Full text Link to item Cite

Essentials of Nephrology

Journal Article · December 1, 2009 Full text Cite

Essentials of nephrology

Chapter · December 1, 2008 Full text Cite

Drug-induced granulomatous interstitial nephritis in a pediatric patient.

Journal Article Pediatr Nephrol · February 2007 Featured Publication Acute interstitial nephritis (AIN) is a known cause of acute renal failure in children. In most instances, drug therapy is the offending agent. Although granuloma formation has been observed in drug-induced interstitial nephritis, it is not a commonly asso ... Full text Link to item Cite

Mutations in INVS encoding inversin cause nephronophthisis type 2, linking renal cystic disease to the function of primary cilia and left-right axis determination.

Journal Article Nat Genet · August 2003 Featured Publication Nephronophthisis (NPHP), an autosomal recessive cystic kidney disease, leads to chronic renal failure in children. The genes mutated in NPHP1 and NPHP4 have been identified, and a gene locus associated with infantile nephronophthisis (NPHP2) was mapped. Th ... Full text Link to item Cite

Basic fibroblast growth factor among children with diarrhea-associated hemolytic uremic syndrome.

Journal Article J Am Soc Nephrol · March 2002 Diarrhea-associated hemolytic uremic syndrome (D+HUS) is characterized by endothelial injury and activation of inflammatory cytokines. Basic fibroblast growth factor (bFGF) is an angiogenic peptide released in response to vascular damage. The plasma concen ... Full text Link to item Cite

The United States National Prospective Hemolytic Uremic Syndrome Study: microbiologic, serologic, clinical, and epidemiologic findings.

Journal Article The Journal of infectious diseases · April 2001 The frequency of Shiga toxin-producing Escherichia coli (STEC) serotypes associated with postdiarrheal hemolytic uremic syndrome (HUS) cases among children and adults in the United States and the proportion with IgM or IgG lipopolysaccharide antibodies to ... Full text Cite

Prevalence and clinical correlates of glomerulopathy in children with sickle cell disease.

Journal Article J Pediatr · June 2000 Featured Publication OBJECTIVES: Glomerular disease and renal failure cause substantial morbidity for patients with sickle cell disease (SCD). Proteinuria is an early manifestation of sickle nephropathy, but the prevalence of proteinuria and its clinical correlations in childr ... Link to item Cite

Thrombocytopenia and absent radii (TAR) syndrome associated with horseshoe kidney.

Journal Article Pediatr Nephrol · January 2000 The TAR syndrome is an inherited disorder characterized by limb abnormalities, especially absent radii, and hypomegakaryocytic thrombocytopenia. Previous reports have included two infants with genitourinary abnormalities. We report a newborn with bilateral ... Full text Link to item Cite

Appearance of immune complex glomerulonephritis following the onset of type I diabetes mellitus in a child.

Journal Article Am J Kidney Dis · November 1997 Featured Publication Renal disease is a frequent late complication of type I diabetes mellitus, occurring almost entirely in adult patients. Typical diabetic nephropathy is characterized by proteinuria, and by the histological lesions of mesangial expansion and basement membra ... Full text Link to item Cite

Perspective of a pediatric nephrology program: an 18 year retrospective.

Journal Article Zhonghua Min Guo Xiao Er Ke Yi Xue Hui Za Zhi · 1997 We analyzed the patient profile in a pediatric nephrology training program, along with data collected over an 18 year period, to determine whether there is merit in the proposition that clinical training can be obtained equally well in internal medicine ne ... Link to item Cite

Nutritional intake in children with renal insufficiency: a report of the growth failure in children with renal diseases study.

Journal Article J Am Coll Nutr · December 1996 OBJECTIVE: This study was designed to assess sequentially the nutrient intake in children with chronic renal insufficiency and its relationship to body size, the level of renal failure, and growth velocity. METHODS: The nutrient intake from 401 4-day food ... Full text Link to item Cite

Metabolic studies of rat renal tubule cells loaded with cystine: the cystine dimethylester model of cystinosis.

Journal Article J Am Soc Nephrol · August 1995 The cause of Fanconi syndrome in cystinosis is enigmatic. It has previously been shown that renal tubules could be loaded with cystine by incubating them with cystine dimethylester (CDE), mimicking the biochemical hallmark of cystinosis. Such tubules have ... Full text Link to item Cite

Insulin-like growth factor-I gene expression in the tibial epiphyseal growth plate of growth hormone-treated uremic rats.

Journal Article Kidney international · May 1995 To identify the molecular mechanisms involved in long bone growth of uremic animals, we evaluated the effects of recombinant human growth hormone (rhGH) supplementation on whole body growth, growth plate morphometrics, and insulin-like growth factor-I (IGF ... Full text Cite

Renal toxicity of ifosfamide in pilot regimens of the intergroup rhabdomyosarcoma study for patients with gross residual tumor.

Journal Article Am J Pediatr Hematol Oncol · November 1994 PURPOSE: The purpose of this review is to characterize the nephrotoxicity noted in newly diagnosed patients under 21 years of age after treatment with ifosfamide-containing chemotherapy regimens and local irradiation for localized gross residual rhabdomyos ... Link to item Cite

PREDICTORS OF GLOMERULAR-DISEASE IN CHILDREN AND ADULTS WITH SICKLE-CELL-ANEMIA

Journal Article JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY · September 1, 1994 Link to item Cite

The peritoneal equilibration test in children.

Journal Article Pediatr Nephrol · December 1993 Eight children, aged 15 months to 17 years 9 months, maintained by continuous ambulatory peritoneal dialysis (CAPD)/continuous cycling peritoneal dialysis and nine adults, aged 20-59 years, managed by CAPD were compared using a standardized peritoneal dial ... Full text Link to item Cite

ESRD: pay now or pay later.

Journal Article Va Med Q · 1993 Link to item Cite

Hypercholesterolemia in rats with chronic renal insufficiency not aggravated by recombinant human growth hormone.

Journal Article Child Nephrol Urol · 1992 The lipid metabolic disorders in chronic renal insufficiency (CRI) are related to increased hepatic lipid synthesis, reduced triglyceride removal coupled with insulin insensitivity and impaired lipoprotein lipase activity. Growth hormone is lipolytic, and ... Link to item Cite

Inhibition of calcium oxalate crystal growth in vitro by uropontin: another member of the aspartic acid-rich protein superfamily.

Journal Article Proc Natl Acad Sci U S A · January 1, 1992 The majority of human urinary stones are primarily composed of calcium salts. Although normal urine is frequently supersaturated with respect to calcium oxalate, most humans do not form stones. Inhibitors are among the multiple factors that may influence t ... Full text Link to item Cite

Growth in chronic renal disease

Journal Article Current Opinion in Pediatrics · January 1, 1992 Full text Cite

Hypercalciuria due to combined growth hormone and calcitriol therapy in uremia: effects of growth hormone on mineral homeostasis in 75% nephrectomized weanling rats.

Journal Article Pediatr Res · December 1991 The administration of growth hormone (GH) in conjunction with calcitriol in uremia may increase urinary calcium and decrease renal phosphate excretion, which could have an adverse effect on the kidney in chronic renal insufficiency. The effect of 40 d of o ... Full text Link to item Cite

Chronic renal insufficiency in infants and children.

Journal Article Clin Pediatr (Phila) · June 1991 Full text Link to item Cite

Diagnostic workup of renal disorders.

Journal Article Current problems in pediatrics · February 1991 Full text Cite

Renal transplantation in children.

Journal Article Int J Artif Organs · January 1991 Link to item Cite

Cystinosis

Journal Article Kidney · December 1, 1990 Cite

Effect of cystine loading and cystine dimethylester on renal brushborder membrane transport.

Journal Article Biosci Rep · October 1990 The effect of loading renal tubule cells with cystine was studied by incubating them with cystine dimethylester. Proline uptake into brushborder membrane vesicles isolated from the cystine loaded cells was not different from that observed into brushborder ... Full text Link to item Cite

Effect of cystine loading on substrate oxidation by rat renal tubules.

Journal Article Pediatr Nephrol · May 1990 Isolated rat renal tubules were loaded with cystine by incubating them with 2 mM cystine dimethylester. The oxidation of 1 mM glucose and lactate was significantly decreased after 20 and 30 min of incubation, in the cystine-loaded tubules compared with con ... Full text Link to item Cite

10-year survey of referrals to a pediatric nephrology program.

Journal Article Child Nephrol Urol · 1990 To provide a realistic picture of the patient case load of a pediatric nephrologist in a teaching hospital, we analyzed the number, the demographics, and the reasons for patient referral to our pediatric nephrology program over a 10-year period. Between Ja ... Link to item Cite

Familial hyperuricemia and renal insufficiency.

Journal Article Child Nephrol Urol · 1990 A kindred is described in which hyperuricemia and renal insufficiency were observed in three generations. The hyperuricemia appeared to precede the renal diseases. Lowering the serum uric acid level to normal did not decrease the progression of renal insuf ... Link to item Cite

Hypophosphatemia in a pediatric patient after major hepatic resection.

Journal Article J Pediatr Surg · November 1989 Hypophosphatemia was noted in a 20-month-old infant following major hepatic resection. The hypophosphatemia appeared to be related in part to renal wasting of phosphate, although this condition was transient. Prompt recognition and treatment prevented seri ... Full text Link to item Cite

The effects of exogenous rat growth hormone therapy on growth of uremic rats fed an 8% protein diet.

Journal Article Pediatric research · September 1989 Although the mechanisms underlying the inhibitory effects of chronic renal insufficiency on growth are poorly understood, large doses of growth hormone (GH) have been used to improve growth. The present study examines the effects of rat GH and a reduced (8 ... Full text Cite

Cystinosis.

Journal Article Semin Nephrol · March 1989 Link to item Cite

Human renal Fanconi syndrome--then and now.

Journal Article Nephron · January 1989 Full text Cite

Lithium-induced nephrotic syndrome.

Journal Article Am J Psychiatry · January 1989 The nephrotic syndrome is a rare, idiosyncratic adverse renal effect of lithium that can occur with therapeutic plasma lithium levels. The syndrome is usually reversed by discontinuation of lithium treatment but may require corticosteroids. Renal biopsies ... Full text Link to item Cite

Chronic renal failure in infants and children.

Journal Article J Pediatr · November 1988 Chronic renal failure is an uncommon problem for pediatricians, but early recognition is important for maximizing growth and minimizing complications. Marked strides have been made in understanding and treating renal osteodystrophy. Recombinant erythropoie ... Full text Link to item Cite

Characteristics of cystine uptake by cultured LLC-PK1 cells.

Journal Article Biochim Biophys Acta · March 11, 1988 The characteristics of the uptake of L-cystine by LLC-PK1 cells were examined. The uptake diminished with time in culture after passage of cells while the uptake of sugar increased. In 48-h-cultured cells at a range of cystine concentrations including phys ... Full text Link to item Cite

Effect of cystine dimethylester on renal solute handling and isolated renal tubule transport in the rat: a new model of the Fanconi syndrome.

Journal Article Metabolism: clinical and experimental · December 1987 The effect of cystine dimethylester on the renal handling of phosphate, glucose, alpha-amino nitrogen, amino acids, and protein in vivo and on the uptake of lysine, glycine, taurine, and alpha-methyl glucoside by isolated renal tubules in vitro was studied ... Full text Cite

Cysteine and glutathione levels in developing rat kidney and liver.

Journal Article Pediatr Res · November 1987 Intracellular levels of cysteine (CSH) and reduced glutathione (GSH) in the kidney and liver of rats from the newborn to the adult period have been determined using a sensitive high performance liquid chromatography method. In the kidney, the intracellular ... Full text Link to item Cite

Characteristics of lysine transport by isolated rat renal cortical tubule fragments.

Journal Article Biochimica et biophysica acta · July 1987 The uptake of L-lysine was examined in isolated renal cortical tubules. Lysine was actively taken up by the renal tubule cells isolated from 7-week-old rats. No metabolism of the transported lysine was found. There was no evidence for sodium-dependence of ... Full text Cite

Cystine and lysine transport in cultured human renal epithelial cells.

Journal Article Metabolism: clinical and experimental · April 1987 The transport of the amino acids, cystine and lysine, was studied in epithelial cell lines propagated from human kidney cortex. Cystine uptake data were reproducible in different cell lines and did not vary over several cell passages of an individual cell ... Full text Cite

Characteristics of lysine uptake by isolated renal cortical tubule fragments from mature and immature dogs.

Journal Article Biochimica et biophysica acta · November 1986 The uptake of L-lysine was examined in isolated renal cortical tubule fragments from adult and 1-week-old dogs. Lysine uptake by adult tubules was initially more rapid than that by the immature tubules. This uptake by mature tubules reached a steady state ... Full text Cite

Characteristics of cultured human renal cortical epithelia.

Journal Article Biochemical medicine and metabolic biology · October 1986 Nine human kidney epithelial cell lines, isolated from small biopsied material and from whole kidney, were propagated in both a hormonally defined medium and a medium supplemented with serum. At confluency, hemicysts or domes, typical of cultured epithelia ... Full text Cite

Developmental aspects of cystine transport in the dog.

Journal Article Pediatr Res · July 1986 Developmental changes in cystine transport by the canine kidney were examined both in vivo and in vitro. Renal clearance studies indicated that cystine was one of the more incompletely reabsorbed amino acids at birth, but its reabsorption approaches adult ... Full text Link to item Cite

Cystine-glutamate transport interactions in rat renal cortical tubules, brushborder vesicles, and cultured renal tubule cells.

Journal Article Biosci Rep · January 1986 Glutamate had no significant effect on the uptake of 0.025 mM cystine by isolated rat renal cortical tubules and brushborder membrane vesicles in contrast to lysine which significantly inhibits cystine transport. Glutamate, however, markedly inhibited cyst ... Full text Link to item Cite

Uptake of proline by brushborder vesicles isolated from human kidney cortex.

Journal Article Biochemical medicine · December 1985 Proline transport into renal brushborder membrane vesicles isolated from human kidney is mediated by two uptake systems. The high-affinity system is stimulated by a Na gradient and appears to be shared with glycine while the low-affinity system is not. Upt ... Full text Cite

Nonsurgical management of obstructive aortic thrombosis complicated by renovascular hypertension in the neonate.

Journal Article The Journal of pediatrics · April 1985 The use of umbilical artery catheters has become commonplace in the intensive care nursery and has facilitated management in the very ill newborn infant. Thrombotic complications associated with the use of such catheters may be as high as 30%. The successf ... Full text Cite

Developmental aspects of sugar transport by isolated dog renal cortical tubules.

Journal Article Pediatr Res · August 1984 alpha-Methyl-D-glucoside (AMG) uptake was examined in isolated renal cortical tubules from newborn, 3-month-old, and adult dogs. All three age groups demonstrated active sugar transport. The initial rate of AMG uptake was similar in the 3-month-old and adu ... Full text Link to item Cite

Absence of a role of gamma-glutamyl transpeptidase in the transport of amino acids by rat renal brushborder membrane vesicles.

Journal Article J Membr Biol · 1984 The role of the enzyme, gamma-glutamyl transpeptidase on the uptake of amino acids by the brushborder membrane of the rat proximal tubule was examined by inhibiting it with AT-125 (L-[alpha S, 5S]-alpha-amino-3-chloro-4,5-dihydro-5-isoxazoleacetic acid). A ... Full text Link to item Cite

Effect of acidosis on glutamine transport by isolated rat renal brush-border and basolateral-membrane vesicles.

Journal Article Biochem J · June 15, 1983 Glutamine uptake was examined in isolated renal brush-border and basolateral-membrane vesicles from control and acidotic rats. In brush-border vesicles from acidotic animals, there was a significant increase in the initial rate of glutamine uptake compared ... Full text Link to item Cite

Developmental changes of glycine transport in the dog.

Journal Article Biochimica et biophysica acta · December 1982 The renal clearance of amino acids was measured in canine pups between 5 days and 12 weeks of age. The reabsorption of glycine was incomplete at 5 and 21 days, indicating a physiologic aminoaciduria of immaturity. An adult pattern of 97-100% reabsorption a ... Full text Cite

Preservation of brush border transport systems for proline and alpha-methyl-D glucoside from rat, dog, and human kidney.

Journal Article Lab Invest · December 1982 The effects of freezing renal tissue from rat, dog, and man on the time course of uptake of proline and alpha-methyl-D-glucoside by subsequently isolated brush border membrane vesicles was examined and compared with uptake patterns by membranes isolated fr ... Link to item Cite

Developmental pattern of cystine transport in isolated rat renal tubules.

Journal Article Biochimica et biophysica acta · August 1982 Isolated renal cortical tubule fragments from rats ranging in age from less than 48 h to 15 weeks were used to examine the pattern of cystine uptake with development. Immature tubules took up cystine with a faster initial rate than mature tubules and did n ... Full text Cite

Homocystine uptake in isolated rat renal cortical tubules.

Journal Article Metabolism · June 1982 Isolated rat renal cortical tubules were used to study the nature of homocystine entry into the tubule cell and its transport interactions with cystine and the dibasic amino acids. The uptake of homocystine with time was progressive, reaching a steady stat ... Full text Link to item Cite

Methods in laboratory investigation. Preservation of brush border transport systems for proline and α-methyl-D-glucoside from rat, dog, and human kidney

Journal Article Laboratory Investigation · January 1, 1982 The effects of freezing renal tissue from rat, dog, and man on the time course of uptake of proline and α-methyl-D-glucoside by subsequently isolated brush border membrane vesicles was examined and compared with uptake patterns by membranes isolated from t ... Cite

Amino acid uptake by isolated renal brush border membrane vesicles in various buffers.

Journal Article Biochim Biophys Acta · August 6, 1981 The uptake of amino acids by isolated rat renal brush border membrane vesicles in a modified Krebs-Ringer bicarbonate buffer and a phosphate buffer was compared to the uptake in the standard membrane vesicle buffer, Tris-Hepes-mannitol. The uptake in the m ... Full text Link to item Cite

Cysteamine therapy in nephropathic cystinosis.

Journal Article N Engl J Med · May 7, 1981 Full text Link to item Cite

Serum and urinary biotin levels during treatment of holocarboxylase synthetase deficiency.

Journal Article Clinica chimica acta; international journal of clinical chemistry · February 1981 Measurements of blood and urine biotin levels have been performed during treatment of a patient with holocarboxylase synthetase deficiency. During the first 24 hours of therapy, the infant progressed from a moribund, shock-like state to a clinically normal ... Full text Cite

Effects of cysteamine therapy in nephropathic cystinosis.

Journal Article The New England journal of medicine · January 1981 We studied the effects of cysteamine in five children with nephropathic cystinosis who were treated for up to 30 months. Cysteamine caused a decline in leukocyte cystine levels to the range seen in clinically unaffected heterozygotes; both plasma and urina ... Full text Cite

Holocarboxylase synthetase deficiency: a biotin-responsive organic acidemia.

Journal Article The Journal of pediatrics · May 1980 The clinical and biochemical features of an infant affected by holocarboxylase synthetase deficiency are presented. The patient was the sibling of the deceased child in whose cultured skin fibroblasts the precise enzymatic disorder was first determined. Th ... Full text Cite

Transport interactions of cystine and dibasic amino acids in isolated rat renal tubules.

Journal Article Metabolism: clinical and experimental · January 1980 Isolated renal cortical tubules prepared from adult male Sprague-Dawley rats were used to study the nature of cystine entry into tubule cells and its transport interactions with dibastic amino acids. The uptake of cystine over time was progressive, reachin ... Full text Cite

Effects of arginine infusion in homocystinuria

Journal Article Pediatric Research · January 1, 1980 Cite

Nephropathic cystinosis: Management with cysteamine

Journal Article Pediatric Research · January 1, 1980 Cite

Hypoxanthine uptake in isolated rat renal cortical tubule fragments.

Journal Article The Journal of clinical investigation · April 1979 Isolated renal tubule fragments prepared from adult Sprague-Dawley rats were used to study the cellular uptake of hypoxanthine. This uptake was rapid, reaching a steady state after 30 min of incubation. Analysis of the intracellular pool during the initial ... Full text Cite

Cystine transport in isolated rat renal tubules

Journal Article Kidney International · January 1, 1978 Cite