... Wyatt RJ, Rivas ML, Julian BA, Quiggins PA, Woodford SY, McMorrow RG, Baehler RW (1987) Regio... more ... Wyatt RJ, Rivas ML, Julian BA, Quiggins PA, Woodford SY, McMorrow RG, Baehler RW (1987) Regionalization in hered-itary IgA nephropathy. ... Kobayashi Y, Rujii K, Hiki Y, Tateno S, Kurokawa M (1988) Steroid therapy in IgA nephropathy: a retrospective study in heavy ...
1 Department of Pediatrics, Baylor College of Medicine, Houston, Texas, USA 2 Department of Pedia... more 1 Department of Pediatrics, Baylor College of Medicine, Houston, Texas, USA 2 Department of Pediatrics, Stanford University Medical Center, Stanford, California, USA 3 Diagnostic Systems Laboratories, Webster, Texas, USA ... Received: April 17, 1995; received in revised form ...
Acid-base disorders are common in sick children. This article is a practical guide to the differe... more Acid-base disorders are common in sick children. This article is a practical guide to the differential diagnosis and treatment of simple and mixed acid-base disorders of children. Special attention is given to fundamentals of acid-base physiology, to clinical use of the Henderson equation, and to interpretation of readily available laboratory tests.
The waiting list for kidney transplantation continued to grow between 1999 and 2008, from 41 177 ... more The waiting list for kidney transplantation continued to grow between 1999 and 2008, from 41 177 to 76 089 candidates. However, active candidates represented the minority of this increase (36 951-50 624, a 37% change), while inactive candidates increased over 500% (4226-25 465). There were 5966 living donor (LD) and 10 551 deceased donor (DD) kidney transplants performed in 2008. The total number of pancreas transplants peaked at 1484 in 2004 and has declined to 1273. Although the number of LD transplants increased by 26% from 1999 to 2008, the total number peaked in 2004 at 6647 before declining 10% by 2008. The rate of LD transplantation continues to vary significantly as a function of demographic and geographic factors, including waiting time for DD transplant. Posttransplant survival remains excellent, and there appears to be greater use of induction agents and reduced use of corticosteroids in LD recipients. Significant changes occurred in the pediatric population, with a dramatic reduction in the use of LD organs after passage of the Share 35 rule. Many strategies have been adopted to reverse the decline in LD transplant rates for all age groups, including expansion of kidney paired donation, adoption of laparoscopic donor nephrectomy and use of incompatible LD.
Evaluation of hypertension in the pediatric age group should be guided by the age at presentation... more Evaluation of hypertension in the pediatric age group should be guided by the age at presentation, the severity of hypertension at presentation, and whether the hypertension is sustained or transient. The common causes of hypertension differ by age group (Table 1). Hypertension is more likely to be secondary and sustained in the younger age groups, whereas primary (essential) hypertension and white coat hypertension are more likely to be the etiology in adolescents [1–5]. The global epidemic of childhood obesity has been associated with an increased incidence of primary hypertension and prehypertension in adolescents worldwide [3–7]. Obese children have as much as a threefold higher risk for developing hypertension compared to nonobese children [7]. The frequent occurrence of primary hypertension in obese adolescents dictates an entirely different evaluation than for younger or nonobese children. Renal disease must be considered in every child with hypertension, because of the prevalence of renovascular and renal parenchymal disorders as the etiology in any age group. Secondary hypertension from renal disorders is more likely to be severe at presentation than primary hypertension. Endocrine causes of hypertension are rare, so special diagnostic studies for these disorders should be reserved for those patients whose history, physical examination, and preliminary evaluation warrant further specific investigation. This chapter outlines the steps in evaluation of childhood hypertension, starting with the confirmation of the diagnosis and evaluation of its duration and severity.
OBJECTIVE: Growth failure is frequently observed in children with chronic kidney disease and thos... more OBJECTIVE: Growth failure is frequently observed in children with chronic kidney disease and those with kidney transplantation (KTx). Pubertal development and growth have a major effect on final height. There are few reports about puberty in children after KTx. We evaluated the effects of KTx on pubertal development and growth in pre-pubertal children. PATIENTS AND METHODS: From 1990 through 2009, KTx was performed in 274 children at our center. We analyzed data from those children who underwent KTx before puberty and retrospectively studied growth, signs of puberty, estimated glomerular filtration rate, and immunosuppressive treatment. RESULTS: In children transplanted before puberty, 65 children (41 boys; median age at KTx, 7.9 years; pre-emptive KTx, 9) developed secondary sexual characteristics. The mean age at the onset of puberty [healthy Japanese children] was 11.
American Journal of Physiology-renal Physiology, Sep 1, 1983
Micropuncture and clearance studies were performed to assess reabsorptive function in the proxima... more Micropuncture and clearance studies were performed to assess reabsorptive function in the proximal and distal nephron of rats with experimental Fanconi's syndrome induced by maleic acid. Anesthetized rats were studied by free-flow micropuncture of the late proximal tubule 90-120 min after continuous intravenous administration of maleic acid, 100 mg X kg-1 X h-1. Compared with control rats, the reabsorption of sodium and phosphate was significantly reduced (P less than 0.001 and less than 0.02, respectively). Tubular fluid-to-ultrafiltrate (TF/UF) chloride concentration ratio was 1.00 +/- 0.02 compared with 1.16 +/- 0.03 (P less than 0.01) in controls, suggesting a nearly total inhibition of proximal bicarbonate reabsorption. Whole kidney fractional excretions of sodium and chloride were increased significantly (P less than 0.02) but could not be explained by enhanced delivery of these solutes out of the late proximal tubule. To assess whether distal nephron reabsorption of sodium and chloride were inhibited by maleic acid, clearance studies were performed during water diuresis in awake rats. During maleic acid administration, 200 mg X kg-1 X h-1, urine flow rate (P less than 0.02) and the fractional excretions of sodium and chloride (P less than 0.001) increased significantly, but fractional free water clearance decreased from 7.16 +/- 0.42 to 4.03 +/- 0.68% (P less than 0.001). In acetazolamide-treated control rats but not in maleic acid-treated rats with similar bicarbonaturia, the magnitude of fractional free water clearance closely approximated the simultaneously measured fractional distal delivery of chloride. These studies suggest that maleic acid inhibits reabsorption at a distal nephron site or sites as well as in the proximal tubule.
IGF-binding protein-3 (IGFBP-3), usually found as glycosylated 41-and 38-kD forms, is the major s... more IGF-binding protein-3 (IGFBP-3), usually found as glycosylated 41-and 38-kD forms, is the major serum IGFBP during extrauterine life. In normal serum ICFBP-3 binds one IGF peptide and one acid-labile (a) subunit in a high-molecular-weight (MW) complex of 150 kD. By RIA, an excess of IGFBP-3 is present in chronic renal failure (CRF) serum, where it reportedly accumulates at low MW (25-55 kD) rather than as part of the 150-kD complex. To further evaluate IGFBP-3 forms in CRF, sera were obtained from seven healthy adolescents and seven adolescents with CRF. By RIA, IGFBP-3 levels were higher in CRF than normal sera (15.4 f 2.2 versus 10.1 f 2.1 wg/mL). High-MW (150-kD) fractions of CRF and normal sera, obtained by neutral size-exclusion chromatography, had equal amounts of IGFBP-3 by RIA. However, a second RIA peak of IGFBP-3, present in low-MW (35-kD) fractions of CRF but not normal sera, could account for the higher IGFBP-3 levels of CRF serum. ['251]IGF ligand blots of whole serum and serum fractions, either with or without prior precipitation by IGFBP-3 antiserum, found levels of 41-and 38-kD IGFBP-3 forms to be similar between CRF and normal whole sera and located these forms in the high-MW (150-kD) fractions of CRF and normal sera. [125111GF ligand blots also identified excess IGFBP in low-MW CRF serum fractions; cross-linking these IGFBP with [1251]IGF-I, followed by precipitation with IGFBP-I,-2, and-3 antibodies, identified high levels of unsaturated IGFBP-I, IGFBP-2, and 19-and 14-kD forms of IGFBP-3 in CRF serum. These studies indicate that I) normal levels of functional 41-and 38-kD IGFBP-3 forms are present in CRF serum, and these forms can be incorporated into the 150-kD serum complex; and 2) high RIA levels of IGFBP-3 in low-MW fractions of CRF serum are at least in part due to 19-and 14-kD IGFBP-3 forms. These studies suggest that the excess unsaturated IGFBP of CRF serum are small enough to enter interstitial tissue spaces where they may modulate IGF-I-mediated mitogenic, metabolic, and differentiative effects.
Results. A given patient cycles between fi ve Markov states: waitlisted, transplanted with a func... more Results. A given patient cycles between fi ve Markov states: waitlisted, transplanted with a functioning living donor or deceased donor graft, post-second graft failure, or deceased (Figure 1). The model accounts for the increased risk of graft failure seen during late adolescence and early adulthood, aging or disease development in the living donor over time, sensitization risks, and differential deceased donor waiting times based upon age-based allocation policies. Given a set of candidate and living donor characteristics, the model provides the expected patient survival over a time horizon of 20 years (Figure 2). Factors most predictive of a survival difference between living-donor-fi rst and deceased-donor-fi rst were level of sensitization and living donor HLA mismatch. Conclusion. The Markov model provides patients, families, and providers with patient-specifi c guidance regarding the most advantageous use of living donor transplantation for children with end-stage renal disease. Most phenotypes benefi t from living-donor-fi rst, but some benefi t from deceased-donor-fi rst.
The clinical course of a 14-year-old boy who developed biopsy-proven minimal change nephrotic syn... more The clinical course of a 14-year-old boy who developed biopsy-proven minimal change nephrotic syndrome (MCNS) prior to institution of therapy for Hodgkin's disease (HD), mixed cellularity histologic subtype, is presented. In the first week of radiotherapy to the major site of disease in the neck, BUN and creatinine levels rose to 98 mg% and 8.4 mg%, respectively. During the second week of treatment, levels gradually decreased becoming normal in the third week. Body weight gradually decreased in the second week of radiotherapy, falling rapidly in the third and fourth week when diuresis was greatest. Although urine protein concentration fell initially, the concentration remained 1 + or more for 10 weeks following radiotherapy. Following radiotherapy to the neck, staging laparotomy demonstrated a single focus of Hodgkin's disease in the spleen. Radiotherapy was given to the splenic pedicle: six courses of multiagent chemotherapy (MOPP) followed. The boy remains free of any evidence of either HD or MCNS 33 + months from the time of the diagnosis. Circulating lymphocytes showed normal responses to stimulation by phytohemaglutinin, Concanavalin A, and pokeweed mitogens. The patient's monocyte-macrophage-mediated antibody-dependent cellular cytotoxicity (ADCC) was low both prior to and following radiotherapy. Lymphocyte-mediated ADCC was normal at both of these times.
... Wyatt RJ, Rivas ML, Julian BA, Quiggins PA, Woodford SY, McMorrow RG, Baehler RW (1987) Regio... more ... Wyatt RJ, Rivas ML, Julian BA, Quiggins PA, Woodford SY, McMorrow RG, Baehler RW (1987) Regionalization in hered-itary IgA nephropathy. ... Kobayashi Y, Rujii K, Hiki Y, Tateno S, Kurokawa M (1988) Steroid therapy in IgA nephropathy: a retrospective study in heavy ...
1 Department of Pediatrics, Baylor College of Medicine, Houston, Texas, USA 2 Department of Pedia... more 1 Department of Pediatrics, Baylor College of Medicine, Houston, Texas, USA 2 Department of Pediatrics, Stanford University Medical Center, Stanford, California, USA 3 Diagnostic Systems Laboratories, Webster, Texas, USA ... Received: April 17, 1995; received in revised form ...
Acid-base disorders are common in sick children. This article is a practical guide to the differe... more Acid-base disorders are common in sick children. This article is a practical guide to the differential diagnosis and treatment of simple and mixed acid-base disorders of children. Special attention is given to fundamentals of acid-base physiology, to clinical use of the Henderson equation, and to interpretation of readily available laboratory tests.
The waiting list for kidney transplantation continued to grow between 1999 and 2008, from 41 177 ... more The waiting list for kidney transplantation continued to grow between 1999 and 2008, from 41 177 to 76 089 candidates. However, active candidates represented the minority of this increase (36 951-50 624, a 37% change), while inactive candidates increased over 500% (4226-25 465). There were 5966 living donor (LD) and 10 551 deceased donor (DD) kidney transplants performed in 2008. The total number of pancreas transplants peaked at 1484 in 2004 and has declined to 1273. Although the number of LD transplants increased by 26% from 1999 to 2008, the total number peaked in 2004 at 6647 before declining 10% by 2008. The rate of LD transplantation continues to vary significantly as a function of demographic and geographic factors, including waiting time for DD transplant. Posttransplant survival remains excellent, and there appears to be greater use of induction agents and reduced use of corticosteroids in LD recipients. Significant changes occurred in the pediatric population, with a dramatic reduction in the use of LD organs after passage of the Share 35 rule. Many strategies have been adopted to reverse the decline in LD transplant rates for all age groups, including expansion of kidney paired donation, adoption of laparoscopic donor nephrectomy and use of incompatible LD.
Evaluation of hypertension in the pediatric age group should be guided by the age at presentation... more Evaluation of hypertension in the pediatric age group should be guided by the age at presentation, the severity of hypertension at presentation, and whether the hypertension is sustained or transient. The common causes of hypertension differ by age group (Table 1). Hypertension is more likely to be secondary and sustained in the younger age groups, whereas primary (essential) hypertension and white coat hypertension are more likely to be the etiology in adolescents [1–5]. The global epidemic of childhood obesity has been associated with an increased incidence of primary hypertension and prehypertension in adolescents worldwide [3–7]. Obese children have as much as a threefold higher risk for developing hypertension compared to nonobese children [7]. The frequent occurrence of primary hypertension in obese adolescents dictates an entirely different evaluation than for younger or nonobese children. Renal disease must be considered in every child with hypertension, because of the prevalence of renovascular and renal parenchymal disorders as the etiology in any age group. Secondary hypertension from renal disorders is more likely to be severe at presentation than primary hypertension. Endocrine causes of hypertension are rare, so special diagnostic studies for these disorders should be reserved for those patients whose history, physical examination, and preliminary evaluation warrant further specific investigation. This chapter outlines the steps in evaluation of childhood hypertension, starting with the confirmation of the diagnosis and evaluation of its duration and severity.
OBJECTIVE: Growth failure is frequently observed in children with chronic kidney disease and thos... more OBJECTIVE: Growth failure is frequently observed in children with chronic kidney disease and those with kidney transplantation (KTx). Pubertal development and growth have a major effect on final height. There are few reports about puberty in children after KTx. We evaluated the effects of KTx on pubertal development and growth in pre-pubertal children. PATIENTS AND METHODS: From 1990 through 2009, KTx was performed in 274 children at our center. We analyzed data from those children who underwent KTx before puberty and retrospectively studied growth, signs of puberty, estimated glomerular filtration rate, and immunosuppressive treatment. RESULTS: In children transplanted before puberty, 65 children (41 boys; median age at KTx, 7.9 years; pre-emptive KTx, 9) developed secondary sexual characteristics. The mean age at the onset of puberty [healthy Japanese children] was 11.
American Journal of Physiology-renal Physiology, Sep 1, 1983
Micropuncture and clearance studies were performed to assess reabsorptive function in the proxima... more Micropuncture and clearance studies were performed to assess reabsorptive function in the proximal and distal nephron of rats with experimental Fanconi's syndrome induced by maleic acid. Anesthetized rats were studied by free-flow micropuncture of the late proximal tubule 90-120 min after continuous intravenous administration of maleic acid, 100 mg X kg-1 X h-1. Compared with control rats, the reabsorption of sodium and phosphate was significantly reduced (P less than 0.001 and less than 0.02, respectively). Tubular fluid-to-ultrafiltrate (TF/UF) chloride concentration ratio was 1.00 +/- 0.02 compared with 1.16 +/- 0.03 (P less than 0.01) in controls, suggesting a nearly total inhibition of proximal bicarbonate reabsorption. Whole kidney fractional excretions of sodium and chloride were increased significantly (P less than 0.02) but could not be explained by enhanced delivery of these solutes out of the late proximal tubule. To assess whether distal nephron reabsorption of sodium and chloride were inhibited by maleic acid, clearance studies were performed during water diuresis in awake rats. During maleic acid administration, 200 mg X kg-1 X h-1, urine flow rate (P less than 0.02) and the fractional excretions of sodium and chloride (P less than 0.001) increased significantly, but fractional free water clearance decreased from 7.16 +/- 0.42 to 4.03 +/- 0.68% (P less than 0.001). In acetazolamide-treated control rats but not in maleic acid-treated rats with similar bicarbonaturia, the magnitude of fractional free water clearance closely approximated the simultaneously measured fractional distal delivery of chloride. These studies suggest that maleic acid inhibits reabsorption at a distal nephron site or sites as well as in the proximal tubule.
IGF-binding protein-3 (IGFBP-3), usually found as glycosylated 41-and 38-kD forms, is the major s... more IGF-binding protein-3 (IGFBP-3), usually found as glycosylated 41-and 38-kD forms, is the major serum IGFBP during extrauterine life. In normal serum ICFBP-3 binds one IGF peptide and one acid-labile (a) subunit in a high-molecular-weight (MW) complex of 150 kD. By RIA, an excess of IGFBP-3 is present in chronic renal failure (CRF) serum, where it reportedly accumulates at low MW (25-55 kD) rather than as part of the 150-kD complex. To further evaluate IGFBP-3 forms in CRF, sera were obtained from seven healthy adolescents and seven adolescents with CRF. By RIA, IGFBP-3 levels were higher in CRF than normal sera (15.4 f 2.2 versus 10.1 f 2.1 wg/mL). High-MW (150-kD) fractions of CRF and normal sera, obtained by neutral size-exclusion chromatography, had equal amounts of IGFBP-3 by RIA. However, a second RIA peak of IGFBP-3, present in low-MW (35-kD) fractions of CRF but not normal sera, could account for the higher IGFBP-3 levels of CRF serum. ['251]IGF ligand blots of whole serum and serum fractions, either with or without prior precipitation by IGFBP-3 antiserum, found levels of 41-and 38-kD IGFBP-3 forms to be similar between CRF and normal whole sera and located these forms in the high-MW (150-kD) fractions of CRF and normal sera. [125111GF ligand blots also identified excess IGFBP in low-MW CRF serum fractions; cross-linking these IGFBP with [1251]IGF-I, followed by precipitation with IGFBP-I,-2, and-3 antibodies, identified high levels of unsaturated IGFBP-I, IGFBP-2, and 19-and 14-kD forms of IGFBP-3 in CRF serum. These studies indicate that I) normal levels of functional 41-and 38-kD IGFBP-3 forms are present in CRF serum, and these forms can be incorporated into the 150-kD serum complex; and 2) high RIA levels of IGFBP-3 in low-MW fractions of CRF serum are at least in part due to 19-and 14-kD IGFBP-3 forms. These studies suggest that the excess unsaturated IGFBP of CRF serum are small enough to enter interstitial tissue spaces where they may modulate IGF-I-mediated mitogenic, metabolic, and differentiative effects.
Results. A given patient cycles between fi ve Markov states: waitlisted, transplanted with a func... more Results. A given patient cycles between fi ve Markov states: waitlisted, transplanted with a functioning living donor or deceased donor graft, post-second graft failure, or deceased (Figure 1). The model accounts for the increased risk of graft failure seen during late adolescence and early adulthood, aging or disease development in the living donor over time, sensitization risks, and differential deceased donor waiting times based upon age-based allocation policies. Given a set of candidate and living donor characteristics, the model provides the expected patient survival over a time horizon of 20 years (Figure 2). Factors most predictive of a survival difference between living-donor-fi rst and deceased-donor-fi rst were level of sensitization and living donor HLA mismatch. Conclusion. The Markov model provides patients, families, and providers with patient-specifi c guidance regarding the most advantageous use of living donor transplantation for children with end-stage renal disease. Most phenotypes benefi t from living-donor-fi rst, but some benefi t from deceased-donor-fi rst.
The clinical course of a 14-year-old boy who developed biopsy-proven minimal change nephrotic syn... more The clinical course of a 14-year-old boy who developed biopsy-proven minimal change nephrotic syndrome (MCNS) prior to institution of therapy for Hodgkin's disease (HD), mixed cellularity histologic subtype, is presented. In the first week of radiotherapy to the major site of disease in the neck, BUN and creatinine levels rose to 98 mg% and 8.4 mg%, respectively. During the second week of treatment, levels gradually decreased becoming normal in the third week. Body weight gradually decreased in the second week of radiotherapy, falling rapidly in the third and fourth week when diuresis was greatest. Although urine protein concentration fell initially, the concentration remained 1 + or more for 10 weeks following radiotherapy. Following radiotherapy to the neck, staging laparotomy demonstrated a single focus of Hodgkin's disease in the spleen. Radiotherapy was given to the splenic pedicle: six courses of multiagent chemotherapy (MOPP) followed. The boy remains free of any evidence of either HD or MCNS 33 + months from the time of the diagnosis. Circulating lymphocytes showed normal responses to stimulation by phytohemaglutinin, Concanavalin A, and pokeweed mitogens. The patient's monocyte-macrophage-mediated antibody-dependent cellular cytotoxicity (ADCC) was low both prior to and following radiotherapy. Lymphocyte-mediated ADCC was normal at both of these times.
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