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1.
Am J Transplant ; 6(6): 1459-65, 2006 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-16686771

RESUMEN

Two children, boys of 8 and 13 years, presented with measles-associated encephalopathy several years after kidney transplantation for congenital nephrotic syndrome. In the absence of prior clinical measles, the neurological symptoms initially eluded diagnosis, but retrospective analysis of stored samples facilitated the diagnosis of measles-associated encephalopathy without recourse to biopsy of deep cerebral lesions. Each had received a single dose of measles mumps and rubella vaccine before 12 months of age. Prior vaccination, reduction of immunosuppression and treatment with intravenous immunoglobulin and ribavirin may have contributed to their survival. Persistent measles virus RNA shedding, present in one child, was not controlled by treatment with i.v. ribavirin. Two years later, both patients continue to have functioning allografts with only minimal immunosuppression. These cases illustrate the difficulty in diagnosing measles-associated encephalopathy in the immunocompromised host, even in the era of molecular diagnostics, and highlight the renewed threat of neurological disease in communities with incomplete herd immunity.


Asunto(s)
Trasplante de Riñón/fisiología , Sarampión/complicaciones , Panencefalitis Esclerosante Subaguda/epidemiología , Adolescente , Biopsia , Encéfalo/patología , Niño , Humanos , Lactante , Vacuna contra el Sarampión-Parotiditis-Rubéola , Resultado del Tratamiento
3.
QJM ; 95(9): 597-607, 2002 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-12205338

RESUMEN

BACKGROUND: The efficacy of allopurinol in autosomal dominant familial juvenile hyperuricaemic nephropathy (FJHN) has been disputed. AIM: To address this question, in the absence of controlled trials. DESIGN: Retrospective long-term follow-up study. METHODS: All kindreds were biochemically screened. Measurements included uric acid clearance, creatinine clearance, serum creatinine, and glomerular filtration rate (GFR). We used five siblings who had died or progressed to transplantation, ten other deceased relatives, and two index cases (one untreated, one non-compliant) as controls to assess the effects of allopurinol. RESULTS: Of eight families with FJHN, six had a strong history of renal disease and early parental death (mean age 41 years, n=10). Of 27 patients started immediately on allopurinol and treated uninterruptedly, 21 responded well, including three children born subsequently. Eight siblings (mean age 19 years) with a normal plasma creatinine at start (<120 micromol/l, mean GFR 80 ml/min/1.73 m(2)) retained stable renal function (mean 14.5 years, mean age 34 years, GFR 85 ml/min/1.73 m(2)). Of the 13 other responders, treated for up to 34 years, 10 with a creatinine <200 micromol/l at diagnosis (mean age 28 years, mean creatinine 137 micromol/l at start) now have a mean creatinine of 210 micromol/l. In contrast, five patients (mean age 26 years) with a creatinine >200 micromol/l (GFR <35 ml/min/1.73 m(2)) when allopurinol commenced, plus one untreated index case, all progressed rapidly (mean 6 years) to end-stage renal failure. In two others (one non-compliant, one initially untreated), GFR fell by >50% in 7 years. Introduction of allopurinol in the latter has stabilized GFR. DISCUSSION: Allopurinol reduced the morbidity and mortality from renal failure seen in untreated siblings and previous generations of these families. Early diagnosis of FJHN is important, so that treatment can begin before irreversible renal damage has developed.


Asunto(s)
Alopurinol/uso terapéutico , Supresores de la Gota/uso terapéutico , Enfermedades Renales/tratamiento farmacológico , Ácido Úrico/sangre , Uricosúricos/uso terapéutico , Adolescente , Adulto , Niño , Femenino , Estudios de Seguimiento , Humanos , Enfermedades Renales/complicaciones , Enfermedades Renales/genética , Masculino , Linaje , Insuficiencia Renal/etiología , Insuficiencia Renal/prevención & control , Estudios Retrospectivos , Síndrome , Resultado del Tratamiento , Uremia/tratamiento farmacológico , Uremia/genética
4.
Paediatr Respir Rev ; 3(2): 135-8, 2002 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-12297060

RESUMEN

Renal disease is relatively rare in cystic fibrosis even though the cystic fibrosis transmembrane regulator (CFTR) protein is expressed in abundance in the kidney. Aberrant CFTR expression probably explains the subtle abnormalities in renal concentrating and diluting ability described in cystic fibrosis and possibly the increased incidence of nephrocalcinosis. It also provides a hypothesis for the important differences in renal handling of some drugs. As the prognosis improves for patients with cystic fibrosis, secondary renal complications, for example glomerulonephritis and amyloidosis, are likely to become more prevalent. There are also a number of potentially nephrotoxic drugs used in the management of cystic fibrosis.


Asunto(s)
Fibrosis Quística/complicaciones , Enfermedades Renales/complicaciones , Amiloidosis/complicaciones , Regulador de Conductancia de Transmembrana de Fibrosis Quística/genética , Nefropatías Diabéticas/complicaciones , Glomerulonefritis por IGA/complicaciones , Humanos , Cálculos Renales/complicaciones , Enfermedades Renales/inducido químicamente , Nefritis Intersticial/complicaciones , Nefrocalcinosis/complicaciones
5.
Pediatr Nephrol ; 14(4): 309-14, 2000 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-10775075

RESUMEN

The growth of short children with chronic renal failure (CRF) and renal transplants was assessed over 10 years following entry into a 1-year trial of recombinant human growth hormone (rhGH) therapy. Patients were divided into three groups: 6 prepubertal patients with CRF (group 1), mean (range) age at start of trial 7.7 (5.0-10.4) years; 6 prepubertal patients with renal transplants (group 2), age 11.9 (9.5-14.6) years; and 6 pubertal patients with renal transplants (group 3), age 15.6 (14.1-18.3) years. In group 1, the mean (range) height standard deviation score (Ht SDS) increased from -2.9 (-3.7 to -2.2) to -1.9 (-2.9 to -0.5) over 4.0 (0.3-9.1) years of rhGH (P=0.04), and was -1.6 (-2.9 to -0.4) after 10 years of follow-up (NS). In group 2 Ht SDS increased from -3.3 (-4.5 to -1.9) to -2.9 (-5.4 to -0.5) over 2.7 (1.0-6.0) years and was -3.0 (-6.3 to -0.1) at final height (NS). In group 3 Ht SDS increased from -3.4 (-4.3 to -2.6) to -3.0 (-3.4 to -2.2) over 1.4 (0.2-2.3) years (NS) and was -2.5 (-3.0 to -1.9) at final height (P=0.03 from stopping rhGH to final height). Final height was attained in 13 patients, in whom Ht SDS increased from -3.2 (-4.3 to -1.9) to -2.6 (-3.9 to -0.5) on rhGH (P=0.004) and to -2.2 (-4.4 to -0.1) after stopping treatment (P=0.04). Four patients died, 2 have chronic hepatitis C, and 1 has had surgery for parathyroid adenomata. In conclusion, the majority of patients had an improvement in Ht SDS while on rhGH, which was maintained after stopping treatment.


Asunto(s)
Estatura/efectos de los fármacos , Hormona de Crecimiento Humana/uso terapéutico , Adolescente , Niño , Preescolar , Esquema de Medicación , Femenino , Trastornos del Crecimiento/tratamiento farmacológico , Trastornos del Crecimiento/etiología , Hormona de Crecimiento Humana/administración & dosificación , Humanos , Fallo Renal Crónico/complicaciones , Trasplante de Riñón , Estudios Longitudinales , Masculino , Complicaciones Posoperatorias , Proteínas Recombinantes/administración & dosificación , Proteínas Recombinantes/uso terapéutico
6.
Nephrol Dial Transplant ; 14(10): 2343-50, 1999 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-10528656

RESUMEN

BACKGROUND: Patients who die within 90 days of commencing renal replacement therapy (RRT) may be recorded by some centres and not others, and hence data on mortality and survival may not be comparable. However, it is essential to compare like with like when analysing differences between modalities, centres and registries. It was decided, therefore, to look at the incidence of deaths within 90 days in the ERA-EDTA Registry, and to try to define the characteristics of this group of patients. METHODS: Between 1 January 1990 and 31 December 1992, 78 534 new patients started RRT in 28 countries affiliated to the ERA-EDTA Registry. Their mean age was 54 years and 31% were over 65 years old. Eighty-two per cent of the patients received haemodialysis (HD), 16% peritoneal dialysis (PD) and 2% had preemptive transplantation as first mode of treatment. RESULTS: From January 1990 to March 1993 the overall incidence of deaths was 19% and 4% of all patients died within 90 days from the start of RRT. Among those dying within 90 days 59% were over 65 years compared to 53% over 65 years in those dying beyond this time (P<0.0001). The modality of RRT did not influence the distribution of deaths before and after 90 days. Vascular causes and malignancy were more common in those dying after 90 days, while there were more cardiac and social causes among the early deaths. Mortality from social causes was twice as common in the elderly, who had a significantly higher chance of dying from social causes within 90 days compared to those aged under 65 years. The overall incidence of deaths within 90 days was 3.9% but there was a wide variation between countries, from 1.8% to 11.4%. Finally, patient survival at 2 years was markedly influenced in different age groups when deaths within 90 days were taken into account. CONCLUSIONS: The incidence of deaths within 90 days from the start of RRT was 3.9%, with a marked variation between countries ranging from 1.8% to 11.4%, which probably reflects mainly differences in reporting these deaths, although variable selection criteria for RRT may contribute. Deaths within 90 days were significantly more frequent in elderly patients with more early deaths resulting from cardiac and social causes, while vascular causes of death and malignancy were more common in those dying after 90 days. Patient survival analyses should take into account deaths within 90 days from the start of RRT, particularly when comparing results between modalities, countries and registries.


Asunto(s)
Sistema de Registros , Terapia de Reemplazo Renal/mortalidad , Adolescente , Adulto , África del Norte , Distribución por Edad , Anciano , Causas de Muerte , Europa (Continente) , Femenino , Humanos , Israel , Fallo Renal Crónico/mortalidad , Fallo Renal Crónico/terapia , Masculino , Persona de Mediana Edad , Análisis de Supervivencia , Factores de Tiempo
7.
Nephrol Dial Transplant ; 14(10): 2351-6, 1999 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-10528657

RESUMEN

BACKGROUND: The prevalence of Renal Replacement Therapy (RRT) is rising steadily, worldwide and in Europe. One reason for this is an increasing number of patients starting RRT, but improving survival on RRT may also be contributing. MATERIAL AND METHODS: In an ERA-EDTA Registry study we have examined survival of patients with Standard Primary Renal Disease, or Diabetes, aged 20 to 75 years, who started RRT with haemodialysis (HD) or peritoneal dialysis (PD) between 1975 and 1992. Altogether close to a quarter of a million patients were included in the analysis which included conventional survival analysis of comparable subgroups of the whole cohort as well as Cox regression. RESULTS: After accounting for age, mode of initial treatment, and diagnosis, an improvement in survival of RRT patients was evident. From Cox regression it was calculated the risk for death decreased by about 5% annually during the time period 1975 1992. Patients who started RRT using PD experienced a higher mortality than those starting with HD. According to Cox regression the relative risk ratio for death was 1.25 for the whole period. The difference in survival between patients starting with PD or HD diminished during the observation period (1975-1992). DISCUSSION: The survival prospects of a patient presenting with end stage renal disease were considerably better in the early 1990s compared to the mid 1970s. This is reassuring despite the fact that mortality on RRT remains high. The higher mortality of RRT patients who started with PD is probably an 'historical' observation as the techniques of this treatment modality have improved considerably since the 1980s which was the time period from which came most of the data for the analysis.


Asunto(s)
Diálisis Peritoneal , Sistema de Registros , Diálisis Renal , Adulto , Distribución por Edad , Anciano , Estudios de Cohortes , Diabetes Mellitus/mortalidad , Diabetes Mellitus/terapia , Europa (Continente) , Humanos , Enfermedades Renales/mortalidad , Enfermedades Renales/terapia , Persona de Mediana Edad , Diálisis Peritoneal/mortalidad , Modelos de Riesgos Proporcionales , Diálisis Renal/mortalidad , Análisis de Supervivencia
8.
J Clin Periodontol ; 25(6): 457-64, 1998 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-9667479

RESUMEN

Drug regimens for transplantation often consist of multiple therapeutic agents and may result in drug-induced gingival overgrowth (DIGO). The aim of this study was to investigate the contribution of individual drugs in renal transplant patients. 147 adults (19-84 years) and 60 juveniles (3-18 years) were scored for DIGO and other clinical variables. Duration of treatment, dosage of drugs per kg body weight and serum cyclosporin levels were recorded. 44% of adults and 27% of children had DIGO. All patients were receiving prednisolone. More adults than children were administered cyclosporin, the reverse was true of azathioprine (p<0.01). Explanatory models were evaluated by stepwise ordinal polynomial logistic regression. Statistically significant explanation (p<0.05) of DIGO was afforded by prednisolone, nifedipine and azathioprine concentrations in adults and by cyclosporin, nifedipine and azathioprine concentrations in juveniles. Prednisolone and azathioprine were inversely related to the degree of DIGO. Plaque and irregularity scores, lip coverage and mouthbreathing status showed significant additional explanation in adults, replacing nifedipine and azathioprine in the final model. Irregularity was additionally explanatory in children, but no other clinical variables. A larger proportion of the variance of DIGO was explained by the available variables in children than in adults (pseudo r2=0.50 versus 0.25). The degree of DIGO in renal transplant patients is influenced by the dosage of a number of individual components of multiple drug therapy independently of the presence of local clinical factors.


Asunto(s)
Sobrecrecimiento Gingival/inducido químicamente , Inmunosupresores/efectos adversos , Trasplante de Riñón , Adolescente , Adulto , Factores de Edad , Anciano , Anciano de 80 o más Años , Análisis de Varianza , Azatioprina/administración & dosificación , Azatioprina/efectos adversos , Peso Corporal , Bloqueadores de los Canales de Calcio/administración & dosificación , Bloqueadores de los Canales de Calcio/efectos adversos , Niño , Preescolar , Ciclosporina/administración & dosificación , Ciclosporina/efectos adversos , Ciclosporina/sangre , Placa Dental/complicaciones , Combinación de Medicamentos , Femenino , Glucocorticoides/administración & dosificación , Glucocorticoides/efectos adversos , Humanos , Inmunosupresores/administración & dosificación , Inmunosupresores/sangre , Trasplante de Riñón/efectos adversos , Labio/fisiopatología , Modelos Logísticos , Masculino , Persona de Mediana Edad , Respiración por la Boca/complicaciones , Respiración por la Boca/fisiopatología , Nifedipino/administración & dosificación , Nifedipino/efectos adversos , Prednisolona/administración & dosificación , Prednisolona/efectos adversos , Factores de Tiempo
10.
Nature ; 379(6564): 445-9, 1996 Feb 01.
Artículo en Inglés | MEDLINE | ID: mdl-8559248

RESUMEN

Kidney stones (nephrolithiasis), which affect 12% of males and 5% of females in the western world, are familial in 45% of patients and are most commonly associated with hypercalciuria. Three disorders of hypercalciuric nephrolithiasis (Dent's disease, X-linked recessive nephrolithiasis (XRN), and X-linked recessive hypophosphataemic rickets (XLRH)) have been mapped to Xp11.22 (refs 5-7). A microdeletion in one Dent's disease kindred allowed the identification of a candidate gene, CLCN5 (refs 8,9) which encodes a putative renal chloride channel. Here we report the investigation of 11 kindreds with these renal tubular disorders for CLCN5 abnormalities; this identified three nonsense, four missense and two donor splice site mutations, together with one intragenic deletion and one microdeletion encompassing the entire gene. Heterologous expression of wild-type CLCN5 in Xenopus oocytes yielded outwardly rectifying chloride currents, which were either abolished or markedly reduced by the mutations. The common aetiology for Dent's disease, XRN and XLRH indicates that CLCN5 may be involved in other renal tubular disorders associated with kidney stones.


Asunto(s)
Canales de Cloruro/genética , Cálculos Renales/genética , Mutación , Secuencia de Aminoácidos , Animales , Secuencia de Bases , Calcio/orina , Células Cultivadas , Canales de Cloruro/química , Canales de Cloruro/metabolismo , ADN , Análisis Mutacional de ADN , Electroquímica , Femenino , Cálculos Renales/orina , Masculino , Datos de Secuencia Molecular , Linaje , Reacción en Cadena de la Polimerasa , Conformación Proteica , Proteínas Recombinantes/química , Xenopus
11.
J Pediatr ; 128(2): 177-83, 1996 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-8636808

RESUMEN

OBJECTIVE: To provide accurate measurement of renal function during treatment with recombinant human growth hormone (rhGH). METHODS: We measured glomerular filtration rate and effective renal plasma flow by clearance of inulin and para-aminohippuric acid before rhGH therapy, after 1 week, and then at 6-month intervals for up to 2 years of treatment in 16 children (mean (SD) age = 13.1 (2.2) years; glomerular filtration rate = 52 (27) ml/min per 1.73 m2). The mean (SD) time from transplantation was 6.5 (3.6) years. RESULTS: Linear growth velocity during rhGH therapy increased from 4.0 (1.8) to 8.8 (2.6) cm/yr (p < 0.0001). One child was withdrawn after 9 months because of abnormal glucose tolerance, and another child received a second renal transplant after 18 months. Glomerular filtration rate increased to 57 (29) ml/min per 1.73 m2 at 1 week (p = 0.004), remained improved at 6 months (63 (30); p = 0.013), but was not significantly better at 1 year (59 (33)). Effective renal plasma flow on day 1 was 237 (127) ml/min per 1.73 m2 and was unchanged on day 8 (244 (123)), at 6 months (271 (149)), and after 1 year (269 (157)). During the study there was no significant change in filtration fraction, blood pressure, or kidney volume, and excretion of microalbumin and N-acetylglucosaminidase was unaltered. There was one rejection episode per 14.8 patient-months in the year before treatment, 1 per 18.9 patient-months during the first year of treatment, and 1 per 13 patient-months during the second year of rhGH therapy. CONCLUSION: Treatment with rhGH improves growth in children with renal transplants. Glomerular filtration rate was increased after 1 week and 6 months of rhGH therapy but returned to baseline values thereafter. The data indicate the need for long-term follow-up of children with renal transplants who are receiving rhGH.


Asunto(s)
Hormona del Crecimiento/farmacología , Trasplante de Riñón , Riñón/efectos de los fármacos , Adolescente , Creatinina/sangre , Femenino , Tasa de Filtración Glomerular/efectos de los fármacos , Humanos , Riñón/irrigación sanguínea , Masculino , Proteínas Recombinantes/farmacología
13.
Transpl Int ; 9(2): 155-60, 1996.
Artículo en Inglés | MEDLINE | ID: mdl-8639258

RESUMEN

We report a successful renal transplant in a highly sensitised paediatric recipient following removal of HLA-specific antibodies by extracorporeal immunoadsorption. The immediate pretransplant cytotoxic titre against the donor was greater than 1:512; this was reduced to negativity by two immunoadsorption sessions prior to transplant surgery. We also describe the presence of unexpected non-HLA-specific antibody activities in this immunoadsorbed patient.


Asunto(s)
Autoanticuerpos , Rechazo de Injerto/terapia , Antígenos HLA/inmunología , Trasplante de Riñón/inmunología , Diálisis Renal/métodos , Niño , Rechazo de Injerto/inmunología , Humanos , Inmunoadsorbentes , Masculino
14.
Pediatr Nephrol ; 9(4): 458-63, 1995 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-7577409

RESUMEN

In normal subjects recombinant human growth hormone (rhGH) increases glomerular filtration rate (GFR) and effective renal plasma flow (ERPF) through the action of insulin-like growth factor-I (IGF-I). We have measured clearance of inulin and para-aminohippuric acid in 18 children with chronic renal failure (CRF) during their 1st year of rhGH treatment to look at the immediate (first 3 h), short-term (1 week) and long-term (1 year) effects of treatment. On day 1 mean (range) age was 9.1 (4.9-13.9) years, GFR 19 (9-58) and ERPF 77 (34-271) ml/min per 1.73 m2. During treatment height velocity increased from 4.5 (1.7-6.5) to 9.5 (4.8-12.7) cm/year (P < 0.0001). Two children required dialysis after 0.75 years and 1 child was electively transplanted after 0.5 years. There were no other serious adverse events. GFR and ERPF were unchanged in the 3 h following rhGH. GFR remained constant on day 8, 22 (6-56) and after 1 year, 20 (9-59) ml/min per 1.73 m2. ERPF increased to 96 (33-276) ml/min per 1.73 m2 on day 8 (P = 0.005), and remained elevated, but not significantly so, at 99 (24-428) ml/min per 1.73 m2 at 1 year. Fasting IGF-I increased from 147 (46-315) ng/ml to 291 (61-673) by day 8 (P < 0.003), and to 341 (101-786) ng/ml at 1 year. There was no correlation between the change in IGF-I and renal function. Blood pressure, albumin excretion and dietary protein intake were unchanged by treatment.(ABSTRACT TRUNCATED AT 250 WORDS)


Asunto(s)
Tasa de Filtración Glomerular/efectos de los fármacos , Hormona del Crecimiento/efectos adversos , Fallo Renal Crónico/fisiopatología , Circulación Renal/efectos de los fármacos , Adolescente , Presión Sanguínea/efectos de los fármacos , Estatura/efectos de los fármacos , Niño , Preescolar , Creatinina/sangre , Femenino , Trastornos del Crecimiento/tratamiento farmacológico , Trastornos del Crecimiento/etiología , Hormona del Crecimiento/administración & dosificación , Hormona del Crecimiento/uso terapéutico , Humanos , Inyecciones Subcutáneas , Factor I del Crecimiento Similar a la Insulina/metabolismo , Fallo Renal Crónico/complicaciones , Fallo Renal Crónico/terapia , Trasplante de Riñón , Masculino , Proteínas Recombinantes/administración & dosificación , Proteínas Recombinantes/efectos adversos , Proteínas Recombinantes/uso terapéutico , Diálisis Renal , Ácido p-Aminohipúrico/orina
15.
Arch Dis Child ; 72(3): 247-50, 1995 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-7741578

RESUMEN

The optimal regimen for investigating children with urinary tract infection (UTI) remains uncertain. Ultrasonography, contrast micturating cystourethrography (MCU), intravenous urography (IVU), and technetium-99m dimercaptosuccinic acid (DMSA) studies were performed in 58 children with UTI under 14 years of age attending two teaching hospitals and the results compared. All four investigations were normal in 12 children. In 36 with vesicoureteric reflux (VUR) on MCU, dilatation was reported on ultrasonography in eight children. Radiological renal scarring was seen in 20 children; it was suspected on ultrasonography in nine, with dilatation alone in four, and a normal report in seven. Duplex kidneys identified on IVU were unrecognised on ultrasonography or DMSA studies; ultrasonography showed no change corresponding to presumed acute defects on DMSA studies that later resolved. Disparities were observed at all ages. This study suggests that ultrasonography is unreliable in detecting VUR, renal scarring, or inflammatory change and, alone, is inadequate for investigating UTI in children.


Asunto(s)
Infecciones Urinarias/diagnóstico , Niño , Preescolar , Femenino , Humanos , Riñón/anomalías , Masculino , Compuestos de Organotecnecio , Succímero , Ácido Dimercaptosuccínico de Tecnecio Tc 99m , Ultrasonografía , Infecciones Urinarias/diagnóstico por imagen , Infecciones Urinarias/patología , Urografía , Reflujo Vesicoureteral/diagnóstico por imagen
16.
Arch Dis Child ; 72(3): 251-5; discussion 255-8, 1995 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-7741579

RESUMEN

The histories and imaging results are presented in 10 children in whom errors had been made in the interpretation of early investigations. Ultrasonography may not detect either vesicoureteric reflux (VUR) or renal scars or inflammation. The reduced nephrogram or renal swelling following a first attack of acute pyelonephritis may not be recognised without renal measurement on an intravenous urogram. Renal scarring may be diagnosed incorrectly on the basis of functional defects of isotope uptake on a technetium 99m-dimercaptosuccinic acid study. In the absence of VUR, the micturating cystogram will not visualise the kidneys.


Asunto(s)
Infecciones Urinarias/diagnóstico , Niño , Preescolar , Errores Diagnósticos , Femenino , Humanos , Lactante , Masculino , Compuestos de Organotecnecio , Radiografía , Cintigrafía , Succímero , Ácido Dimercaptosuccínico de Tecnecio Tc 99m , Ultrasonografía , Infecciones Urinarias/diagnóstico por imagen , Reflujo Vesicoureteral/diagnóstico por imagen
18.
Pediatr Nephrol ; 7(2): 156-8, 1993 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-8476710

RESUMEN

A 15-year-old boy with Alport's syndrome and hereditary motor and sensory neuropathy type I is described. An association between hereditary motor and sensory neuropathy and a nephropathy has been reported in 12 cases in the literature. Although showing features in common with Alport's syndrome and with a familial tendency, these 12 cases are clinically and histologically distinct from our patient who is the unfortunate inheritor of two genetic disorders, one maternally and one paternally derived. Recognition of an association between nephropathy and neuropathy, although rare, is important.


Asunto(s)
Neuropatía Hereditaria Motora y Sensorial/complicaciones , Nefritis Hereditaria/complicaciones , Glomeruloesclerosis Focal y Segmentaria/patología , Neuropatía Hereditaria Motora y Sensorial/genética , Neuropatía Hereditaria Motora y Sensorial/patología , Humanos , Lactante , Trasplante de Riñón , Masculino , Nefritis Hereditaria/genética , Nefritis Hereditaria/patología , Linaje
19.
J Am Acad Dermatol ; 28(1): 51-5, 1993 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-8425970

RESUMEN

BACKGROUND: Renal allograft transplantation is associated with an increased incidence of malignant melanoma. The development of excess melanocytic nevi may be an indicator of this risk. OBJECTIVE: This study determines the prevalence of melanocytic nevi in children who have received renal allografts. METHODS: Total and regional melanocytic nevi counts were made in 38 children (27 boys, 11 girls) with a renal allograft and in 38 individually age- and sex-matched healthy controls; counts were related to age, sex, skin type, and duration of immunosuppression. RESULTS: There was a significant increase in the total number of nevi in the renal transplant group compared with the control group (p < 0.05), with most marked increases occurring on the back and at acral sites. A strong positive correlation between nevi count and duration of immunosuppression independent of age was observed (p < 0.005). CONCLUSION: Excess numbers of melanocytic nevi occur in children with renal allografts. These patients constitute a risk group for malignant melanoma and require continued assessment.


Asunto(s)
Trasplante de Riñón/efectos adversos , Nevo Pigmentado/etiología , Neoplasias Cutáneas/etiología , Adolescente , Estudios de Casos y Controles , Niño , Preescolar , Femenino , Humanos , Terapia de Inmunosupresión/efectos adversos , Masculino , Melanoma/etiología , Melanoma/prevención & control , Nevo Pigmentado/epidemiología , Prevalencia , Análisis de Regresión , Factores de Riesgo , Neoplasias Cutáneas/epidemiología , Neoplasias Cutáneas/prevención & control , Factores de Tiempo
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