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1.
Arq. bras. med. vet. zootec. (Online) ; 70(3): 815-822, maio-jun. 2018. tab
Artículo en Portugués | LILACS, VETINDEX | ID: biblio-911510

RESUMEN

As doenças infecciosas são reconhecidamente causadoras de declínios populacionais de animais silvestres e algumas delas podem representar ameaça à saúde pública. O presente estudo objetivou investigar a ocorrência de Salmonella spp. em Psittaciformes exóticos e nativos mantidos em cativeiro na região central do Rio Grande do Sul, além de comparar os resultados obtidos por meio do método bacteriológico convencional e da reação em cadeia da polimerase (PCR), mediante a utilização de material fecal do ambiente, evitando-se o estresse da contenção. Durante os meses de agosto/2016 e setembro/2016 foram coletadas amostras de fezes frescas de 90 gaiolas em dois criatórios, representando 180 aves. Com as duas técnicas empregadas nas análises, as amostras foram negativas para Salmonella spp., contudo foram detectadas bactérias da família Enterobacteriaceae: Escherichia coli, Cedecea sp. e Citrobacter freundii. Considera-se importante a continuidade do monitoramento dos criatórios, já que o patógeno investigado pode estar em aves portadoras e se manifestar em situações de estresse, representando riscos enquanto zoonose e prejuízos à saúde das aves.(AU)


Infectious diseases are known to cause decline in wildlife population and some of them may represent a threat to public health. This study aimed to investigate the occurrence of Salmonella spp. in exotic and native Psittaciformes kept in captivity in the central region of Rio Grande do Sul, Brazil, and to comparing the results obtained through the conventional bacteriological method and the polymerase chain reaction (PCR), using fecal matter collected from the environment, avoiding the containment stress. From August to September 2016, samples of fresh feces were collected from 90 cages in two breeding grounds, representing 180 birds. According to both analysis techniques the samples were negative for Salmonella spp., however, bacteria from the Enterobacteriaceae family: Escherichia coli, Cedecea sp. and Citrobacter freundii were detected. Continuous monitoring of breeding grounds is important since the pathogen investigated can be found in carrier birds and manifest itself in stressful situations presenting risks such as zoonosis and reduced bird health.(AU)


Asunto(s)
Animales , Psittaciformes/microbiología , Salmonella/patogenicidad
3.
Arq. bras. med. vet. zootec ; 67(3): 727-731, May-Jun/2015. graf
Artículo en Portugués | LILACS | ID: lil-753920

RESUMEN

A piometra é uma infecção aguda ou crônica do útero que ocorre frequentemente em cadelas não castradas, podendo também ocorrer em gatas domésticas e selvagens, sendo poucos os estudos relacionados à piometra em grandes felídeos. O objetivo deste relato foi descrever um caso de piometra em uma leoa (Panthera leo) de cativeiro, as lesões de necropsia e histológicas, bem como os resultados da análise microbiológica. Uma leoa com aproximadamente 23 anos, pertencente a um criadouro conservacionista de Santa Maria-RS, foi encontrada morta pela manhã em seu recinto. Após coleta de dados, procedeu-se à necropsia e à coleta de material para análise histopatológica e bacteriológica. A análise microbiológica revelou predomínio das bactérias Streptococcus sp. e Escherichia coli no conteúdo purulento do útero, caracterizando como piometra, e a bactéria predominante em plasma, fígado e medula óssea foi E. coli. De acordo com o laudo histopatológico, as alterações observadas nessa leoa sugerem um quadro de septicemia grave, sendo a origem do foco infeccioso bacteriano, provavelmente, a piometra. Considera-se importante chamar a atenção dos médicos veterinários de animais selvagens para um diagnóstico precoce dessa doença, que é comum em cadelas, mas que pode acometer também felídeos selvagens e levá-los à morte.


Pyometra is an acute or chronic uterus infection that occurs often in not spayed dogs, but may also occur in wild and domestic cats, and there are few studies related to pyometra in big cats. The aim of this report was to describe a case of pyometra in a lion (Panthera leo) in captivity, the gross and microscopic lesions found at necropsy, and the results of the microbiological analysis. A female with approximately 23 years of age, belonging to a Conservationist Breeding Center located in Santa Maria-RS-Brazil was found dead in her enclosure in the morning. After data collection, we proceeded to the autopsy and collection of material for histopathological and bacteriological analyzes. Microbiological analysis revealed a predominance of the bacteria Streptococcus sp and Escherichia coli in the uterus content, characterized as pyometra and the predominant bacterium in the plasma, liver and bone marrow was Escherichia coli. According to histopathology, the changes observed in this lioness suggest a framework of severe septicemia, being the source of the bacterial infection, probably, pyometra. It is considered important to draw the attention of zoo and wildlife veterinarians for an early diagnosis of this common disease in dogs, which can also affect large wild cats and lead them to death.


Asunto(s)
Animales , Femenino , Escherichia coli/aislamiento & purificación , Leones/microbiología , Piómetra/veterinaria , Streptococcus/aislamiento & purificación , Endocarditis/veterinaria , Neumonía/veterinaria , Sepsis/veterinaria
5.
Clin Genet ; 80(6): 581-5, 2011 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-21108633

RESUMEN

Heterozygous humans for PAX2 mutations show autosomal dominant papillorenal syndrome (PRS), consisting of ocular colobomas, renal hypo/dysplasia and progressive renal failure in childhood. PAX2 mutations have also been identified in patients with isolated renal hypo/dysplasia. Twenty unrelated children and young adults with kidney and urinary tract malformations and no ocular abnormalities were retrospectively recruited for PAX2 mutational analysis. All patients had undergone renal transplantation after end-stage renal disease. We identified two new sequence variations: (i) a deletion causing a frameshift (c.69delC) and (ii) a nucleotide substitution determining a splice site mutation (c.410+5 G/A) by predictive analysis. Therefore, we suggest PAX2 molecular analysis to be extended to all patients with congenital malformations of kidney and urinary tract (CAKUT).


Asunto(s)
Riñón/anomalías , Factor de Transcripción PAX2/genética , Anomalías Urogenitales/genética , Adolescente , Secuencia de Bases , Niño , Análisis Mutacional de ADN , Anomalías del Ojo/genética , Femenino , Mutación del Sistema de Lectura , Pruebas Genéticas , Humanos , Riñón/patología , Fallo Renal Crónico/genética , Fallo Renal Crónico/patología , Trasplante de Riñón , Masculino , Datos de Secuencia Molecular , Estudios Retrospectivos , Alineación de Secuencia , Anomalías Urogenitales/patología , Adulto Joven
6.
Q J Nucl Med Mol Imaging ; 54(4): 363-71, 2010 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-20823804

RESUMEN

Pediatric hydronephrosis may correspond to very different clinical situations, ranging from fully benign reversible dilatation to severe obstructive nephropathy. The genetic research is difficult, mainly because the condition is probably polygenic and the embryology of the urinary system is very complex and depends on a multifaceted interaction of genetic and environmental factors. Molecular biology has gained new insights in the complicated urinary system and in the mechanisms of obstructive nephropathy. Some mediators (tumor growth factor, tumor necrosis factor, renin angiotensin system, etc.) could be considered molecular markers of obstruction and it has been proposed to introduce them in clinical decision making, in order to make an accurate selection of patients needing surgical correction. Scintigraphy has been a standard procedure in the management of pediatric hydronephrosis for decades and has been used in many clinical studies designed to evaluate the role of selected molecular markers in clinical settings. The relationships between scintigraphic parameters and molecular mediators seems promising, in particular for the evaluation of the Reanin Angiotensin System, which plays many roles in the natural history of pediatric hydronephrosis. Angiotensin up-regulation is a turning point in many pediatric hydronephrosis and can be unveiled by captopril scintigraphy, which allows a timely diagnosis of obstruction, before irreversible parenchymal injury and loss of renal function.


Asunto(s)
Hidronefrosis/diagnóstico por imagen , Animales , Biomarcadores/metabolismo , Niño , Modelos Animales de Enfermedad , Humanos , Hidronefrosis/genética , Hidronefrosis/patología , Hidronefrosis/fisiopatología , Biología Molecular , Medicina Nuclear , Cintigrafía , Sistema Renina-Angiotensina/fisiología
7.
Am J Transplant ; 10(4): 828-836, 2010 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-20420639

RESUMEN

Minimizing steroid exposure in pediatric renal transplant recipients can improve linear growth and reduce metabolic disorders. This randomized multicenter study investigated the impact of early steroid withdrawal on mean change in height standard deviation score (SDS) and the safety and efficacy of two immunosuppressive regimens during the first 6 months after transplantation. Children received tacrolimus, MMF, two doses of daclizumab and steroids until day 4 (TAC/MMF/DAC, n=98) or tacrolimus, MMF and standard-dose steroids (TAC/MMF/STR, n=98). Mean change in height SDS was 0.16 +/- 0.32 with TAC/MMF/DAC and 0.03 +/- 0.32 with TAC/MMF/STR. The mean treatment group difference was 0.13 (p < 0.005 [95% CI 0.04-0.22]), 0.21 in prepubertal (p = 0.009 [95% CI 0.05-0.36]) and 0.05 in pubertal children (p = ns). Frequency of biopsy-proven acute rejection was 10.2%, TAC/MMF/DAC, and 7.1%, TAC/MMF/STR. Patient and graft survival and renal function were similar. Significantly greater reductions in total cholesterol and triglycerides but significantly higher incidences of infection and anemia were found with TAC/MMF/DAC (p < 0.05 all comparisons). Early steroid withdrawal significantly aided growth at 6 months more so in prepubertal than pubertal children. This was accompanied by significantly better lipid and glucose metabolism profiles without increases in graft rejection or loss.


Asunto(s)
Anticuerpos Monoclonales/administración & dosificación , Crecimiento , Inmunoglobulina G/administración & dosificación , Inmunosupresores/administración & dosificación , Fallo Renal Crónico/cirugía , Trasplante de Riñón , Esteroides/administración & dosificación , Tacrolimus/administración & dosificación , Adolescente , Anticuerpos Monoclonales Humanizados , Niño , Preescolar , Daclizumab , Humanos
9.
J Urol ; 176(6 Pt 1): 2668-73; discussion 2673, 2006 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-17085190

RESUMEN

PURPOSE: We evaluated clinical and biological variables, and their meaning as reliable markers of chronic interstitial nephropathy in a selected group of children with prenatally detected hydronephrosis who underwent pyeloplasty because of congenital unilateral ureteropelvic junction obstruction. MATERIALS AND METHODS: We reviewed the clinical, prenatal and postnatal ultrasonographic, and scintigraphic records of children for whom intraoperative biopsy records were available. We performed histological analysis, and evaluated tubulointerstitial immunostaining for vimentin and alpha-smooth muscle actin, and the immunohistochemical and mRNA expression of the renin-angiotensin system peptides and transforming growth factor-beta1. RESULTS: The children were divided in 2 groups according to the absence (group 1) or presence (group 2) of chronic interstitial nephropathy in the biopsy. Patients in group 2 were significantly younger at prenatal diagnosis (p = 0.031), and had decreased split renal function (p = 0.005) and worse drainage (p = 0.035) on preoperative diuretic renography. No differences were found in terms of degree of hydronephrosis, or its prenatal and postnatal variation. Group 2 biopsies exhibited greater immunostaining for alpha-smooth muscle actin and vimentin (p = 0.004 and p = 0.047, respectively), and transforming growth factor-beta1 mRNA levels (p = 0.06). Vimentin and alpha-smooth muscle actin positivity correlated with renin, angiotensin II receptors 1 and 2, and transforming growth factor-beta1 mRNA levels, and all correlated with preoperative split renal function and post-void washout. CONCLUSIONS: In congenital unilateral ureteropelvic junction obstruction chronic interstitial nephropathy and poor postoperative recovery seem to be associated with an earlier diagnosis of hydronephrosis, functional loss greater than 10% and worse scintigraphic drainage. Moreover, there is a strong correlation between molecular fibrogenic markers and histologically and scintigraphically demonstrated renal damage.


Asunto(s)
Hidronefrosis/diagnóstico , Nefritis Intersticial/diagnóstico , Obstrucción Ureteral/complicaciones , Actinas/metabolismo , Progresión de la Enfermedad , Femenino , Enfermedades Fetales/diagnóstico por imagen , Humanos , Hidronefrosis/congénito , Técnicas para Inmunoenzimas , Inmunohistoquímica , Lactante , Túbulos Renales/metabolismo , Túbulos Renales/patología , Masculino , Nefritis Intersticial/etiología , Nefritis Intersticial/metabolismo , Nefritis Intersticial/patología , Reacción en Cadena de la Polimerasa , Pronóstico , Estudios Retrospectivos , Ultrasonografía Prenatal , Obstrucción Ureteral/congénito , Obstrucción Ureteral/cirugía , Vimentina/metabolismo
10.
Kidney Int ; 70(7): 1342-7, 2006 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-16900087

RESUMEN

Type 1 nephronophthisis (NPHP) with homozygous deletions of nephrocystin [NPHP1, DEL] has been considered a pure renal disorder, but co-occurrence of extrarenal symptoms, mainly retinitis pigmentosa, is observed in a subset of patients. Recently, [NPHP1, DEL] has been detected in three patients with Joubert syndrome-related disorders (JSRDs), who associated neurological signs with a peculiar neuroradiological malformation known as the 'molar tooth sign' (MTS). To define the frequency of JSRD spectrum in NPHP1 patients, we re-examined 56 cases with [NPHP1, DEL] and found an overall incidence of 8.9% (five out 56 patients). All had small hyperechoic kidneys and had developed advanced renal failure within 15 years. Two patients presented the complete features of JSRD with cerebello-renal-retinal association and MTS. Two others showed, instead, severe intentional tremor and thick superior cerebellar peduncles on brain magnetic resonance imaging (MRI), and one of them had associated retinopathy. The fifth patient presented with hypotonia, developmental delay, central deafness, and ataxia associated with Leber congenital amaurosis and liver fibrosis but with normal brain MRI. Marked intrafamilial variability of associated extrarenal symptoms was observed in familial cases. Deletion extension did not differ in patients with isolated renal phenotype and in those with associated neurological symptoms. In conclusion, neurological defects varying from subtle involvement of cerebellum with thickened peduncle to both JSRD and diffuse central hypotonia are frequent in [NPHP1, DEL] patients. Prevalence of such association may justify systematic neurological and neuroradiological evaluation.


Asunto(s)
Enfermedades Renales/genética , Adolescente , Adulto , Factores de Edad , Cerebelo/anomalías , Niño , Femenino , Eliminación de Gen , Homocigoto , Humanos , Incidencia , Enfermedades Renales/complicaciones , Enfermedades Renales/diagnóstico , Enfermedades Renales/diagnóstico por imagen , Enfermedades Renales/epidemiología , Fallo Renal Crónico/etiología , Imagen por Resonancia Magnética , Masculino , Fenotipo , Prevalencia , Retinitis Pigmentosa/complicaciones , Síndrome , Factores de Tiempo , Ultrasonografía
11.
Neurology ; 65(4): 606-8, 2005 Aug 23.
Artículo en Inglés | MEDLINE | ID: mdl-16116126

RESUMEN

Coenzyme Q10 (CoQ10) deficiency has been associated with various clinical phenotypes, including an infantile multisystem disorder. The authors report a 33-month-old boy who presented with corticosteroid-resistant nephrotic syndrome in whom progressive encephalomyopathy later developed. CoQ10 was decreased both in muscle and in fibroblasts. Oral CoQ10 improved the neurologic picture but not the renal dysfunction.


Asunto(s)
Enfermedades Renales/etiología , Enfermedades Renales/prevención & control , Encefalomiopatías Mitocondriales/complicaciones , Encefalomiopatías Mitocondriales/tratamiento farmacológico , Ubiquinona/análogos & derivados , Atrofia/etiología , Atrofia/patología , Atrofia/fisiopatología , Encéfalo/efectos de los fármacos , Encéfalo/patología , Encéfalo/fisiopatología , Preescolar , Coenzimas , Creatinina/sangre , Progresión de la Enfermedad , Diagnóstico Precoz , Transporte de Electrón/efectos de los fármacos , Transporte de Electrón/genética , Femenino , Humanos , Lactante , Enfermedades Renales/fisiopatología , Imagen por Resonancia Magnética , Masculino , Mitocondrias/efectos de los fármacos , Mitocondrias/metabolismo , Encefalomiopatías Mitocondriales/metabolismo , Músculo Esquelético/efectos de los fármacos , Músculo Esquelético/patología , Músculo Esquelético/fisiopatología , Recuperación de la Función/efectos de los fármacos , Recuperación de la Función/fisiología , Resultado del Tratamiento , Ubiquinona/deficiencia , Ubiquinona/uso terapéutico
12.
Transplant Proc ; 37(2): 856-8, 2005 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-15848555

RESUMEN

This open-label, longitudinal, long-term study of de novo pediatric renal transplant recipients was designed to investigate the pharmacokinetics (PK) of mycophenolic acid (MPA) and its possible interaction with cyclosporine (CsA). Thirty-four children on an immunosuppressive regimen of CsA, prednisone, and mycophenolate mofetil (MMF, 300-400 mg/m2 twice daily) were investigated at 6, 30, 180, and 360 days after transplantation. Considerable interindividual variability in the areas under the concentration curve (AUC(0-12)) of MPA was observed during the follow-up, although the dose of MMF remained the same over the same time. Predose levels (C0) increased significantly during the first 6 months after transplantation: C0 at 6 and 180 days after transplantation was 0.8 +/- 0.6 and 1.9 +/- 1.1 microg/mL (P < .0001). A significant time-dependent increase in the AUC of MPA was also observed during the first 6 posttransplant months: AUC(0-12) at 6 and 180 days after transplantation was 23.3 +/- 10.8 and 40 +/- 11.6 mg*h/L (P = .003). MPA concentrations 3 and 4 hours after MMF intake were the individual time points that best correlated with the full MPA AUC (r = 0.8 and 0.79; P < .001). The abbreviated MPA AUC (0-4 hours) correlated reasonably with the full AUC (r = 0.87; P < .001). Finally, a significant reduction in CsA dose during the first 6 posttransplant months (P < .001) matched the significant increases in both MPA C0 and full MPA AUC, thus demonstrating the interaction of the 2 immunosuppressive drugs. These observations suggest the need for therapeutic drug monitoring when adjusting the dose of MMF in children.


Asunto(s)
Trasplante de Riñón/fisiología , Ácido Micofenólico/análogos & derivados , Niño , Ciclosporina/uso terapéutico , Relación Dosis-Respuesta a Droga , Interacciones Farmacológicas , Monitoreo de Drogas/métodos , Humanos , Trasplante de Riñón/inmunología , Tasa de Depuración Metabólica , Ácido Micofenólico/sangre , Ácido Micofenólico/farmacocinética , Ácido Micofenólico/uso terapéutico , Periodo Posoperatorio
13.
J Urol ; 165(6 Pt 2): 2296-9, 2001 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-11371966

RESUMEN

PURPOSE: A growing body of evidence identifies the renin-angiotensin system as a key factor in the onset and progression of renal damage in chronic partial obstruction, which often represents a complex diagnostic challenge. A prospective study was undertaken to evaluate the role of captopril mercaptoacetyltriglycine-3 (MAG-3) renography as an early diagnostic test of obstruction. We report the results in a subgroup of children who underwent surgical correction for pyeloureteral obstruction. MATERIALS AND METHODS: Pyeloplasty was performed in 12 patients, including 10 males, 2 to 72 months old (median age 7) with unilateral hydronephrosis, including normal renal function and blood pressure. Basal and captopril enhanced diuretic renography with 99mtechnetium MAG-3 was performed within 24 hours using the same hydration and diuretic stimulus (0.75 mg./kg. furosemide), and 0.75 mg./kg. captopril was administered orally 60 to 90 minutes before scintigraphy. RESULTS: No adverse effects or modifications of the blood pressure were observed after captopril administration. The diuretic response was deeply worsened by angiotensin converting enzyme inhibition in each hydronephrotic kidney even when the basal study was only slightly abnormal (15-minute washout basal -27 +/- 16%, after captopril -9 +/- 13, p <0.005). After surgical correction the diuretic washout during angiotensin inhibition appeared normal in all patients (15-minute washout -56 +/- 14%). Separate renal function and parenchymal transit of MAG-3 were not modified by angiotensin converting enzyme inhibition, preoperatively or postoperatively. CONCLUSIONS: Our data confirm the influence of angiotensin on the kidney excretory system in human hydronephrosis and suggest a role for captopril enhanced diuretic renography in the early diagnosis of pyeloureteral obstruction. Further work is needed to evaluate angiotensin converting enzyme inhibition as a protective agent in obstructive nephropathy.


Asunto(s)
Inhibidores de la Enzima Convertidora de Angiotensina , Captopril , Hidronefrosis/diagnóstico por imagen , Hidronefrosis/fisiopatología , Renografía por Radioisótopo , Radiofármacos , Tecnecio Tc 99m Mertiatida , Niño , Preescolar , Femenino , Humanos , Lactante , Masculino , Estudios Prospectivos , Sistema Renina-Angiotensina/fisiología
14.
Pediatr Nephrol ; 16(1): 1-7, 2001 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-11198593

RESUMEN

To evaluate the efficacy of renal transplantation in small pediatric patients, we have reviewed 41 allografts performed in 39 children (28 M/11 F) less than 6 years of age between 1987 and 1998 in the North Italy Transplant Program. Of these patients, 39 had a cadaver donor and 2 a living-related donor, with ages ranging from 20 days to 35 years. The mean follow-up was 56 months. Graft survival was 74.5% and 70.5% at 1 and 5 years, respectively. The causes of graft lost were acute rejection (4), graft vascular thrombosis (4), and hemolytic uremic syndrome recurrence (1). Only 1 patient has died due to chickenpox. Double and triple immunosuppressive therapies were used in 63% and 37% of patients, respectively, on the basis of different center protocols, without differences in graft survival. Steroids were successfully administered on alternate days in 37% of patients, 6-12 months after transplantation. Thrombosis was reported in 2 of 6 kidneys from donors less than 1 year of age and in 2 of 35 donors older than 1 year (P < 0.05). Thirty rejections occurred in 23 patients: 7 episodes were steroid resistant and were treated with ATG/OKT3. Thirty-four infections were reported in 16 of 41 patients; of these 17 were viral, 14 bacterial, and 3 due to Mycoplasma. Four surgical complications were reported: 1 graft artery stenosis, 1 ureteral stenosis, 1 urinary leak, and 1 lymphocele. Mean height standard deviation score improved from -2.0 +/- 1.3 pre transplantation to -1.8 +/- 1.4, -1.5 +/- 1.3, and -1.5 +/- 1.5 at 1, 2, and 5 years post transplantation. Linear growth was significantly better in infants treated with alternate-day steroids. Hypertension was a frequent complication, since 19 of the 30 patients with a 5-year follow-up were still being treated with antihypertensive drugs. In conclusion, graft survival in patients less than 6 years old is satisfactory and similar to that obtained in children aged from 6 to 18 years (70.5% vs. 78.9% at 5 years, P = NS). Consequently, since there are many difficulties in managing infants on maintenance dialysis, an early transplant should be considered. Donors older than 24 months carry a low risk of vascular thrombosis and may be successfully grafted in infants.


Asunto(s)
Trasplante de Riñón , Desarrollo Infantil , Preescolar , Femenino , Estudios de Seguimiento , Tasa de Filtración Glomerular , Rechazo de Injerto/epidemiología , Trasplante de Corazón , Humanos , Terapia de Inmunosupresión , Incidencia , Recién Nacido , Infecciones/etiología , Enfermedades Renales/cirugía , Trasplante de Riñón/efectos adversos , Trasplante de Hígado , Masculino , Complicaciones Posoperatorias , Recurrencia , Análisis de Supervivencia , Trombosis/etiología , Resultado del Tratamiento
15.
Ann Hematol ; 79(8): 452-4, 2000 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-10985366

RESUMEN

We report an 8-year-old girl who presented with hemolytic-uremic syndrome (HUS) as the onset manifestation of acute lymphocytic leukemia (ALL). The patient was admitted to the hospital for renal failure, thrombocytopenia, and anemia during a HUS outbreak. She was discharged 2 weeks later with normal renal function. One month later the girl presented with clinical and laboratory signs consistent with a diagnosis of ALL. The short time interval between HUS and ALL suggests that HUS was probably an early manifestation of acute leukemia.


Asunto(s)
Síndrome Hemolítico-Urémico/diagnóstico , Leucemia-Linfoma Linfoblástico de Células Precursoras/diagnóstico , Niño , Diagnóstico Diferencial , Brotes de Enfermedades , Femenino , Síndrome Hemolítico-Urémico/epidemiología , Humanos
16.
J Chromatogr B Biomed Sci Appl ; 744(2): 241-7, 2000 Jul 21.
Artículo en Inglés | MEDLINE | ID: mdl-10993511

RESUMEN

We report a new high-performance liquid chromatography method developed for measuring inulin in plasma and urine using ion moderated partition chromatography and evaporative light-scattering detection. Samples are deproteinized with a zinc acetate and phosphotungstic acid solution and added with melezitose as an internal standard. The chromatographic separation is carried out in 16 min at a flow-rate of 0.6 ml/min using deionized water as the mobile phase. Within-run precision, measured at four different concentrations (0.050 mg/ml, 0.150 mg/ml, 0.300 mg/ml and 1.200 mg/ml), ranges from 1.7 to 3.4% in plasma and from 1.5 to 3.5% in urine. Similarly, between-run precision is in plasma from 2.0 to 4.3% and in urine from 2.0 to 4.4%. Analytical recovery ranges from 97.9 to 100.1% in plasma and from 99.1 to 99.7% in urine, respectively. Detection limit (signal-to-noise ratio=3) is 5 microg/ml both in plasma and urine. The method is simple, sensitive, without interference due to hexoses or drugs commonly taken by patients with renal diseases, and offers the advantage of measuring inulin without previous hydrolysis of the molecule.


Asunto(s)
Cromatografía Líquida de Alta Presión/métodos , Inulina/análisis , Adolescente , Adulto , Calibración , Niño , Preescolar , Femenino , Humanos , Inulina/sangre , Inulina/orina , Luz , Masculino , Reproducibilidad de los Resultados , Dispersión de Radiación , Sensibilidad y Especificidad
17.
Clin Nephrol ; 53(4): suppl 8-9, 2000 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-10809427

RESUMEN

BACKGROUND: It is hypothesized that in acute and chronic CsA nephrotoxicity, in vivo models CsA side-effects are mediated by Renin-Angiotensin II (RAS)-TGF-beta-1 pathway. However, to induce chronic nephrotoxicity, CsA administration has to be combined with a low salt diet, which causes hemodynamic changes and RAS up-regulation. MATERIALS AND METHODS: In order to define any direct correlation between CsA and nephrotoxicity, we studied in normal sodium fed rats, the chronic effects of CsA administration (group-1 treated with 12.5 mg/Kg/day of CsA subcutaneously; group 2 received daily placebo; group 3 interrupted CsA injection after 60 days), on renal TGF-beta-1 and collagen III expression, and on TGF-beta-1, collagen III and IV deposition. Sacrifices were performed after 2, 4, 8 and 12 weeks (wks) and kidneys were harvested for immunohistological studies and RT/PCR analysis. RESULTS: No difference of TGF-beta-1 expression and deposition was found among groups. Starting from the 2nd week of treatment, an increased collagen III deposition was evident in vessels and in outer medulla with subsequent extension at the 4th week to medullary rays and to cortex interstitium. The deposition paralleled the renal collagen III mRNA up-regulation: it was significantly higher in group 1 than in group 2 (p < 0.009 at 2nd wk; p < 0.016 at 4th wk). Collagen IV deposition did not differ between groups at any point. CONCLUSIONS: Our results suggest that chronic CsA administration can induce, in normal fed rats, the process of interstitial fibrogenesis through TGF-beta non-related mechanisms.


Asunto(s)
Colágeno/genética , Ciclosporina/farmacología , Inmunosupresores/farmacología , Riñón/efectos de los fármacos , Riñón/metabolismo , Animales , Ciclosporina/farmacocinética , Inmunosupresores/farmacocinética , Masculino , ARN Mensajero/biosíntesis , Ratas , Ratas Wistar , Factor de Crecimiento Transformador beta/metabolismo
18.
Am J Kidney Dis ; 35(1): 44-51, 2000 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-10620543

RESUMEN

Autosomal recessive nephronophthisis (NPH) is a renal disorder histologically characterized by tubulointerstitial lesions that are, in some cases, associated with extrarenal manifestations such as tapeto-retinal degeneration or liver fibrosis. The disease is usually pauci-symptomatic in an early phase but invariably evolves to end-stage renal failure in childhood or early adulthood. The recent discovery of the NPHP1 gene (nephrocystin) has prompted research into putative genotype-phenotype correlations. We screened a population of 68 Italian children (10 multiplex families, 47 sporadic cases) with a clinical and histopathologic picture of NPH and found a large homozygous deletion at 2q13 involving nephrocystin in 30 cases, and heterozygous deletion associated with new point mutations at exons 15 (Tyr518Ter) and 17 (Arg585Ter) of the gene in two other cases. The remaining 36 children had no apparent molecular defects of nephrocystin. In spite of this genetic heterogeneity, the two groups, with and without detectable molecular defects of nephrocystin, showed similar renal defects and comparable cumulative survival considering the start of dialysis as an end-point. The unique difference observed was a less frequent requirement of dialysis in NPH1 patients with pure renal form. Finally, tapeto-retinal degeneration was associated with renal lesions in seven cases presenting deletion of the nephrocystin gene and in five sporadic cases without molecular defects. These data show that a molecular defect of nephrocystin is involved in approximately 50% of patients with NPH, and another 50% require further molecular characterization. Research therefore should now be aimed at characterizing a new locus. In spite of the molecular heterogeneity, NPH in children presents similar renal and extrarenal manifestations, thus suggesting the involvement of common pathological routes.


Asunto(s)
Tamización de Portadores Genéticos , Pruebas Genéticas , Fallo Renal Crónico/genética , Nefritis Intersticial/genética , Proteínas Adaptadoras Transductoras de Señales , Adolescente , Adulto , Niño , Preescolar , Aberraciones Cromosómicas/genética , Deleción Cromosómica , Trastornos de los Cromosomas , Cromosomas Humanos Par 2 , Proteínas del Citoesqueleto , Exones , Femenino , Genes Recesivos/genética , Genotipo , Humanos , Italia , Fallo Renal Crónico/diagnóstico , Masculino , Proteínas de la Membrana , Nefritis Intersticial/diagnóstico , Linaje , Fenotipo , Mutación Puntual/genética , Proteínas/genética , Retinitis Pigmentosa/diagnóstico , Retinitis Pigmentosa/genética
19.
J Urol ; 163(2): 556-60, 2000 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-10647684

RESUMEN

PURPOSE: We investigated glomerular filtration rate and renal function reserve after the surgical relief of partial obstruction. MATERIALS AND METHODS: We evaluated 4 boys and 1 girl 9 to 14 years old who underwent pyeloplasty because of unilateral ureteropelvic junction obstruction. Contralateral normal kidneys served as controls. The glomerular filtration rate (inulin clearance), and urinary excretion of prostaglandin E2, thromboxane B2 and endothelin were determined at baseline and after a meal of 4 gm./kg. cooked unsalted red meat on day 4 postoperatively. Tests were repeated the following day 1 hour after the oral administration of 20 mg./kg. aspirin, an inhibitor of prostaglandin E2 synthesis. Urine was collected separately through a bladder catheter and another catheter placed in the upper renal pelvis at surgery. RESULTS: Glomerular filtration rate at baseline was significantly greater in normal than in surgically treated kidneys (77.2 ml. per minute, range 60 to 98 versus 63.6, range 43 to 78, p = 0.04). Aspirin did not change baseline inulin clearance in normal kidneys but it significantly decreased the glomerular filtration rate in operated renal units (-4% versus -26.4%, p = 0.04). The concentration of all vasoactive compounds was not significantly different in the urine specimens of normal and operated kidneys. The administration of aspirin resulted in a significant decrease in mean urinary prostaglandin E2 excretion plus or minus standard error in operated but not in normal renal units (0.64+/-0.12 ng. per minute versus 0.27+/-0.06, p = 0.04). When expressed as mean versus baseline values, protein induced glomerular hyperfiltration seemed lower in operated than in contralateral intact kidneys (6.9% and 12.4%, respectively). CONCLUSIONS: In the immediate postoperative period previously obstructed kidneys maintain renal function via mechanisms that depend on the activation of prostaglandin, mimicking normal renal function. This effect is decreased by drugs that inhibit prostaglandin E2 production. Therefore, renal damage may be present when the glomerular filtration rate appears normal.


Asunto(s)
Aspirina/farmacología , Dinoprostona/antagonistas & inhibidores , Pelvis Renal/cirugía , Obstrucción Ureteral/cirugía , Adolescente , Niño , Dinoprostona/orina , Endotelinas/orina , Femenino , Tasa de Filtración Glomerular , Humanos , Masculino , Cuidados Posoperatorios , Tromboxano B2/orina
20.
Int J Artif Organs ; 23(1): 49-54, 2000 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-12118837

RESUMEN

UNLABELLED: Photopheresis (ECP) is a new immunomodulatory therapy in which recipient lymphocytes are treated extracorporeally with 8-methoxypsoralen and ultraviolet light. The treatment seems to induce an inhibition of both humoral and cellular rejection after transplantation. OBJECTIVE: Since recurrent rejection (RR) continues to be a severe complication after heart transplantation (HTx) and the immunosuppressive regimes used for the treatment are often associated with increased morbidity and mortality, we investigated whether ECP could have a beneficial effect on the number and severity of rejection episodes. METHODS: Eleven HTX recipients (5 M and 6 F, mean age 48.5 yrs) with RR were enrolled in the study. ECP was performed at weekly intervals during the 1st month, at 2 week intervals during the 2nd and 3rd month, and then monthly for another 3 months. RESULTS: The fraction of biopsies (EMB) with a grade 0/1A rejection increased during ECP from 46% to 72% while the EMB showing a 3A/3B rejection decreased from 42% to 18%. It is also noteworthy that out of the 78 EMB performed during ECP only one showed a 3B rejection in comparison with 13 out of 110 EMB in the pre-ECP period. Six rejection relapses were observed in a total follow-up of 60 months, two of them occurring during the tapering of oral steroid. Four relapses were reversed by ECP, one by i.v. steroids and the last by methotrexate after the failure of both i.v. steroids and ECP. The mean doses of immunosuppressive drugs resulted lower after 6 months of ECP: steroids were reduced from 13 to 8.25 mg/day, cyclosporine from 375 to 285 mg/day, azathioprine from 55 to 35 mg/day. CONCLUSIONS: ECP is a well tolerated treatment. Its administration allows better RR control and significant reduction in immunosuppressive therapy.


Asunto(s)
Rechazo de Injerto/prevención & control , Trasplante de Corazón , Fotoféresis/métodos , Adulto , Biopsia , Femenino , Rechazo de Injerto/patología , Humanos , Inmunosupresores/uso terapéutico , Masculino , Persona de Mediana Edad , Resultado del Tratamiento
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