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Renal biopsies from 19 boys and 11 girls, most with moderate or severe forms of hemolytic uremic syndrome (HUS) of the classic diarrhea-associated type, were analyzed as part of their long-term follow-up. Patients were biopsied because of late or persistent proteinuria, hypertension, and prolonged renal failure. The median length of follow-up was 11.2 years (range 0.9-22.0 years). Four histological groups were identified: focal segmental glomerulosclerosis and hyalinosis (FSGSH) (17 patients), diffuse mesangial proliferative glomerulonephritis (DMPGN) (9 patients), diffuse glomerulosclerosis (2 patients), and minimal glomerular changes (2 patients). The median interval between the onset of disease and renal biopsy was significantly shorter in DMPGN than in FSGSH (P < 0.001). The pathological findings may be the expression of two different stages of the same dynamic process: a regular sequence of glomerular lesions consisting of early DMPGN, followed by FSGSH. This lesion would ultimately lead to the final stage of global glomerulosclerosis. At the last examination, only a quarter of the patients had normal renal function. These observations also confirm that prolonged oligoanuria during the acute stage of HUS frequently results in an unfavorable long-term prognosis.

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A new diagnostic schema for infective endocarditis (IE), the Duke criteria, has been compared with the previously published criteria of von Reyn in adult patients. This study was designed to analyze the clinical characteristics of a group of pediatric patients with IE and to compare the diagnostic efficiency of both sets of criteria. We reviewed retrospectively the clinical records of 38 patients, 22 with predisposing heart disease (Subgroup A) and 16 with no known cardiologic abnormality (Subgroup B). Ventricular septal defect was the most frequent preexisting heart disease (31.8%) and central venous catheters were the most frequent predisposing factor (68.7%). Comparison of the clinical features between subgroups (A vs. B) showed differences only for the presence of a new regurgitant murmur (9% vs. 44%, P < 0.05) and a hemoglobin value < or = 10 g/dl (50% vs. 94%, P < 0.05). The most frequent microorganisms isolated were viridans streptococci (36%) in Subgroup A and Staphylococcus aureus (50%) in Subgroup B. Of the 6 pathologically confirmed cases all would have been classified as clinically definite by the Duke criteria, as compared with 2 of 6 being defined as probable and one being rejected by von Reyn criteria. Of the 32 cases clinically defined 19 (59%) were classified as definite by the Duke criteria, and 11 (34%) were probable by the von Reyn criteria (difference 25%, P < 0.01). Although no case of IE was rejected by Duke criteria, 8 (25%) were rejected by von Reyn criteria (difference 25%, P < 0.01), with all 8 classified as possible by Duke criteria. We conclude that the Duke criteria were superior to the von Reyn criteria for the diagnosis of pediatric IE, including more cases as definite and significantly fewer cases as rejected.

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A review of extrarenal involvement in diarrhoea-associated haemolytic-uraemic syndrome (HUS) is based on 64 of our autopsied patients and an update of the literature. Large bowel pathology was the commonest (29 cases), followed by the central nervous system (21 cases), the heart (19 cases) and the pancreas (19 cases). The severity of systemic involvement was associated with the magnitude of renal compromise and the prognosis of the acute phase. Diarrhoea-associated HUS is described as a multiorgan entity, due to extensive microvascular damage and thrombosis. At present mortality during the acute phase is not confined to renal failure; systemic involvement can also lead to death.

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Eighteen records from children with renal transplants (RT) and classical haemolytic-uraemic syndrome (HUS) were reviewed. The mean oliguric period was 17.9 +/- 7.5 days; the interval between acute phase and end-stage renal disease (ESRD) was 9.3 +/- 5.2 years. HUS was the most frequent cause of renal transplantation (23.4%). There were no significant differences between patients with HUS and controls (children with RT but without HUS), regarding renal function, frequency of rejections, renal survival (HUS 65%, controls 57%) or patient survival (94.4% and 96.6%, respectively) after 9 years. None had clinical or histopathological evidence of HUS recurrence in the allograft. Of all children with living-related donors (LRD), renal survival after 3 years was longer for those who received cyclosporin A (CSA) (HUS and controls 86%) than for those who did not receive it (HUS 50%, controls 53%). Classical HUS is a frequent cause of ESRD in Argentina. The duration of the acute oliguric period is a good predictor of the likelihood of progression to chronicity. In the classical form of HUS there is no recurrence in the allograft. CSA and LRD can be used without risk in renal transplantation of children with classical HUS.

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Creatinine clearance and microalbuminuria were measured before and after an oral protein load in 17 children with a history of haemolytic uraemic syndrome, 11 with a single kidney, and 15 controls, all of them normotensive and without evidence of renal damage, to look for indirect evidence of glomerular hyperfiltration. While creatinine clearance increased significantly after the protein load in controls, it did not change in patients with either haemolytic uraemic syndrome or a single kidney. Basal microalbuminuria was significantly higher in those with haemolytic uraemic syndrome when compared with those with a single kidney and controls. It increased significantly in all groups after a water load; this increase was significantly higher in haemolytic uraemic syndrome. After the protein load microalbuminuria returned to baseline. In conclusion, children with a history of haemolytic uraemic syndrome have an abnormal renal functional reserve like children with a single kidney. Only patients with haemolytic uraemic syndrome exhibited an increased microalbuminuria, however, suggesting that it may be the expression of a pathophysiological mechanism involved in haemolytic uraemic syndrome and not in single kidney, that could account for their different prognosis.

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Serum creatine kinase (CK) and CK isoenzymes (CK-MM, CK-MB and CK-BB) were measured in 35 healthy and 25 children with hemolytic uremic syndrome (HUS) at 48 h, 7 and 15 days after admittance. Total serum CK activity was measured with a commercially available kit ("CK-NAC", by Merck, cat 14327) and CK isoenzymes using the Helena laboratories method. The interassay coefficients of variation with these methods are the following: for the total CK activity, 10.95 and 9.15% for an enzyme activity of 42 and 142 U/L respectively; for the activity of the isoenzymes, 6.8, 8.0 and 15.1% for activities of 102, 67 and 30 U/L. Total CK activity at 48 h in HUS patients defined two groups, group 1 (G1) which is not different from the control group (CG) and group 2 (G2) which had a significantly higher activity, p less than 0.0005. The increase in total CK remained significant until the first week. Increase in total CK resulted from the increase in CK isoenzymes: CK-MM, CK-MB and CK-BB. Highly significant correlation coefficients (p less than 0.0005) were obtained between total CK and their isoenzymes. When we examined both groups of patients in relation to clinical parameters, no difference could be found although G2 showed higher urea and fibrinogen degradation products with significantly decreased platelet counts. Although the reasons for enzyme release are not understood, anoxia and chemical toxins have been incriminates.(ABSTRACT TRUNCATED AT 250 WORDS)

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El síndrome urêmico hemolítico (SUH) es una enfermedad sistémica. En el presente trabajo se trata de demostrar el compromiso de la musculatura esquelética a través de la determinación seriada de creatinakinasa (CK) total y sus tres isoenzimas (CK-MM, CK-MB, CK-BB). La población estudiada que comprondió 25 niños con SUH, en relación a los valores de CK total y sus isoenzimas quedó dividida en dos grupos, G1 que se comporta como el control (35 niños sanos) y G2 en el que el aumento significativo de CK total se correlaciona en forma significativa con el aumento de las tres isoenzimas, aún hasta una semana de evolución. A los 15 días, sólo en G2 se encontró aumento sostenido de CK-BB que correlacionó significativamente a los niveles de urea plasmática y al grado de plaquetopenia. Interesante fue observar que el G2 presentaba en forma significativa una mayor concentración de urea plasmática, el mayor grado de plaquetopenia y cualitativamente una mayror concentración de productos de degradación de fibrinógeno. El aumento de Ck-MM se correlacionó en forma significativa con el aumento de CK-MG y CK-BB, además el aumento de CK-MB se correlacionó al aumento de CK-BB. Estos resultados señalarían un origen común de los tres isoenzimas, esto es el músculo estriado. En favor de esta hipótesis concurren los siguientes hallazgos: a) La actividad de CK-MB fue 7,0 ñ 1,8% de la actividad de CK total lo que concuerda con la concentración presente en el músculo esquelético. b) Por la isoenzima CK-BB tiene...

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Serum creatine kinase (CK) and CK isoenzymes (CK-MM, CK-MB and CK-BB) were measured in 35 healthy and 25 children with hemolytic uremic syndrome (HUS) at 48 h, 7 and 15 days after admittance. Total serum CK activity was measured with a commercially available kit ([quot ]CK-NAC[quot ], by Merck, cat 14327) and CK isoenzymes using the Helena laboratories method. The interassay coefficients of variation with these methods are the following: for the total CK activity, 10.95 and 9.15

for an enzyme activity of 42 and 142 U/L respectively; for the activity of the isoenzymes, 6.8, 8.0 and 15.1

for activities of 102, 67 and 30 U/L. Total CK activity at 48 h in HUS patients defined two groups, group 1 (G1) which is not different from the control group (CG) and group 2 (G2) which had a significantly higher activity, p less than 0.0005. The increase in total CK remained significant until the first week. Increase in total CK resulted from the increase in CK isoenzymes: CK-MM, CK-MB and CK-BB. Highly significant correlation coefficients (p less than 0.0005) were obtained between total CK and their isoenzymes. When we examined both groups of patients in relation to clinical parameters, no difference could be found although G2 showed higher urea and fibrinogen degradation products with significantly decreased platelet counts. Although the reasons for enzyme release are not understood, anoxia and chemical toxins have been incriminates.(ABSTRACT TRUNCATED AT 250 WORDS)

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El síndrome urÛmico hemolítico (SUH) es una enfermedad sistémica. En el presente trabajo se trata de demostrar el compromiso de la musculatura esquelética a través de la determinación seriada de creatinakinasa (CK) total y sus tres isoenzimas (CK-MM, CK-MB, CK-BB). La población estudiada que comprondió 25 niños con SUH, en relación a los valores de CK total y sus isoenzimas quedó dividida en dos grupos, G1 que se comporta como el control (35 niños sanos) y G2 en el que el aumento significativo de CK total se correlaciona en forma significativa con el aumento de las tres isoenzimas, aún hasta una semana de evolución. A los 15 días, sólo en G2 se encontró aumento sostenido de CK-BB que correlacionó significativamente a los niveles de urea plasmática y al grado de plaquetopenia. Interesante fue observar que el G2 presentaba en forma significativa una mayor concentración de urea plasmática, el mayor grado de plaquetopenia y cualitativamente una mayror concentración de productos de degradación de fibrinógeno. El aumento de Ck-MM se correlacionó en forma significativa con el aumento de CK-MG y CK-BB, además el aumento de CK-MB se correlacionó al aumento de CK-BB. Estos resultados señalarían un origen común de los tres isoenzimas, esto es el músculo estriado. En favor de esta hipótesis concurren los siguientes hallazgos: a) La actividad de CK-MB fue 7,0 ñ 1,8% de la actividad de CK total lo que concuerda con la concentración presente en el músculo esquelético. b) Por la isoenzima CK-BB tiene... (AU)

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La inyección intramuscular de penicilina benzatínica puede ocasionar complicaciones graves por lesión del nervio ciático o inyección accidental intraarterial. Se presentan 3 pacientes con lesiones isquémicas graves en miembros inferiores que provocaron amputaciones distales en todos, sumándose en el tercero mielitis transversa. Los hallazgos histopatológicos en 2 de ellos fueron compatibles con lesiones en vasos y arterias de pequeño tamaño. Se recomienda no utilizar las inyecciones intramusculares de penicilina benzatínica en menores de 2 años, en especial desnutridos o prematuros

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La inyección intramuscular de penicilina benzatínica puede ocasionar complicaciones graves por lesión del nervio ciático o inyección accidental intraarterial. Se presentan 3 pacientes con lesiones isquémicas graves en miembros inferiores que provocaron amputaciones distales en todos, sumándose en el tercero mielitis transversa. Los hallazgos histopatológicos en 2 de ellos fueron compatibles con lesiones en vasos y arterias de pequeño tamaño. Se recomienda no utilizar las inyecciones intramusculares de penicilina benzatínica en menores de 2 años, en especial desnutridos o prematuros (AU)

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Se exponen algunos de los problemas mas complejos que acompanan al diagnostico y seguimiento de los ninos con enfermedad mortal. Se describen en detalle para el equipo de salud, los aspectos practicos a fin de instrumentar la ayuda indispensable y oportuna al paciente y su familia

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Se ha llevado a cabo un estudio clinico y bioquimico completo de una paciente de 4 anos con porfiria variegata (PV) en fase aguda y en remision. La paciente fue tratada con solucion glucosada al 5%, evidenciando recuperacion clinica y bioquimica luego de 4 dias de iniciada la terapia.Desde el punto de vista bioquimico, se encontraron niveles significativamente aumentados de ALA y PBG urinarios durante la crisis, que se normalizaron alcanzada la remision. las porfirinas urinarias y fecales tambien aumentadas, evolucionaron segun lo esperado y fue importante y definitorio el patron de excrecion fecal, que mostro concentraciones elevadas de cropo y protoporfirina. Se realizo asimismo un estudio bioquimico completo en 7 familiares consanguineos, de tres generaciones, estableciendose que la abuela materna, la madre y los tres hermanos son portadores de una porfiria aguda

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Se exponen algunos de los problemas mas complejos que acompanan al diagnostico y seguimiento de los ninos con enfermedad mortal. Se describen en detalle para el equipo de salud, los aspectos practicos a fin de instrumentar la ayuda indispensable y oportuna al paciente y su familia

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Se ha llevado a cabo un estudio clinico y bioquimico completo de una paciente de 4 anos con porfiria variegata (PV) en fase aguda y en remision. La paciente fue tratada con solucion glucosada al 5%, evidenciando recuperacion clinica y bioquimica luego de 4 dias de iniciada la terapia.Desde el punto de vista bioquimico, se encontraron niveles significativamente aumentados de ALA y PBG urinarios durante la crisis, que se normalizaron alcanzada la remision. las porfirinas urinarias y fecales tambien aumentadas, evolucionaron segun lo esperado y fue importante y definitorio el patron de excrecion fecal, que mostro concentraciones elevadas de cropo y protoporfirina. Se realizo asimismo un estudio bioquimico completo en 7 familiares consanguineos, de tres generaciones, estableciendose que la abuela materna, la madre y los tres hermanos son portadores de una porfiria aguda

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