Efficacy of a flexible schedule for administration of a Leptospira borgpetersenii serovar Hardjo bacterin to beef calves.

OBJECTIVE: To determine whether a flexible vaccination regimen provides protection against challenge exposure with a virulent Leptospira borgpetersenii serovar Hardjo isolate. ANIMALS: Fifty-five 4-week-old calves seronegative for antibodies against L borgpetersenii serovar Hardjo. PROCEDURES: Calves were assigned to 3 groups and administered 2 doses of adjuvant (control calves; n = 11), 1 dose of serovar Hardjo bacterin and 1 dose of adjuvant (22), or 2 doses of the serovar Hardjo bacterin (22); there was a 16-week interval between dose administrations. Three weeks after the second dose, all calves were challenge exposed by use of conjunctival instillation of a heterologous strain of L borgpetersenii serovar Hardjo for 3 consecutive days. Urine samples for leptospiral culture were collected for 5 weeks after challenge exposure; at that time, all calves were euthanized and kidney samples collected for leptospiral culture. RESULTS: Antibody titers increased in both leptospiral-vaccinated groups of calves. A significant increase in antibody titers against L borgpetersenii serovar Hardjo was detected after administration of the second dose of L borgpetersenii serovar Hardjo bacterin and challenge exposure. In 10 of 11 adjuvant-treated control calves, serovar Hardjo was isolated from both urine and kidney samples. Leptospira borgpetersenii serovar Hardjo was not isolated from the urine or kidney samples obtained from any of the 21 remaining calves that received 1 dose of bacterin or the 20 remaining calves that received 2 doses of bacterin. CONCLUSIONS AND CLINICAL RELEVANCE: Protection in young calves was induced by vaccination with 1 or 2 doses of a serovar Hardjo bacterin.

Extranodal rosai-dorfman disease presenting as a solitary mass with human herpesvirus 6 detection in a pediatric patient.

In Rosai-Dorfman disease (RDD), exclusive extranodal involvement with lesions limited to the kidneys is very uncommon and has been described only in adult patients. Occasionally, human herpesvirus 6 (HHV-6) has also been detected in RDD tissue samples. We present the case of a 7-year-old boy referred to our center presenting a single solid mass in the right kidney measuring 3.4 cm, detected both on contrast computed tomography and magnetic resonance imaging. Surgical excision was successfully completed, and the pathology report informed characteristic histopathology and immmunohistochemistry features of RDD. Human herpesvirus 6 was detected and amplified by polymerase chain reaction, as well as by immunohistochemistry. We discuss imaging and histology-based differential diagnoses in the pediatric age group. Although RDD is a rare histiocytic disorder of unknown etiology and pathogenesis, the presence of HHV-6 observed in this case supports the possibility of an abnormal immunologic response linked to viral presence.

Miofibromatosis infantil / Infantile myofibromatosis

La miofibromatosis infantil es una enfermedad del período neonatal o de la infancia temprana; consiste en la aparición de uno o múltiples tumores fibrosos que afectan piel, tejido celular subcutáneo, músculos, huesos y/o vísceras. Son tumores con tendencia a la involución espontánea, y su pronóstico está condicionad por la presencia o no de compromiso visceral. Se presenta el caso de un recién nacido de 48 horas de vida, con una tumoración en miembro superior derecho, sin compromiso visceral, que presentó involución espontánea en un plazo de 8 meses.

Infantile myofibromatosis is an illness of the neonatal period or early childhood. It consists of the appearance of one or more fibrous tumors which affect the skin, subcutaneous tissue, muscles, bones and/or viscera. These tumors tend to undergo spontaneousinvolution, and the prognosis is conditioned by the presence or absence of visceral liability. The case of a 48-hour newborn is described. The infant presented a tumour in the upper right member, which did not revealedvisceral liability and which resulted in spontaneous involution over an eight month span.

[Tubulointerstitial nephritis associated with parvovirus beta19 infection]. / Nefritis tubulo intersticial asociada a parvovirus B19.

A previously healthy 9 year old girl developed nephrotic syndrome with hypertension, microhematuria and normal renal function. The patient evolved as steroid resistant nephrotic syndrome whose initial renal biopsy was consistent with diffuse proliferative mesangial glomerulonephritis with focal segmental glomerulosclerosis. At the time of cyclophosphamide and prednisone treatment, she developed a prolonged febrile syndrome. She also had severe anemia following an aplastic crisis induced by human parvovirus B19 infection and acute renal failure secondary to a severe tubulointersticial disease. Bone marrow and renal tissue, tested by polimerase chain reaction were positive for parvovirus, while the patient's blood was negative. The renal involvement did not improve requiring chronic dialysis support. We believe that the initial glomerular disease could have been due to a parvovirus infection followed by un unexpected acute tubular interstitial nephritis, rapidly progressing to chronic renal disease. This case represents, to our knowledge, the first time that a direct relationship between parvovirus infection and acute tubulointerstitial disease has been demonstrated.

[Hemolytic uremic syndrome. Treatment of secondary glomerulopathy]. / Síndrome urémico hemolítico. Tratamiento de la glomerulopatía secundaria.

Chronic renal failure (CRF) is the most severe complication of hemolytic uremic syndrome (HUS). In 1996, the histological sequence of changes in patients with long lasting oligoanuric periods was clarified. In the last years different therapeutic schemes have been proposed in order to slacken the development of terminal CRF in different renal conditions secondary to diabetes and other diseases. Some of these cases can suffer the onset of renal failure at adolescence. In this review, response to two treatment schemes in different patients with HUS and proteinuria with or without hypertension or renal failure is commented. Early indication of poor sodium diet and strict control of protein intake at the very moment of hospital discharge is strongly recommended, as well as angiotensin II conversion inhibiting enzymes (iACE) at the appearance of proteinuria.

Nefritis tubulo intersticial asociada a parvovirus B19. / [Tubulointerstitial nephritis associated with parvovirus beta19 infection]

A previously healthy 9 year old girl developed nephrotic syndrome with hypertension, microhematuria and normal renal function. The patient evolved as steroid resistant nephrotic syndrome whose initial renal biopsy was consistent with diffuse proliferative mesangial glomerulonephritis with focal segmental glomerulosclerosis. At the time of cyclophosphamide and prednisone treatment, she developed a prolonged febrile syndrome. She also had severe anemia following an aplastic crisis induced by human parvovirus B19 infection and acute renal failure secondary to a severe tubulointersticial disease. Bone marrow and renal tissue, tested by polimerase chain reaction were positive for parvovirus, while the patients blood was negative. The renal involvement did not improve requiring chronic dialysis support. We believe that the initial glomerular disease could have been due to a parvovirus infection followed by un unexpected acute tubular interstitial nephritis, rapidly progressing to chronic renal disease. This case represents, to our knowledge, the first time that a direct relationship between parvovirus infection and acute tubulointerstitial disease has been demonstrated.

Síndrome urémico hemolítico. Tratamiento de la glomerulopatía secundaria. / [Hemolytic uremic syndrome. Treatment of secondary glomerulopathy]

Chronic renal failure (CRF) is the most severe complication of hemolytic uremic syndrome (HUS). In 1996, the histological sequence of changes in patients with long lasting oligoanuric periods was clarified. In the last years different therapeutic schemes have been proposed in order to slacken the development of terminal CRF in different renal conditions secondary to diabetes and other diseases. Some of these cases can suffer the onset of renal failure at adolescence. In this review, response to two treatment schemes in different patients with HUS and proteinuria with or without hypertension or renal failure is commented. Early indication of poor sodium diet and strict control of protein intake at the very moment of hospital discharge is strongly recommended, as well as angiotensin II conversion inhibiting enzymes (iACE) at the appearance of proteinuria.

Nefritis tubulointersticial por estreptococo hemolítico del grupo A. Causa infrecuente de insuficiencia renal aguda en edad pediátrica / Tubele-interstitial nephritis due to Group A b-hemolytic Streptococcus

La nefritis tubulointersticial en la edad pediátrica es una causa poco frecuente de insuficiencia renal aguda. Presentamos a una niña de 2 años de edad que presentó insuficiencia renal aguda oligoanúrica en el contexto de una enfermedad invasiva por estreptococo ß-hemolítico del grupo A, con requerimiento de diálisis peritoneal para el manejo de la insuficiencia renal aguda. Mediante el aislamiento del estreptococo ß-hemolítico en el hemocultivo se hizo el diagnóstico de la enfermedad invasiva. La biopsia renal en la etapa aguda mostró un infiltrado intersticial linfocitario, característico de la nefritis tubulointersticial aguda. El tratamiento médico de sostén y dialítico se acompañó de recuperación de la filtración glomerular, no así de la función tubular, la que aún sigue comprometida a los 3 meses del episodio agudo  

Nefritis tubulointersticial por estreptococo ß-hemolítico del grupo A. Causa infrecuente de insuficiencia renal aguda en edad pediátrica / Tubele-interstitial nephritis due to Group A b-hemolytic Streptococcus

La nefritis tubulointersticial en la edad pediátrica es una causa poco frecuente de insuficiencia renal aguda. Presentamos a una niña de 2 años de edad que presentó insuficiencia renal aguda oligoanúrica en el contexto de una enfermedad invasiva por estreptococo ß-hemolítico del grupo A, con requerimiento de diálisis peritoneal para el manejo de la insuficiencia renal aguda. Mediante el aislamiento del estreptococo ß-hemolítico en el hemocultivo se hizo el diagnóstico de la enfermedad invasiva. La biopsia renal en la etapa aguda mostró un infiltrado intersticial linfocitario, característico de la nefritis tubulointersticial aguda. El tratamiento médico de sostén y dialítico se acompañó de recuperación de la filtración glomerular, no así de la función tubular, la que aún sigue comprometida a los 3 meses del episodio agudo ÿ

Rapid onset of protection following vaccination of calves with multivalent vaccines containing modified-live or modified-live and killed BHV-1 is associated with virus-specific interferon gamma production.

The objective of this study was to determine the effect of vaccination with commercially-available multivalent vaccines containing either modified-live (MLV) bovine herpesvirus-1 (BHV-1) (Bovishield) or MLV plus killed (MLV + K) BHV-1 (Reliant Plus) on protection against challenge at 5 days after a single vaccination. An additional objective was to determine whether cell-mediated immunity as measured by virus-specific interferon gamma (IFN-gamma) production by peripheral blood mononuclear cells (PBMC) was associated with any early protection induced by vaccination. Clinical signs, serum neutralizing (SN) titers, and nasal virus isolation (VI) titers were also measured. The 12-16-week-old dairy cross-calves seronegative for antibodies to BHV-1 were vaccinated with a multivalent vaccine containing MLV BHV-1 (n = 19), a multivalent vaccine containing MLV + K BHV-1 (n = 19), or a control multivalent vaccine not containing BHV-1 (n = 10) on day 0 and challenged intranasally on day 5. PBMC were isolated on days 0, 3, 5, 8, 10, 14 and 19. PBMC were incubated in vitro with spent media, live BHV-1, or heat-inactivated BHV-1 for 72 h. Supernatants were assayed for bovine IFN-gamma by ELISA. Bovine herpesvirus-1-specific IFN-gamma production was expressed as percent of the kit positive control, with value for spent media subtracted. Clinical signs were monitored daily. Serum VN titers were measured on days 0-5 and 19. Nasal VI titer was measured every other day from days 5 to 19. Interferon gamma production was higher on day 5, and was significantly increased post-challenge, in both vaccine groups compared to controls. There was no difference between vaccine groups on any day. There was no significant difference in SN titer among groups on any day. Virus isolation titer was significantly higher in controls on days 6 and 8 compared to both vaccine groups. Temperatures were significantly higher and nasal discharge was present more often post-challenge in controls compared to vaccine groups. Vaccination 5 days prior to challenge with commercially-available vaccine containing MLV or MLV + K BHV-1 was associated with increased BHV-1-specific IFN-gamma production, decreased viral shedding, lower temperatures and less nasal discharge post-challenge. Cell mediated immune responses as measured by IFN-gamma production are stimulated rapidly following BHV-1 vaccination of calves.

Acute renal failure in typical Kawasaki disease.

Few cases of Kawasaki disease with acute renal failure have been described and only three articles report histological findings. We present an 8-year-old boy with typical Kawasaki disease and acute renal failure who did not require dialysis and had a complete recovery. Pathological findings in percutaneous biopsy included tubulointerstitial nephropathy with mild mesangial expansion, without vessel involvement or deposits in basal membrane. These findings were similar to those previously reported. We also detected apoptotic bodies in tubules.

Trisomy 3 in two paediatric post-transplant lymphomas.

Few cytogenetic data are available concerning the chromosomal constitution of post-transplant lymphomas. We report two paediatric cases of trisomy 3, as a primary anomaly, in post-transplant lymphoproliferative disease (PTLD) associated with B immunophenotype. Using cytogenetic analysis and fluorescence in situ hybridization on chromosome preparations, we found trisomy 3 in both patients and an extra X chromosome in one. Clinical, histological and immunophenotypical data are presented. Trisomy 3 has been observed in different types of non-Hodgkin's lymphomas but it is relatively rare in B-cell lymphomas, with the exception of marginal zone lymphoma and mantle cell lymphoma. To our knowledge, trisomy 3 is an uncommon cytogenetic finding in PTLD. Further cytogenetic studies of these lymphoproliferative disorders might contribute to evaluate the role of these chromosomal anomalies in the pathogenesis of this disease.

Tyrosine kinase c-Src constitutes a bridge between cystic fibrosis transmembrane regulator channel failure and MUC1 overexpression in cystic fibrosis.

Cystic fibrosis (CF), a disease caused by mutations in the cystic fibrosis transmembrane regulator (CFTR) chloride channel, is associated in the respiratory system with the accumulation of mucus and impaired lung function. The role of the CFTR channel in the regulation of the intracellular pathways that determine the overexpression of mucin genes is unknown. Using differential display, we have observed the differential expression of several mRNAs that may correspond to putative CFTR-dependent genes. One of these mRNAs was further characterized, and it corresponds to the tyrosine kinase c-Src. Additional results suggest that c-Src is a central element in the pathway connecting the CFTR channel with MUC1 overexpression and that the overexpression of mucins is a primary response to CFTR malfunction in cystic fibrosis, which occurs even in the absence of bacterial infection.

Síndrome urémico hemolítico: estudio anatomoclínico en 59 pacientes / Hemolytic-uremic syndrome: a pathologic-clinical study of 59 patients

Nuestro objeto es analizar las lesiones renales y extrarrenales del SUH y correlacionarlas con los datos clínicos en una serie (la mayor publicada) de 59 niños muertos dentro de las 5 semanas de evolución, todos autopsiados, con examen del encéfalo en 29. Su edad media fue l6,5 meses (5 á 72); 56 comenzaron con diarrea (forma clásica) y en 3 el pródromo fue respiratorio. El fallo renal fue grave, con más de 14 días de oligoanuria en 57 por ciento; todos tenían microangiopatía trombótica (MAT) glomerular, frecuentemente asociada a MAT arterial (n:28) y/o necrosis cortical (N:38). En la forma clásica hubo signos digestivos severos; en la mitad había necrosis colónica con MAT; requirió cirugía en 9. El 95 por ciento tuvo signos neurológicos, severos en la mayoría, con tendencia a persistir y agravarse y hemorragias retinianas en 68 por ciento; las lesiones cerebrales fueron hemorrágicas en la mayoría, y de MAT en 33 por ciento, frecuente en plexos coroideos. En algunos hubo disfunción cardíaca con signos de isquemia; en 30 por ciento se halló MAT miocárdica, mortal en 5. En páncreas había MAT selectiva en los islotes de Langerhans, causa en uno de hiperglucemia intensa, y de pancreatitis fatal en el otro. Los 3 con formas respiratorias tuvieron igual daño renal, neumonías virales letales y ausencia de MAT extrarrenal. Conclusiones: el daño extrarrenal es importante en la morbilidad y mortalidad de la fase aguda del SUH. Su lesión principal es la MAT, vinculada al daño endotelial de la verotoxina. (AU)

Síndrome urémico hemolítico: estudio anatomoclínico en 59 pacientes / Hemolytic-uremic syndrome: a pathologic-clinical study of 59 patients

Nuestro objeto es analizar las lesiones renales y extrarrenales del SUH y correlacionarlas con los datos clínicos en una serie (la mayor publicada) de 59 niños muertos dentro de las 5 semanas de evolución, todos autopsiados, con examen del encéfalo en 29. Su edad media fue l6,5 meses (5 á 72); 56 comenzaron con diarrea (forma clásica) y en 3 el pródromo fue respiratorio. El fallo renal fue grave, con más de 14 días de oligoanuria en 57 por ciento; todos tenían microangiopatía trombótica (MAT) glomerular, frecuentemente asociada a MAT arterial (n:28) y/o necrosis cortical (N:38). En la forma clásica hubo signos digestivos severos; en la mitad había necrosis colónica con MAT; requirió cirugía en 9. El 95 por ciento tuvo signos neurológicos, severos en la mayoría, con tendencia a persistir y agravarse y hemorragias retinianas en 68 por ciento; las lesiones cerebrales fueron hemorrágicas en la mayoría, y de MAT en 33 por ciento, frecuente en plexos coroideos. En algunos hubo disfunción cardíaca con signos de isquemia; en 30 por ciento se halló MAT miocárdica, mortal en 5. En páncreas había MAT selectiva en los islotes de Langerhans, causa en uno de hiperglucemia intensa, y de pancreatitis fatal en el otro. Los 3 con formas respiratorias tuvieron igual daño renal, neumonías virales letales y ausencia de MAT extrarrenal. Conclusiones: el daño extrarrenal es importante en la morbilidad y mortalidad de la fase aguda del SUH. Su lesión principal es la MAT, vinculada al daño endotelial de la verotoxina.