Renal candidiasis associated with papillary necrosis in a captive tiger (Panthera tigris).

A 14-year-old male tiger developed anorexia with elevated blood urea nitrogen and creatinine levels. The patient had a palpable abdominal mass and demonstrated neutrophilic leukocytosis and anaemia. Leukocytes, yeast and bacteria were present in the urine. The animal was non-responsive to therapy and was subsequently euthanised. Extensive acute renal papillary necrosis (RPN) with pyelonephritis, chronic nephritis and polycystic renal disease were evident during gross and microscopic pathology examinations. The histologic occurrence of fungal spores and pseudohyphae morphologically consistent with Candida species were observed within the necrotic papillary regions of the kidney and within multiple foci of mild parakeratotic hyperkeratosis present in the gingiva and tongue. Candida albicans along with a slight growth of Escherichia coli were recovered from kidney cultures. Possible contributory factors for the renal candidiasis and associated RPN include predisposing oral candidiasis, polycystic renal disease, ischaemic nephrosclerosis, age-associated or other forms of immunodeficiency and therapy with meloxicam, a non-steroidal anti-inflammatory drug. The absence of apparent lower urinary tract involvement coupled with the presence of intravascular renal 'Candida emboli' suggest that chronic oral candidiasis was the probable source of the kidney infection.

[Sickle cell trait complications: A case series of 6 patients]. / Complications du trait drépanocytaire : à propos d'une série de 6 cas.

INTRODUCTION: Patients with sickle cell trait (SCT) are commonly considered as asymptomatic carriers. However, some clinical manifestations may occur. METHODS: Here we present a retrospective descriptive study about SCT subjects with at least one complication diagnosed in a sickle cell disease referral center, in Paris, between 2008 and 2019. We also performed a literature review on the complications of SCT subjects. RESULTS: Six patients (between 19 and 65 years old) were included. SCT was already known only for 4 of them at the time of the complication. Four patients presented with a splenic infarct after a stay in high altitude or a plane trip, one of them was associated with papillary necrosis; one patient had isolated papillary necrosis, and the last one had splenic sequestration. These complications happened for most of them after exposure to an unusual situation of hypoxia or deshydratation. Five out of 6 patients had a marked elevated C reactive protein. CONCLUSION: SCT may cause acute ischemic complications in a context of prolonged hypoxia or dehydration. The most commonly reported are the splenic infarct and the renal papillary necrosis. A study of hemoglobin should be considered in these clinical situations in patients with compatible ethnic origin.

Renal papillary necrosis in patients with sickle cell disease: How to recognize this 'forgotten' diagnosis.

INTRODUCTION: Renal papillary necrosis is not commonly seen in daily practice, but can have severe consequences when it is not diagnosed in time. It is known to be associated with sickle cell hemoglobinopathies; however a wide range of etiologies are possible, and it is therefore not the first diagnosis clinicians consider in patients with sickle cell disease who present with hematuria. METHODS: A literature search was performed to summarize the current knowledge about renal papillary necrosis associated with sickle cell disease. These findings are illustrated with a case of a 9-year old girl with sickle cell disease who was referred with painless gross hematuria. RESULTS: Typical radiologic signs for renal papillary necrosis are necrotic cavities that fill with contrast, small collections of contrast peripheral to the calyces in the papillary region (ball-on-tee sign), calcification of the papillary defect, filling defects, hydronephrosis, blunted papillary tip, clefts in the renal medulla filled with contrast, hyperattenuated medullary calcifications, non-enhanced lesions surrounded by rings of excreted contrast, and clubbed calyces. DISCUSSION: This study focuses on the pathophysiology of renal papillary necrosis associated with sickle cell disease, the possible symptoms, as well as the diagnostic steps, with a special interest in particular presentation on old (retrograde pyelography) and new (computed tomography) gold standard in radiologic imaging, and the management for this pathology. CONCLUSION: This study aims to remind clinicians of this "forgotten" diagnosis and what signs to look for in pediatric patients with sickle cell disease who present with hematuria. In pediatric cases radiation protection is important, therefore knowing what radiologic signs can be found on retrograde pyelography can lead to early identification of this pathology without having to proceed to computed tomography.

Genitourinary manifestations of sickle cell disease.

Sickle cell disease is a common genetic disorder characterized by sickling of red blood cells under conditions of reduced oxygen tension. In turn, sickling leads to intravascular hemolysis and vaso-occlusive events with subsequent tissue ischemia-reperfusion injury affecting multiple organs, including the genitourinary system. Our review of the genitourinary manifestations of sickle cell disease focuses on sickle cell nephropathy, priapism, and other genitourinary complications such as papillary necrosis and renal medullary carcinoma.

Renal actinomycosis.

Actinomycosis of the kidney is rare and less than 50 cases have been reported in the English literature. Reported presentations are pyelonephritis, renal abscesses or pyonephrosis. To date, one case of actinomycosis associated necrotising papillitis has been reported. We describe the second case of such a rare association of actinomycosis with papillary necrosis.

An update on sickle cell nephropathy.

Sickle cell disease (SCD) is a major health problem in many countries. Sickle cell nephropathy (SCN) is now a well-characterized entity with specific manifestations, risk factors and prognosis. The presence of sickled erythrocytes in the renal medullary vessels is the hallmark of the disease with a variety of renal complications. Renal manifestations of SCD include renal ischemia, microinfarcts, renal papillary necrosis and renal tubular abnormalities with variable clinical presentations. Proximal tubule dysfunction generally impairs urinary concentration, while more distal tubule dysfunction may impair potassium excretion, leading to hyperkalemia. Glomerular disease with proteinuria may develop due to ischemia and results in a compensatory increase in the renal blood flow and glomerular filtration rate; such hyperfiltration, combined with glomerular hypertrophy, probably contributes to glomerulosclerosis. Acute and chronic kidney disease are the expected outcomes of the disease. Both dialysis and kidney transplantation are effective renal replacement therapies for end-stage renal disease due to SCN, with a higher advantage for transplantation. Whether bone marrow transplantation in the early stage of the disease can halt the progression of SCN is unknown and awaits clinical studies.

[Spectrum of renal manifestations in sickle cell disease]. / Atteintes rénales au cours de la drépanocytose.

Sickle cell disease (SCD), the most common hemoglobinopathy, is an increasing cause of chronic kidney disease. In the last decade, we have witnessed a better understanding in the characterization of clinical manifestations and pathogenesis of sickle cell nephropathy. The spectrum of renal diseases during SCD includes various renal manifestations such as impairment of urinary concentrating ability, defect in urine acidification, renal papillary necrosis and proteinuria related to glomerular injury leading to progressive end-stage renal disease. Endothelial dysfunction related to chronic hemolysis and the relative renal hypoxia caused by vaso-occlusive sickle red blood cells are probably two key factors for SCN development. Optimal therapeutic management (including the use of blockers of the renin-angiotensin system) of patients with proteinuria remains to be determined. Renal replacement therapy with dialysis is required in SCD patients with end-stage renal disease but these patients should probably undergo kidney transplantation that requires careful management.

Early allograft calcifications after kidney transplantation.

Early allograft calcifications after kidney transplantation (KT) have already been reported, but the clinical implications of this finding are not clear thus far. Patient-related factors such as age, gender, underlying renal disease, and dialytic modality, seem to be irrelevant. It has been postulated that factors promoting the development of metastatic calcifications, including elevated calcium phosphate product and severe secondary hyperparathyroidism, could play a causal role. Here we report a case of a KT patient who developed early kidney calcifications which were associated with severe allograft dysfunction.

Afectación renal en la enfermedad falciforme / Kidney abnormalities in sickle cell disease

La enfermedad falciforme (EF) es la hemoglobinopatía estructural más frecuente en el mundo. Su afectación renal clínicamente significativa ocurre más frecuentemente en los individuos homocigotos que en los heterocigotos o con hemoglobinopatías mixtas, con la excepción del carcinoma medular renal, que es más frecuente en los portadores del rasgo falciforme. Como en otras manifestaciones de la EF, los fenómenos vasooculsivos son el principal mecanismo etiopatogénico. Sus manifestaciones más frecuentes son la hematuria macroscópica asintomática, las alteraciones funcionales tubulares y la necrosis papilar. Menos frecuentes, pero de peor pronóstico, son la glomerulopatía que conduce a enfermedad renal crónica y el carcinoma medular renal. Debido a la alta tasa de inmigración en España y a una mayor supervivencia de estos pacientes se espera una prevalencia creciente en nuestro país. En 2009, la Asociación Española de Hematología y Hemoterapia elaboró las Guías de manejo de la enfermedad falciforme. Este artículo reproduce el capítulo sobre afectación renal actualizado para su difusión en NEFROLOGÍA (AU)

Patients with sickle cell disease exhibits numerous kidney structural and functional abnormalities, changes that are seen along the entire length of the nephron. Changes are most marked in patients with homozygous sickle cell anemia, but are also seen in those with compound heterozygous states and the sickle cell trait. The renal features of sickle cell disease include some of the most common reasons for referral to nephrologists, such as hematuria, proteinuria, tubular disturbances and chronic kidney disease. Therapy of these conditions requires specialized knowledge of their distinct pathogenic mechanisms. Spanish Haemathology and Hemotherapy Association has recently publicated their Clinical Practice Guidelines of SCD management. Renal chapter is reproduced in this article for Nephrology difussion (AU)

[Co-inheritance of autosomal dominant polycystic kidney disease and sickle cell trait in African Americans]. / Co-herencia de poliquistosis renal autosómica dominante y hemoglobina con rasgo falciforme en afroamericanos.

BACKGROUND: Macroscopic haematuria secondary to renal cyst rupture is a frequent complication in autosomal dominant polycystic kidney disease (ADPKD). Sickle-cell disease is an autosomal recessive haemoglobinopathy that involves a qualitative anomaly of haemoglobin due to substitution of valine for the glutamic acid in the sixth position of 3-globin gene on the short arm of chromosome 11. For the full disease to be manifested, this mutation must be present on both inherited alleles. The severity of the disease is proportional to the quantity of haemoglobin S (Hb S) in the red cells; sickle-cell trait (Hb S <50%) and homozygous sickle-cell disease (Hb S >75%). In sickle-cell disease, the abnormal Hb S loses its rheological characteristics and is responsible of the various systemic manifestations including those of the kidney, such as macroscopic haematuria secondary to papilar necrosis. Despite the generally benign nature of the sickle-cell trait, several potentially serious complications have been described. Metabolic or environmental changes such as hypoxia, acidosis, dehydration, hyperosmolality or hyperthermia may transform silent sickle-cell trait into a syndrome resembling sickle-cell disease with vaso-occlusive crisis due to an accumulation of low deformable red blood cells in the microcirculation originating haematuria from papilar necrosis. On the other hand, it has been demonstrated an earlier onset of end-stage renal disease (ESRD), in blacks with ADPKD and sickle-cell trait when compared with blacks with ADPKD without the trait. PATIENTS AND METHODS: We studied 2 african-american families (4 patients) which presented with both ADPKD and sickle-cell trait (Hb S <50%). The diagnosis of sickle-cell trait was confirmed by haemoglobin electrophoresis. The renal volume was measured by magnetic resonance imaging (MRI). RESULTS: The proband subject in family 1 presented frequent haematuria episodes, associated to increase of renal volume, developed very early ESRD and was dialyzed at the age of 39 years. The other 3 patients in family 2 presented different degree of renal function. CONCLUSIONS: The presence of sickle haemoglobin should be determined in african-american and west-african patients with ADPKD because it is an important prognostic factor. Coherence of sickle-cell trait may have influence on ADPKD evolution to ESRD and other complications, such as cystic haemorrhages. MRI can identify intracystic haemorrhage and permit renal volume measure.

Complications associated with sickle cell trait: a brief narrative review.

Sickle cell trait occurs in approximately 300 million people worldwide, with the highest prevalence of approximately 30% to 40% in sub-Saharan Africa. Long considered a benign carrier state with relative protection against severe malaria, sickle cell trait occasionally can be associated with significant morbidity and mortality. Sickle cell trait is exclusively associated with rare but often fatal renal medullary cancer. Current cumulative evidence is convincing for associations with hematuria, renal papillary necrosis, hyposthenuria, splenic infarction, exertional rhabdomyolysis, and exercise-related sudden death. Sickle cell trait is probably associated with complicated hyphema, venous thromboembolic events, fetal loss, neonatal deaths, and preeclampsia, and possibly associated with acute chest syndrome, asymptomatic bacteriuria, and anemia in pregnancy. There is insufficient evidence to suggest an independent association with retinopathy, cholelithiasis, priapism, leg ulcers, liver necrosis, avascular necrosis of the femoral head, and stroke. Despite these associations, the average life span of individuals with sickle cell trait is similar to that of the general population. Nonetheless, given the large number of people with sickle cell trait, it is important that physicians be aware of these associations.

Nuevo posicionamiento para realizar la pieloplastia laparoscópica. Nuestra experiencia / New position for laparoscopic pyeloplasty. Our experience

Objetivo: Nuestra intención con el presente artículo pretende compartir nuestra experiencia en relación con la realización de la pieloplastia laparoscópica y nuestras aportaciones en este sentido. Métodos: Hemos realizado 12 pielopastias laparoscópicas a otros tantos pacientes entre marzo del 2004 y enero 2006. A lo largo de nuestra serie hemos ido codificando la técnica quirúrgica a medida que nos hemos ido encontrando con dificultades durante la realización de la misma. Mediante la única modificación del posicionamiento del paciente hemos logrado una importante mejora en nuestra técnica. Resultados: Describimos en este artículo como realizábamos la intervención en nuestros primeros casos y de que forma estamos realizándola en este momento, gracias al nuevo posicionamiento. Asímismo damos cuenta de cuales son las ventajas que apreciamos. Conclusiones: Con nuestra técnica logramos una importante reducción en el tiempo quirúrgico, mejoras en cuanto a seguridad de la técnica y reducción de las complicaciones quirúrgicas (AU)

Objectives: To share our experience performing laparoscopic pyeloplasty and our contributions to this surgery. Methods: Between March 2004 and January 2006 we have performed 12 laparoscopic pyeloplasties in 12 patients. We modified our technique as we found difficulties during operations. By the only modification of patient position we have achieved a significant improve in our technique. Results: We describe how we performed the operation in the first cases and how we do it today, with the new position. We also describe the advantages observed. Conclusions: With our technique we achieve an important surgical time reduction, improvements in safety and reduction of surgical complications (AU)

Acute renal papillary necrosis with complete bilateral ureteral obstruction in a child.

A 9-year-old girl presented with apparent meningococcal septicemia and developed acute renal failure after 48 hours of treatment with antibiotics and analgesics. Early ultrasound scanning demonstrated mild bilateral hydronephrosis and hydroureter. Intravenous urography showed slow contrast uptake with delay nephrogram and no contrast entering the bladder. Repeat ultrasonography revealed bilateral papillary irregularity and echogenic debris in the distal ureters. Bilateral double-J stents were inserted cystoscopically, resulting in prompt polyuria and a return of normal renal function. Although rare, recognition of sloughed papilla in papillary necrosis causing ureteral obstruction can lead to early management with no long-term sequelae.

Pielonefritis aguda en la gestación / Acute pyelonephritis in pregnancy

La pielonefritis aguda es la complicación médicagrave mas común durante la segunda mitaddel embarazo. La presencia de una bacteriuriaasintomática no diagnosticada en el primertrimestre de gestación es un factor predisponentede primer orden. Los cambios anatómicosy funcionales que el embarazo y su ambientehormonal ocasionan sobre el aparato urinariotambién facilitan el desarrollo de infeccionesurinarias y potenciales pielonefritis.Las pielonefritis agudas no complicadas de lamujer gestante están mayoritariamente ocasionadaspor enterobacterias gram negativas queascienden desde el tracto urinario inferior.Un diagnóstico certero, fundamentado en parámetrosclínicos y analíticos, junto con un eficaztratamiento antibioterápico disminuyen lascomplicaciones maternas y fetales que se ocasionanen un 20% de las pielonefritis agudasgraves

The acute pyelonephritis is the medical serious complication commonly during the second half of the pregnancy. The presence of an asymtomatic bacteriuria not diagnosed in the first trimester of gestation is a predisposition factor of the first order. The anatomical and functional changes that the pregnancy and his hormonal environment cause on the urinary device also facilitate the development of urinary infections and potential pyelonephritis. The acute pyelonephritis not complicated of the pregnancy woman are caused for the most part for negative gram enterobacterias that ascend from the lowest urinary tract. An accurate diagnosis based on clinical and analytical parameters, together with an effective antibiotic treatment they diminish the mother and foetal complications that are caused in 20% of the acute serious pyelonephritis