Nephrotic syndrome and AA amyloidosis revealing adult-onset cryopyrin-associated periodic syndrome.

Cryopyrin-associated periodic syndrome (CAPS) is due to gain-of-function mutations in the cryopyrin gene, which determines an overactive inflammatory response. AA amyloidosis is a complication of this syndrome. A 53-year-old man was referred to us because of lower limb edema. Past history: at the age of 20, he complained of arthralgia/arthritis and bilateral hypoacusis. At the age of 35, he presented posterior uveitis, several episodes of conjunctivitis, and progressive loss of visual acuity. Laboratory tests disclosed nephrotic syndrome, and renal biopsy showed AA amyloidosis. He was given anakinra with improvement of arthritis. A genetic study revealed the p.D303N mutation in the cryopyrin gene, and he was diagnosed as having AA amyloidosis due to CAPS. Twenty-one months after starting anakinra, the arthritis has disappeared, although nephrotic-range proteinuria persisted. It is important to be aware of cryopyrin-associated periodic syndrome because it can cause irreversible complications, and there is effective therapy.

Tocilizumab treatment for nephrotic syndrome due to amyloidosis in Behcet's disease.

Renal involvement is an unusual but significant Behcet´s disease (BD) complication and AA amyloidosis appears to be the most common etiology. IL-6 is a pro-inflammatory cytokine with an important role in AA amyloidosis development. Tocilizumab (TCZ) is a humanized anti-IL-6 receptor antibody that has emerged as an effective and specific treatment in AA amyloidosis secondary to chronic inflammatory disorders. We report on a patient diagnosed with BD who developed nephrotic syndrome caused by renal AA amyloidosis with an excellent response to TCZ therapy.

Imipenem-induced Clostridium difficile diarrhea in a patient with chronic renal failure.

An 80-year-old man was diagnosed to have pneumonia and advanced chronic kidney disease. He presented with anuria and hemodialysis, by temporary femoral catheter, was initiated. He was empirically treated with imipenem/cilastatin 500 mg/24 h after hemodialysis. After 10 days of antibiotic intake, he developed severe diarrhea. Diagnosis of Clostridium difficile (CD)-associated diarrhea was confirmed by detection of the toxins A and B in his stool. Imipenem therapy was discontinued; Vancomycin 500 mg orally every 6 h and 1000 mg per rectum every day was added. After two weeks of this treatment, the patient reported complete resolution of the diarrhea and stool samples were negative for Clostridium toxin. In this case, the most possible cause of CD colitis was considered to be imipenem because of the temporal relationship between exposure to the drug and onset of symptoms.

Gitelman syndrome due to p.A204T mutation in CLCNKB gene.

A 45-year-old woman presented with phenotypical features suggestive of Gitelman syndrome (adult age at diagnosis, normal-low blood pressure, hypokalaemia, metabolic alkalosis, hypomagnesaemia, and hypocalciuria). Mutational analysis revealed no significant abnormality in SLC12A3 gene, but homozygous p.A204T mutation was found in the CLCNKB gene. This is a founder effect mutation described in Spanish patients with classic and atypical Bartter syndrome. This report confirms previous descriptions and expands the clinical spectrum of this mutation.

Cryoglobulinemic glomerulonephritis in chronic hepatitis B infection.

Hepatitis B virus (HBV) infection is an uncommon cause of cryoglobulinemia. Renal cryoglobulinemia has been rarely reported in the setting of chronic hepatitis B infection. We describe a case of chronic hepatitis B infection with cryoglobulinemic glomerulonephritis (Gn) and provide information about the treatment and the evolution over a 30-month follow-up. A 41-year-old woman with chronic hepatitis B infection developed nephrotic syndrome and acute renal failure; other investigations revealed type 2 cryoglobulinemia; HBV DNA was detected in the cryoprecipitate. Renal biopsy showed findings of cryoglobulinemic Gn. She was given lamivudine, corticosteroids, plasma exchange, and mycophenolate mofetil. The renal function improved, nephrotic syndrome remitted, and HBV DNA became undetectable; there was no compromise of the liver function.

Obstructive nephropathy secondary to AA-type amyloidosis in ankylosing spondylitis.

AA-type amyloidosis of the genitourinary tract is a rare phenomenon and few cases are described in the literature. We report a 42-year-old man with ankylosing spondylitis, who developed hematuria, bilateral hydronephrosis, and renal failure caused by AA amyloidosis.

Remission of minimal change disease in Type 2 diabetes after streptococcus bacteremia.

A 41-year-old man with Type 2 diabetes developed sudden onset of nephrotic syndrome. He initially refused a renal biopsy. However, 3 months later, the nephrotic syndrome persisted and percutaneous renal biopsy was performed. The study with light microscopy, immunofluorescence and electron microscopy showed minimal change disease. Three weeks after biopsy, before immunosuppressive therapy was begun, the patient presented Group A Streptococcus (GAS) bacteremia and acute renal failure which needed hemodialysis. Afterwards, the renal function recovered and complete remission of the nephrotic syndrome, maintained during a 22-month follow-up, was observed. We discuss the possible mechanisms implicated in the remission. This report extends the spectrum of infections associated with remission of minimal change disease (MCD).

Severe renal complications in chronic myelomonocytic leukemia.

A 56-year-old man presented with peripheral monocytosis and massive nephrotic syndrome. He was diagnosed as having chronic myelomonocytic leukemia and membranous glomerulonephritis. He received prednisone, chlorambucil and hydroxyurea, but the nephrotic syndrome persisted. Seven months after diagnosis, he was started on cyclosporin A; 1 month later he developed acute renal failure due to radiolucent bilateral renoureteral stones. His kidney function recovered after placing ureteral catheters and urine alkalization. Afterward, he was given mycophenolate mofetil, and proteinuria decreased to subnephrotic levels (1 g/24 hours). This case highlights 2 severe renal complications in this type of leukemia. To the best of our knowledge, there are only 2 previous cases of glomerulonephritis, histologically proven, associated with chronic myelomonocytic leukemia. On the other hand, reversible acute renal failure due to radiolucent bilateral renoureteral stones has never been reported. Also, as far as we know, mycophenolate mofetil was successfully used here for the first time for treating glomerulonephritis-related chronic myelomonocytic leukemia.

Goodpasture's syndrome in a patient using cocaine--a case report and review of the literature.

Glomerulonephritis is a very rare form of cocaine-induced renal pathology. We report a 26-year-old woman having inhaled cocaine, who presented with antiglomerular basement membrane glomerulonephritis and pulmonary hemorrhage. She was treated with immunosuppressive therapy and plasmapheresis. Maintenance hemodialysis was required on discharge. We stress the importance of early detection and treatment of this renal association for the potential fatal consequences implicated.

Autosomal dominant polycystic kidney disease with congenital absence of contralateral kidney.

Autosomal dominant polycystic kidney disease is the most frequent hereditary kidney disorder, accounting for 8-10% of the cases of end-stage renal disease. It is characterized by bilateral multiple renal cysts, nevertheless, asymmetric enlargement of the kidneys is frequently observed, and this can lead to diagnostic confusion. We report the rare occurrence of autosomal dominant polycystic disease confined to a right kidney and congenital absence of the contralateral one. Unexpected early onset of terminal renal failure in this hypertensive 23-year-old male is discussed with the review of the literature.

[Hypersensitivity reaction to systemic corticosteroids in a hemodialysis patients]. / Reacción de hipersensibilidad por corticoides sistémicos en una paciente en hemodiálisis.

Hypersensitivity reactions are a feared complication of hemodialysis considering their potential for a serious and eventually fatal course. This association could reflect a number of different exposures, including concomitant medications. Like this, systemic steroid-induced hypersensitivity reactions do occur, althought their incidence is extremely rare. A few reactions related to metylprednisolone have been reported in renal transplantation. The diagnosis could be confirmed by the patient history and performing skin testing, oral or parenteral challenge or by the evidence of specific IgE. We report on a 39-year-old woman undergoing dialysis therapy in whom severe bronchospasm was induced by corticosteroids. She has a positive systemic reaction on the oral steroid challenge test. A successful desensitization regimen for safe administration was achieved. The recognition of high-risk subjects and alternative therapeutic approach are critical, mainly in the management of patients while receiving corticosteroids or a renal allograft.

¿Se siguen las normas de prevención de la insuficiencia renal en los pacientes tratados con IECA/ARA II? / No disponible

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Reacción de hipersensibilidad por corticoides sistémicos en una paciente en hemodiálisis / Hypersensitivity reaction to systemic corticosteroids in a hemodialysis patient

Las reacciones de hipersensibilidad que ocurren durante la hemodiálisis son unacomplicación de curso potencialmente fatal. Se describen asociadas a una seriede factores que incluyen la administración concomitante de medicación. Por otraparte y aunque se consideran raras, se han comunicado reacciones de hipersensibilidada corticoides sistémicos. Algunos casos, en relación a metilprednisolona,se han evidenciado en la inducción del trasplante renal. El diagnóstico se confirmaen base a la historia clínica y a tests cutáneos y de provocación oral o parenteral,o incluso por la identificación de IgE específicas. Describimos el caso deuna mujer de 39 años en hemodiálisis de mantenimiento que presentaba reaccionesde hipersensibilidad inducidas por esteroides demostradas por la pruebade provocación oral. En la enferma se logró inducir tolerancia a metilprednisolonamediante un régimen de desensibilización. La identificación de hipersensibilidada esteroides sistémicos es necesaria en pacientes que los precisen como terapiade mantenimiento o para un futuro trasplante renal

Hypersensitivity reactions are a feared complication of hemodialysis consideringtheir potential for a serious and eventually fatal course. This association could reflecta number of different exposures, including concomitant medications. Like this,systemic steroid-induced hypersensitivity reactions do occur, althought their incidenceis extremely rare. A few reactions related to metylprednisolone have beenreported in renal transplantation. The diagnosis could be confirmed by the patient history and performing skin testing, oral or parenteral challenge or by the evienceof specific IgE. We report on a 39-year-old woman undergoing dialysis therapyin whom severe bronchospasm was induced by corticosteroids. She has a positivesystemic reaction on the oral steroid challenge test. A successful desensitizationregimen for safe administration was achieved. The recognition of high-risk subjectsand alternative therapeutic approach are critical, mainly in the managementof patients while receiving corticosteroids or a renal allograft