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.

Ultrasound based application for intraglandular mapping of breast cancer.

PURPOSE: Breast cancer is the most common malignant tumor among women worldwide. Breast cancer is one of the few cancers that can be early detected, and the survival rate of the women whose breast cancers are detected on their initial stage is virtually 100%. At the present time, ultrasound (US) is the most important imaging test together with the mammogram for the diagnostic evaluation of the breast. Recent studies have shown that ultrasound, in addition to mammography, helps doctors to spot significantly more cancers compared with mammograms alone. This work intends to standardize the process of the US breast examination, the storage and marking of the US images and their subsequent visualization and comparison. METHODS: It presents an innovative technique for the intraglandular mapping of breast cancer in a 3D scene. An anatomical based model of the breast is used for storage of the US images. Hardware equipment needed for the breast examination is described. Soft application programmed on Apple tools is fully described. The database for the storage is presented. RESULTS: First clinical applications of the presented tool are reported. Currently, the system is being distributed free of charge to clinical personal in order to evaluate its benefits. CONCLUSIONS: A first version of an application to standardize the process of the US breast examination is presented. First reports show the feasibility of the system to be applied on clinics.

Hemorragia pulmonar crónica como primera manifestación de una poliangeítis microscópica asociada a tiroiditis autoinmune / Chronic pulmonary bleeding as the first sign of microscopic polyangiitis associated with autoimmune thyroiditis

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Hepatotoxicidad tras tratamiento con ciclofosfamida en un paciente con vasculitis MPO-ANCA / Hepatotoxicity following cyclophosphamide treatment in a patient with MPO-ANCA vasculitis

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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.

[Conservative approach in major renal trauma]. / Manejo conservador de los traumatismos renales de alto grado.

INTRODUCTION: Genitourinary trauma amount to an 8-10% of abdominal trauma with the kidney being the most affected organ in 50% of cases, especially the left one. The choice of treatment will depend on the kind of lesion found in the affected renal unit and on the patient's clinical conditions. OBJECTIVE: The aims of this study are twofold: to determine the applicability of conservative treatment in major renal trauma and to assess the evaluation and emergence of possible complications. MATERIAL AND METHODS: We have analysed 309 cases of renal trauma dealt with in our department between January 1984 and January 2006, analyzing such variables as the etiology of the trauma, associated lesions in other organs, the therapeutic approach adopted as well as the presence of complications, both in the long and short run. RESULTS: Out of a total of 309 renal trauma analyzed, a 94.1% (291 cases) were blunt renal trauma. The distribution by grade was: Grade I, 213 cases (69%); Grade II, 39 cases (12.6%); Grade III/IV, 32 cases (10.3%); Grade V, 25 cases (8%). We have given a conservative approach in the 84.6% of the grade III/IV cases (24 cases). 4 grade III nephrectomies were carried out in the Emergency Room because of haemodynamic instability, other 4 grade IV nephrectomies were done for the same reason, one of which was a partial nephrectomy, 48-72 hours after the trauma. The treatment for grade V was nephrectomy in 67%. CONCLUSIONS: According to our experience and in the light of the results obtained, we consider the conservative approach adequate for major renal trauma as long as the patient is haemodynamically stable.

Manejo conservador de los traumatismos renales de alto grado / Conservative approach in major renal trauma

Introducción: Los traumatismos genitourinarios constituyen el 8-10% de los traumatismos abdominales y en el 50% de los casos es el riñón el órgano más afectado, sobretodo el izquierdo. La decisión de adoptar un tratamiento conservador o quirúrgico en estos casos dependerá del tipo de lesión que encontremos en la unidad renal afecta y de las condiciones clínicas del paciente. Objetivo: Los objetivos del estudio son determinar la posibilidad de llevar acabo un tratamiento conservador en los traumatismos renales de alto grado y estudiar la evaluación y aparición de complicaciones en los mismos. Material y métodos: Hemos analizado retrospectivamente los 309 casos de traumatismos renales acontecidos en nuestro Servicio en el período de tiempo comprendido entre Enero 1984 y Enero 2006, estudiando variables como la etiología del traumatismo, lesiones asociadas en otros órganos, actitud terapéutica adoptada y presencia de complicaciones, tanto a largo como a corto plazo. Resultados: De los 309 traumatismos renales analizados, el 94,1% (291 casos) fueron traumatismos renales cerrados o contusos. La distribución por grados del total de traumatismos fue: Grado I: 213 casos (69%), grado II: 39 casos (12.6%), grados III y IV: 32 casos (10,3%) y grado V: 25 casos (8%). Realizamos tratamiento conservador en el 84,6% de los casos etiquetados como grados III y grado IV (24 casos en total). Se practicaron 4 nefrectomías de urgencia en los grado III por inestabilidad hemodinámica y 4 nefrectomías en grados IV por el mismo motivo, una de ellas parcial, diferidas a las 48- 72 horas del traumatismo. En 67% de los traumatismos grado V se realizó nefrectomía de urgencia. Conclusiones: De acuerdo a nuestra experiencia y a los resultados obtenidos, consideramos adecuado el manejo conservador en los traumatismos renales de alto grado siempre y cuando las condiciones hemodinámicas del paciente lo permitan

Introduction: Genitourinary trauma amount to an 8-10% of abdominal trauma with the kidney being the most affected organ in 50% of cases, especially the left one. The choice of treatment will depend on the kind of lesion found in the affected renal unit and on the patient’s clinical conditions. Objective: The aims of this study are twofold: to determine the applicability of conservative treatment in major renal trauma and to assess the evaluation and emergence of possible complications. Material and methods: We have analysed 309 cases of renal trauma dealt with in our department between January 1984 and January 2006, analyzing such variables as the etiology of the trauma, associated lesions in other organs, the therapeutic approach adopted as well as the presence of complications, both in the long and short run. Results: Out of a total of 309 renal trauma analyzed, a 94,1% (291 cases) were blunt renal trauma. The distribution by grade was: Grade I, 213 cases (69%); Grade II, 39 cases (12,6%); Grade III/ IV, 32 cases (10,3%); Grade V, 25 cases (8%). We have given a conservative approach in the 84,6% of the grade III/ IV cases (24 cases). 4 grade III nephrectomies were carried out in the Emergency Room because of haemodynamic instability, other 4 grade IV nephrectomies were done for the same reason, one of which was a partial nephrectomy, 48-72 hours after the trauma. The treatment for grade V was nephrectomy in 67%. Conclusions: According to our experience and in the light of the results obtained, we consider the conservative approach adequate for major renal trauma as long as the patient is haemodynamically stable

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.

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

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