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.

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.

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.

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.

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.

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.

Acute renal failure associated with an accidental overdose of colchicine.

CASE SUMMARY: A 47-year-old man with a history of polyarticular gout was admitted to the nephrology service because of severe renal insufficiency (creatinine 6.25 mg/dl). Three days before admission he had a pain crisis in his knees and ankles and self-administered 20 x 1 mg granules of colchicine p.o. over a period of 4 - 5 hours together with six suppositories each containing 100 mg of indomethacin. The patient began vomiting within 24 hours, experienced diarrhea which persisted for three days and then came to the hospital. The patient reported oliguria during the preceding 24 hours. In hospital, attempts to correct water and electrolyte balance were initiated. The patient became stabilized hemo-dynamically, the diarrhea disappeared within 24 hours, diuresis resumed and the renal function progressively improved. Leukopenia and thrombopenia were diagnosed, the transaminases increased: AST = 79 U/l, ALT = 132 U/l on the eighth day after taking the colchicine. The serology for hepatitis A, B, C and HIV viruses was negative; the serology for CMV and VEB revealed a previous infection. After being discharged from hospital 11 days after admission, the patient presented with the following parameters: hematocrit 39%, leukocytes 5,920/microl (3 470 neutrophils), prothrombin time 13 seconds, urea 44 mg/dl, creatinine 1.29 mg/dl, AST 16 U/l and ALT 35 U/l. DISCUSSION: The patient mistakenly ingested 20 mg ofcolchicine p.o. (0.22 mg/kg). The intoxication was associated with gastroenterocolitis, dehydration and renal failure during the first three days after ingestion. The patient also developed leukopenia, thrombopenia and mild hepatocellular injury. Renal failure due to colchicine intoxication is due to various factors such as depletion of volume/hypotension, rhabdomyolysis and multiorgan failure. In this case, the hypovolemia was probably the fundamental cause of the acute renal insufficiency as demonstrated by the quick recovery after administering fluids. It is possible that indomethacin may have enhanced the toxic effect of colchicine on the kidneys and bone marrow. Some colchicine intoxications, as in this case, are caused by an error in interpreting the dose for treating an acute attack of gout. A way to prevent these errors would be to use a low-dose treatment protocol.

Postinfectious diffuse proliferative glomerulonephritis and acute renal failure in an HIV patient.

Postinfectious proliferative glomerulonephritis may occur in HIV-infected patients, although it is not a common cause of severe acute renal failure in them. We report a woman with HIV infection, who developed hypocomplementemic acute nephritic syndrome 10 days after an upper respiratory infection. Systemic diseases were excluded. The serum creatinine level increased to 6.6 mg/dl. Renal biopsy showed diffuse endocapillary proliferative glomerulonephritis, with mesangial and capillary walls, granular deposits of IgG and C3 by immunofluorescence. She was given corticosteroids with progressive normalization of her renal function. No opportunistic infections have occurred during 1-year follow-up.

Henoch-Schönlein purpura associated with clarithromycin. Case report and review of literature.

OBJECTIVE: To report a case of Henoch-Schönlein purpura that appears to be related to the intake of clarithromycin for pharyngitis/tonsillitis. CASE SUMMARY: We describe a case of Henoch-Schönlein associated with clarithromycin therapy in a 48-year-old white man with no history of allergic drug reactions. Four days after starting therapy, he came to our hospital emergency room because of a non-pruritic palpable purpuric rash on the trunk and extremities and arthralgias involving elbows and knees. Administration of clarithromycin was suspended, in a few days, arthralgias and skin lesions quickly resolved. Three weeks later, the patient presented again with abdominal pain, dark-red urine and swelling of the legs. Urinalysis revealed proteinuria of 11 g/24 h and hematuria. A percutaneous renal biopsy showed a diffuse endocapillary proliferative glomerulonephritis with segmental areas of fibrinoid necrosis within glomeruli, on immunofluorescence study granular deposits of IgA and C3 were present in the mesangium and capillary walls. A diagnosis of HSP was made. We suspected that the causative agent might be clarithromycin since this was the only drug added before the cutaneous and renal condition appeared. CONCLUSIONS: Our case and the previous case suggest that HSP may represent a potential adverse effect of clarithromycin, clinicians should be alerted to this potentially severe side effect of such a widely used drug. In accordance with the data obtained and based on the Naranjo algorithm, the adverse reaction could be considered possible.

[Palliative treatment of esophageal neoplastic stenosis using bipolar electrocoagulation probe]. / Traitement palliatif des sténoses néoplasiques oesophagiennes par sonde d'électrocoagulation bipolaire (BICAP).

OBJECTIVE, PATIENTS AND METHODS: The objective of this study was to evaluate the results of palliative bipolar electrocoagulation probe (BICAP) treatment in 26 patients with non surgical, obstructive esophageal cancer. The mean tumor length was 7.2 cm. The strictures were located as follows: 3 in the cervical esophagus, 8 in the thoracic esophagus, 13 in the distal third of the esophagus and 2 involved both the cervical and thoracic esophagus. Most lesions were circumferential (73% versus 27% non circumferential) and exophytic (78% versus 22% sub mucosal). Coagulation was carried out under direct endoscopic control in the forward direction. The success of treatment was evaluated on the basis of the degree of reopening achieved (easy passage of an endoscope 12 mm in diameter) and good functional results (improvement of dysphagia, scored from 0 to 4 using a standard grading scale, for more than 15 days). RESULTS: Twenty-six patients underwent a total of 45 BICAP treatments (31 initial sessions, 14 repeated sessions). Reopening was achieved in 92% of cases and good functional results were obtained in 85% (mean dysphagia score: 3.2 before treatment versus 1.1 after treatment). The improvement of dysphagia resulted in a significant improvement of general performance status and stabilization or weight improvement in 21 patients. The mean number of sessions necessary for good initial results was 1.2 +/- 0.4. After the initial treatment by BICAP, radiotherapy or radiochemotherapy were respectively associated in 4 and 10 patients. The median duration of improvement was significantly longer in patients who underwent radiochemotherapy as compared with patients treated by BICAP alone (22 weeks versus 4 weeks). During the follow-up, 9 patients required several BICAP treatments and at the end of their disease, 12 patients underwent other palliative procedures. Major complications occurred in 4 cases (2 esotracheal fistulas, 1 hemorrhage, and 1 aspiration pneumonia) and mortality related to the procedure was 8%. CONCLUSIONS: Palliative BICAP treatment of obstructing esophageal and cardial cancer provide quick relief of dysphagia but repeated treatment sessions are necessary to maintain initial improvement. The procedure requires a short hospitalization stay and can be easily accomplished in all cases regardless of the tumor features.

[Rhabdomyolysis and anuric kidney failure induced by the treatment with a gemfibrozil-cerivastatin combination]. / Rabdomiólisis y fracaso renal anúrico inducidos por el tratamiento combinado de gemfibrozil y cerivastatina.

A 67-year-old man treated with gemfibrozil for a year development rhabdomyolysis and anuric renal failure after addition of cerivastatin. The clinical features and serological studies ruled out other causes of rhabdomyolysis. Drugs were stopped and hemodialysis was carried on for 14 days until diuresis occurred. The renal function improved steadily to a serum creatinine of 1.2 mg/dl two months later. On the basis of its pharmacokinetic profile cerivastatin appears to have less interactions than other statins. There are only two reports of rhabdomyolysis and acute renal failure due to fibrates and cerivastatin combination. This patient shows the potential risk of a fibrates-cerivastatin combination. When this association is required it is necessary to avoid other nephrotoxic and myopathic factors and to monitor CK levels closely.

[Extracapillary IgA nephropathy associated with infection with hepatitis C virus and hepatic cirrhosis]. / Nefropatía IgA extracapilar asociada con infección por el virus de la hepatitis C y cirrosis hepática.

We describe a 36 year old man who was admitted to the hospital with dyspnea, edema of the lower limbs, arterial hypertension and oliguric renal failure. He had microhematuria and nephrotic range proteinuria, immunological tests were normal or negative. Renal biopsy revealed global (55%) or segmental glomeruloesclerosis, remaining glomeruli showed extracapillary proliferation (25%). Immunofluorescence study disclosed IgA mesangial deposits. He was also diagnosed as having liver cirrhosis with positive serology against hepatitis C virus. He was treated with dialysis, antihypertensive drugs and steroids with improvement of the renal function. However, ten months later maintenance hemodialysis became necessary. We emphasize two points: first IgA glomerulonephritis is rarely associated with hepatitis C infection, and second crescentic IgA nephropathy has been infrequently reported in liver cirrhosis.

[Complications of uretero-renoscopy]. / Complicaciones de la uretero-renoscopia.

INTRODUCTION: Since its clinical introduction ureteroscopy (URS) has experienced an impressive development due to the technical improvements of new and smaller urological armamentarium. Currently, ureteroscopy is a worldwide procedure with a varied number of diagnostic and therapeutic possibilities. However, the technique has complications. MATERIAL AND METHODS: We analyse the complications of URS in a series of 4.645 ureteroscopic procedures performed from january 1990 to december 2001, 2972 (64%) female and 1673 (36) male. The objectives of URS were diagnostic (haematuria) and therapeutic (ureteral stones, tumors, strictures and placement of stents). RESULTS: Fever was the most frequent complication (11.7%), with sepsis in 15 patients. Another complications were: ureteral perforation (1.2%), ureteral avulsion (0.06%), renal injury (0.04%), extrusion (0.4%) and uretero-iliac fistulae (0.02%). The most common treatment was conservative with endourological approach. CONCLUSION: Carefully performed ureteroscopy is a superb tool for the urologist either for diagnostic or therapeutic purposes with a low ratio of complications. The majority of these complications can be solved with conservative management.

[Extracorporeal shockwave lithotripsy in single kidneys. Our experience]. / Litotricia extracorpórea por ondas de choque en monorrenos. Nuestra experiencia.

Forty patients with renal lithiasis in single kidney were treated with extracorporeal shock wave lithotrity in our unit. Nine patients required emergency urinary by-pass, because of original picture of obstructive anuria, and in another 21 cases a double-J catheterism was conducted as prophylaxis prior to lithotrity. Treatment was carried out with analgesia and ambulatory, except for 10 patients with calculi of less than 10 mm where by-pass was not performed, and who were kept in preventive hospitalization for 24 hours. Average of sessions per patients was 1.59 (range 1-7). After 6 months follow-up there are 24 free renal units (60%), 12 (30%) with debris that can be expelled, failure in 4 (10%): 2 with debris that can be expelled and 2 which were not fragmented. Renal function has not deteriorated during follow-up, except for 2 patients with obstructive uropathy, that subsequently normalized following resolution of the condition. No significant differences were found in the treatment of calculi of less than 10 mm with or without double-J. ESWL is considered to be the choice approach for lithiasis in patients with one single kidney, due to is efficacy and low morbidity, safety in the ambulatory environment, even for calculi of less than 10 mm with no urinary by-pass.

[Pyeloureteral fungus ball in patients with urinary lithiasis. Treatment with ureterorenoscopy]. / Fungus ball pieloureteral en pacientes con litiasis urinaria. Tratamiento con ureterorrenoscopia.

INTRODUCTION: C. albicans is the most frequent fungus causing opportunist infections of the urinary tract. Agglutination of necrotic tissue nucleus (papilary necrosis), mucosus debri and chirurgic or lithiasic debri, originates a fungus ball, which can obstruct the urinary tract at any level. CLINICAL DATA: We present 2 patients with obstructive urinary lithiasis developing fungus ball in the dilated upper urinary tract. Patient 1 was bilaterally affected and never had systemic symptoms. Treatment consisted of uretherorrenoscope for removal of fungus ball, bilateral urinary diversion and anphotericine B irrigations and systemic. Patient 2 developed a candidemia and was treated with systemic liposomal anphotericine B, uretherorrenoscopy with removal of ureteric calculus and fungus ball, and placement of a double J catheter and nephrostomy tube for anphotericine B irrigation. DISCUSSION: Urinary lithiasis is a risk factor for fungal infection of the upper urinary tract, provided there is no other predisponent factor for opportunistic fungal infections. Calculi facilitates fungal growth by means of obstruction and subsequent retrograde estasis and creating a nucleus for growth, aggregation and ramification of mycelium. The infection pathway is probably ascendent and may turn into fungemia and septicaemia. Systemic antimicotics, urinary diversion and local irrigations with antimicotics, and endourologic technics, are the three basic items in the treatment. Ureterorrenoscopy plays an important roll as a diagnostic and therapeutic tool as it may solve the obstruction and allows removal of fungus ball.

[The treatment of ureteral lithiasis with a pulsed dye laser]. / Tratamiento de la litiasis ureteral con láser pulsado colorante.

PURPOSE: Pulsed dye laser lithotripsy has shown to be an effective and safe procedure for treatment of ureteral calculi. We report our experience. MATERIAL AND METHODS: A total of 1000 patients were treated with pulsed dye laser between January 1990 and February 1996. In 450 patients the procedure was performed under general or regional anaesthesia; the remaining 550 patients were treated with the aid of a mild sedation. RESULTS: In the mild sedation group a total of 417 out of 550 patients (75.8%) had their calculi completely cleared (85.6% of effectiveness in women with pelvic lithiasis). Different facts caused the treatment to fail in the remaining patients: severe pain, vagal reaction and technical difficulties. In the anesthesia group the procedure was effective in 346 cases (76.9%). Laser failed to clear calculi in 104 patients in this group due to technical impossibility and persistence of ureteral fragments. Additional treatment options in order to achieve resolution of the calculi were: ESWL, both ESWL and laser, Dormia basket, Zeiss stent, three-prong grasping forceps, ureterolithotomy and nephrectomy. Morbidity was higher in the group treated under anesthesia, being haematuria the most frequent adverse effect. CONCLUSIONS: We believe that ureteroscopy and laser lithotripsy is an effective, safe and low morbidity procedure for the treatment of ureteral calculi in women with distal calculi. It is often suitable to perform it with mild sedation with the subsequent benefit for both the patient and health care system regarding to cost-effectiveness balance.

[Renal traumatism: diagnostic protocol and therapeutic indications]. / Traumatismos renales: protocolo diagnóstico e indicaciones terapéuticas.

Review of 158 cases of renal traumatism attended ar the Urology Service of "La Fe" Hospital from 1984 to 1995. We analyze the indications of radiological study of the polytraumatized patient, the different classifications of renal traumatism and the changes produced in the diagnosis protocol, after substituting urography and arteriography for ultrasonography and CT in the Urgencies Area. We explain the treatment, complications and evolution of the patients in our series. 153 of the cases are closed traumatism and 5 of them are penetrating. Following the Chatelain's classification, 78.5% of the reviewed cases can be describes as traumatism grades I and II and in all the instances a conservative treatment was followed. Grades III and Iv (18.3%) were mostly treated surgically (82.7%) with 20.8% of complications that were solved conservatively except for one case, which required surgery. Every penetrating traumatism was dealt surgically and we practised nephrectomy in one of them (20%). We finally suggest, as the best moment for the reconstructive surgery, the period between the 3rd and the 10th day after the traumatism, due to the high rate of nephrectomy on the previous days and the technical difficulties which the haematoma fibrosis conveys on the subsequent.