Immunoglobulin G4-Related Kidney Disease.

Immunoglobulin G4-related disease (IgG4-RD) is an emerging fibro-inflammatory condition known to involve potentially every organ system in the body, especially the kidneys, and is characterized by interstitial nephritis, obstructive nephropathy, and in rare cases, glomerular nephropathy. In this article, we report two cases of IgG4-RD with renal manifestations, hospitalized in the Department of Internal Medicine of the Principal Military Hospital of Instruction of Tunis. In conclusion, the clinical course of IgG4-related kidney disease varies widely and it can sometimes be difficult to diagnosis. A multidisciplinary approach can be useful. Early diagnosis and appropriate therapy are very important.

Two Peculiarities of the Nutcracker Syndrome: Rare Association with Multiples Thrombosis and Successful Medical Treatment (Pentoxifylline).

The nutcracker syndrome (NCS) is a rare condition characterized by the entrapment of the left renal vein between the superior mesenteric artery and the aorta. Clinically, it presents with hematuria, flank pain, and symptoms of pelvic venous congestion. It is more frequent in females. Conservative treatment is usually proposed children but in adults, NCS has to be treated by surgical or endovascular methods. Drug therapy is not proposed for the treatment of NCS. We report a case of a young patient who presented with recurrent gross hematuria. Our patient was treated with pentoxifylline three times daily and anti-coagulation, with a favorable outcome.

Epidemiology, diagnosis, and etiology of acute kidney injury in the elderly: A retrospective analysis.

The increased incidence of the acute kidney injury (AKI) in the elderly is becoming a disturbing reality in our days, mainly with the aging of the general population, and the predisposition of old persons to chronic diseases, drug toxicity, and infections. The aim of this study was to investigate the epidemiological, clinical, and biological features and to assess variant etiologies and outcomes of AKI in the elderly. Data were collected from the medical records of patients older than 65 years age having AKI admitted in the Internal Medicine Department of the Military Hospital of Tunis from January 2006 to June 2014. One hundred and seventeen cases were included in the study. The median age was 74.2 years. Male:female ratio was 1.6. Hypertension and diabetes were the most frequently found comorbidities seen in 69.2% and 48.7% of patients, respectively. The percentage of patients having acute-on-chronic renal failure was 13.7%. The AKI was manifested by nausea or vomiting in 33.3% of cases. One patient had hematemesis. The other symptoms were dyspnea in 14.5% of cases, uremic encephalopathy in 6.8% of cases, and oligoanuria in 16.2% of cases. The AKI was discovered fortuitously in 31.6% of cases and was diagnosed early within the 48 h after admission in 94% of cases and after this delay, in 6% of cases. It was ranked Acute Kidney Injury Network 1, 2, or 3, respectively, in 29.9%, 24.8%, and 45.3% of cases. Organic etiologies were observed more frequently (53.8%) followed by functional etiologies (37.6%) and then by obstructive ones (8.5%). Hemodialysis was performed for 9.4% of the patients. Of all the patients, 70.1% had favorable outcome, 49.6% of patients recovered totally. There was aggravation of the AKI in 29.9% of cases. Death occurred in eight cases (6.8%). The epidemiological, clinical, biological, and etiological profile of AKI in the elderly emphasizes the effect of aging of the human being on determining the pathology.

Toxocariasis and nephrotic syndrome.

We describe a case of toxocariasis as a rare cause of nephrotic syndrome in an adult woman. This rare association was confirmed by elevated Toxocara-specific immunoglobulin M titers. Renal biopsy was not done because of prolonged activated partial thromboplastin time. Our patient was treated with prednisone and albendazole. These treatments resulted in partial remission of renal symptoms as well as the abatement of the Toxocariasis infection. The relationship between toxocariasis infection and glomerular disease is still unclear. In the literature, exceptional renal impairment secondary to toxocariasis have been described, especially in children. To the best of our knowledge, this is the second case of nephrotic syndrome associated with toxocariasis in adults.

A case of abdominal aortic thrombosis associated with the nephrotic syndrome.

Thromboembolic disease is an important and frequent complication in patients with the nephrotic syndrome (NS), and the consequences are often severe. Usually, the venous system is affected. Arterial thrombosis has rarely been reported and occurs mainly in children. We report the case of a 27-year-old man with a history of NS due to focal and segmental glomerulosclerosis resistant to steroids and cyclosporine, admitted for bilateral pain in the calves. Aortogram revealed a suspended thrombus in the abdominal aorta just below the origin of the renal arteries with embolism into the left tibioperoneal trunk and the right anterior tibial artery. Endarterectomy was performed followed by systemic heparinization with a good outcome. Arterial thrombosis is rare and must be prevented.

Acute tubulointerstitial nephritis and uveitis syndrome: A report on four adult cases.

Acute tubulointerstitial nephritis and uveitis (TINU) syndrome is a rare disease, generally presenting in children and young women. The interstitial nephritis may precede, follow, or develop concurrent to the uveitis. We report the clinical features and outcomes of four adult patients, aged 41-70 years with the TINU syndrome.

Severe metastatic calcifications in a hemodialysis patient.

Tissue calcification is a common complication in patients on continuous hemodialysis (HD) for chronic renal failure; however, severe calcification is unusual. Three distinct clinical types of extraosseous calcifications are found in uremic patients: vascular calcification, periarticular (tumoral) calcification, and visceral calcification (heart, lung, and kidney). We report a case of a young chronic HD patient who presented with extensive metastatic calcifi cations both vascular, visceral specially localized in the lungs, and periarticular with progressively increasing multiple subcutaneous swellings. This evolution was secondary to noncompliance of the patient to the treatment of a malignant hyperparathyroidism with a marked elevation of phosphocalcium product.

Retroperitoneal fibrosis: A retrospective review of clinical presentation, treatment and outcomes.

Retroperitoneal fibrosis (RPF) is a rare disease and has a high risk of developing chronic kidney disease (CKD). This retrospective study was carried out with the objective to study the epidemiological, clinical and therapeutic characteristics of RPF and identify the risk factors associated with its progression to CKD. All 30 cases (24 males and five females) of RPF admitted from January 1985 to December 2013 in the Military Hospital of Tunis were included in this study. The mean age was 50.5 years. Presentation was with lower back pain, acute renal failure and inflammatory syndrome in 93%, 56% and 43% of the cases, respectively. Sixteen patients (54%) had a creatinine clearance <60 mL/min at the time of diagnosis. Erythrocyte sedimentation rate and C-reactive protein of >30 mm/h and 10 mg/L were observed in 56% and 53% of cases, respectively. The abdominal computed tomography scan showed ureterohydronephrosis in 63% of the cases. Classes I, II and III according to Scheel's radiological classification were found in, respectively, 16%, 13% and 70% of cases. Biopsy of RPF was performed in 20% of the cases, and all showed an inflammatory infiltrate without signs of vasculitis. RPF was idiopathic in 85% of the cases. Oral corticosteroid therapy was started for all patients. After a mean follow-up time of 53.2 months, an initial favorable response was noted in 76% of the cases. Fifty-three percent of the patients have presented one or more relapses during follow-up and 20% progressed to CKD. Most relapses were successfully treated by corticosteroids; only five patients had required additional immunosuppressive therapy. Two patients died. Elevated creatinine at diagnosis, high urea, clearance of creatinine lower than 60 mL/min and the use of ureteral stents were identified as risk factors for development of CKD.