A case of exorbitism in association with Wegener's granulomatosis with renal involvement.

Wegener's granulomatosis (WG) is a necrotizing granulomatous vasculitis involving the nose, paranasal sinuses, lungs, and kidneys. Ocular involvement can occur in about 50% of cases. There are very few reports of WG with orbital inflammation and exorbitism. We report a case of a female patient who presented with exorbitism related to orbital inflammation secondary to WG, with renal involvement. A 29-year-old woman with a previous history of recurrent pan-sinusitis presented with bilateral exophthalmos and renal failure with rapidly progressive glomerulonephritis. Computed tomography showed extensive bilateral soft tissue in the retro-orbital area. Immunologic tests showed the presence of type-C anti-neutrophil cytoplasmic antibodies and renal biopsy revealed pauci immune crescentic glomerulonephritis. The patient was treated with corticosteroids and pulses of cyclophosphamide followed by azathioprine and trimethoprim-sulfamethoxazole. After a follow-up of 10 months, the renal outcome was favorable with improvement of renal function but there was persistence of exorbitism and loss of visual function. Our case suggests that WG should be considered in the differential diagnosis of persistent bilateral exophthalmos. Prompt recognition of this early manifestation is important for the institution of early treatment.

Dissecting aortic aneurysm in maintenance hemodialysis patients.

The dissecting aortic aneurysm (DAA) is a rare pathology that may result in fatal outcome. We report follow up of three cases of DAA patients undergoing maintenance hemo-dialysis who were managed conservatively.

[Study of association of the SNP19 polymorphism of calpain 10 gene with type 2 diabetes in ethnic sub-groups of the Tunisian population: gene-environment interaction]. / Etude de l'association du polymorphisme SNP19 du gène calpaïne 10 avec le diabète de type 2 chez deux groupes ethniques dans la population tunisienne : interaction gène-environnement.

Calpaïn 10 (CAPN10) is the first diabetes gene to be identified through a genome scan followed by positional cloning, encoding the cysteine protease, the calpaïn 10 encodes for a ubiquitously expressed protease implicated in the two fundamental pathophysiological aspects of T2DM insulinoresistance and insulinosecretion. Many investigators, but not all, have subsequently found association between calpaïn 10 polymorphism and type 2 diabetes (T2DM) as well as insulin action and insulin secretion. The aim of this study was to determine whether there is an association between specific polymorphism SNP19 in CAPN10 gene and T2DM in two ethnic groups from Djerba Island. Overall, 162 patients with type 2 of diabetes and 110 healthy volunteers who served as controls for genetic characterization with no family history of diabetes were included in the present study. They consisted of 159 women and 113 men. Their mean +/- SD age was 56,47 +/- 11,86 years. All subjects were genotyped according to SNP 19 polymorphism in CAPN10 gene with PCR method to perform case-control study. After adjusting for gender and age, we found an association with a high risk of T2DM in Djerba Island only in Arab sub-group.

Gastrointestinal angiodysplasia in chronic renal failure.

Gastrointestinal (GI) hemorrhage is a frequent and sometimes life-threatening complication of end-stage renal failure. Angiodysplasia (AD), vascular malformation, is the most common cause of recurrent lower-intestinal hemorrhage in patients with renal failure. We report four chronic hemodialysis patients with AD. All patients presented with severe anemia requiring transfusion. GI hemorrhage ceased spontaneously in three cases and after treatment with argon plasma coagulation in another. Diagnosis of AD is usually challenging, since its cause is still unknown, and its clinical presentation is variable. Lesions are multiple in 40-75% of cases, often located in the stomach and duodenum but can affect the colon and the jejunum. Diagnosis is improved by endoscopy which has a much higher sensitivity compared to angiography. Capsular endoscopy may reveal the hemorrhage site in the small intestine when regular endoscopy fails, and therapeutic intervention usually include argon plasma coagulation.

Interstitial and glomerular renal involvement in sarcoidosis.

Sarcoidosis is a systemic disease characterized by chronic granulomatous inflammation. Chronic kidney disease (CKD) was reported in less than 1% of patients of sarcoidosis. The prevalence of tubulo-interstitial nephritis (TIN) in sarcoidosis varies from 7 to 27%. In this retrospective study, we present 15 patients with interstitial or glomerular renal involvement secondary to sarcoidosis diagnosed in our center from 1975 to 2006. Patients were 13 (96.6%) females and two males with a mean age of 56.5 years. CKD was present in 14(93.3%) patients, proteinuria in 13(96.6%), and nephrotic syndrome in one. Pulmonary involvement was present in 10 (66.6%) patients. Renal biopsy performed in 12 (80%) patients revealed TIN lesions in 10 (66.6%) patients, extracapillary proliferative glomerulonephritis (GN) in one, and membranous GN type 2 in another. Corticosteroid therapy using prednisolone 0.5 to 1 mg/kg per day was used in 12(80%) patients. Ten (66.6%) patients were followed up for a mean period of 25 months (ranged from 2 to 48 months). The outcome was favorable with 7 (46.6%) patients improved their renal function, 6 (40%) remained with a moderate CKD, one normalized his renal function, and one died suddenly after 2 months of initiating the treatment corticosteroids. We conclude that corticosteroid treatment is efficient in TIN and variably efficient in GN. Patients with sarcoidosis may cause advanced renal failure, which renders it a serious nephrological condition.

Renal amyloidosis followed more than 5 years: report of 12 cases.

Renal involvement with amyloidosis is common but causes patient survival to be poor, rarely reaching 5 years. In this study, we retrospectively reviewed clinical and biological characteristics as well as treatments and outcomes of patients with renal amyloidosis followed for more than 5 years. Between 1975 and 2003, 485 patients were diagnosed with renal amyloidosis including only 12 patients who were followed more than 5 years. The six men and six women of mean age 42.4 years (range 18 to 66 years) displayed renal signs of lower limb edema in all cases; hypertension in four cases, proteinuria on urinalysis in all cases with microscopic hematuria in five cases. Biological tests showed nephrotic syndrome in 11 patients, normal renal function in nine patients, and renal failure in three patients whose mean creatinine was 481.6 micromol/L (range 294 to 726). The amyloidosis was AA type in 11 cases and non-AA in one case. An etiologic survey revealed spondylarthropathy in one patient, pulmonary tuberculosis in two patients, chronic bronchitis in three patients, hepatic hydatic cyst in one patient, Mediterranean familial fever in two patients, Crohn's disease in one patient, Hodgkin's lymphoma in one patient, and multiple myeloma in one patient. Specific treatment was initiated with colchicine in seven patients. At a 110-month mean follow-up (range 53 to 153 months), remission of nephrotic syndrome was observed in four cases, progression to chronic renal failure in two patients, and to end-stage renal failure in five cases (range 53 to 196 months), with stabilization of renal function in seven patients. In conclusion, primary amyloid disease should be optimally suppressed in patients with renal involvement. The role of this treatment in remission of renal amyloidosis is not well established. This efficacy of the treatment has been demonstrated in some patients with improved survival.

Visceral leishmaniasis in a patient with sicca syndrome and nephropathy.

A 63-year-old woman presented with severe volume depletion and pre-renal azotemia. She had xerostomia, xerophthalmia and cervical lymhadenopathy. Urine examination revealed proteinuria, hematuria and glycosuria. Laboratory studies, after volume repletion, revealed hyper-gammaglobulinemia. Renal biopsy showed interstitial nephropathy and salivary-gland biopsy showed glandular atrophy and diffuse fibrosis. Diagnosis of leishmaniasis was established by bone marrow examination and serology. The patient was treated with pentavalent antimonial (Glucantime) with an excellent response. The treatment, however, had to be interrupted because of transient nephrotoxicity. After a break of four weeks, the antimonial was reinstituted with no more side effects. Both the sicca syndrome and the nephropathy responded very well to the treatment at nine months follow-up. In this case the presentation of visceral leishmaniasis was atypical, probably because of the partially suppressed immunity. The clue to the diagnosis was the polyclonal hypergammaglobulinemia.