Profil étiologique des microangiopathies thrombotiques rénales au Centre Hospitalier et Universitaire de Tunis: Etiological profile of renal thrombotic microangiopathy in Tunis University Hospital

Context and objective. Renal thrombotic microangiopathy (TMA) is a histological lesion that can be observed in several pathological settings. The present study aimed to describe the different etiological contexts in which TMA has been observed and provide particularities. Methods. A retrospective and descriptive study over a 16-year period, from January 2000 to January 2016 was conducted at the Tunis University Hospital. All patients aged over 16 years and presenting with TMA at renal biopsy were analyzed. Results. 100 cases of renal TMA were collected. The mean age of patients at the time of renal biopsy was 38.5 ± 12.9 years. The clinical picture was dominated by hypertension observed in 70 patients. Results of the laboratory investigations were as follows: kidney injury was noted in 92 patients and biological TMA was observed in 10 patients. At the renal biopsy, arteriolar TMA was seen in all patients and glomerular TMA was seen in 15 patients. Seventy-three patients reached end stage renal disease. TMA was observed mainly in association with glomerular nephropathy outside lupus nephropathy in 47 cases, lupus nephropathy in 19 cases, malignant hypertension in 18 cases and post-renal transplantation in 9 cases. TMA was related to atypical hemolytic uremic syndrome in one case and 6 sporadic cases were observed. Conclusion. Renal TMA has been observed in several pathological situations. Comparative studies with matched control cases in each group but without TMA lesions should be performed to identify risk factors for developing TMA and the influence of TMA on renal prognosis in each etiological setting. Contexte et objectif. La microangiopathie thrombotique (MAT) rénale est une lésion histologique caractérisée par la présence de microthrombi obstruant les vaisseaux de la microcirculation rénale. Actuellement, les patients atteints de MAT rénale sont répartis en fonction du contexte étiologique. L’objectif de la présente étude était de décrire les différents contextes pathologiques au cours desquels une MAT rénale a été observée. Méthodes. Il s’agissait d’une étude documentaire (16 ans). L’étude a inclus tous les patients adultes ayant des lésions de MAT à la ponction biopsie rénale (PBR). Résultats. Parmi 3240 PBR réalisées, la MAT a été observée chez 100 patients soit une incidence moyenne de 6,7 cas/an. L’âge moyen était de 38,5+/-12,9 ans avec une prédominance masculine. Les lésions de MAT rénale ont été associées à plusieurs contextes pathologiques : les néphropathies glomérulaires (47%), les néphropathies lupiques (19%), l’HTA maligne (18%), post transplantation rénale (9%), postgreffe de cellules souches hématopoïétiques (1%), post partum (2%), un contexte néoplasique (1%), une origine médicamenteuse (1%), un syndrome des antiphospholipides primaire (1%), et un syndrome hémolytique et urémique (1%). Conclusion. La MAT rénale est fréquemment secondaire et s’associe à plusieurs contextes pathologiques. Cependant un SHUa/PTT sous-jacents devrait toujours être recherchés

Reversible nephrotic syndrome secondary to pulmonary hydatid disease

Most patients with pulmonary hydatidosis are children. The disease may be asymptomatic or revealed by unusual events such as a glomerulopathy. An 8-year-old boy from a rural part of Tunisia presented with generalised oedema and macroscopic haematuria. There was no familial history of renal disease. He had a normal blood pressure (100/60 mmHg), and a pleural effusion was detected. Urinalysis showed nephrotic range proteinuria (375 mg/kg/d) and microscopic haematuria. His serum total protein concentration was 40 g/l and his serum albumin was 10 g/l. Renal biopsy showed capillary wall thickening and duplication, and mesangial cell proliferation in the glomeruli, characteristic of mesangiocapillary glomerulonephritis. Renal and abdominal ultrasound images showed increased echogenicity of the kidneys and mild ascites. Radiology revealed three large pulmonary hydatid cysts. The largest cyst occupied the entire right upper lobe and compressed the superior vena cava. Hydatid disease was confirmed by a strongly positive serum enzyme-linked immunosorbent assay (ELISA) for echinococcus. The patient was treated with high protein intake, dipyramidol and captopril; both right lung cysts were resected, followed by the left pulmonary cyst 4 weeks later. Hydatid cyst was confirmed histopathologically. He recovered well, the serum ELISA for echinococcus became negative, and follow-up urine examination and thoracic computerised tomography were normal 6 months after surgery, confirming good renal recovery and absence of pulmonary hydatid disease

Tuberculosis in children undergoing hemodialysis

Gargah Tahar1, Goucha-Louzir Rim2, Lakhoua Mohamed Rachid11Department of Pediatric Nephrology, 2Department of Nephrology, Charles Nicolle Hospital, Tunis, TunisiaAbstract: Tuberculosis (TB) remains a public health problem in Tunisia. Its incidence is higher in immunocompromised hosts than in the general population. In children and during hemodialysis, TB is characterized by the frequency of extrapulmonary localizations and diagnostic difficulties. The aim of this retrospective study is to evaluate the incidence of TB in Tunisian children undergoing hemodialysis and to determine its clinical features as well as the results of chemotherapy.Method: This retrospective study includes seven TB children among 112 children on hemodialysis at the pediatric nephrology department in Charles Nicolle Hospital from 2002 to 2008. The diagnosis of TB was established by a combination of clinical, radiological, biochemical, microbiological, and histological examinations. Treatment with anti-TB drugs, the results of therapy, and the outcome of patients were noted.Results: There were four girls and three boys aged 10 to 16 years (mean, 13 years). They had been on hemodialysis for 2 to 5 years (mean, 3 years). Noted clinical features were weight loss and fever in five cases, chest pain in one case, cervical lymph node in one case, and spinal pain in one case. The organ systems involved were pleural in two cases, pulmonary in one case, peritoneal in one case, cervical lymphatic in one case, and spinal in one case. One patient was treated empirically with a good response. Diagnosis was made by isolation of mycobacterium TB in three cases, by specific histological signs observed in a lymph node biopsy in one case, in peritoneal biopsy in one case, and in discovertebral biopsy in one case. In the remaining patient, the clinical and radiological presentations were compatible with pulmonary TB. All patients received four anti-TB drugs: isoniazid, rifampicin, pyrazinamide, and ethambutol. One patient died with miliary TB. The other patients had favorable outcomes.Conclusions: TB in hemodialysis children has a nonspecific clinical presentation. Extrapulmonary locations are most common. Diagnosis is often difficult, but successful outcomes are possible when made at an early stage.Keywords: child, hemodialysis, tuberculosi

Multiple Myeloma in a Young Adult with Renal Involvement

Abstract Multiple myeloma (MM) results from malignant plasma cell disorder. It represents approximately 10% of hematological malignancies and it is typically diagnosed in the elderly with a median age of 70 years and has a steep increase in incidence with advancing age. N Engl J Med. 2004, 1860; Clin Interv Aging. 2020, 619. The incidence of MM has been increasing over time, mostly due to population aging. Mayo Clin Proc. 2010, 225 However, certain MMs are diagnosed at young age even under 40 years old (2%). Leuk Lymphoma. 1998, 493; Blood. 2010, 5501. We report a case of a MM in a thirty‐four‐year‐old woman whose circumstance of discovery was acute kidney failure

Nondiabetic renal disease in patients with type 2 diabetes

Diabetic nephropathy (DN) is one of the major complications of type 2 diabetes mellitus (T2DM). The diagnosis of DN is mostly clinical. Kidney biopsy is indicated only if nondiabetic renal disease (NDRD) is suspected. This study is aimed to assess the prevalence of NDRD and to determine predictor and prognostic factors of DN, NDRD. It was a retrospective analytic study including T2DM patients in whom renal biopsies were performed at our department from 1988 to 2014. Seventy-five patients were included. Mean age was 52.7 years with sex ratio at 1.56. Renal biopsy findings were isolated NDRD in 33 cases, NDRD superimposed on DN in 24 cases, and isolated DN in 18 cases. Most common NDRD found were focal segmental glomerulosclerosis (21%) and membranous nephropathy (19%). Multivariate analysis showed that the absence of ischemic heart disease [odds ratio (OR) = 0.178, 95% confidence interval (CI) = 0.041–0.762], absence of peripheral vascular disease (OR = 0.173, 95% CI = 0.045–0.669), and presence of hematuria (OR = 7.200, 95%CI = 0.886–58.531) were independent predictors of NDRD. 24 patients reached end-stage renal disease 55% in DN group, 16% in DN associated to NDRD group, and 30% in NDRD group. The prevalence of NDRD found in our study confirmed usefulness of renal biopsy in patients with T2DM, especially in those without degenerative complications, hypertension, and insulin therapy

Acute kidney injury with granulomatous interstitial nephritis and vasculitis revealing sarcoidosis

Sarcoidosis is an inflammatory disease that affects mostly the lungs and lymph glands. Renal involvement is rare and especially vasculitis. We report a case who presented an acute kidney failure and had sarcoidosis with vasculitis and nodular splenic involvement. A 35-year-old woman presenting a Lofgren syndrome was hospitalized for acute renal failure with cervical lymphadenopathy without other clinical findings. Laboratory data disclosed elevated angiotensin converting enzyme serum level. Abdominal ultrasound showed a multinodular spleen. Renal histology revealed granulomatous interstitial nephritis with necrotizing vasculitis. Outcome was favorable after the institution of high dose corticosteroids along with cyclophosphamide. Renal involvement is rare in sarcoidosis. However, the diagnostic delay should be avoided to improve the outcome

Encapsulating peritoneal sclerosis after kidney transplantation: Success of medical treatment

Encapsulating peritoneal sclerosis (EPS) is an infrequent but serious complication of long-term peritoneal dialysis (PD). EPS may become clinically apparent when patients are on PD (classical EPS) or after undergoing kidney transplantation (post-transplantation EPS). This presentation of EPS seems to occur shortly after kidney transplantation in former PD patients. In this report, we present our experience in our first case of patient diagnosed with EPS after kidney transplantation

Predictive factors of mortality in a tunisian cohort with systemic lupus erythematosus

Mortality in systemic lupus erythematosus (SLE) has decreased with the advent of immunosuppressive therapy and the development of hemodialysis. This study aims to evaluate the survival rate, factors of poor prognosis, and causes of death in SLE in a Tunisian series. The records of all SLE patients followed up in a single center during 1974–2014 were reviewed. The causes of death were identified. Prognostic factors of survival were analyzed by multivariate analysis using the comparison of the survival rates by the log-rank test. Two hundred ninety-nine patients with SLE were included (274 women, 25 men) aged meanly of 27.52 years at diagnosis. The death occurred in 50 cases (16.7%). The mean age at death was 28.46 years (14–69 years). The patient survival rates at 5, 10, and 20 years were 83.8%, 78.6%, and 56.7%, respectively. The leading causes of death were active SLE (50%) and infectious complications (36%). Independent factors of poor prognosis identified by multivariate analysis were myocarditis (P = 0.029), splenomegaly (P = 0.0015), and worsening of renal function (P = 0.004). Remission was identified as a protective factor (P = 0.047). Our study shows that renal disease remains the primary cause and the main predictor of death in SLE, which is consistent with the literature data