Crystalline Nephropathy in Renal Transplant: A Series of 4 Cases.

Crystals are particles of endogenous inorganic or organic composition that can trigger kidney injury when deposited or formed inside the kidney. The most common forms of crystalline nephropathies (CNs) are nephrocalcinosis and oxalate nephropathy. The causes of early allograft dysfunction are changing constantly, and recently calcium oxalate (CaOx) crystal deposition has been added to this list. CaOx deposition in renal allograft is important and probably under-recognized cause of delayed graft function that requires adequate awareness with early intervention to improve the allograft outcome. Here, we describe four cases of irreversible renal graft injury due to CNs.

[A multicenter study on profile of hypertension in focal segmental glomerulosclerosis in Tunisia]. / Profil de l'hypertension artérielle dans la hyalinose segmentaire et focale (HSF). Étude multicentrique de 116 cas en Tunisie.

UNLABELLED: Hypertension in focal segmental glomerulosclerosis is frequent and responsible for the progression of the disease. It could be a circumstance of the diagnosis of FSG or a complication of the nephrotic syndrome. PURPOSE: To determine the prevalence of hypertension among patients with FSG diagnosed in Tunisia and to describe the profile of patients with FSG having hypertension in contrast with who do not. PATIENTS AND METHODS: It was a retrospective multicentric study based on 116 patient files having FSG located in 5 specialized centers in Tunisia. RESULTS: The prevalence of hypertension among our patients was 41%, with a feminine predominance, their mean age was 36.34 ± 15.71 years. The systolic blood pressure among the patients with hypertension was 153.18 mmHg. The nephrotic syndrome was impure due to hypertension in 14.5% of the cases. The patients affected by hypertension were more obese. Proteinuria was higher among those not having hypertension than those with it, who score an average value of 5.67 ± 4.51 g/24h, with an insignificant difference. Serum creatinine at presentation was significantly higher among patients with hypertension. Vascular lesions were present at the renal biopsy among 39.45% of patients affected by hypertension, associated with renal failure in 58.50% of patients. The etiopathogenic treatment of FSG was essentially based on steroids full dose. CONCLUSION: Hypertension is often present in FSG and its' treatment must be as soon as possible in order to slow the progression of kidney chronic disease.

BK virus-associated hemophagocytic syndrome in a renal transplant recipient.

Hemophagocytic syndrome (HPS) is a life-threatening hematological disorder in immunocompromised patients. Reactive HPS is observed in patients with systemic infection, neoplasia or auto-immune diseases. It is a rare hematological disorder after renal transplantation and must be suspected when fever and pancytopenia are seen in association with viral infections. HPS is usually associated with infection with the Cytomegalovirus and Epstein-Barr viruses. We report here a case of BK-virus-associated HPS.

Secondary oxalosis due to excess vitamin C intake: a cause of graft loss in a renal transplant recipient.

Renal oxalate deposition can be seen with primary hyperoxaluria, malabsorptive states, ethylene glycol toxicity and, rarely, with excessive vitamin C ingestion. We report a case of secondary hyperoxaluria in which the diagnosis was not considered initially because there was no past history of urinary calculi and no evidence of nephrocalcinosis on plain X-ray of the abdomen and ultrasonography. The disease was detected and diagnosed only after kidney transplantation. Secondary oxalosis can cause graft loss or delayed graft function. Biopsy of the allograft should be carefully examined for oxalate deposits even in the absence of a family history. When oxalosis is diagnosed, intensifying hemodialysis (HD) to eliminate calcium oxalate can help in the recovery of renal function in some cases. Systematic vitamin C supplementation in HD patients should be avoided as it can be a cause of secondary oxalosis.

Invasive fungal infections in renal transplant recipients: about 11 cases.

UNLABELLED: Invasive fungal infections are a major complication and an important cause of morbidity and mortality among solid organ transplant recipients. Their diagnosis is difficult and their prognosis is often pejorative. OBJECTIVE: The aim of this study was to report the cases of invasive fungal infections in renal transplant recipients in Habib Bourguiba Sfax university hospital and to identify the main fungal agents. MATERIALS AND METHODS: It is a retrospective study of invasive fungal infections in renal transplant recipient reported in our hospital from January 1995 to February 2013. RESULTS: Invasive fungal infections were diagnosed in 11 cases (3.4%) among 321 renal transplant recipients. These infections included four cases of pneumocystosis, two cases of candidiasis, two cases of aspergillosis, two cases of cryptococcosis and one case of mucormycosis. There were six men and five women. The mean age was 37 years. The infection was late in 63% of cases (>3 months after transplantation). The prolonged corticosteroid and immunosuppressive therapy were the main risk factors (100%) followed by renal failure (45%), graft rejection (45%), broad spectrum antibiotics (45%), CMV infection (36%), neutropenia (36%) and dialysis (18%). The evolution under treatment was favourable only in two cases (18%). CONCLUSION: Invasive fungal infections are not common among kidney transplant recipients. However, they remain an important cause of morbidity and mortality in this group of patients. Prevention, early diagnosis and appropriate management are necessary to improve prognosis and reduce mortality rate.

Sirolimus for the treatment of Kaposi sarcoma after renal transplantation: a series of 10 cases.

The incidence of Kaposi sarcoma (KS) has substantially increased among immunocompromised patients, suggesting a role for immunosuppressive drugs. The aim of this study was to evaluate the incidence, features, and outcome of KS among 307 kidney transplantation patients at our center between January 1994 and June 2010. During the study period, the 10 patients who developed KS (3.25%) showed a mean age at transplantation of 35.8 ± 8.7 years (range, 22 to 49 years). The mean interval between transplantation and occurrence of KS was 24.7 ± 21.36 months (range, 6 to 64 months). The mean time of antithymocyte globulin induction was 9.5 days (range, 6 to 13 days). KS was restricted to the skin in 7 cases, among which, one presented with associated Hodgkin lymphoma. Visceral involvement (one lung and one colon) was observed in two cases. One patient presented with a gastric KS without skin lesions. Immunosuppressive treatment was reduced, then withdrawn in three cases, resulting in regression of KS a few weeks later, but with graft loss requiring hemodialysis at 1, 3 and 4 months. Among the remaining 7 cases, we stopped mycophenalate mofetil (MMF) and switched from calcineurin inhibitors to sirolimus. Allograft function remained stable after the switch. Only one patient who already had allograft dysfunction due to biopsy-proven chronic allograft nephropathy. Deteriorated progressively, undergoing hemodialysis at 2 years after KS diagnosis. In conclusion, we observed a relatively high incidence of KS among our cases. The introduction of sirolimus resulted in complete regression of KS lesions with preserved graft function.

Atypical presentation of a brown tumor in a kidney transplant recipient: a case report.

Brown tumor is a rare complication of secondary hyperparathyroidism. It is exceptionally encountered after kidney transplantation. We here report on a 54-year-old male recipient who developed a brown tumor localized in the right forearm, and whose initial presentation was atypical, mimicking a bone tumor. Hence, diagnosis of brown tumors should be suggested by clinicians in a context of hyperparathyroidism.

[Management of arterial hypertension in Tunisia: the challenge of a developing country]. / Prise en charge de l'hypertension artérielle en Tunisie: le défi d'un pays en voie de développement.

Tunisia is a north-African country where epidemiological and socio-economical transition lead cardio-metabolic diseases at the forefront of health concerns. Cardiovascular disease becomes the leading cause of death. Epidemiological studies noted that 30,6% of adults are hypertensive. Only 38,8% from those diagnosed with hypertension were aware about their disease. From those, 84% take antihypertensive treatment. Tunisian health authorities developed in 1993 a national program for diabetes and hypertension care. Hypertension benefits from a full support by the social security fund for policyholders and the state for the poor. Thus, hypertension followed by public health centers is controlled in 42.9% of cases.

[Prevalence and diagnostic value of antinuclear antibodies without identified antigenic target: a retrospective study of 90 patients]. / Prévalence et valeur diagnostique des anticorps antinucléaires de spécificité antigénique indéterminée : étude rétrospective à propos d'une série de 90 patients.

PURPOSE: The objective of this study was to determine the clinical relevance and the diagnostic significance of positive antinuclear antibodies (ANA) without identified antigenic target by the usual characterization technique. PATIENTS AND METHODS: Retrospective study conducted in the Laboratory of Immunology of Habib Bourguiba Hospital (Sfax, Tunisia) during 18 months. The inclusion criteria were the presence of an ANA titer greater or equal to 1/320 with negative characterization result. ANA screening was performed by indirect immunofluorescence (IIF) on Hep2 cells. Each positive serum was tested by IIF on Crithidia luciliae (anti-native DNA) and by immunodot (anti-nucleosome, anti-histone, anti-Sm, anti-RNP, anti-SSA, anti-SSB, anti-Scl 70, anti-PM-Scl, anti-Jo1, anti-PCNA and anti-ribosomal protein). Sera of systemic lupus erythematosus (SLE), myositis, and scleroderma patients were tested for anti-Ku, anti-PL7, anti-PL12 and anti-Ro-52 using dot myositis. RESULTS: Sera of 90 patients were studied: 18 men and 72 women (average age: 44 years). Drug-induced ANA was found in eight patients. The most frequent clinical symptoms were joint (56.7%), cutaneous (54.4%) and constitutional symptoms (45.6%). The diagnosis of an autoimmune disease was suspected in 49 patients (54.5%) and confirmed in 30 (33.3%) including 20 cases of connective tissue disease: myositis (n=6), scleroderma (n=5), Sjögren's syndrome (n=3), SLE (n=4), rheumatoid arthritis (n=6) and antiphospholipid syndrome (n=4). Other autoimmune diseases were less frequent. The anti-Ku antibody was detected in the majority of patients with connective tissue disease. The diagnosis of non-autoimmune diseases was established in 25.5% of patients. Eighteen patients (20%) had no diagnosis orientation. CONCLUSION: Our study demonstrated the diagnostic value of the presence of ANA even in the absence of known antigenic target, confirmed the role of the IIF as "gold standard" test for ANA screening, and suggested the usefulness of the addition of Ku antigen in the immunodot classic profile.

Does kidney transplantation with multiple arteries affect graft survival?

INTRODUCTION: We compared short- and long-term outcomes of renal transplants with single versus multiple arteries. PATIENTS AND METHODS: We retrospectively analyzed data from kidney transplants from 208 living donors performed between 1994 and 2010. Renal grafts were divided into two groups: single renal artery (n = 164) versus multiple renal arteries (n = 44). The groups were compared regarding early and late vascular and urological complications. Patient and graft survivals were compared using Kaplan-Meier survivorship curves with comparisons using the log-rank test. RESULTS: Both groups were comparable regarding acute rejection episodes, posttransplant hypertension, postsurgery renal artery stenosis, and urologic complications. Only hemorrhagic complications and renal artery thrombosis were significantly higher in the multiple renal arteries group (P = .027 and .03, respectively). Warm ischemia time was significantly longer in the multiple renal arteries group without any influence on the incidence of acute tubular necrosis (P = .2). Mean creatinine clearance at 1 year was 65 versus 50 mL/min/1.73 m(2) (P = .5) and at 5 years, 60 versus 55 mL/min/1.73 m(2) (P = .1) for the single versus multiple renal arteries groups, respectively. Return to hemodialysis was necessary for 18.8% of the single and 16.1% of the multiple renal arteries group. CONCLUSION: The use of an allograft with multiple renal arteries is a safe, successful surgical procedure, that does not influence patient or graft survivals or increase surgical complication rates provided the surgical team is evolved with technical skill.

Evolution of impaired renal function after external continent urinary diversion (Mitrofanoff principle).

OBJECTIVE: To study the evolution of impaired renal function after external continent urinary diversion (Mitrofanoff principle) (ECUD-M) associated with ileocystoplasty. PATIENTS AND METHODS: Over 18 years from 1992 to 2009, ECUD-M with ileocystoplasty was performed in 120 patients with mean age of 25.5 years. Renal impairment was evident in 43 patients (17 children and 26 adults). RESULTS: Ninety percent of patients demonstrated a neurologic bladder and mild to moderate renal failure. Initially, all patients underwent continuous bladder drainage for a mean of 3 weeks. Renal function improved in 35 patients, although with persistent mild renal insufficiency. The other patients demonstrated moderate persistent residual renal insufficiency. During a mean follow-up of 10 years (range, 1-18 years), renal function returned to normal in 13 patients, stabilized at lower values in 15, and remained moderate in 5. After a mean follow-up of 8 years (range, 6-12 years), renal failure gradually worsened, increasing to higher values in 6 patients and leading to hemodialysis in 4. One patient underwent living-donor kidney transplantation, with good evolution. CONCLUSION: ECUD-M with ileocystoplasty can lead to normalization unless stabilizationof impaired residual renal function by eliminating the obstructive factor provides self-adequate management of the diversion. The procedure delays for the need forhemodialysis therapy, and enables patients to prepare for kidney transplantation into a previously reconstructed lower urinary tract.

Primary adenocarcinoma in a kidney allograft: a case report and review of the literature.

De novo tumors are common complications after solid organ transplantation. Lymphoma and skin cancers are the most frequently observed malignancies. However, graft carcinomas can be observed to be five times more frequent after kidney transplantation compared to their incidence in the general population. We report a case of a 49-year-old female who developed an early adenocarcinoma of the graft as revealed by acute renal failure. She underwent transplantectomy and chemotherapy with hemodialysis therapy. Carcinoma of the graft is a rare but serious complication usually occurring late after transplantation. Close monitoring of a kidney recipient using abdominal ultrasound may detect this complication at early stages, which may improve the prognosis. Similarly, good screening of donors may prevent tumor transmission.

Early onset of paucisymptomatic cryptococcal meningitis in a kidney transplant patient: a case report and review of the literature.

Fungal infections of the central nervous system are rare and are more frequently encountered in immunocompromised patients. Cryptococcocal infection is the most common opportunistic fungal infection after Candida and Aspergillus in organ transplant recipients. Atypical manifestations and nonspecific neuroradiological findings due to the lack of inflammatory response in these immunocompromised patients are responsible for a delay in diagnosis. This diagnosis should be considered even in atypical neurological signs, and additional tests (cerebrospinal fluid examination, magnetic resonance, etc) that may help to suggest the correct diagnosis should be used. We report a case of cryptococcal meningitis in a renal transplant recipient, which was misdiagnosed for several months because of an atypical presentation of headaches without fever or neurological signs.

Simultaneous Hodgkin's disease and Kaposi sarcoma in a renal transplant recipient.

A 38-year-old women underwent first cadaver kidney transplantation. Her panel reactive antibody was 0%, and she had never previously been transfused nor pregnant. She received induction therapy with antithymoglobulin (ATG) as standard protocol and maintained on immunosuppressive treatment of cyclosporine A, mycophenolate mofetil (MMF), and prednisone. Nine months after transplantation, she presented with anorexia, asthenia and weight loss. Cutaneous Kaposi's sarcoma and a Hodgkin disease were diagnosed. MMF was discontinued and cyclosporine A was switched to sirolimus. She also received a poly-chemotherapy associated with 4 courses of rituximab. Twelve months later, the patient had normal graft function and both malignancies were in complete remission.

[Microalbuminuria and left ventricular geometry in essential arterial hypertension]. / Microalbuminurie et geometrie ventriculaire gauche dans l'hypertension arterielle essentielle.

The association between left ventricular hypertrophy and microalbuminuria is actually admitted. Recent studies shown that patients with microalbuminuria have higher left ventricular mass index, worst systolic function and often concentric geometric kind of left ventricular hypertrophy. The efficiency of an adapted and early therapeutic on a potential relationship between reduction of microalbuminuria and improvement of target organ injury, principally heart and kidney, is still to evaluate. The aim of our study is to review geometric and hemodynamic aspect of left ventricular hypertrophy in hypertensive micro-albuminuria patients.

[Esophageal leiomyomatosis revealing an Alport syndrome]. / Léiomyomatose oesophagienne révélatrice d'un syndrome d'Alport.

Alport syndrome is a rare progressive hematuric nephropathy associated with sensorineural deafness. Leiomyomatosis associated with Alport syndrome is quite rare. We report a particular case of Alport syndrome which was diagnosed in the setting of an oesophageal leiomyomatosis. Alport syndrome and leiomyomatosis are caused by mutation of the genes encoding for the alpha chain of type IV collagen. In view of the important clinical and genetic implications, renal function and urinary status should be controlled in any patient with oesophageal leiomyomatosis.

[Ocular findings in Alport syndrome: 32 case studies]. / Atteinte oculaire au cours du syndrome d'Alport. A propos de 32 cas.

BACKGROUND: Alport syndrome is an inherited disease resulting in kidney failure, hearing loss, and ocular abnormalities. The purpose of this study was to describe the incidence and type of ocular abnormalities and to determine inheritance of this syndrome in our population. PATIENTS AND METHODS: A total of 32 patients, from ten different families in South Tunisia, underwent a complete ocular examination. Inheritance was determined using pedigrees and genotyping. RESULTS: The best corrected visual acuity was 7.6/10. Biomicroscopy showed polymorphous dystrophy in 3%, anterior lenticonus in 28%, lens opacities in 3%, cataract in 19%, and retinal flecks in 37%. The genetic survey found five families with X-linked Alport syndrome, four families with recessive autosomal disease, and one family with dominant autosomal disease. DISCUSSION: Ocular abnormalities have been reported in 9%-82% of Alport syndrome patients. They are rare in childhood and increase in frequency and severity with age. The types of ocular defects described mostly involve the lens, the retina and more rarely the cornea. The most common changes are anterior lenticonus and perimacular retinal flecks. In approximately 85%, Alport syndrome is X-linked. In the remaining 15%, the transmission is autosomal recessive and exceptionally autosomal dominant. CONCLUSION: Ocular examination is a precious help for Alport syndrome diagnosis. It can also determine the prognosis of nephropathy.

Risk genotypes and haplotypes of the GLUT1 gene for type 2 diabetic nephropathy in the Tunisian population.

OBJECTIVE: Diabetic nephropathy (DN) is a long-term complication of both type 1 and type 2 diabetes. Genetic studies on DN have been of little help so far, since several genetic association studies have shown conflicting results. Here we report the findings of a case-control study on five SNPs in the glucose transporter 1 (GLUT1) gene. The study investigated the association of five GLUT1 genotypes and haplotypes with DN. RESEARCH DESIGN AND METHODS: All subjects, 126 DN (cases) and 273 type 2 diabetes (controls), were genotyped using the polymerase chain reaction restriction fragment length polymorphism. RESULTS: The TT and the AA genotypes of the Haell and Enh2 SNP1, increased the risk of DN. The study also identified CGT as the highest risk haplotype (4.4-fold) followed by CAT with an increased risk of DN of 2.6-fold. CONCLUSIONS: The GLUT1 gene confers susceptibility to DN in type 2 diabetes patients in the Tunisian population.

Free-living amoebae (FLA): detection, morphological and molecular identification of Acanthamoeba genus in the hydraulic system of an haemodialysis unit in Tunisia.

UNLABELLED: The free-living amoebae (FLA) are ubiquitous and opportunistic protozoa. They can induce human and animal diseases. The aim of our study was to detect the FLA and Acanthamoeba genus in the hydraulic system of an hemodialysis unit. It was a prospective study of 46 water samples. The first collect (23) was before cleaning and after the haemodialysis sessions and the second (23) after cleaning and before the hemodialysis sessions. RESULTS: the morphological study enabled us to detect morphotypic diversity. The predominant morphotypes were the acanthopodial forms (29%). At the entrance of hemodialysis unit there were acanthopodial (44%) and monotactic (25%) forms; at the outlet, acanthopodial and fan-shaped forms (25% each). In addition, Acanthamoeba genus was present in 39% (1st collect) and 18% (2nd collect). The amplification of the FLA 18S rDNA gene was negative in only one sample localized in the last stage of water treatment unit (WTU). The amplification of the 18S rDNA (ASA.A1) Acanthamoeba gene was positive in 15 samples. CONCLUSIONS: we noted that, in the hemodialysis unit, the purification techniques used in the WTU were effective, but there is a problem of water stagnation in the drain, which constitutes an appropriate condition for the biofilms formation. It is then necessary to use a filter with a low porosity (0.2 microm) at the entrance of the hemodialysis unit and if possible to change the drain.

Factors predisposing to post-renal transplant erythrocytosis: a retrospective study.

A retrospective study was conducted on 143 consecutive renal transplant recipients who had a functioning graft for three months or longer, to evaluate the prevalence of post-transplant erythrocytosis (PTE) and its potential risk factors. True PTE was defined as hematocrit (Ht) above 52% and hemoglobin (Hb) above 18 g/dl in males, and Ht above 50% and Hb above 17 g /dl in females. A total of 31 patients (21.6%) developed PTE; none had any evidence of polycythemia vera (PV), or secondary polycythemia due to reduced arterial oxygen, kidney or hepatic tumors, or relative erythrocytosis due to a decrease in plasma volume by overuse of diuretics. Thirty-one non-polycythemic patients (Hb 12.9 +-1.6 g/dl) matched for sex, age and renal function were used as case controls. PTE was more common in males (p= 0.043). The majority of our patients developed PTE within the first year post-transplantation and all had excellent renal function at the time of diagnosis. Also, PTE was found to be related to duration on dialysis prior to transplantation (p= 0.0013) and acute rejection (p= 0.0031).