Light-chain deposition disease of the kidney: a case report.

A 41-year-old man was admitted for evaluation of nephrotic syndrome associated with microhematuria, hypertension, and moderate renal failure. In serum and urine samples, monoclonal IgG-lambda was detected. Bone marrow examination showed normal representation of all cell lines with normal range of plasma cells. Renal biopsy demonstrated diabetes-like nodular glomerulosclerosis. Immunofluorescence failed to demonstrate the presence of kappa or lambda light chains in the kidney. Electron microcopy showed granular electron-dense deposits along the glomerular basement membranes and in the mesangial nodules. The patient was diagnosed as having light-chain deposition disease (LCDD) without evidence of plasma cell dyscrasia. This report was designed to stress the significant challenges that remain in the diagnosis of LCDD-related glomerulopathy. The salient morphological features that help in making an accurate diagnosis are discussed.

[Etiologic profile of amyloidosis of the elderly in Tunisia]. / Profil étiologique de l'amylose en Tunisie chez les sujets âgés.

BACKGROUND: The amyloidosis is one of the histologic lesions the most frequently associated to the aging. AIM: To identify the several etiologies of amyloidosis in elderly patients according to immunohistochemical type. METHODS: Retrospective study on 10 years, in Internal medicine and Nephrology department in Charles Nicolle Hospital. Tunis. Tunisia. Diagnosis of amyloidosis was retained after histological confirmation with specific colorations, in patients aged 65 years or more at the diagnose of amyloidosis. RESULTS: The study enrolled 51 patients with amyloidosis. In 67% of cases it was AA amyloidosis. The etiology the most frequently observed with this type was the tuberculosis, followed by chronic pulmonary infections. We retained diagnose of non AA amyloidosis in 21% of patients. The multiple myeloma has been the most frequent etiology observed, but no cause was identified at about half of patients. Twelve per cent of amyloidosis were not typed. CONCLUSION: From our study, we can remark the high predominance of AA amyloidosis, probably because of frequency of tuberculosis which is still high in our country, this is not in accordance with occidental data, were AL amyloidosis and senile amyloidosis predominate in elderly.

[Acute tubular necrosis in kidney transplantation]. / La nécrose tubulaire aiguë en transplantation rénale.

BACKGROUND: The acute tubular necrosis (ATN) is common after kidney transplantation. Acute tubular necrosis (ATN) is multifactorial and represents one of the main causes of the delayed graft function. Its impact on graft and patients survival is documented. AIMS: To study the prevalence of the ATN in kidney transplanted patients, the acute rejection rate and their impact on the graft and the patient survival. METHODS: We retrospectively studied the frequency of ATN, its causes and its impact on patient and graft survival in 255 kidney transplanted patients between 1986-2006. RESULTS: Thirty-nine patients had ATN (15.29%). They are 25 men and 14 women with mean age of 30.1 ± 12.6 years (8-61) followed for an average of 98 ± 61.76 months. The majority was treated by hemodialysis (79.48%) and half of them were transplanted from kidney of deceased donor. All patients received anti lymphocyte serum and the majority anticalcineurins (69.23%). The outcome was favorable in 26 patients (66.66%) with recovery of diuresis and normalization of renal function after 6 weeks on average. An acute rejection was diagnosed in 21 patients (53.48%). The mean creatinine at 1, 5 and 10 years was 135.3, 159.9 and 121.4 µmol / l. Eight patients had creatinine ² 130 µmol / l at 10 years. Ten patients died from infectious and cardiovascular causes. By comparing the 2 groups ATN + and ATN - we found a statistically significant correlation between ATN and cold ischemia (10 ± 10.9 vs 1.2 ± 4.7 hours, p <0.0001) and the interval between the start of dialysis and transplantation (42.18 ± 38.44 vs. 31.1 ± 25.2 months, p= 0.02). No statistical correlation was found between the ATN and gender, age of recipient and donor, warm ischemia, acute rejection, chronic rejection and graft and patient survival at 1, 5 and 10 years. CONCLUSION: The ATN is more common among transplanted patients from deceased donors. It had good evolution in the majority of cases and it's correlated to cold ischemia and duration of dialysis. Finally, it has no impact on patients and graft survival.

Membranoproliferative glomerulonephritis with isolated C3 deposits: case report and literature review.

Membranoproliferative glomerulonephritis with isolated C3 deposits (MPGNC3) is an uncommon condition characterized by overt glomerular C3 deposits in the absence of immunoglobulins and intramembranous dense deposits. Here the authors describe the clinical and morphological features of primary MPGNC3 in a 13-year-old boy and critically review the previously published cases. The patient presented with nephrotic syndrome and microscopic hematuria. Blood tests revealed very low circulating C3 levels. The renal biopsy exhibited subendothelial, subepithelial, and mesangial deposits, with C3 but not immunoglobulins seen on immunofluorescence. This case and the review of the literature indicate that the serum complement profile with decreased levels of C3 and normal levels of classical pathway components together with glomerular deposits containing exclusively complement C3 is highly suggestive of alternative pathway activation. The diagnosis of acquired and/or genetic complement abnormalities in some cases supports that complement dysregulation is implicated in the pathogenesis of MPGNC3. Such data show great promise to provide new therapy strategies based on modulation of the complement system activity.

Clinicopathological characteristics of obesity-associated focal segmental glomerulosclerosis.

Obesity-related glomerulopathy (ORG) is a secondary form of focal segmental glomerulosclerosis (FSGS) occurring in obese patients with a body-mass index higher than 30 kg/m(2). It is typically manifested by nephrotic-range proteinuria without full nephrotic syndrome, and progressive renal insufficiency. Characteristic morphologic features include the consistent presence of glomerulomegaly, predominance of perihilar variant of FSGS, and the relatively mild fusion of visceral epithelial cell foot processes. The concept of podocyte depletion as a driver of the glomerular scarring in obesity-associated FSGS is well documented. The underlying mechanisms are likely to be related in part to the oxidative stress and the impairment of the integrity of the slit diaphragm and cell adhesion resulting mainly from angiotensin II and transforming growth factor-ß. These proapoptotic cytokines are upregulated in obesity in response to insulin resistance, compensatory hyperinsulinemia and glomerular hyperfiltration-hypertension mediated mechanical stress. This review is designed to discuss the clinicopathologic features of obesity-associated FSGS, with a focus on the podocyte injury, which is involved in the onset and progression of the glomerulosclerotic process. Ultrastructural glomerular lesions are documented.

Incidence of seroma in sublay versus onlay mesh repair of incisional hernia.

A best evidence topic has been constructed using a described protocol. The three-part question addressed was: In patient undergoing open mesh repair of incisional hernia, is there any difference in the rate of seroma between Sublay and Onlay technique? The best evidence showed that Sublay repair has a lower seroma rate in comparison to onlay repair.

Does anastomotic technique affects the recurrence rate of Crohn's disease after ileocolic resection?

A best evidence topic has been constructed using a described protocol. The three-part question addressed was: does the anastomotic technique after ileocolic resection affects the recurrence rate in patients with Crohn's disease? Using OVID interface and PubMed interface, 16 articles were found; out of this 6 studies were deemed to be suitable to answer the question. The outcomes assessed were anastomotic recurrence rate. The best evidence showed that Kono-S ileocolic anastomotic technique is associated with significantly low recurrence rate in patient with Crohn's disease.

Locally advanced breast cancer treated with neoadjuvant chemotherapy: Is breast-conserving surgery feasible?

A best evidence topic has been constructed using a described protocol. The three-part question addressed was: is breast-conserving surgery feasible after neoadjuvant chemotherapy for locally advanced breast cancer? Using the reported search, 19 articles were found, out of these 6 studies were deemed to be suitable to answer the question. The outcomes assessed were local recurrence rate. The best evidence showed that breast conserving surgery is safe in terms of local recurrence.

[Calciphylaxis in chronic renal failure: 3 cases]. / Calciphylaxie au cours de l'insuffisance rénale chronique: etude de 3 cas.

BACKGROUND: Calciphylaxis is a small vessel disease responsible for vascular calcification and skin necrosis. It occurs in association with chronic renal failure and has a poor prognosis. BUT: Report new cases. We report 3 cases of calciphylaxis occurred in patients with chronic renal failure secondary to interstitial nephritis in 1 case, diabetic nephropathy in 1 case and thrombotic microangiopathy in 1 case. CASES: They were 2 females an 1 man aged of 44, 3 years meanly. Hyperphosphoremia and hyperparathyroidism were the essential risk factors. All patients died by sepsis. This course was precipitating by corticotherapy in 2 cases. CONCLUSION: Early recognition and treatment of risk factors is mandatory to reduce mortality in uremic patients with calciphylaxis.

Best evidence topic: Is ileocolic anastomotic leak rate higher in handsewn or stapler's anastomosis?

A best evidence topic has been constructed using a described protocol. The three-part question addressed was: is ileocolic anastomotic leak rate higher in handsewn or stapler's anastomosis? Using the reported search, 150 papers were found. 6 studies were deemed to be suitable to answer the question. The outcomes assessed were anastomotic leaks rate in hands Sewn and stapler's ileocolic anastomosis. The evidence does not provide an agreed consensus for which modalities of anastomosis have higher anastomotic leaks rate. Until a high quality randomized control trial is performed, the authors recommend an individual approach in a term of selection of which anastomotic modalities to be used.

[Peritoneal dialysis in the elderly]. / La dialyse peritoneale chez le sujet age.

BACKGROUND: Peritoneal dialysis is used more and more as treatment of substitution of the end stage renal disease at the old subjects of more than 65 years and its effectiveness and its good tolerance were shown. AIMS: To study the epidemiological and clinical profile of these patients, the indication and the advantages of PD, the various complications, the elements of prognostic and the survival of the patients and the technique. METHODS: We made a retrospective study including a series of 13 old patients of more than 65 years and treaties by PD during the period extending from the 1983/2/11 to the 2005/12/31. They are 10 men and 3 women on average of 70 +/- 3.1 year and representing 3.62% of the totality of the patients. The diabetic and vascular nephropathies represent the first cause of ESRD. RESULTS: The PD was used of first intention at 53.84% of the patients whose majority suffers from a malnutrition due to advanced age, anorexia, psychological disorders, bad dental state and uraemia. The cardiovascular complications are frequent in this age bracket explaining heavy morbidity. The pulmonary and urinary infectious complications are also frequent. The more frequent peritonitis compared to the literature, are comparable between the 2 age brackets < and = with 65 years and the mode of PD (APD or CAPD). The time separating the beginning from PD and which has occurred of the peritonitis is shorter in APD. The lesions of renal osteodystrophy are found among 6 patients: 3 cases of hyperparathyroidism and 3 cases of adynamic osteopathy. The return in HD is rare due to dysfunction of the catheter. The survival of the patients is 92.8% at 1 year and 60.8% at 5 years; that of the technique is worse with 88.3% at 1 year and 33.7% at 5 years Eight patients died (61.5%) because of cardiovascular diseases and of the infections. We found a correlation statistically significant between the survival of the patients and the mode of PD, on the other hand any correlation was not found with the age or the sex. The survival of the technique is not correlated to a significant degree with the age, the sex and the mode of PD. CONCLUSION: Peritoneal dialysis is used of first intention at more half of the old subjects and remains a last recourse for haemodialysis which have an initially vascular problem. The family support and the good nurse allow these patients to adhere well to the technique. The cardiovascular diseases and the infections are responsible for heavy morbi-mortality. The survival of the technique is worse than that of the patients.

[Interest of bone alkaline phosphatase in hemodialysis patients]. / Interet de la phosphatase alcaline osseuse chez les hemodialyses chroniques.

BACKGROUND: The renal osteodystrophy represent a major complication in hemodialysis. AIM: To evaluate the value of plasma bone-alkaline phosphatase (bAP) in the diagnosis of the type of renal osteodystrophy among hemodialysis patients and to seek a possible correlation between the bAP, total alkaline phosphatases (tAP) and the intact parathormone (iPTH). METHODS: We studied 67 chronic hemodialysis patients. Plasma bAP was determined by immunoenzymatic technic. iPTH (1-84) was measured by electrochimiluminescence. RESULTS: We found that bAP levels were normal (10-20 ng/ml) in 17 patients, low (< 10 ng/ml) in 4 and high (> 20 ng/ml) in the 46 other patients. There is a good positive correlation between the plasmatic rate of bAP and the following parameters: the period of dialysis (R = 0.316, p = 0.009), plasmatic rate of tAP (r = 0.781, p < 10(-3)) and the rate of iPTH (r = 0.650, p < 10(-3)). There is a good positive correlation between the plasmatic rate of bAP and the rates of the tAP and of iPTH, the correlation between bAP and the iPTH being more significant. A rate of bAP higher than 20 ng/ml had a sensitivity of 93.5%, specificity of 63.3% in favour of a rate of iPTH >400 pg/ml and consequently of the biological diagnosis of hyperparathyroidism. In addition, 4 patients have a bAP <10 ng/ml with iPTH < 150 pg/ml evoking an adynamic osteopathy. CONCLUSION: plasma bAP provides useful information about bone remodelling in hemodialysis patients.