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Karyomegalic interstitial nephritis is a rare cause of hereditary chronic interstitial nephritis, described for the first time over 40 years ago.A 36-year-old woman, of Turkish origin, presented with chronic kidney disease and high blood pressure. She had a history of recurrent upper respiratory tract infections but no familial history of nephropathy. Physical examination was unremarkable. Laboratory tests showed serum creatinine at 2.3 mg/dL with an estimated glomerular filtration rate of 26 mL/min/1.73m, and gamma-glutamyl transpeptidase and alkaline phosphatase at 3 and 1.5 times the upper normal limit. Urinalysis showed 0.8 g/day of nonselective proteinuria, microscopic hematuria, and aseptic leukocyturia. Immunological tests and tests for human immunodeficiency and hepatitis B and C viruses were negative. Complement level and serum proteins electrophoresis were normal. Analysis of the renal biopsy showed severe interstitial fibrosis and tubular atrophy. Numerous tubular cells had nuclear enlargement with irregular outlines, hyperchromatic aspect, and prominent nucleoli. These findings were highly suggestive of karyomegalic interstitial nephritis, which was further confirmed by exome sequencing of FAN1 gene showing an identified homozygous frameshift mutation due to a one-base-pair deletion in exon 12 (c.2616delA).The present case illustrates a rare but severe cause of hereditary interstitial nephritis, sometimes accompanied by subtle extrarenal manifestations. Identification of mutations in FAN1 gene underscores recent insights linking inadequate DNA repair and susceptibility to chronic kidney disease.

We report the case of a patient suffering from atypical hemolytic uremic syndrome with inaugural intra-alveolar hemorrhage. Clinical features and detection of circulating anti-glomerular basal membrane antibodies first raise the possibility of a Goodpasture syndrome. Renal biopsy allows to correct the diagnosis. Partial remission is obtained thanks to specific care and eculizumab infusions., (Copyright © 2015 Association Société de néphrologie. Published by Elsevier SAS. All rights reserved.)

Thrombotic microangiopathy includes a set of conditions characterized by the association of mechanical hemolytic anemia, thrombocytopenia and organ failure which accurate diagnosis is sometimes difficult. We report the case of a patient who presented a thrombotic microangiopathy (TMA) due to an atypical hemolytic and uremic syndrome (HUS) associated with an immunoglobulin A (IgA) nephropathy with a favorable outcome under corticosteroid.

Introduction: Using iodine povidone in internal way may be responsible of severe adverse effects, sometimes causing death of the patients., Exegese: A 36th years old woman, with a secondary sterility has benefitted of an uterine opacification by iodine povidone before a laparotomy and a salpingotomy. In post surgery she presented an anuric acute renal failure and a severe anaemia which have needed a transfusion. Outcome was favourable with a recuperation of diuresis and a full normalization of the renal function., Conclusion: The authors recommend to respect the indications of iodine povidone.

Septicaemia concerning Lactobacillus jensenii is exceptional. This bacteria commensal of the normal human flora is known for its low pathogenicity. We report here the observation of a woman, 50 years old, who was admitted in our service for an obstructive acute renal failure and who has presented a septicaemia due to L. jensenii, with a favourable issue by use of antibiotics (amoxicillin-clavulanic acid). This observation permits to report the clinical and bacteriological characteristics of L. jensenii. The importance of the immunodepressed status (diabetes mellitus, chronic renal failure) and use of the endoureteral acts is mentioned.

We report the case of a patient with predominantly renal sarcoidosis. Renal failure responded well to systemic corticosteroid therapy. The clinical presentation was particular, notably the absence of lung involvement and the presence of cutaneous lymphedema.

Introduction: IgD myeloma is a rare disease, comprising only 1-2% of all cases of myeloma., Exegesis: A 71-year-old woman was admitted with acute renal failure, hypercalcemia and IgD lambda multiple myeloma. Dialysis was necessary. Six monthly cures of chemotherapy of induction according to the protocol VAD (vincristine, doxorubicin and dexamethasone) allowed to achieve moderate chronic renal failure (serum creatinine = 120 mumol/L). Sixteen months later, the patient developed an abdominal mass due to an IgD plasmocytoma in spite of treatment with interferon alpha and dexamethasone. Chemotherapy with melphalan and dexamethasone allowed to the disappearance of plasmocytoma and remission. The death occurred 36 months after the diagnosis., Conclusion: This observation allows to display the particularities of IgD myeloma: remarkable preponderance of lambda-type light chains, small or no visible monoclonal spike on serum electrophoresis, frequent extraosseous spread of tumor, renal failure and presence of osteolytic lesions. Over the last years, management and prediction of the survival time of IgD myeloma patients have improved.