Primary membranous nephritis (PMN) is an autoimmune disease induced by the deposit of antibodies (Ab) to the phospholipase receptor A2 receptor (PLA2R) on podocytes. In this context, we aimed to ...assess the relationships between anti-PLA2R Ab, PLA2R rs4664308 SNP, PLA2R mRNA levels and PMN susceptibility and outcome. Sixty-eight PMN patients, 30 systemic lupus erythematosus (SLE) patients with secondary MN and 30 healthy control subjects served for anti-PLA2R Ab measurement by ELISA and PLA2R rs4664308 SNP genotyping by a commercial real-time PCR. Twenty patients with tubulo-interstitial nephritis (TIN) were used as controls for renal PLA2R mRNA quantification in PMN patients from kidney biopsies. PLA2R mRNA quantification was carried-out by real-time PCR after RNA extraction. Forty-three (63.2%) PMN patients received initial therapy consisting of alternating monthly cycles of corticosteroids and cyclophosphamide. Twelve (17.6%) patients had resistant PMN to initial therapy and were consecutively treated by cyclosporine or tacrolimus. Anti-PLA2R Ab and renal PLA2R mRNA could be useful markers for PMN outcome predicting. The PLA2R rs6446308 SNP is associated with PMN susceptibility in Tunisians.
Catheter-related infections (CRI) are a major cause of morbidity and mortality in chronic hemodialysis (HD) patients. In this paper, we share our experience with CRI in HD patients. We recorded 49 ...cases of CRI among 167 patients during a period of 40 months (January 2018-April 2021). The incidence of CRI was 3.7 per 1000 catheter-days. The revealing symptoms were dominated by fever or chills (90%). Inflammatory signs were observed in 74% of cases with respectively concurrent exit-site (51%) and tunnel infection (6%). The biological inflammatory syndrome was found in 74% of patients (average CRP level = 198.9 mg/l). Blood cultures were performed in all cases and were positive in 65% of cases. Thirteen patients have been diagnosed with Infection complications, which were respectively infective endocarditis in 7 cases, septic arthritis in 3 cases, infective myositis in one case, cerebral thrombophlebitis in 1 case and mediastinitis in 1 case. The death occurred in eleven patients, it was due to septic shock in 9 cases, pulmonary embolism in one case and neurologic alterations related to cerebral thrombophlebitis. The mean seniority in HD was 16.5 months in the group with CRI and 3.7 months in the group without CRI (p < 0.04). We did not notice significant difference in mortality between tunnelled and non-tunnelled catheters. CRI does not seem to be more severe in patients with diabetes. Duration of use of the HD catheter (p < 0.007) and ferritin level (p < 0.0001) were independent factors that predispose to CRI in our population.
Fulminant lupus pneumonitis is a rare complication of SLE. We report a case of 75 years‐old male patient with SLE who developed pneumonia and severe respiratory failure requiring mechanical ...ventilation. Refractory respiratory distress complicating noninfectious fulminant lupus pneumonitis did not respond to methylprednisolone and intravenous immunoglobulin treatment.
FLP in SLE of the elderly is a rare and severe condition. Each practitioner has to focus on respiratory failure, which is severe and life‐threatening.
Autosomal recessive polycystic kidney disease (ARPKD) is the most common childhood-onset ciliopathy. Intracranial aneurysms (ICA) are a serious complication of autosomal dominant polycystic kidney ...disease (ADPKD). However, there are only three reports about ICA in an adult patient with ARPKD. We describe a rare case of a 29-year-old man with ARPKD presenting with subarachnoid hemorrhage secondary to a ruptured intracranial aneurysm. The diagnosis of ARPKD was at the age of eight years based on typical ultrasonography findings with polycystic kidneys and liver disease. Magnetic resonance cholangiography showed a nonobstructive dilatation of intrahepatic bile ducts. Liver biopsy showed hepatic fibrosis. None of the family members was affected. At the age of 15 years, he had progressed to end-stage kidney disease, and hemodialysis was started. The patient had always a severe arterial hypertension. At the age of 29 years, he complained of headaches with an uncontrolled hypertension and disturbance of consciousness, computed tomography angiography showed subarachnoid hemorrhage and multiple cerebral aneurysms. Early neurologic screening of intracranial aneurysm should be recommended in ARPKD like in ADPKD patients.
Thalidomide, which is an angiogenesis inhibitor and immunomodulator that reduces tumor necrosis factor-alpha, has regained value in the treatment of multiple myeloma. Serious pulmonary complications ...due to thalidomide use remain relatively uncommon. We describe a case of bronchiolitis obliterans organizing pneumonia (BOOP) due to thalidomide. A 51-year-old man with IgG lambda myeloma was treated with thalidomide and dexamethasone. Seven days after the beginning of chemotherapy, the patient presented a fever and a persistent cough. Auscultation revealed crackles in both pulmonary bases. The chest X-ray showed a diffuse bilateral alveolar-interstitial syndrome. Computed tomography scan revealed bilateral pulmonary involvement, with bilateral interstitial alveolar infiltration and ground-glass pattern consolidations. Pulmonary infection, malignant tumor, and lung involvement of multiple myeloma were excluded through various tests. Thalidomide-induced BOOP was suspected, and the drug was withdrawn and replaced by Melphalan. The patient had complete resolution of his symptoms and radiologic pulmonary involvement on discontinuation of the drug. In the absence of other etiologies, physicians should be cognizant of this potential complication in patients receiving thalidomide who present with respiratory symptoms.
Takayasu arteritis is a rare and chronic granulomatous vasculitis that affects the large vessels. Takayasu arteritis targets the aorta and its branches and is still of unknown etiology. It often ...affects female patients under 50 years of age. A relationship between Takayasu arteritis and tuberculosis has been suggested for a long time.
We report a severe case of Takayasu arteritis in a 10-year-old Tunisian child revealed by renovascular hypertension with concomitant pulmonary tuberculosis.
Our patient is among only a few cases of Takayasu arteritis published worldwide affecting young infants and adolescents, which underlines the strong relationship between Takayasu arteritis and tuberculosis.
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