- Atypical presentation of atypical haemolytic uraemic syndrome. [Journal Article]
- BCBMJ Case Rep 2018 Feb 11; 2018
- A 17-year-old girl presented with fever, myalgia, vomiting for 1 month and oliguria and dyspnoea for 4 days. She was tachycardic,hypertensive, with pedal oedema and decreased breath sounds. She had h...
A 17-year-old girl presented with fever, myalgia, vomiting for 1 month and oliguria and dyspnoea for 4 days. She was tachycardic,hypertensive, with pedal oedema and decreased breath sounds. She had high serum creatinine (3 mg/dL), anaemia, thrombocytopenia, leucocytosis and eosinophilia with schistocytes. Lactate dehydrogenase, transaminases were high , with low haptoglobin and high ferritin (5269 ng/mL). Complement C3/C4 and fibrinogen were normal. Urinalysis showed large blood and protein and stool studies were negative. Her ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) was normal. Kidney biopsy showed acute interstitial nephritis (AIN) in addition to thrombotic angiopathy. The differentials - haemolytic uraemic syndrome (HUS), thrombotic thrombocytopenia (TTP) and haemophagocytic lymphohistiocytosis (HLH) were ruled out. Her genetic testing was abnormal for large CFHR1-CFHR3 homozygous deletion and heterozygous missense variant in exon 2 of DGKE making the diagnosis of atypical HUS. She received eculizumab and was discharged on oral steroids for AIN and biweekly eculizumab infusions with excellent recovery.
- Chronic kidney disease mortality trends in selected Central America countries, 1997-2013: clues to an epidemic of chronic interstitial nephritis of agricultural communities. [Journal Article]
- JEJ Epidemiol Community Health 2018 Feb 02
- CONCLUSIONS: This study provides the most comprehensive mortality analysis of this epidemic published to date and confirms an excess of CKD-N18 mortality and its relation with the epidemic of CINAC. The overall trends and the mortality pattern among women, children and adolescents suggest that the heat stress-dehydration hypothesis cannot fully explain this epidemic and that other environmental factors, more likely agricultural practices and agrochemicals, may be causally involved.
- Safety of Anti-Staphylococcal Penicillins versus Cefazolin: A Systematic Review and Meta-Analysis. [Journal Article]
- AAAntimicrob Agents Chemother 2018 Feb 05
- Recent studies and experience suggest that cefazolin might be as equally effective as anti-staphylococcal penicillins for methicillin-susceptibleStaphylococcus aureus(MSSA) with a better safety profi...
Recent studies and experience suggest that cefazolin might be as equally effective as anti-staphylococcal penicillins for methicillin-susceptibleStaphylococcus aureus(MSSA) with a better safety profile and lower cost. The objective of these meta-analyses was to compare the safety of anti-staphylococcal penicillins and cefazolin. PubMed, EMBASE, International Pharmaceutical Abstracts databases and websites for clinical trial registries through June 23, 2017 were searched. In addition, recent abstracts from infectious disease and pharmacy conferences were reviewed. We estimated Peto odds ratios (ORs) with 95% confidence intervals (CIs) using random-effects models. One analysis focused on hospitalized patients and the other on outpatients. Eleven retrospective studies of hospitalized patients and three of outpatients were included. In hospitalized patients, lower nephrotoxicity (Peto OR, 0.225; 95% CI, 0.127-0.513), acute interstitial nephritis (Peto OR, 0.189; 95% CI, 0.053-0.675), hepatotoxicity (Peto OR, 0.160; 95% CI, 0.066-0.387), and drug discontinuation due to adverse reactions (Peto OR, 0.192; 95% CI, 0.089-0.414) were found with cefazolin. In outpatients, lower nephrotoxicity (Peto OR, 0.372; 95% CI, 0.192-0.722), hepatotoxicity (Peto OR, 0.313; 95% CI, 0.156-0.627), and hypersensitivity reactions (Peto OR, 0.372; 95% CI, 0.201-0.687[rsqb] were observed with cefazolin. Compared to anti-staphylococcal penicillins, cefazolin was associated with significant reductions in nephrotoxicity and hepatotoxicity in hospitalized patients and outpatients. Additionally, cefazolin was associated with less likelihood of discontinuation due to side effects in hospitalized patients and hypersensitivity reactions in outpatients. Cefazolin should be considered a first-line option for patients with MSSA infections in which efficacy is presumed to be similar to anti-staphylococcal penicillin therapy.
- Successful use of rituximab in glomerular basement membrane nephritis associated with HIV interstitial nephritis secondary to Castleman disease . [Journal Article]
- CNClin Nephrol 2018 Feb 09
- We report a case of glomerular basement membrane crescentic glomerulonephritis and multicentric Castleman disease-associated interstitial nephritis in a patient with human immunodeficiency virus (HIV...
We report a case of glomerular basement membrane crescentic glomerulonephritis and multicentric Castleman disease-associated interstitial nephritis in a patient with human immunodeficiency virus (HIV) infection. The patient received corticosteroids, cyclophosphamide, and plasmapheresis, and within 3 weeks, there was worsening thrombocytopenia, anemia, and renal function requiring initiation of hemodialysis. He then received 8 weekly doses of rituximab, and there was steady improvement in renal function, such that he stopped dialysis within 6 weeks and has remained in disease remission at 1-year follow-up. This is the first case report of acute kidney injury caused by both antiglomerular basement membrane disease and multicentric Castleman disease, with a favorable response to rituximab. .
- The atypical chemokine receptor 2 limits renal inflammation and fibrosis in murine progressive immune complex glomerulonephritis. [Journal Article]
- KIKidney Int 2018 Jan 22
- The atypical chemokine receptor 2 (ACKR2), also named D6, regulates local levels of inflammatory chemokines by internalization and degradation. To explore potential anti-inflammatory functions of ACK...
The atypical chemokine receptor 2 (ACKR2), also named D6, regulates local levels of inflammatory chemokines by internalization and degradation. To explore potential anti-inflammatory functions of ACKR2 in glomerulonephritis, we induced autologous nephrotoxic nephritis in C57/BL6 wild-type and Ackr2-deficient mice. Renal ACKR2 expression increased and localized to interstitial lymphatic endothelium during nephritis. At two weeks Ackr2-/-mice developed increased albuminuria and urea levels compared to wild-type mice. Histological analysis revealed increased structural damage in the glomerular and tubulointerstitial compartments within Ackr2-/- kidneys. This correlated with excessive renal leukocyte infiltration of CD4+ T cells and mononuclear phagocytes with increased numbers in the tubulointerstitium but not glomeruli in knockout mice. Expression of inflammatory mediators and especially markers of fibrotic tissue remodeling were increased along with higher levels of ACKR2 inflammatory chemokine ligands like CCL2 in nephritic Ackr2-/- kidneys. In vitro, Ackr2 deficiency in TNF-stimulated tubulointerstitial tissue but not glomeruli increased chemokine levels. These results are in line with ACKR2 expression in interstitial lymphatic endothelial cells, which also assures efflux of activated leukocytes into regional lymph nodes. Consistently, nephritic Ackr2-/- mice showed reduced adaptive cellular immune responses indicated by decreased regional T-cell activation. However, this did not prevent aggravated injury in the kidneys of Ackr2-/- mice with nephrotoxic nephritis due to simultaneously increased tubulointerstitial chemokine levels, leukocyte infiltration and fibrosis. Thus, ACKR2 is important in limiting renal inflammation and fibrotic remodeling in progressive nephrotoxic nephritis. Hence, ACKR2 may be a potential target for therapeutic interventions in immune complex glomerulonephritis.
- Combined membranous nephropathy and tubulointerstitial nephritis as a rare renal manifestation of IgG4-related disease: a case-based literature review. [Journal Article]
- CCCEN Case Rep 2018 Feb 01
- IgG4-related disease (IgG4-RD) is a newly recognized immune-mediated multisystemic disease characterized by a fibro-inflammatory condition with tissue infiltration of IgG4-positive plasma cells and o...
IgG4-related disease (IgG4-RD) is a newly recognized immune-mediated multisystemic disease characterized by a fibro-inflammatory condition with tissue infiltration of IgG4-positive plasma cells and often associated with elevated serum IgG4 levels. Typical renal involvement of IgG4-RD presents as tubulointerstitial nephritis (TIN), membranous or membranoproliferative nephropathy. We are presenting a case with combined IgG4 membranous nephropathy and TIN, as well as a literature review on pathophysiology, diagnosis and treatment of IgG4-RD. A 62-year-old man presented with weight loss and fatigue. Labs showed significant proteinuria and hematuria with elevated serum creatinine (2.5 mg/dL). CT/PET scan found scattered lymphadenopathy without increased FDG uptake. Kidney biopsy showed glomerular lesions as well as severe interstitial fibrosis and tubular atrophy. Immunohistochemistry study was negative for anti-phospholipase A2 receptor antibodies and showed interstitial lymphocytic infiltration with IgG4 positive plasma cells. Patient also had elevated serum IgG4 level and IgG4 to total IgG ratio. Prednisone treatment was initiated soon after the diagnosis was made, patient responded well with proteinuria and hematuria both resolved. IgG4-related disease (IgG4-RD) is a newly increasingly recognized immune-mediated multisystemic disease; IgG4-related membranous nephropathy should be included in the differential diagnosis for patients with proteinuria.
- iRhom2 promotes lupus nephritis through TNF-α and EGFR signaling. [Journal Article]
- JCIJ Clin Invest 2018 Jan 25
- Lupus nephritis (LN) often results in progressive renal dysfunction. The inactive Rhomboid 2 (iRhom2) is a newly identified key regulator of A disintegrin and metalloprotease 17 (ADAM17), whose subst...
Lupus nephritis (LN) often results in progressive renal dysfunction. The inactive Rhomboid 2 (iRhom2) is a newly identified key regulator of A disintegrin and metalloprotease 17 (ADAM17), whose substrates, such as TNF-α and heparin-binding EGF (HB-EGF), have been implicated in the pathogenesis of chronic kidney disease. Here we demonstrate that deficiency of iRhom2 protects the lupus-prone Fcgr2b-/- mice from developing severe kidney damage without altering anti-double stranded (ds) DNA Ab production, by simultaneously blocking the HB-EGF/EGFR and the TNF-α signaling in the kidney tissues. Unbiased transcriptome profiling of kidneys and kidney macrophages revealed that TNF-α and HB-EGF/EGFR signaling pathways are highly upregulated in Fcgr2b-/- mice; alterations that were diminished in the absence of iRhom2. Pharmacological blockade of either TNF-α or EGFR signaling protected Fcgr2b-/- mice from severe renal damage. Finally, kidneys from LN patients showed increased iRhom2 and HB-EGF expression, with interstitial HB-EGF expression significantly associated with chronicity indices. Our data suggest that activation of iRhom2/ADAM17-dependent TNF-α and EGFR signaling plays a crucial role in mediating irreversible kidney damage in LN, thereby uncovering a novel target for selective and simultaneous dual inhibition of two major pathological pathways in the effector arm of the disease.
- [Renal involvement in amyloidosis and sarcoidosis]. [Journal Article]
- DMDtsch Med Wochenschr 2018; 143(2):101-109
- Amyloidosis is a rare disease characterized by extracellular deposition of fibrils. Among the most common forms of systemic amyloidosis with renal involvement are AL-amyloidosis based on plasma cell ...
Amyloidosis is a rare disease characterized by extracellular deposition of fibrils. Among the most common forms of systemic amyloidosis with renal involvement are AL-amyloidosis based on plasma cell dyscrasia and AA-amyloidosis in chronic inflammatory diseases. Depending on the affected renal compartment, the clinical appearance of renal amyloidosis varies. The pattern of renal amyloid deposition can be glomerular, interstitial, tubular or even vascular. Renal amyloid deposits are detected by renal biopsy. Patients with glomerular deposits typically show severe nephrotic syndrome with volume overload. Patients with predominantly tubulo-interstitial or vascular deposits typically exhibit lower proteinuria and progressive renal impairment. Treatment strategies for renal amyloidosis are primarily based on the treatment of the underlying disease including chemotherapy or stem cell transplantation in AL-amyloidosis or treatment of chronic inflammatory diseases in AA-amyloidosis. Granulomatous interstitial nephritis is the most common renal lesion occurring in sarcoidosis. Therapy of granulomatous interstitial nephritis is mainly based on the use of glucocorticoids.
- [Renal Involvement in Connective Tissue Diseases]. [Journal Article]
- DMDtsch Med Wochenschr 2018; 143(2):89-100
- Renal involvement is common and heterogenous in connective tissue diseases and has a main influence on prognosis and mortality. In systemic lupus erythematosus proliferative glomerulonephritis is the...
Renal involvement is common and heterogenous in connective tissue diseases and has a main influence on prognosis and mortality. In systemic lupus erythematosus proliferative glomerulonephritis is the most common manifestation, while in primary Sjogren's syndrome interstitial nephritis with tubular dysfunction is the predominant pathological feature. In systemic sclerosis the most serious renal manifestation is scleroderma renal crisis characterized by abrupt onset of hypertension and acute kidney injury associated with an increase in plasma renin activity. Risk factors for scleroderma renal crisis are diffuse cutaneous involvement, treatment with corticosteroids > 15 mg prednisolone/day and treatment with calcineurin inhibitors.Regular measurement of urine sediment, proteinuria-to-urine creatinine ratio, tubular proteinuria, measurement of plasma creatinine, and office as well as home blood pressure monitoring are strongly recommended. Diagnostic kidney biopsy is essential in differentiating the different types of lupus nephritis and renal involvement in sjogren's syndrome.The optimal treatment of lupus nephritis varies with the classification of the morphological findings present on renal biopsy. The treatment of interstitial nephritis in sjogren's syndrome consists of immunosuppression e. g. corticosteroids. Renal tubular acidosis should be corrected by sodium alkali and potassium alkali. Angiotensin-converting enzyme inhibitors play a major role in the treatment of scleroderma renal crisis, they should be continued also in patients progressing to end-stage renal disease.
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- Sunitinib-Induced Acute Interstitial Nephritis in a Thrombocytopenic Renal Cell Cancer Patient. [Journal Article]
- CRCase Rep Oncol Med 2017; 2017:6328204
- Sunitinib, a multitargeted tyrosine kinase inhibitor (TKI), is currently the standard of care for patients with metastatic renal cell carcinoma. Renal adverse events associated with sunitinib include...
Sunitinib, a multitargeted tyrosine kinase inhibitor (TKI), is currently the standard of care for patients with metastatic renal cell carcinoma. Renal adverse events associated with sunitinib include proteinuria, renal insufficiency secondary to focal segmental glomerulosclerosis (FSGS), and thrombotic microangiopathy. We describe the second reported instance of biopsy-proven sunitinib-induced acute interstitial nephritis (AIN), in a challenging case complicated by thrombocytopenia. The case illustrates the importance of early diagnosis and intervention in ensuring long-term recovery from renal complications. Four other cases of AIN reported along with inhibition of the vascular endothelial growth factor (VEGF) by either TKI (sunitinib and sorafenib) or antibodies (bevacizumab) suggest a possible class effect. Given our experience, we recommend monitoring renal function with VEGF inhibition, and in the case of renal failure in the setting of an unclear diagnosis, we recommend prompt biopsy.