Hydralazine Induces Myeloperoxidase and Proteinase 3 Anti-Neutrophil Cytoplasmic Antibody Vasculitis and Leads to Pulmonary Renal Syndrome

We report a case of hydralazine-induced ANCA-associated glomerulonephritis with pulmonary hemorrhage. A 62-year-old Hispanic man with hypertension, who was being treated with hydralazine 100 mg three times a day for four and half years, presented to the hospital with severe anemia. He had acute kidney injury and urinalysis showed proteinuria, dysmorphic RBCs, and rare RBC cast. CT scan of the chest revealed bilateral pulmonary ground-glass infiltrates. Transbronchial biopsy was consistent with pulmonary hemorrhage. Serologic tests showed high titer PR3 ANCA and, to a lesser extent, MPO ANCA. Kidney biopsy revealed focal segmental necrotizing glomerulonephritis with crescents, without evidence of immune complex deposits. Hydralazine was discontinued and the patient was treated with corticosteroids and intravenous cyclophosphamide. At one-year follow-up, he had no symptoms and anemia had resolved. Kidney function improved dramatically. Serology showed undetectable PR3 ANCA and minimally elevated MPO ANCA. To our knowledge, hydralazine-associated PR3 ANCA has not been previously reported. The possibility of ANCA systemic vasculitis should be included in the differential diagnosis of any patient with hydralazine use and pulmonary renal syndrome. This is a potentially life threatening condition requiring prompt cessation of the drug and treatment with glucocorticoids and immunosuppression.

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Successful treatment of pulmonary mucormycosis caused by Rhizopus microsporus with posaconazole

European Journal of Medical Research ◽

10.1186/s40001-021-00602-x ◽

2021 ◽

Vol 26 (1) ◽

Author(s):

F. Yuan ◽

J. Chen ◽

F. Liu ◽

Y. C. Dang ◽

Q. T. Kong ◽

...

Keyword(s):

Fungal Culture ◽

Rhizopus Microsporus ◽

Pulmonary Tissue ◽

Case Presentation ◽

Common Cause ◽

Pulmonary Mucormycosis ◽

Threatening Condition ◽

Life Threatening ◽

One Year

Abstract Background Mucormycosis is a rare fungal infection occurring chiefly in the lung or the rhino-orbital-cerebral compartment, particularly in patients with immunodeficiency or diabetes mellitus. Among Mucorales fungi, Rhizopus spp. are the most common cause of mucormycosis. Case presentation We report a case of pulmonary mucormycosis caused by Rhizopus microsporus in a young patient with diabetes but no other apparent risk factors. The diagnosis mainly relied on clinical manifestation, positive pulmonary tissue biopsy, and fungal culture. The patient was successfully treated with posaconazole oral suspension and remains asymptomatic at one-year follow-up. Conclusions Pulmonary mucormycosis is a life-threatening condition and posaconazole is an effective treatment for pulmonary mucormycosis caused by Rhizopus microspores.

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Successful Treatment of Pulmonary Mucormycosis Caused by Rhizopus Microsporus With Posaconazole

10.21203/rs.3.rs-302265/v1 ◽

2021 ◽

Author(s):

Fan Yuan ◽

Jun Chen ◽

Fang Liu ◽

Yongchao Dang ◽

Qingtao Kong ◽

...

Keyword(s):

Fungal Culture ◽

Rhizopus Microsporus ◽

Pulmonary Tissue ◽

Case Presentation ◽

Common Cause ◽

Pulmonary Mucormycosis ◽

Threatening Condition ◽

Life Threatening ◽

One Year

Abstract Background: Mucormycosis is a rare fungal infection occurring chiefly in the lung or the rhino-orbital-cerebral compartment, particularly in patients with immunodeficiency or diabetes mellitus. Among Mucorales fungi, Rhizopus spp. are the most common cause of mucormycosis. Case presentation: We report the case of pulmonary mucormycosis caused by Rhizopus microsporus in a young patient with diabetes but no other apparent risk factors. The diagnosis has mainly relied on clinical manifestation, positive pulmonary tissue biopsy, and fungal culture. The patient was successfully treated with posaconazole oral suspension and remains asymptomatic at one-year follow-up.Conclusions: Pulmonary mucormycosis is a life-threatening condition and based on direct microscopy, histopathology, and culture for the diagnosis.

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A Toddler Presenting with Pulmonary Renal Syndrome

Case Reports in Nephrology and Dialysis ◽

10.1159/000477662 ◽

2017 ◽

Vol 7 (2) ◽

pp. 73-80 ◽

Cited By ~ 3

Author(s):

Florence A. Aeschlimann ◽

Rae S.M. Yeung ◽

Ronald M. Laxer ◽

Diane Hebert ◽

Ashley Cooper ◽

...

Keyword(s):

Lupus Erythematosus ◽

Treatment Options ◽

Renal Involvement ◽

Pulmonary Hemorrhage ◽

Disease Systemic Lupus Erythematosus ◽

Threatening Condition ◽

Life Threatening ◽

History Of ◽

Pulmonary Renal Syndrome ◽

Clinically Significant

Pulmonary renal syndrome refers to an association of pulmonary and glomerular disease and includes disorders, such as the ANCA-associated vasculitides, anti-glomerular basement membrane antibody disease, systemic lupus erythematosus, and IgA vasculitis (Henoch-Schönlein purpura). We present the medical history of a 26-month-old boy with an extensive purpuric rash, involving the limbs, trunk, and face, who developed clinically significant pulmonary hemorrhage and renal involvement. Rapid recognition of this rare but potentially life-threatening condition is crucial. In this report, we discuss the differential diagnosis, diagnostic studies, and treatment options to consider when facing a young child presenting with a pulmonary renal syndrome.

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Patient with Antineutrophil Cytoplasmic Antibody Associated Small Vessel Vasculitis, Acute Renal Failure, and Coronavirus Disease-19 Pneumonia: A Diagnostic and Therapeutic Challenge

Open Access Macedonian Journal of Medical Sciences ◽

10.3889/oamjms.2020.5510 ◽

2020 ◽

Vol 8 (T1) ◽

pp. 542-547

Author(s):

Biljana Gerasimovska-Kitanovska ◽

Gjulshen Selim ◽

Zvezdana Petronijevik ◽

Blagica Pecanova ◽

Gjorgi Stojchev ◽

...

Keyword(s):

Renal Failure ◽

Acute Renal Failure ◽

Systemic Vasculitis ◽

Renal Involvement ◽

Rapidly Progressive Glomerulonephritis ◽

Pulmonary Hemorrhage ◽

Small Vessel ◽

Small Vessel Vasculitis ◽

Necrotizing Glomerulonephritis ◽

Pulmonary Renal Syndrome

BACKGROUND: Antineutrophil cytoplasmatic antibody (ANCA)-associated vasculitis (AAV) has a predilection for the kidney and more than three quarters of patients have renal involvement with rapidly progressive glomerulonephritis. Small-vessel systemic vasculitis may present as pulmonary-renal syndrome and is characterized by necrotizing glomerulonephritis and pulmonary hemorrhage. Diagnosis and therapy for AAV in coronavirus disease (COVID) COVID-19 pandemic require multi-disciplinary collaboration due to the affection of multiple systems and risks associated with immunosuppressive medications. CASE REPORT: A 69-year-old non-smoker, non-diabetic female presented in the outpatient unit at the department of pulmonology with dry cough, malaise, and sub-febrile temperature, lasting for 1 month. The patient had a high suspicion of severe pulmonary-renal syndrome, ANCA-AAV, and acute renal failure requiring hemodialysis. She was treated with corticosteroids, cyclophosphamide, and plasma exchange. The treatment led to temporary improvement. Infections with COVID-19, Enterococcus in the urine, and Acinetobacter in the tracheal aspirate further complicated the clinical picture and despite antibiotic treatment, use of tocilizumab and convalescent plasma, the outcome was lethal. CONCLUSION: It is important to establish the diagnosis and distinguish accurately between vasculitis and infection to provide adequate and timely therapy.

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Endoscopic follow-up of cyanoacrylate obliteration of gastric varices

Arquivos de Gastroenterologia ◽

10.1590/s0004-28032009000100020 ◽

2009 ◽

Vol 46 (1) ◽

pp. 81-84 ◽

Cited By ~ 7

Author(s):

Fernanda Prata Martins ◽

Erika Pereira de Macedo ◽

Gustavo Andrade de Paulo ◽

Frank Shigueo Nakao ◽

José Celso Ardengh ◽

...

Keyword(s):

Abdominal Pain ◽

Portal Hypertension ◽

Gastric Varices ◽

Study Endpoint ◽

Mild Abdominal Pain ◽

Threatening Condition ◽

Life Threatening ◽

Related Mortality ◽

Overall Mortality

Bleeding from gastric varices is a life-threatening condition. We report our experience with cyanoacrylate injection. Twenty three patients with portal hypertension and gastric varices underwent intra-variceal injection of a cyanoacrylate/lipiodol solution (1:1). Study endpoint was variceal obliteration. Mean follow-up was 25.3 months. Variceal obliteration was achieved in 87% of patients. Recurrence occurred in one patient (4.3%) and rebleeding in another case (4.3%). Mild abdominal pain was described in 13% of patients. Overall mortality was 21.7% and rebleeding related mortality rate was 4.3%. Our results confirm that cyanoacrylate injection is effective and safe to eradicate gastric varices.

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Abstract P147: Association Of Rosuvastatin Use With Risk Of Rhabdomyolysis Across Chronic Kidney Disease Status

Circulation ◽

10.1161/circ.143.suppl_1.p147 ◽

2021 ◽

Vol 143 (Suppl_1) ◽

Author(s):

Jung-Im Shin ◽

Yao Qiao ◽

Aditya Surapaneni ◽

Lesley Inker ◽

Derek Fine ◽

...

Keyword(s):

Chronic Kidney Disease ◽

Kidney Disease ◽

Proportional Hazards ◽

Disease Status ◽

Cox Proportional Hazards ◽

Lower Egfr ◽

Threatening Condition ◽

Life Threatening ◽

End Stage

Introduction: Statin-induced rhabdomyolysis is a rare, but potentially life-threatening condition. It is unknown whether specific statins carry a greater risk of rhabdomyolysis and whether the risk differs between patients with and without chronic kidney disease (CKD). The objective of this study was to investigate the association of rosuvastatin use vs. atorvastatin use with the risk of rhabdomyolysis across CKD status. Hypothesis: Rosuvastatin use is associated with a higher risk of rhabdomyolysis as compared to atorvastatin use and the risk is greater among those with CKD than those without CKD. Methods: We identified adult patients who initiated rosuvastatin or atorvastatin between January 1, 2004 and December 31, 2018 and were free of end-stage kidney disease at the time of prescription in the Geisinger Health System. The association between rosuvastatin use and rhabdomyolysis was assessed using Cox proportional hazards regression models with an interaction between rosuvastatin use and CKD (i.e., estimated glomerular filtration rate <60 ml/min/1.73 m 2 ) in an inverse probability of treatment weighted (IPTW) sample. Results: Of 8,748 rosuvastatin users (mean [SD] age, 59.7 [12.6] years; 49.8% female; 11.8% CKD) and 31,770 atorvastatin users (mean [SD] age, 59.1 [12.6] years; 48.2% female; 11.9% CKD), 0.7% and 0.4% patients developed rhabdomyolysis, respectively, during a median follow-up of 5.1 years. Rosuvastatin use was associated with a higher risk of rhabdomyolysis in patients with CKD (hazard ratio [HR], 3.29; 95% CI, 1.53-7.09), but not in those without CKD (HR, 1.29; 95% CI, 0.82-2.03; p-interaction=0.04). A higher risk of rhabdomyolysis associated with rosuvastatin use in lower eGFR was also observed in the analysis with continuous eGFR ( Figure ). Conclusions: The findings suggest that rosuvastatin use in patients with CKD may be associated with excess risk of rhabdomyolysis as compared to atorvastatin.

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P0709RENAL OUTCOMES AND MORTALITY FOLLOWING TRANSCATHETER AORTIC VALVE IMPLANTATION IN CHRONIC KIDNEY DISEASE PATIENTS

Nephrology Dialysis Transplantation ◽

10.1093/ndt/gfaa142.p0709 ◽

2020 ◽

Vol 35 (Supplement_3) ◽

Author(s):

Amir Shabaka ◽

Rafael Lucena Valverde ◽

Andres Escudero ◽

Gabriela Tirado-Conte ◽

Luis Nombela Franco

Keyword(s):

Kidney Disease ◽

Serum Creatinine ◽

Kidney Function ◽

Severe Aortic Stenosis ◽

Kidney Injury ◽

Transcatheter Aortic Valve ◽

Aortic Valve Implantation ◽

One Year ◽

Valve Implantation

Abstract Background and Aims Transcatheter aortic valve implantation (TAVI) is indicated for the treatment of patients with severe aortic stenosis with a high surgical risk that are rejected for surgical valve placement. The aim of this study is to evaluate the effect of TAVI on long-term kidney function in stage 3-4 chronic kidney disease (CKD) patients. Method We performed a single-center retrospective observational study of 529 consecutive patients that underwent TAVI between August 2007 and January 2018. We included patients with stage 3 or 4 CKD, with at least two stable measurements of glomerular filtration rate during the three months before TAVI. We excluded patients that presented with an acute kidney injury during the three months that preceded TAVI. Results 165 patients (31.3%) of the patients who underwent TAVI had a stable stage 3 or 4 CKD before implantation. Their mean age was 83.61 ±5.08 years, BMI of 27.79 ± 4.54 kg/m2, baseline serum creatinine of 1.62 ± 0.49 mg/dl for an eGFR measured by MDRD-4 of 40.19 ± 10.79 ml/min, EuroScore-II of 6 (IQR 4-8). 42 patients (28.6%) had a post-procedural acute kidney injury (AKI), 2 of them required temporary renal replacement therapy and 30 patients (71.4%) recovered to their baseline kidney function. Mean contrast media administered was 162.0 ±77.8 ml. Mean serum creatinine decreased from 1.64 ± 0.51 mg/dl to 1.46 ± 0.52 ml/min after 1 year of follow-up (p=0.032). Only 2.4% of patients showed a >50% increase in serum creatinine after 1 year. Median follow-up was 3.8 years (IQR 2.3-5.7 years) during which time no patient progressed to end-stage kidney disease. 33.3 % of patients died during follow-up, with a one-year survival rate of 87% and two-year survival of 82%. Post-interventional major bleeding (14.6 vs 5.8%, p<0.001) and the need for post-interventional pacemaker implantation (25 vs 13.3%, p<0,001)were the only risk factors associated with the development of AKI. Contrast volume was not associated with the development of AKI. AKI was associated with an increased in-hospital stay (13.2 vs 7.4 days, p<0.001), but was not associated with either a reduced kidney function at end of follow-up or an increased mortality (Log-rank X2=1.72, p=0,578). Conclusion Despite the high incidence of post-interventional AKI after TAVI, our study did not show an association between AKI and increased mortality or reduced renal survival. In most cases after AKI patients recovered to their baseline kidney function. There is an improvement in kidney function after one year of treatment of severe aortic stenosis with TAVI in patients with CKD Stage 3-4.

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P0599THE LYSOSOMAL URINE ENZYMES ACTIVITY IN CHILDREN AFTER ACUTE KIDNEY INJURI

Nephrology Dialysis Transplantation ◽

10.1093/ndt/gfaa142.p0599 ◽

2020 ◽

Vol 35 (Supplement_3) ◽

Author(s):

Olga Lavrenchuk ◽

Lesya Korol ◽

Ludmila Migal ◽

Ingretta Bagdasarova

Keyword(s):

Enzyme Activity ◽

Kidney Disease ◽

Lysosomal Enzymes ◽

Reference Group ◽

Kidney Injury ◽

Activity Levels ◽

Healthy Children ◽

Ckd Progression ◽

One Year

Abstract Background and Aims The urgency of the problem of acute kidney injury (AKI) in children is due to the high risk of chronic kidney disease (CKD) as an outcome. Lysosomal enzymes in urine are considered to be informative markers of renal parenchyma damage. The aim of study was to determine the activity of lysosomal enzymes β-galactosidase (GAL) and N-acetyl-β-D-glucosamynidase (NAG) in urine as markers of CKD progression in children after AKI. Method 41 children were examined after AKI with achieving self-diuresis and clinical improvement. The functional state of the kidneys was evaluated by the glomerular filtration rate (Schwartz GFR), the level of albuminuria. Urine enzyme activity was determined and cross-linked to mmol urinary creatinine. The control (reference) group consisted of 28 children who were conditionally healthy, without kidney disease. Results The NAG activity was 8 times higher level than in the reference group of healthy children (87/46;193 vs. 15/11;18, p<0.001), and GAL was elevated in 3 times either (34/22;45 vs. 10/6;13, p<0.001) in patients with a disease period of up to 2 years after AKI. In patients with follow-up period 2 or more years enzyme activity levels decreased, but remained 4 times higher (NAG: 49/31;109, p <0.001 compared to control; GAL: 35/25;44, p <0.001 compared to control). The high levels of enzyme activity was documented in 73.2% of patients after one year of follow up, but the GFR decrease was found in 22.0%, and albuminuria - only in 34.1%. Conclusion The urine activity of NAG and GAL in children is an informative marker for diagnosing recovery after AKI or CKD progression with renal function decline. This method is non-invasive, inexpensive, and simple to conduct.

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Tracheal development after left pulmonary artery reimplantation: an individual study

Scientific Reports ◽

10.1038/s41598-020-74890-4 ◽

2020 ◽

Vol 10 (1) ◽

Author(s):

Xiaoyang Hong ◽

Ruijie Li ◽

Zhe Zhao ◽

Jiangheng Guan ◽

Hui Wang ◽

...

Keyword(s):

Pulmonary Artery ◽

Pulmonary Arteries ◽

Left Pulmonary Artery ◽

Weaning Failure ◽

Threatening Condition ◽

Ventilation Weaning ◽

Life Threatening ◽

The Mean ◽

Tracheal Carina

Abstract Pulmonary artery sling (PA sling) often presents as a life-threatening condition requiring urgent surgical correction. We reported 32 cases of PA sling in children who were followed up postoperatively in the past 6 years. All patients with PA slings who were admitted to the hospital from January 2012 to December 2017 and underwent surgery were retrospectively analyzed. The mean age of the 32 patients at repair was 16.97 months (range, 15 days to 128 months). Six patients required ventilator assistance for respiratory failure. All children underwent left pulmonary artery (LPA) reimplantation (n = 32), and 3 patients needed reimplantation slide tracheoplasty (n = 3) due to ventilation weaning failure. Four patients died, 27 patients survived until discharge, and 18 patients were followed up. Pulmonary computed tomography imaging and echocardiography were performed in 18 patients who were followed up. After LPA reimplantation, the tracheal carina area was significantly enlarged compared to that preoperation (p = 0.0002). In this follow-up cohort study, 75% of the patients who underwent LPA reimplantation survived until discharge. The survivors had subsequently well-developed pulmonary arteries and tracheas.

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