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Innere Medizin (Heidelberg, Germany) Oct 2023A 40-year-old Syrian man presented to the emergency department with a 5-day history of anuria. He had previously excreted dark urine. Major rhabdomyolysis and crush...
A 40-year-old Syrian man presented to the emergency department with a 5-day history of anuria. He had previously excreted dark urine. Major rhabdomyolysis and crush kidney were found, meaning that hemodialysis was immediately initiated. A detailed patient history in the patient's mother tongue revealed indications of metabolic myopathy. The PYGM-associated glycogen storage disease type V (McArdle disease) was confirmed by next generation sequencing panel diagnostics. The most important treatment approach is to avoid rhabdomyolysis through only moderate physical exertion.
Topics: Male; Humans; Adult; Rhabdomyolysis; Glycogen Storage Disease Type V; Muscular Diseases; Physical Exertion; Metabolism, Inborn Errors
PubMed: 37296329
DOI: 10.1007/s00108-023-01539-5 -
Annals of Medicine and Surgery (2012) Nov 2023Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis is a rare co-occurrence with systemic sclerosis, in around 2.5-9% of patients. The clinical...
INTRODUCTION AND IMPORTANCE
Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis is a rare co-occurrence with systemic sclerosis, in around 2.5-9% of patients. The clinical manifestations and prognosis of vasculitis in systemic sclerosis depend on organ involvement. It presented with rapidly progressive acute renal failure without malignant hypertension, and with pitting hand and foot ulcers get along with purpuric vasculitis in some cases reports. Reports had found that survival in those with pulmonary-renal syndrome is poor. However, high-dose corticosteroids and cyclophosphamide increase the survival percent in those patients.
CASE PRESENTATION
An 81-year-old female was admitted for newly diagnosed acute renal failure and highly elevated C-reactive protein levels. She was diagnosed with systemic sclerosis 8 years previously, with a 3-year history of interstitial lung disease, and a 2-year history of pulmonary hypertension. Treatment included home oxygen on demand, prednisone 5 mg/day, and azathioprine 75 mg daily. On physical examination, she had sclerodactyly, both extremities ulcers, severe livedo reticularis, and hyperpigmented papules on her hand and feet. Laboratory findings included a markedly positive MPO (p-ANCA), and anti-Scl-70. She was treated with pulse methylprednisolone without any improvement. After a day, she developed anuria and became comatose. Then, she developed cardiac arrest, leading to death.
CLINICAL DISCUSSION
The presence of ANCA in systemic sclerosis patients ranges from 2.5 to 9% of systemic sclerosis patients. It presented with rapidly progressive acute renal failure without malignant hypertension, and with pitting hand and foot ulcers. The treatment with high-dose corticosteroids and cyclophosphamide is benefit. Survival in those with pulmonary-renal syndrome is poor.
CONCLUSION
The presence of ANCA-associated vasculitis is rarely reported with scleroderma. It occurs most commonly in women with limited or Calcinosis, Raynaud phenomenon, Esophageal dysmotility, Sclerodactyly, and Telangiectasia (CREST) variants of scleroderma, as well as those with overlap features. Severe manifestations including pulmonary-renal syndrome and death may occur.
PubMed: 37915665
DOI: 10.1097/MS9.0000000000001347 -
Clinical Nephrology. Case Studies 2023We present two atypical cases of calciphylaxis presenting with ocular ischemic pathology - both without the hallmark cutaneous manifestations - to raise awareness of...
PURPOSE
We present two atypical cases of calciphylaxis presenting with ocular ischemic pathology - both without the hallmark cutaneous manifestations - to raise awareness of this rare yet highly disabling condition.
OBSERVATIONS
We report two cases of ophthalmic calciphylaxis presenting as (1) anterior ischemic optic neuropathy (AION) and cilioretinal artery occlusion in a 76-year-old woman with pre-dialysis kidney failure, and (2) AION with contralateral central retinal artery occlusion (CRAO) in a 44-year-old man on hemodialysis.
CONCLUSION AND IMPORTANCE
These cases highlight the need for judicious clinical suspicion of calciphylaxis in patients with kidney failure, presenting with microvascular ischemic ophthalmic pathology such as AION or CRAO. Confirmation with temporal artery biopsy is essential to direct targeted individualized multi-disciplinary treatment of calciphylaxis and avoid unnecessary steroid exposure in cases masquerading as giant cell arteritis (GCA).
PubMed: 38169875
DOI: 10.5414/CNCS111088 -
Peritoneal Dialysis International :... Sep 2023Incremental peritoneal dialysis (PD), defined as less than Full-dose PD prescription, has several possible merits, including better preservation of residual kidney... (Randomized Controlled Trial)
Randomized Controlled Trial
BACKGROUND
Incremental peritoneal dialysis (PD), defined as less than Full-dose PD prescription, has several possible merits, including better preservation of residual kidney function (RKF), lower peritoneal glucose exposure and reduced risk of peritonitis. The aims of this study were to analyse the association of Incremental and Full-dose PD strategy with RKF and urine volume (UV) decline in patients commencing PD.
METHODS
Incident PD patients who participated in the balANZ randomised controlled trial (RCT) (2004-2010) and had at least one post-baseline RKF and UV measurement was included in this study. Patients receiving <56 L/week and ≥56 L/week of PD fluid at PD commencement were classified as Incremental and Full-dose PD, respectively. An alternative cut-point of 42 L/week was used in a sensitivity analysis. The primary and secondary outcomes were changes in measured RKF and daily UV, respectively.
RESULTS
The study included 154 patients (mean age 57.9 ± 14.1 years, 44% female, 34% diabetic, mean follow-up 19.5 ± 6.6 months). Incremental and Full-dose PD was commenced by 45 (29.2%) and 109 (70.8%) participants, respectively. RKF declined in the Incremental group from 7.9 ± 3.2 mL/min/1.73 m at baseline to 3.2 ± 2.9 mL/min/1.73 m at 24 months ( < 0.001), and in the Full-dose PD group from 7.3 ± 2.7 mL/min/1.73 m at baseline to 3.4 ± 2.8 mL/min/1.73 m at 24 months ( < 0.001). There was no difference in the slope of RKF decline between Incremental and Full-dose PD ( = 0.78). UV declined from 1.81 ± 0.73 L/day at baseline to 0.64 ± 0.63 L/day at 24 months in the Incremental PD group ( < 0.001) and from 1.38 ± 0.61 L/day to 0.71 ± 0.46 L/day in the Full-dose PD group ( < 0.001). There was no difference in the slope of UV decline between Incremental and Full-dose PD ( = 0.18).
CONCLUSIONS
Compared with Full-dose PD start, Incremental PD start is associated with similar declines in RKF and UV.
Topics: Female; Humans; Adult; Middle Aged; Aged; Male; Peritoneal Dialysis; Glomerular Filtration Rate; Dialysis Solutions; Peritoneum; Kidney; Kidney Failure, Chronic
PubMed: 37259236
DOI: 10.1177/08968608231175826 -
Cureus Dec 2023Teratogenic agents have been shown to have drastic and detrimental effects on fetuses if exposed to the agent during uterine life. The most sensitive time for a... (Review)
Review
Teratogenic agents have been shown to have drastic and detrimental effects on fetuses if exposed to the agent during uterine life. The most sensitive time for a developing fetus is during the first trimester, and teratogenic exposure during this time can lead to severe deformities in the fetus. The Food and Drug Administration has categorized teratogenic agents based on the severity of their effect on the fetus; these categories include A, B, C, D, and X. Category A is the safest, with the most dangerous, and highly contraindicated in pregnant patients being Category X. This review article will discuss the teratogenic agents leflunomide, isotretinoin, thalidomide, warfarin, tetracycline, and angiotensinogen-converting enzyme inhibitors. Leflunomide can cause cranioschisis, exencephaly, and vertebral, head, and limb malformations. Isotretinoin's main teratogenic effects include central nervous system malformations, hydrocephalus, eye abnormalities, cardiac septal defects, thymus abnormalities, spontaneous abortions, and external ear abnormalities. Thalidomide has been shown to cause limb deformities, bowel atresia, and heart defects when the embryo is exposed to the agent during development. Warfarin can lead to spontaneous abortion and intrauterine death, as well as nasal hypoplasia, hypoplasia of extremities, cardiac defects, scoliosis, and mental retardation when exposed in utero. Tetracycline's teratogenic effects include gastrointestinal distress, esophageal ulceration and strictures, teeth discoloration, hepatotoxicity, and calcifications. Angiotensinogen-converting enzyme inhibitors can cause skull hyperplasia, anuria, hypotension, renal failure, lung hypoplasia, skeletal deformation, oligohydramnios, and fetal death. Teratogenic effects can be avoided if the pregnant patient is educated on the teratogenic effects of these agents.
PubMed: 38222129
DOI: 10.7759/cureus.50465 -
Journal of Clinical Medicine Jul 2023Unlike other adverse drug reactions, visceral organ involvement is a prominent feature of drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome and... (Review)
Review
Unlike other adverse drug reactions, visceral organ involvement is a prominent feature of drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome and correlates with mortality. The aim of this study was to systematically review cases published in PubMed-indexed, peer-reviewed journals in which patients had renal injury during the episode of DRESS syndrome (DS). We found 71 cases, of which 67 were adults and 56% were males. Female sex was associated with higher mortality. Chronic kidney disease (CKD) was present in 14% of patients who developed acute kidney injury (AKI) during DS. In 21% of cases, the kidneys were the only visceral organ involved, while 54% of patients had both liver and kidney involvement. Eosinophilia was absent in 24% of patients. The most common classes of medication associated with renal injury in DS were antibiotics in 34%, xanthine oxidase inhibitors in 15%, and anticonvulsants in 11%. Among antibiotics, vancomycin was the most common culprit in 68% of patients. AKI was the most common renal manifestation reported in 96% of cases, while isolated proteinuria or hematuria was present in only 4% of cases. In cases with AKI, 88% had isolated increase in creatinine and decrease in glomerular filtration (GFR), 27% had AKI concomitantly with proteinuria, 18% had oliguria, and 13% had concomitant AKI with hematuria. Anuria was the rarest manifestation, occurring in only 4% of patients with DS. Temporary renal replacement therapy was needed in 30% of cases, and all but one patient fully recovered renal function. Mortality of DS in this cohort was 13%, which is higher than previously reported. Medication class, latency period, or pre-existing CKD were not found to be associated with higher mortality. More research, particularly prospective studies, is needed to better recognize the risks associated with renal injury in patients with DS. The development of disease-specific biomarkers would also be useful so DS with renal involvement can be easier distinguished from other eosinophilic diseases that might affect the kidney.
PubMed: 37510691
DOI: 10.3390/jcm12144576 -
Kidney International Sep 2023Avoiding excessive dialysis-associated volume depletion may help preserve residual kidney function (RKF). To establish whether knowledge of the estimated normally...
Bio-impedance spectroscopy added to a fluid management protocol does not improve preservation of residual kidney function in incident hemodialysis patients in a randomized controlled trial.
Avoiding excessive dialysis-associated volume depletion may help preserve residual kidney function (RKF). To establish whether knowledge of the estimated normally hydrated weight from bioimpedance measurements (BI-NHW) when setting the post-hemodialysis target weight (TW) might mitigate rate of loss of RKF, we undertook an open label, randomized controlled trial in incident patients receiving HD, with clinicians and patients blinded to bioimpedance readings in controls. A total of 439 patients with over 500 ml urine/day or residual GFR exceeding 3 ml/min/1.73m were recruited from 34 United Kingdom centers and randomized 1:1, stratified by center. Fluid assessments were made for up to 24 months using a standardized proforma in both groups, supplemented by availability of BI-NHW in the intervention group. Primary outcome was time to anuria, analyzed using competing-risk survival models adjusted for baseline characteristics, by intention to treat. Secondary outcomes included rate of RKF decline (mean urea and creatinine clearance), blood pressure and patient-reported outcomes. There were no group differences in cause-specific hazard rates of anuria (0.751; 95% confidence interval (0.459, 1.229)) or sub-distribution hazard rates (0.742 (0.453, 1.215)). RKF decline was markedly slower than anticipated, pooled linear rates in year 1: -0.178 (-0.196, -0.159)), year 2: -0.061 (-0.086, -0.036)) ml/min/1.73m/month. Blood pressure and patient-reported outcomes did not differ by group. The mean difference agreement between TW and BI-NHW was similar for both groups, Bioimpedance: -0.04 kg; Control: -0.25 kg. Thus, use of a standardized clinical protocol for fluid assessment when setting TW is associated with excellent preservation of RKF. Hence, bioimpedance measurements are not necessary to achieve this.
Topics: Humans; Anuria; Dielectric Spectroscopy; Renal Dialysis; Urea; Kidney; Kidney Failure, Chronic; Randomized Controlled Trials as Topic
PubMed: 37263353
DOI: 10.1016/j.kint.2023.05.016 -
Kidney Research and Clinical Practice Aug 2023This study was performed to investigate the feasibility of incremental peritoneal dialysis (iPD) in older patients.
BACKGROUND
This study was performed to investigate the feasibility of incremental peritoneal dialysis (iPD) in older patients.
METHODS
In this retrospective cohort study, we enrolled peritoneal dialysis (PD) patients with age ≥ 60 years old at our center from 2008 to 2017. The patients were divided into two groups based on the daily PD exchanges: iPD group (≤3 × 2 L exchanges), and full dose group (≥4 × 2 L exchanges). Kaplan-Meier curves and multivariate Cox regression models were applied to evaluate the risks of anuria and mortalities between groups.
RESULTS
A total of 238 patients (186 in full dose group and 52 in iPD group) were enrolled. The mean age was 67.8 ± 5.7 years, and 45.8% were females. The baseline glomerular filtration rate was 4.15 ± 2.39 mL/min/1.73 m2 . Multivariate Cox regression models showed that patients in the iPD group patients had significantly decreased risk of anuria (hazard ratio [HR], 0.44; 95% confidence interval [CI], 0.24-0.81; p = 0.008), and all-cause mortality (HR, 0.59; 95% CI, 0.36-0.98; p = 0.04). Additionally, the incidence of peritonitis was significantly lower in the iPD group than that in the full dose group (0.115 vs. 0.197 episodes per person-year, p = 0.03) during the 36 months of PD commencement.
CONCLUSION
Older patients with iPD were independently associated with better preservation of residual kidney function and survival outcomes. Moreover, iPD regimens are also associated with reduced incidence of peritonitis. The iPD strategy might offer a feasible option for older patients.
PubMed: 37559224
DOI: 10.23876/j.krcp.22.202 -
Diagnostics (Basel, Switzerland) Nov 2023(1) Background: Uremic pruritus (UP) is a common and taxing symptom in patients on maintenance hemodialysis (MHD). We have previously shown that blood lead levels (BLLs)...
(1) Background: Uremic pruritus (UP) is a common and taxing symptom in patients on maintenance hemodialysis (MHD). We have previously shown that blood lead levels (BLLs) and blood aluminum levels (BALs) were separately positively associated with UP in MHD patients. We also found that blood cadmium levels (BCLs) were positively associated with all-cause mortality and cardiovascular-related mortality in MHD patients. We wondered whether there is any correlation between BCLs and UP after adjusting for BLLs and BALs. (2) Methods: Patients enrolled in this study were all from three hemodialysis (HD) centers at Chang Gung Memorial Hospital, Lin-Kou Medical Center, including both the Taipei and Taoyuan branches. Correlations between UP and BLLs, BALs, BCLs, and other clinical data were analyzed. (3) Results: Eight hundred and fifty-three patients were recruited. Univariate logistic regressions showed that diabetes mellitus, hepatitis B virus infection, hepatitis C virus infection, HD duration, hemodiafiltration, dialysis clearance of urea, normalized protein catabolic rate, non-anuria, serum albumin levels, log (intact-parathyroid hormone levels), total serum cholesterol levels, serum low-density lipoprotein levels, log (blood aluminum levels), and log (blood lead levels) were associated with UP. Although log BCLs were not significantly associated with UP ( = 0.136) in univariate analysis, we still included log BCLs in multivariate logistic regression to verify their effect on UP given that our aim in this study was to verify associations between serum heavy metals and UP. Multivariate logistic regressions showed that log BLLs (OR: 27.556, 95% CI: 10.912-69.587, < 0.001) and log BALs (OR: 5.485, 95% CI: 2.985-10.079, < 0.001) were positively associated with UP. The other logistic regression, which stratified BLLs and BALs into high and low BLLs and BALs, respectively, showed that high BLLs or high BALs (low BLLs and low BALs as reference) (OR: 3.760, 95% CI: 2.554-5.535, < 0.001) and high BLLs and high BALs combined (low BLLs and low BALs as reference) (OR: 10.838, 95% CI: 5.381-21.828, < 0.001) were positively correlated with UP. (4) Conclusions: BLLs and BALs were positively correlated with UP. BCLs were not correlated with UP. Clinicians should pay more attention to the environmental sources of lead and aluminum to prevent UP.
PubMed: 38066806
DOI: 10.3390/diagnostics13233565 -
Clinical Kidney Journal Sep 2023The combination of anti-glomerular basement membrane (GBM) disease and immunoglobulin A nephropathy (IgAN) has been well documented in sporadic cases, but lacks overall...
BACKGROUND
The combination of anti-glomerular basement membrane (GBM) disease and immunoglobulin A nephropathy (IgAN) has been well documented in sporadic cases, but lacks overall assessment in large collections. Herein, we investigated the clinical and immunological characteristics and outcome of this entity.
METHODS
Seventy-five consecutive patients with biopsy-proven anti-GBM disease from March 2012 to March 2020 were screened. Among them, patients with concurrent IgAN were identified and enrolled. The control group included biopsied classical anti-GBM patients during the same period, excluding patients with IgAN, other glomerular diseases or tumors, or patients with unavailable blood samples and missing data. Serum IgG and IgA autoantibodies against GBM were detected by enzyme-linked immunosorbent assay, as were circulating IgG subclasses against GBM.
RESULTS
Fifteen patients with combined anti-GBM disease and IgAN were identified, accounting for 20% (15/75) of all patients. Among them, nine were male and six were female, with an average (± standard deviation) age of 46.7 ± 17.3 years. Thirty patients with classical anti-GBM disease were enrolled as controls, with 10 males and 20 females at an average age of 45.4 ± 15.3 years. Patients with combined anti-GBM disease and IgAN had restricted kidney involvement without pulmonary hemorrhage. Compared with classical patients, anti-GBM patients with IgAN presented with significantly lower levels of serum creatinine on diagnosis (6.2 ± 2.9 vs 9.5 ± 5.4 mg/dL, = .03) and less occurrence of oliguria/anuria (20%, 3/15 vs 57%, 17/30, = .02), but more urine protein excretion [2.37 (1.48, 5.63) vs 1.11 (0.63, 3.90) g/24 h, = .01]. They showed better kidney outcome during follow-up (ESKD: 47%, 7/15 vs 80%, 24/30, = .03). The autoantigen and epitope spectrum were comparable between the two groups, but the prevalence of circulating anti-α3(IV)NC1 IgG1 (67% vs 97%, = .01) and IgG3 (67% vs 97%, = .01) were lower in patients with IgAN.
CONCLUSIONS
Concurrent IgAN was not rare in anti-GBM disease. Patients showed milder kidney lesions and better recovery after immunosuppressive therapies. This might be partly explained by lower prevalence of anti-GBM IgG1 and IgG3 in these patients.
PubMed: 37664576
DOI: 10.1093/ckj/sfad068