Could mesangial C3 deposition be an independent prognostic marker in immunoglobulin A nephropathy?

BACKGROUND: Immunoglobulin A nephropathy (IgAN) is a common primary glomerulonephropathy. There is evidence that mesangial C3 deposition plays a role in the development of the disease. The aim of this study was to examine the effect of C3 deposition on the prognosis of IgAN patients. METHOD: The study included 1135 patients with biopsy-confirmed IgAN from the database of the Turkish Nephrology Association Glomerular Diseases Working Group (TSN-GOLD). Patients were excluded from the study if they were aged < 18 or > 75 years or if C3 staining had not been performed in the immunofluorescent analysis. C3 deposition was defined as an immunofluorescence intensity of C3 ≥ 2 + within the mesangium. The primary endpoints were the development of end-stage renal disease, a 30% decrease in glomerular filtration rate compared to the basal value or an elevation in proteinuria to a nephrotic level (3.5 gr/day). RESULTS: Mesangial C3 deposition was observed in 603 (53.1%) patients. No statistically significant difference was found at baseline between the groups with and without mesangial C3 deposition, as for age, sex, BMI, proteinuria level, or the presence of hypertension. In the follow-up period with a mean duration of 78 months, no significant difference was found between the two groups regarding the primary endpoints (p = 0.43). A significant correlation between C3 deposition and segmental glomerulosclerosis (S1) according to the Oxford MEST-C classification was found (p = 0.001). CONCLUSION: Although a correlation was observed between mesangial C3 deposition and the S1 MEST-C classification, mesangial C3 deposition was not a prognostic factor in IgAN.

De Novo Malignancies After Kidney Transplant: Where Do We Stand and Does the Head and Neck Region Require More Caution?

OBJECTIVES: The aim of this study was to investigate the characteristics of de novo malignancies arising in kidney transplant recipients followed in a tertiary hospital in Turkey and to examine the tumors in the head and neck region as a subgroup. MATERIALS AND METHODS: Data from kidney transplant recipients treated at our institution between January 2010 and July 2022 were retrospectively analyzed in this single-center study. Data regarding malignancies were noted according to the pathologists' reports. In situ malignancies and those arising after graft loss were not evaluated. RESULTS: The study population comprised 231 patients (165 men; 71.4%) with a median follow-up of 11 years (2853 patient-years). The recipients had a higher cancer risk than the general population (standardized incidence rate = 3.04; 95% CI, 1.82-4.26). Thirty de novo malignant tumors were detected in 24 patients (10.4%). The mean age at diagnosis of cancer was 54.88 ± 11.44 years. The median time from transplant to cancer diagnosis was 11.5 years (range, 7-18.8 y). Nonmelanoma skin cancers (56.7% of all tumors) were the most common malignancies. Twenty-two lesions (73.3%) that developed in 17 patients (7.4%) were localized to the head and neck region: 15 (68.2%) were cutaneous and 7 (31.8%) were noncutaneous. The median time from transplant to head and neck cancer diagnosis was 12 years (range, 7.5-17.5 y). Mortality rate was higher in cancer patients (10 [41.7%] vs 17 [8.2%]; P < 0.01). CONCLUSIONS: The incidence of de novo malignancy in kidney transplant recipients was relatively higher compared with previous data. Nonmelanoma skin cancers were the most common type. Three-quarters of all lesions were in the head and neck region, and two-thirds were of cutaneous origin.

The relationship between depression, anxiety, quality of life levels, and the chronic kidney disease stage in the autosomal dominant polycystic kidney disease.

PURPOSE: Decreased quality of life, anxiety, depression, and other negative psychosocial factors in autosomal dominant polycystic kidney disease (ADPKD) may lead to the patient's attitudes that reduce treatment effectiveness. We aimed to evaluate the relationship between the depression, anxiety, perceived social support, genetic psychosocial risk and quality of life levels, and chronic kidney disease (CKD) stage in ADPKD and to investigate the relationship between these variables/parameters and the dietary compliance that is an essential factor in the course of the disease. METHODS: 100 ADPKD patients were enrolled in this cross-sectional study. EuroQol-5D-3L (EQ-5D-3L) health-related quality of life index, EuroQol-5D-3L visual analog scale (EQ-5D-3L VAS), multidimensional scale of perceived social support (MSPSS), patient health questionnaire (PHQ)-9, and genetic psychosocial risk instrument (GPRI) were applied to the patients. RESULTS: There is a relationship with negative regression coefficient between the CKD stage and the total scores of the EQ-5D-3L and EQ-5D-3L VAS scales (p < 0.000 and ß = - 5.355, p < 0.000, and ß = - 8.394, respectively). There is a relationship with positive regression coefficient between the CKD stage and MSPSS total score and level (p < 0.000 and ß = 0.364, p < 0.000 and ß = 0.331, respectively). There is no relationship between the CKD stage and GPRI total score (p = 0.800). In addition, there is a relationship with positive regression coefficient between the dietary compliance and EQ-5D and EQ-5D VAS total scores (p = 0.006 and ß = 2.687, p = 0.004 and ß = 3.148, respectively). There is a relationship with negative regression coefficient between the dietary adherence and PHQ-9 total score and CKD stage (p = 0.003, p = 0.006, and ß = - 0.692, respectively). CONCLUSION: As the CKD stage increases in the ADPKD patients, the quality of life decreases, whereas the level of anxiety and depression increases. It has been seen that the ADPKD patients with more depressive complaints have less dietary compliance. In this particular patient group, the early detection and treatment of psychosocial difficulties and the work to improve the quality of life that affect the course of the ADPKD may be as important as the medical treatment. To determine the needs of ADPKD patients with multiple physical and psychosocial difficulties and to perform appropriate interventions, we think that there is a necessity for a specific scale that evaluates these effective components together in the ADPKD process.

Comparison of geriatric nutritional risk index and creatinine index in short-term mortality prediction in maintenance hemodialysis patients.

BACKGROUND: The aim of this study is to analyze and compare the predictive values of the Geriatric Nutritional Risk Index (GNRI) and Creatinine Index (CI) in the short-term mortality of maintenance hemodialysis patients and to determine their best cut-offs. METHODS: A total of 169 adult hemodialysis patients were included in this retrospective, cross-sectional, and single-center study. The demographic, clinical, and laboratory data of the month in which the patients were included in the study were obtained from their medical files and computer records. All-cause death was the primary outcome of the study during a 12-month follow-up after baseline GNRI and CI calculations. RESULTS: The mean age of the study population was 57 ± 16 years (49.7% were women, 15% were diabetic). During the one-year observation period, 19 (11.24%) of the cases died (8 CV deaths). The optimal cut-off value for GNRI was determined as 104.2 by ROC analysis [AUC = 0.682 ± 0.06, (95% CI, 0.549-0.815), p = 0.01]. The low GNRI group had a higher risk for all-cause and CV mortality compared to the higher GNRI group (p = 0.02 for both in log-rank test). The optimal sex-specific cut-off was 12.18 mg/kg/day for men [AUC = 0.723 ± 0.07, (95% CI, 0.574-0.875), p = 0.03] and was 12.08 mg/kg/day for females [AUC = 0.649 ± 0.13, (95% CI, 0.384- 0.914), p = 0.01]. Patients with lower sex-specific CI values had higher all-cause and CV mortality (p = 0.001 and p = 0.009 in log-rank test, respectively). In multivariate cox models, both GNRI [HR = 4.904 (% 95 CI, 1.77-13.56), p = 0.002] and sex-specific CI [HR = 5.1 (95% CI, 1.38-18.9), p = 0.01] predicted all-cause mortality. The association of GNRI with CV was lost [HR = 2.6 (CI 95%, 0.54-13.455), p = 0.22], but low CI had a very strong association with CV mortality [HR = 11.48 (CI 95%, 1.25 -104), p = 0.03]. DISCUSSION: In hemodialysis patients, GNRI and CI have similar powers in predicting all-cause short-term mortality. The association of CI with all-cause death depends on gender. On the other hand, sex-specific CI predicts CV mortality better than GNRI.

Pregnancy and its outcomes in hemodialysis patients in Turkey.

BACKGROUND/AIM: This study aimed to investigate pregnancy frequency and evaluate the factors affecting live births in hemodialysis (HD) patients. MATERIALS AND METHODS: Female HD patients whose pregnancy was retrospectively reported between January 1, 2014, and December 31, 2019. The duration of HD, primary disease, whether the pregnancy resulted in abortion, stillbirth, or live birth, whether the HD duration was prolonged after diagnosing the pregnancy and whether it accompanied preeclampsia were recorded. RESULTS: In this study, we reached 9038 HD female patients? data in the study. A total of 235 pregnancies were detected in 145 patients. The mean age was 35.42 (35 ± 7.4) years. The mean age at first gestation was 30.8 ± 6.5 years. The average birth week was 32 (28 - 36) weeks. 53.8% (no = 78) of the patients had live birth, 51.7% (no = 70) had at least one abortion in the first 20 weeks, and 13.1% (no = 19) had at least one stillbirth after 20 weeks. The rate of patients' increased numbers of dialysis sessions during pregnancy was 71.7%. The abortion rate was 22.4% in those with increased HD sessions, whereas 79.3% in those not increased HD sessions (p < 0.001). Live birth frequency was 67.2% in the increased HD sessions group and 3.4% in those who did not differ in HD sessions (p < 0.001). CONCLUSION: For the first time, we reported pregnancy outcomes in HD female patients, covering all regions of Turkey. It has been observed that; increasing the number of HD sessions in dialysis patients will decrease fetal and maternal complications and increase live birth rates.

Retinal Microcirculation Alterations in Microalbuminuric Diabetic Patients With and Without Retinopathy.

PURPOSE: To investigate the effect of microalbuminuria (MA) on superficial vessel density (SVD), deep vessel density (DVD), and choriocapillaris vessel density (CVD) in type-2 diabetic patients. METHODS: Twenty patients without diabetic retinopathy (DR) and MA (group 1), 20 patients without DR but with MA (group 2) and 30 patients with mild DR and MA (group 3) were enrolled in this prospective and cross-sectional study. SVD, DVD, and CVD of all patients were screened with optical coherence tomography angiography (OCTA). The relationships between these values and age, diabetes duration and metabolic parameters were also evaluated. RESULTS: The whole macular SVD value was 50.15 ± 4.52 in group 1 and 47.81 ± 4.12 in group 2 (p = .04). The whole macular DVD value was 47.66 ± 2.76 in group 1, 44.37 ± 3.39 in group 3 (p = .02). Parafoveal DVD value was 52.58 ± 3.47 in group 1, 51.84 ± 2.23 in group 2, and 49.23 ± 3.38 in group 3 (p G1&3 = .001, p G2&3 = .02). Perifoveal DVD value was 47.92 ± 3.30 in group 1, 43.96 ± 4.19 in group 2, and 42.85 ± 2.98 in group 3 (p G1&2 = .02 and p G1&3 < .001). There were inverse correlations between diabetes duration, urea, creatinine, albumin, urinary sodium and some DVD values (p < .05, for all). Also, there were inverse correlations between parafoveal and perifoveal DVD values and MA (p = .002 and p = .031). Additionally, inverse correlations were determined between diabetes duration, creatinine, urea, serum Na and some CVD values (p < .05 for all).Conclusion: Decreased SVD and DVD values measured by OCTA in type-2 diabetic patients, whether they have mild DR or not, may be associated with MA causing early retinal microvascular changes.

Is dialysis adequacy a useful predictor for sexual function in males and females with end-stage renal disease?

INTRODUCTION: The development of sexual dysfunction (SD) in dialysis patients is multifactorial. We aimed to evaluate whether adequate dialysis had an effect on the development of SD in male and female patients undergoing dialysis due to end stage renal disease. Anxiety, depression, health-related quality of life and the other risk factors related to dialysis were also evaluated in terms of SD. METHODS: Seventy men and 57 women undergoing haemodialysis (HD) or peritoneal dialysis (PD) and 65 healthy male volunteers and 48 healthy female volunteers, age-matched, were included in the study. The International Index of Erectile Function, Female Sexual Function Index, Beck Depression Inventory, Beck Anxiety Inventory and The Short Form-36 Health Survey were applied to all participants. The cut off value of Kt/V was determined as 1.3 for HD and 1.7 for PD to assess dialysis adequacy. Per gender, all the participants were divided into three groups as control, adequate dialysis and non-adequate dialysis. RESULTS: Dialysis adequacy [OR: 3.225, 95%CI (1.213-8.620), p=.019] was found as a more decisive factor for male SD, while dialysis adequacy [OR: 3.015, 95%CI (.991-7.250), p=.041] and depression [OR: 4.280, 95%CI (1.705-10.747), p=.002] were more significant for female SD. In addition, a strong relationship was found between male SD and physical functioning (r: .524, p=.032), social functioning (r: .565, p=.042), general health (r: .693, p=.037) perception, while female SD was found to be strongly associated with anxiety (r: -.697, p=.002) and depression (r: -.738, p=.001). DISCUSSION: Dialysis adequacy was found to be the most important factor in reducing SD. Non-adequate dialysis resulted in worse sexual function, higher levels of depression and anxiety. Its negative effect on health-related quality of life was only seen in men.

COVID-19 infection in a kidney transplant recipient-special emphasis on pharmacokinetic interactions: A case report.

BACKGROUND: Solid organ transplant recipients are considered to be at high-risk of developing coronavirus disease 2019 (COVID-19)-related complications. The optimal treatment for this patient group is unknown. Consequently, the treatment of COVID-19 in kidney transplant recipients should be determined individually, considering patient age and comorbidities, as well as graft function, time of transplant, and immunosuppressive treatment. Immunosuppressive treatments may give rise to severe COVID-19. On the contrary, they may also lead to a milder and atypical presentation by diminishing the immune system overdrive. CASE SUMMARY: A 50-year old female kidney transplant recipient presented to the transplant clinic with a progressive dry cough and fever that started three days ago. Although the COVID-19 test was found to be negative, chest computed tomography images showed consolidation typical of the disease; thus, following hospital admission, anti-bacterial and COVID-19 treatments were initiated. However, despite clinical improvement of the lung consolidation, her creatinine levels continued to increase. Ultrasound of the graft showed no pathology. The tacrolimus blood level was determined and the elevation in creatinine was found to be related to an interaction between tacrolimus and azithromycin. CONCLUSION: During the COVID-19 pandemic, various single or combination drugs have been utilized to find an effective treatment regimen. This has increased the possibility of drug interactions. A limited number of studies published in the literature have highlighted some of these pharmacokinetic interactions. Treatments used for COVID-19 therapy; azithromycin, atazanavir, lopinavir/ritonavir, remdesivir, favipiravir, chloroquine, hydroxychloroquine, nitazoxanide, ribavirin, and tocilizumab, interact with immunosuppressive treatments, most importantly with calcineurin inhibitors. Thus, their levels should be frequently monitored to prevent toxicity.

Effect of microalbuminuria on macular thickness in patients with type-2 diabetes mellitus.

PURPOSE: To investigate the effect of microalbuminuria on macular thickness in patients with type-2 diabetes mellitus with no or mild diabetic retinopathy and to investigate the relationship between macular thickness and metabolic parameters. MATERIALS AND METHODS: Fifty eight eyes of 58 patients without diabetic retinopathy (group 1) in microalbuminuria stage, 42 eyes of 42 patients with mild diabetic retinopathy (group 2) in microalbuminuria stage, and 50 eyes of 50 patients without diabetic retinopathy and microalbuminuria (group 3) were included in this study. After detailed ophthalmologic examination, all patients underwent spectral domain-optical coherence tomography measurements. Macular thickness was noted from nine different areas (fovea, four parafoveal, and four perifoveal areas) and compared between groups. The correlations between macular thickness and age, duration of diabetes mellitus, microalbuminuria, serum urea, creatinine, glycosylated hemoglobin (HbAIc), albumin, sodium (Na), and urinary Na were evaluated. RESULTS: The mean age was 53.29 ± 6.49 in group 1, 55.86 ± 6.97 in group 2, and 52.98 ± 5.66 years in group 3 (p = 0.06). The macular thickness values of superior, inferior, and nasal parafoveal areas were significantly different between groups (p = 0.001, p = 0.006, and p = 0.03, respectively). Bonferroni post test revealed that this difference originated from the difference between group 2 and 3 (p < 0.05 for all values). There were significant negative correlations between the macular thickness values of parafoveal areas and serum urea, HbA1c, albumin, microalbuminuria levels (p < 0.05 for all values). CONCLUSION: In this study, a significantly decreased parafoveal macular thickness was measured in patients with mild diabetic retinopathy and microalbuminuria compared to patients without diabetic retinopathy and microalbuminuria.

Corneal Endothelial Morphology and Thickness Changes in Patients with Gout

Objectives: To investigate corneal endothelial cell density (ECD), morphology, and central corneal thickness (CCT) in patients with gout compared with healthy subjects. Materials and Methods: Fifty eyes of 50 gout patients and 50 eyes of 50 healthy subjects without gout or any other systemic disease were included in this study. After detailed ophthalmologic examination, specular microscopy (Tomey EM-4000; Tomey Corp) measurement was performed for all participants. ECD, average cell area (ACA), coefficient of variation (CV), hexagonality ratio, and CCT values were recorded. Results: Mean ECD and hexagonality ratio were lower (p=0.004 and p=0.002) and CV, ACA, and CCT values were higher (p=0.001, p=0.007, and p=0.001) in patients with gout when compared to healthy subjects. There were significant correlations between gout disease duration and CD and hexagonality ratio (p=0.019 and p=0.043) and also between uric acid value and hexagonality ratio and CCT (p=0.044 and p=0.003). Conclusion: Altered corneal endothelial function was found in patients with gout when compared to healthy subjects and the alteration increased as gout duration and uric acid value increased.

Could corneal densitometry be used as a diagnostic and screening tool for ocular involvement in patients with gout?

PURPOSE: To evaluate the corneal and tear film characteristics in patients with gout and compare these data with those of healthy subjects and to investigate the correlation of corneal densitometry with uric acid value and duration of disease. METHODS: Forty-one eyes of 41 patients with gout (group 1) and 40 eyes of 40 healthy subjects (group 2) were included in this study. Detailed ophthalmologic examinations were performed on all participants. Corneal tomographic analyses were performed with the Pentacam HR Scheimpflug imaging system (Oculus, Wetzlar, Germany). Maximum keratometry (Kmax), central corneal thickness (CCT), corneal volume (CV), and corneal densitometric values were compared between groups. Tear function tests including Schirmer, tear breakup time, and fluorescein staining were performed and compared between groups. RESULTS: Groups were similar in terms of age and gender. Kmax, CCT, and CV values were similar between groups. The outcomes of tear function tests were similar between groups. Corneal densitometric values for the 0-2 and 2-6 mm zones of the anterior and center layers were significantly higher in group 1 compared to group 2. This densitometric increase was positively correlated with uric acid levels and gout duration. CONCLUSIONS: Corneal densitometry values of the 0-2 and 2-6 mm zones of the anterior and central layers were higher in patients with gout than those of the control subjects, and the densitometric values increased as uric acid level and disease duration increased.

Increased Corneal Ectasia Risk in Patients with Familial Mediterranean Fever.

PURPOSE: To evaluate the corneal tomographic parameters in patients with familial Mediterranean fever (FMF) and to compare data with those of healthy control subjects. MATERIALS AND METHODS: Forty eyes of 40 patients with FMF (FMF group) and 35 eyes of 35 healthy subjects (control group) were included to this prospective study. All participants underwent complete ophthalmological examination and tomographic analysis with Pentacam HR. Maximum keratometry, front and back astigmatism, thinnest pachymetry, maximum anterior and posterior elevation best-fit-sphere (BFS) at the 5.0 mm zone, front and back difference elevation, final D, average and maximum pachymetric progression index, and maximum Ambrósio relational thickness parameters were noted. RESULTS: Groups were similar in terms of age and gender (p = 0.77 and 0.35). Maximum keratometry, thinnest pachymetry, front and back astigmatism, and maximum anterior elevation BFS at the 5.0 mm values were similar between groups (p = 0.22, 0.52, 0.49, 0.29, and 0.31, respectively). Maximum posterior elevation BFS at the 5.0 mm and back difference elevation values were higher than control group in FMF group (p = 0.001 and 0.04). The mean values of final D, average and maximum pachymetric progression index were higher, and Ambrósio relational thickness was lower in FMF group compared to control group (p = 0.03, 0.002, 0.006, and 0.01, respectively). CONCLUSIONS: FMF patients carry increased corneal ectasia risk compared to healthy subjects. The corneal tomographic characteristics and the association of keratoconus with FMF may be important in a detailed refractive surgery work-up.

Association between central aortic pulsatility and glomerular filtration rate in patients with coronary artery disease.

OBJECTIVE: Aortic stiffness and chronic kidney disease share common risk factors. Increased aortic stiffness is a predictor of lower estimated glomerular filtration rate (eGFR) at lower levels of renal functions. We aimed to investigate the association between invasively measured central aortic pulsatility (AP) as an indicator of aortic stiffness and eGFR in a population with coronary artery disease and without overt renal disease. METHODS: This study had a cross-sectional design. Data were retrospectively collected. We evaluated 72 patients (44 males and 28 females; mean age 59.0±10.3 years) with coronary artery disease. eGFR was calculated with dividing the Cockcroft-Gault formula by body surface area. Direct measurements of aortic blood pressures were utilized to calculate pulse pressure and AP. Multiple linear regression analysis was performed to test the relationship between eGFR and AP, independent from potential confounders. RESULTS: eGFR was significantly correlated with age (r=0.489, p<0.001), body surface area (r=0.324, p=0.006), weight (r=0.323, p=0.006), aortic pulse pressure (r=-0.371, p=0.001), and AP (r=-0.469, p<0.001). In multiple linear regression analysis, AP was independently associated with eGFR (p=0.035), beside the age and body surface area. An AP cut-off level of >0.71 had 84% sensitivity and 72% specificity in predicting eGFR of <90 mL/min per 1.72 m2 (receiver-operating characteristic area under curve: 0.851, 95% CI: 0.760-0.942, p<0.001). CONCLUSION: We found an independent relationship between invasively measured AP and eGFR in patients with coronary artery disease. Moreover, a higher AP may predict lower eGFR. These results may be utilized to predict eGFR from AP during invasive procedures.

Low serum pancreatic enzyme levels predict mortality and are associated with malnutrition-inflammation-atherosclerosis syndrome in patients with chronic kidney disease.

PURPOSE: Serum levels of amylase and lipase are frequently increased in patients with chronic kidney disease (CKD). Relatively low serum pancreatic enzyme levels in CKD may represent a state of pancreatic insufficiency and may contribute to protein-energy wasting (PEW). We aimed to investigate the relationships of serum pancreatic enzyme levels with PEW, inflammation, and mortality in CKD patients. METHODS: Two hundred and thirty CKD patients (male/female: 144/86; mean age, 59 ± 16 years) were enrolled. Serum total α-amylase and lipase activities were measured by enzymatic colorimetric assays. Mean follow-up time was 18 ± 10 months. Forty-seven patients (20 %) died during this period. RESULTS: Serum amylase levels were increased in 95 patients (41 %) and serum lipase levels were increased in 71 patients (30 %) out of the 230 patients. Diabetic patients had significantly lower serum amylase levels than non-diabetic ones (86 ± 46 vs. 111 ± 60 IU/L, p < 0.0001). Patients with ischemic heart disease also had significantly lower serum amylase (82 ± 37 vs. 108 ± 60 IU/L, p < 0.0001) and lipase levels (39 ± 36 vs. 57 ± 57 IU/L, p = 0.007). Serum amylase and lipase levels were directly correlated with serum creatinine (r = 0.173, p = 0.009 and r = 0.374, p < 0.0001) and albumin (r = 0.410, p < 0.0001 and 0.287, p < 0.0001), and inversely correlated with CRP (r = -0.223, p = 0.001 and r = -0.147, p = 0.027). The Kaplan-Meier analysis revealed survival advantages for both high-amylase and high-lipase groups in end-stage renal disease (ESRD) (CKD stage 5) patients (log rank, p < 0.001 and p = 0.02, respectively). In the Cox regression analysis, serum amylase was found to be an independent predictor for mortality. CONCLUSION: Serum amylase activity was found to be an independent predictor of mortality in ESRD patients. Relatively low serum pancreatic enzyme levels in CKD may be regarded as a novel component of the malnutrition-inflammation-atherosclerosis syndrome.

An unusual case of renal failure due to solitary plasmacytoma: parenchymal invasion of the kidney.

Solitary extramedullary plasmacytoma (EMP) is a rare plasma cell disorder mostly involving the upper airway; however, retroperitoneal infiltration is very rare. Kidney injury associated with EMP is exceptionally rare with only anecdotal reports. Herein we report a case of retroperitoneal EMP causing renal failure by the way of direct renal parenchymal infiltration. Renal parenchymal invasion should be considered in aggressive and refractory plasma cell dyscrasias with unexplained renal failure.

Reversible posterior leukoencephalopathy in the course of Goodpasture syndrome.

Reversible posterior leukoencephalopathy syndrome (RPLS) is characterized by headache, altered consciousness, seizures, and cortical blindness. The most frequent etiological factors are hypertension, kidney diseases, and immunosuppressive drugs such as steroids and cyclophosphamide. Herein we present a case of a 22-year-old female patient presented with alveolar hemorrhage and acute renal failure necessitating hemodialysis. In renal biopsy, necrotizing crescentic glomerulonephritis and immunofluorescence pattern compatible with Goodpasture syndrome were found. Anti-glomerular basement membrane antibody result was positive. At follow-up, respiratory failure ensued, steroid pulse treatment was started, and she was transferred to intensive care unit (ICU). In the ICU, she had visual disturbances and blindness together with seizures. Cranial magnetic resonance imaging (MRI) revealed irregular T2- and fluid-attenuated inversion recovery (FLAIR)-weighted lesions in bilateral occipital lobes. On clinical and radiological grounds, RPLS was diagnosed. With the supportive and anti-hypertensive treatment, RPLS was resolved without a sequela. Subsequent cranial MRI was totally normal. In the literature, RPLS associated with Goodpasture syndrome was reported only once. Hypertension and methylprednisolone might be the responsible etiologies in this case.

Cardiomyopathy mimicking left ventricular noncompaction in a patient with lupus nephritis.

A 37-year-old female patient was admitted with exertional dyspnea. Her serum creatinine was 2.4 mg/dL and anti-nuclear antibody was positive in a titer of 1/320. Renal biopsy revealed diffuse proliferative lupus nephritis. Echocardiography and cardiac magnetic resonance (MR) imaging showed increased apical trabeculations compatible with left ventricular noncompaction (LVNC), which is a rare genetic cardiomyopathy. The patient expressed a marked improvement in exertional dyspnea after the immune-suppressive treatment for systemic lupus erythematosus (SLE). Control echocardiography revealed a significant increase of ejection fraction. SLE may cause a kind of cardiomyopathy with high resemblance to LVNC. Discrimination of these two similar clinical entities is important because SLE-induced cardiomyopathy is potentially reversible after the immune-suppressive treatment for SLE.

Focal segmental glomerulosclerosis associating Kimura disease.

Kimura disease presents as a benign subcutaneous mass. Although it principally affects the skin and soft tissues, there is a high prevalence of related renal disease. We report a case of Kimura disease from western Asia, presenting itself as nephrotic syndrome, and this case was seen at a nontransplant kidney presenting with focal segmental glomerulonephritis.