Allelic Variants of the Complement Genes in Acute Postinfectious Glomerulonephritis.

Acute postinfectious glomerulonephritis (APIGN) is one of the most common causes of acute glomerulonephritis in children. It may lead to inflammation and proliferation of glomerular tissue through immunologic mechanisms (Balasubramanian R, Paediatr Int Child Health 2017;37:240-247).

How many cores should be taken from each region of interest when performing a targeted transrectal prostate biopsy?

Background: The number of core biopsies required per region of interest (ROI) is controversial, as is the localization of the core to be taken from a lesion. This study aimed to determine the ideal biopsy core number and location in a multiparametric magnetic resonance imaging guided targeted prostate biopsy (TPB), without reducing the clinically significant prostate cancer (csPC) detection rate. Materials and methods: Data of patients who had PI-RADS ≥3 lesions on multiparametric magnetic resonance imaging and underwent a TPB in our clinic between October 2020 and January 2022 were reviewed, retrospectively. The first and second cores were taken from the central part of the ROI, whereas the third and fourth cores were taken from the right and left peripheries of the ROI. We compared the csPC detection success of single-, 2-, 3-, and 4-core samplings. Results: Software-based transrectal TPB was performed on 251 ROIs in a total of 167 patients. Internal Society of Urological Pathology Grade Group ≥2 cancer was detected in at least one core in 64 (25.4%) lesions. Moreover, csPC was detected in 42 (65.6%) ROIs in first-core biopsies; in 59 (92.2%) ROIs in first- and second-core biopsies; in 62 (96.9%) ROIs in first-, second-, and third-core biopsies; and in 64 (100%) ROIs in first-, second-, third-, and fourth-core biopsies. Using McNemar's test for comparison, a significant difference was found in terms of csPC detection success between performing first-core and second-core biopsies (65.6 - 92.2%, p < 0.001); by contrast, no significant difference was observed in csPC detection success between 2-core and 3-core biopsies (92.2% - 96.9%, p = 0.24). Furthermore, no significant difference existed between performing second-core and fourth-core biopsies in terms of csPC detection success (92.2%-100%, p = 0.07). Conclusion: We concluded that taking 2-core biopsies from the center of each ROIs during a transrectal TPB is sufficient for diagnosing csPC.

Granulomatous Mediastinitis: A Rare Cause of Pulmonary Hypertension.

A rare case of a patient with chronic obstructive pulmonary disease who developed secondary anthracofibrosis to biomass exposure, fibrosing mediastinitis due to anthracotic enlarged lymph nodes in the mediastinum, and pulmonary hypertension because of compres- sion of the lymph nodes on the pulmonary arteries is presented. This is a case report of a 71-year-old female patient who has been followed up with chronic obstructive pulmonary disease for 10 years, has no history of smoking, and has been exposed to biomass for many years. The patient, who had been hospitalized in various centers for the last 3 years due to progressive shortness of breath and dry cough, applied to us with dry cough and dyspnea complaints. On echocardiography, systolic pulmonary arterial pressure was found to be 59 mmHg. For the etiology of pulmonary hypertension, dual-energy thoracic computed tomography was performed with the suspicion of chronic thromboembolic pulmonary hypertension. No filling defect compatible with thromboembolism was detected. In right heart catheterization, mean pulmonary artery pressure was 27 mmHg, pulmonary capillary tip pressure was 7 mmHg, and pulmonary vascular resistance was 3.71 woods units. Endobronchial ultrasound was applied to the patient with the preliminary diagnoses of lymphoma, anthracosis, fibrosing mediastinitis, and infection. Widespread anthracosis was observed in all lobes and segments macroscopically. The lymph node in the subcarinal area was interpreted as anthracotic lymph node. Anthracosis is defined as black pigmentation involving the mucosal, and submucosal layers of the tracheobronchial tree and the lung parenchyma. If anthracosis is associated with luminal obliteration and/or mucosal proliferation causing obstruction, it is considered anthracofibrosis. In this case, we saw that secondary anthracofibrosis, fibrosing mediastinitis due to anthracotic enlarged lymph nodes in the mediastinum, and pulmonary hypertension may develop because of compression of the lymph nodes on the pulmonary arteries, and we wanted to draw attention to it was a rare case.

Value of Neutrophil-Lymphocyte and Platelet-Lymphocyte Ratios in the Evaluation of Acute Rejection and Chronic Allograft Nephropathy in Children With Kidney Transplantation.

OBJECTIVES: Neutrophil-to-lymphocyte ratio and platelet (thrombocyte)-to-lymphocyte ratio have become accepted markers of inflammation in recent years and are used to assess disease activity in some diseases. In this study, we investigated the relationship between these values and acute rejection attacks, as well as their role in determining chronic allograft nephropathy, in follow-up of pediatric kidney transplant recipients. MATERIALS AND METHODS: Our study included 58 kidney transplant recipients (age 5-18 years) with at least 5-year follow-up at our center. Patients with history of secondary transplant, concomitant malignancy, and shorter follow-up were excluded. Medical history and laboratory parameters pretransplant and at 1, 3, and 6 months and 1, 2, 3, 4, and 5 years posttransplant, as well as kidney biopsy reports, were reviewed. RESULTS: Both neutrophil-to-lymphocyte (P = .003) and thrombocyte-to-lymphocyte (P = .002) ratios were significantly higher during acute rejection attacks. Although both values were higher in patients with chronic allograft nephropathy at 5 years posttransplant, differences were not statistically significant (P = .69 and P = .55). When patients with and without chronic allograft nephropathy within 5 years were compared, those who developed chronic allograft nephropathy had significantly higher neutrophil- tolymphocyte and thrombocyte-to-lymphocyte ratios at all periods in the first 2 and 4 years posttransplant, respectively. Among patients who had acute rejection attacks, those who subsequently developed chronic allograft nephropathy had higher neutrophil-tolymphocyte ratio in the first 3 years posttransplant, with higher thrombocyte-to-lymphocyte ratio at all posttransplant periods. CONCLUSIONS: This is the first study on neutrophil- tolymphocyte and thrombocyte-to-lymphocyte ratios in pediatric kidney transplant recipients. Our results indicated that both values can be useful and easily accessible markers in acute rejection diagnosis and determining chronic allograft nephropathy development risk, which are 2 major causes of kidney graft loss posttransplant. Pediatric studies with larger populations are needed to support our findings.

Which one is better for predicting extraprostatic extension on multiparametric MRI: ESUR score, Likert scale, tumor contact length, or EPE grade?

PURPOSE: To evaluate the European Society of Urogenital Radiology (ESUR) score, the Likert scale, tumor contact length (TCL) > 1 cm, and EPE (extraprostatic extension) grade in predicting EPE at multiparametric magnetic resonance imaging (mp-MRI). METHODS: Seventy-nine patients who underwent 3-T MRI and were histopathologically confirmed by microblocks were enrolled in this retrospective study. The index lesions were interpreted by two experienced radiologists. Apparent diffusion coefficient (ADC) values were also noted. Weighted κ statistics were used to compare interreader agreement. Univariate logistic regression analysis was performed to define independent predictors of EPE status. Multivariable logistic regression and receiver operating characteristic (ROC) analysis were performed to compare the MRI-based methods and clinical variables (ISUP grade, prostate volume and PSA density) + MRI-based methods for pathologic EPE prediction by using the area under the curve (AUC) value. RESULTS: The mean age was 64.5 years ± 6.2. 33/79 (41.8%) patients had pathologic EPE. As ESUR score showed weak interreader agreement (κ = 0.537), Likert scale, TCL, and EPE grade showed moderate agreement (κ = 0.608, κ = 0.747, κ = 0.647 respectively). Univariate ROC analysis result showed that all MRI-based score systems, mean ADC value, the ISUP grade, prostate volume, PSA density were the best variables in predicting EPE. ROC analysis results of four MRI-based methods showed good diagnostic performance. At multivariate analysis, all clinical models showed excellent diagnostic performance. CONCLUSION: All four MRI-based methods had good diagnostic performance. Furthermore, consisting of both qualitative and quantitative parameters and being less reader experience dependent, EPE grade was a promising method in predicting EPE. All clinical models showed excellent diagnostic performance.

Role of silymarin (Silybum marianum) in the prevention of colistin-induced acute nephrotoxicity in rats.

Silymarin (Silybum marianum) has some protective effects against drug toxicity (cisplatin, acetaminophen, adriamycin, gentamicin etc.). Colistin is a strong antimicrobial, which is frequently used in the treatment of resistant gram-negative bacterial infections in recent years although it has nephrotoxic potential. This study was aimed to determine the role of silymarin against colistin-induced acute nephrotoxicity (CIN). Rats were randomly divided into four groups. The control group was treated with tap water whereas groups 2 and 3 received silymarin (orally, 100 mg/kg/day) and colistin (intraperitoneally, 750.000 IU/kg/day) for seven days, respectively. Group 4 received both 750,000 IU/kg/day colistin and 100 mg/kg/day silymarin for seven days. After euthanasia, histopathological and biochemical examinations were completed for the kidney tissue specimens and blood samples. All parameters of the control and silymarin groups were similar. Severe weight loss was seen in the groups receiving colistin (groups 3 and 4). Silymarin significantly increased glutathione peroxidase and superoxide dismutase levels when administered with colistin in group 4 only. Acute tubular injury, tubular necrosis, meduller congestion, interstitial inflammation and apoptotic indices of colistin group were significantly higher than the control group. The administration of colistin with silymarin (group 4) was able to make some improvements in tubular necrosis and significant increase in antioxidant capacity. Silymarin increased antioxidant enzyme activity only when used in combination with colistin. The effects of silymarin may become more pronounced when used at higher doses or with a longer duration of treatment and may prevent nephrotoxicity.

The Histopathological Effect of Aloe Vera on the Wound Healing Process in a Surgically Created Tubularized Incised Plate Urethroplasty Model on Rats.

BACKGROUND: Hypospadias is a common congenital anomaly which is determined as an abnormal urethral opening on the ventral face of penis. The purpose of this rat model study was to research the effect of topically applied Aloe vera after a tubularized incised plate urethroplasty (TIPU). METHODS: The TIPU model was applied to male Wistar albino rats. A total of 30 rats were randomly grouped into 3 groups of 10. Group I was assigned as the control group, treated with 0.9% saline only twice a day for 15 days. Group II received topical Aloe vera gel once a day and Group III received Aloe vera gel twice a day. Spongiofibrosis was graded as 0: none, 1+:≤10% tissues involved, 2+:10%-49% tissues involved, 3+: ≥ 50% tissues involved. RESULTS: A higher degree of fibrosis and inflammation was determined in the Group I subjects than in Groups II and III. Fibrosis of grade 3+ was observed in 33% of the control group and not in any of the two Aloe groups (p = 0.043). Inflammation of grade 3+ was seen in 66.7% of the control group, in 10% of Group II, and in 33% of Group III (p = 0.02). CONCLUSIONS: The topical application of Aloe vera to a surgically created tubularized incised plate urethroplasty model decreased inflammation and fibrosis that may affect the success rates of this operation.

The role of histopathological and biochemical parameters for predicting metastatic disease on 68 Ga-PSMA-11 PET in prostate cancer.

BACKGROUND: The aim of this study was to evaluate the role of histopathological and biochemical parameters in the prediction of the presence and number of PSMA positive lesions consistent with the metastatic spread of prostate cancer on 68 Ga-PSMA PET images. METHODS: Biochemical, histopathological and imaging data of 302 prostate cancer patients who underwent 68 Ga-PSMA-11 PET/CT or PET/MR imaging for primary staging were retrospectively analyzed. Patients were divided into two groups as "PET positive" and "PET negative" according to the presence of pathologic extraprostatic PSMA involvement. "PET positive" patients were additionally divided into two groups: oligometastatic (1-3 metastatic lesion) and multimetastatic (>3 metastatic lesions). RESULTS: The mean age of patients was 66.8 ± 7.6 years. Imaging modality was PET/MR in 223 (73.8%) and PET/CT in 79 (26.2%) of patients. Total PSA, PSA density (PSAD), ALP, and tumor ratio in biopsy specimens were found to be significantly higher in "PET positive" group compared to "PET negative" group and in multimetastatic group compared to oligometastatic group. PET positivity was observed in 3.8% of the low-intermediate risk groups (ISUP 1-3 and total PSA ≤ 20 ng/ml and PSAD < 0.15 ng/ml/cc). This ratio was 46% in the high-risk group (ISUP 4-5 or total PSA > 20 ng/ml or PSAD ≥ 0.15 ng/ml/cc) with a relative risk of 12 (p < .001). The prediction models to predict the PET positivity and the presence of distant metastasis had AUCs of 0.901 and 0.925, respectively; with ALP, total PSA, and tumor ratio in needle biopsy specimen as significant independent predictors (p < .05). CONCLUSIONS: In this study, 68 Ga-PSMA-11 PET positivity was significantly higher in the high-risk patient group than in the low-intermediate risk groups. The prediction models used for predicting the PET positivity and the presence of distant metastasis on PET imaging were successful with high discriminatory powers. In addition to total PSA and ISUP GG, ALP and tumor ratio in biopsy specimens can be used to identify high-risk patients.

Case Report: A Human Case of Onchocerca lupi Mimicking Nodular Scleritis.

We report a case of human ocular onchocerciasis by zoonotic Onchocerca lupi presenting as nodular scleritis. Molecular analyses were used to confirm diagnosis at species level. In addition to few existing reports of human infection by O. lupi in Turkey, this case further suggests that the pathogen might be more common than previously reported, thus requiring further attention and investigations.

Relation between Cajal Cell Density and Radiological and Scintigraphic Outcomes in Patients with Ureteropelvic Junction Obstruction.

INTRODUCTION: In this study, we aimed to investigate the correlation between Cajal cell density and preoperative and postoperative radiological and scintigraphic parameters in ureteropelvic junction obstruction (UPJO). METHODS: The study group consisted of 41 renal units (38 consecutive patients; 13 female and 25 male) surgically treated for UPJO. UPJ specimens from patients were immuno-stained with CD117 (c-kit) antibody for interstitial Cajal cells (ICCs). The relation between Cajal cell density and preoperative and postoperative radiological and scintigraphic parameters was evaluated. RESULTS: The mean age of the patients was 8.52 ± 8.86 (0-35) years. The density of Cajal cells was defined in 2 groups for convenient analysis as 0-5 cells (low) in 19 (46.3%) patients and >5 cells (moderate-high) in 22 (53.6%). There was significant difference between the preoperative and postoperative anteroposterior diameters of the related kidneys in both Cajal groups (p = 0.001-low, p = 0.000-moderate-high) independent of Cajal cell density. Regression in hydronephrosis postoperatively was determined in both Cajal groups (77.8%-low, 64.7%-moderate-high); however, there was no difference between them (p = 0.39). Preoperative T1/2 was significantly longer in the low Cajal group (p = 0.02). Postoperative T1/2 decreased in both low (p = 0.000) and moderate-high (p = 0.001) Cajal groups, but no difference was found between them (p = 0.24). There was significant improvement in the kidney differential function after surgery in the low Cajal density group (p = 0.015) while there was no correlation between the scintigraphic success or improvement and Cajal cell density (p = 0.51). DISCUSSION/CONCLUSION: ICC deficiency/density could not be shown as a predictive factor for the determination of success rate of pyeloplasty. Despite the lack of any evidence for the degree of deficiency as an indicator for the severity of obstruction and prediction of surgical success, further studies are needed for confirmation.

Ezrin is a prognostic biomarker in patients with clear cell metastatic renal cell carcinoma receiving sunitinib.

OBJECTIVE: Sunitinib is a novel oral multitargeted tyrosine kinase inhibitor with antitumor and antiangiogenic activities. This study evaluates ezrin expression in sunitinib-treated metastatic clear cell renal cell carcinoma (ccRCC) patients and elucidates its role as a possible marker for survival. MATERIALS AND METHODS: The expression of ezrin was measured by immunohistochemistry in 80 patients with ccRCC treated by first-line sunitinib between January 2007 and June 2012. Kaplan-Meier curves and log-rank tests were used for analysis of progression-free survival and overall survival (OS), and a multivariate Cox proportional hazard model was employed to identify factors with an independent effect on the survival. RESULTS: In multivariate analysis, liver metastasis (P = 0.018; hazard ratio [HR]: 3.707 (1.257-10.931) and overexpression of ezrin (P = 0.006; HR: 2.993 (1.373-6.523 95% confidence interval) were remained significant factors influencing OS. Overexpression of ezrin in the patients who had progressed in the first 3 months was higher than in the patients who had progressed after 3 months (P = 0.003). The median OS was longer in patients with low levels of ezrin expression (27 months) compared to patients overexpressing ezrin (12 months) (P = 0.001). CONCLUSION: This is the first study in the literature showing that ezrin status is related with prognosis in patients with metastatic ccRCC.

Diagnostic accuracy of 68 Ga-PSMA PET/MRI and multiparametric MRI in detecting index tumours in radical prostatectomy specimen.

OBJECTIVE: To evaluate the diagnostic accuracy of the 68 gallium (68 Ga) prostate-specific membrane antigen (PSMA) positron emission tomography/magnetic resonance imaging (PET/MRI) and multiparametric MRI (mpMRI) by region-based comparison of index tumour localisations using histopathological tumour maps of patients who underwent radical prostatectomy because of clinically significant prostate cancer. PATIENTS AND METHODS: The study included 64 patients who underwent radical prostatectomy after primary staging with mpMRI and 68 Ga-PSMA PET/MRI. Diagnostic analysis was performed by dividing the prostate into four anatomic regions as left/right anterior and left/right posterior. The extension of the lesions in mpMRI and the pathological uptake in 68 Ga-PSMA PET/MRI were matched separately for each region with the extension of the index tumour into each region. RESULTS: The sensitivity, specificity, positive predictive value, negative predictive value, positive likelihood ratio, negative likelihood ratio, and the accuracy of mpMRI and 68 Ga-PSMA PET/MRI are shown as 55.7%, 91.8%, 80.6%, 77.2%, 78.1%, and 60.8%, 94.3%, 86.8% 79.8%, 83.5%, respectively. 68 Ga-PSMA PET/MRI has higher sensitivity and specificity compared with mpMRI. However, no statistically significant difference was found (P = .464). Combined imaging had significantly higher diagnostic accuracy compared with mpMRI and 68 Ga-PSMA PET/MRI (change in AUC: 0.084 and 0.046, P < .001 and P = .028, respectively), while no statistically significant difference was found between mpMRI and 68 Ga-PSMA PET/MRI (change in AUC: 0.038, P = .246). CONCLUSION: 68 Ga-PSMA PET/MRI had higher clinical diagnostic accuracy in prostate cancer compared with mpMRI. Diagnostic accuracy was significantly increased in the combined use of both imaging modalities.

Effects of phospholipase A2 receptor and thrombospondin type-1 domain-containing 7A expression in glomerular basement membranes on treatment response and renal outcome in membranous nephropathy.

BACKGROUND: The aim of this study was to define the clinicopathologic features of phospholipase A2 receptor (PLA2R) and/or thrombospondin type-1 domain-containing 7A (THSD7A) associated membranous nephropathy(MN) focusing on their impact to disease relapse and response to treatment. METHODS: A total of 201 patients were enrolled for baseline clinical and histopathological features and 102 patients with a clinical follow-up for more than 1 year were evaluated for outcomes. Immunohistochemical staining was performed with PLA2R and THSD7A antibodies on kidney biopsies and glomerular staining was evaluated. RESULTS: PLA2R expression was observed in 75% of the patients' biopsies; however, THSD7A expression was present only in 7 patients' biopsies (3.5%). No significant difference was found between histopathological and clinical features of PLA2R positive and negative patients, collectively. Glomerular PLA2R expression was significantly associated with complete and complete/partial remission with first-line treatment; however, overall complete, and complete/partial remission rates did not differ from PLA2R negative patients (p = 0.2 and p = 0.8). Male gender, the presence of IgG4 staining and a necessity of immunosuppressive treatment were significantly associated with glomerular PLA2R expression. One patient, who developed end-stage renal disease, had glomerular expression for both PLA2R and THSD7A. Three patients with THSD7A-positive MN achieved complete remission. CONCLUSIONS: The probability of achieving complete remission is high in patients with PLA2R-positive MN for whom the relapse rate was also higher. The overall renal outcome did not differ from PLA2R negative cases. Low incidence of THSD7A-positive MN reduces the possibility of future randomized controlled trials.

Kidney biopsy in the elderly: diagnostic adequacy and yield.

PURPOSE: The number of kidney biopsies (KB) performed in elderly patients has been increasing. Safety and usefulness of elderly KB have been well established, whereas much less is known about diagnostic adequacy and yield in this patient population. METHODS: We performed a retrospective study of KBs in 428 patients from April 2015 to December 2017 at an academic institution. We compared KB from 50 patients aged over 64 (elderly) with KB from 378 patients aged between 18 and 64. RESULTS: Gender ratio, body mass index, systolic and diastolic BP, creatinine values, incidences of AKI at the time of biopsy, INR/aptt values, and platelets were similar between the two groups. eGFR and number of transplant biopsies were lower in the elderly biopsy group. The glomerular yield was similar between the two groups (22 ± 14 vs. 22 ± 13, p = 0.869). The likelihood of obtaining more than ten glomeruli was 87% and 88%, respectively, without a significant difference. Inadequate samples were encountered in 6% of the elderly and 5.6% of the non-elderly KB, again without a significant difference. Samples taken by nephrologist had higher glomerular yield for both groups (25 ± 13 vs. 18 ± 12 overall, 26 ± 14 vs. 18 ± 14 for elderly, p < 0.001 both). Inadequate biopsies were lower in the nephrologist group when all patients were considered (3% vs. 9%, p = 0.025). Results were numerically similar for the elderly patients, but the difference was not statistically significant (2% vs. 8%, p = 0.322). No deaths occurred in both arms. Minor complications were not different for each group (4.5% vs. 4%). There were no major complications in elderly patients. However, the difference did not reach statistical significance. CONCLUSION: The world is aging, leading to an increased number of KB in older patients. KB in the elderly is a safe, effective, and an indispensable tool for the nephrologist. This study suggests there is no need to fear lower diagnostic adequacy in the decision making of a KB for an elderly patient.

Combining clinical features and MEST-C score in IgA nephropathy may be a better determinant of kidney survival.

Introduction. IgA nephropathy (IgAN) is a heterogeneous disease with highly variable clinical and histopathological features. We investigated the effects of Oxford classification and clinical features on renal survival in patients with IgAN.Methods. This retrospective observational study conducted from 2013 to 2017. Ninety-seven patients who were followed up more than six months were examined.Results. A total of 97 patients (68% male and median age 40 years) were enrolled in this study. 13% of patients developed end stage renal disease (ESRD) within the median of 37 months of follow-up. Need for renal replacement therapy at the time of diagnosis, serum creatinine level of higher than 1.97 mg/dl, serum albumin level less than 3.5 gr/dl, 24-hour urine protein level of higher than > 3.5 g/day, the percentage of glomerulosclerosis higher than 53%, T2 score and total MEST-C score higher than two were found to be significant predictors of development of ESRD. None of the clinical or histopathological features were found to be significant predictor of steroid treatment sensitivity except T1-2 scores.Conclusion. We think that IgA nephropathy is a heterogeneous disease that requires clinical and histopathological features to be evaluated together, but not individually, to determine renal survival.What is new. Iga nephropathy is a heterogeneous disease and modern pathologic classification systems is not enough to predict to prognosis. Histopathological features to be evaluated with clinical features, but not individually, to determine renal survival. Also glucocorticoid treatment response seems to be independent from clinical and histopathological features except T1-2 score.

Renal Biopsy Prognostic Findings in Children With Atypical Hemolytic Uremic Syndrome.

BACKGROUND: The aim of this study was to investigate the histopathological findings in kidney biopsies in children with atypical hemolytic uremic syndrome (aHUS) and to determine whether specific pathological findings in aHUS have a prognostic value. METHODS: Renal biopsy specimens of 29 patients who were recorded in the national Turkish aHUS registry database were available for review. Histopathological findings were compared with the clinical and laboratory features at the presentation and the final outcome. RESULTS: The mean age at presentation and follow-up period was 4.9 ± 3.9 and 3.9 ± 3.0 years, respectively. The median time interval from the first symptom to biopsy was 10 days. Vascular thrombosis and interstitial fibrosis were significantly related to chronic kidney disease (CKD) requiring dialysis or kidney transplantation during follow-up (5.6-fold, for both). Glomerular necrosis, cortical necrosis, and glomerular sclerosis were markedly associated with CKD without dialysis (6.2-fold, 13.3-fold, and 8.8-fold, respectively). However, presence of endothelial swelling, subendothelial widening, and fragmented erythrocytes was found to be correlated with a favorable final outcome. CONCLUSIONS: Presence of vascular thrombosis, cortical necrosis, and glomerular sclerosis in histopathological evaluation correlated with developing CKD. Chronic changes in the interstitial compartment were also related to poor prognosis, a finding that has been shown for the first time in pediatric aHUS cases.