Alport syndrome misdiagnosed with IgA nephropathy from familial history: a case report and brief review.

BACKGROUND: Alport syndrome is a rare inherited disease resulting from a primary disorder of the glomerular basement membrane. This disease results from mutations in genes encoding alpha chains of type IV collagen. In the differential diagnosis of this disease, IgA nephropathy is the most common primary glomerular disease with gross or microscopic hematuria. CASE PRESENTATION: A 50-year-old woman was presented with microscopic hematuria and proteinuria of under one gram. Due to the diagnosis of IgA nephropathy in family members, she was treated and followed up for 4 years as a possible case of IgA nephropathy. Eye examination and audiometry were normal. She underwent renal biopsy with an exacerbation of proteinuria. There was no finding in favor of IgA nephropathy in the histological examination, but the findings of electron microscopy and family history favored Alport syndrome. CONCLUSIONS: This case demonstrates the importance of accurate history and electron microscopy in the complete histological evaluation and diagnosis of glomerular disease. Although in most cases the two can be differentiated based on clinical manifestations, laboratory findings, and histopathological examination, sometimes the association of these two diseases in the families involved or the lack of accurate history and complete histological examinations can complicate the diagnosis.

Cadaveric kidney transplantation in an ESRD patient with Klippel-Feil syndrome-A case report.

Klippel-Feil syndrome (KFS) is a congenital skeletal disorder with classic clinical triad consisting of short neck, low hairline, and limitation of neck movement. In addition to skeletal deformities, diagnosed cases of KFS may have other anomalies such as cardiac, neurologic, and genitourinary disorder. We report a case of KFS with associated anomalies such as short neck, limitation of neck movement, scoliosis of cervical spine, and dextrocardia who received successful cadaveric kidney transplantation. This article aims to present kidney transplantation as option of treatment in KFS patient with ESRD and emphasize on the difficulty in airway management of this patient.

Comparing Therapeutic versus Prophylactic Enoxaparin Therapy in Severe COVID-19 Patients: A Randomized Clinical Trial.

Background: Coronavirus disease 2019 (COVID-19) has been associated with a hypercoagulopathy state; however, the efficacy of different anticoagulant regimens in preventing thrombotic events is not clear. We aimed to compare therapeutic versus prophylactic enoxaparin therapy in severe COVID-19 patients. Methods: In this single-center, open-label, randomized controlled trial, adult patients with severe COVID-19 presentations and an increased D-dimer level of more than 4 times the normal upper limit were randomly assigned to receive either prophylactic or therapeutic dose of enoxaparin. All patients were observed for at least 4 months regarding the overall survival as the primary outcome. Hospitalization duration, the need for intensive care unit (ICU) admission, the need for mechanical ventilation, and major adverse events (MAEs) were also analyzed as the secondary outcomes. Survival analysis was done via Kaplan-Meier curves and the Log-rank test. Cox regression was used, adjusting for baseline variables. Results: Overall, 237 patients (152 men and 85 women) were randomized to either arm (121 to prophylactic and 116 to therapeutic groups). The mortality rate was 27 (22.3%) and 52 (44.8%) in prophylactic and therapeutic arms, respectively. Prophylactic enoxaparin was associated with better survival in the log-rank test (P < 0.001; HR, 0.42). Additionally, a significantly lower rate of ICU admission, a lower rate of MAEs, and shorter hospitalization were observed in the prophylactic arm (P < 0.001, P = 0.009, and P = 0.028, respectively). Conclusion: The results of the current study were in favor of anticoagulant treatment with prophylactic doses of enoxaparin. Still, due to the limitations of this paper, we suggest that these findings be treated cautiously.

Choriocarcinoma transmitted with the transplant: Case study.

Choriocarcinoma is a rare kind of cancer, which may be either gestational or non-gestational. Choriocarcinoma is responsible for about a quarter of all documented neoplastic aneurysms. It is a descriptive case report of choriocarcinoma transmission from a donor, following kidney donation. A 45-year-old woman got a kidney from a 25-year-old woman who was taken to the hospital due to a non-traumatic cerebral hemorrhage. She delivered a healthy baby 48 days before her brain death. The transplant was successfully done. Five weeks' post-transplantation, the recipient had pain and erythema in the surgical area. Regarding the high level of beta-human chorionic gonadotropin in her blood, diagnostic tests were performed. Following the confirmation of the cancer, a five-phase chemotherapy plan with various pharmaceutical regimens was initiated. Liver function test values rose after the final round of chemotherapy, and the patient developed hepatic encephalopathy. Considering the thrombocytopenia, dialysis, or hemoperfusion, which are normally performed to reduce liver enzymes, were not initiated. Finally, she died due to the hepatic failure and disseminated intravascular coagulation. Although the nephrologists disagree on the optimal course of treatment, it seems that nephrectomy would be helpful in such instances. Physicians should be aware of the possibility of transplant-related choriocarcinoma in female donors of reproductive age who die because of intracerebral brain hemorrhage for unclear reasons. Every donor must undergo a thorough examination. It is critical to get documents, clarify history, and interview relatives.

Long-Term Impact of the COVID-19 Associated AKI: The Relationship between Kidney Recovery and Mortality in a 10-Month Follow-Up Cohort Study.

INTRODUCTION: Coronavirus-2019 disease (COVID-19)-associated acute kidney injury (AKI) and its short and mid-term effect on kidney has been well established in the previous literature, indicating a high number of AKI in hospitalized patients associated with high rates of mortality, followed by high rates of unresolved kidney injury at the time of discharge. However, the long-term impact of AKI and its resulting lack of recovery at the time of discharge has not been investigated. Herein, we sought to explore the possible relationship between AKI and unresolved kidney injury and post-discharge mortality. METHOD: In this cohort study, patients hospitalized with COVID-19 who survived until discharge were followed for a median of 9.6 months. AKI during hospitalization based on the staging according to Kidney Disease: Improving Global Outcomes (KDIGO) criteria and kidney injury status at discharge and other comorbidities and mortality during the follow-up period were recorded. The desired association was investigated using Cox proportional hazards regression after adjustment for potential confounders. RESULT: Among 1,017 discharged patients, 298 patients (29.3%) experienced AKI during hospitalization according to KDIGO criteria, of whom 178 patients (59.7%) were diagnosed with unresolved kidney injury at the time of discharge. After adjusting for potential confounders, Cox regression indicated that AKI stage 3 (hazard ratio (HR): 4.56, 95% confidence interval (CI): 1.89-10.99, p = 0.001) and unresolved kidney injury at the time of discharge (HR: 2.09, 95% CI: 1.18-3.73, p = 0.011) were significantly associated with mortality during the post-discharge period. Additionally, Kaplan-Meier curves for overall survival indicated an increased risk of mortality in patients with stage 2, stage 3 AKI, and unresolved kidney injury at the time of discharge (p < 0.001). CONCLUSION: Overall, it was shown that patients with COVID-19 who develop AKI, mainly stage 2 and 3, and patients with unresolved kidney injury at the time of discharge, were at an increased risk of mortality, even after hospitalization for an extended period of time.

Clinical, Functional, and Mental Health Outcomes in Kidney Transplant Recipients 3 Months After a Diagnosis of COVID-19.

BACKGROUND: Kidney transplant patients are at high risk for coronavirus disease 2019 (COVID-19)-related mortality. However, limited data are available on longer-term clinical, functional, and mental health outcomes in patients who survive COVID-19. METHODS: We analyzed data from adult kidney transplant patients in the European Renal Association COVID-19 Database who presented with COVID-19 between February 1, 2020, and January 31, 2021. RESULTS: We included 912 patients with a mean age of 56.7 (±13.7) y. 26.4% were not hospitalized, 57.5% were hospitalized without need for intensive care unit (ICU) admission, and 16.1% were hospitalized and admitted to the ICU. At 3 mo follow-up survival was 82.3% overall, and 98.8%, 84.2%, and 49.0%, respectively, in each group. At 3 mo follow-up biopsy-proven acute rejection, need for renal replacement therapy, and graft failure occurred in the overall group in 0.8%, 2.6%, and 1.8% respectively, and in 2.1%, 10.6%, and 10.6% of ICU-admitted patients, respectively. Of the surviving patients, 83.3% and 94.4% reached their pre-COVID-19 physician-reported functional and mental health status, respectively, within 3 mo. Of patients who had not yet reached their prior functional and mental health status, their treating physicians expected that 79.6% and 80.0%, respectively, still would do so within the coming year. ICU admission was independently associated with a low likelihood to reach prior functional and mental health status. CONCLUSIONS: In kidney transplant recipients alive at 3-mo follow-up, clinical, physician-reported functional, and mental health recovery was good for both nonhospitalized and hospitalized patients. Recovery was, however, less favorable for patients who had been admitted to the ICU.

COVID-19 Infection in Kidney Transplant Recipients From a Single Center in Iran.

OBJECTIVES: COVID-19 has been spreading rapidly throughout the world, with nearly every country thus far documenting this infection. In this study, our aim was to evaluate the risk factors for increased mortality in deceased donor kidney transplant recipients with COVID-19 at a single center in Iran. MATERIALS AND METHODS: This was a retrospective study in a single center. During the 17-month ongoing COVID19 pandemic in Iran, there were 153 deceased donor kidney recipients at our center with suspected COVID19 symptoms. Of these patients, 138 had positive COVID-19 tests, and thus a therapeutic regimen was commenced for these patients. Data were analyzed with SPSS version 16 software. RESULTS: The patients were predominantly male (83, 60.1%) with a median age of 47.09 ± 13.75 years and a median time since transplant of 51 months (IQR, 1-276 months). Among these patients, 84 (60.8%) had hypertension and 43 (31.2%) had diabetes mellitus. We observed a significant relationship between disease severity and mortality (P < .001). After risk adjustments for age, presence of diabetes mellitus and hypertension and blood group type were factors that showed a significantly higher risk of death. CONCLUSIONS: Deceased donor kidney transplant recipients with confirmed COVID-19 experienced less fever as an initial symptom. However, recipients with COVID-19 and an underlying disease had a higher rate of mortality, severity of infection, and progression of disease. Appropriate management of renal complications and vaccinations in deceased donor kidney transplant recipients may help lead to more favorable outcomes.

Association of clinical characteristics, antidiabetic and cardiovascular agents with diabetes mellitus and COVID-19: a 7-month follow-up cohort study.

BACKGROUND: The prognostic factors of long-term outcomes in hospitalized patients with diabetes mellitus and COVID-19 are lacking. METHODS: In this retrospective cohort study, we evaluated patients aged ≥ 18-years-old with the COVID-19 diagnosis who were hospitalized between Feb 20 and Oct 29, 2020, in the Sina Hospital, Tehran, Iran. 1323 patients with COVID-19 entered in the final analysis, of whom 393 (29.7%) patients had diabetes. We followed up patients for incurring in-hospital death, severe COVID-19, in-hospital complications, and 7-month all-cause mortality. By doing univariate analysis, variables with unadjusted P-value < 0.1 in univariate analyses were regarded as the confounders to include in the logistic regression models. We made adjustments for possible clinical (model 1) and both clinical and laboratory (model 2) confounders. RESULTS: After multivariable regression, it was revealed that preadmission use of sulfonylureas was associated with a borderline increased risk of severity in both models [model 1, OR (95% CI):1.83 (0.91-3.71), P-value: 0.092; model 2, 2.05 (0.87-4.79), P-value: 0.099] and major adverse events (MAE: each of the severe COVID-19, multi-organ damage, or in-hospital mortality) in model 1 [OR (95% CI): 1.86 (0.90-3.87), P-value: 0.094]. Preadmission use of ACEIs/ARBs was associated with borderline increased risk of MAE in the only model 1 [OR (95% CI):1.83 (0.96-3.48), P-value: 0.066]. CONCLUSIONS: Preadmission use of sulfonylureas and ACEIs/ARBs were associated with borderline increased risk of in-hospital adverse outcomes. SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1007/s40200-021-00901-4.

The Tsunami of COVID-19 Infection Among Kidney Transplant Recipients: A Single-Center Study from Iran.

BACKGROUND: Although most evidence supports the fact that kidney transplant (KT) recipients are at significant risk of morbidity and mortality, risk factors of accruing COVID-19 in this population have remained poorly defined. METHODS: All KT recipients who had been transplanted in Sina Hospital and were actively followed between March 1996 and January 2021 were enrolled in a retrospective manner. The demographic characteristics, immunosuppressive treatment before KT, and death were gathered by calling patients with a designed questionnaire. RESULTS: 108 (about 21%) of 523 KT recipients were diagnosed with COVID-19. The mean age of COVID-19 patients was 46.9 ± 13.6, of whom 43% were women. In the multivariate model, body mass index (BMI) ≥ 30 independently increased the risk of COVID-19 incidence with OR 2.00 (95% CI 1.23, 3.26) (P = 0.00), and besides, having diabetes had a marginal association with COVID-19 incidence (OR 1.62 [95% CI 0.98, 2.66]; P = 0.057). The mortality rate of COVID-19 was 15%. In the multivariate model, only pre-transplantation diabetes significantly increased the risk of death by COVID-19 with OR of 3.90 (95% CI 1.00-15.16) (P = 0.04). CONCLUSION: Given the higher incidence rate in KT recipients with obesity and diabetes and higher mortality rate in KT recipients with diabetes as the cause of ESRD, more attention should be paid to KT recipients with these risk factors.

Hybrid Percutaneous Brachiofemoral Shunt and Open Abdominal Aortic Aneurysm Repair in a Kidney Transplant Recipient.

Abdominal aortic aneurysm (AAA) repair in kidney transplant recipients may cause ischemia in the transplanted kidney. As a result, various techniques have been described for protection of the renal allograft during AAA repair including temporary shunt, extracorporeal bypass, cold renal perfusion, endovascular aortic aneurysm repair (EVAR), and operation without renal allograft protection. We successfully treated a 56-year-old man, a case of kidney transplantation with AAA, using a temporary hybrid percutaneous brachiofemoral shunt using vascular prosthesis with a long 7-French (Fr) catheter sheath introducer (CSI) in the aortic arch via the right brachial artery and 8 Fr CSI in the right femoral artery that were connected together with a 7 Fr guiding catheter, before aortic cross-clamping and repair of AAA using a Dacron tube graft. The patient recovered well from the surgery without any complication and was discharged on the 6th postoperative day. To our knowledge, this is the first report of using a temporary hybrid percutaneous brachiofemoral shunt for renal allograft protection in AAA repair surgery in a patient with kidney transplantation, and we think that this temporary shunt is an easy, safe, and rapid method for renal allograft protection from ischemia.

The Risk Factors and Clinical Outcomes Associated with Acute Kidney Injury in Patients with COVID-19: Data from a Large Cohort in Iran.

INTRODUCTION: Kidney involvement, ranging from mild hematuria and proteinuria to acute kidney injury (AKI) in patients with coronavirus disease-2019 (COVID-19), is a recent finding with various incidence rates reported among hospitalized patients with COVID-19. Given the various AKI rates and their associated risk factors, lack of AKI recovery in the majority of patients hospitalized with COVID-19, and limited data regarding AKI in patients with COVID-19 in Iran, we aim to investigate the potential risk factors for AKI development and its incidence in patients hospitalized with COVID-19. METHODS: In this retrospective cohort study, we enrolled adult patients referred to the Sina Hospital, Iran, from February 20 to May 14, 2020, with either a positive PCR test or a highly susceptible chest computed tomography features consistent with COVID-19 diagnosis. AKI was defined according to the kidney disease improving global outcomes criteria, and patients were stratified based on their AKI staging. We evaluated the risk indicators associated with AKI during hospitalization besides in-hospital outcomes and recovery rate at the time of discharge. RESULTS: We evaluated 516 patients with a mean age of 57.6 ± 16.1 years and a male-to-female ratio of 1.69 who were admitted with the COVID-19 diagnosis. AKI development was observed among 194 (37.6%) patients, comprising 61.9% patients in stage 1, 18.0% in stage 2, and 20.1% in stage 3. Out of all patients, AKI occurred in 58 (11.2%) patients during the hospital course, and 136 (26.3%) patients arrived with AKI upon admission. AKI development was positively associated with all of the in-hospital outcomes, including intensive care unit admissions, need for invasive ventilation, acute respiratory distress syndrome (ARDS), acute cardiac injury, acute liver injury, multiorgan damage, and mortality. Patients with stage 3 AKI showed a significantly higher mortality rate, ARDS, and need for invasive ventilation than other stages. After multivariable analysis, male sex (odds ratio [OR]: 11.27), chronic kidney disease (CKD) (OR: 6.89), history of hypertension (OR: 1.69), disease severity (OR: 2.27), and high urea levels (OR: 1.04) on admission were independent risk indicators of AKI development. Among 117 (28.1%) patients who experienced AKI and survived, only 33 (28.2%) patients made a recovery from the AKI, and 84 (71.8%) patients did not exhibit full recovery at the time of discharge. DISCUSSION/CONCLUSION: We found that male sex, history of CKD, hypertension, disease severity, and high serum urea were independent risk factors associated with AKI in patients with COVID-19. Also, higher stages of AKI were associated with increased risk of mortality and in-hospital complications. Our results indicate a necessity for more precise care and monitoring for AKI during hospitalization in patients with COVID-19, and lack of AKI recovery at the time of discharge is a common complication in such patients.

The Prevalence of Renal Stones and Outcomes of Conservative Treatment in Kidney Transplantation: A Systematic Review and Meta-Analysis.

BACKGROUND: Nephrolithiasis is a rare complication in transplanted kidneys and limited information is available about its therapeutic options. This study aimed to review the conservative management of urinary lithiasis and its outcomes in renal transplanted patients. METHODS: A systematic review and meta-analysis of the scientific literature were performed in the Medline, Scopus, and Embase databases. Inclusion criteria were studies which include patients with kidney stones in transplanted kidney no matter de-novo or donated stones and use conservative treatment for all or part of their patients. Exclusion criteria were bladder and ureteral stones, full text unavailable, conference paper. (from January 1st, 1980, to the 19th May 2020). The results of included studies, in the final analysis, were combined using a random-effect model and using metaprop method prevalence and 95% CI of renal stone were reported. RESULTS: A total of 8 studies (14988 transplant patients) met the inclusion criteria for the final analysis. A total of 195 patients suffered from renal stone and the prevalence of renal stone was 1.3% (95% CI: 0.89% - 1.7%). The mean age of these patients was 43 years. analysis, the prevalence of conservative treatment in 195 patients was 35% (95% CI: 19%-51%). The mean stone size ranged from 0.29 cm to 1 cm. Three studies reported a stone-free rate of %100. Except for two studies that did not report complications, other studies reported zero percent. CONCLUSION: More than one-third of nephrolithiasis were conservatively managed in transplanted patients. Despite limited data, conservative treatment could be done in less than 4 mm stones with high Stone Free Rate (SFR) and very low complications.

COVID-19 progression in kidney transplant recipients: a single-center case series.

The novel coronavirus disease 2019 (COVID-19) is a respiratory infection that has received much attention due to its rapid expansion. Currently, it has been revealed that patients with underlying disease, especially those with kidney disease are more prone to develop complications. Some studies associate kidney transplantation as a risk factor for COVID-19 progression; however, epidemiologic data that demonstrate this are amazingly rare. Considering the importance of the topic, we report on six kidney transplant recipients (median age 47 [41-55]) with confirmed or clinically suspected COVID-19. The most common admission presentations were fever (83.3%), dyspnea, and myalgia. At baseline, immunosuppressive therapy was ceased, prednisolone dose was increased, and all patients received antiviral treatment including hydroxychloroquine and umifenovir. After a median follow-up of 11.5 days from admission, six patients (100%) developed acute kidney injury (AKI), 50% required intensive care unit (ICU) admission, and two patients (33.3%) deceased as a result of deterioration in respiratory status. Overall, these findings demonstrate that respiratory involvement may be a risk indicator of in-hospital mortality in kidney recipients with COVID-19. In addition, AKI development in kidney recipients with COVID-19 is of utmost importance given the higher AKI occurrence in these patients compared with others. Therefore, more intensive attention should be paid to kidney transplant recipients with COVID-19.

Adaptation of a quality of life questionnaire for Iranian patients with esophageal cancer.

INTRODUCTION: Evaluation of quality of life is very important in cancer patients. Esophagus-specific quality of life questionnaire (QLQ-OES18) is a disease-specific questionnaire for assessing quality of life in esophageal cancer (EC). So we aimed to translate and evaluate the reliability and validity of the QLQ-OES18 when applied to Iranian patients. MATERIALS AND METHODS: This study was designed as cross-sectional study on 62 newly confirmed EC in two referral hospital in Tehran, Iran. Reliability of the subscales was evaluated by intraclass correlation coefficients. Pearson's correlations of an item with its own scale and other scales were calculated to assess convergent and discriminant validity. Clinical validity was also evaluated by known-group comparisons. RESULTS: Cronbach's alpha was higher than 0.7 in most subscales. All subscales met the standards of convergent and discriminant validity. Also QLQ-OES18 had discriminatory power for differentiation between patient's groups with different clinical status. CONCLUSION: Our results provide evidences that Persian version of QLQ-OES18 is a valid and reliable questionnaire when applied to a sample of Iranian patients with EC and is recommended for use in clinical research.

Methemoglobinemia in aluminum phosphide poisoning.

INTRODUCTION: Acute aluminum phosphide (AlP) poisoning is one of the most common causes of acute pesticide poisoning in Iran. Hydrogen phosphide or phosphine gas is produced following reaction of AlP with water even at ambient humidity. Methemoglobinemia is a rare finding following phosphine poisoning. In this paper, two cases of fatal AlP poisoning complicated by methemoglobinemia are reported. CASE REPORT: Two patients presented following suicidal ingestion of AlP tablets. In the Emergency Department (ED), they received gastric lavage with sodium bicarbonate and potassium permanganate. Both of them received supportive care. In each case, hematuria and hemolysis were significant events. The patients also showed a decrease in O(2) saturation in spite of high FIO(2). Methemoglobin levels of 40% and 30% were detected by co-oximetry. Neither patient responded to treatment (ascorbic acid in one case, methylene blue in the other). Both patients died due to systemic effects of phosphine poisoning. DISCUSSION AND CONCLUSION: Hemolysis and methemoglobinemia may complicate the course of phosphine poisoning that seems resistant to methylene blue and ascorbic acid. Therefore, other treatments including hyperbaric oxygen therapy and exchange blood transfusion should be considered.