Management of hepatorenal syndrome and associated outcomes: a systematic reviews.

BACKGROUND: Hepatorenal syndrome (HRS), a multiorgan condition of acute kidney injury, is seen in advanced liver disease. This study aims to evaluate the current treatment for HRS. METHODS: The authors searched PubMed, Scopus and Google Scholar literature. After quality assessment, 31 studies were included in this review. The Preferred Reporting Items for Systematic Reviews and Meta-Analyses methodology and the population, intervention, comparison and outcome scheme were used. We included human-controlled trials that evaluate the current treatment for HRS. Two authors independently screened articles for inclusion, extracted data and assessed the quality of included studies. RESULTS: This study investigated the studies conducted on the effects of different treatments on follow-up of HRS patients. We gathered 440 articles, so 31 articles remained in our study. Of which 24 articles were conducted on terlipressin versus placebo or other treatments (midodrine/octreotide, norepinephrine, etc) that showed the higher rate of HRS reversal was detected for terlipressin in 17 studies (10 of them were significant), 2 studies achieved an insignificant lower rate of the model for end-stage liver disease score for terlipressin, 15 studies showed a decreased mortality rate in the terlipressin group (4 of them were significant). CONCLUSION: This review showed that terlipressin has a significantly higher reversal rate of HRS than the other treatments. Even the results showed that terlipressin is more efficient than midodrine/octreotide and norepinephrine as a previous medication, in reverse HRS, increasing patient survival.

Frequency and timing of renal biopsy complications.

Background: Percutaneous renal biopsy is the primary diagnostic tool for renal diseases. In this study, we evaluated renal biopsy complications and the timing of complications. Methods: A cross-sectional study was performed on adult patients who underwent renal biopsy. The data gathering sheet collected patient characteristics. Complications were categorized as minor and major which needed an intervention. Data were analyzed using SPSS, and a p-value <0.05 was considered significant. Results: This cross-sectional study was conducted on 215 patients (mean age: 33.1±16.4 and 54.4%: women) who underwent percutaneous renal biopsy in Shiraz Nemazi Hospital for one year. Of the 298 complications that occurred, 90.2% were minors (56.1%of them microscopic hematuria). Moreover, 2 (0.7%) patients developed major complications and received a transfusion. In addition, most of the complications (98.9%, 295 ones) developed within 8 hours post-procedure. Only hemoglobin drop was significantly higher in women (41.0% vs. 21.4%, P=0.003). Conclusion: This study indicates that renal biopsy is a safe procedure; the results revealed that the significant post-biopsy complications were rare and occurred in the first 8 hours.

Comparative Study of Intravenous Immunoglobulin and Leflunomide Combination Therapy With Intravenous Immunoglobulin Single Therapy in Kidney Transplant Patients With BK Virus Infection: Single-Center Clinical Trial.

OBJECTIVES: Nephropathy due to BK virus infection is a major cause of graft dysfunction and loss. No specific treatment has been developed for the BK virus. Here, we compared the combination of intravenous immunoglobulin and leflunomide versus intravenous immunoglobulin to treat BK virus nephropathy after renal transplant. MATERIALS AND METHODS: This study was a randomized controlled clinical trial. Sixteen kidney transplant patients with BK virus infection were randomly divided into 2 groups; 1 group received intravenous immunoglobulin, and another group received leflunomide and intravenous immunoglobulin. P < .05 was considered statistically significant. RESULTS: Results of a polymerase chain reaction test for BK virus after 2 months of treatment were negative in 3 patients in the intravenous immunoglobulin group and in 7 patients in the intravenous immunoglobulin + leflunomide group. The amount of BK virus decreased significantly in each group, and a significant difference was observed between the 2 groups after 3 months (P = .014). The average level of creatinine in the intravenous immunoglobulin group at 1, 2, and 3 months after treatment was 1.7 ± 0.23, 1.8 ± 0.5, and 1.5 ± 0.3, respectively, and in the intravenous immunoglobulin + leflunomide group was 2.1 ± 0.75, 1.76 ± 0.37, and 1.4 ± 0.18, respectively (P > .05). CONCLUSIONS: Although BK viral load decreased significantly in both groups, there was a significant difference between patients who received intravenous immunoglobulin versus those who received the combination of intravenous immunoglobulin + leflunomide after 3 months. The addition of leflunomide to the intravenous immunoglobulin treatment seems to have a better effect in reducing BK viral load. However, further studies with a larger sample and longer duration are needed.

COVID-19 Associated Acute Kidney Injury: The Incidence and Associated Factors in Different KDIGO Stages Among the Hospitalized Patients.

INTRODUCTION: Acute kidney injury (AKI) is the most common reported renal complication associated with COVID-19. In this study, we evaluated the frequency of AKI, the predisposing factors, and its impact on the patient's outcomes in COVID-19. METHODS: By collecting retrospective data, we conducted a crosssectional study on hospitalized severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infected patients in a COVID-19- designated hospital in Shiraz, Iran, from March 2020 to June 2020. Patients' characteristics and laboratory findings were recorded in data gathering sheets. Data were analyzed using SPSS Software Version 16. A P value < .05 was considered significant. RESULTS: This study was conducted on 980 patients with COVID-19 (mean age: 51.2 ± 16.2 years and men: 54.8%), of which 32.6% developed AKI during their hospitalization period, and 1.3% ended up requiring renal replacement therapy. Patients with higher AKI stages experienced more severe/critical COVID-19 (stage 3: 71.0%, stage 2: 44.8%, stage 1: 6.5%; P < .001). The multivariate analysis showed that the proteinuria had the highest relationship with AKI (OR = 6.77 [95% CI: 4.39 to 10.41], P < .001), followed by in-hospital death (OR = 5.14 [95% CI: 1.86 to 14.47], P = .002). In addition, in-hospital death was more observed in higher stages of AKI (OR = 12.69 [95% CI: 3.85 to 42.09], P < .001). CONCLUSIONS: Hospitalized patients with COVID-19 are vulnerable to AKI, especially those who experienced more severe COVID-19 or require mechanical ventilation, which considerably affects the patients' mortality. The high incidence of AKI in our patients demonstrated that it should be considered as one of the common complications of COVID-19, and diagnostic measures, particularly in severe or critical cases, are recommended.  DOI: 10.52547/ijkd.7636.

Sodium-Glucose Cotransporter-2 Inhibitors in Patients with Non-diabetic Chronic Kidney Disease: A Systematic Review.

Sodium-glucose cotransporter-2 (SGLT2) inhibitors modulate kidney function in diabetic chronic kidney disease trials. Furthermore, recent studies have showed their effect on kidney dysfunction in non-diabetic chronic kidney disease (CKD). Here, we focus on the impact of SGLT2 inhibitors on some renal parameters in nondiabetic CKD by discussing completed and ongoing trials. Different databases and search engines of Web of Science, PubMed, Google Scholar, Scopus, SID, and Magiran were searched until November 2022. We included human studies that evaluated the effect of SGLT2 inhibitors in non-diabetic CKD participants. Two authors independently screened the articles for inclusion, extracted the data, and assessed the quality of the included studies. The primary outcomes were the effect of the SGLT2 inhibitors on proteinuria, GFR and blood pressure. A total of 46 full texts were assessed for eligibility, and further review. After reviewing the full texts, seven eligible articles were entered included in this study. We suggest that SGLT2 inhibitors provide renal protection by modifying predisposing factors in the development of CKD, specifically albuminuria and GFR decrease. Other beneficial effects of these agents on blood pressure and sympathetic nerve activity might be considered as a possible mechanism for improving renal hemodynamics. We believe SGLT2 inhibitors could be considered as an effective add-on therapy in non-diabetic CKD patients.  DOI: 10.52547/ijkd.7309.

Exposure to needle stick injuries among health care workers in hemodialysis units in the southwest of Iran: a cross-sectional study.

BACKGROUND: Needle stick injury (NSI) is the most common cause of infection with blood-borne pathogens (BBP) among healthcare workers (HCWs). This study aimed to assess the prevalence of NSI and it's contributing factors among HCWs of hemodialysis (HD) units in southwest Iran. METHODS: A cross-sectional study was performed in 13 HD centers in Shiraz, Iran. A total of 122 employees were enrolled in our study. We used self-administrated questionnaires to collect data about demographics, experiences regarding NSIs, and general health status. The statistical tests used in this study were Chi-square and Independent T-test. A P-value < 0.05 is considered significant. RESULTS: The mean age of the study population was 36.1 ± 7.8 years (72.1%: women). Exposure to NSIs was reported by 23.0% of them at least once during the previous six months. NSI prevalence was significantly higher among those with higher age (p = 0.033), work experience > 10 years (p = 0.040), and those who graduated earlier (p = 0.031). The intravenous injection was the most common procedure leading to NSI, and being in a hurry was the most common cause. The average general health was 3.7 ± 3.2, higher among those not exposed to NSI (p = 0.042). CONCLUSION: NSI is a prevalent hazard in HCWs of HD units. The high rate of NSI and unreported cases, besides the lack of adequate information, indicates the necessity of implementing protocols and strategies for improving the safety of this personnel. It is difficult to compare the result of this study with those performed among HCWs in other settings; hence, further studies are needed to determine whether HCWs of these units are more exposed to NSIs.

Comparison between olfactory training and curcumin therapy in improving olfactory dysfunction in patients with chronic kidney disease.

Objective: Olfactory dysfunction can be seen in chronic kidney disease (CKD) patients. We aimed to investigate the effects of olfactory training and curcumin on olfactory dysfunction in CKD patients and compare their impact with a placebo. Methods: We conducted a double-blind, randomized, placebo-controlled trial in CKD patients, 2021-2022. We enrolled 60 participants in our study into three groups (curcumin, training, and control). Participants were randomized into trials and control groups and assessed using the Iran-smell identification test (Iran-SIT), a questionnaire of olfactory disorders (QOD), and a self-assessment tool. P-value < 0.05 was considered statistically significant. Results: We gathered 58 participants (mean age of 56.1 ± 2.5, 56.9% men). All the tests showed that curcumin improved olfactory function after the trial, though it was significant in QOD (17.5 ± 11.8 vs. 13.1 ± 9.7, p = 0.045) and self-assessment results (8.5 ± 3.1 vs. 9.5 ± 4.0, p = 0.047). Moreover, compared to baseline, training patients experienced an increase in their olfactory function in Iran-SIT (15.3 ± 4.9 vs. 18.8 ± 2.7, p = 0.001), QOD (19.0 ± 10.4 vs. 12.2 ± 9.9, p = 0.003), and self-assessment tools (6.8 ± 1.8 vs. 8.2 ± 3.1, p = 0.027). In contrast, the olfactory function was unchanged in control in all the tests (p > 0.05). Also, the improved change of Iran-SIT and QOD scores during the trial was more significant in training compared to the curcumin group (p < 0.002). Conclusion: The findings of this study indicate that olfactory training, even more than curcumin, can improve olfactory function in CKD patients. This information may help manage olfactory dysfunction in the CKD population.

Post-COVID-19 vaccine acute encephalitis in an adult patient: A case report and literature review.

Several vaccines were approved after COVID-19 pandemic, which have been fast-tracked for emergency use. The short- and long-term safety profile has been an area of concern. We presented a patient with encephalitis followed by hyponatremia who developed hallucination and seizure 1 day after receiving the second dose of Sinopharm vaccine.

Coronavirus Disease 2019 and Hypertension: How Anti-hypertensive Drugs Affect COVID-19 Medications and Vice Versa.

BACKGROUND: As a medical problem, hypertension is one of the most common disorders in cardiovascular disease. High blood pressure has been identified as one of the most familiar risk factors for the ongoing COVID-19 pandemic. We planned to explore the possible interactions between anti-hypertensive agents and drugs targeting SARS-CoV-2 with broad investigations of these medications' mechanism of action and adverse effects. METHODS: Two co-authors searched the electronic databases (PubMed, Scopus, and Google Scholar) to collect papers relevant to the subject. The keywords searched were angiotensin-converting enzyme inhibitors (ACEI), angiotensin-II receptor blockers (ARBs), sympatholytic drugs (alpha-1 blockers, beta-blockers), vasodilators (calcium channel blockers, nitrates, and hydralazine), diuretics, chloroquine, hydroxychloroquine, lopinavir/ritonavir, remdesivir, favipiravir, interferons, azithromycin, anti-cytokine agents, glucocorticoids, anticoagulant agents, nitric oxide, and epoprostenol. RESULTS: QT prolongation, arrhythmia, hypokalemia, hypertriglyceridemia are the most dangerous adverse effects in the patients on COVID-19 medications and anti-hypertensive drugs. CONCLUSION: This review emphasized the importance of the potential interaction between drugs used against COVID-19 and anti-hypertensive agents. Therefore, caution must be exercised when these medications are being used simultaneously.

Inequity and disparities mar existing global research evidence on Long COVID.

Since the pandemic began in December 2019, SARS-Cov2 has accentuated the wide gap and disparities in socioeconomic and healthcare access at individual, community, country, and regional levels. More than two years into the current pandemic, up to three-fourths of the patients are reporting continued signs and symptoms beyond the acute phase of COVID-19, and Long COVID portends to be a major challenge in the future ahead. With a comprehensive overview of the literature, we found that most studies concerning long COVID came from high and upper-middle income countries, and people of low-income and lower-and-middle income regions and vulnerable groups with comorbid conditions have been neglected. Apart from the level of income, there is a significant geographical heterogeneity in investigating the Post-Acute Sequelae of COVID-19 (PASC) or what we call now, long COVID. We believe that these recognizing health disparities is crucial from equity perspective and is the first step toward global health promotion.

Efficacy and Safety of Turmeric Dietary Supplementation on Proteinuria in CKD: A Systematic Review and Meta-analysis of RCT.

The beneficial effects of oral turmeric extract on proteinuria levels have been investigated in several human and animal studies. We conducted a systematic review and meta-analysis to evaluate the significance of this new treatment in CKD patients for the first time. We searched ISI Web of Science, PubMed/Medline, Google Scholar, Scopus, SID, and Magiran until March 2021 to identify human-controlled trials that evaluated the effect of turmeric on proteinuria in chronic kidney disease patients. A total of six trials met the selection criteria and were reviewed in our study and four of them were included in the meta-analysis. In these studies, the results showed not only a significant decrease in the level of proteinuria of the trial groups, who had received curcumin but also a significant change in the level of proteinuria between the trial and control groups (SMD = -0.72, 95% CI: -1.10 to 0.35). The results of this meta-analysis demonstrates that turmeric/curcumin oral supplementation significantly improves urinary protein excretion in patients who suffer from chronic kidney diseases with proteinuria; thus, it can be considered as a potential treatment modality in this population.  DOI: 10.52547/ijkd.6772.

Association Between IL-17A, FOXP3, and CTLA4 Genes Expression and Severity of Lupus Nephritis

INTRODUCTION: Elevated levels of interleukin 17A (IL-17A) have been found in systemic lupus erythematosus (SLE). Forkhead box protein P3 (FOXP3) activates T-regulation lymphocytes and is a master regulator cell function. The cytotoxic T-lymphocyte-associated protein 4 (CTLA4) gene plays a similar role. We investigated the role of these expressions in SLE patients with/without nephritis. METHODS: The present study was a case-controlled study including 49 patients with SLE and 26 healthy controls. The genes expression of IL-17A, FOXP3, and CTLA4 were measured by quantitative Real-Time PCR. The relation between lupus nephritis and disease activity with IL-17A, FOXP3, and CTLA4 genes expression was evaluated. RESULTS: IL-17A, FOXP3, and CTLA4 expressions in T-cells were significantly higher in SLE patients than controls (P < .0001). When comparing the nephritis group and no nephritis group to the control group individually, the expression of mentioned genes is also higher (P < .05). There was no significant difference regarding IL-17A, FOXP3, and CTLA4 genes expression in the nephritis group and no nephritis group (P > .05). But there was a low expression of FOXP3 and IL-17A in patients with the higher stage of nephritis (P < .05). CONCLUSION: Our findings elevated IL-17A, FOXP3, and CTLA4 expressions significantly contribute to SLE pathophysiology. This study provides new insight into the function of IL-17A, FOXP3, and CTLA4 in disease setting. The heterogeneity of SLE patients is reflected in the multiple abnormalities found in the immune system. Finding such variations can provide targets for better manipulation of the immune system. DOI Code: DOI: 10.52547/ijkd.6537

Survival Rate of Renal Transplant and Factors Affecting Renal Transplant Failure.

OBJECTIVES: The most important complication in kidney transplant is acute/chronic rejection. In this study, we investigated the factors affecting kidney rejection and transplant survival. MATERIALS AND METHODS: In this survival analysis study, 352 patients (mean follow-up of 12.9 ± 4.4 years) who underwent renal biopsy due to increased creatinine level from 2012 to 2016 were identified by glomerular filtration rate level and rejection. Probable factors affecting renal function and survival rate after transplant rejection were assessed. P < .05 was considered as significant. RESULTS: Among our study patients, 40.9% developed early and 59.1% developed late acute kidney injury. Graft survival rates at 1 and 5 years were 98.9% and 68.5%, respectively, which was significant when rejection type was considered (P = .002). In addition, patient survival rates at 1 and 5 years were 99.7% and 98.6%, respectively. Graft survival at 5 years was significantly lower among older subjects, those with diabetes, those who received deceased donor organs, and those with late acute kidney injury (P < .002). Patient survival was significantly higher among young patients, those with systemic lupus erythematosus, those who received living donor organs, and those without cytomegalovirus infection (P < .003). CONCLUSIONS: We observed that recipient age, type of donor, underlying disease, infection, and late acute kidney injury had great negative impacts on renal dysfunction and survival. In our center, because of the large number of kidney transplants from deceased donors, the necessity of antithymocyte globulin induction therapy was considered, since this study showed that patients who received rabbit antithymocyte globulin induction had better outcomes.

Sialic acid: an attractive biomarker with promising biomedical applications.

This broad, narrative review highlights the roles of sialic acids as acidic sugars found on cellular membranes. The role of sialic acids in cellular communication and development has been well established. Recently, attention has turned to the fundamental role of sialic acids in many diseases, including viral infections, cardiovascular diseases, neurological disorders, diabetic nephropathy, and malignancies. Sialic acid may be a target for developing new drugs to treat various cancers and inflammatory processes. We recommend the routine measurement of serum sialic acid as a sensitive inflammatory marker in various diseases.

Diagnosis and Treatment of Latent Tuberculosis Infection in Kidney and Liver Transplant Recipients in Iranian Candidates for Transplant.

OBJECTIVES: The rates of tuberculosis and its mortality are higher in solid-organ transplant recipients than in the general population. In this study, we compared the rate of active tuberculosis disease conversion in solid organ transplant recipients based on pretransplant tuberculin skin test results and also its association with treatment. MATERIALS AND METHODS: This cross-sectional study included kidney/liver transplant recipients who had pretransplant tuberculin skin test indurations of ≥5 mm and who were seen from March 2009 to March 2019 at the Shiraz Nemazi Hospital Transplant Center (Shiraz, Iran). Data were analyzed using SPSS software, and P < .05 was considered significant. RESULTS: Of 6289 solid-organ transplant recipients seen at our center over the 10-year period, 334 recipients (mean age of 46.0 ± 13.8 years; 67.6% men) had tuberculin skin test indurations of ≥5 mm. Of these 334 recipients, 76.3% had kidney transplant, and the remainder had liver transplant. Of patients who received complete treatment for latent tuberculosis, the rate of conversion to active tuberculosis was lower than in those who did not adhere to medication (8.6% vs 43.7%; P < .001). In addition, the rate of active tuberculosis development was higher in patients who had pretransplant tuberculin skin tests results of ≥10 mm compared with those who had results of 5 to 9 mm (15.8% vs 3.4%; P < .001). CONCLUSIONS: Latent tuberculosis diagnosis and treatment before solid-organ transplant can reduce active tuberculosis conversion and its associated morbidity and mortality. We recommend modifying the cutoff point considered for tuberculin skin test positivity for solid-organ transplant candidates in Iran to ≥10 mm, although further evaluations are needed.

Chronic Kidney Disease Following Liver Transplant: Associated Outcomes and Predictors.

OBJECTIVES: Liver transplant as a life-saving procedure in patients with end-stage liver disease may have some complications such as renal dysfunction. Improved postoperative management and immuno- suppressive therapy have increased long-term survival and thus increased late complications like chronic kidney disease. Our study aimed to investigate outcomes of chronic kidney disease in liver transplant recipients and the incidence, progression rates, and adjustable risk factors of chronic kidney disease after liver transplant. MATERIALS AND METHODS: Related studies published in English were elicited from various international sources like the ISI Web of Science, PubMed/Medline, Google Scholar, and Scopus. RESULTS AND CONCLUSIONS: Chronic kidney disease as a long-term complication is common in liver transplant recipients whose survival is affected by renal function. Risk assessment of renal function before liver transplant and some nonrenal causes of chronic kidney disease after transplant could help reduce the risks associated with future renal outcomes.

Detection of human papillomavirus (HPV) DNA in benign laryngeal lesions and role of cigarette smoking as an inducing factor.

The association of human papillomavirus (HPV) in laryngeal malignancies is largely known. This study evaluated the association between HPV and benign laryngeal lesions and also the role of smoking and alcohol consumption in increasing the prevalence of HPV in such benign lesions. Seventy-eight specimens of benign laryngeal lesions including 26 polyps, 26 dysplasia, and 26 other lesions such as nodules and cysts were enrolled in this study. Polymerase chain reaction (PCR) technique was used to detect HPV DNA in the tissues. The role of smoking and alcohol consumption in the prevalence of HPV was also evaluated through appropriate statistical tests. This study showed that the prevalence of HPV in benign laryngeal lesions was not statistically significant. The Cohen's effect size for comparing polyps vs. other lesions was nearly 0.7, indicating that HPV prevalence in laryngeal polyps may be clinically meaningful. Another finding in our study is the role of smoking in increasing the HPV prevalence in laryngeal polyps (P = 0.034). In benign laryngeal polyps, HPV prevalence may be clinically important. Smoking acts as a co-factor to induce HPV infection in laryngeal polyps in our study.

Long COVID, a comprehensive systematic scoping review.

PURPOSE: To find out what is known from literature about Long COVID until January 30, 2021. METHODS: We undertook a four-step search with no language restriction. A preliminary search was made to identify the keywords. A search strategy of all electronic databases resulted in 66 eligible studies. A forward and backward search of the references and citations resulted in additional 54 publications. Non-English language articles were translated using Google Translate. We conducted our scoping review based on the PRISMA-ScR Checklist. RESULTS: Of 120 papers, we found only one randomized clinical trial. Of the 67 original studies, 22 were cohort, and 28 were cross-sectional studies. Of the total 120 publications, 49.1% focused on signs and symptoms, 23.3% on management, and 10.8% on pathophysiology. Ten publications focused on imaging studies. The results are also presented extensively in a narrative synthesis in separated sections (nomenclature, diagnosis, pathophysiology, risk factors, signs/symptoms, management). CONCLUSIONS: The controversies in its definition have impaired proper recognition and management. The predominant symptoms were: fatigue, breathlessness, arthralgia, sleep difficulties, and chest pain. Recent reports also point to the risk of long-term sequela with cutaneous, respiratory, cardiovascular, musculoskeletal, mental health, neurologic, and renal involvement in those who survive the acute phase of the illness.

Potential Mechanisms of the SARS-CoV-2-induced AKI Progression to CKD: A Forward-Looking Perspective.

Coronavirus disease 2019 (COVID­19) was identified in December 2019 and is still expanding in most parts of the world. The wide variety of affected organs is likely based upon the shared expression of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) important entry-receptor angiotensin-converting enzyme 2 (ACE2). For this reason, the broad distribution of ACE2 receptors in different tissues plays a crucial role in the multi-organ dysfunction and fatality due to COVID-19. Because of the high prevalence of acute kidney injury (AKI) in patients with COVID-19, we review the molecular understanding into viral infection mechanisms and implications for AKI. Furthermore, mechanisms of the AKI to chronic kidney disease (CKD) progression, such as the relative contribution of immune cell reaction, fibroblasts activation, endothelial dysfunction, and subsequent hypoxia may contribute to the association of AKI with worse outcomes during this virus pandemic. We highlight the state of the knowledge on SARS-CoV-2-dependent mechanisms for AKI and list the potential management choices for the prevention of AKI aggravation and the impending possibility of CKD. Finally, we intend to provide a much better understanding of why Coronavirus induces AKI and its subsequent progression to CKD in the coming years and further discuss the acute and long-term renal consequences.

Kidney Transplant and Autosomal Recessive Polycystic Disease: A Case Report and Literature Review of 2 Brothers.

Autosomal recessive polycystic disease is a rare hepatorenal disorder. End-stage renal disease and liver fibrosis are serious presentations of this disease. Here, we report 2 brothers with autosomal recessive polycystic disease who presented with abnormal abdominal protrusion and hepatosplenomegaly during infancy and eventually underwent renal transplant. Congenital hepatic fibrosis and nephromegaly followed by renal failure developed, after which renal transplant was successfully performed. The remaining compli-cation after transplant was hematemesis and melena due to esophageal varices. Autosomal recessive polycystic disease has a broad spectrum of symptoms; similar pre-sentations with manifestations in siblings may explain some unknown genetic causes of this rare disease.