Background:Schimke immunoosseous dysplasia (SIOD) is a condition marked by spondyloepiphyseal dysplasia (SED), leading to short stature, nephropathy, and T-cell immunodeficiency.Case presentation: A 15-year-old male was referred to the nephrology clinic with a gradual onset of lower-limb swelling. Clinical examination revealed short stature. Laboratory studies revealed renal impairment and nephrotic-range proteinuria. Kidney biopsy showed global sclerosis in 3 of 28 glomeruli, segmental sclerosis in 16 of 28 glomeruli, and 90% foot-process effacement on electron microscopy. A skeletal survey showed flattened thoracolumbar vertebral bodies, a characteristic feature of SIOD. Flow cytometry revealed a low CD4 count. Whole-exome sequencing confirmed that the proband was homozygous for the p.(R611C) variant in the SMARCAL1 gene. The patient was treated with a calcineurin inhibitor (CNI), angiotensin receptor blockers (ARBs), and prophylactic anticoagulation. Initially, he experienced improvement in serum albumin levels and proteinuria. However, his urine protein and creatinine levels subsequently increased, prompting discontinuation of CNI. The patient progressed to end-stage kidney disease (ESKD) and required hemodialysis 18 months after the initial presentation.Discussion: This report describes a case of SIOD, a rare multisystem disorder characterized by short stature due to SED and nephrotic syndrome, distinguishing it from other hereditary nephrotic syndromes. The patient presented unusually in adolescence with nephrotic-range proteinuria and skeletal abnormalities, representing a rarely reported juvenile variant. Genetic testing revealed a novel homozygous SMARCAL1 mutation, p.(R611C), which was confirmed in his younger sibling. However, the phenotypic expression differed, reflecting the weak genotype-phenotype correlation in SIOD. Unlike many SIOD cases, our patient did not experience recurrent infections despite abnormal immune parameters, suggesting a milder immunodeficiency and making kidney transplantation with cautious immunosuppression a feasible option. Current therapeutic challenges include the lack of effective disease-specific treatments, limited success with conventional transplantation due to infection and malignancy risks, and emerging but logistically complex approaches such as combined stem-cell and kidney transplantation. Conclusion:SIOD is a rare genetic disorder with variable presentation and outcomes. Our patient, carrying a novel SMARCAL1 mutation and presenting with juvenile-onset disease, progressed to ESKD but had only mild immune dysfunction, making kidney transplantation a potential treatment option. Early recognition is essential to avoid unnecessary treatments, guide supportive care, and enable timely referral for advanced therapies. Clinicians should consider SIOD in patients presenting with short stature and nephrotic syndrome to optimize outcomes.

Background:Acute kidney injury requiring dialysis (AKI-D) is a severe medical condition that is common and associated with a high rate of morbidity and mortality. Identifying predictors of kidney recovery in patients with AKI-D might lead to better care and improved kidney and patient survival. This study aims to assess the long-term clinical outcomes of patients who had AKI-D during their hospitalization and remained on dialysis at discharge and identify predictors of renal recovery after discharge. Methods:We retrospectively studied adult patients hospitalized between January 2016 and December 2022 who had AKI-D during their hospitalization and continued receiving dialysis after discharge. Patients who had less than three months of follow-up, underwent kidney transplantation, or died within three months of dialysis initiation were excluded from the study. Results:Of the 64 patients in the study, 20 (31%) achieved renal recovery, while 44 (69%) remained dialysis dependent. The average time to renal recovery was 93 ± 61 days. Recovered AKI-D patients had significantly lower baseline and average weekly predialysis serum creatinine after discharge and significantly higher intensive care unit admission, length of hospital stay, vasopressor use, and number of dialysis sessions than non-recovered patients. Using multivariate analysis, we identified vasopressor use as the only independent predictor of renal recovery after discharge in patients with AKI-D (odds ratio, 16.244 [95% CI, 1.22-217.17]; P = 0.035). Conclusion:Renal recovery after discharge can be seen in up to one-third of patients with AKI-D, even if they have advanced chronic kidney disease at baseline or require dialysis for more than three months. The chance of renal recovery is higher in patients who require vasopressor use during hospitalization. Thus, patients with AKI-D should be closely monitored after discharge, and guidelines on managing such patients need to be created.

Immunosuppression in kidney transplantation elevates the risk of malignancies, particularly immune-driven and virus-related cancers like Kaposi sarcoma (KS). KS typically manifests as single or multiple skin lesions following kidney transplantation but can also affect other organs. Involvement of the kidney allograft by KS is exceptionally rare, with only a few cases documented. In this report, we present all known cases of KS involving kidney allografts in adult transplant recipients in Qatar, accompanied by a brief review of the literature.

Dialysis treatment carries a high burden for patients, families, and care givers. Elderly patient with limited mobility and multiple comorbidities specifically suffer poor quality of life on dialysis. This population unlikely to be candidate for traditional home hemodialysis (HHD) modality. Our study presenting our novel experience in the state of Qatar with providing Assisted HHD (AHHD) for elderly patients with limited mobility in their home environment.

Abstract Background and Aims There are a growing number of patients supported by dialysis in older age. In Qatar, 58% of haemodialysis patients are above 60 years. The dialysis care of these patients can be challenging due to frailty, chronic health conditions, diminished QOL, and function. One important reason to favor Assisted Home Haemodialysis (AHHD), especially in the older population is the greater flexibility in conducting dialysis sessions. Our aim is to assess the efficacy of AHHD program in comparison to in-center dialysis. Method This is retrospective study to evaluate AHHD program in Qatar between July 2021 to December 2023 and compare to in-center haemodialysis in complications, compliance, infectivity and cost. Results The AHHD population increased gradually to 122 patients in December 2023. Patients and families reported great satisfaction 98.9% with the AHHD experience as they reported less fatigue post dialysis, eliminated the burden of transportation, and improved quality of life by having HD provided in a home environment. AHHD patients had a significantly lower infection rate (5%) during the COVID-19 outbreak than those receiving in-center dialysis. AHHD freed up crucial slots in hemodialysis centers, increasing the availability of dialysis slots by 10%. Mortality rate was 20.6% while 5 % of patient returned back to in-center dialysis. No major clinical or technical complications had been reported during follow up. The program had resulted in 25% reduction in total cost mainly due to reduction in ambulance use. Conclusion AHHD service increases access to hemodialysis care for elderly patients. The AHHD in Qatar showed great success over 30 months and there was a remarkable increase in the number of patients who started the AHHD with better outcomes regard to QOL and infectivity. It was cost-effective and showed an obvious outcome of reducing ambulance transport utilization and improving patients’ QOL and satisfaction.

Abstract Background and Aims Acute kidney injury requiring dialysis (AKI-D) is a serious medical condition that becomes increasingly common and associated with high rate of mortality. Identifying modifiable predictors of AKI-D patients’ outcomes will lead to better care and improve kidney and patient survival. This study aimed to assess long-term outcomes (ESRD, HD-independence, or death) of AKI-D patients after hospital discharge as well as to identify the clinical predictors of their outcomes. Method We followed 64 AKI-D patients who survived till hospital discharge and continued receiving dialysis treatment in outpatient dialysis center. Follow up continued for 6 months between January 2016 and December 2022 for patients discharged from Hamad General Hospital to receive dialysis in Fahd Ben Jassim Kidney center. All demographic data, patients’ comorbidities, hospital course, laboratory values and outpatient dialysis details were collected and analysed. Follow up of recovered patient was continued for 2 years either in nephrology or low clearance clinic. Results During follow up, 20 patients (31.3%) recovered enough kidney function to stop dialysis while 44 patients (68.7%) were declared as ESRD. The average time to recovery was 106 ± 81 days. No significant difference was noticed between both groups regard to patients’ age, gender or comorbidities. Recovered patients had more ICU admission rate compared to non-recovered patients (60% and 29.5% of patients respectively, p 0.028) and more use of vasopressors (p 0.002). Moreover, the recovered patients received significantly more dialysis sessions during hospital stay than non-recovered patients (p 0.009). Conclusion Close monitoring of AKI-D patients during ICU admission, use of vasopressors, and frequent dialysis sessions may facilitate recovery of kidney function. More research is needed to identify the modifiable predictors of AKI-D patients’ outcomes.

average number of primary care medical visits/patient was 3.6±2.6;individual counseling sessions and goals were 3.7±0.7 and 1.9±0.7,respectively.The most common goals were improved physical activity (75%), blood glucose (46%), blood pressure (15%), serum lipid (4%), weight loss (17%), and adherence to medications (31.%).A multivariate logistic regression analysis showed that receiving CHW's home visits, food assistance, and clothing increased the likelihood of successful goal attainment [(RR=3.17,95%CI 1.62-6.23,P=0.001), (RR=3.03,95%CI 1.08-8.45,P=0.034), (RR=9.23,95%CI 1.7-947.44,P=0.008)], respectively.The household size [RR=0.79,95%CI 0.67-0.94,P=0.009] was associated with lower goal attainment.Medically underserved patients living with or at-risk for CKD successfully set and attained specific self-management goals, with a preference for goals focused on improving physical activity and glucose levels.The likelihood of attaining their goals appeared to be enhanced when patients also received food, clothing, and home visits from CHWs, and reduced when there were more than four members per household.

The literature outlining the overall psychiatric effects of dialysis procedures in patients with chronic kidney disease (CKD) is sparse.The aim of this study was to investigate the subsequent development of substance use disorders in dialysis-dependent ESRD/CKD stage 5 patients.A retrospective cohort analysis was conducted using TriNetX database.Using validated ICD-10 codes, cohort 1 identified those with ESRD or CKD stage 5 who are dependent on renal dialysis, while the control group cohort 2 identified patients with ESRD or CKD stage 5 who are not receiving renal dialysis.After propensitymatching for demographics and related comorbidities, we analyzed the incidence of subsequent substance abuse disorders and clinical outcomes 30 days, 90 days, 1 year, and 5 years thereafter.Values were presented as adjusted Risk Ratios (aRR) with 95% CI.At 30 days, increased risk was observed for opioid abuse or dependence (aRR [95% CI])=(1.25[1.01,1.54])and nicotine dependence (1.16[1.08,1.24]).At 90 days, greater risk for opioid abuse or dependence (1.46[1.26,1.69]),cannabis abuse or dependence (1.35[1.15,1.58]),and nicotine dependence (1.40[1.33,1.48]).The 1-year time frame demonstrated increased risk for alcohol abuse or dependence (1.17[1.10,1.23]),opioid abuse or dependence (1.47[1.33,1.62]),cannabis abuse or dependence (1.58[1.42,1.76]),and nicotine dependence (1.42[1.37,1.48]).Within 5 years, increased risk were observed for alcohol abuse or dependence (1.28[1.22,1.33]),opioid abuse or dependence (1.70[1.59,1.82]),cannabis abuse or dependence (1.84[1.74,2.04]),sedative-hypnotic-anxiolytic abuse (1 .31[1.14, 1.50]) or dependence and nicotine dependence (1.43[1.39,1.47]).The risk for substance use disorder development consistently increased as the time of observation was extended from 1 month to 5 years on maintenance dialysis in CKD patients.These results prove the need for increased awareness and provision of resources for this population.

The literature outlining the overall psychiatric effects of dialysis procedures in patients with chronic kidney disease (CKD) is sparse.The aim of this study was to investigate the subsequent development of substance use disorders in dialysis-dependent ESRD/CKD stage 5 patients.A retrospective cohort analysis was conducted using TriNetX database.Using validated ICD-10 codes, cohort 1 identified those with ESRD or CKD stage 5 who are dependent on renal dialysis, while the control group cohort 2 identified patients with ESRD or CKD stage 5 who are not receiving renal dialysis.After propensitymatching for demographics and related comorbidities, we analyzed the incidence of subsequent substance abuse disorders and clinical outcomes 30 days, 90 days, 1 year, and 5 years thereafter.Values were presented as adjusted Risk Ratios (aRR) with 95% CI.At 30 days, increased risk was observed for opioid abuse or dependence (aRR [95% CI])=(1.25[1.01,1.54])and nicotine dependence (1.16[1.08,1.24]).At 90 days, greater risk for opioid abuse or dependence (1.46[1.26,1.69]),cannabis abuse or dependence (1.35[1.15,1.58]),and nicotine dependence (1.40[1.33,1.48]).The 1-year time frame demonstrated increased risk for alcohol abuse or dependence (1.17[1.10,1.23]),opioid abuse or dependence (1.47[1.33,1.62]),cannabis abuse or dependence (1.58[1.42,1.76]),and nicotine dependence (1.42[1.37,1.48]).Within 5 years, increased risk were observed for alcohol abuse or dependence (1.28[1.22,1.33]),opioid abuse or dependence (1.70[1.59,1.82]),cannabis abuse or dependence (1.84[1.74,2.04]),sedative-hypnotic-anxiolytic abuse (1 .31[1.14, 1.50]) or dependence and nicotine dependence (1.43[1.39,1.47]).The risk for substance use disorder development consistently increased as the time of observation was extended from 1 month to 5 years on maintenance dialysis in CKD patients.These results prove the need for increased awareness and provision of resources for this population.

COVID-19 carries a high risk of morbidity and mortality in dialysis patients. Multiple SARS-CoV-2 variants have been identified since the start of the COVID-19 pandemic. The current study aimed to compare the incidence and outcomes of the COVID-19 Omicron dominant period versus other pre-Omicron period in hemodialysis patients. In this observational, analytical, retrospective, nationwide study, we reviewed adult chronic hemodialysis patients between March 1, 2020, and January 31, 2022. Four hundred twenty-one patients had COVID-19 during the study period. The incidence of COVID-19 due to the Omicron dominant period was significantly higher than other pre-Omicron period (30.3% vs. 18.7%, P<0.001). In contrast, the admission rate to ICU was significantly lower in the Omicron dominant period than in the pre-Omicron period (2.8% vs. 25%, P<0001) but with no significant difference in ICU length of stay. The mortality rate was lower in the Omicron dominant period compared to the pre-Omicron period (2.4% vs. 15.5%, P<0.001). Using multivariate analysis, older age [OR 1.093 (95% CI 1.044-1.145); P<0.0001] and need for mechanical ventilation [OR 70.4 (95% CI 20.39-243.1); P<0.0001] were identified as two independent risk factors for death in hemodialysis patients with COVID-19. In Conclusion, the COVID-19 Omicron variant had a higher incidence and lower morbidity and mortality than pre-Omicron period in our hemodialysis population.

The literature outlining the overall psychiatric effects of dialysis procedures in patients with chronic kidney disease (CKD) is sparse.The aim of this study was to investigate the subsequent development of substance use disorders in dialysis-dependent ESRD/CKD stage 5 patients.A retrospective cohort analysis was conducted using TriNetX database.Using validated ICD-10 codes, cohort 1 identified those with ESRD or CKD stage 5 who are dependent on renal dialysis, while the control group cohort 2 identified patients with ESRD or CKD stage 5 who are not receiving renal dialysis.After propensitymatching for demographics and related comorbidities, we analyzed the incidence of subsequent substance abuse disorders and clinical outcomes 30 days, 90 days, 1 year, and 5 years thereafter.Values were presented as adjusted Risk Ratios (aRR) with 95% CI.At 30 days, increased risk was observed for opioid abuse or dependence (aRR [95% CI])=(1.25[1.01,1.54])and nicotine dependence (1.16[1.08,1.24]).At 90 days, greater risk for opioid abuse or dependence (1.46[1.26,1.69]),cannabis abuse or dependence (1.35[1.15,1.58]),and nicotine dependence (1.40[1.33,1.48]).The 1-year time frame demonstrated increased risk for alcohol abuse or dependence (1.17[1.10,1.23]),opioid abuse or dependence (1.47[1.33,1.62]),cannabis abuse or dependence (1.58[1.42,1.76]),and nicotine dependence (1.42[1.37,1.48]).Within 5 years, increased risk were observed for alcohol abuse or dependence (1.28[1.22,1.33]),opioid abuse or dependence (1.70[1.59,1.82]),cannabis abuse or dependence (1.84[1.74,2.04]),sedative-hypnotic-anxiolytic abuse (1 .31[1.14, 1.50]) or dependence and nicotine dependence (1.43[1.39,1.47]).The risk for substance use disorder development consistently increased as the time of observation was extended from 1 month to 5 years on maintenance dialysis in CKD patients.These results prove the need for increased awareness and provision of resources for this population.

Abstract Background and Aims Home hemodialysis (HHD) usually done as self-care by patient themselves through a portable haemodialysis (HD) machine under training and monitoring by dialysis team. HHD offers greater patient autonomy, cost benefits, treatment-related flexibility, and improved quality of life compared to traditional in-centre HD. Uptake of HHD is limited by patient motivation, cognitive and/or physical barriers, as well as lack of support of family and community. Assisted HHD (AHHD) is a new concept where dialysis team provides HD at home. Usually, it is done by a visiting dialysis nurse using mobile HD machine. Its use is limited due to financial and logistical restriction. We like to present our unique experience in providing AHHD in the State of Qatar. Planning for our AHHD program started in July 2020. Because of COVID-19 pandemic, it faced many challenges and delays. We started first patient in July of 2021. In our program, we use traditional HD machine (not portable) with connection set up in the house in a dedicated room. Special training was provided to AHHD staff regarding the special care needed for home setting (including social, complication of home dialysis settings, decision making, follow up of protocols and policies, etc.) Method We performed a retrospective study between July 1st, 2021 - December 31st, 2022. Our primary objectives were efficiency and safety of AHHD, and secondary objective was cost effectiveness. We included adult chronic HD patients (on HD >3 months) using ambulance (or eligible for it) with functional dialysis access. We excluded patients who are not suitable for home environment (psychiatric illness, aggressive behaviour, etc.). Data were collected from our national electronic health record system. Results 946 patients screened for the program. 237 were eligible (exclusion mostly due to lack of national insurance coverage or not meeting mobility/transport criteria). 121 patients refused to participate (mostly for feeling safer in the clinic setting or improper home environment), 40 patients were undecided, and 76 patients accepted and started AHHD. Age was 73+/-11 years. We had 32 males and 44 females. Mean follow up period were 7 months. 12 patients died and 2 patients returned to dialysis centre during follow up period. Only 15 out of the total 126 hospitalizations were related to dialysis (mostly due to volume overload and non-compliance with dialysis schedule and time). We had 55 patients with permcath and 21 with AV fistulas. We had 8 incidents of dialysis catheter malfunction (6 required tissue plasminogen activator installation in the house setting and only two needed catheter exchange (one had catheter related infection)). No reported significant access bleeding or hypotension episodes. We had 20 technical incidents during the study related to electricity or water supply failures. All incidents were resolved without much interruption of treatment. The program overall was cost effective and reduced cost by over 25% (mostly related to saving of ambulance cost). Patients and their families were very satisfied with the program overall. Conclusion We present a unique successful program related to providing AHHD. Targeting certain dialysis population showed great care, safety, cost saving, better QOL and satisfaction.

Hamad, Abdullah I.; Mohamed, Mohamed; Elshirbeny, Mostafa; Filali, Mossab; Alkhayyat, Dina N.; Abd El Wahed, Ahmed Adel H.; Alzalma, Ismail; Alkadi, Mohamad M.; Al-Malki, Hassan A. Author Information

Patients with end-stage kidney disease (ESKD) are at increased risk for SARS-CoV-2 infection and its complications compared with the general population. Several studies evaluated the effectiveness of COVID-19 vaccines in the dialysis population but showed mixed results. The aim of this study was to determine the effectiveness of COVID-19 mRNA vaccines against confirmed SARS-CoV-2 infection in hemodialysis (HD) patients in the State of Qatar. We included all adult ESKD patients on chronic HD who had at least one SARS-CoV-2 PCR test done after the introduction of the COVID-19 mRNA vaccines on 24 December 2020. Vaccinated patients who were only tested before receiving any dose of their COVID-19 vaccine or within 14 days after receiving the first vaccine dose were excluded from the study. We used a test-negative case–control design to determine the effectiveness of the COVID-19 vaccination. Sixty-eight patients had positive SARS-CoV-2 PCR tests (cases), while 714 patients had negative tests (controls). Ninety-one percent of patients received the COVID-19 mRNA vaccine. Compared with the controls, the cases were more likely to be older (62 ± 14 vs. 57 ± 15, p = 0.02), on dialysis for more than one year (84% vs. 72%, p = 0.03), unvaccinated (46% vs. 5%, p < 0.0001), and symptomatic (54% vs. 21%, p < 0.0001). The effectiveness of receiving two doses of COVID-19 mRNA vaccines against confirmed SARS-CoV-2 infection was 94.7% (95% CI: 89.9–97.2) in our HD population. The findings of this study support the importance of using the COVID-19 mRNA vaccine in chronic HD patients to prevent SARS-CoV-2 infection in such a high-risk population.

Hamad, Abdullah I.; Elshirbeny, Mostafa; Ali, Mohamed Y.; Ghonimi, Tarek A.; Ibrahim, Rania A.; Yasin, Fadumo Y.; Singh, Poonam R.; Aly, Sahar; Abuhelaiqa, Essa; Al-Malki, Hassan A.; Alkadi, Mohamad M. Author Information