Health-related quality of life, mental health and caregiver burden in children with autosomal recessive polycystic kidney disease (ARPKD)

Objective: To examine whether established patient-reported outcome measures are suitable for capturing the impact of ARPKD in children and their families. Method(s): We assessed 44 children with ARPKD (40 families) with respect to patients' health-related quality of life ((hr- QOL) using PedsQLTM ESRD module) and mental health (strength and difficulties questionnaire (SDQ)) as well as family and caregiver burden (Impact on family score (IFS) und Ulm inventory of parental caregiver QOL (ULQIE)) and compared them to published data and 36 healthy control children matched for age and time. Result(s): Patients were aged 9.5 +/- 5.9 years (vs. controls 8.8 +/- 5.0, p = ns) and 21 (48%) were female (vs. 19 controls (53%), p = ns). Mean eGFR was 81 ml/min*1.73m2 (range 4 - 165);7 received dialysis and 11 had functioning kidney transplants (KTX, 2 combined with liver transplants). Eight patients had developmental delay secondary to medical complications, while chronic illness was an exclusion criterion for healthy controls. 61 caregivers of affected children had same gender-distribution (61% vs. 60% mothers) and age (both 42 +/- 7 years) and number of dependent children (1.8 +/- 0.9 vs. 2.0 +/- 0.8) as 57 caregivers of healthy children. The mean proxy reported PedsQL Total score was 77.5 +/- 10.6 (range 59 - 96). It correlated significantly to eGFR (r = 0.5, p < 0.01, (also within the subpopulations pre- and post-KTX)). Parents reported greater mental health problems in affected than in control children with a higher SDQ total score mainly due to higher scores in the hyperactivity and peerinteraction subscales. ULQIE revealed that parents of affected children had significantly lower levels of physical functioning, self-fulfillment and general QOL, but despite higher emotional burden scores they indicated similar satisfaction with family life. Impact on family scores were in a similar range to those of children with moderate to severe disabilities. Conclusion(s): The good spread of PedsQLTM ESRD-scores and their correlation to renal function indicates that it captures significant aspects of ARPKD, however, it may need further adjustment to include liver complications. All four chosen instruments revealed significant impact of ARPKD on hrQOL and mental health of affected children as well as family life and parental wellbeing in comparison to healthy controls. More problems with peer-interactions may also be due to more stringent shielding of chronically ill children from social contacts during the COVID pandemic compared to healthy children.

POST-VACCINATION SARS-COV-2 IGG ANTIBODY TITER IN DIALYSIS PATIENTS

Background: Dialysis patients have a higher COVID-19 fatality rate than the general population and are priority candidates for SARS-CoV-2 vaccination. However, dialysis patients are immunocompromised, suggesting that they may develop a less immune response to COVID-19 vaccination than healthy individuals. Objective and Methods: A total of 358 hemodialysis patients who were twicevaccinated with BNT162b2 were included. SARS-CoV-2 IgG antibody titer was measured within 7 days to 1 month, 1∼2 months, and 3∼4 months after the second vaccination, and factors influencing antibody titer were statistically investigated. SARS-CoV-2 IgG measurement was performed using SARS-CoV-2 IgG II Quant Reagent (Abbott), which is a reagent to quantitatively measure IgG antibodies against the receptor-binding domain of SARS-CoV-2 spike protein. Results: The patients were 240 males (67%) and 118 females, ranging from 37 to 95 years old, with a median age of 70 years. Causes of kidney failure were diabetes mellitus in 35.2%, hypertensive kidney disease in 7.3%, glomerular disease in 30.5%, and polycystic kidney disease in 4.5% of the patients. Comorbidities were hypertension in 64.3% and diabetes in 48.9%. Steroids or immunosuppressive drugs were used in 9% of the patients. SARS-CoV-2 IgG antibody titers at 7 days to 1 month, 1 to 2 months, and 3 to 4 months (median 10, 42, and 98 days) after the second vaccination have the median of 4092 AU/mL(with interquartile range: 1354, 7592), 2199 (927, 4692), and 789 (323, 1559), respectively. Post-vaccination SARS-CoV-2 IgG titers were significantly correlated with Kt/V, the presence of autoimmune diseases, the use of steroids or immunosuppressive drugs, malignancy treatment, and serum albumin and hemoglobin levels. Multivariate analysis showed that the factors that decreased post-vaccination SARS-CoV-2 IgG titer were the use of steroids and immunosuppressive drugs, the presence of malignant tumors under treatment, and hypoalbuminemia. Conclusion: Compared to healthy subjects in previous reports, dialysis patients had lower SARS-CoV-2 IgG titers after COVID-19 vaccination, suggesting that the vaccine may not be sufficiently effective. In addition, SARS-CoV-2 IgG titers are likely to be even lower in patients at high risk for decreased immune response due to medications or comorbidities. Additional vaccination may be essential for hemodialysis patients who are expected to have low SARS-CoV-2 IgG titers.

HYDROXYCHLOROQUINE CAN BREAK YOUR HEART

SESSION TITLE: The Cardiac Intensivist 2 SESSION TYPE: Rapid Fire Case Reports PRESENTED ON: 10/18/2022 12:25 pm - 01:25 pm INTRODUCTION: Hydroxychloroquine and chloroquine are medications derived from aminoquinoline. They are disease-modifying antirheumatic drugs used in the treatment of systemic lupus erythematosus (SLE). Although well tolerated, they do have side effects such as retinopathy, vacuolar myopathy, neuropathy, and as seen in our patient, cardiotoxicity. CASE PRESENTATION: Patient is a 48 year old female with a past medical history significant for chronic kidney disease secondary to autosomal dominant polycystic kidney disease, SLE on hydroxychloroquine who presented to the emergency department complaining of weakness. On arrival the patient was found to be in cardiogenic shock. Her transthoracic echocardiogram revealed a reduced ejection fraction of 37% and a large pericardial effusion concerning for tamponade physiology. Her COVID-19 PCR test was positive. She was taken for emergent pericardiocentesis which revealed 300cc of exudative fluid. Patient’s right heart catheterization revealed mean pulmonary capillary wedge pressure of 23 mmHg, pulmonary artery pressures of 44 mmHg/24 mmHg, mean 31mmHg, cardiac index 1.1L/min/m² by thermodilution, 1.7 L/min/m² by Fick. Following right heart catheterization and intra aortic balloon pump placement, the patient was admitted to the medical intensive care unit (MICU) and placed on intravenous inotropic and vasopressor support. Shortly after arrival to the MICU, patient had an increase in vasopressor requirements. Bedside ultrasound revealed cardiac tamponade. Patient had approximately 400cc of bloody pericardial fluid removed from her pericardial drain. The decision was made for emergent venoarterial extracorporeal membrane oxygenation (ECMO) to be initiated. Endomyocardial biopsy was performed which revealed vacuolization in the cytoplasm of several myocytes as well as lymphocytes in the interstitium of the endocardium. The vacuoles found in the cardiac myocytes were PAS positive. These biopsy results are consistent with hydroxychloroquine cardiotoxicity. The patient’s hydroxychloroquine was discontinued. In addition to hemodynamic support, she also received intravenous immunoglobuluin and systemic steroids. After a prolonged hospitalization she was successfully discharged. DISCUSSION: Cardiotoxicity is a rare adverse reaction seen with hydroxychloroquine. A 2018 systematic review revealed 127 cases of cardiac toxicity associated with the use of hydroxychloroquine or chloroquine. Most patients had been treated with the medication for a prolonged period of time and the toxicity is dose dependent. The mechanism behind hydroxychloroquine and chloroquine induced cardiomyopathy is believed to be secondary to lysosomal dysfunction as a result of toxic phospholipid accumulation in cardiomyocytes. CONCLUSIONS: In patients with new onset cardiomyopathy, a detailed medication reconciliation should be conducted to evaluate for toxins such as hydroxychloroquine and chloroquine. Reference #1: Della Porta, A., Bornstein, K., Coye, A., Montrief, T., Long, B., & Parris, M. A. (2020). Acute chloroquine and hydroxychloroquine toxicity: A review for emergency clinicians. The American Journal of Emergency Medicine. Reference #2: Abbi, B., Patel, S., Kumthekar, A., Schwartz, D., & Blanco, I. (2020). A Case of Cardiomyopathy With Long-term Hydroxychloroquine Use. JCR: Journal of Clinical Rheumatology, 26(8), e300. Reference #3: Chatre, C., Roubille, F., Vernhet, H., Jorgensen, C., & Pers, Y. M. (2018). Cardiac complications attributed to chloroquine and hydroxychloroquine: a systematic review of the literature. Drug safety, 41(10), 919-931. DISCLOSURES: no disclosure on file for Joseph Adams;no disclosure on file for Suliman Alradawi;No relevant relationships by George Kalapurakal No relevant relationships by Mohammed Siddiqui

THE TIME TO NEGATIVE CONVERSION AMONG ADULT COVID-19 PATIENTS ON CHRONIC HEMODIALYSIS ADMITTED AT THE PHILIPPINE GENERAL HOSPITAL

BACKGROUND AND AIMS: In the Philippines, the shortage of dialysis centers that cater to ESKD individuals who tested positive for COVID-19 uniquely presents a logistic challenge, as these patients remain admitted in COVID-19 hospitals with hemodialysis units despite the clinical resolution of their disease. The majority of free-standing hemodialysis units require proof of negative conversion despite recommendations from local guidelines. As proof of negative conversion remains important in many practical settings, the negative conversion rate of SARS-CoV-2 infection has been the subject of several investigations. However, negative conversion rates particularly for ESKD patients with COVID-19 infection are lacking. Hence, this study aims to determine the time to negative conversion of COVID-19 RT-PCR testing among adult COVID-19 patients on chronic hemodialysis admitted at the Philippine General Hospital (PGH), a tertiary government hospital assigned as one of the COVID-19 referral centers in the country. This knowledge will allow for a more systematic and evidence-based implementation of the test-based approach, ultimately determining whether such an approach can be shifted to a symptom or time-based procedure in order to shorten the isolation period and conserve resources, especially in resource-limited settings. METHOD: This is a retrospective cohort study. All adult patients on chronic hemodialysis who were admitted at PGH after the diagnosis of COVID-19 by RTPCR between March 2020 and February 2021 were included. Patients who were asymptomatic for COVID-19, whose charts could not be retrieved, whose COVID-19 RT-PCR results were missing and those who died or got discharged without having a negative COVID-19 RT-PCR result were excluded in this study. Descriptive statistics were used in summarizing the data. Time to negative conversion is the primary outcome measure. RESULTS: A total of 90 patients who were on chronic hemodialysis and tested positive for COVID-19 via RT-PCR admitted at PGH at the specified time period met the inclusion and exclusion criteria. A total of 60% were males and the median age was 55 years old. The mean HD vintage was 2.95 years. Among the causes of ESKD, 46% was from hypertension, 31% was due to diabetes mellitus, 15% due to chronic hemodialysis, 0.80% was caused by autosomal polycystic kidney disease, 2.50% was due to obstructive uropathy and the remaining 4.0% of patients with ESKD were due to other causes such as NSAID nephropathy, gouty nephropathy, etc. Of these, 17% had mild, 53% had moderate, 18% had severe and 12% had critical COVID-19. The mean number of days from the onset of symptoms to clinical recovery is 22.48 days;the median was 18 days. One patient had clinical recovery only after 84 days. The median time to first negative conversion was 24.5 days, with a mean of 26.65 days. There were 6.67% who achieved negative conversion on the first week;15.56% on the second week;24.44% on the third week;26.67% on the fourth week;8.89% on the fifth week;6.67% on the sixth week;4.44% on the seventh week;5.56% on the eighth week;and 1.11% on the ninth week. After 28 days, 90% of the patients had clinical recovery, but 15% of them still had positive RT-PCR results. CONCLUSION: Among adult patients on chronic hemodialysis who were admitted at PGH after the diagnosis of COVID-19 by RT-PCR between March 2020 and February 2021, the median time to negative conversion was 24.5 days.

COVID-19 SEQUELS IN NEPHROLOGY CKD PATIENTS MORE PRONE TO FATALITY

BACKGROUND AND AIMS: This strange, prolonged, very exhausting and costly pandemic still is remaining in our world showing us that we are so weak, so unprotected, so fragile. It initiated and continued mostly like a respiratory syndrome but soon after it was seen that it has a systemic impact and consequences in our body. There were patients presented with hematuria, proteinuria and deteriorating of kidney function after the infection. Direct kidney involvement, cytokine storm, coagulopathy, rhabdomyolysis, antibiotic use, sepsis and haemodynamic instability then acute tubular necrosis, all of these factors contribute in this picture of kidney failure. Very difficult to treat and to rehabilitate a part of these patients are going through kidney replacement therapy and a part are dying. METHODS: We studied a population of 77 patients that had passed the COVID-19 infection, confirmed by RT PCR. Males 58% and females 42%. Mean age was 67.1 years old, the BMI 29.6. Comorbidities were present in our cohort in 59.7% only one comorbidity and in 31.1% of patients two comorbidities. Mean time from COVID-19 infection was 56 days. There were CKD 70% patients and 30% non-CKD. Diagnoses of CKD patients were: hypertensive nephrosclerosis 49%, diabetic nephropathy 32%, chronic glomerulonephritis 9%, ADPKD 8% and transplanted patients with CAN 1%. RESULTS: It resulted that our patients had higher D dimer (mean 3660 mcg/dL = 7-fold higher), PCR (mean 35 mg/dL, 7-fold higher) and ferritin (mean 634 ng/dL). Levels of lymphocytes remained lower (mean 13.9%). Proteinuria was found in 92% and hematuria was found in 88% of our patients. There were a significant correlation between need for KRT and diabetic nephropathy was p = 0.037 and also between need for KRT and proteinuria presence was P = 0.048. It was seen that higher the BUN levels lower the lymphocytes counts (P = 0.028) and lower the time post COVID lower the albumin levels (P = 0.007). It was seen that patients with CKD were more prone to need for haemodialysis treatment during the hospitalization and the mortality rate was higher in this group of patients. CONCLUSION: COVID-19 infection and its short term sequels play an important role on morbidity and mortality of CKD patients. It was a decisive factor for KRT in these patients. CKD patients with their immunosuppression, several drugs used, anemia, hypertension, diabetes are more prone for post COVID-19 complications, hospitalizations, dialysis and then death. Being aware of this dangerous infection and being vaccinated are the mainstay and crucial things for being healthy and being alive.

Extubated VV-ECMO for COVID-19 ARDS in an Immunosuppressed Pediatric Renal Transplant Patient

Extracorporeal membrane oxygenation (ECMO) for coronavirus disease 2019 (COVID-19) associated acute respiratory distress syndrome (ARDS) in adults is common, and outcomes are similar to those for non-COVID-19 ARDS on ECMO1. However, children are much less likely to have severe COVID-19 disease, and thus the need for extracorporeal support is rare2,3. Even pediatric solid organ transplant recipients with active COVID-19 infections typically do not require ECMO.4 We present the case of an immunosuppressed pediatric patient with COVID-19-related ARDS who had an excellent outcome on VV-ECMO. An 11-year-old female with a history of polycystic kidney disease status post renal transplant three years earlier was admitted to the pediatric intensive care unit (PICU) with COVID-19 pneumonia. She developed severe ARDS and required intubation on hospital day (HD) 19. The patient's condition deteriorated despite trials of prone positioning, nitric oxide, and neuromuscular blockade. Due to her refractory hypoxemia, she was cannulated to venovenous (VV) ECMO on HD 28 utilizing a 27 french Avalon bi-caval dual lumen cannula. Laboratory testing demonstrated severely impaired immune function due to her baseline immunosuppressive regimen of mycophenolate, prednisone, and tacrolimus. In addition, she had received B cell depleting therapy with Rituximab several months prior to admission for management of transplant rejection. During her ECMO run, she developed pulmonary aspergillus in addition to her COVID-19 infection. Thus, the overall treatment goal was to minimize immunosuppression sufficiently to manage the COVID-19 and aspergillus infections while not triggering rejection of her transplanted kidney. On admission, her mycophenolate dose and tacrolimus trough goal were reduced. When her status worsened shortly after intubation, mycophenolate was discontinued, and tacrolimus trough goal was further decreased. For treatment of acute COVID-19 pneumonia she received remdesivir, dexamethasone, tocilizumab, and convalescent plasma. Farther into her course, she was also treated for suspected MIS-C with anakinra, IVIG, and methylprednisolone. COVID-19 polymerase chain reaction (PCR) and cycle threshold times were monitored weekly. Gradually her cycle threshold times increased, and her COVID-19 PCR became negative on HD 61. With this laboratory and clinical improvement, her mycophenolate was restarted at 50% of the previous dose, and the tacrolimus trough goal was increased to its previous level. There were no concerns for graft rejection during her hospitalization. However, continuous renal replacement therapy (CRRT) was required in tandem for the first four days of extracorporeal support. Following this, she maintained good renal function with her transplanted kidney for the remainder of her hospital course. Five days after VV-ECMO cannulation she was extubated to a high-flow nasal cannula. With weaning of neurosedative infusions, she was able to participate in pulmonary clearance and physical rehabilitation while on ECMO. With aggressive rehab she could sit on the edge of her bed and even transfer to a chair with assistance. After 33 days of ECMO support, she was decannulated on high-flow nasal cannula. She left the PICU on HD 83 and was discharged home without supplemental oxygen on HD 104. Our case experience suggests that extubated VV-ECMO can be a safe and effective rescue therapy for COVID-19-related ARDS in children, even in the setting of immunosuppression. Modification of immunosuppressive regimens in the setting of acute COVID-19 disease is not well described in the pediatric population.3 Our case study demonstrates it is possible to balance the risk of transplant rejection with infection control and ECMO therapy. Immunocompromised pediatric patients with COVID-19 can be considered candidates for ECMO support.

Outcomes from a dedicated Tolvaptan clinic for patients with Autosomal Dominant Polycystic Kidney Disease (ADPKD)

Introduction: Tolvaptan was approved for use in patients with ADPKD by the Scottish Medicines Consortium in January 2016, following the results of the TEMPO 3:4 trial, which showed that Tolvaptan reduced the rate of decline in kidney function- and increase in total kidney volume- compared to placebo.1 In July 2017, a dedicated Tolvaptan clinic was set up for patients with ADPKD from NHS Greater Glasgow and Clyde and NHS Forth Valley. Eligible patients are referred from their local general nephrology clinic, and counselled on treatment with Tolvaptan by a dedicated team comprised of consultant nephrologists, advanced nurse practitioners and renal pharmacists. Those who choose to proceed with treatment are reviewed monthly in the ADPKD clinic for the first 18 months of treatment before returning to three monthly follow up at their local nephrology clinic.We aimed to analyse the outcomes of patients referred to the ADPKD clinic since July 2017. Method: All patients referred to the Glasgow ADPKD clinic between July 2017 and January 2021 were identified. Prospectively recorded data was collected from SERPR and Clinical Portal and analysed retrospectively. In those who did not commence treatment with Tolvaptan, the documented reason was recorded. In those who commenced Tolvaptan, further data were gathered including duration of treatment, dose achieved, side effects reported, any LFT derangement, and serum creatinine measurements over time. An assessment of whether patients met the Renal Association criteria for eligibility for treatment with Tolvaptan was also made. Results: 78 patients who attended the ADPKD clinic were identified. 60 patients commenced treatment with Tolvaptan. Of those who did not start treatment, the most common reason recorded was anticipated side effects and consequent impact upon quality of life (7/18 patients). 25 of the 60 patients who started Tolvaptan stopped the drug during this period, after a median length of treatment of 11 months (IQR 2-18 months). The most common reason for stopping Tolvaptan was also side effects -23% of our patients stopped for this reason compared to 8% in the trial. The median length of treatment in those patients still on Tolvaptan was 21 months (IQR 14-34.5 months). 29 patients had a break in treatment due to the COVID-19 pandemic. 7 patients (11.7%) had treatment suspended due to deranged LFTs, and 2 (3.3%) of those patients did not resume Tolvaptan, compared to 1.2% of patients in the trial. In retrospect, 4 of the 60 patients commenced on treatment may not have met the criteria for eligibility for treatment with Tolvaptan suggested by the Renal Association. Discussion: Tolvaptan appears to be well-tolerated in the majority of patients. As expected, a significant minority of patients do not tolerate Tolvaptan due to expected side effects. A higher proportion of our patients stopped Tolvaptan due to aquaresis side effects that in TEMPO, but this is not unexpected, and the vast majority of patients in our clinic elected to resume Tolvaptan after an enforced break due to the COVID-19 pandemic. Fewer patients in our clinic were maintained on less than full dose Tolvaptan when compared with the trial. A higher percentage of our patients had Tolvaptan discontinued due to deranged LFTs than in the trial, although this was only 2 patients. The impact of Tolvaptan on CKD progression outwith the trial setting will become clearer over time.

eP270: Incorporating genetic services into adult kidney disease care

Introduction: Chronic kidney disease (CKD) is a debilitating disorder associated with significant morbidity and mortality. CKD diagnoses can have overlapping, non-specific clinical symptoms and histology findings, and the underlying etiology can remain unknown. Recent studies have shown that 1 in 10 adults with CKD has a genetic component to their disease. However, genetic services are limited in this patient population and disproportionally impact those from medically underserved communities. Therefore, an adult kidney genetics clinic was developed within the Division of Nephrology at a large urban academic medical center to increase access to genetic services and testing in adults with kidney disease. Methods: In June 2019, the Division of Nephrology at Columbia University Irving Medical Center created a Kidney Genetics Clinic staffed by genetic counselors (GC) and nephrologists. Initially, appointments were held in-person but transitioned to telemedicine beginning in May 2020 due to the COVID-19 pandemic. The clinic utilized two appointment types: full genetic consults (staffed by a GC and nephrologist) and genetic counseling visits (staffed by a GC only). Genetic counselors implemented several genetic education initiatives to increase clinic referrals and increase provider interest. These included bi-monthly genetic case seminars, monthly genetic research sign-out rounds, a continuing education course focusing on clinical genetics, and genetic counseling student rotations. Results: Between June 2019 and June 2021, the clinic received 277 referrals, averaging 11 per month. Of those referred, 83% were scheduled, and 212 patients underwent genetic evaluation. The median wait time from referral to appointment was 37 days, and the no-show rate was 8%. The majority (89%) of appointments were via telehealth, either by phone or video, while the rest occurred in person. Genetic counseling visits accounted for 21% of patient appointments, and the remaining ones were full genetic consults. Most patients who attended their genetics appointment were in the NY tri-state area (87%), but 12% resided in nine additional states, three other countries, and one US territory. The primary insurance was Medicare in 10% of patients and Medicaid in 17%. Most patients described themselves as white (n=126), while 47 patients reported Hispanic or Latino ethnicity, 36 identified as Black, 15 Asian, and 4 Native Hawaiian or Pacific Islander. The average age of the patient population was 44 years old (ranging from 18 to 87). Patients seen in the genetics clinic were referred for a variety of indications and included several different kidney diagnoses, including: CAKUT (n=6), tubulointerstitial disease (n=26), suspected or clinical diagnosis of a collagenopathy (n=38), focal segmental glomerulosclerosis (FSGS) (n=28), tubulopathy or electrolyte disorder (n=16), cystic kidney disease (including PKD) (n=24), hematuria and/or proteinuria (n=31), complement dysregulation (n=8), tumor or cancer (n=4), and CKD of unknown etiology (n=23). Six patients had a known genetic diagnosis, and 23 were healthy relatives of individuals with a known genetic diagnosis or potential kidney donors. Of patients seen in the kidney genetics clinic, 42% reported a family history of CKD or a personal or family history of a genetic diagnosis. A total of 186 clinical genetic tests were ordered on 174 patients;nine tests still have results pending, and ten were canceled. Genetic tests that were ordered included: small (>50) and large gene panels (n=146), exome sequencing (n=6), microarrays (n=4), and single gene and targeted variant testing (n=20). In patients that did not undergo genetic testing, reasons included: not clinically indicated, testing already ordered, and financial concerns. A diagnostic or candidate diagnostic positive result was reported in 29% of patients, involving 18 different genes. Pathogenic or likely pathogenic variants were most common in COL4A4 (n=11), followed by PKD1 (n=8). Similarly, the highest diagnostic yield was in patients with a referral ndication of a suspected collagenopathy (diagnosis in 50%) or cystic disease (diagnosis in 50%). A non-diagnostic positive finding was identified in 9% of patients and included results such as secondary findings (n=1), carrier status (n=5), and risk factors, such as an APOL1 high-risk genotype (n=9). The identification of a genetic diagnosis in patients impacted several areas of clinical care, including referrals to specialists, kidney donor selection, clinical trial eligibility (for example, in patients with a genetic Alport diagnosis), and increased access to medications (such as tolvaptan in patients with PKD1 variants). In addition, those with non-diagnostic findings were referred to ongoing research studies at the medical center to elucidate the genetics of kidney disease. Conclusion: Here, we describe the successful creation and implementation of an adult kidney genetics clinic at a large medical center. By utilizing a combination of in-person appointments and telemedicine, the clinic was able to provide access to genomic services across a broad geographic region and to a diverse patient population of adults with kidney disease. Genetic education efforts were an integral component of the clinic's success, as it increased visibility and helped providers identify patients who would benefit from genetic services, as evidenced by the high percentage of referred patients scheduling appointments and high diagnostic yield in patients undergoing testing. Identifying genetic diagnoses in this patient population has several clinical implications, including changes in management, eligibility for genetically stratified clinical trials, and treatment decisions. Continued and ongoing access to genomic services is a fundamental component of patient care in adults with kidney disease.