The Division of Pediatric Nephrology at UT Southwestern is among the largest pediatric nephrology programs in the United States and is the main provider of clinical services at Children's Medical Center for children with kidney disease and hypertension. The clinical service of the Division consists of the following components: hemodialysis and peritoneal dialysis, inpatient services, clinic services and testing services. Each of these services is coordinated to provide outstanding care for children who have kidney disease and/or hypertension.

At the same time, we are also committed to the training of outstanding physicians and scientists, as well as discovery of new knowledge that will help tomorrow’s patients. Division faculty are devoted to improving the lives of children with renal disease by performing cutting edge research.

Faculty

There are ten pediatric nephrologists in the Division, each with a special area of expertise, including renal development, chronic and congenital kidney disease, X-linked hypophosphatemia, bone disease, nephrotic syndrome, hypertension, and renal tubular disorders.

Two new faculty joined the Division in 2019

Abbie Bauer, M.D. Assistant Professor

B.S. Biology and Bio-Physics, with honors Creighton University, Omaha, NE, 2008 M.D. UT Health Science Center San Antonio, 2013

Postdoctoral Training

Residency, Pediatrics

Oregon Health & Science University, Portland, OR, 2013 – 2016

Fellowship, Pediatric Nephrology

University of Washington School of Medicine, Seattle, WA, 2016 – 2019

Interests:

AKI, QI, Renal Replacement Therapy, Caregiver Burden/Quality of life

Jyothsna Gattenini, M.D. Associate Professor,

Interim Division Chief

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Aditi Dokras, M.D. Assistant Professor

B.S. Human Biology University of California, San Diego, CA, 2007 M.D. St. George’s University School of Medicine, Granada, West Indies, 2013

Postdoctoral Training

Residency, Pediatrics

University of Maryland Medical Center, Baltimore, MD, 2013 – 2016

Fellowship, Pediatric Nephrology

University of California, Los Angeles/Mattel Children’s Hospital, 2016 – 2019

Interests:

Treatment of rejection in kidney transplant recipients and optimizing graft outcomes. Practices

that slow progression of chronic kidney disease. Medication non-adherence impact and

interventions for all renal disease patients.

Honors / Awards

2019 Promotions

Michel Baum – Associate Vice Chair for Faculty Advancement

Keri Drake – Assistant Professor

Jyothsna Gattineni – Interim Division Chief

Matthias Wolf – Associate Professor

Best Pediatric Specialists in Dallas, D Magazine

Jyothsna Gattineni

Raymond Quigley

Mouin Seikaly

Matthias Wolf

Texas Super Doctors, Texas Monthly

Michel Baum

Mouin Seikaly

Jyothsna Gattineni

Capstone Project selected by the Dean – LEAD class of 2019

Invited Lectures

Jyothsna Gattenini

Prune Belly Syndrome Symposium, Dallas, TX, March 2019 o Prune Belly Syndrome: A Nephrologist’s Perspective

Prune Belly Syndrome Network Convention, Phoenix, AZ, July 2019 o Prune Belly Syndrome: A Nephrologist’s Perspective

American Heart Association, Philadelphia, PA, November 2019 o “Prolonged Exposure to Elevated FGF23 Does Not Cause Left Ventricular Hypertrophy in Aged Mice”

Mouin Seikaly

American Society of Pediatric Nephrology, Pathology Webinar, November 2019 o Content Expert Speaker, “IgA Nephropathy”

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Matthias Wolf

University Children’s Hospital, Center for Pediatric Nephrology, Gastroenterology and Metabolic Disorders, MHH Hannover, Germany, September 2019

o "The influence of proteins in human urine on development of kidney stones"

Epithelial Physiology and Cell Biology, Telluride Science Research Center, Telluride, CO, July 2019 o "Klotho ameliorates kidney and heart disease in the UmodC93F/C93F mouse"

American Society of Nephrology, Kidney Week 2019, Washington DC, November 2019 o "Magnesium balance disorders: Principles and Case Discussions"

American Society of Nephrology, Kidney Week 2019, Washington DC, November 2019 o “TRPM6, Uromodulin, and Renal Magnesium Balance"

Conference Presentations

Drake KA, Chaney C, Das A, Carroll T.

Poster Presentation, ReBuilding a Kidney (RBK) Meeting, Bethesda, MD, June 2019 “Stromal YAP/TAZ Signaling Non-autonomously Regulates NPCs and the Renal Microvasculature”

Mansuri A, Modem V, Quigley R.

Poster Presentation, Pediatric Academic Society Conference, Baltimore, MD, April 2019 “Successful Acute Management of Inborn Errors of Metabolism (IEM) in Neonates by Continuous Renal Replacement Therapy”

Raphael Wilson, Neije Mukherjee-Roy, Denisse Leza-Rincon, Yipin Wu, Priyatharshini Alphonse, Jyothsna Gattineni.

American Heart Association, Philadelphia, PA, Month 201. “Prolonged Exposure to Elevated FGF23 Does Not Cause Left Ventricular Hypertrophy in Aged Mice”

Education and Training

The Division of Pediatric Nephrology is one of a hand full of Nephrology Programs to be funded by an NIH T32 grant. This grant has funded the training of some of the most successful and productive pediatric nephrologists in the country. Fellows spend a total of 12 months on inpatient clinical service during the three years of training. All fellows spend one day a week seeing outpatients in the nephrology clinic. They also spend time in the metabolic bone clinic at Texas Scottish Rite Hospital for Children. The rest of their training is devoted to clinical or basic research. The trainees may go into one of the Division’s basic science laboratories or one of the laboratories of others on campus who study renal disease. Some trainees have elected to spend a fourth year of training in renal transplantation.

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Research Activities

The Division of Pediatric Nephrology has four basic research laboratories devoted to research into clinically important issues.

Baum Laboratory

The laboratory has published more than 140 research papers and 70 reviews in chapters in areas related to understanding of how ions are transported across the renal epithelia in both children and adults. The Baum laboratory has been funded by the NIH for more than 25 years and investigated the regulation of salt transport by the neonatal and adult kidney as well as the transporters involved in salt transport. The laboratory has shown that some of the mechanisms involved in both active and passive salt transport are different in neonates than adults. Recently the laboratory has focused on the mechanism of hypertension with prenatal programming. Small for gestational age infants and very premature infants develop hypertension and chronic kidney disease. The Baum laboratory uses a rat model to explore the pathophysiology for the increase in blood pressure and kidney injury with programming. Most recently they have made significant discoveries that have shown that hypertension and kidney injury can be prevented by changes in the postnatal environment. Gattineni Laboratory

FGF23 is a phosphaturic hormone that is elevated in many metabolic bone diseases. It is the first hormone to increase in the blood in patients with chronic kidney disease and the levels increase progressively as renal disease worsens. The Gattineni laboratory has characterized the receptors for this hormone, and in so doing, has generated a mouse that has a 50-fold increase in FGF23 levels. Dr. Gattineni is using this mouse to determine if FGF23 contributes to the bone, vascular and cardiac disease seen in patients with chronic kidney disease. Wolf Laboratory

The Wolf laboratory studies the mechanism and regulation of magnesium and calcium transport. Calcium and magnesium are transported in the distal tubule by channels. This laboratory has made seminal contributions showing how these channels traffic to and stay in the membrane to transport calcium and magnesium. Several diseases, including diabetes, have dysregulated magnesium transport that can make the disease much worse. Dr. Wolf is examining why there is dysregulation of magnesium transport in diabetes and if there are factors that can normalize magnesium transport and improve the outcome of patients with diabetes.

Drake Laboratory

Dr. Drake studies renal development using mouse models. The kidney undergoes dramatic changes during the course of prenatal development. There are thousands of steps mediated by hormones and receptors to produce the filtering part of the kidney and the tubules that regulate salt transport. Abnormal renal development can lead to kidney problems including absence of the kidneys if everything does not go just right. Dr. Drake is exploring the signaling necessary to ensure proper kidney formation and function.

Clinical Research

Pediatric Nephrology faculty members perform clinical research to study the pathogenesis and therapy for children with chronic kidney disease, end stage renal disease, hypertension, and transplantation. Patients with end stage renal disease are at risk for developing metabolic bone disease and opportunistic infections. The Division has made significant contributions examining therapy for these complications. Faculty also care for a large number of children with metabolic bone disease and have made significant strides in improving the care and outcome for patients with X-linked hypophosphatemic rickets.

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Clinical Activities

Dialysis

The Division of Pediatric Nephrology cares for 60-70 patients with end stage renal disease, which places it among the three largest providers of such care for children in the United States. Approximately half of these patients are treated with peritoneal dialysis and half with hemodialysis. Under the direction of Drs. Michel Baum and Raymond Quigley, the Dialysis Program has met all of the federal and state requirements necessary to provide services for the children under its care. The Division provides 24-hour/day and seven-day/week on call services for emergency dialysis to support children with acute kidney injury hospitalized at Children’s.

Inpatient Services The Division provides care to all hospitalized children with hypertension and renal disease at Children’s. The average inpatient census is ten patients. In addition, most of the rheumatology patients that receive intravenous immunosuppressive medication are admitted to the Nephrology service. The division also provides inpatient consultation and manages acute dialysis and continuous renal replacement therapy for the ICU. There is an Attending Physician on call for consultation 24 hours/day and seven days/week. All consults are provided within 24 hours of the time requested.

In addition to providing inpatient services for patients with chronic kidney disease and dialysis requiring hospitalization, the Division also admits and cares for patients who have received renal transplants. This includes patients who have new renal transplants and those who have complications from a transplant such as rejection or opportunistic infection.

Clinic Services Division faculty provide outpatient treatment for children with kidney disease and hypertension in Dallas and surrounding areas. An Attending Physician staffs clinic five days/week. The division also provides outpatient care for those patients who have received renal transplants in the Solid Organ Transplant Program clinic.

Testing Services The Division provides testing services for children with chronic kidney disease and disorders of bone and mineral metabolism. These services are directed by Dr. Mouin Seikaly and include:

Blood Pressure o Measurement of blood pressure in an outpatient and inpatient setting has been found to be unreliable to

assess whether or not the patient has hypertension. The Division offers 24-hour blood pressure monitoring, which is the gold standard for assessment of hypertension.

Bone Mineral Density o The Division oversees a service dedicated to the quantification of bone mineral density. Many patients

suffering from endocrine, rheumatologic, orthopedic and kidney diseases have decreased bone mineral density, which may require therapy to improve bone strength.

Renal Function o Renal function has traditionally been assessed using serum creatinine levels. Unfortunately, serum

creatinine has been found to be an inaccurate measure of renal function in children. Creatinine levels not only reflect renal function but also muscle mass which can be quite low in children with chronic disease. To circumvent this limitation, the Division has been providing an accurate measure of renal function using the Glofil technique, which is the gold standard for assessment of glomerular filtration rate in children.

Metabolic Bone Disease

Dr. Mouin Seikaly runs the Metabolic Bone disease clinic at Texas Scottish Rite Hospital for Children and consults for disorders of metabolic bone disease at Children’s Medical Center. Metabolic bone disease comprises a number of inherited disorders which can often be treated. Dr. Seikaly is a world’s expert on the diagnosis and treatment of metabolic bone disease and has made research discoveries that have translated to improved patient care.

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Patient Statistics 2016 2017 2018 2019

Total number of admissions to C5 354 391 473

New Nephrology Clinic outpatient visits

1,014

Dallas: 883 Plano: 131

1,107

Dallas: 994 Plano: 113

1,058

Dallas: 880 Plano: 178

878

Dallas: 736 Plano: 142

Follow up Nephrology Clinic outpatient visits

3,591

Dallas: 3,118 Plano: 473

3,377

Dallas: 2,969 Plano: 408

3,447

Dallas: 3,045 Plano: 402

2,078

Dallas: 1,633 Plano: 445

Patients seen at Legacy 641

New Patient: 131 Follow Up: 473 Nurse Visit: 37

568

New Patient: 113 Follow Up: 408 Nurse Visit: 47

602

New Patient: 178 Follow Up: 402 Nurse Visit: 22

612

New Patient: 142 Follow Up: 445 Nurse Visit: 25

Acute hemodialysis procedures: Inpatient hemodialysis

429 697 729 735

Patients treated with CVVH 76 84 110

Renal transplants: Living and Deceased 14 11 22 33

Average number of hemodialysis patients 30 33 35 31

Average number of peritoneal dialysis patients 36 38 29.5 28

Current Grant Support

Elizabeth Brown

Grantor: National Institutes of Diabetes and Digestive and Kidney Disease (NIDDK), and the Office for Rare Diseases Research (ORDR) at the National Institutes of Health (NIH), and The NephCure and Halpin Foundations Title of Project: Nephrotic Syndrome Study Network (“NEPTUNE”) Role: Co -Investigator Dates: 2013 - Present

Grantor: National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK) at the National Institutes for Health (NIH). Title of Project: Cure Glomerulonephropathy (CureGN) Role: Co-Investigator Dates: 2013 - Present

Sponsor: Retrophin, Inc Title of Project: Study of Sparsentan in Patients With Primary Focal Segmental Glomerulosclerosis (FSGS) (DUPLEX) Role: Co-Investigator

Date: 2019

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Jyothsna Gattineni

Grantor: NIH-National Institute of DDK Diseases Title of Project: Consequences of Elevated Fibroblast Growth 23 in the Presence and Absence of Kidney Disease Role: Principal Investigator Dates: 07/2018 – 06/2023

Raymond Quigley

Grantor: Children’s Mercy Hospital Title of Project: International Pediatric Peritoneal Dialysis Network (IPPN) Role: Principal Investigator Dates: 06/2010 – 06/2020

Grantor: Baxter Healthcare Corporation Title of Project: Clinical Evaluation of the Prismafelx HF20 Set Role: Principal Investigator Dates: 08/2016 – 08/2021

Grantor: AMGEN Title of Project: An Open-label, Single-arm Study to Assess the Safety and Tolerability of Cinacalcet HCI in Addition to Standard of Care in Pediatric Subjects Age 28 Days to < 6 Years with Chronic Kidney Disease and Secondary Hyperparathyroidism Receiving Dialysis Role: Principal Investigator Dates: 01/2013 – 01/2022

Grantor: Genentech Inc.

Title of Project: An open-label, SINGLE-arm, multicenter study to ascertain the optimal starting dose of MIRCERAâ

given subcutaneously for the maintenance treatment of anemia in pediatric patients with chronic kidney disease on dialysis or not yet on dialysis Role: Principal Investigator Dates: 10/2018 – 10/2023

Mouin Seikaly

Grantor: Children's Research Institute Title of Project: Chronic Kidney Disease in Children (CKID IV) Role: Principal Investigator Dates: 08/2018 – 07/2019

Matthias Wolf

Grantor: NIH-National Institute of DDK Diseases Title of Project: Renal Regulation of the Magnesium Channel TRPM6 by Uromodulin Role: Principal Investigator Dates: 04/2017 – 02/2020 Grantor: US Department of Defense Title of Project: Urinary magnesium regulation by Mucin-1 and Uromodulin - an unexpected role in the development of type 2 diabetes mellitus Role: Principal Investigator Dates: 09/2019 – 09/2022

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Peer-Reviewed Publications

1. Alhamoud I, Huang R, Lacelle C, Burguete D, Hendricks AR, Torrealba JR, Seikaly MG. Allograft outcomes of treated children with kidney transplant who developed plasma cell-rich acute rejection (PCAR): A single center's experience. Pediatr Transplant. 2019 Sep;23(6):e13500. PMID: 31437388

2. Baum M. Editorial: Renal transplantation: a brief history and outlook for the future. Curr Opin Pediatr. 2019 Apr;31(2):210-212. PMID: 30730316

3. Burns EC, Burns B, Newgard CD, Laurie A, Fu R, Graif T, Ward CS, Bauer A, et al. Pediatric Minor Traumatic Brain Injury With Intracranial Hemorrhage: Identifying Low-Risk Patients Who May Not Benefit From ICU Admission. Pediatr Emerg Care. 2019 Mar;35(3):161-169. PMID: 27798539

4. Wang CS, Troost JP, Greenbaum LA, et al, Brown E, Lin JJ, Kaskel F, Gipson DS. Text Messaging for Disease Monitoring in Childhood Nephrotic Syndrome. Kidney Int Rep. 2019 May 7;4(8):1066-1074. PMID: 31440697

5. Drake KA, Ellington N, Gattineni J, Torrealba JR, Hendricks AR. Clinicopathological features of C3 glomerulopathy in children: a single-center experience. Pediatr Nephrol. 2020 Jan;35(1):153-162. PMID: 31667615

6. Drake KA, Fessler AR, Carroll TJ. Methods for renal lineage tracing: In vivo and beyond. Methods Cell Biol. 2019;154:121-143. PMID: 31493814

7. Hou J, Renigunta V, Nie M, Sung A, et al., Wolf MTF. Phosphorylated claudin-16 interacts with Trpv5 and regulates transcellular calcium transport in the kidney. Proc Natl Acad Sci U S A. 2019 Sep 17;116(38):19176-19186. PMID: 31488724

8. Hwang CS, Gattineni J, MacConmara M. Utilizing increased risk for disease transmission (IRD) kidneys for pediatric renal transplant recipients. Pediatr Nephrol. 2019 Oct;34(10):1743-1751. PMID: 31243535

9. Jain J, Legan SK, Alhamoud I, Gattineni J, Baum M. Effect of sex on glomerular filtration rate in programmed rats by prenatal dexamethasone. Physiol Rep. 2019 Jul;7(12):e14154. PMID: 31243892

10. MacConmara M, El Mokdad A, Gattineni J, Hwang CS. Donation after cardiac death kidneys are suitable for pediatric recipients. Pediatr Transplant. 2019 Nov;23(7):e13540. PMID: 31278813

11. Onder AM, Flynn JT, Billings AA, et al, Quigley R, et al; Midwest Pediatric Nephrology Consortium. Correction to: Predictors of patency for arteriovenous fistulae and grafts in pediatric hemodialysis patients. Pediatr Nephrol. 2019 Aug;34(8):1483-1484. PMID: 30684015

12. Potter AS, Drake K, Brunskill EW, Potter SS. A bigenic mouse model of FSGS reveals perturbed pathways in podocytes, mesangial cells and endothelial cells. PLoS One. 2019 Aug 28;14(8):e0216261. PMID: 31461442

13. Rees L, Baum M. Writing a paper for publication. Pediatr Nephrol. 2019 Aug;34(8):1307-1309. PMID: 30109446

14. Wolf MTF, Zhang J, Nie M. Uromodulin in mineral metabolism. Curr Opin Nephrol Hypertens. 2019 Sep;28(5):481-489. PMID: 31205055

15. Wong DG, Arevalo MK, Passoni NM, Iqbal NS, Jascur T, Kern AJ, Sanchez EJ, Satyanarayan A, Gattineni J, Baker LA. Phenotypic severity scoring system and categorisation for prune belly syndrome: application to a pilot cohort of 50 living patients. BJU Int. 2019 Jan;123(1):130-139. PMID: 30113772

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Book Chapters

1. Baum M. (2019) Renal Transplantation: a brief history and outlook for the future. In: Current Opinion in Pediatrics (31: 2: pp 210-212). Philadelphia, PA: Lippincott Williams & Wilkins Ltd.

2. Brown E. (2019) Glomerular Diseases Associated with Hepatitis B and C Infection, Pediatric. In: Trachtman H, Herlitz L, Lerma EV, Hogan JJ (Eds.), Glomerulonephritis (1st ed.) New York, NY: Springer.

3. Okumura K, Vidi S, Desai D. (2019) Renal Auto Transplantation for Renovascular Hypertension Associated with Bifurcated Renal Artery Stenosis in a Pediatric Patient. In: Stylianos S, et al (Eds.), Journal of Pediatric Surgery Case Reports (42: pp 42-44) New York, NY: Elsevier