Rare Kidney Diseases

Specialists dedicated to treating and supporting adult and pediatric patients with rare kidney diseases.

Rare kidney diseases treated at UI Hospitals & Clinics

The University of Iowa has special expertise in the evaluation and management of several of these diseases. Atypical hemolytic uremic syndrome (aHUS) is such a disorder for which new treatments have been identified and several patients from around the country have been successfully transplanted here.

Another type of kidney disease called C3 glomerulopathy (C3GN) or membranoproliferative glomerulonephritis (MPGN), which also includes dense deposit disease (DDD), has a genetic and immunologic basis and can be difficult to treat when it returns in the transplanted kidney.

The Rare Renal Disease Clinic and the Renal Genetics Clinic are among the world’s best in diagnosing and treating rare genetic kidney diseases in children and adults. In the United States, a rare disease is defined as a disease that affects fewer than 200,000 people in the country. There are at least 150 different disorders that are considered to be rare kidney diseases. This means that only a few thousand people in the United States may have one of these diseases, so specialized care providers may be hard to find.

UI Health Care specialists are dedicated to treating and supporting patients with rare kidney diseases. These specialists also research the causes of rare kidney diseases in the laboratory. Clinical trials are ongoing, and some patients may qualify for enrollment in studies of new therapies.

We provide state-of-the-art genetic testing through the Molecular Otolaryngology and Renal Research Laboratories, based at the University of Iowa Roy J. and Lucille A. Carver College of Medicine. The Genetic Renal Panel provides a comprehensive screen of complement genes for the diagnosis of complement mediated kidney disease. These are first steps toward treatment and management.

Patients may choose to come here on their own or have their current provider contact us for referral. We ask that all new patients bring their medical records with them if they have been seen outside of University of Iowa Health Care.

Rare Renal Disease Clinic

Director: Carla M. Nester, MD
Carla M. Nester, MD, is a fellowship-trained and board-certified pediatric nephrologist and adult nephrologist.

The Rare Renal Disease Clinic is a national and international clinical referral center for children and adults who have been diagnosed with:

  • Atypical hemolytic uremic syndrome (aHUS)
  • C3 glomerulopathy (C3G), including dense deposit disease (DDD), also sometimes called type 2 MPGN
  • C3 glomerulonephritis (C3GN)
  • Membranoproliferative glomerulonephritis type 1 and 3 (MPGN 1 and 3)

Patients are seen every Wednesday in the Pediatric Specialty Clinic, located in the John Colloton Pavilion (JCP) near elevator F, level 2. Please talk to our schedulers to arrange appointments on other days of the week.

Rare Kidney Diseases Group Meetings

We also offer our weekly meeting for patients and doctors. This meeting is directed by Richard Smith, MD and Carla Nester, MD. Physicians, patients, and families have their email-submitted questions answered at this renal group meeting.

For patients and families affected by C3G, we also provide support through Kidneeds, a local non-profit organization dedicated to studying genetic kidney diseases. Founded by Richard Smith, MD and Lynne Lanning, Kidneeds raises money for research of C3G and provides information and understanding for C3G patients and their families and caregivers. Kidneeds also sponsors an annual get-together on the University of Iowa campus for patients and their families with C3G.

Renal Genetics Clinic

Director: Christie P. Thomas, MBBS
Christie P. Thomas, MBBS, is a fellowship-trained and board-certified adult nephrologist.

The Renal Genetics Clinic is dedicated to adults who have been diagnosed with rare and inherited kidney diseases other than aHUS, C3G, C3GN, or MPGN 1, and for patients who have kidney diseases that have not been identified. We provide diagnosis and treatment for over 50 different genetic kidney diseases, with special focus on the following disorders:

  • Alport syndrome
  • APOL1 related nephropathy
  • Bartter syndrome
  • Branchio-oto-renal syndrome
  • Cystinosis
  • Fabry disease
  • Familial kidney stone disease
  • Familial hypertension
  • Gitelman syndrome (familial hypokalemia-hypomagnesemia)
  • Hypokalemia (low potassium states)
  • Hypomagnesemia
  • Inherited FSGS syndromes
  • Medullary cystic kidney disease
  • Nephronophthisis
  • Renal cysts and diabetes
  • Renal tubular acidosis

We also offer a clinical evaluation and genetic counseling and testing for patients who may carry a kidney disease gene:

  • Screening for family members at risk for any inherited kidney disease
  • Evaluation for possible genetic kidney disease for patients planning a kidney transplant
  • Counseling and testing living kidney donors for genetic kidney diseases
  • Coordinate therapies tailored to specific genetic diseases

Patients are seen every Monday in the Medicine Specialty Clinics, located in the Pomerantz Family Pavilion (PFP) near elevator L, level 3. Please talk to our schedulers to arrange appointments on other days of the week.

Renal disease clinic

The Organ Transplant Center partners with the Rare Renal Disease and Renal Genetics Clinic, a multi-disciplinary clinic whose purpose is to serve the Iowa community as well as physicians throughout the United States and abroad in the evaluation and management of rare renal diseases in the adult and pediatric patient.

This clinic has a national reputation in complement mediated (a critical part of the immune system) renal diseases, such as atypical hemolytic uremic syndrome and the C3 glomerulopathies, including dense deposit disease.

The clinical team works with Richard Smith, MD and the Molecular Otolaryngology and Renal Research Laboratory at Iowa to provide genetic screening, specific testing, and molecular diagnosis.

Clinical experts at the University of Iowa sponsor an annual conference on atypical HUS and partner with the Foundation for Children with Atypical HUS to provide education and support for patients and their families.

Care Team