Patient Presentation
A 6-month-old male came to clinic for his health maintenance visit. He had an eventful past medical history including being diagnosed with posterior urethral valves in-utero with ablation and vesicostomy after birth. His kidney function was currently stable but the family was anticipating his kidney function to progress to end stage kidney disease as there already had been some decline. Although he was growing, he had poor weight gain and linear growth and also had had 1 urinary tract infection. He seemed to be developing appropriately and was saying consonants, sitting up and interacting with the family appropriately. The family history was negative for genetic or renal disease.

The pertinent physical exam showed a thin male growing at the 3rd percentile for weight and 5% for length. Blood pressure was borderline at 102/64. He had the vesicostomy in place along with an NG tube that provided overnight supplemental nutrition. His examination was otherwise unremarkable.

The diagnosis of a patient status-post posterior urethral valve surgery with chronic renal disease was confirmed. The pediatrician offered support to the parents and discussed what he could do to help the family. “You need to be working with the kidney doctors and surgeons mainly, but I can help if he gets sick or you aren’t sure who to call. You can always call me and my partners,” he offered. “I am also willing to be there when some of the big decisions are going to have to be made such as when to do a transplant or start dialysis or something else. I’m not a kidney specialist but I know about kids and your family so I can be there to help you if you want,” he said.

Discussion
Kidneys are amazing in that they filter the blood, eliminate toxins, balance fluids and electrolytes plus have a lot of reserve to do the job they need to do even when injured. Therefore, depending on the problem, chronic renal disease (CRD) often allows time before renal replacement therapy (RRT) is needed. RRT currently is dialysis (peritoneal or hemodialysis) or renal transplant. In general, the patient’s own kidneys do the best job for them but there is always a balance between the problems of CRD and its management, versus the potentially improved renal function with RRT. RRT itself though has a different set of management problems.

Causes of end stage renal disease include:

• *Congenital
  • Hypoplasia/dysplasia (10%)
  • Obstructive uropathies (9.5%)
• *Cystic
  • Polycystic kidney disease
• *Hereditary
• Primary glomerular disease (25%) – mainly focal segmental glomerulosclerosis
• Secondary glomerulonephritis and vasculitis (12%) – mainly lupus nephritis

Congenital, cystic and hereditary combined are the most common grouping which accounts for about 38%.

Primary care providers can support patients and families in their treatment and management decisions, they can promptly evaluate and coordinate care with specialists for acute problems, and provide immunization for all non-live virus vaccines to patients and make sure that family members have all of their own vaccines including live-virus vaccines. Mental health support at all times but especially around anticipated life transitions can help families too. Counseling about long-term health behaviors is important to try to decrease the risk of malignancy and cardiovascular problems including scrupulous use of sun protection measures, no tobacco use, little or no alcohol use, weight management and nutrition, and avoiding dehydration.

Learning Point
ESRD itself carries some innate problems including anemia and mineral bone disorders. Life expectancy varies greatly depending on the underlying disease, management and treatment. Overall, life expectancy is reduced for both genders

RRT options and their advantages and disadvantages includes:

Dialysis
The number of children on dialysis who have other co-morbidities is increasing and therefore these patients may be more complex. Both types of dialysis can have problems with the catheter or infections. For children, peritoneal dialysis is usually preferred over hemodialysis.

Peritoneal dialysis is used as the first dialysis modality for 80-90% of patients. It doesn’t need vascular access, there are fewer dietary restrictions and better catchup growth. It can also be done at home usually overnight, which may be more helpful to the overall family functioning. But this also means that much of the care is reliant on the family to do the dialysis, give multiple medications, monitor feedings, blood pressure, etc.

Hemodialysis has the advantage that much of the care is done by others but there still is the problem of obtaining and maintaining vascular access, increased risk of hemodynamic instability, risk of infection, amount of time to travel and be at a dialysis center. There is also an increased risk of anemia.

Kidney transplant

Advantage

• For children kidney transplant offers many advantages and currently is the preferred form of RRT if possible
• For many patients, shorter times for dialysis and preemptive transplant (patient has kidney transplant without dialysis) may provide longer graft survival
• With transplant, patients have a 10 year survival of 90-95%
• Graft survival is about 12-15 years in children. Therefore children with ESRD often require more than one transplant over their lifetime
• Living related donor transplants have better long-term outcomes than non-living related donors

Problems

• Size – newborns and infants may not be large enough to receive a transplant. Patients need to be around 8-10 kg in size generally.
• Graft – acute or chronic grade rejection, progression of the CRD or recurrence of the primary kidney disease
  Rejection can be difficult to diagnose particularly as there is renal reserve especially in young children who have a large graft relative to their muscle mass, and therefore creatinine levels may have no change. Acute rejection can also be asymptomatic or present with mild fever or mild hypertension.

• Immunosuppression/Infection – patient is immunocompromised, primary viral diseases may cause severe disease such as cytomegalovirus, Epstein-Barr virus and BK polyoma virus
  Diarrhea can cause serious problems as even mild dehydration can significantly alter the kidney function and absorption and metabolism of the immunosuppressant medications.
  UTI is also common.

• Malignancy is very common in adult survivors who had pediatric kidney transplant. ~40% in one study with most being nonmelanoma skin cancer but also a variety of other malignancies
• Cardiometabolic problems – hypertension, diabetes, hyperlipidemia, obesity, for children and adults hypertension, cardiovascular and valve disease are the main long-term causes of mortality
• Growth/development – short stature, developmental delay, delayed puberty, rickets, psychosocial problems related day-to-day activities but also during transition times such as puberty, emerging adulthood and fertility.

Questions for Further Discussion
1. How is renal transplant similar and different to other transplants?
2. What factors influence growth after renal transplant? A review can be found here
3. What causes proteinuria? A review can be found here

Related Cases

Disease: Posterior Urethral Valves | Kidney Failure | Kidney Transplantation | Dialysis

Symptom/Presentation: Growth Problems | Health Maintenance and Disease Prevention | Short Stature | Syndromes

Specialty: General Pediatrics | Nephrology / Urology

Age: Infant

To Learn More
To view pediatric review articles on this topic from the past year check PubMed.

Evidence-based medicine information on this topic can be found at SearchingPediatrics.com and the Cochrane Database of Systematic Reviews.

Information prescriptions for patients can be found at MedlinePlus for these topics: Kidney Failure, Chronic Kidney Disease Kidney Transplant, and Dialysis.

To view current news articles on this topic check Google News.

To view images related to this topic check Google Images.

To view videos related to this topic check YouTube Videos.

Neild GH. Life expectancy with chronic kidney disease: an educational review. Pediatr Nephrol. 2017;32(2):243-248. doi:10.1007/s00467-016-3383-8

Winterberg PD, Garro R. Long-Term Outcomes of Kidney Transplantation in Children. Pediatr Clin North Am. 2019;66(1):269-280. doi:10.1016/j.pcl.2018.09.008

Sanderson KR, Warady BA. End-stage kidney disease in infancy: an educational review. Pediatr Nephrol. 2020;35(2):229-240. doi:10.1007/s00467-018-4151-8

Author
Donna M. D’Alessandro, MD
Professor of Pediatrics, University of Iowa