Managing Advanced Chronic Kidney Disease: Cincinnati Fetal Center

If amniotic fluid levels are restored to normal through shunting or amnioinfusion, the likelihood of pulmonary survival increases to approximately 80%.

If amniotic fluid levels are not returned to normal, the pulmonary outcome is poor.

Many infants who are pulmonary survivors have reduced reserve lung function, similar to reduced reserve renal function, and can rapidly transition from pulmonary survivor to non-survivor if injured.

Babies with renal problems who had normal amniotic fluid after replacement have developed chronic lung disease after delivery, demonstrating they are not normal from a respiratory standpoint.

Any kind of urine output is much better than no urine output, even if the urine is of poor quality and does not contain cleared metabolites.

Babies with obstructive uropathy often have a concentrating defect and produce large volumes of urine.

At this center, the size required for safe transplantation is typically between 8 and 10 kg, which usually occurs in the second year of life.

Lower urinary tract management aims to reduce the risk of urinary tract infection through antibiotic prophylaxis and bladder irrigations, and to manage bladder pressure with anticholinergics and catheterization programs guided by urodynamic studies.

Obstructive uropathy bladders can be very high pressure and can change over time, requiring regular follow-up and potentially changing management strategies.

Hypertension is not commonly seen in these babies because they have high urine output and are not volume overloaded, and they tend to lose sodium due to tubular dysfunction.

Normal GFR for a newborn at 1 month of age is about 50, and it takes a whole year to reach what is accepted as normal GFR in older individuals, which is about 100.

Reasonable predictions based on clearance and creatinine cannot be made until a patient has spent the first year of life establishing their kidney function.

The GFR criteria in chronic kidney disease guidelines do not apply to children less than 2 years of age; these children can only be categorized as having normal, moderately reduced, or very severely reduced age-adjusted GFR.

Dialysis is indicated when chronic kidney disease management fails, specifically when the baby stops growing (including head circumference) or when hyperkalemia or metabolic acidosis cannot be medically managed.

Peritoneal dialysis is the technically least difficult way to provide dialysis in small children.

Urine output remains a significant advantage because it is very difficult to manage fluid balance with dialysis alone if there is no residual diuresis.

If parents want aggressive care, full respiratory support should be provided for at least the first 3 to 4 days, as many infants requiring high levels of support (including oscillator or pulmonary vasodilators) will begin to improve after this period.

If the baby is not showing signs of stabilizing and improving after the first 3 to 4 days, the reality that the infant is likely not a pulmonary survivor must be discussed with parents.

The question of pulmonary survival must be readdressed anytime there is an episode of lung injury, such as from sepsis.

When peritoneal dialysis is not available due to peritonitis, short-term hemodialytic strategies can be attempted, though they are challenging due to the need for blood priming and large catheters in small blood vessels.

Aquapheresis (ultrafiltration with convective clearance) using slightly smaller catheters has been successfully used to maintain an anuric baby with intraperitoneal problems.

Temporary hemodialysis catheters in newborns are difficult to maintain because there is not much catheter design for small children, resulting in excess extravascular catheter that moves despite securing, and the 8 French catheter is limited to jugular veins.

Tunneled hemodialysis catheters are easier to maintain from a movement standpoint but are still very large and likely to cause local thrombosis or stenosis in the central circulation.

Nutrition in babies with urine output is easier than in oliguric or anuric babies because the formula does not need to be as concentrated.

High-output babies lose electrolytes and may require supplementation of potassium and phosphorus, which are typically restricted in older chronic kidney disease patients.

The density of formula is essentially an inverse function of urine output.

The need to provide adequate protein intake may necessitate dialysis in some babies because their BUN cannot be managed otherwise.

It is relatively unusual to expect these babies to drink spontaneously in amounts sufficient to supply adequate nutrition.

Breast milk can be incorporated into specialized formulas that meet the baby's specific needs when mothers are motivated to provide it.

The majority of these children have an NG tube or gastrostomy tube, which they keep even around transplant time for medication administration.