What pediatric urologist should know on the chronic renal insufficiency/chronic

renal failure and renal replacement therapy in children

(Cooperation with a pediatric nephrologist)

Postgraduate teaching course, Prague, October 16, 2005

Prof. Jan Janda, MD

Dptm. of Pediatrics, Section for Pediatric Nephrology

University Hospital Motol, Prague, CZ

Chronic renal insufficiency and chronic renal failure in children

■ This issue constitutes a stimulating challenge for the pediatric nephrologist, but also for the pediatric urologist.

■ Pediatric urologist must be an integrated member of the team taking care of patients with this diagnosis

Prenatal diagnosis and severe congenital anomalies of urinary tract

■ Major development in dialysis and other elimination methods (renal replacement therapy = RRT) improved significantly the prognosis of CRI/CRF in this age.

■ Cooperation with pediatric urologists starts even before the child´s birth (prenatal ultrasound diagnosis, even prenatal surgery!!), particularly in children severe anomalies

Prenatal diagnosis and severe congenital anomalies of urinary tract

Some of these inborn defects may result in early deterioration of renal function resulting and a severe complex of problems (impact on thriving, normal somatic/psychical development, growth, etc., still prior the renal replacement therapy is necessary.

To understand each other, the common nomenclature is useful

Chronic renal insufficiency (CRI) in children is mostly defined as plasma creatinine level (Pcr)> 2mg%= 2mg/dl, i.e. ca 176 μmol/L (and >ca 130 μmol/L in infant and toddlers) lasting longer than 6 months (to avoid temporary creatinine elevation, e.g. after acute renal failure).

Grading of the chronic renal insufficiency according the GFR

Stage 1. Kidney damage with normal or even increased GFR (higher than

90 ml/min/ 1.73 m2)

Stage 2. Mild decrease of GFR (between 60-89 ml/min/ 1.73 m2 )

Stage 3. Moderate decrease of GFR (between 30-59 ml/min/ 1.73 m2 )

Grading of the chronic renal insufficiency according the GFR

Stage 4. Severe decrease of GFR (between 15-29

ml/min/ 1.73 m2 )

Stage 5. Chronic renal failure (GFR lower than 15 ml/min/ 1.73 m2- see below!

In this proposal the lower limit of GFR is not more 80 ml/min/ 1.73 m 2l, but 90 ml ml/min/ 1.73 m 2!!

■ American National Kidney Foundation (2002)

Definition of chronic renal failure

■ Chronic renal failure (CRF) is defined as End Stage Renal Disease (ESRD) requiring Renal Replacement Therapy (RRT)

■ This situation may be also called as „renal death“. The kidneys are not more able to fulfill their basic function and without an active intervention (dialysis or successful renal transplantation) the patients cannot survive.

What GFR -value heralds situation when RRT is necessary?

Unfortunately, it is not possible to set the exact level of Pcr, mostly the Pcr reach some 700- 800 μmol/L in school children, but the intervention RRT may become urgent at much lower levels in toddlers or even in infants.Exceptionally, RRT may commence with normal GFR! (congenital nephrotic syndrome)

Evaluation of renal function, also for paediatric urologists:

■ Glomerular filtration rate (GFR)Glomerular filtration rate (GFR) may be may be assessed using the classical test (creatinine assessed using the classical test (creatinine clearence with the urine-collectionclearence with the urine-collection

■ This procedure is limited, particularly in This procedure is limited, particularly in infants, toddlers, pre-school children infants, toddlers, pre-school children (inaccurate urine collection).(inaccurate urine collection).

■ Attempts to use Cystatin A as a marker of Attempts to use Cystatin A as a marker of GFRGFR

Simple assessment of GFR in children older than 1 year

■ The following equation introduced by Schwartz in The following equation introduced by Schwartz in 70thies is used as a routine worldwide:70thies is used as a routine worldwide:

■ GFR= GFR= (body height (body height cmcm x 48 x 48

■ PPcr cr (μmol/L)(μmol/L)

■ This equation gives the GFR in ml/Min./1.73 mThis equation gives the GFR in ml/Min./1.73 m22 BSA BSA and may be used approximately since the first and may be used approximately since the first birthday.birthday.

■ The problem is, the Schwartz formula does not work The problem is, the Schwartz formula does not work so good in children with very high plasma creatinine so good in children with very high plasma creatinine

levels.levels.

The plasma creatinine level is age-dependent (better height-dependent).

■ There is another useful equation you can There is another useful equation you can calculate the calculate the upper limit of plasma creatinine upper limit of plasma creatinine levellevel (P(P

cr maximal cr maximal )) in childhood: in childhood:

■ PPcr(max)cr(max)= height= heightcmcm x 0.61 x 0.61

■ So, e.g. the maximal creatinine level (in So, e.g. the maximal creatinine level (in umol/L) in a child aged 2 years (having height umol/L) in a child aged 2 years (having height some 86 cm) must not exceed 53some 86 cm) must not exceed 53

μmol/L)- μmol/L)- which is which is in adults out of range (but too low!! )in adults out of range (but too low!! )

Incidence of chronic renal insufficiency in children

Epidemiology of the CRI/CRF in children: The incidence of CRI in childhood (taken as

GFR lower than ca 80 ml/ Min./1.73 m2/) does not differ substantially worldwide, the figures show ca 7-10 cases pmcp/ 1 year

(pmcp= per million children population)new nomenclature:

pmarppmarp = per million of age related population

The incidence/prevalence of CRI/CRF/RRT (European Pediatric Registry)

■ The former European register of EDTA collapsed in The former European register of EDTA collapsed in 1994, but now 1994, but now some new data are already available: some new data are already available: more than 3000 patients aged less than 20 years and more than 3000 patients aged less than 20 years and starting RRT between 1980 and the end of 2000 starting RRT between 1980 and the end of 2000 registeredregistered..

■ The incidence of End-Stage Renal-Disease The incidence of End-Stage Renal-Disease rose from rose from 7.1 pmcp in the 1980-1984 to 9.9 pmarp in the 7.1 pmcp in the 1980-1984 to 9.9 pmarp in the 1985-1989 and remained stable thereafter. 1985-1989 and remained stable thereafter. The The prevalence of RRTprevalence of RRT increased from 22.9 pmcp in 1980 increased from 22.9 pmcp in 1980 to as high as 62.1 pmarp in 2000.to as high as 62.1 pmarp in 2000.

The prevalence of CRF/RRT= children surviving on RRT

(from the European Pediatric Registry)

■ The prevalence data range between 0 in some The prevalence data range between 0 in some developing countries to ca 50 patients pmcp or even developing countries to ca 50 patients pmcp or even more in developed countries. The correlation is often more in developed countries. The correlation is often difficult (different criteria, different definition of the difficult (different criteria, different definition of the child- up 15 or up 18, or even up 20 years).child- up 15 or up 18, or even up 20 years).

■ Higher figures of prevalence occurring in the Higher figures of prevalence occurring in the last years are on account of infants and last years are on account of infants and toddlers included in RRT programtoddlers included in RRT program

Number of New Patients Entering RRT

over Time (pmarp)

0

5

10

15

20

1990 1992 1994 1996 1998 2000

0-4

5-9

10-14

15-19

Per million age related population

Incidence of chronic renal failure in children/adolescents

■ Among the children population on RRT the Among the children population on RRT the school children and adolescents prevailschool children and adolescents prevail (ca (ca 35-45% and ca 30% resp.). 35-45% and ca 30% resp.).

■ The proportion of preschool children, toddlers The proportion of preschool children, toddlers and infants is lower than 10%, but an and infants is lower than 10%, but an increasing tendency is reported during the last increasing tendency is reported during the last years (emerging ethical issues in newborns and years (emerging ethical issues in newborns and infants with combined handicap)infants with combined handicap)

Primary Renal Disease by Gender (pmarp)

0

1

2

3

4

Pye

lonep

hritis

Hyp

oplas

ia D

yspl

asia

Glo

mer

ulonep

hr./sc

lero

sis

Hae

moly

tic U

raem

ic S

yndr

ome

Poly

cyst

ic k

idney

s

Her

edita

ry n

ephro

path

y

Mis

cella

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Unkn

own

FemaleMale

Per million age related population (0-19 yrs)

What are the symptoms of chronic renal insufficiency?

■ DiuresisDiuresis decreased, not always! decreased, not always! ■ E.g. poE.g. polyuria is often the leading symptom in lyuria is often the leading symptom in

nephronophtisis nephronophtisis resulting in secondary resulting in secondary bed wettingbed wetting (a (a possible possible pitfall for urologistspitfall for urologists examinig children with examinig children with enuresis !!enuresis !!))

■ AnaemiaAnaemia (paleness), (paleness), headaches headaches (hypertension)(hypertension)■ Growth retardation Growth retardation (growth charts - tracking phenomen)(growth charts - tracking phenomen)

■ Losing the body weightLosing the body weight

What are the symptoms of chronic renal insufficiency?

■ FatigueFatigue, drowsiness, , drowsiness, losinglosing appetiteappetite, restriction of , restriction of physical activitiesphysical activities (sport), sleeping following an (sport), sleeping following an interesting TV-programme, moviesinteresting TV-programme, movies

■ SchoolSchool: sleeping during the classes and unexpected : sleeping during the classes and unexpected bad notes, limited contacts with classmatesbad notes, limited contacts with classmates

■ Losing interest on previous interesting activities Losing interest on previous interesting activities

Conservative treatment of CRI

■ Fluid and salts intakeFluid and salts intake■ Acidobasic regulation (bicarbonate)Acidobasic regulation (bicarbonate)■ Phosphate-binding drugs Phosphate-binding drugs (Ca(Ca++++free drugs preferred)free drugs preferred)

■ Diet- restriction in children mostly not Diet- restriction in children mostly not recommended, caloric intake often only via recommended, caloric intake often only via nasogatrig tubing or PEG!nasogatrig tubing or PEG!

■ Vitamins- D-vitamin derivates (Rocaltrol, D- no Vitamins- D-vitamin derivates (Rocaltrol, D- no polyvitamines compounds (avoid A and E!! )polyvitamines compounds (avoid A and E!! )

Fluids loss and its management■ Very important message for paediatric Very important message for paediatric

urologistsurologists:: relevant fluid loss (diarrhea, relevant fluid loss (diarrhea, vomiting) may result in low blood pressure and vomiting) may result in low blood pressure and limited kidney blood supply - sequel is a limited kidney blood supply - sequel is a decrease of GFRdecrease of GFR!!

■ Mostly, there is a recovery of this complication after Mostly, there is a recovery of this complication after early rehydration, early rehydration, but very often, the value of GFR but very often, the value of GFR does not return to original level! does not return to original level! The parents must be The parents must be informed on this emergency situation and early fluid informed on this emergency situation and early fluid parenteral application must be provided!parenteral application must be provided!

Conservative treatment of CRI

■ ErythropoetinErythropoetin fighting the severe anaemia fighting the severe anaemia■ Antihypertensive treatmentAntihypertensive treatment if high blood if high blood

pressure present pressure present ■ Growth hormonGrowth hormon- starting early!- starting early!■ Message for surgeonsMessage for surgeons: avoid damage of vessels : avoid damage of vessels

in arms (then later possible problems with in arms (then later possible problems with construction of the shunt -fistula)construction of the shunt -fistula)

Decision to start the renal replacement therapy in children

■ Complex evaluation by_Complex evaluation by_■ paediatric nephrologistpaediatric nephrologist■ paediatric urologistpaediatric urologist■ primary care pediatricianprimary care pediatrician■ team of nursesteam of nurses■ psychologist, teachers and thenpsychologist, teachers and then■ FAMILYFAMILY

Indication for the RRT

■ Main difference in RRT in children x adults:Main difference in RRT in children x adults:■ Commencement of RRT in this age automatically Commencement of RRT in this age automatically

takes in account the renal transplantation. takes in account the renal transplantation. ■ Reason: even the best long lasting dialysis cannot Reason: even the best long lasting dialysis cannot

provide an appropriate development/growth in provide an appropriate development/growth in paediatric patients and it is an immense burden paediatric patients and it is an immense burden (very often higher stress for the family than for (very often higher stress for the family than for the child!)the child!)

Preparation for renal replacement therapy

■ Discussion with parents/children, decision to Discussion with parents/children, decision to start the RRTstart the RRT

■ To present posibilities of:To present posibilities of:◆ a) hemodialysisa) hemodialysis◆ b) peritoneal dialysisb) peritoneal dialysis◆ c) preemptive renal transplantation (mostly using a c) preemptive renal transplantation (mostly using a

graft from relative living donor)graft from relative living donor)◆ d) witholding of the active treatment (ethical issue)d) witholding of the active treatment (ethical issue)

Treatment modalities in children

■ HemodialysisHemodialysis: earlier the most common form : earlier the most common form of RRT, but of RRT, but peritoneal dialysisperitoneal dialysis became became popular by the end of 80s. Today, the children popular by the end of 80s. Today, the children are mostly treated at home by are mostly treated at home by automated automated peritoneal dialysis=peritoneal dialysis=APDAPD, using “cyklers”, using “cyklers”

■ Pre-emptive renal transplantation Pre-emptive renal transplantation (renal (renal transplantation without previous dialysis) transplantation without previous dialysis) accounted for 18% of the first treatment accounted for 18% of the first treatment modality in the 1995-2000 treated children. modality in the 1995-2000 treated children.

Team of the paediatric dialysis-transplantation center

■ Paediatric nephrologistsPaediatric nephrologists■ Specialized nurses, dieticiansSpecialized nurses, dieticians■ Surgeons- Surgeons- pediatric urologistpediatric urologist, vascular surgeon, vascular surgeon■ AnesthetistAnesthetist■ Psychologist, social workerPsychologist, social worker■ Teacher Teacher ■ Close contacts with the family Close contacts with the family

Thank you for your attention

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