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Kronična bubrežna bolest

Strukturirano praćenje

i liječenje prema smjernicama

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Sadržaj

Uzrok kronične b.b. u djece
Tko ima kroničnu bubrežnu bolest?
Koji su čimbenici progresije?
Liječenje

reverzibilnog oštećenja
radi usporavanje progresije
komplikacija

Redovitost kontrola
Indikacije za RRT

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Čuvanje makronutrijenata

Volumen i sastav tjelesnih tekućina

Krvni

tlak

Eritropoeza

(eritropoetin)

Acido-bazna ravnoteža

Eliminacija otpadnih tvari

Metabolizam kosti

(1,25-OH2-D3)

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Uzrok KBB

NAPRTCS* 2014 Annual Transplant Report

*North American Pediatric Renal Trials and Collaborative Studies

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Tko ima kroničnu bubrežnu bolest?

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Criteria for CKD (either of the following present for 3 months*)
GFR <60 mL/min/1.73 m2 †
GFR >60 mL/min/1.73 m2	Evidence of structural damage or signs of functional renal abnormality (proteinuria, albuminuria, RTA, histology)

*does not apply to newborns or infants <3 months of age; duration is necessary to distinguish from acute kidney injury (AKI)

† does not apply to children <2 years of age

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GFR<30 priprema za bubrežno nadomjesno liječenje:

Transplantacija
Hemodijaliza
Peritonejska dijaliza

Neprimjenjivo u djece <2 godine!!

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Schwartz GJ et al. Pediatr Nephrol. 2007;22:1839.

KDIGO:

odstupanja GFR više od 1 SD zahtjeva pozornije praćenje

više od 2 SD je ozbiljno oštećenje

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pedz.de/

en/gfr.html

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https://www.kuleuven-kulak.be/egfr_calculator/

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Čimbenici progresije

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Wingen AM et al. Lancet 1997;349:1117.

NAPRTCS. Mitsnefes M et al. J Am Soc Nephrol 2003;14:2618.

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CKiD study:

Warady BA et al. Am J Kidney Dis 2015;65:878.

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CKiD study:

Warady BA et al. Am J Kidney Dis 2015;65:878.

ESCAPE trial:

Wuhl E et al. NEJM 2009; 361:1639.

ItalKid Project:

Ardissino G et al. Pediatr Nephrol 2004;19:172.

Neglomerularni uzrok	Glomerularni uzrok
Proteinurija* Povišen krvni tlak† Hipoalbuminemija Mlađa djeca Dječaci Visok fosfor Anemija Dislipidemija	Proteinurija* Povišen krvni tlak† Hipoalbuminemija

*Neovisni čimbenici progresije u ItalKid projektu & ESCAPE pokusu.

†Također u ESCAPE pokusu.

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4C study http://dx.doi.org/10.1016/j.kint.2017.05.006

Acidoza: HCO3 ≤18 mmol/L

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ePTFP [mg/dl] = 10.31 x U_{P [mmol/L]} x S_crea/U_crea

ePTFp = koncentracija fosfata u distalnom kraju proksimalnog tubula

Surogat koncentracije FGF23

ePTFp > 2,32 mg/dl → progresija u ESRD tijekom 5 godina

Clin Sci (Lond) . 2021 Aug 13;135(15):1915-1927. doi: 10.1042/CS20201453.

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ItalKid Project: Ardissino G et al. Pediatrics 2003;111(4 Pt 1):e382

Adolescencija

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Hiperuricemija u CKD G3-5?

Terapija alopurinolom kod urata >420 umol/L

588 odraslih pacijenata, 165 liječeno alopurinolom (58±13 god)

Urati HR 1.09 (95% CI 0.84–1.40), p=0.53 (univar. regresija)

Potrebna dalja istraživanja, uključujući na djeci

PLoS One. 2019; 14(6): e0218510.

doi: 10.1371/journal.pone.0218510

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Do sada ključne točke

Djeca <2 godine normalno imaju nižu GFR

Proteinurija, povišene vrijednosti krvnog tlaka, acidoza i povišen FGF23 su neovisni čimbenici rizika progresije KBB u ESRD

Vodeći uzroci KBB u djece su renalna hipodisplazija, kongenitalna opstruktivna uropatija i fokalna segmentalna glomeruloskleroza

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G1 & G2

GFR>60 mL/min/1.73 m2

Većinom bez simptoma

Potrebno ih je pratiti

radi prevencije

progresije

Edukacija i isticanje čimbenika rizika

Usporavanje progresije KBB

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Liječenje KBB

Liječenje reverzibilnog oštećenja

Smanjena prefuzija bubrega!?
Primjena nefrotoksičnih lijekova!?

Usporenje progresije

Stroga kontrola krvnog tlaka
Smanjenje proteinurije i kontrola acidoze

Liječenje komplikacija KBB

Koje su to komplikacije KBB?

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1a shock, volume depletion, drugs that reduce kidney perfusion, such as NSAID, ACE-I, ARB (#ref)

Mechanism in healthy kidney during low ECV is: reduced sodium excretion (by aldosterone, FeNa is low)

In CKD, patients are unable to normally reabsorb Na, and loss which leads to further damage

So, in acute disease judicious trial with hypovolemia is fluid repletion

1b NSAID, contrasts, aminoglycosides, amphotericin B, cyclo, tacro – avoid use or use with caution with therapeutic drug level monitoring

Bear in mind that cimetidine, TMP, cipro, flucocytosine elevate cerum cretinine secondary to interference with tubular secretion, but BUN is normal

2 progressive long-term kidney damage 2/2 adaptive hyperfiltration with increased intraglomerular perfussion & pressure which leads to glomerular scaring

2a slows progression and reduce cardiovascular risk ESCAPE trial, ACE-i&ARBs are superior to other antiHTN and are recommended to control BP and reduce proteinuria

2b studies in children have not shown a benefit of low protein diet on CKD progression (age-appropriate recommended daily allowance for proteins): lipid lowering th and anemia treatment in kidney slowing CKD progression are inconclusive

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Čuvanje makronutrijenata

Volumen i sastav tjelesnih tekućina

Krvni

tlak

Eritropoeza

(eritropoetin)

Acido-bazna ravnoteža

Eliminacija otpadnih tvari

Metabolizam kosti

(1,25-OH2-D3)

sick

G3-5

Poremećaj Na i volumena tekućina

Hiperkalemija

Hipertenzija

Metabolička acidoza

Anemija

Uremija

Mineralno-koštana bolest

Neuhranjenost

Zastoj rasta

Dislipidemija

Kardiovaskularne bolesti

Endokrini poremećaj

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G4 & G5

Retencija Na i edemi

restrikcija unosa NaCl

(2-3 g/day)

Tiazid*

1-3 mg/kg/d (max 50 mg/d), rani stadiji

Furosemid

0.5-2 mg/kg/d /1 ili 2 doze, kasni stadij

Povećanje ultrafiltracije

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↑K

Povećan katabolizam

Previsok unos K

Metabolička acidoza

↓GFR

↓aldosteron

H+

X

Prehrana siromašna K

Polistiren sulfonat (Sorbisterit)

RRT

Soda bikarbona ako je HCO3<22

X

Poremećen prijelaz u stanice

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CKD: SERUM BICARB ALGORITHM

Kraut JA et al. Am J Kidney Dis 2015 Oct 15

CILJ: HCO₃^->22 mmol/L

NaHCO₃ 2-3 mol/kg/dan

/3-4 doze (početna doza)

titrirati prema rezultatima kontrolnih nalaza

G4 & G5

Metabolička acidoza

KDBZ

The kidney has the principal role in the maintenance of acid-base balance. Therefore, a decrease in renal ammonium excretion and a positive acid balance often leading to a reduction in serum bicarbonate concentration are observed in the course of chronic kidney disease (CKD).

The decrease in serum bicarbonate concentration is usually absent until glomerular filtration rate decreases to <20 to 25mL/min/1.73 m², although it can develop with lesser degrees of decreased kidney function. Non-anion gap acidosis, high-anion gap acidosis, or both can be found at all stages of CKD.

The acidosis can be associated with muscle wasting, bone disease, hypoalbuminemia, inflammation, progression of CKD, and increased mortality. Administration of base may decrease muscle wasting, improve bone disease, and slow the progression of CKD.

Base is suggested when serum bicarbonate concentration is <22 mEq/L, but the target serum bicarbonate concentration is unclear. Evidence that increments in serum bicarbonate concentration > 24 mEq/L might be associated with worsening of cardiovascular disease adds complexity to treatment decisions.

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VanDeVoorde RG et al. Adv Chronic Kidney Dis. 2011;18:355.

Hipertenzija

Dislipidemija

Hiperglikemija

Pretilost

Kardiovaskularne bolesti

vodeći uzrok smrti u KBB

Echo: LVH

KDBZ

Hypertension which is secondary primarily due to volume expansion and activation RAS is common in children with CKD: in G1 max 63% to 80% in G4-5.

Hypertension along with dyslipidemia, abnormal glucose metabolism and obesity is the leading risk factor for cardiovascular diseases in CKD which account for 30% of mortality even in transplant patients, and there are leading cause of death.

Risk for CV diseases is 100X higher compared to general pediatric population.

Mechanism is early atherosclerosis.

A long-term follow-up study of young adults with childhood-onset CKD found that 50% of the deceased patients had died from cerebro- or cardiovascular events.

Coronary artery calcification is one of the earliest cardiovascular markers detected in children with ESRD.

*The increased prevalence of obesity in childhood has also been seen among children with CKD, as approximately 17% of enrolling patients in the CKiD study were obese with a body mass index >95th percentile (Hypertension, 52 (2008), pp. 631–637)

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The ESCAPE Trial Group. NEJM 2009;361:1639.

41.7%

vs.

29.9%

HR=0.65; 95% CI 0.44-0.94; p=0.02

KDBZ

Hypertension is found in >50% of pediatric CKD. Recent studies using ABPM indicate that children with CKD also have a high prevalence of masked hypertension.

Similar to adults, long-standing and uncontrolled hypertension in children is associated with the progression of CKD and development of end-organ damage including early cardiomyopathy and premature atherosclerosis. Aggressive treatment of hypertension should be an essential part of pediatric CKD care, not just to prevent the development of symptomatic cardiovascular disease but also to delay progression of CKD. Recent findings from the European Effect of Strict Blood Pressure Control and ACE Inhibition on Progression of Chronic Renal Failure in Pediatric Patients (ESCAPE) trial have shown that the aggressive treatment of blood pressure, to below the 50th percentile, has even greater benefit in children with CKD, unlike results seen in adult studies.

ESCAPE: 385 children, aged 3-18 y, GFR=15-80, ramipril 6 mg/m2/d, intensified group with target 24h-MAP<50.c., conventional group MAP 50-95.c., followed for 5 y., primary end-point=decline of 50% in GFR or progressing to ESRD, secondary end-points=changes in BP, GFR, and urinary protein excretion.

FIGURE: A total of 29.9% of the patients in the group that received intensified blood-pressure control reached the primary end point, as assessed by means of a Kaplan–Meier analysis, as compared with 41.7% in the group that received conventional blood-pressure control (hazard ratio, 0.65; confidence interval, 0.44 to 0.94; P = 0.02).

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Systolic & diastolic BP percentiles based upon age and gender

+Promjena životnih navika

the European Society of Hypertension guidelines for BP management in children and adolescents recommend target BP below the 75th percentile without proteinuria and below the 50th percentile with proteinuria, likely in lieu of the new findings from the ESCAPE trial (J Hypertens, 27 (2009), pp. 1719–1742)

Routine screening ambulatory BP monitoring is not currently recommended by the K/DOQI guidelines.

Ambulatory BP also better predicts end-organ damage and mortality outcomes in adults. In children with CKD, masked hypertension determined by ABPM was associated with significantly increased risk of cardiac hypertrophy (J Am Soc Nephrol, 21 (2010), pp. 137–144)

Routine use of echocardiography is recommended by the Fourth Report to screen for end-organ injury in children with hypertension or those with pre-hypertension and cardiovascular risk, which includes CKD (Pediatrics, 114 (2 suppl. 4th report) (2004), pp. 555–576).

Lifestyle modifications are often the first recommended treatment in adults and children with mild hypertension (Adv Chronic Kidney Dis. 2011;18:355.)=weight reduction, exercise, low salt in diet.

*BUT, the efficacy of lifestyle modifications in children with CKD and hypertension has not been evaluated and should not be the only prescribed therapy.

The preferred antihypertensive agents for the treatment of hypertension in CKD are ACE inhibitors or angiotensin receptor blockers (ARBs) because of their added effects on proteinuria and the slowing of CKD progression. They may also reduce renal sympathetic activity while also having secondary antifibrotic effects, aiding with cardiac remodeling, as additional rationale for selecting these first-line agents.

The beta-blocker metoprolol was shown to have anti-proteinuric effects comparable with ramipril in the African American Study of Kidney Disease and Hypertension trial,³⁰ while carvedilol has also been shown to have improved anti-proteinuric effects as compared with other beta-blockers.

Diuretics may be considered in children with CKD that do not have salt-losing nephropathies. Thiazide diuretics may be used in patients with earlier stages of CKD, but they too are not effective in children when the GFR decreases below 30 mL/min/1.73 m². Loop diuretics may be considered in CKD stages 4 and 5. Calcium channel blockers (CCB) are also potent antihypertensive medications and may be considered for adjunctive therapy, although their use was associated with uncontrolled hypertension in the CKiD study.

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KBB: proteinurija

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0.5-5 g : Hg <110

5-12 g : Hg <115

12-15 g : Hg <120

>15 f : Hg<120

>15 y m : Hg<130

Od G2 određivati Hg

GFR<43 mL/min

Anemija

Ako je normokromna

normocitna

Serum B12 & folat

Hemokult

Pretrage željeza:

Fe, TIBC, TSAT, feritin

Retikulociti

(norm 0.5-1.5%)

↑

↓

Hemoliza

CKD

↓B12/folat

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Pretrage željeza:

Fe, TIBC, TSAT, feritin

TSAT<20%

Ferritin norm or ↑

Feritin<100 ng/mL

Absolutni deficit Fe

ESA + FE i.v.

Functional Fe deficiency

or

Inflammatory Fe block

Elemental Fe 2-6 mg/kg/d

Monitor Hg & iron studies

TSAT>20%

Feritin>100

Ali Hg<110

ESA+nastavak Fe

Hg 1-2 wk dok nije stabilan, tada svakih 1 mj

Pretrage željeza 1-3 mj

Ferritin drops

& Hg rises

Functional

Fe deficiency

↑Ferritin & no

erythropoiesis

Inflammatory

Fe block

Cilj H 110-120

Izbjegavati Hg>130

CVI incidenti

START

Cilj Hg>110,

TSAT>20 &

feritin>100

Kad norm

svakih 1-3 mo

KDBZ

TSAT=%transferrin Sat=Fe/TIBC*100

-Absolute Fe deficiency is like if TSAT<20% & ferritin<100 ng/mL or <200 ng/mL in dialysis pts bc of functional Fe deficiency.

-Functional Fe deficiency or inflammatory Fe block 🡪 TSAT<20% and ferritin normal or high.

-ESA=erythropoiesis stimulating agent

-ALL patients on ESA REQUIRE iron supplementation to prevent deficiency

-ESA should not be started until iron studies have been evaluated

-KDIGO: ESA primarily to reduce number of transfusions

-ESA: rHuEPO or darbepoetin-alpha in ESRD 🡪 stimulating division & differentiation of erythroid progenitor cells, increased rets release from bone marrow, approved in >1 y when Hg<100 + dialysis (Fe loss 2g/year)

-incomplete response to ESA is 2/2 iron deficiency (adequate iron stores are necessary for max benefit), ALSO: infection, inflammation, chronic blood loss, osteitis fibrosa, aluminum toxicity, low B12, low folate, malnutrition, hemoglobinopathies, hemolysis, carnitine deficiency, Cu deficiency

-rHuEPO dose: 80-120 U/kg/wk in 2-3 div doses, on dialysis or <5y 300 U/kg/wk, expected Hg increase is 10-20 over 2-4 wks

-darbopoetin-alpha dose: 0.25-0.75 ug/kg/wk, once a week

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Anemija: terapijske opcije

Per os željezo 2-6 mg/kg/dan:

Ferrum

Ferosanol kapi (novorođenčad i dojenčad)

IV željezo 0.5 mg/kg svaka 1-2 tjedna

Venofer

Dodaj Folacin 5 mg po

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Nutritivni status

KDOQI: evaluate nutritional status periodically
	G2	G3	G4	G5	Dialysis
Infants	2 wks–3 mo				2 wks–2 mo
1-3 y	1–3 mo
>3 y	6–12 mo	6 mo	3–4 mo		N/A

3-day diet record,

Percentiles (weight, height, BMI, head circ),

Weight/height index,

Normalized protein catabolic rate in HD adolescents

CKD therapy goals:

Height > 3.c.

Weight 10-85 c.

BMI 10-85 c.

ENERGY	100% of estimated req for age
PROTEIN	100-140% of DRI for G3 100-120% for G4-5 Dialysis secondary to protein loss: +0,2 g/kg/day NO protein restriction
MICRONUTs	100% of DRI for B1, B2, B5, B12, A, C, E, K, folate, Cu, and Zn In G5 loss of vitamine A clearance and high risk of hypervitaminosis A : no intake of vit A in G5

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Age	DRI (g/kg/day)	Recommended for CKD G3 (g/kg/day) (100-140% DRI)	Recommended for CKD G4-5 (g/kg/day) (100-120% DRI)	Recommended for HD (g/kg/day)	Recommended for PD (g/kg/day)
0-6 months	1.5	1.5 to 2.1	1.5 to 1.8	1.6	1.8
7-12 months	1.2	1.2 to 1.7	1.2 to 1.5	1.3	1.5
1-3 years	1.05	1.05 to 1.5	1.05 to 1.25	1.15	1.3
4-13 years	0.95	0.95 to 1.35	0.95 to 1.15	1.05	1.1
14-18 years	0.85	0.85 to 1.2	0.85 to 1.05	0.95	1

Recommended dietary protein intake in children with chronic kidney disease

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Adekvatan per os unos!

Nutricionist

Dnevnik prehrane kroz 3 dana

PEG (uvijek prije PD)

Enteralne formule za dojenčad i malu djecu

Većina djece ima premali kalorijski unos i neadekvatan unos makro i mikronutrijenata

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Dislipidemija je u 45% pacijenata s KBB

Ako je TGC > 5,65 mmol/L inicijalno se uvode promjene životnih navika:

prehrana s malim udjelom zasićenih masnih kiselina
svakodnevna tjelesna aktivnost
ograničenje neaktivnosti na <2h/dan

Kod LDL > 3,36 mmol/L uz izostanak terapijskog učinka promjene životnih navika indicirana je:

primjena statina u dječaka >10 godina i djevojčica nakon menarhe.

KDBZ

The prevalence of dyslipidemias in children with CKD and ESRD is high, being present in 39-65 % of patients. Elevated lipid levels in children withoutrenal disease are a risk factor for cardiovascular disease (CVD).

Dislipidemija raste s padom glomerularne filtracije.

The pathogenesis ofdyslipidemia in CKD features various factors, including increased levels of triglycerides, triglyceride-rich lipoproteins, apolipoprotein C3 (ApoC-III), decreased levels of cholesterylester transfer protein and high-density lipoproteins, and aberrations in serum very low-density and intermediate-density lipoproteins.

Dodatni rizici za dislipidemiju su proteinurija nefrotskih razmjera, pretilost, mlađa dob i PD (CKiD study, Bonthuis et al Nephrol Dial Transplant 2014;29:594).

Sva djeca s CKD starija >2g probir na dislipidemiju ili u vrijeme postavljanja dijagnoze, od tada 1x godišnje

Diet is more restrictive in G5

If TGC>5,65 mmol/L -> dietary modification

Very-low fat diet (<15%), use MCT+fish oil

KDIGO does not recommend statins to kids with CKD, but if LDL very high (>3,36 mmol/L) and dietary/lifestyle modifications fail, add statins to boys >10 y and postmenarchal girls

If initial risk assessment indicates that a child with advanced CKD has 2 or more co-morbidities for CVD, first-line treatment should consist of non-pharmacologic management such as therapeutic lifestyle changes and dietary counseling. Pharmacologic treatment of dyslipidemiamay reduce the incidence of CVD in children with CKD/ESRD, but randomized trials are lacking. Statins are the only class of lipid-lowering drugs currently approved by the U.S. Food and Drug Administration (FDA) for use in the pediatric population.

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Sekundarni hiperparatireoidizam

Zastoj u rastu

Renalna osteodistrofija (Osteitis fibrosa cystica)

Patološke frakture

Patološke kalcifikacije

Hiperfosfatemija

Hiperparatireoidizam

Hipovitaminoza D

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↑PTH

↓1,25(OH)₂-D

↑Phosphate

↓Calcium

CaR

Parathyroid

CKD

POJEDNOSTAVLJENA PATOFIZIOLOGIJA KOJA NIJE SASVIM TOČNA

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Fosfat

FGF23-Klotho

Ca

+

Primarni CCP

PTH

kalcitriol

Arterioscler Thromb Vasc Biol. 2021 May 5; 41(5): 1607–1624.

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PTH secretion

Renal osteodystrophy

Ca level increase

1

2

3

1 inhibition of expression of NaPiIIa cotransporter in proximal tubule

2 inhibition of 1α-hydroxylase

3 expression of 24-hydroxylase

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	Pregradnja kosti	Osteoid	Fibroza	Uzrok
Osteitis fibrosa	↑	↓	↑	Visok PTH
Adinamična	↓	↓	↓	Pretjerana supresija PTH
Osteomalacija	↓	↑	↓	deficit vitamina D
Miješana osteodistrofija	↑	↑	↑

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Signs & symptoms

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CKD

↓calcitriol

↑P

P retention

↓production

inhibition

of production

↓intestinal

absorption

removing inhibitory

effect on PTH release

↑PTH

↑urinary P

excretion

↑FGF23

FGFR1

Klotho

proxymal

tubular cells &

parathyroid

inhibition of expression of

NaPiIIa cotransporter

↓renal P

reabsorption

Osteocytes

Osteoblasts

inhibition of

1α-hydroxylase, expression of 24-hydroxylase

expression declines

w/ decreasing GFR

PTH

P

Ca

calcitriol

decreased

clearance

-

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Hiperfosfatemija

Fosfat je vaskularni toksin
U hiperfosfatemiji nastaju kalciproteinske čestice (CPP) s proupalnim učinkom i vaskularnom kalcifikacijom te razvojem ateroskleroze
CCP je povezan s ubrzanim starenjem
Porast P potiče lučenje FGF23 koji preko koreceptora Klotho potiče eliminaciju fosfata iz organizma

Pediatr Nephrol . 2013 Apr;28(4):583-93.

Clin Sci (Lond). 2021 Aug; 135(15): 1915–1927.

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G2 ili više kontrola Ca, P, PTH

Visok

Cilj (pg/mL):

G2-3: 35-70

G4: 70-110

G5: 200-300

Dia: 100-300

Monitoriraj 25-OH-D

G3 ili više worse

ograničenje unosa fosfora

START

Visok P: 80% DRI	Normalan P 100% DRI

<75

>75

Kolekalciferol

START

Ca<2,37

P norm/↓

kalcitriol

START

↓D3

↑vezači P

↑restrikcije P

NE

DA

vezači fosfora

START

G5

PTH>300

CaCO3

Renvela (sevelamer karbonat)

Oleovit D3

KDBZ

Ergokalciferol/kolekalciferol doze:

Serumski 25-OH-D 30-75: 2000 IU/dan/3 mo ili 50000 IU/mjesečno / 3 mo

Serumski 25-OH-D 12-27: 4000 IU/dan/3 mo ili 50000 IU/svaki dr tjedan / 3 mo

Serumski 25-OH-D <12: 8000 IU/dan/1 mo zatim 4000 IU/dan/2 mo ili 50000 IU/tjedno /1 mo zatim 50000 IU 2x mjesečno sljedeća 2 mj

Kolekalciferol je 87% učinkovitiji u dizanju i održavanju serumskog 25-OH-D i proizvodi 2-3x veće zalihe vitamina nego ekvimolarne doze D2. Nema dokaza za sekvestraciju u masti niti za D2 niti D3. Budući da je učinkovitiji i jeftiniji preporučuje se D3.

Kolekalciferol 4000 IU/dan kroz 3 mjeseca.�Kontrola Ca, P: 1 za mjesec dana od početka terapija pa za 3 mjeseca.�Kontrola 25-OH-D za 3 mjeseca.�Kada 25-OH-D bude >75 planirati će se terapija održavanja s 400-1000 IU/dan.

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Ca x P

<5,0 mmol²/L²

>5,0 mmol²/L²

PTH ↓/↓normal

PTH 1-3x normal

PTH ↑target

PTH ↓/↓normal

PTH normal - ↑

PTH ↑ ↑ ↑

Stop active vit D

Use ↓Ca dialysate

Reduce Ca-cont

P-binder

Continue current P-binder & active vit D

Increase active vit D

Consider ADBD

Stop active vit D

Use ↓Ca dialysate

Use Ca-free

P-binder

Consider subtotal parathyroidectomy

↑Ca

↑P

Increase P-binder

Diatery counsel

Stop active vit D

DOI: 10.1007/s00467-005-2082-7

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Pediatr Nephrol. 2020 Mar;35(3):485-491. doi: 10.1007/s00467-019-04399-0.

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Pediatr Nephrol. 2020 Mar;35(3):493-500. doi: 10.1007/s00467-019-04400-w.

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Age	100% DRI (mg/day)	80% DRI (mg/day)
0-6 mo	100	80
7-12 mo	275	220
1-3 y	460	368
4-8 y	500	400
9-19 y	1250	1000

Dietary phospohorus restriction (UpToDate)

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Rast

↓GFR

↑proizvodnja u jetri

↓klirens

IGFBP

↑↑IGFBP

↓↓slobodni IGF-1

Nizak rast

↑GH

↑ukupni IGF-1

Poremećaj volumena i sastava tekućina

Met. acidoza

Anemija

Mineralno-

koštana bolest

Neuhranjenost

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Potporne mjere

Prehrana
Korekcija metaboličke acidoze
Korekcija gubitka elektrolita i tekućine
Liječenje mineralno-koštane bolesti
Liječenje anemije

RRT: HD 3x, HD 6x, PD, Tx
Steroidi: SDD, ukidanje, lijekovi koji štede steroide
rhGH

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Preventing & correcting complications of CKD. Catch-up growth without rhGH (only supportive measures) is observed only in <5y

1) a) EN when spontaneus intake is insufficient. Gastrostomy or NG tube.

2) HD 3x/wk results in further deterioration in growth (2/3 of patients)

HD 6x/wk+nutrition=significant improvements

PD is better for children, especially <6y, more effective in solute/fluid removal than HD, but still growth retardation

Tx is optimal, still no true catch up for >5y

3) Are bad for growth, use SDD, early withdrawal, tacro, MMF – does not infuence graft survival

Before rhGH ½ of patients with childhood onset ESRD achived final height <3rd c.

Earlier th is better at younger age (<6y) and milder stages of CKD

Indications for rhGH:

FIRST, is there any Unresolved modifiable Factor:

BMI < 10% (malnutrition)

HCO3< 22 (acidosis)

Renal salt wasting (fluid requirement >180 ml/kg/day)

PTH > 70 (G2-3), >110 (G4)

Hypothyroid

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Naziv	Indikacija	Primjena	Doza
Tiazid	Hipervolemija	po	1-3 mg/kg/dan (max 50 mg/dan)
Furosemid	Hipervolemija	po, iv	0,5-2 mg/kg/dan u 1 ili 2 doze
Soda bikarbona	Acidoza	po	2-3 mmol/kg/dan u 3-4 doze
Sorbisterit	Hiperkalemija	po, p.rec.	5-10 mL/kg u min 3 dnevne doze (15 g u 150 mL 5% glukoze)
Ferrum, Ferosanol, Venofer	Anemija	po, iv	2-6 mg/kg/dan (po); 0.5 mg/kg svaka 1-2 tj iv.
Neorecormon	Anemija	sc	3 x 20 IU/kg tj (max 750 IU/kg/tj)
Oleovit D3	Deficit vit D	po	2.000 IU/dan /3 mj pa titracija
Rocatrol	Osteodistrofija	po	0,25-0,50 ug /dan ili SDD
CaCO3	Visok P	po	3x dan uz obrok (0,1-0,3 g/kg/dan)
Renvela	Visok P	Po	3x0,4-1,6 g/dan uz obrok

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KBB komplikacije	Lab	Status
1. Stadij KBB	ureja, kreatinin, cistatin C, eGFR
2. Hipervolemija & ↑Na	Na, Cl	Edemi, hropčići, hepatomegalija, galopni ritam
3. Hiperkalemija	K
4. Metabol. acidoza	HCO3
5. Hipertenzija		RR, KMAT
6. Proteinurija	bjelančevine/kreatinin, urin
7. Anemija	Hg, Fe, TIBC, TSAT, feritin, vitamin B12, folat, hemokult	Blijedilo
8. Dislipidemija	kolesterol, LDL, HDL, TGC
9. Rast i razvoj	albumin	TM, TV, OG
10. CKD-BMD	Ca, P, PTH, 25-OH-D, ePTFp	Deformiteti ekstremiteta

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Renalni panel

Na, K, Cl, Ca, Mg, P

urea, kreatinin, cistatin C

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Parametar	Ciljane vrijednosti
RR	24h-MAP<50. centile; RR <75. c. odnosno <50. c. kod proteinurije
Proteinurija	<18 mg/mmol kreatinina, <4 mg/m2/h
HCO3	22-24 mmol/L
Hg	>110 (do 5 godina), >115 (5-12 godina), >120 (12-15 godina), >120 (djevojčice >15 godina), >130 (dječaci >15 godina), kod EPO 110-120 uz izbjegavanje >130
Željezo	TSAT>20% i feritin >100 ng/mL
Rast	TV >3. centile, TM i BMI 10-85. centile
K i Ca	unutar referentnog raspona
P	u donjoj polovici referentnog intervala (G1-4); 1,29-1,93 mmol/L (G5, 1-12 godina); 1,13-1,78 mmol/L (G5 adolescenti)
vit D	>75 nmol/L, <120 nmol/L
PTH	35-70 pg/mL (G2-3), 70-110 (G4), 200-300 (G5), 100-300 (dijaliza)
ePTFp	<2,32 mg/dl

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Dodatne informacije

slaven-abdovic.from.hr

KBB: predavanje, smjernice liječenja i šalabahter

Prehrana u bubrežnim bolestima

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