Table of contents
01 03
Case Illustrations Case Discussion
Renal Tubular
02 04
Acidosis Points to Remember
01
Case Illustrations
Case Illustrations
• Main complaint : general weakness
A 4 years old girl • Polyuria and polydipsi since 2 years old
was admitted in Dr. • She drank almost 3 L/day
Saiful Anwar • Urination > 10x/day, volume > 3L (clear),
General Hospital dysuria (-)
on January 25th 2022 • Difficulty to gain of weight and height
• Difficulty of gait, general weakness
• Bloating
Physical Examination
Weakness on upper & lower
extremities
Meteorismus
BB/U :
11 kg (-3 SD) Short stature
PB/U :
87 cm (<-3 SD) Mild moderate malnutrition
2 th 1 bln
Problem List
Polyuria
Polydipsia
General Weakness
Refractory Hypokalemia
Metabolic acidosis
Short Stature
Malnutrition
Nephrogenic Diabetes Tubulopathy ?
Insipidus ?
Our Kidney
(Pediatrics in Review Vol.22 No.8 August 2021)
Our Kidney Tubulus
(Pediatrics in Review Vol.22 No.8 August 2021)
The Nephron & Electrolytes
(Pediatrics in Review Vol.22 No.8 August 2021)
Renal Regulation of Acid Base Metabolism
Hydrogen Excretion and Bicarbonate Reabsorption in the Proximal Tubule
Ammonia Synthesis and Excretion in the Proximal Tubule
Hydrogen Excretion and Bicarbonate Reabsorption in the Loop of Henle
Metabolism of Ammonia in the Loop of Henle
Hydrogen and Ammonium Excretion in the Distal and Connector Tubules
Hydrogen Excretion in the Collecting Tubule
Excretion of Ammonia in the Collecting Tubule
(Renal Tubular Acidosis in Children: New Insights in Diagnosis and Treatment, Springer, 2022)
(Nagami et al, 2017)
(Nagami et al, 2017)
Physiology of Renal Potassium Handling
K+ renal handling. Various factors that
modulate K+ physiology in the distal nephron
are indicated within the rectangle
(Murillo-de-Ozores, 2022)
02
Renal Tubular Acidosis
How can effect to growth?
Definition
1. Renal Tubular Acidosis (RTA) is a syndrome resulting
from either a defect in proximal tubule bicarbonate
(HCO3-) reabsorption or a defect in distal tubule
hydrogen ion (H-) secretion, or both.
2. This results in normal anion gap hyperchloremic
metabolic acidosis (HCMA).
(Pediatric Nephrology, 4th ed, 2021)
History of RTA
LIGHTWOOD (Calcification in BAINES et al
kidney, anorexia, constipation, In adults
failure to thrive)
1843 1936
1935 1945
“A CHRISTMAS CAROL” BUTTLER et al
By Charles Dickens In children
What Charles Dicken’s character is theorized to
have suffered from RTA?
Tiny Tim
• Growth retardation
• Bone disease
• Intermittent muscle weakness (hypokalemia)
• Kidney stones
• Progressive renal failure
• Death
Lewis DW, Am J Dis Child. 1992 Dec; 146(12): 1403-7
Growth Retardation in RTA
Metabolic acidosis :
Renal calcium wasting
Liberation calcium from
the bone
Direct inhibition of Ca
transport within the nephron
(J Am Soc Nephrol 27: 3511–3520, 2016)
Bone Disease in RTA
Acidosis mediates its effect on bone involves the release of PGE2
Stimulates the expression of receptor activator of nuclear factor k-B
Altering osteoclast differentiation and increasing resorption
Osteoclast activity is stimulated
Osteoblast activity is inhibited by lower pH
(J Am Soc Nephrol 27: 3511–3520, 2016)
Intermittent Muscle Weakness
Increased flow rate
Decreased HCO3 to distal tubule
Metabolic acidosis Bicarbonaturia
reabsorbtion (defects in H+
secretion)
causing increased
K excretion
Muscle weakness Hypokalemia
(urinary K+
wasting)
(J Am Soc Nephrol 27: 3511–3520, 2016)
Kidney Stones
Metabolic acidosis
Defect in secreting H+
Alkaline urinary pH
Calcium phosphate precipitation
Hypercalciuria
Low urinary citrate
Kidney Stones (nephrocalcinosis)
(J Am Soc Nephrol 27: 3511–3520, 2016)
Polyuria
Impaired urinary concentration ability
Salt-losing nephropathy
Induced partly by the hypercalciuria
Chronic hypokalemia from potassium wasting → contributes to
the lack of urine concentrating ability
Damage to the papillae and their collecting systems caused by
interstitial nephritis of chronic hypokalemia also may contribute
(Pediatrics in Review Vol.22 No.8 August 2021)
Classification of RTA
(J Am Soc Nephrol 27: 3511–3520, 2016)
Clinical Characteristics of RTA
(J Am Soc Nephrol 27: 3511–3520, 2016)
Kapan curiga RTA pada anak ?
Bayi Anak Remaja
• Gagal tumbuh • Perawakan pendek • Urolitiasis
• Muntah & dehidrasi dan BB sulit naik
• ISK • Poliuria dan
• Syok (kolaps polydipsia
sirkulasi) • Riket dengan
• Uropati obstruktif terlambat berjalan,
bowing of legs,
frontal bossing
• Urolitiasis
(Adv Ther (2021) 38:949–968)
Stepwise Approach to the Diagnosis of RTA
[Link] 2. Estimates 3. Look for 4. Additional 5. Differentiate
of plasma anion urine pH, urine associated functional type of RTA
gap & K+ AG, and urine defects studies
osmolal gap
(Pediatric Nephrology, 4th ed, 2021)
1. Determination of plasma anion gap & K+
Check blood urea, creatinine, sodium, potassium,
chloride, and bicarbonate
RTA manifest as a normal anion gap hyperchloremic
metabolic acidosis with normal GFR
Plasma anion gap : Na – (Cl + HCO3) < 12
Hypokalemia is accosiated with type 1 and 2 RTA
(Pediatric Nephrology, 4th ed, 2021)
2. Estimates urine pH, urine AG, and urine osmolal gap
Should be measured on a fresh voided, early morning urine specimen
Urine pH < 5.5 suggest RTA type 2 or 4
Urine pH > 5.5 → acidosis suggest distal RTA
Urine anion gap = (Na + K) – Cl
Urine anion gap > 40 mmol/L suggest RTA type 1 or 4
Check urine osmolality
(Pediatric Nephrology, 4th ed, 2021)
3. Look for associated defects
In suspected type 1 In suspected type 2 Audiometry to detect
RTA RTA hearing loss
• Kidney ultrasound • Look for Fanconi • Suggested subtypes
→ medullary syndrome of type 1 RTA
nephrocalcinosis • Urinary amino acid
• Urinary calcium quantification
excretion to look for • Glucosuria on
hypercalciuria dipstick examination
• Urinary citrate
excretion to look for
hypocitraturia
(Pediatric Nephrology, 4th ed, 2021)
4. Additional functional studies
Bicarbonat loading test
Ammonium chloride loading test
Furosemide loading test
• Single oral dose of furosemide at 2 mg/kg (max 40 mg)
• Urine pH is measured hourly for 8 hours
• Urine K is measured at the end of 8 hours
• Interpretation : in hypokalemic type 1 or distal RTA → urine pH does
not fall but the urine K excretion increase
(Pediatric Nephrology, 4th ed, 2021)
5. Differentiate type of RTA
(Pediatric Nephrology, 4th ed, 2021)
Penatalaksanaan RTA
Koreksi asidosis Memperbaiki Koreksi kelainan Pencegahan
metabolik, pertumbuhan tulang nephrocalcinosis
hypokalemia, (BB dan TB)
hiperkalsiuria,
hipofosfatemia dan
hypocitraturia
(Renal Tubular Acidosis in Children: New Insights in Diagnosis and Treatment, Springer, 2022)
Penatalaksanaan RTA
Koreksi asidosis metabolik
• Tipe 1 RTA
• Bayi : 5-8 mmol/kg/hari
• Anak : 2-4 mmol/kg/hari
• Dewasa : 1-2 mmol/kg/hari
• Tipe 2 RTA
• 10-20 mmol/kg/hari
• dosis besar diperlukan pada bayi
(Renal Tubular Acidosis in Children: New Insights in Diagnosis and Treatment, Springer, 2022)
Penatalaksanaan RTA
• Natrium Bicarbonat (Nabic) →
Tipe Alkali oral 500 mg mengandung 6 mmol
mana yang Na+ dan 6 mmol HCO3-
dipilih? • Natrium sitrat/asam sitrat
• Potasium sitrat
(Renal Tubular Acidosis in Children: New Insights in Diagnosis and Treatment, Springer, 2022)
Penatalaksanaan RTA
• Terapi alkali sebaiknya dalam dosis
terbagi tiap 6 jam khususnya pada
Regimen dosis tipe 2 RTA.
alkali
• Pada RTA tipe 1 dosis lebih tinggi
diperlukan pada malam hari.
(Renal Tubular Acidosis in Children: New Insights in Diagnosis and Treatment, Springer, 2022)
Penatalaksanaan RTA
Koreksi • Hipokalemia
gangguan • Hiponatremia
elektrolit
(Renal Tubular Acidosis in Children: New Insights in Diagnosis and Treatment, Springer, 2022)
Penatalaksanaan RTA
• Diagnosis awal/dini dan koreksi
asidosis metabolik
Pencegahan • Diuretik thiazide jika masih refrakter
Nephrocalcinosis setelah koreksi asidosis metabolik
& urolitiasis
→ HCT dosis 1-1.5 mg/kg, 1-2 x/hari
(dosis maksimum 50 mg/x)
(Renal Tubular Acidosis in Children: New Insights in Diagnosis and Treatment, Springer, 2022)
03
Case Discussion
ANALISIS GAS DARAH 26 Januari Normal Range
pH 7,3 7,35-7,45
PCO2 26,1 mmHg 35-45
PO2 38,3 mmHg 80-100
HCO3 11,2 mmol/L 21-28
BE -11,8 mmol/L (-3)-(+3)
Saturasi O2 70,6 % >95%
Hb 10,9 g/dL
Suhu 37 °C
Metabolic Acidosis
Hyperchloremia, Hypokalemia, Anion Gap = 8
Kaliuresis
HEMATOLOGI 25 Januari
Hb 13,20 g/Dl
WBC 18,17 103/µL
Hematocrite 36,30 %
Platelet 517.000
Ureum 14,6 mg/dL Normal GFR
Kreatinin 0,23 mg/dL
Case Illustrations
A 4-year-old girl Treatment
• Main complaint : general weakness 1. Low sodium diit
2. High potassium diit
• Polyuria 3. Oral bicarbonat
• Polydipsi since 2 years old 4. Intravenous and orally potassium
• Bloating (KSR)
• Refractory hypokalemia 5. Hidrochlorotiazide
BB/U : • Normal GFR 6. Ibuprofen
11 kg (-3 SD) • Metabolic acidosis (HCO3 < 12)
• pH urine : 7
PB/U :
• Normal anion gap
87 cm (<-3 SD) • Short stature, malnutrition
2 th 1 bln
Diagnosis : RTA type 1 or 2 ??
04
Points to Remember
Points to Remember
RTA is a disease that occurs when the kidneys fail to excrete acids into the urine, which causes a
person’s blood to remain too acidic.
Without proper treatment, chronic acidity of the blood leads to growth retardation, kidney stones,
bone disease, chronic kidney disease, and possibly total kidney failure.
If RTA is suspected, additional information about the sodium, potassium, and chloride levels in
the urine and the potassium level in the blood will help identify which type of RTA a person has.
In all cases, the first goal of therapy is to neutralize acid in the blood, but different treatments may
be needed to address the different underlying causes of acidosis.
(National Kidney and Urologic Diseases Information Clearinghouse)
(Charles Dickens, 1812-1870)
“An idea, like a ghost, must be spoken to a little
before it will explain itself.”
