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Renal tubular acidosis :umbrella_with_rain_drops: (Mx:leaves: (:tulip:More…
Renal tubular acidosis :umbrella_with_rain_drops:
Kidney
Function
:fireworks:
Waste removal
Fluid homeostasis
Electrolyte homeostasis
Acid-base homeostasis*
Blood pressure regulation
Erythropoiesis
Gluconeogenesis
Approach
to
Metabolic acidosis
:tropical_fish:
High anion gap
Normal anion gap
Definition
:linked_paperclips:
Impaired urinary acidification ➯ Clinical state of systemic
hyperchloremic
acidosis
reserved
for individual with poor urinalysis acidification in well-func kidney
RTA causing
Metabolic acidosis — Normal AG acidosis
Causes
:information_source:
met. acidosis caused by either.. or..
Failure to reabsorb sufficient bicarbonate ions from filtrate in the Proximal tubule
Insufficient secretion of H+ ion into Distal tubule
Types
:pizza:
:cake:
Type 1
Distal RTA
H+
fails to secrete
into lumen of nephron by Alpha-intercalated cells of medullary collecting duct of Distal nephron
Labs
:male-scientist:
Normal AG Metabolic acidosis
Elyte: HypoK, HypoCa, HyperCl
Clinical found
:frame_with_picture:
Urinary stone formation
Nephrocalcinosis
Bone demineralisation
Sjogren’s syndrome
Note: if urinary stone formation & nephrocalcinosis
เป็นเยอะๆ นานๆ
➯ Obstruction นานๆ ➯ Renal failure
Etiology
:bread:
Primary etiology
— usu found in :baby:**
Idiopathic, sporadic
Hereditary
AR
AD
Second etiology
Autoimmune ds
Sjogren’s syndrome
SLE
Primary biliary cirrhosis
Thyroiditis
Drugs
Aspiration of toluene
Lithium
Amphotericin B
Ibuprofen
Ifosfamide
Other etiologies
Hypergammaglobulinemia
Sickle-cell anemia
Obstructive uropathy & Chronic pyelonephritis
Dx
:golf:
Failure to thrive (if met. acidosis นานๆ)
Normal AG Metabolic acidosis
HypoK
Urine pH > 5.5
Positive
urinary AG (by Na + K - Cl > 0)
Nephrocalcinosis
:cake:
Type 2
Proximal RTA
Proximal tubular cell
fail to reabsorb
filtered bicarbonate fr urine
Usu.
associated
with
Fanconi syndrome
— “phosphaturia, glucosuria, aminoaciduria, uricosuria & tubular proteinuria”
:warning:
unlike Fanconi’s anemia !
Etiology
:bread:
Primary etiology
Idiopathic, sporadic
Inherited
Inherited renal ds (Idiopathic Fanconi)
AD
:star:
m/c
Sporadic
X-linked (Dent ds)
AR
Inherited syndromes
Cystinosis
Tyrosinemia type 1
Galactosemia
Oculocerebral dystrophy (Lowe syndrome)
Wilson ds
Hereditary fructose intolerance
Second etiology
Intrinsic renal ds
Sjogren’s syndrome
SLE
Primary biliary cirrhosis
Thyroiditis
Drugs
Gentamicin
Cisplatin
Sodium valproate (aed)
Ifosfamide
Heavy metals
Lead
Cadminum
Mercury
Hematologic ds — Myeloma
Organic compounds eg Toluene
Nutrional: Kwashiorkor
Hormonal: primary hyperparathyroidism
Dx
:golf:
Failure to thrive (if met. acidosis นานๆ)
Normal AG Metabolic acidosis
HypoK
Urine pH > 5.5
Vit.D def, rickets
Glucosuria, Aminoaciduria, Phosphaturia
:cake:
Type 3
Combined proximal & distal RTA
rarely disiussed
:cake:
Type 4
Absolute hypoaldosteronism or aldosterone insensitivity
not actually a tubular disorder
Associate
with Mild (normal AG) metabolic acidosis
Mech
Impaired aldosterone secretion or Distal tubule resistance to aldosterone
Impaired func of Na/K-H (cation) exchange mechanism
:arrow_down: H & K secretion
Urine pH < 5.5
HyperK ➯ inhibits Ammnoniagenesis ➯ :arrow_down: H excretion
Major causes of HyperK (type 4) RTA
:bread:
Hypoaldosteronism
DM
Interstitial nephritis
Addison’s ds
Nephrosclerosis
Amyloidosis
SLE
Transplant rejection
Analgesic abuses
NSAIDs
Spironolactone
Aldosterone-Resistance states
Tubulointerstitial ds
Pseudohypoaldosteronism (Type I & II)
Obstructive uropathy
Dx
:golf:
Normal AG Metabolic acidosis
HyperK
Urine pH
< 5.5
Positive
urinary AG (by Na + K - Cl > 0)
Summary
:grapes:
Hx & PE
:bookmark_tabs:
Hx
:notebook:
Growth & development
Recent or Recurrent diarrheal illness
FxHx of mental retardation
Failure to thrive
End-stage renal disease
Infant deaths / Miscarriage
PE
:silhouettes:
Growth parameters
Volume status
Dysmorphic features suggesting an underlying syndrome
Mx
:leaves:
:snow_cloud:
Correct acidosis
: 5-20 mEq/kg of alkali
:star::snow_cloud:
Bicarbonates
(solution,polycitra): 5-8 mEq/kg
:star:
mainstay of Rx in all types of RTA
:snow_cloud:
Potassium citrate
as part of alkali
:snow_cloud:
Dietary Na restriction
:snow_cloud:
Hydrochlorthiazine
To contract ECF
:arrow_up: proximal HCO3 reabsorption
:snow_cloud:
Phosphate supplements
(neutral phosphare, joulie solution)
necessary in Fanconi syndrome
: 1-3 gm/day
:snow_cloud:Small doses of
vit D
may enable healing of rickets — usu found in Type 2
:tulip:
More specfic
Type 4 RTA
:snow_cloud:
Na-K exchange resin
may need Chronic Rx For HyperK
Type 1 RTA
:snow_cloud:Sufficient
bicarb
to reverse acidosis & stops bone dissolution and hyperCa (common in distal RTA)
Type 2 RTA
:snow_cloud:
Bicarb
:snow_cloud:
Oral phosphate supplement
:question: Vit D to offset the secondary hyperparathyroidism that complicates oral phosphate therapy