1. TMO MEDICAL “B” UNIT. LRH, PESHAWAR
DR. SHAFIQ AHMAD
TMO MEDICAL “B” UNIT.
LRH, PESHAWAR

2. Case History
A Nineteen years old thin boy presented with six months history of pain both the lumber areas with generalized abdominal cramps and body aches. He was also experiencing difficulty in walking, along with epigastric pain and occasional vomiting for the last two months.
No h/o loose motion, joints pains and fever.
A month ago he was again hospitalized in DHQ Nowshehra for vomiting and partially responded to anti-emetic and PPI.

3. ON EXAMINATION
He was a thin lean boy of average height, afebrile and normotensive. Systemic examination was unremarkable. Gait was normal although he was complaining of pain during walking and on standing from sitting position.

4. INVESTIGATION FULL BLOOD COUNT Hb 13gm % TLC 7500/mm3
with normal differential count
Blood Urea 50mg %
S.Creatinine 1.1mg%
S.Calcium 8.5mg%
S.Amylase 171 U/L
Urine microscopy NAD

5. Bilateral nephrocalcinosis
ABDOMINAL ULTRA SOUND
Bilateral nephrocalcinosis

6. Serum Electrolytes & Arterial Blood Gases Analysis
S.Na+ 139meq/L
S.Cl - 113meq/L
S.K+ 3.4meq/L
PH 7.34
PCO2 35.5mmHg
HCO3 18.4mmol/L

7. Ammonium chloride challenge test 0
Ammonium chloride challenge test 0.1 gm/kg Ammonium chloride was given orally and urinary and plasma pH recorded every two hrs. There was no drop in urinary pH below tough the plasma pH fell to This test confirmed the diagnosis of…….

8. Type I distal Renal Tubular hypokalemic, hyperchloremic
DIAGNOSIS
Type I distal Renal Tubular
hypokalemic, hyperchloremic
metabolic acidosis

9. RENAL TUBULAR ACIDOSIS
Definition :
Its a systemic acidosis, resulting due to impaired ability of the Renal tubules to “ ACIDIFY” the urine normally and there will be little or no over all reduction in the Renal function test.
The disease is characterized by
Hypokalemic,Hyperchloremic, metabolic acidosis with Normal Serum Anion Gape [ Na+ ( Cl+HCo3)]

10. DISEASE PRESENTATION
Disease can present in different ways depending upon which aspect of Renal acid handling has been effected.
There may be defect in HCo3 reabsorption in P.C.T
Decrease Amoniogensis in D.C.T.
There may be defective proton secretion.

11. CLASSIFICATION Type I Type II may be inherited or acquired
Type IV : is acquired and associated with decrease Aldasteron level or tubular Hypo responsiveness to Minerlocorticoids.
Type III (distal RTA):

12. DISTAL RTA / TYPE I characterized by:
Hypokalemic , Hyperchloremic metabolic acidosis .
Excess Bicarbonaturia
Inability to decrease urinary PH below 5.5
all this happens because:
Of either excessive backward diffusion of H+ from lumen to the blood or…..
Inadequate transport of H+ ions.

13. ETIOLGY CONGENITAL Familial Marfan syndrome Ehler Danlos syndrome
Sickle cell disease
Hereditary elliptocytosis

14. ACQUIRED PRIMARY may be idiopathetic SECONDARY
Various systemic diseases can lead to
distal RTA

15. HYPERGAMMAGLOBULNAEIMIC STATES
Amylodosis
Cronic liver disease
Cryglobulinaemia
AUTO IMMUNE DISEASE
Sjogoren’s syndrome
Thyroiditis
Auto immune hepatitis
Primary bilary cirrhosis
SLE

16. RENAL TRANSPLANT REJECTION
DRUGS
Amphotericine – B
Lithium
NSAID
Lead
NEPHROCALSINOSIS
Chronic hyper calciurea
Medullary spongy kidney
Chronic Pyleonephritis
RENAL TRANSPLANT REJECTION

17. SYMPTOMS and COMPLICATIONS:
Anorexia
Fatigue
Renal colic
Polyuria/ polydypsia
Bone pain and weakness
( due to rickets in children and osteomalacia)
Constipation
Recurrent UTI
Renal failure

18. INVESTIGATION Electrolyte Arterial Blood Gases Analysis.
Decrease K+ , increase in Chloride
Arterial Blood Gases Analysis.
Decrease in ph HCo3 less than 21 mmol/L
Urine analysis
urinary PH never below 5.5
Decrease urinary NH4
Increase in Ca+
Decrease citrate level
Ammonium chloride challenge test.

19. Treatment Treat the cause: NaHCo3 supplement:
enough Alkali should be given to filtrate the daily metabolic acid load. Usual range is 0.5 to 2mmol/kg/d.
NaHCo3 and shohls solution ( Na+ citrate 1 mmol + citric acid 1mmol)
Potassium Alkali
Thiazide Diuretic : may reduce plasma Vol and then increase PCT reabsorption and HCo3
Vitamin D:

20. TYPE II RTA Less common than type I
Occurs as a part of generalized disorder of P.C.T , function , presenting as
Hyper chloremic acidosis
Other features of Fanconi syndrome that is glycosuria , Aminoacidurea , phosphaturea

21. MECHANISM
The main defect is failure to secrete adequate H+ ions or selective defect in the P.C.T ability to reabsorb filtered HCO3.
About 90% of filtered bicarbonate is absorb by P.C.T, D.C.T has a limited ability to absorb bicarbonate .
When to much bicarbonate is left in the filtrate is delivered to D.C.T, that is over whelming the absorptive capacity of D.C.T, the tubules dose not function adequately and leads to bicarbonate Urea.

22. Eventually the distal delivery of filtered HCO3 declines because the Plasma HCO3levels drops as a result of progressive Urinary loss.
When Plasma HCO3 level drops to 15—18 mmol/L,the distal Nephrone will start function normally, as lower filtered load of HCO3 can be reabsorbed by P.C.T resulting in normal delivery of HCO3 to D.C.T and able to absorb all the HCO3 .
At this point HCO3 Urea disappears and Urinary PH can be acidic.

23. ETLOGOY CONGENITAL Hereditary Autosomal Dominant cystinosis
Galactosaemia
Wilson Disease
von –Greke’s Disease
Hereditary fructose intolerance

24. Acquired
Auto immune :with increase immunoglobulin e.g sjorgren,s syndrome
Drugs : acetazolamide (by inhibiting carbonic anhydrase) lead , tetracyline , copper .
Others : hyper parathyroidsim , amyloidsis,
nephrotic syndrome
Dysprotenanic slate i.e. myloma

25. SYMPTOMS Polyurea Polydypsia Muscle weakness rare
( caused by Hypokalemic Myopathy)
Bone pain from ricket/osteomalacia –rare.

26. INVESTIGTION protein positive FBC--- normal UREA – Creatinin – normal
S.Electrolytes S.K+ decrease
Cl- increase .
Decrease in Ca++ .
Decrease phosphate .
ABGs
Decrease PH ,Decrease HCo3
URINE ANALYSIS
PH increase 5.5
Aminoacidurea
protein positive

27. TREATMENT
Large doses of Alkali i.e mmol/kg/day. Because it is rapidly excreted .
Potassium Citrate may be required
Thiazide diuretic with low salt diet reduces the amount of HCo3 required .

28. TYPE FOUR RTA
This type also called HYPER KALAMIC DISTAL RTA or HYPORENIMIC HYPOALDESTERONISM --- acquired disorder

29. ETIOLOGY Chronic interstitial nephritis Diabetic nephropathy
Primery adrenal disease for example ADDISONS
Hereditary inborn error of steroid synthesis but it is rare
Drugs : AC inhibitor, heparin , trimethoprim , NSAID, spirnolactone.
Obstructive uropathy
Sickle cell disease

30. MECHANISM
weather there is decrease Aldasteron level or Renal Resistance to the effect of Aldasteron , it reduces the ability of distal Nephrone to secret H+ ions. Hyperkalaemia is directly due to loss of Aldasteron action . This causes suppression of Renal production of NH4 which further exacerbates the Acidosis

31. SYMPTOMS
Polyurea , Polydypsia
Lion pain if reflux uropathy is present

32. INVESTIGATION S.Electrolyte S.Na + - normal ( decrease if Addison )
Increase S.K+
PH + decrease
Bicarbonate decrease
Ca++ Normal

33. TREATMENT
Aim: To decrease K+ as acidosis usually improve once Hyperkalemic block of NH4 production is removed
Low potassium diet
Minerlocorticoids supplements i.e.
FLUDROCORTISONE mg/day
This Minerlocorticoids replacement should not be the approach for the patients with hypertension or heart failure history

34. RENA DEFECT SERUM K+ URINARY PH+ URINARY ANION GAPE TITRAT ABLE ACID
TREATMENT
GIT
HCO3 LOSS
Non
<5.5 __
Na+, K+, HCO3
Are Required.
RTA I
Distal H+
Secretion
>5.5 +
NaHCO3
RTA II
Proximal
H+ secretion
HCO3 reab
Normal
NaHCO3 , K+ Alkali
Thiazide Diuretic
RTA
III
NH3 production IV
Distal Na+reab
K+/ H+
Fludrocortisone
Ditary K+
Furosemide

35. THANK YOU