Renal Tubular Acidosis in briefb(8).pptx

Renal Tubular Acidosis

1. A 30-year-old man presented with sudden weakness of all four limbs Dr Priom Roy  FCPS Part-II Trainee Medicine Unit-IV Dhaka Medical College & Hospital

2. Case Summary Mr  X, 30-year-old-male, normotensive, nondiabetic, nonsmoker, non-alcoholic, hailing from Raipura , N arsingdi admitted in DMCH on 13th S eptember , 2022 with the complaints of- Generalized weakness for   2 years Difficulty in swallowing for same duration Sudden onset weakness of all four limbs for 8 hours.

3. Case Summary (continued) Reasonably well 2 years back Developed generalized weakness & difficulty in swallowing; both developed gradually   Despite weakness he could perform daily activities; weakness had no diurnal variation,   neither associated  with  heat or cold  intolerance, fever, oral ulcer, joint pain, rash; neither was there any worsening following heavy  meal or strenuous activities.

4. Case Summary (continued) There was no history of diarrhoea , blood loss or any respiratory illness H ad difficulty in swallowing- worsened during initiation; more often to solid than liquid. For  that reason, he used to take frequent sips of water during meal; there was history of frequent vomiting during or after meals.

5. Case Summary (continued) H e developed sudden onset weakness of all four limbs, 8 hours prior admission which was not associated with any  pain, numbness, bowel or bladder involvement Had similar episodes of acute limb weakness (4-5 times), in recent past which responded to conservative management.

6. Case Summary (continued) On query, he informed about unintentional significant weight loss (7kg  in last 2 months), associated with loss of appetite No family history of consanguineous marriage among parents; family members have no such illness Immunized as per EPI schedule & vaccinated against  COVID-19.

7. General Examination (during admission) Appearance I ll l ooking Build, Nutrition Average Decubitus On choice Anemia Mildly anemic Jaundice, Clubbing Absent Koilonychia, Leukonychia Absent Oedema, Dehydration Absent Lymph node Absent 13/08/2022

8. General Examination (continued) Bony tenderness Absent Pigmentation Absent Thyroid gland Not enlarged Body hair Normal Pulse 88/min (regular) Blood pressure 90/60 mmHg with no postural drop Respiration 18 breaths/min Temperature 98.8 F

9. General Examination (continued) Bed side urine examination Protein Positive Sugar Nil

10. Neurological Examination (on admission) 1) Higher c erebral f unctions : Normal 2) Cranial nerves including fundoscopy : Intact 3) Sensory system : Normal

11. Neurological Examination(motor system) Motor examination of lower limbs: Right Left Power 1/5 (both proximal & distal) 1/5 (both proximal & distal) Tone Reduced Reduced Jerk Diminished Diminished Planter Equivocal Equivocal

12. Neurological Examination(motor system) Motor examination of upper limbs: Right  Left Power 3/5 (both proximal & distal) 3/5 (both proximal & distal) Tone Reduced Reduced Jerk Normal Normal

13. Systemic Examination (other system) Precordium Normal Respiratory system Normal Abdomen Normal Musculoskeletal system Normal (except less power as mentioned)

14. Weight loss Difficulty in swallowing Vomiting Problem list Generalized weakness followed by sudden weakness of all 4 limbs

15. Provisional diagnosis?

16. Provisional diagnosis Periodic paralysis

17. Differential diagnosis Polymyositis Guillain-Barre syndrome Myasthenia gravis

18. Investigations CBC: Haemoglobin 8.5 g/dl WBC 18,000/ cmm Neutrophil 71% Lymphocyte 24% Platelet 4,02,000/ cmm ESR 25 mm in 1st hr

19. Investigations (continued) PBF : RBC- Normocytic normochromic morphology WBC- Matured with increased total count Platelet- Normal Comment: Normocytic normochromic a naemia .

20. Investigations (continued) Urine R/E Albumin Trace Sugar Nil Pus cell 0-2/HPF RBC 0-1/HPF Cast Nil

21. Investigations (continued) RBS 6.2 mmol/L Serum b ilirubin 0.80 mg/dl SGPT 33 U/L Serum urea 25 mg/dl TSH 3.50 uIU /ml Serum c reatinine 3.32 mg/dl

22. Investigations (continued) Serum electrolytes : ​ 13.9.22​ 14.9.22​ 15.9.22​ 19.9.22​ 22.9.22​ 25.9.22​ 1.10.22​ Sodium​ 128​ 127​ 139​ 118​ 130​ 127​ 136​ Potassium​ 2.0​ 2.4​ 3.15​ 1.4​ 2.0​ 2.19​ 2.37​ Chloride​ 104​ 96​ 102​ 91​ 98​ 105​ 108​ HCO3​ 18​ 8​ 12​ 10​ 20​ 20​ 17​

23. Investigations (continued) Test Name​ 14.9.22​ 15.9.22​ 19.9.22​ 22.9.22​ 25.9.22​ S. Magnesium​ 1.15​ 1.12​ 1.23​ 1.15​ 1.0​ S. Calcium​ 5.8​ 6.0​ 4.6​ 5.5​ 4.8​

24. Investigations (continued) Intact PTH​ 175  pg /ml​ S. Albumin ​ 3.9 g/dl​ S. P hosphate ​ 4.1 mg/dl​ HBsAg​ Negative​ Anti HCV​ Negative​ Anti HBc (total)​ Negative​ Anti HIV  (1&2)​ Negative​ LDH 155 U/L

25. ECG Comment: Normal

26. Chest X-Ray P/A view Comment: Normal

27. Plain Xray Abdomen A/P view in erect posture Comment : Normal

28. USG of whole a bdomen

29. Urinary electrolytes: Test Result Reference value 24hrs urine Na+ 78 mmol/24hrs 40-220 mmol/24hrs 24hrs urine K+ 127 mmol/24hrs 25-125 mmol/24hrs 24hrs urine Cl- 94 mmol/24hrs 110-250 mmol/24hrs 24hrs urine c alcium 352 mg/24hrs 100-300 mg/24hrs 24hrs urine volume 3100 ml/24hrs

30. Investigations (continued) ABG Report Blood Gas 19.09.22 26.09.22 pH 7.29 7.21 pCO2 12.3 mmHg 18.6 mmHg HCO3- 8.9 mmol /L 7.1 mmol/L Na+ 125 mmol /L 128 mmol/L K+ 1.78 mmol /L 1.75 mmol /L Cl- 102 mmol /L 105 mmol /L Anion gap 15.88 mmol /L 17.65 mmol /L

31. Investigations (continued) pH: Test Name​ 21/09/22 ​ 27/09/22 ​ Urine for pH​ 6.0​ 5.5​

32. Comment: Positive findings Modified Acid Load Test Before taking drugs Urine for pH 6.0 1 hour after taking drugs Urine for pH 6.0 2 hour after taking drugs Urine for pH 6.0 3 hour after taking drugs Urine for pH 5.5 4 hour after taking drugs Urine for pH 5.5 5 hour after taking drugs Urine for pH 5.5

33. Hyponatremia H ypokalemia H ypocalcemia H ypomagnesaemia Generalized weakness followed by sudden weakness of all 4 limbs V omiting Difficulty in swallowing Weight loss Metabolic acidosis Dilemma Normal anion gap

34. A t this point our final dx Distal Renal tubular acidosis

35. W hat is the cause of Distal RTA ?

36. Flashback On history, he tells us about difficulty in swallowing. But he denies dryness of eyes

37. Investigations (continued) ANA Screening – Negative ENA Profile – Anti-ds-DNA– Negative SmD1​ Positive​ Histone​ Negative​ U1-SM/RNP​ Negative​ SS-A/Ro​ Negative​ SS-B/La​ Negative​ Scl70​ Negative​ Jo-1​ Negative​

38. Schirmer tear test On R/E : <5mm wet On L/E : <5mm wet Comment : Severe Dry Eye

39. Schirmer test  Tear production was measured using the Schirmer test. A small piece of sterile filter paper, supplied in a standard kit, is placed in the lateral third of the lower eyelid. The extent of wetting in a given time is measured. Wetting of less than 5 mm in five minutes is considered abnormal.

40. Salivary gland biopsy Non keratinized squamous epithelium with infiltration of inflammatory cells

41. Final Diagnosis Considering lacking of some evidence & exclusion of other diseases our diagnosis is- Distal Renal tubular acidosis due to Primary Sjogren syndrome

42. Treatment: Tab. Mycophenolate Mofetil Tab. Spironolactone Tab. Sodium Bicarbonate Tab. Pilogen Tab. Magnesium Oxide Cap. Calcitriol Syrup. Potassium Chloride

43. He was advised to follow up 1 month later. After 1 month of Rx; his sicca symptoms disappeared, renal function improved & S. electrolytes corrected.

44. Ocular symptoms Dryness of eyes for more than 3 months   Presence of autoantibodies (anti-Ro/SSA and/or anti-La/SSB Oral symptoms Dryness of mouth Ocular signs P ositive schirmer tear test   Salivary gland histopathology demonstrating foci of lymphocytes   Tests indicating impaired salivary gland function Diagnosis of Sjogren’s syndrome according to AECG criteria

45. Patients who have symptoms and findings that satisfy four or more of the American-European Consensus Group (AECG) criteria probably have SS.

46. Our patient has Ocular signs Positive schirmer tear test   Oral symptoms Dryness of mouth Salivary gland histopathology demonstrating foci of inflammatory cells  

47. Exclusion criteria  Prior head and/or neck irradiation Infection with hepatitis C virus Acquired immunodeficiency syndrome (AIDS) Graft-versus-host disease Lymphoma Sarcoidosis Recent use of medications with anticholinergic properties

48. Take home massage Patient presenting with Generalized weakness & hypokalemia, we should think about Renal tubular acidosis. If diagnosis is Renal tubular acidosis, we should search for cause.

49. Thank You

50. Case Report 2 At the same time, a patient with similar complaints was admitted in our unit A 60-year-old male with a history of multiple hospitalizations for hypokalemic paralysis was brought to our unit with the complaints of weakness of all four limbs for 4 hours​. The patient reported that his symptoms were similar to his previous episodes.

51. Case Report 2 On query, he admitted to have infrequent, intermittent dry mouth and dry eyes with grittiness & itching for several months A review of history & medical records supplemented by investigations did not reveal any abnormality related to GIT disturbances, urinary symptoms, thyroid abnormalities , joint pain, rashes. There was no family history of autoimmune diseases.

52. Case Report 2 His vital signs were within normal limits and physical examination was unremarkable except mild anemia & dental caries Results of initial lab tests showed sodium 132 mmol /L, potassium 2.0 mmol /L , chloride 91 mmol /L & bicarbonate 17 mmol /L S. Creatinine was 1.9 Arterial blood gas pH was 7.21 & HCO3 was 12 .

53. Case Report 2 24 h our urine sodium was 75 mmol /L, urine potassium was 29.51 mmol /L, urine chloride was 68 mmol /L & urine calcium was 546 mg/24hrs. Urinalysis showed a urinary pH of 6.5 without blood or protein. Modified acid load test was positive. USG of W/A shows bilateral renal parenchymal disease with nephrolithiasis.

54. Case Report 2 With a normal anion gap of 18 and normal anion gap metabolic acidosis, he was diagnosed with distal RTA . His autoimmune panel was positive for ANA and anti-Ro/SSA antibody and negative for anti-double-stranded DNA ( dsDNA ), anti-Smith, anti-La/SSB, and anti-U1-ribonucleoprotein (RNP) antibodies . Schirmer tear test was positive.

55. Case Report 2 Based on sicca symptoms, positive schirmer tear test and positive anti-Ro/SSA antibodies, a diagnosis of Sjogren’s syndrome was made & Rx was given. H e was advised to follow up 1 month later After 1 month of Rx, his sicca symptoms disappeared, renal function improved & S. electrolytes corrected.