dr. Aulia_Case Cholangio + Impending Thyroid storm.pptx

Case presentation

1. Aulia, dr. | Yessy Puspitasari , dr., Sp.PK, Subsp.G.E.H . (K) 1 CASE PRESENTATION Monday, June 24 th 2024

2. 2 Main complaint : yellowish in body History of illness :  The patient r eferred from Bhakti Husada Hospital with diagnosis: Obs. Deep icteric pro-evaluation + AF (8/5–9/5). Patient complained yellowish in his body since Eid this year, the complaint was accompanied by nausea and vomiting. Weakness since 1 week, worsening since 1 day before admittance, and feeling short of breath (+) intermittent. Complained of frequent chest palpitations for the past 14 years, accompanied with shortness of breath that improves with rest. The shortness of breath does not improve with changes in position, the patient sleeps with one pillow at night. The patient denied having chest pain. The patient also complained yellow eyes and facial area since approximately 1 month (before Eid) and worsening this week. The patient complained of drastic weight loss (50 kg  39 kg) and decreased appetite. Tea-colored urine since the past month. Black stools and putty-colored stools were denied by the patient, there were no complaints of swelling in the feet or abdominal enlargement. Mrs. Y, 44 y.o 13080794

3. 3 History Past lllness Diabetes mellitus, Hypertension, Hearth failure, and Coronary Artery were denied History of kidney, liver, and Cerebrovascular disease were denied History of Medication : --

4. 4 Physical Examinations Objective Weak, Alert, GCS 456 BP 112/71 mmHg HR 112 bpm RR 29 X/minutes Temp 37,3 °C O 2 Saturation 98%, O2 3 lpm Head / Neck A- / I + / C - / D - Meningeal sign (-) Chest: Retraction (-) Heart: S1-S2 single, murmur/gallop- Lung: Ves +/+, Rh -/- , Wh -/- Abdominal Soft, Bowel Sound (+) N , Hepatomegaly (-), Splenomegaly (-), Abdominal aorta pulsation (+) Extremity W arm +/+, E dema - / -, CRT <2 seconds

5. 5 Hematology ER (9/5) Ward (10/5) Ward (13/5) Ward (15/5) Ref. Range WBC (10 3 / uL ) 15.86 14.16 10.06 12.64 3,37-10 ,0 % Eo 0.3 0.1 0.0 0.0 0,6-5,4 % Ba 0.3 0.2 0.1 0.1 0,3-1,4 % Neu 77.0 79.7 87.8 87.0 39,8-70,5 % Ly m 11.1 8.1 4.7 5.5 23,1-49,9 % Mo 11.3 11.9 7.4 7.4 4,3-10,0 RBC (10 6 / uL ) 3.95 4.00 4.07 4.01 3,69-5,46 HGB (g/ dL ) 10.1 9.7 7.9 9.6 11-14,7 HCT (%) 28.3 29.4 31.1 28.7 41,3-52,1 MCV ( fL ) 71.6 73.5 76.4 71.6 86,7-102,3 MCH (pg) 25.6 24.3 19.4 23.9 27,1-32,4 MCHC (g/ dL ) 35.7 33.0 25.4 33.4 29,7-33,1 RDW (%) 23.7 21.3 19.8 19.6 12,2-14,8 PLT (10 3 / uL ) 148 87 122 105 150-450 PDW ( fL ) -- -- -- -- 9,6-15,2 MPV ( fL ) -- -- -- -- 9,2-12 P-LCR (%) -- -- -- -- 19,7-42,4 IG% 0.6 0.7 0.5 0.8 0-0,5 Laboratory Results

6. 6 Scattergram 09/05/24

7. 7  Clinical Chemistry ER (9/5) Ward (10/5) Ward (11/5) Ward (13/5) Ward (15/5) Reference Value K ( mmol /L) 4.10 5.00 -- 3.40 3.50 3,5 - 5,1 Na (mmol/L) 133 134 -- 140 135 136 - 145 Cl (mmol/L) 104.0 101.0 -- 104.0 93.0 98 - 107 BUN (mg/dL) 29.7 -- -- 31.9 21.0 7 - 18 Serum Creatinin (mg/dL) 0.8 -- -- 0.7 0.6 0,6 - 1,3 eGFR (mL/min/1,73m 2 ) 112 -- -- 117 122 Albumin (g/dL) 2.32 -- 2.15 2.52 2.48 3,4 - 5,0 AST (U/L) 116 -- -- -- 50 5 - 34 ALT (U/L) 61 -- -- -- 37 0 - 55 Direct Bilirubin (mg/dL) 22.20 -- -- 18.80 16.81 <0,2 Total Bilirubin (mg/dL) 31.50 -- -- 26.20 24.16 0,2 - 1 Kalsium -- 8.8 -- -- -- 8,5 – 10,5 Magnesium -- 1.90 -- -- -- 1,8 – 2,4 Fosfat (mg/dL) -- 4.75 -- -- -- 2,5 – 4,5

8. 8 Coagulation study ER (9/5) Reference Range PPT 18.7 9 – 12 S APTT 33.0 23 – 33 S

9. 9 URINALYSIS ER (9/5) Reference Range SG 1.011 1,003-1,030 pH 6.00 4,5-8 Colour Yellow Clearity Jernih Bilirubin 3+ Negative Urobilinogen Normal Normal Protein 1+ Negative Blood Negatif Negative Glucose Negatif Negative Ketone Negatif Negative Leucocyte Negatif Negative Nitrite Negatif Negative A:C Ratio (mg/ gCr ) 150 <30 P:C Ratio (g/ gCr ) >= 0.50 <0,15 Albumin 80 10-30 mg/L

10. 10 Immunology Ward (10/5) Ward (15/5) Referen c e Range HBsAg ( Indeks ) -- Non Reactive 0.57 <=0,99(NR) 1-50(Equivocal) >50(R) Anti HCV ( Indeks ) -- Non Reactive 0.18 <0,80 non reaktif =0,80 - < 1.00 Equivocal =1.00 reaktif ANA Test ( AU/mL) 16.90 -- < 40,0 : Negative, >= 40,0 : Positive Free T4 (FT4) ( ng/dL) 13.90 7.01 0.70-1.48 TSH Ultrasensitif ( µUI/mL) 0.005 0.037 2-12 Tahun : 0.64- 6.27 12-18 Tahun : 0.51-4.94 >18 Tahun : 0.55-4.78 Complemen C3 ( mg/dL) -- 21.00 82 - 185 Complemen C4 ( mg/dL) -- 2.9 15 - 53

11. 11 Pathogen Detection Result (11/05) Blood No aerob, anaerob bacterial and fungal growth Blood Culture

12. 12 Radiology Result Thorax AP : (9/5/2024) Conclusion : Cor and pulmo do not show any abnormalities ECG : (9/5/2024) Conclusion : Atrial fibrillation rhythm with rapid ventricular response 114x/minute, normal frontal axis, horizontal axis CWR (Clockwise Rotation)

13. 13 Radiology Result Echo TTE ( Transthoracal Echocardiography) (09/05/24): 1. Normal left ventricular dimensions ( LVIDd 4.76 cm) without LVH ( LVDMi 97.1 g/m2; RWT 0.366) 2. Normal left ventricular systolic function (EF by TEICH 58.6%) 3. Normokinetic left ventricular segmental analysis. Normal left ventricular diastolic function ( E'Sept 0.09 m/s; E'Lat 0.15 m/s; MV E Vel 1.14 m/s; E/A 1.89; E/E' AVG 8.75) 4. Normal right ventricular dimensions. Normal right ventricular systolic function (TAPSE 2 cm) 5. Normal left atrial dimensions 6. Normal right atrial dimensions 7. No thrombus or intracardiac vegetation was found 8. Heart valves : Mitral: MR trivial Aorta: mild AR (AR Sdec 1.76 m/s; AR PHT 560 msec) Tricuspid: Normal Pulmonal: Normal 9. No pleural or pericardial effusion was found 10.Dimensi Aorta: Annulus Aorta 1.75 cm | Sinus Valsava 2.93 cm | Sinotubular Junction 2.53 cm | Ascending Aorta 2.49 cm | Aorta Descendens 2.77 cm | Aorta Abdominalis : 1.74 cm

14. 14 Radiology Result USG Abdomen : (9/5/2024) Choledocolithiasis GB looks sludge Left pleural effusion Currently there are no abnormalities in the Liver / GB / Spleen / Pancreas / Right and left Kidney / Bladder / Uterus / Right and left Adnexa. CT Angiography Thoracoabdominalis : (10/5/2024) Celiac trunk, superior and inferior mesenteric arteries and external and internal iliac arteries on the right and left appear good Ascending aorta, aortic arch and descending aorta appear good No AVM ( arteri-venus malformation) or fistulation is seen No calcification is seen in the abdominal aorta No mass is seen in the thoracic cavity, pelvis and abdominal cavity

15. 15 Therapy O2 NK 3lpm Diet TKTP 2100kkal/24 hours low fat Inf. Amiodarone 1200 mg in D5 500cc : PZ 500cc 1:1 within 24 hours Trf . Albumin 20% 100cc in 4 hours Inj. Metoclopramid 3x1 PO UDCA 3x500mg Inj. Cefoperazone sulbactam 2x2 g IV Inj. Metronidazole 3x500mg IV PO Sucralfate syr 3xC1 PO Lansoprazole caps 2x30mg PO Amiodarone 2x20mg PO paracetamol 3x500 mg Raber with Cardio

16. 16 Resume Anamnesis: Female, 44 y.o , yellowish in body, eyes and facial area, fever, weakness, feeling palpitation, and shortness of breath Physical Examinations : HR ↑, RR ↑, Icteric +/+ Lab: CBC : Hb ↓ , HCT ↓, MCV ↓, MCH ↓, % Lym ↓, PLT ↓, WBC ↑, % Neu ↑, IG ↑ CC : BUN ↑, AST ↑, ALT ↑, Direct Bil ↑, Total Bil ↑ , Albumin ↓ Coagulation study : PPT ↑ Immunology: FT4 ↑, TSH ↓, C3 ↓, C4 ↓ Radiology: USG: Choledocolithiasis ECG: Atrial fibrillation rhythm with rapid ventricular response C holangitis with choledocholithiasis + Atrial fibrillation + Impending Thyroid Storm + Hyperbillirubinemia + Microcytic hypochromic anemia

17. 17 Problem List How to establish the diagnosis choledocholithiasis ? What is the cause of increment TBIL and DBIL? W hat is the pathophysiology of hyperthyroid? How to establish Impending Thyroid Storm? What is the cause decrement C3 & C4?

18. 18 THANK YOU Moderator Yessy Puspitasari , dr. , Sp. PK(K), Subsp.G.E.H . (K) PPDS Aulia Ashar, dr.

19. 19 Choledocholithiasis

20. 20 USG Abdomen : (9/5/2024) Choledocolithiasis

21. 21 T.Bill 31.5 / D.Bill 22.2 USG : Choledolithiasis

22. 22 Hepatocyte with cell organelles (schematic representation) and localization of the diagnostically most important enzymes etc 1. Stellate Kupffer cell 2. Space of Disse 3. Granular endopl . retic: ChE 4. Smooth endopl . retic 5. Mitochondrion: GlDH,ASAT 6. Bile canaliculi: ALP,LAP,G-GT 7. Nucleus 8. Lysosomes :hydrolases 9. Cytoplasm: LDH,ALAT,ASAT Iron Kuliah dr. Leonita , SpPK (K), 2017

23. 23 Newsome PN, et al. Gut 2018;67:6–19. doi:10.1136/gutjnl-2017-314924

24. 24 Newsome PN, et al. Gut 2018;67:6–19. doi:10.1136/gutjnl-2017-314924

25. 25 Ravi P, Thugu T, Singh J, et al. 2023. Gallstone Disease and Its Correlation With Thyroid Disorders: A Narrative Review. Cureus 15(9): e45116. DOI 10.7759/cureus.45116

26. 26 Hyperbilirubinemia

27. Kuliah Tes Fungsi Hati , dr Yessy T. Bill D. Bill 31.50 22.20 27

28. Hiperbillirubinemia (According to etiology) Kuliah Tes Fungsi Hati , dr Yessy 28

29. 29 https://doi.org/10.1515/almed-2021-0047

30. Kawathalkar , 2010 (Essentials of Clinical Pathology) Test Pre-hepatic Hepatic Post-hepatic Total bil + ++ +++ Conjugated bil Normal Increased Increased Unconjugated bil Increased Increased Normal AST/ALT Normal +++ + ALP/GGT Normal + +++ Urine Bilirubin Absent Present Present Urine urobilinogen Increased Variable Decreased Urine colour Normal Normal (but maybe dark depending on cause) Dark Stool colour Normal Normal Pale T. Bill D. Bill AST ALT Urobilinogen Urin Billirubin 31.50 22.20 116 61 Normal 3+ 30

31. Classification of Jaundice Pre-hepatic: pathology occuring prior to the liver Any cause of increased haemolysis (e.g. Spherocytosis , thalassaemia , sickle cell disease, transfusion reaction, auto-immune, malaria etc.) and some drugs Causes unconjugated hyperbilirubinaemia Intra-Hepatic: pathology occuring within the liver All the causes of hepatitis/cirrhosis (e.g. Alcohol, viral, auto-immune, primray biliary cirrhosis, haemochromatosis , wilsons , alpha-1 antitrypsin deficiency etc.), inherited condition on previous slide and some drugs Can result in hepatocyte destruction and therefore unconjugated hyperbilirubinaemia or in bile cannaliculi destruction and therefore conjugated hyperbilirubinaemia or both Post-hepatic: pathology occuring after conjugation of bilirubin within the liver (obstructive jaundice) Any cause of biliary obstruction (e.g. Gallstones) Causes conjugated hyperbilirubinaemia 31

32. 32

33. 33

34. 34

35. 35

36. 36

37. 37 Hyperthyroid

38. 38 Free T4 (FT4) ( ng/dL) 13.90 TSH Ultrasensitif ( µUI/mL) 0.005 European Thyroid. 2018. DOI: 10.1159/000490384

39. 39 Lancet Diabetes Endocrinol. 2023; 11: 282–98. https://doi.org/10.1016/S2213-8587(23)00005-0

40. 40 Slide Kuliah dr. Ferdy R. Marpaung , SpPK (K)

41. 41 Slide Kuliah dr. Ferdy R. Marpaung , SpPK (K)

42. 42

43. 43 European Thyroid Journal. 2018. DOI: 10.1159/000490384

44. 44 European Thyroid Journal. 2018. DOI: 10.1159/000490384

45. 45

46. 46   https://doi.org/10.3390/ijms24076835

47. 47 Impending Thyroid Storm

48. 48 Lancet Diabetes Endocrinol. 2023; 11: 282–98. https://doi.org/10.1016/S2213-8587(23)00005-0 Score : 35 Impending Storm Temp 37,3 T.Bill / D.Bill ↑ HR 112x/bpm

49. 49 Sarcognato S, et al . Autoimmune biliary diseases: primary biliary cholangitis and primary sclerosing cholangitis. Pathologica . 2021. https://doi. org/10.32074/1591-951X-245

50. 50 Sarcognato S, et al . Autoimmune biliary diseases: primary biliary cholangitis and primary sclerosing cholangitis. Pathologica . 2021. https://doi. org/10.32074/1591-951X-245 ALP : No Data GGT : No Data T. Bill : 31.5 AST : 116 ALT : 61

51. 51 Sarcognato S, et al . Autoimmune biliary diseases: primary biliary cholangitis and primary sclerosing cholangitis. Pathologica . 2021. https://doi. org/10.32074/1591-951X-245 ANA Test ( AU/mL) 16.90 < 40,0 : Negative, >= 40,0 : Positive

52. 52 Sarcognato S, et al . Autoimmune biliary diseases: primary biliary cholangitis and primary sclerosing cholangitis. Pathologica . 2021. https://doi. org/10.32074/1591-951X-245 ALP : No Data Anti-cardiolipin autoantibodie : No Data Anti-TPO autoantibodies : No Data ANA : 16.90 AST : 116 ALT : 61

53. 53 Ravi P, Thugu T, Singh J, et al. 2023. Gallstone Disease and Its Correlation With Thyroid Disorders: A Narrative Review. Cureus 15(9): e45116. DOI 10.7759/cureus.45116

54. 54 Ravi P, Thugu T, Singh J, et al. 2023. Gallstone Disease and Its Correlation With Thyroid Disorders: A Narrative Review. Cureus 15(9): e45116. DOI 10.7759/cureus.45116

55. 55 Low C3 & C4

56. 56

57. 57

58. 58 Prolong PPT & APTT

59. 59 PPT 18.7 APTT 33.0 Albumin (g/dL) 2.32 AST (U/L) 116 ALT (U/L) 61

60. 60 Mathieu Vinken and Vera Rogiers (eds.), Protocols in In Vitro Hepatocyte Research , Methods in Molecular Biology, vol. 1250, DOI 10.1007/978-1-4939-2074-7_23

61. 61 https://www.medmastery.com/guides/liver-lab-clinical-guide/prothrombin-time-and-what-it-reveals-about-liver-function

62. 62 Increment of BUN BUN (mg/dL) 29.7 31.9 21.0 Serum Creatinin (mg/dL) 0.8 0.7 0.6 Ratio > 20:1 > 20:1 > 20:1

63. 63 Atrial fibrillation

64. 64 Elevated BUN in HRS Blood urea nitrogen (BUN) is a measure of the amount of urea nitrogen in the blood. Urea is a waste product of protein metabolism that is normally excreted in the urine. In HRS, the kidneys' ability to filter and excrete urea is impaired, leading to an accumulation of urea in the blood and an increase in BUN levels. Increased Urea Production: In HRS, protein breakdown in the liver may increase due to factors like muscle wasting and catabolism. This increased urea production further contributes to the elevation of BUN levels. Enhanced Cellular Activity: Thyroid hormones bind to specific receptors in cells, initiating a complex signaling pathway that amplifies cellular activity. This increased cellular activity leads to a surge in metabolic processes, including energy production and protein breakdown. Enhanced Protein Degradation: Thyroid hormones augment protein breakdown, a process known as proteolysis. This increased proteolysis releases amino acids, which can be used for energy production or building new proteins.

65. 65 Increment AST & ALT

66. 66 Kuliah Tes Fungsi Hati , dr Yessy AST (U/L) ALT (U/L) 116 61

67. 67 Kuliah Tes Fungsi Hati , dr.Yessy

68. 68

69. 69 (Kuliah dr. Leonita Anniwati SpPK (K)) AST HATI (SITOSOL, MITOKONDRIA) OTOT JANTUNG OTOT RANGKA GINJAL OTAK PANKREAS PARU LEUKOSIT ERITROSIT KURANG SPESIFIK UNTUK MENILAI FUNGSI HATI DAPAT MENINGKAT PADA LATIHAN FISIK BERLEBIH INFARK MIOKARD Pincus MR, 2011

70. 70 (Kuliah dr. Leonita Anniwati SpPK (K)) – TERUTAMA TERDAPAT DI SITOSOL HEPATOSIT ENZIM SPESIFIK HATI PENINGKATAN ALT MENETAP LEBIH LAMA DIBANDINGKAN AST Pincus MR, 2011 A L T

71. 71 doi :  10.1177/2324709616651092

72. 72 Anemia Hematology ER (9/5) Ward (10/5) Ward (13/5) Ward (15/5) Ref. Range HGB (g/ dL ) 10.1 9.7 7.9 9.6 11-14,7 MCV ( fL ) 71.6 73.5 76.4 71.6 86,7-102,3 MCH (pg) 25.6 24.3 19.4 23.9 27,1-32,4 MCHC (g/ dL ) 35.7 33.0 25.4 33.4 29,7-33,1

73. 73

74. 74

75. 75

76. 76 Trombocytopenia Hematology ER (9/5) Ward (10/5) Ward (13/5) Ward (15/5) Ref. Range PLT (10 3 / uL ) 148 87 122 105 150-450 PDW ( fL ) -- -- -- -- 9,6-15,2 MPV ( fL ) -- -- -- -- 9,2-12 P-LCR (%) -- -- -- -- 19,7-42,4

77. 77

78. 78 Int Ophthalmol (2023) 43:3355–3362 https://doi.org/10.1007/s10792-023-02742-x

79. 79 Decrement of %Lymphocyte Hematology ER (9/5) Ward (10/5) Ward (13/5) Ward (15/5) Ref. Range % Neu 77.0 79.7 87.8 87.0 39,8-70,5 % Ly m 11.1 8.1 4.7 5.5 23,1-49,9

80. 80 Int Ophthalmol (2023) 43:3355–3362 https://doi.org/10.1007/s10792-023-02742-x

81. 81 https://doi.org/10.1016/j.jtauto.2022.100159

82. 82 https://doi.org/10.1016/j.jtauto.2022.100159 Hematology ER (9/5) Ward (10/5) Ward (13/5) Ward (15/5) Ref. Range % Neu 77.0 79.7 87.8 87.0 39,8-70,5 % Ly m 11.1 8.1 4.7 5.5 23,1-49,9 Ratio 7 9.8 18.6 18.8

83. 83 European Journal of Applied Physiology (2021) 121:1803–1814 https://doi.org/10.1007/s00421-021-04668-7

84. 84 Hypoalbuminemia

85. 1. Decreased production in liver (insufficient amino acids) -. Chronic severe malnutrition, chronic hepatic disease 2. Albumin as negative acute phase reactant → s timulate down regulation of albumin production. -. In response to trauma, Inflammation , neoplasm. 3. Increased loss of albumin ( Kidney problem , Severe hemorrhage, Severe exudative dermatopathy, Nephrotic syndrome) 4. Sequestration ( in body cavities caused by Peritonitis) 5. Increased albumin catabolism -. Chronic infection, neoplasm, trauma, inflammation Hypoalbuminemia 85

86. Albumin as negative acute phase reactant In inflammation condition, the body save amino acid more (the main structure to protein), so the inflammatory cytokines stimulate the down regulation of albumin production to save amino acid which can be used to produce positive acute protein plasma. Mirsaedi , 2018. doi.org /10.1016/j.ejim.2018.04.016 86

87. 87 Atrial Fibrilasi pada Hipertiroid

88. 88 Bekiaridou ,  et al.  The bidirectional relationship of thyroid disease and atrial fibrillation. 2022. https://doi.org/10.1007/s11154-022-09713-0