CASE PRESENTATION ONCIRRHOSIS OF LIVER WITH PORTAL HYPERTENSION, HEPATIC ENCEPHALOPATHY AND GRADE II OESOPHAGEAL VARICES WITH CONGESTIVE GASTROPATHY.

1. AKHIL JOSEPH REG.NO : 13QO4O2

2.  Cirrhosis is a slowly progressing disease in which healthy liver tissue is replaced with scar tissue, eventually preventing the liver from functioning properly. The scar tissue blocks the flow of blood through the liver and slows the processing of nutrients, hormones, drugs, and naturally produced toxins.

3.  As the disease worsens, a person may become tired, weak, itchy, have swelling in the lower legs, develop yellow skin, bruise easily, have fluid build up in the abdomen, or develop spider-like blood vessels on the skin. The fluid build-up in the abdomen may become spontaneously infected. Other complications include hepatic encephalopathy, bleeding from dilated veins in the esophagus or dilated stomach veins, and liver cancer. Hepatic encephalopathy results in confusion and possibly unconsciousness.

4. EPIDEMIOLOGY  Cirrhosis is the ninth leading cause of death in the United States and is responsible for 1.2% of all US deaths • Many patients die in their fifth or sixth decade of life • The prevalence is higher in non-Hispanic blacks and Mexican Americans, those living below the poverty level, and those with less than a 12th grade education • Each year, 2000 additional deaths are attributed to fulminant hepatic failure (FHF), that may be caused viral hepatitis, drugs (e.g., acetaminophen), toxins (e.g., Amanita phalloides, the yellow deathcap mushroom), autoimmune hepatitis, Wilson disease, or a variety of less common etiologies. Cryptogenic causes are responsible for one third of fulminant cases.

5. ETIOLOGY  It has many possible causes; sometimes more than one cause is present in the same person. Globally, 57% of cirrhosis is attributable to either hepatitis B (30%) or hepatitis C (27%).Alcohol consumption is another important cause, accounting for about 20% of the cases.  Alcoholic liver disease (ALD). Alcoholic cirrhosis develops for 10–20% of individuals who drink heavily for a decade or more.  Non-alcoholic steatohepatitis (NASH). In NASH, fat builds up in the liver and eventually causes scar tissue.  Chronic hepatitis C.  Chronic hepatitis B.  Primary biliary cirrhosis. Damage of the bile ducts leading to secondary liver damage.

6.  Primary sclerosing cholangitis. PSC is a progressive cholestatic disorder presenting with pruritus, steatorrhea, fat-soluble vitamin deficiencies, and metabolic bone disease.  Wilson's disease. Autosomal recessive disorder characterized by low serum ceruloplasmin and increased hepatic copper content on liver biopsy and elevated 24-hour urine copper.  Cystic fibrosis  Hepatotoxic drugs or toxins  Autoimmune hepatitis. This disease is caused by the immunologic damage to the liver causing inflammation and eventually scarring and cirrhosis.

7. PATHOPHYSIOLOGY

8. CLINICAL PRESENTATION SYMPTOMS  Gastrointestinal: bleeding, dark stool from digested blood, fluid in the abdomen, nausea, passing excessive amounts of gas, vomiting blood, or water retention  Whole body: fatigue, loss of appetite, or reduced hormone production  Skin: web of swollen blood vessels in the skin or yellow skin and eyes  Weight: weight gain or weight loss  Also common: swollen legs, yellow eyes, bleeding, breast enlargement, breast enlargement in men (Gynecomastia), bruising, dark urine, enlarged veins around belly button, flapping hand tremor, itching, mental confusion, Poor concentration and memory, muscle weakness, red palms, swelling, swelling in extremities, or swollen veins in the lower esophagus(Bleeding esophageal varices )

9. ASCITES WITH PORTAL HYPERTENSION

10. SIGNS  Jaundice  Scratch marks secondary to pruritus  Spider angiomata/naevi (mainly found on the trunk and face)  Skin telangiectasia's ('paper money skin')  Palmar erythema  Bruising  Petechia or purpura  Hair loss  White nails (sign of hypoalbuminemia)  Finger clubbing  Dupuytren's contracture

11. RISK FACTORS  Obesity/overweight increases the risk for liver disease. Obesity often results in the accumulation of fat cells in the liver. Acids that are secreted by these fat cells (called fatty acids) can cause a reaction in the body that destroys healthy liver cells and results in scarring (sclerosis) and liver damage.  The risk for developing liver disease varies, depending on the underlying cause and the particular condition. General risk factors for liver disease include alcoholism, exposure to industrial toxins, heredity (genetics), and long-term use of certain medications.  Age and gender also are risk factors for liver disease. These factors vary, depending on the particular type of disease. For example, women between the ages of 35 and 60 have the highest risk for primary biliary cirrhosis and men aged 30-40 are at higher risk for primary sclerosing cholangitis.

12. COMPLICATIONS  Anemia, thrombocytopenia and coagulopathy (folate deficiency, hemolysis, hypersplenism, cholestasis)  Esophageal varices (portal hypertension)  Ascites  Spontaneous bacterial peritonitis  Hepatocellular carcinoma  Cirrhotic cardiomyopathy  Hepatopulmonary syndrome  Portopulmonary hypertension.

13. DIAGNOSIS  The gold standard for diagnosis of cirrhosis is a liver biopsy, through a percutaneous, transjugular, laparoscopic, or fine-needle approach. A biopsy is not necessary if the clinical, laboratory, and radiologic data suggests cirrhosis. Furthermore, there is a small but significant risk to liver biopsy, and cirrhosis itself predisposes for complications caused by liver biopsy.The best predictors of cirrhosis are ascites, platelet count <160,000/mm3, spider angiomata, and Bonacini cirrhosis discriminant score greater than 7.

14. normal liver tissue

15. The liver in this histologic picture has wide bands of fibrous connective tissue separating small islands of hepatic tissue.

16. LAB FINDINGS Thrombocytopenia - typically multifactorial. Due to alcoholic marrow suppression, sepsis, lack of folate, sequestering in the spleen as well as decreased thrombopoietin. However, this rarely results in platelet count < 50 000/mL.  Aminotransferases - AST and ALT are moderately elevated, with AST > ALT. However, normal aminotransferases do not preclude cirrhosis.  Alkaline phosphatase - slightly elevated but less than 2-3 times the upper limit of normal.  Gamma-glutamyl transferase – correlates with AP levels. Typically much higher in chronic liver disease from alcohol.  Albumin - levels fall as the synthetic function of the liver declines with worsening cirrhosis since albumin is exclusively synthesized in the liver

17.  Bilirubin - Levels normal when compensated but may elevate as cirrhosis progresses.  Prothrombin time - increases since the liver synthesizes clotting factors.  Globulins - increased due to shunting of bacterial antigens away from the liver to lymphoid tissue.  Serum sodium - hyponatremia due to inability to excrete free water resulting from high levels of ADH and aldosterone.  Leukopenia and neutropenia - due to splenomegaly with splenic margination.  Coagulation defects - the liver produces most of the coagulation factors and thus coagulopathy correlates with worsening liver disease.

18. IMAGING  Ultrasound is routinely used in the evaluation of cirrhosis. It may show a small and nodular liver in advanced cirrhosis along with increased echogenicity with irregular appearing areas. Other findings suggestive of cirrhosis in imaging are an enlarged caudate lobe, widening of the liver fissures and enlargement of the spleen. An enlarged spleen (splenomegaly), which normally measures less than 11–12 cm in adults, is suggestive of cirrhosis with portal hypertension in the right clinical setting. Ultrasound may also screen for hepatocellular carcinoma, portal hypertension, and Budd-Chiari syndrome (by assessing flow in the hepatic vein).  Computed tomography (CT) scanning and/or magnetic resonance imaging (MRI)

19. ENDOSCOPY  Gastroscopy (endoscopic examination of the esophagus, stomach, and duodenum) is performed in patients with established cirrhosis to exclude the possibility of esophageal varices. If these are found, prophylactic local therapy may be applied (sclerotherapy or banding)

20. GRADING OF DISEASE  The severity of cirrhosis is commonly classified with the Child-Pugh score. This score uses bilirubin, albumin, INR, presence and severity of ascites, and encephalopathy to classify patients in class A, B, or C. Class A has a favourable prognosis, while class C is at high risk of death. It was devised in 1964 by Child and Turcotte and modified in 1973 by Pugh and others. Although it was originally used to predict mortality during surgery, it is now used to determine the prognosis, as well as the required strength of treatment and the necessity of liver transplantation.  Modified Maddrey's discriminant function.  The modified Maddrey's discriminant function) was originally described by Maddrey and Boitnott to predict prognosis in alcoholic hepatitis. It is calculated by a simple formula: (4.6 x (PT test - control))+ S.Bilirubin in mg/dl  Prospective studies have shown that it is useful in predicting short term prognosis especially mortality within 30 days. A value more than 32 implies poor outcome with one month mortality ranging between 35% to 45%. Corticosteroid therapy or pentoxifylline have been used with mixed results for patients whose increased mortality is indicated with a value greater than 32.

21. SCORING Points Class One year survival Two year survival 5–6 A 100% 85% 7–9 B 81% 57% 10–15 C 45% 35% Measure 1 point 2 points 3 points Total bilirubin, μmol/L (mg/dL) <34 (<2) 34–50 (2–3) >50 (>3) Serum albumin, g/dL >3.5 2.8–3.5 <2.8 Prothrombin time, prolongation (s) <4.0 4.0–6.0 > 6.0 Ascites None Mild (or suppressed with medication) Moderate to Severe (or refractory) Hepatic encephalopathy None Grade I–II Grade III–IV The score employs five clinical measures of liver disease. Each measure is scored 1–3, with 3 indicating most severe derangement. Chronic liver disease is classified into Child–Pugh class A to C, employing the added score from above. INTERPRETATION

22. Modified Maddrey's discriminant function  to predict prognosis in alcoholic hepatitis. It is calculated by a simple formula:  (4.6 x (PT test - control))+ S.Bilirubin in mg/dl.  Prospective studies have shown that it is useful in predicting short term prognosis especially mortality within 30 days. A value more than 32 implies poor outcome with one month mortality ranging between 35% to 45%.

23. DEMOGRAPHIC DETAILS OF PATIENT  NAME : XYZ  SEX : Male  DEPT : EICU  DOA : 15-01-2017  AGE : 56  I.P NO : 1624  UNIT :  DOD : 24-01-2017

24. REASON FOR ADMISSION:  C/O bleeding from gums after tooth extraction.  K/C/O alcoholic liver disease , admitted 6 months back.

25. HISTORY OF PRESENT ILLNESS:  Pt was apparently alright 2 days back the he had tooth extraction (3) following which he complains of bleeding from gums. He also had C/O abdominal distension and pedel edema.  Abdominal distension since 6 months.  K/C/O alcoholic liver disease, admitted for the same.  H/O pedal edema - pitting type, no h/o fever, no h/o pain in abdomen, no h/o vomiting of blood, no h/o passasge of dark coloured stools.

26. PAST MEDICAL HISTORY Admitted for ALD six months back. Not a K/C/O DM, HTN, TB, ASTHMA, EPILEPSY. No H/O previous surgeries.

27. Family history  Diet : veg  Sleep: disturbed  Appetite : normal and regular.  Habits : chronic alcoholic since 25 years, left 6 months back.

28. General physical examination.  Pt is moderatly built and nourished.  Not oriented to TPP. ON EXAMINATION  GC : fair  Afebrile  PR: 108 bpm  BP: 110/70 mmHg  SPO2 : 99% + + - - - +  P I C K L E

29. SYSTEMIC EXAMINATION  CVS : S1 S2 +, NO MURMUR  RS : B/L AE equal  CNS : pt. drowsy  P/A :palpation + no local rise in temperature non-tender hepatomegaly + inspection : distension +, umbilicus inverted.

30. INVESTIGATIONS  Complete hemogram with ESR  LFT  RBS  Blood urea  Serum electrolytes  BT  CT  PT  APTT  INR  Chest X-ray  USG abdomen and pelvis.

31. PROVISIONAL DIAGNOSIS LIVER CIRROSIS ?

32. LABORATORY FINDINGS HB : 6.9 g/dl ↓ ( 13.5- 17.5 g/dl )  WBC: 11,200 cells/µl. ↑  RBC: 2.07Million/µl. ↓  PLT: 1,12,000 cells/comm. ↓  DLC: polymorphs: 78% basophils: 00% eosinophils : 02% lymphocytes: 20% monocytes: 00% ESR : 120mm ↑ (<15mm) Complete hemogram  PCV : 17.2 % ( 40-50) ↓  MCH : 33.3 pg ( 27-32) ↑  MCHC : 40% ( 32- 46 )  MCV : 83.3 fl ( 82 - 101)

33.  ELECTROLYTES : Sodium : 138 (135-147 mEq / L ) Potassium :3.8( 3.5-5.2 mEq / L ) Chlorides : 105 ( 95-107 mEq / L ) RBS: 106 mg/dl (60-140 mg/dl ). HIV : Non-Reactive HBsAg : NEGATIVE HCV : NEGATIVE BT : 3 mnts. 30 sec (1-6 min ) CT : 5min. 30sec ( 5-10 min ) PT: test : 20 sec , control : 13sec ↑ INR : 1.5 (0.8-1.1) ↑ APTT : test : 40sec, control : 22sec. ↑

34.  PSS : normocytic normochromic anemia with neutrophilia.  Blood group : O+ve RFT  Sr. Urea : 32  Creatinine : 0.7

35. LFT  ALT : 24 U/L (6-38)  AST : 71 U/L ( 6-40) ↑  ALP : 63 U/L ( 35-140)  BILIRUBIN T : 7.3 MG% ( 0.2 – 1.0)↑ D : 2.8 MG% ( 0.1 - 0.4 ) ↑ I : 4.5 MG % ( 0.1 – 0.6)↑ Total protien : 6.7 ( 6.4-8.3 gm/dl) Albumin : 2.9 ( 3.5 – 5 gm/dl ) ↓ Globulin : 3.8 ( 2.3 – 3.5 gm/dl) ↑ A/G ratio : 1:1.3 Ammonia : 68mcg/dl ( 9.5-49) ↑

36. Ascitic fluid examination  Protien : 1.5 gm %  Chloride : 120mEq/L  Sugar : 173mg%  Gram stain : Few lymphocytes seen, no organisms.  ZN stain : No AFB

37. URINE ROUTINE  Pus cells : 1-2/ hpf  Epithelial cells : 1-2/hpf

38. USG ABDOMEN  1. CIRRHOSIS OF LIVER WITH PORTAL HYPERTENSION AS EVIDENCED BY SPLEENOMEGALYAND GROSS TENSE ASCITES.  2. CHOLELITHIASIS

39. TREATMENT CHART

40. BRAND NAME GENERIC NAME DOSE ROUTE FREQUENCY D A Y 1 D A Y 2 D A Y 3 D A Y 4 D A Y 5 D A Y 6 D A Y 7 D A Y 8 D A Y 9 D A Y 10 IVF NS DNS WITH 2 AMP OPTINEURON 2 PINT 1 PINT IV 1-0-0-1 √ √ √ √ √ √ √ √ √ √ √ √ √ INJ. XONE CEFTRIAXONE 1 GM IV 1-0-1 √ √ √ √ √ TAB OFLOX OFLOXACIN 400MG P/O 1-0-1 √ √ √ √ √ INJ. PAN PANTOPRAZOL E 40 MG IV 1-0-0 √ √ √ √ √ √ √ √ √ √ INJ. VITAMIN K VITAMIN K IM 1-0-1 √ √ √ √ √ √ INJ. HEPAMERZ L-ORNITHINE L-ASPARTATE I AMP IN 100 MLNS 1-1-1 2-2-2 (SACHET) √ √ √ √ √ 1- 1- 1 √ √ √ √ INJ. EMSET ONDANSTRON 4 MG IV 1-1-1 √ √ √ TAB. UDIBON URSODEOXYCH OLIC ACID SILYMARIN 300MG 140MG P/O 1-0-1 √ √ √ √ √ √ √ √ √ √ INJ. PAUSE TRANEXAMIC ACID I AMP IN 100 ML NS 1-0-1 √ √ √ √ √ T A B √ SYP.DUPHAL AC LACTULOSE 15ML P/O 1-1-1 √ √ √ √ √ √ √ √ √ √

41. BRAND NAME GENERIC NAME DOSE ROUTE FREQUENCY D A Y 1 D A Y 2 D A Y 3 D A Y 4 D A Y 5 D A Y 6 D A Y 7 D A Y 8 D A Y 9 D A Y 10 TAB. DYTOR PLUS TORASEMIDE SPIRONOLACTO NE 10 MG 50 MG P/O 1-0-0 √ √ √ √ √ INJ. 25% DEXTROSE DEXTROSE IV 1-1-1 √ √ √ √ TAB. RIFAGUT RIFAXIMIN 400MG P/O 1-1-1 √ √ √ √ √ √ √ √ √ INJ. OCTREOTID E OCTREOTIDE ACETATE 5 AMP IN 1OO ML NS IV @ 30ML / HR √ √ INJ.METRO GYL METRONIDAZO LE 100ML IV 1-1-1 √ √ √ √ T H. ALBUMIN HUMAN ALBUMIN 100 ML IV 1-0-0 √ SYP. POTKLOR KCL 2TSP P/O 2 TSP WITH WATER √ √ √ √ √ √ TAB. OROFER XT TOTAL ZN, FOLIC ACID,ELEMENT AL IRON, METHYLCOBAL AMIN 22.5MG 1.1MG 100MG1 .55MG P/O 0-1-0 √ √ √ Β- PROTIEN POWDER PROTIENS 2 TSP WITH MILK P/O 1-1-1 √

42. FOLLOW UP  DAY 1  PR : 120bpm  BP: 130/70 mmhg O/E  afebrile S/E  Pt is conscious and oriented.  CVS : S1S2 +  RS : B/L AEE  CNS : irritable and drowsy  P/A : hepatomegaly  1 PINT PCV TRANSFUSED

43. Abdominal Girth : 94cm Oral suctioning – hourly RT FEED : 100ML 1-1-1-1

44. ADVICE  INJ. METROGYL 100ML IV 1-1-1  INJ. XONE 1GM IV 1-0-1  INJ. PAN 40 MG IV 1-0-0  INJ. VITAMIN K 1 AMP IN 100ML NS IV 1-0-1  INJ. HEPAMERZ 2 AMP IN 100ML NS IV 1-1-1  INJ. EMSET 4MG IV 1-1-1  TAB. UDIBON 1-0-1  INJ. Pause 1 AMP IN 100ML NS 1-0-1  SYP. DUPHALAC 2 TSP √-√-√  INJ. 25% DEXTROSE 1-1-1  DUPHALAC BOWEL WASH 1-1-1-1-1-1  INJ. 1 PINT DNS WITH 2 AMP OPTINEURON 1-0-0-1  TAB. RFAGUT 400MG 1-1-1  INJ. OCTREOTIDE 5AMP IN 1 PINT NS @ 30ML/ HR

45. DAY 2 Pt on Mechanical Ventilation BP: 120/80 mmhg PR: 92 bpm Systemic examination CNS : conscious and oriented, B/L PERL + CVS : s1s2 + P/A : soft, distended. Spo2 : 98%

46. Investigations (17-01-2017)  Hb : 7.2  PT: T : 23.8 sec C: 13.5 sec INR: 1.86 APTT T: 28 sec C: 22 sec Creatinine : 1.0  Treatment As Per Chart.

47. Day 3  No fresh complaints.  PR: 72 bpm  BP: 110/80 mmhg  SPO2: 98%  CNS: conscious and oriented. Flaps +  RS: B/L AEE  CVS: s1s2 +  P/A: soft, distented. Free fluid +  Abdominal Girth : 91cm

48. Investigations HB : 6.6 g/dl ↓ ( 13.5- 17.5 g/dl )  WBC: 4800 cells/µl.  RBC: 2.04Million/µl. ↓  PLT: 73,000 cells/comm. ↓  DLC: polymorphs: 72% basophils: 00% eosinophils : 03% lymphocytes: 25% monocytes: 00%  ELECTROLYTES : Sodium : 144 (135-147 mEq / L ) Potassium :2.9 ( 3.5-5.2 mEq / L ) Treatment  As per chart

49. Day 4 No fresh complaints PR: 88bpm BP: 110/80 mmhg Spo2 : 96% CNS : conscious and oriented RS : B/L AEE CVS: s1 s2 + P/A: soft, distended.

50. Investigations  Potassium : 2.9 mEq/L CHILD PUGH SCORE  Score : 11  CLASS – C  1 YR MORTALITY – 55% , Two year survival – 35% Modified Maddrey's discriminant function – 54.7 TREATMENT  1 pint PCV transfused.  As per chart.

51. Day 5  Patient shifted to wards  No fresh complaints  PR: 86 bpm  BP: 114/76 mmhg  CNS: conscious and oriented RS: B/L AEE CVS: S1S2 + P/A: soft, distented.  Abdominal Girth : 90cm

52. Investigations  Potassium : 3.1 mEq/L TREATMENT  1 pint PCV transfused  As per chart.

53. Day 6  No fresh complaints  PR: 82 bpm  BP: 108/70 mmhg CNS: conscious and oriented RS: B/L AEE CVS: S1S2 + P/A: soft, distented, non-tender.

54. Investigations HB : 7.9 g/dl ↓ ( 13.5- 17.5 g/dl )  WBC: 5,100 cells/µl. ↓  RBC: 2.47Million/µl. ↓  PLT: 74,000 cells/comm. ↓  DLC: polymorphs: 60% basophils: 00% eosinophils : 04% lymphocytes: 34% monocytes: 02% RFT  Sr. Urea : 24  Creatinine : 1.0 TREATMENT  As per chart.

55. Day 7  No fresh complaints  PR: 88 bpm  BP: 110/70 mmhg CNS: conscious and oriented RS: B/L AEE CVS: S1S2 + P/A: soft, distented, non-tender. Abdominal Girth : 90cm

56. Investigations  ELECTROLYTES : Sodium : 136 (135-147 mEq / L ) Potassium :4.1( 3.5-5.2 mEq / L ) Chlorides : 101 ( 95-107 mEq / L ) LFT  ALT : 22 U/L (6-38)  AST : 57 U/L ( 6-40)  ALP : 49 U/L ( 35-140)  BILIRUBIN T : 4.6 MG% ( 0.2 – 1.0) D : 1.4 MG% ( 0.1 - 0.4 ) I : 3.2 MG % ( 0.1 – 0.6) Total protien : 5.9 Albumin : 2.0 Globulin : 3.9 A/G ratio : 1:1.9

57.  TREATMENT  1 pint PCV transfused  As per chart.

59. Treatment Advice  1 pint PCV transfused.  As per chart.

61. Investigations HB : 8.3 g/dl ( 13.5- 17.5 g/dl )  WBC: 5,300cells/µl.  RBC: 2.54Million/µl.  PLT: 1,50,000 cells/comm.  DLC: polymorphs: 70% basophils: 00% eosinophils : 02% lymphocytes: 208 monocytes: 00% ESR : 42 mm (<15mm) Complete hemogram  PCV : 23.3 % ( 40-50)  MCH : 32.5 pg ( 27-32) ↑  MCHC : 35.4% ( 32- 46 )  MCV : 92 fl ( 82 - 101)

62. PT test : 14 sec , control : 13sec INR : 1.0 (0.8-1.1) APTT test : 28sec, control : 22sec. Peripheral Smear Study Impression : NORMOCYTIC HYPOCHROMIC ANEMIA

63.  UPPER GI ENDOSCOPY Impression : GRADE II OESOPHAGEAL VARICES WITH CONGESTIVE GASTROPATHY. Patient attendees are not willing for surgical intervention / banding.  TREATMENT AS PER CHART.

64. ESOPHAGEAL VARICES CONGESTIVE GASTROPATHY

65. Final Diagnosis  CIRRHOSIS OF LIVER WITH PORTAL HYPERTENSION, HEPATIC ENCEPHALOPATHY AND GRADE II OESOPHAGEAL VARICES WITH CONGESTIVE GASTROPATHY.

66. DISCHARGE MEDICATION BRAND NAME GENERIC NAME DOSE ROUTE FREQUENC Y DURATION TAB. PAN PANTOPRAZOLE 40MG P/O 1-0-0 15 DAYS TAB. UDIBON URSEODEOXYCHOLIC ACID SILYMARIN 300MG 140MG P/O 1-0-1 15 DAYS TAB. RIFAGUT RIFAXIMIN 400MG P/O 1-1-1 15 DAYS HEPAMERZ SACHET WITH WATER L-ORNITHINE L-ASPARTATE 3 GM P/O 1-1-1 15 DAYS TAB. DYTOR PLUS TORASEMIDE SPIRONOLACTONE 10 MG 50MG P/O 1-1-0 15 DAYS SYP. DUPHALAC LACTULOSE 10ML P/O 1-1-1 15 DAYS B-PROTIEN POWDER WITH MILK PROTIEN 2 TSP P/O 1-1-1 15 DAYS TAB. OROFER XT TOTAL ZN, FOLIC ACID, ELEMENTAL IRON, METHYLCOBALAMIN 22.5MG 1.1MG 100MG 1.55MG P/O 0-1-0 15 DAYS

67. REVIEW  Review in medical OPD on WEDNESDAY after 15 days or incase of any emergencies.

68. PHARMACEUTICAL CARE PLAN

69. SUBJECTIVE EVIDENCES  C/O bleeding from gum after tooth extraction.  K/C/O alcoholic liver disease , admitted 6 months back.  Abdominal distension since 6 months.  B/L pedel edema – pitting type.

70. OBJECTIVE EVIDENCES HB : 6.9 g/dl ↓ ( 13.5- 17.5 g/dl )  WBC: 11,200 cells/µl. ↑  RBC: 2.07Million/µl. ↓  PLT: 1,12,000 cells/comm. ↓  ESR : 120mm ↑ (<15mm) PT: test : 20 sec , control : 13sec ↑ INR : 1.5 (0.8-1.1) ↑ APTT : test : 40sec, control : 22sec. ↑ PSS : normocytic normochromic anemia with neutrophilia.

71. LFT  ALT : 24 U/L (6-38)  AST : 71 U/L ( 6-40) ↑  ALP : 63 U/L ( 35-140)  BILIRUBIN T : 7.3 MG% ( 0.2 – 1.0)↑ D : 2.8 MG% ( 0.1 - 0.4 ) ↑ I : 4.5 MG % ( 0.1 – 0.6)↑ USG ABDOMEN  1. CIRRHOSIS OF LIVER WITH PORTAL HYPERTENSION AS EVIDENCED BY SPLEENOMEGALY AND GROSS TENSE ASCITES.  2. CHOLELITHIASIS UPPER GI ENDOSCOPY Impression : GRADE II OESOPHAGEAL VARICES WITH CONGESTIVE GASTROPATHY.

72. ASSESSMENT  BY OBSERVING THE SUBJECTIVE AND OBJECTIVE EVIDENCES THE PATIENT WAS DIAGNOSED AS CIRRHOSIS OF LIVER WITH PORTAL HYPERTENSION, HEPATIC ENCEPHALOPATHY AND GRADE II OESOPHAGEAL VARICES WITH CONGESTIVE GASTROPATHY.

73. PLANNING GOALS TO BE ACHIEVED  To improve patients quality of life.  To prevent further progress of complications.  To reduce the gum bleeding.  To normalise the blood counts.  To reduce the ESR level.  To reduce the abdominal distension.  To reduce mortality rate.

74. GOALS ACHIEVED  Patients quality of life improved.  Minimised the further progress of complications.  Gum bleeding stopped.  Blood counts and levels are improving.  ESR level is decreased.  Abdominal distension reduced.

75. MONITORING PARAMETERS  CBC with complete hemogram.  ESR  LFT  Prothrombin time and INR  Ascites, abdominal distension, bleeding.  Sign and symptoms.

76. PHARMACIST INTERVENTION  Major drug-drug interactions.  POTASSIUM CHLORIDE – SPIRONOLACTONE Concurrent use of potassium and spironolactone may results in hyperkalemia.  METRONIDAZOLE-ONDANSETRON,  OCTREORIDE ACETATE- OFLOXACIN  ONDANSETRON- OCTREORIDE ACETATE  OFLOXACIN - ONDANSETRON  METRONIDAZOLE – OFLOXACIN  METRONIDAZOLE - OCTREORIDE ACETATE, all these maycauses QT interval prolongation.

77.  Moderate drug-drug interactions  ceftriaxone torsemide Cephalosporin antibiotics like cefTRIAXone can occasionally cause kidney problems, and using it with torsemide may increase that risk. The interaction is more likely to occur when the cephalosporin is given at high dosages by injection into the vein or when it is given to the elderly or individuals with preexisting kidney function impairment.  ofloxacin lactulose Ofloxacin can cause an irregular heart rhythm that may be serious and potentially lifethreatening, although it is a relatively rare side effect. The risk is increased if you have low blood levels of magnesium or potassium, which can occur with bowel cleansing preparations or excessive use of medications that have a laxative effect.

78.  Torsemide - Pantoprazole Using pantoprazole together with torsemide may cause a condition called hypomagnesemia, or low blood magnesium. Drugs known as proton pump inhibitors including pantoprazole can cause hypomagnesemia when used for a prolonged period. Banding has to be done to prevent the rupture of esophageal varices, rupture and bleeding further lead to more complications and increased mortality rate.

79. PATIENT COUNSELLING ABOUT DISEASE ;  Cirrhosis is a slowly progressing disease in which healthy liver tissue is replaced with scar tissue, eventually preventing the liver from functioning properly. The scar tissue blocks the flow of blood through the liver and slows the processing of nutrients, hormones, drugs, and naturally produced toxins.  Portal hypertension is an increase in the blood pressure within a system of veins called the portal venous system. Veins coming from the stomach, intestine, spleen, and pancreas merge into the portal vein, which then branches into smaller vessels and travels through the liver.

80.  Hepatic encephalopathy (HE) is the occurrence of confusion, altered level of consciousness, and coma as a result of liver failure. In the advanced stages it is called hepatic coma. It may ultimately lead to death. It is caused by accumulation in the bloodstream of toxic substances that are normally removed by the liver.  Esophageal varices are extremely dilated sub- mucosal veins in the lower third of the esophagus. They are most often a consequence of portal hypertension, commonly due to cirrhosis; patients with esophageal varices have a strong tendency to develop bleeding.

81.  Portal hypertensive gastropathy ( congestive gastropathy ) refers to changes in the mucosa of the stomach in patients with portal hypertension ; by far the most common cause of this is cirrhosis of the liver. These changes in the mucosa include friability of the mucosa and the presence of ectatic blood vessels at the surface.

82. ABOUT MEDICATION  Take medications on time.  Don’t skip the medication.  Pantoprazole can be taken with or without food.  Rifaximin can be taken with or without food.  Udibon has to be taken with food.  Hepamerz sachet dissolved in a glass of water and taken after the meals.  Lactulose can be taken either with or without food. However, if you find it makes you feel sick this can be reduced by taking it with water, fruit juice or meals. You can take lactulose undiluted, or you can mix the required dose of lactulose into a drink of water or fruit juice.  Swallow each dose straight away. Don't keep it in your mouth as the sugar content may lead to tooth decay

83. LIFE STYLE MODIFICATIONS  Lifestyle changes cannot cure cirrhosis, but they can help to delay or stop progression of the disease, reduce the severity of symptoms, and help prevent complications.  Avoid drinking alcohol.  Eat a balanced diet.  Avoid raw seafood.  Discuss the appropriate amount of protein you need to eat, an adult who weighs 68 kilograms needs at least 54 grams of protein each day  Depending on your condition, you may either need to increase or decrease protein intake.  Take any vitamin or mineral supplements recommended by your doctor.  A low-salt diet may be needed to reduce fluid retention.  Get your doctor’s approval for all medicines. For example, acetaminophen (such as Tylenol) can speed up liver damage. Aspirin and other nonsteroidal anti-inflammatory drugs (NSAIDs)-for example, ibuprofen and naproxen -increase the risk of variceal bleeding if you have enlarged veins (varices) in the digestive tract.

84.  Get vaccines for flu, pneumonia, and hepatitis.  Put your feet up to reduce swelling.  Be careful with herbs and dietary supplements. Herbal remedies, herbal combinations and dietary supplements aren’t subject to the same approval process as medication and some may harm the liver. A few that have caused liver problems are cascara, chaparral, comfrey, kava and ephedra.  Stop smoking. Quitting tobacco use will improve your overall health, which may help make you a better candidate for a liver transplant if you need one.  Increased caffeine consumption is most beneficial for people who are at high risk for developing chronic liver disease, It's important to note if you already have cirrhosis, coffee isn’t powerful enough to reverse damage

85. STANDARD TREATMENT Complication Treatment Approach Monitoring Parameter Outcome Assessment Ascites Diet, diuretics, paracentesis, Daily assessment of weight Prevent or eliminate ascites and its secondary complications Variceal bleeding Pharmacologic prophylaxis Endoscopy, vasoactive drug therapy (octreotide), ligation or sclerotherapy, volume resuscitation, pharmacologic prophylaxis Child-Pugh score, endoscop, CBC, evidence of overt bleedingy, CBC Acute: control acute bleed Chronic: variceal obliteration, reduce portal pressures Hepatic encephalopathy Ammonia reduction (lactulose, cathartics), elimination of drugs causing CNS depression, limit excess protein in diet Grade of encephalopathy, EEG, psychological testing, mental status changes, concurrent drug therapy Maintain functional capacity, prevent hospitalization for encephalopathy, decrease ammonia levels, provide adequate nutrition

86. LIVER TRANSPLANTATION  The complications seen in patients with chronic liver disease are essentially functional as a secondary effect of the circulatory and metabolic changes that accompany liver failure. Consequently, liver transplantation is the only treatment that can offer a cure for complications of end-stage cirrhosis. However, patient selection, evaluation, and pre- and postsurgical management are beyond the scope of this review. The surgical procedure is very demanding and ranges from 4 to 18 hours depending on outcome. Numerous anastomoses and sutures, and many disconnections and reconnections of abdominal and liver tissue, must be made for the transplant to succeed, requiring an eligible recipient and a well-calibrated live or cadaveric donor match. The large majority of liver transplants use the entire liver from a non-living donor for the transplant, particularly for adult recipients. A major advance in pediatric liver transplantation was the development of reduced size liver transplantation, in which a portion of an adult liver is used for an infant or small child

87. Liver donor requirements  Any member of the family, parent, sibling, child, spouse or a volunteer can donate their liver. The criteria for a liver donation include:  Being in good health  Having a blood type that matches or is compatible with the recipient's, although some centres now perform blood group incompatible transplants with special immuno suppression protocols  Having a charitable desire of donation without financial motivation  Being between 18 and 60 years old  Being of similar or bigger size than the recipient  Before one becomes a living donor, the donor must undergo testing to ensure that the individual is physically fit. Sometimes CT scans or MRIs are done to image the liver. In most cases, the work up is done in 2–3 weeks.

88. Band Ligation / Banding  Endoscopy band ligation is used to treat enlarged veins in the esophagus, the tube connecting the throat to the stomach. If left untreated esophageal veins (varices) can spontaneously rupture and cause severe bleeding.  The procedure is performed during an upper gastrointestinal endoscopy. A local anesthetic is given to numb the throat and sedation medication will also be given through IV to help you relax/sleep through the procedure.  A scope is placed in the mouth down to the esophagus. When varices are found, tiny elastic bands are placed around the enlarged veins in the esophagus to tie them off so they can’t bleed. The banded varices are then eventually sloughed after a few days and the esophagus is much less likely to bleed after it’s healed.

CASE PRESENTATION ON CIRRHOSIS OF LIVER WITH PORTAL HYPERTENSION, HEPATIC ENCEPHALOPATHY AND GRADE II OESOPHAGEAL VARICES WITH CONGESTIVE GASTROPATHY.

Akhil Joseph