Case presentation

Dr. Rampratap Swami

2nd yr Senior Resident

Department of Gastroenterology

SNMC, Jodhpur

PATIENT INFORMATION

• Name -X

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• Age/sex – 62 yr/ Female

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• Address: Garasani, Jodhpur (Rural)

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• Date of Admission - 23/08/24

BACKGROUND

• Patient was admitted to our ward 25 days back with C/o jaundice and Abdominal distension, fatigue, generalised weakness & altered sensorium. No oliguria or GI bleed

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• On examination- jaundice, moderate ascites, mild pedal edema, flaps +

INVESTIGATIONS

• Hb – 10.4 g/dL
• TLC – 8560 mm3(N-72 %)
• PLT – 65000/mm3
• PT/INR- 24.2/1.65
• Urea/Cr – 34/0.86
• RBS – 165
• TB/DB –7.2/4.6
• SGOT/SGPT – 230/160
• ALP- 112
• TP/ALB /Globulin– 6.4/2.0/4.4
• Na⁺/K⁺ -125/3.7

URINE (R/M)

• Pus Cells- 0-1/HPF
• Epithelial cells- 1-2/HPF
• Albumin- nil
• Bacteria - nil
• URINE CULTURE-Negative

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• Ascitic fluid analysis-SAAG 1.3 ,Protein -2.3 ,TLC-120 (90 % PMNL),ADA-4.5,
• Ascitic fluid culture-Negative

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• Hbsag,Anti HCV-Negative
• S.Ceruloplasmin-23
• S.Ferritin-234
• ANA (by IFA)-1 :320 positive ,cytoplasmic pattern
• IgG total -2050(ULN-1600)
• Procalcitonin-<0.02

PREVIOUS INVESTIGATIONS

Further management

• No history s/o alcohol use, CAM intake or any metabolic disorder.
• Diagnosis of probable AIH was made in view of - Female gender, high ANA titre ,high globulin ,No Alcohol or CAM intake or metabolic disorders & Negative viral markers
• She was diagnosed as ACLF (APASL)
• AARC- 8 ,CTP-C 10 ,MELDNa-23
• Etiology- Probable AIH
• Discharged after 5 days on oral steroids, with short course (7 days) of oral cephalosporins, diuretics and beta blockers, advised for follow up after 7 days in OPD.
• She didn’t came for follow up because of clinical improvement as history given by relatives and was taking treatment.

Re admitted after 25 days on 23/08/24

• Yellowish discoloration of eyes since 4 days.

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• Abdominal distension since last 4 days.

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• Fever (undocumented) high grade since 4 days and cough with expectoration.

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• Shortness of breath since last 2 days.

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• Altered sensorium since last 2 days.

GENERAL AND SYSTEMIC EXAMINATION

• BP - 100/66 mmHg
• Pulse rate – 114/min, regular
• SpO₂ - 95% on Oxygen support via Face mask @ 4L/min
• Patient was icteric and tachypenic (RR- 30/min)
• Pedal edema (+).
• CNS – Patient was disoriented, drowsy, not arousable or responding to verbal commands, arousable only on Deep painful stimuli .Pupils were B/L RRR to light, B/L plantar were mute.

GENERAL AND SYSTEMIC EXAMINATION

• Per Abdomen - Distended, Shifting dullness present.
• Respiratory system - B/L air entry present, with bronchial breathing (Right side), coarse crepitations present through out the lung fields in right lung .
• Cardiovascular System - S1, S2(+), no added heart sounds/murmurs.

INITIAL WORKUP & MANAGEMENT

• In view of poor GC patient was shifted to ICU .
• Oxygen supplementation started @ 6L/min via face mask. Nebulisation done with Levosalbutamol and IPTB.
• Patient was started on empirical broad spectrum IV Antibiotics (Piperacillin-tazobactam) and anti-coma measures.
• All routine blood and urine investigations sent along with cultures and ABG.
• Ryle’s tube was inserted and Foleys catheterization done.
• CXR was done.
• Anti-coma measures started.

CXR (23/08/24)

INVESTIGATIONS

• 23/08/24
• Hb– 10.0 g/dL
• TLC – 13400 mm3 (N-87.2%)
• PLT – 40,000/mm3
• PT/INR- 24.2/1.79
• Urea/Cr – 43/0.67
• RBS –124
• TB/DB – 14.8/8.8
• SGOT/SGPT – 125/77
• ALP-156
• TP/ALB – 6.4/2.8
• Na⁺/K⁺ - 128/2.3

URINE (R/M)

• Pus Cells- 4-5/HPF
• Epithelial cells- 1-2/HPF
• Albumin- nil
• Bacteria - nil

• NTpro BNP –500
• Trop I – Negative
• PCT - 2.4 (positive)

• Sputum for gram stain and AFB-Few gram positive cocci ,AFB -Negative

Provisional diagnosis

• ACLF

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• CHRONIC LIVER DISEASE WITH PORTAL HTN WITH ASCITES

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• Hepatic encephalopathy grade III

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• Etiology-Probable AIH

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• Precipitant-Infection -Pneumonia (community acquired )

Serial ABG

TREATMENT & SEQUENCE OF EVENTS - 23/08/24

• Patient was managed with broad spectrum antibiotics (Piptaz), Albumin, anti-coma measures, supplemental oxygen, and LOLA infusion.

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• KCL infusion started for hypokalemia.

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• Urine output was around 300ml in last 12 hours.

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• Oxygen support was increased to 8L/min via face mask.

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• Advised for intubation and MV i/v/o poor GCS and respiratory status but consent not given by relatives.

TREATMENT & SEQUENCE OF EVENTS - 24/08/24

• Patients GCS further dropped and patient slipped into grade IV HE. SO₂ dropped to 88% on 10L/min O2 support. ABG done which shows pO₂ of 68.
• After taking consent for intubation, patient was intubated in the morning around 10 AM and put on mechanical ventilation on VC-AC mode @ PEEP of 8 and FiO₂ of 60%.
• Patient’s BP dropped to 88/56 and she was started on Nor adrenaline infusion @ 1.2 mg/hr.
• Peripheral IV access was lost so a central line was inserted through subclavian route and a repeat CXR was done which was same as previous X-ray

TREATMENT & SEQUENCE OF EVENTS - 24/08/24

• Same treatment was continued with broad spectrum antibiotics �(Piptaz and Clindamycin and Levofloxacin was added), Albumin, anti-coma measures, and LOLA infusion.
• Inj. SBC was added 30 ml TDS.
• Urine output was around 600ml in last 12 hours.
• Blood, urine and tracheal swab were sent for C/S.
• Patient was maintaining BP of 110/58 on nor adrenaline infusion @1.2 mg/hr

TREATMENT & SEQUENCE OF EVENTS - 24/08/24

• At around 5 PM, Patient’s BP fall to 84/46 and SpO₂ was 91% on FiO₂ 70%. ABG done and FiO₂ increased to 100%.
• Nor adrenaline infusion increased to 2.4 mg/hr.
• Inj. SBC 100 ml was given stat.
• ECG done which showed Sinus tachycardia with non specific ST-T changes.
• Trop I was sent which was negative.
• Patient BP stabilized at 96/58 on Nor adrenaline infusion @ 2.4 mg/hr.

TREATMENT & SEQUENCE OF EVENTS - 24/08/24

• At around 8 PM, patient went into cardiac arrest.
• CPR initiated as per ACLS protocol.
• After around 30 mins of CPR, there was no ROSC and ECG shows no electrical activity.
• Despite resuscitatory efforts patient couldn’t be revived and succumbed to her illness, and declared dead @ 8.40 PM on 24/08/24

FINAL DIAGNOSIS

• ACUTE ON CHRONIC LIVER FAILURE (PPT- PNEUMONIA)
• PNEUMONIA WITH SEPTIC SHOCK WITH TYPE 1 RF
• HEPATIC ENCEPHALOPATHY GRADE IV
• CHRONIC LIVER DISEASE WITH PORTAL HTN WITH ASCITES, (ETIOLOGY – probable AUTOIMMUNE) .
• AARC – 10
• CTP – C (14)
• MELD Na- 19
• CLIF-C – 65
• CAUSE OF DEATH – TYPE 1 RF WITH SEPTIC SHOCK (Blood and Urine culture-Negative)

Issues for discussion

• Whether one should start steroids or not in ACLF with probable AIH ?

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• How much is the risk of acquiring infection after steroids in ACLF ?

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• What are the predictive factors for successful treatment with steroids with low risk of acquiring infections ?

Conclusion: Higher scores CLIF-C ACLF, AARC and CTP, Pneumonia and presence of drug resistant bacteria are

indicators of poor prognosis in ACLF and thereby should be taken as an indication for more aggressive management.

Take Home Points

• Steroids should be started cautiously in ACLF due to AIH even with Low CTP and MELD scores

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• Factors predictive of acquiring infections in ACLF with AIH and who were given steroids are
• CTP >11
• MELD Na>24

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• Factors predicting non response to steroids
• Advanced age
• Presence of hepatic encephalopathy
• >_ F3 fibrosis

Re-ACLF an entity or an illusion ?- A Prospective observational study from tertiary care centre

• Total 212 ACLF (APASL) patients from June 23 to nov 2024
• Etiology-Majority were alcoholic(78 %) ,followed by Hep B(6 %) and MASLD(4%) and Autoimmune (3%) and Cryptogenic(3%)

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• 41 death
• 171 discharged
• We kept follow up of these patients
• Out of these 171 ACLF patients 26 patients devolved ReACLF and 8 died .

The analysis in 26 patients revealed diverse precipitating factors contributing to Re-ACLF episodes, predominantly continuation/ restart of alcohol consumption seen in 12 (46.15 %)  patients, infections (predominantly UTI, LRTI) in 8 (30.77%) , acute PVT in 2 (7.6 %), acute viral hepatitis in 1 (3.8 %),Lower gi bleed in 1 (3.8 %)and Hyponatremia in 1 (3.8 %)and cryptogenic in 1 (3.8%)patient.Mean hospital stay duration was longer in Re ACLF (11.6 days ) v/s 1 st episode of ACLF (8.6 days).Mean duration between two ACLF episodes were 96 days . Poor prognosis (8 out of 26 died in ReACLF episode and none were transplanted )were seen in Re-ACLF patients. AARC-ACLF score was higher (Mean 11.2 )from previous ACLF episode(mean 9.2) on admission.