1. Case Based Discussion LAP TOP 23 rd August 2015 LAP TOP 23 rd August 2015

2. Case- 1 4 year old girl child Presented with yellowish discoloration of eyes and urine x 4 days H/o Prodrome of fever and vomiting No H/o pedal edema, clay colored stool, itching, altered sensorium and bleeding Developmentally normal and Hepatitis B Vaccine given; Hepatitis A not given 4 year old girl child Presented with yellowish discoloration of eyes and urine x 4 days H/o Prodrome of fever and vomiting No H/o pedal edema, clay colored stool, itching, altered sensorium and bleeding Developmentally normal and Hepatitis B Vaccine given; Hepatitis A not given Dr Pradeep Kumar Sharma Dr Sanjay Sehta, Dr Utkarsh Dr Pradeep Kumar Sharma Dr Sanjay Sehta, Dr Utkarsh

3. Examination Liver 5cm BCM, Span 11cm; Slight Tender; round Border ; smooth surface Spleen : Not palpable Shifting Dullness + Bowel Sounds : Normal Rest System :WNL Liver 5cm BCM, Span 11cm; Slight Tender; round Border ; smooth surface Spleen : Not palpable Shifting Dullness + Bowel Sounds : Normal Rest System :WNL

4. Clinical Impression 4 year old child with prodromal symptoms and Jaundice ; Hepatomegaly with ascities S/o Hepatitis of Infective Etiology Acute Viral Hepatitis Malaria Dengue Malaria Dengue Enteric Fever

5. USG Abdomen: Hepatomegaly with Hypoechoic Liver; Ascites ; and Minimal Rt Pleural Effusion USG Abdomen: Hepatomegaly with Hypoechoic Liver; Ascites ; and Minimal Rt Pleural Effusion 760/1230

6. Investigation Contd. PS for MP Negative Dengue /NS-1 Negative Typhidot Negative Leptospira Negative HbsAg, Anti HCV and Anti HEV Negative Ig M Anti HAV Positive LKM /SMA/ANA Negative Ceruloplasmin 35 mg/dl PS for MP Negative Dengue /NS-1 Negative Typhidot Negative Leptospira Negative HbsAg, Anti HCV and Anti HEV Negative Ig M Anti HAV Positive LKM /SMA/ANA Negative Ceruloplasmin 35 mg/dl All Viral Markers required ? PT/INR must in all cases of AVH ? Ascites and Pleural Effusion in AVH ?

7. Final Diagnosis Acute Viral Hepatitis (HAV related) with ascites and pleural effusion and anemia 1234512345 1234512345 Dietary Advice ? When to admit patient with Acute Viral Hepatitis ? IV Fluids ? Any Specific Medications ? Vitamin Supplements ? Liver Tonics ? Serial Monitoring of Liver Functions- When and What ? MANAGEMENTMANAGEMENT

8. Answers by experts High Enzymes favor AVH Ascites in 13% cases of AVH Normal Diet, no restrictions Admit if f/o Hepatic encephalopathy, Pt prolonged and Liver size decreased No specific medicines except UDCA in prurities PT/INR and Serological markers (HBsAg, Ig M Anti HAV, IgM Anti HEV) No serial monitoring required, LFT after 2-3 months to see for normalization High Enzymes favor AVH Ascites in 13% cases of AVH Normal Diet, no restrictions Admit if f/o Hepatic encephalopathy, Pt prolonged and Liver size decreased No specific medicines except UDCA in prurities PT/INR and Serological markers (HBsAg, Ig M Anti HAV, IgM Anti HEV) No serial monitoring required, LFT after 2-3 months to see for normalization

9. Case- 2 45 day boy, Normal Delivery; BW 3.5kg D0 D4 D10 D20 D30 D45 Ante Natal Uneventful Breast Feeds TB 16 D 1.2 Phototherapy TB 16 D 1.2 Phototherapy Jaundice, Pigmented Stool and High Colored Urine Poor Wt Gain 10gm/d Poor feeding Poor Wt Gain 10gm/d Poor feeding ? Seizure at D 42 Progressive Abdominal Distention HIDA Excretory Prof Mala Kumar, Dr Chavvi Nanda Dr Ashutosh Verma, Dr Salman Khan Prof Mala Kumar, Dr Chavvi Nanda Dr Ashutosh Verma, Dr Salman Khan

10. History Contd… History of 2 Sib deaths (<100 days).. 1 had seizures with aspiration and 1 had Jaundice with Ascites with ? septic shock 4 CM BCM 2 CM BCM Free Fluid + Examination To look in Eye ? Repeated Hypoglycemia

11. Summary D45 male child Jaundice, High Colored Urine & Pigmented stools Neonatal Cholestasis – Intrahepatic Sick Child with Ascites, Organomegaly, Cataract and Hypoglycemia. Family History of Sib Deaths with similar illness. D45 male child Jaundice, High Colored Urine & Pigmented stools Neonatal Cholestasis – Intrahepatic Sick Child with Ascites, Organomegaly, Cataract and Hypoglycemia. Family History of Sib Deaths with similar illness. Galactosemia Tyrosinemia Hereditary Hemochromatosis Mitochondrial Disorders Metabolic Liver Disorders with early onset ascites ?

12. How To Investigate ? Hb 10.8gm%; TLC 16,700 (P 78%) Platelet 210000 CRP Positive LFT (Bil 7.2/ D 4.0, SGOT 134, SGPT 198, ALP 887,GGT 24,Pr 6.2 Alb 2.4 ) PT 24 ; INR 2.0 (not Correctable to Vit. K) Blood Culture E. Coli Hb 10.8gm%; TLC 16,700 (P 78%) Platelet 210000 CRP Positive LFT (Bil 7.2/ D 4.0, SGOT 134, SGPT 198, ALP 887,GGT 24,Pr 6.2 Alb 2.4 ) PT 24 ; INR 2.0 (not Correctable to Vit. K) Blood Culture E. Coli Blood Ascetic Fluid High SAAG TLC 350 (All Lymphcyotsis) Culture : Sterile High SAAG TLC 350 (All Lymphcyotsis) Culture : Sterile Specific Test Urine Non Glucose Reducing Sugar S Ferritin S Alpha Feto Protein Urine Non Glucose Reducing Sugar S Ferritin S Alpha Feto Protein ++++ Normal ++++ Normal GAL- 1- PUT ABSENT

13. Diagnosis : Galactosemia Management ? Spectrum of Infantile Metabolic Liver Disease in India ?

14. Answers by experts Look in eyes for Cataract Cherry red spot, posterior embryotoxon and chorioretinitis HIDA not essential if Stool pigmented Most common MLD in infants is Galactosemia. Diagnosis is essential cause it can be managed and treatement Look in eyes for Cataract Cherry red spot, posterior embryotoxon and chorioretinitis HIDA not essential if Stool pigmented Most common MLD in infants is Galactosemia. Diagnosis is essential cause it can be managed and treatement

15. Case 3 7 year old boy presented Pain abdomen for the past 3 year Intermittent symptoms Periumblical, never nocturnal Lasts 5 to 20 minutes, 2 to 3 times a day No weight loss, fever, vomiting, loose stools Examination Normal growth parameters No abnormal physical finding 7 year old boy presented Pain abdomen for the past 3 year Intermittent symptoms Periumblical, never nocturnal Lasts 5 to 20 minutes, 2 to 3 times a day No weight loss, fever, vomiting, loose stools Examination Normal growth parameters No abnormal physical finding Prof. R. Ahuja Dr. Sanjay Niranjan, Dr Prashant Bhargava Prof. R. Ahuja Dr. Sanjay Niranjan, Dr Prashant Bhargava

16. 1 yr 2yr 3yr 4yr ATT (6 mo) ATT (6 mo) Ultrasound abdomen “Multiple mesenteric lymph nodes largest measuring 1 cm” “ Sub centrimetric Lymphnodes” “ Gaseous Distention of Bowel Loops” “ Abdomen is tender Sonographically” Treatment History No response Interpretation ?

17. Is this abdominal tuberculosis ?? Periumblical pain No red flags --- ? Normal growth Correct Diagnosis : Functional abdominal pain Counseling Fiber supplements Subsequent visits - pain resolved Management of ATT Induced Hepato-toxicity ? How to suspect & Confirm Alternate ATT How to resume

18. Case 4 –SOL Liver 5 year Boy; Wt: 18kg & Ht 105cm ; H/o Skin Infection x 3weeks back H/o High Grade Fever – 7 days with Pain RUQ Examination : Toxic Look ; Febrile ; Pallor +; Jaundice Absent Tender Hepatomegaly No Guarding, BS – Normal Hb 8.9gm%;TLC 33400, P 80%, CRP Positive LFT :WNL 5 year Boy; Wt: 18kg & Ht 105cm ; H/o Skin Infection x 3weeks back H/o High Grade Fever – 7 days with Pain RUQ Examination : Toxic Look ; Febrile ; Pallor +; Jaundice Absent Tender Hepatomegaly No Guarding, BS – Normal Hb 8.9gm%;TLC 33400, P 80%, CRP Positive LFT :WNL 3 CM BCM Dr PK Shukla Dr. Amit Rastogi, Dr Abhishek Bansal Dr PK Shukla Dr. Amit Rastogi, Dr Abhishek Bansal

19. Questions Microbiology of Liver Abscess ? Role of amoebic serology ? Role CT Scan/MRI in liver abscess ? Drugs for treatment ? How long ? Single time aspiration vs Precutaneous Drainage ? When to remove drain ? Sonologist says its not liquefied, no use attempting STA or PCD …How True ? Microbiology of Liver Abscess ? Role of amoebic serology ? Role CT Scan/MRI in liver abscess ? Drugs for treatment ? How long ? Single time aspiration vs Precutaneous Drainage ? When to remove drain ? Sonologist says its not liquefied, no use attempting STA or PCD …How True ? USG reveals a hypoechoic mass with irregular borders and internal septation in Rt. Lobe of liver S/O Abscess(Vol 130ml)

20. Case 5 –Incidentally detected SOL Liver 9 months Boy; Wt: 9kg & Ht 70cm Normal growth and development Incidentally detected hepatomegaly while visit for MMR vaccine Examination : Hepatomegaly firm, non tender No splenomegaly, Rest system wnl Investigations: CBC & LFT : WNL 9 months Boy; Wt: 9kg & Ht 70cm Normal growth and development Incidentally detected hepatomegaly while visit for MMR vaccine Examination : Hepatomegaly firm, non tender No splenomegaly, Rest system wnl Investigations: CBC & LFT : WNL 3 CM BCM Dr Ashutosh Pandey, Dr J D Rawat, Dr. S K Rai, Dr. Anurag Katiyar Dr Ashutosh Pandey, Dr J D Rawat, Dr. S K Rai, Dr. Anurag Katiyar

21. Questions FNAC VS Biopsy ? FNAC First chemotherapy or surgery ? Chemo Chemotheray regimen ? How to follow up after surgery ? With AFP FNAC VS Biopsy ? FNAC First chemotherapy or surgery ? Chemo Chemotheray regimen ? How to follow up after surgery ? With AFP AFP:50125 ng/ml ? Hepatoblastoma

22. Thank you