The document discusses the liver and chronic liver disease. It begins by describing liver anatomy and function. It then discusses various causes of cirrhosis and portal hypertension, including infections, genetic disorders, bile duct blockages, drugs/toxins, and alcoholic liver disease. Symptoms of chronic liver disease are described, ranging from early fatigue to later jaundice, edema, and risk of liver cancer. Complications of cirrhosis like ascites and varices are also outlined. The diagnosis and treatment of chronic liver disease focuses on managing underlying causes, preventing further damage, and treating complications.

6. I, caudate lobe; II-IV theI, caudate lobe; II-IV the
left-; V-VIII the rightleft-; V-VIII the right
hemiliver

8. Functional unit:Functional unit: acinusacinus
PT:PT: PV, arteriole, bilePV, arteriole, bile
ductuleductule
Zone 1Zone 1 is well O2; moreis well O2; more
resistant than zone 3.resistant than zone 3.
SinusoidsSinusoids lack BM; havelack BM; have
fenestrated endoth. &fenestrated endoth. &
Kupffer cells. BileKupffer cells. Bile
canaliculi join to formcanaliculi join to form
bile ductulesbile ductules
BM: basement membrane
THV: terminal hepatic venule
PT: Portal triad

10. Cirrhosis & PortalCirrhosis & Portal
HypertensionHypertension

11. At the end we will learnAt the end we will learn
 CLD is rare in children (1/5000)CLD is rare in children (1/5000)
 CLD may beCLD may be idiopathicidiopathic
 Most CLD are preventableMost CLD are preventable
 Commonest c/of PH is cirrhosisCommonest c/of PH is cirrhosis
 Commonest c/of HCC is HBVCommonest c/of HCC is HBV
 HCV is curableHCV is curable
CLD: chr. liver d. PH: portal HTN. HCC: HC CaCLD: chr. liver d. PH: portal HTN. HCC: HC Ca

12. DefinitionDefinition
Chr. liver d.:Chr. liver d.: progressive destruction & regen. ofprogressive destruction & regen. of
Liver parenchyma lastingLiver parenchyma lasting >6mo>6mo leading to cirrhosis.leading to cirrhosis.
It includes:It includes: inf., metabolic, genetic, drugs, idiopathic,., metabolic, genetic, drugs, idiopathic,
structural, HCC & AIDstructural, HCC & AID
Cirrhosis:Cirrhosis: scarring of Liver from injury/long-term d.scarring of Liver from injury/long-term d.
blocking BF:blocking BF: HC failure &HC failure &
Portal HTN developPortal HTN develop
Chr. Hepatitis:Chr. Hepatitis: chr. inflamchr. inflam
within L. leading to cirrhosiswithin L. leading to cirrhosis
& liver failure; persistent& liver failure; persistent
for a period of 6mofor a period of 6mo
BF: blood flow. D: disorder/diseaseBF: blood flow. D: disorder/disease

14. Etiology of Cirrhosis & PHEtiology of Cirrhosis & PH
Infections:Infections:
Liver disorders:Liver disorders:
Bile ductal blockages:Bile ductal blockages:
Drugs & toxins:Drugs & toxins:

15. Liver disorders:Liver disorders:
– Autoimmune hepatitis:Autoimmune hepatitis:
– Chr.Chr. alcoholismalcoholism
– Hereditary:Hereditary:
 HemochromatosisHemochromatosis
 αα1-antitrypsin deficiency1-antitrypsin deficiency
 GSDGSD
 GalactosemiaGalactosemia
 CFCF
 Fructose intoleranceFructose intolerance
 TyrosinemiaTyrosinemia
 Wilson DWilson D
– Indian childhood cirrhosisIndian childhood cirrhosis

16. Autoimmune Liver DiseasesAutoimmune Liver Diseases
Progressive inflam. by autoantibodies; rare in children:Progressive inflam. by autoantibodies; rare in children: 2%2%
of LD in specialized pediatric L. centersof LD in specialized pediatric L. centers
Broad clinical spectrum.Broad clinical spectrum. Quickly responds to steroidsQuickly responds to steroids
& immunosuppressants& immunosuppressants
CommonCommon SS:SS: tiredness & unwellness, anorexia, schooltiredness & unwellness, anorexia, school
deterioration.deterioration. HSM, abnormal LFT,HSM, abnormal LFT, autoantibodiesautoantibodies,,
characteristic livercharacteristic liver biopsybiopsy
DxDx is often byis often by exclusionexclusion. Anti-sm Ab, anti-liver kidney. Anti-sm Ab, anti-liver kidney
microsome type I Ab, ANA. Biopsy may be necessarymicrosome type I Ab, ANA. Biopsy may be necessary forfor
type & severity of L. damage & inflamtype & severity of L. damage & inflam
2 types:2 types: AIH & AI sclerosing cholangitisAIH & AI sclerosing cholangitis

17. Inherited liver diseasesInherited liver diseases
>100 liver diseases;>100 liver diseases; some are inherited. 2 common aresome are inherited. 2 common are
haemochromatosis & alpha-1 antitrypsin d.haemochromatosis & alpha-1 antitrypsin d.
Haemochromatosis:Haemochromatosis: excess Fe is absorbed & deposited. Theexcess Fe is absorbed & deposited. The
commonest is hereditary. 2y HC occurs in hemolytic a.commonest is hereditary. 2y HC occurs in hemolytic a.
Genetic HC affects M x5. As F menstruate, are unlikelyGenetic HC affects M x5. As F menstruate, are unlikely
to show SS till >10y after MP.to show SS till >10y after MP. More in N EuropeMore in N Europe
SS:SS: Hepatomegaly, joint p, fatigue, unexplained wt loss,Hepatomegaly, joint p, fatigue, unexplained wt loss,
darkening of skin ("bronzing“), AP, loss of libido. DMdarkening of skin ("bronzing“), AP, loss of libido. DM
& heart d. may dev.; also cirrhosis, HCC, testicular& heart d. may dev.; also cirrhosis, HCC, testicular
atrophy, & chr AP. Blood iron is confirmatory. HC DNAatrophy, & chr AP. Blood iron is confirmatory. HC DNA
test is done; also for screeningtest is done; also for screening

18. Rx.Rx. of haemochromatosisof haemochromatosis
 to remove excess Fe, reduce SS or complications. Fe isto remove excess Fe, reduce SS or complications. Fe is
removed by phlebotomy: ½L blood is removed/w for 2-3yremoved by phlebotomy: ½L blood is removed/w for 2-3y
until Fe is reduced; then less frequently: based onuntil Fe is reduced; then less frequently: based on
individual circumstancesindividual circumstances
 Avoid dietary Fe, most commonly offal, red meat &Avoid dietary Fe, most commonly offal, red meat &
fortified BF cereals, also multi-vitamin prepn.fortified BF cereals, also multi-vitamin prepn.
 It is also a good idea to limitIt is also a good idea to limit vitamin Cvitamin C
 No alcoholNo alcohol
 If HC has caused cirrhosis, the risk of HCC is higher. As aIf HC has caused cirrhosis, the risk of HCC is higher. As a
result, screening should be performed/6moresult, screening should be performed/6mo

19. Alpha-1 antitrypsin d.Alpha-1 antitrypsin d.
an imp. Liver protein alpha-1 antitrypsin,an imp. Liver protein alpha-1 antitrypsin, is either lacking oris either lacking or
low. Pts. are able to make it; but, the disease preventslow. Pts. are able to make it; but, the disease prevents
it from entering blood & accumulates in liverit from entering blood & accumulates in liver
 A1AT protects lungs from damage.A1AT protects lungs from damage. The d. causes the lungsThe d. causes the lungs
become damaged: difficulty breathingbecome damaged: difficulty breathing
 1% COPD have A1AT deficiency. Risk of cirrhosis is high1% COPD have A1AT deficiency. Risk of cirrhosis is high
SS:SS: first symptoms appear usually in lungs: COPD:first symptoms appear usually in lungs: COPD: SoB/SoB/
wheezing, unexplained wt loss & barrel-shaped chestwheezing, unexplained wt loss & barrel-shaped chest AsAs
it progresses, SS of cirrhosis may appearit progresses, SS of cirrhosis may appear
 A1AT in blood will confirmA1AT in blood will confirm

20. Rx.:Rx.: No cure. Replacement has been triedNo cure. Replacement has been tried
 Rx COPD & cirrhosis: ABT, inhaled medications. DiureticsRx COPD & cirrhosis: ABT, inhaled medications. Diuretics
& other measures to reduce edema& other measures to reduce edema
 No alcohol, smokingNo alcohol, smoking
 Healthy dietHealthy diet
 Flu & pneumonia vax.Flu & pneumonia vax.
 Treat inf.Treat inf. asapasap. Occasionally the lungs or liver deteriorate. Occasionally the lungs or liver deteriorate
despite Rx. Lung/liver transplant may be neededdespite Rx. Lung/liver transplant may be needed
 With appropriate Rx A1AT is usually not fatal. But,With appropriate Rx A1AT is usually not fatal. But,
complications a/with them can be fatalcomplications a/with them can be fatal
 It is v. imp. that people with inherited LD do all they canIt is v. imp. that people with inherited LD do all they can
to stay healthyto stay healthy

21. Bile ductal blockagesBile ductal blockages
– Cong.: biliary atresiaCong.: biliary atresia
– Sclerosing cholangitis
– Gallbladder surgeryGallbladder surgery

22. Drugs & toxinsDrugs & toxins
– Paracetamol. arsenic, INHParacetamol. arsenic, INH
– MethotrexateMethotrexate
– Excess vitamin A, ironExcess vitamin A, iron
– Afla toxinAfla toxin
InfectionsInfections
– Hepatitis B, C, DHepatitis B, C, D
– Schistosomiasis, BrucellosisSchistosomiasis, Brucellosis
– Echinococcosis, advanced or cong. syphilisEchinococcosis, advanced or cong. syphilis

23. Indian Childhood CirrhosisIndian Childhood Cirrhosis
a CLD of children (1–3y) due to deposition of Cu in liver.a CLD of children (1–3y) due to deposition of Cu in liver. It isIt is
a non-Wilsonian Cu overload by ingested Cu occurs ina non-Wilsonian Cu overload by ingested Cu occurs in
genetically susceptible infantsgenetically susceptible infants
 It had a v. high CFR before but has eventually becomeIt had a v. high CFR before but has eventually become
preventable, treatablepreventable, treatable
 Now rareNow rare
CFR: case fatality rateCFR: case fatality rate

24. The commonestThe commonest surgicalsurgical c/of cirrhosis inc/of cirrhosis in
childhood is biliary atresiachildhood is biliary atresia
The commonestThe commonest medicalmedical causes arecauses are
– infectionsinfections
– alpha-1- antitrypsin Dalpha-1- antitrypsin D
– metabolic LDmetabolic LD

25. C.F. of C.L.D.C.F. of C.L.D.
Often asymptomatic in early!Often asymptomatic in early! Start with L. failure.Start with L. failure.
Severity depends on damageSeverity depends on damage
– Early:Early: fatigue, weakness, AN, AP,fatigue, weakness, AN, AP, wt. losswt. loss
– Later:Later: jaundice,jaundice, dark urinedark urine, itching, edema, ascites,, itching, edema, ascites,
poor healing, drug toxicity, testicular atrophy, poorpoor healing, drug toxicity, testicular atrophy, poor
hair,hair, hepatic facies,hepatic facies, easy bruising; esophageal,easy bruising; esophageal,
stomach & anal varices;stomach & anal varices;
kidney failure, gall stoneskidney failure, gall stones
A small number getA small number get liver cancerliver cancer

26. CF have 2 componentsCF have 2 components
SS of Hepatocellular failureSS of Hepatocellular failure
– pallor, edema,pallor, edema, fatigue,fatigue, jaundice,jaundice, itchy skin, dark urine,itchy skin, dark urine,
ANVANV,, wt. loss, palmar erythemawt. loss, palmar erythema
– AP & swelling, pale stool, hepatic facies, spider nevi,AP & swelling, pale stool, hepatic facies, spider nevi,
pigmentationpigmentation
– testicular atrophy, poor hair, gynecomazia, poor libidotesticular atrophy, poor hair, gynecomazia, poor libido
SS of Portal HypertensionSS of Portal Hypertension
– ascites, distended vein (caput medusa), splenomegaly,ascites, distended vein (caput medusa), splenomegaly,
varices at PS junctions: hematemesis,varices at PS junctions: hematemesis, bloody/tar-bloody/tar-
colored stoolcolored stool

27.  Muehrcke's nailsMuehrcke's nails
 Terry’s nailsTerry’s nails

29. Kayser–Fleischer ringKayser–Fleischer ring
at limbus: depositionat limbus: deposition
of Cu in Descemet m.:of Cu in Descemet m.:
characteristic in mostcharacteristic in most
neuro-Wilson& 50% ofneuro-Wilson& 50% of
hepatic WDhepatic WD
Arcus senilis

31. Mechanism of Ascites in CirrhosisMechanism of Ascites in Cirrhosis
 Portal hypertension:Portal hypertension: commonestcommonest
 Hepatic arterial vasodilatationHepatic arterial vasodilatation
 HypoalbuminemiaHypoalbuminemia
 2y hyperaldosteronism2y hyperaldosteronism
 Raised ADHRaised ADH

32. Complications of CirrhosisComplications of Cirrhosis
 Palmar erythemaPalmar erythema
 HypogonadismHypogonadism
 Darkening of skinDarkening of skin
 GynecomaziaGynecomazia
 HypersplenismHypersplenism
 Spider neviSpider nevi
 Caput medusaCaput medusa
 Ascites, edemaAscites, edema
 Varices: hgeVarices: hge
 ItchingItching
 Abdominal infx.Abdominal infx.
 EencephalopathyEencephalopathy
 Raised drug toxicityRaised drug toxicity
 Liver cancerLiver cancer

35. Spider naevi (arterial
spider, vascular spider,
naevus araneus, naevus
arachnoidius, spider
angioma

36. DiagnosisDiagnosis
 LFTLFT
 CT, USG, laparoscopyCT, USG, laparoscopy
 BiopsyBiopsy
 Others:Others:
– FibroscanFibroscan
– Measuring the amount of caffeine in the salivaMeasuring the amount of caffeine in the saliva
– Measuring the pressure within the liver veinMeasuring the pressure within the liver vein
– Ascitic fluid analysisAscitic fluid analysis

37. TreatmentTreatment
Nothing cures scar tissue.Nothing cures scar tissue. Goals are:Goals are:
– Rx underlying causesRx underlying causes
– Preventing further damagePreventing further damage
– Rx symptoms & complicationsRx symptoms & complications
 Fight inf.Fight inf.
 Cut down absorption of wastes/toxinsCut down absorption of wastes/toxins
 FEB. Salt restrictionFEB. Salt restriction
 HemostasisHemostasis

38.  Liver transplant SOSLiver transplant SOS
 Vax. against flu, pneumonia, hepatitisVax. against flu, pneumonia, hepatitis
 No hepatotoxic drugsNo hepatotoxic drugs
 No alcoholNo alcohol
 Diet: extra calories & a generous protein to helpDiet: extra calories & a generous protein to help
liver regenerateliver regenerate
 If cirrhosis is advanced limit proteinIf cirrhosis is advanced limit protein

39. PORTALPORTAL
HYPERTENSION INHYPERTENSION IN
CHILDRENCHILDREN

40. IntroductionIntroduction
 Portal vein:Portal vein: SV with SMV: carries 70% of liver blood.SV with SMV: carries 70% of liver blood.
Ends in sinusoidsEnds in sinusoids
 Double capillariesDouble capillaries
 PH:PH: raised pressure in PV >raised pressure in PV >11mmHg11mmHg or a splenic pulpor a splenic pulp
pressure of >16mmHg. Clinically this is not measuredpressure of >16mmHg. Clinically this is not measured
directly until a TIPS is decideddirectly until a TIPS is decided
SV: splenic vein. SMV: superior mesenteric vein. PH: portal hypertensionSV: splenic vein. SMV: superior mesenteric vein. PH: portal hypertension
TIPS: transjugular intrahepatic portosystemic shuntTIPS: transjugular intrahepatic portosystemic shunt

42. PH:PH: splenomegalysplenomegaly && porto-systemic shunts at:porto-systemic shunts at:
 Lower end ofLower end of eophaguseophagus via GE veins:via GE veins: hemat./malenahemat./malena
 AnalAnal veins:veins: hemorrhoidshemorrhoids
 UmbilicalUmbilical veins:veins: caput medusacaput medusa
 Abdominal wall & retroperitoneum:Abdominal wall & retroperitoneum: distended veinsdistended veins
GE: gastroesophagealGE: gastroesophageal

44. Eesophageal varicesEesophageal varices
• Gastroesophageal junction to 15cm upGastroesophageal junction to 15cm up
• Slow oozing or sudden severe hgeSlow oozing or sudden severe hge
Gastric varicesGastric varices
Extension of esophageal varices or varices inExtension of esophageal varices or varices in
fundus & upper body. Can occur alonefundus & upper body. Can occur alone
Rectal varices:Rectal varices: hemorrhoidshemorrhoids

46. Gastric varicesGastric varices
Rectal varicesRectal varices

47. Aetiology of Portal HTNAetiology of Portal HTN
• IntrahepaticIntrahepatic:: 80%80%
• CirrhosisCirrhosis is the commonest; scar tissue blocks BF &is the commonest; scar tissue blocks BF &
slows liver functionsslows liver functions
•Schistosomiasis, cong. hepatic fibrosis, etc.Schistosomiasis, cong. hepatic fibrosis, etc.
• PrehepaticPrehepatic:: 20%20%
•PV thrombosis:PV thrombosis:
• extension of obliterative process of umb. veinextension of obliterative process of umb. vein
• omphalitis (in newborn)omphalitis (in newborn)
• PosthepaticPosthepatic (rare): cons. pericarditis, tricuspid(rare): cons. pericarditis, tricuspid
incompetence,incompetence, Budd-Chiari syn.Budd-Chiari syn. (occlusion of HV)(occlusion of HV)

48.  Cong. hepatic fibrosis:Cong. hepatic fibrosis: hematemesis, normal LFTs,hematemesis, normal LFTs,
hepatomegaly. May have polycystic disease& otherhepatomegaly. May have polycystic disease& other
renal D. Etiology is unknownrenal D. Etiology is unknown
 Hepato-portal sclerosis:Hepato-portal sclerosis: thickening of intrahepatic PVthickening of intrahepatic PV
obstructs BF: leads to collateral veins in porta hepatisobstructs BF: leads to collateral veins in porta hepatis
 Suprahepatic obstructionSuprahepatic obstruction c/by a web in the IVC orc/by a web in the IVC or
Budd-Chiari syn. Extremely rare. CF may mimicBudd-Chiari syn. Extremely rare. CF may mimic
constrictive pericarditis. DD by echo-, venographyconstrictive pericarditis. DD by echo-, venography

49. CF of portal hypertensionCF of portal hypertension
may not always be specificmay not always be specific
 Splenomegaly (hypersplenism: pancytopenia)Splenomegaly (hypersplenism: pancytopenia)
 Caput medusaCaput medusa
 Hematemesis, melena or PR bleedHematemesis, melena or PR bleed
 AscitesAscites
 Encephalopathy: poor liver function & diversion of bloodEncephalopathy: poor liver function & diversion of blood
away from liveraway from liver

50. DD of uncommon CLDDD of uncommon CLD
ConditionsConditions PresentationPresentation
Budd-Chiari SynBudd-Chiari Syn AscitesAscites
Primary sclerosingPrimary sclerosing
Cholangitis (PSC)Cholangitis (PSC)
Abnormal LFT/jaundiceAbnormal LFT/jaundice
Primary bil. cirrhosis (PBC)Primary bil. cirrhosis (PBC) Abnormal LFT, malaise,Abnormal LFT, malaise,
lethargy, itchinglethargy, itching
Caroli diseaseCaroli disease Abdominal pain/sepsisAbdominal pain/sepsis
Simple liver cystSimple liver cyst Pain/coincidental findingsPain/coincidental findings
Polycystic liver diseasePolycystic liver disease Pain/hepatomegalyPain/hepatomegaly

51. Diagnosis:Diagnosis: by detecting CLD, encephalopathy, ascites,by detecting CLD, encephalopathy, ascites,
varices.varices. Investigations:Investigations: esophagoscopy, Ba-swallow,esophagoscopy, Ba-swallow,
USG, celiac axis angiogramUSG, celiac axis angiogram
PV Thrombosis:PV Thrombosis: Dx is difficultDx is difficult
may present within 5y age as hematemesis with onlymay present within 5y age as hematemesis with only
splenomegaly & low plateletsplenomegaly & low platelet
• H/of umbilical V cannulation & abdo. sepsis (40%), orH/of umbilical V cannulation & abdo. sepsis (40%), or
trauma/pancreatitis. PVT is mainly congenital. LFTs intrauma/pancreatitis. PVT is mainly congenital. LFTs in thesethese
are normal. USG may confirm PVT: collateral veinsare normal. USG may confirm PVT: collateral veins in thein the
porta hepatis replacing the PVporta hepatis replacing the PV

52. TreatmentTreatment
Esophageal variceal bleeding:Esophageal variceal bleeding:
By the time pt. reaches ERBy the time pt. reaches ER
- 1/31/3rdrd
– stopped bleeding– stopped bleeding
- 1/31/3rdrd
– still oozing– still oozing
- 1/31/3rdrd
– still bleeding heavily– still bleeding heavily
Initial measures: ICUInitial measures: ICU
- BT: up to 10 unitsBT: up to 10 units
- LFT: coagulation profileLFT: coagulation profile
- If <50,000, platelet transfusion- If <50,000, platelet transfusion
- Endoscopic evaluation & treatment- Endoscopic evaluation & treatment

53. • Encephalopathy: may need ventilatorEncephalopathy: may need ventilator
• Bronchial aspiration – a frequent complicationBronchial aspiration – a frequent complication
• If profuse bleed prohibits endoscopy:If profuse bleed prohibits endoscopy:
• Insert Sengstaken – Blakemore tube & tamponade;Insert Sengstaken – Blakemore tube & tamponade;
deflate after 12h to avoid pressure necrosisdeflate after 12h to avoid pressure necrosis

55. Endoscopic sclerotherapyEndoscopic sclerotherapy
- Ethanolamine oleate- Ethanolamine oleate
- Sodium tetradesyl sulphate- Sodium tetradesyl sulphate
- 5% phenol in Almond oil- 5% phenol in Almond oil
- Butyl cyanoacrylate- Butyl cyanoacrylate

56. DrugsDrugs
• I/V Vasopressin. SE: Abdo. crampsI/V Vasopressin. SE: Abdo. cramps
• I/V Octreotide (Somatostatin analogue): EquallyI/V Octreotide (Somatostatin analogue): Equally
effective, safeeffective, safe
TIPSSTIPSS (transjugular intrahepatic portosystemic stent)(transjugular intrahepatic portosystemic stent)
revolutionized the Rxrevolutionized the Rx
• Main Rx for drug resistant cases or in endoscopicMain Rx for drug resistant cases or in endoscopic
therapy failuretherapy failure

58. Surgical shuntSurgical shunt
(Replaced by TIPSS & liver transplant)(Replaced by TIPSS & liver transplant)
Surgical shunts:Surgical shunts:
• splenorenalsplenorenal
• portocavalportocaval
SE: encephalopathy (40%)SE: encephalopathy (40%)
No benefit for pts. who have no bleedingNo benefit for pts. who have no bleeding

59. C.I.C.I. of Liver transplantof Liver transplant
• Age >65 yAge >65 y
• H/o IHD, HF, Chr. Respiratory diseaseH/o IHD, HF, Chr. Respiratory disease
• Previous Surgical shunts (relative C.I.)Previous Surgical shunts (relative C.I.)

60. Rx of Ascites of CLDRx of Ascites of CLD
• Salt restrictionSalt restriction
• DiureticsDiuretics
• ParacentesisParacentesis
• Peritoneovenous shuntingPeritoneovenous shunting
• TIPSSTIPSS
• Liver transplantLiver transplant

61. MCQMCQ
 Hepatitis A & E are common c/of CLDHepatitis A & E are common c/of CLD
 Vaccines for HBV protects against HDVVaccines for HBV protects against HDV
 Haemorrhoids in cirrhosis are due to HC failureHaemorrhoids in cirrhosis are due to HC failure
 Palmer erythema is normal in pregnancyPalmer erythema is normal in pregnancy
 Commonest c/of PHT is cirrhosisCommonest c/of PHT is cirrhosis

62. MCQMCQ
 Commonest surgical c/of cirrhosis in children isCommonest surgical c/of cirrhosis in children is
biliary atresiabiliary atresia
 Edema in CLD starts in legsEdema in CLD starts in legs
 CLD may be idiopathicCLD may be idiopathic
 Autoimmune LD have good prognosisAutoimmune LD have good prognosis
 A1AT protects lungsA1AT protects lungs

66. Next lecture:Next lecture:
UTI in ChildrenUTI in Children

67. THANK YOU

68. Cirrhosis: OverviewCirrhosis: Overview
Many children are Dx with cirrhosisMany children are Dx with cirrhosis
 L. is the 2L. is the 2ndnd
largest organ: processes nutrients. It is also alargest organ: processes nutrients. It is also a
big filter for blood: removing poisons & toxins as well asbig filter for blood: removing poisons & toxins as well as
byproducts from metabolism. It helps to control BGL &byproducts from metabolism. It helps to control BGL &
cholesterol, & makes coagulantscholesterol, & makes coagulants
 It has regenerative ability: large portion can be removed:It has regenerative ability: large portion can be removed:
will grow back with no problemswill grow back with no problems
 But this ability is not unlimited. If the damage is chr., theBut this ability is not unlimited. If the damage is chr., the
repair mechanisms fail & it begins to scar: when much of Lrepair mechanisms fail & it begins to scar: when much of L
will be replaced with scar:will be replaced with scar: cirrhosiscirrhosis

69.  As it's hard to reverse this scarring once started, the mainAs it's hard to reverse this scarring once started, the main
goal of Rx is to keep it from advancing further, generallygoal of Rx is to keep it from advancing further, generally
by addressing whatever underlying medical condition hasby addressing whatever underlying medical condition has
caused the liver to scar. If uncontrolled, cirrhosis can havecaused the liver to scar. If uncontrolled, cirrhosis can have
serious complications like PH & hepatopulmonary syn. Itserious complications like PH & hepatopulmonary syn. It
can also increase risk of HCCcan also increase risk of HCC
 While cirrhosis in adults is often c/by alcohol, in childrenWhile cirrhosis in adults is often c/by alcohol, in children
d. of biliary tract & inherited or genetic conditions are thed. of biliary tract & inherited or genetic conditions are the
most common reasons. It is important to stress thatmost common reasons. It is important to stress that nono
amount of alcohol consumption by a parent, even duringamount of alcohol consumption by a parent, even during
pregnancy, can cause a child to develop cirrhosispregnancy, can cause a child to develop cirrhosis

71. Cirrhosis of the liver Symptoms &Cirrhosis of the liver Symptoms &
CausesCauses
We understand that you may have a lot of questions when your child is diagnosed with
cirrhosis.
What exactly is it?
What are the causes of cirrhosis?
What are potential complications in my child’s case?
What are the treatments?
What are possible side effects from treatment?
How will it affect my child long term?
We’ve tried to provide some answers to those questions here, & when you meet with
our experts, they can explain your child’s condition & treatment options fully.
What is the liver, & what does it do?
The liver is the body’s second largest organ, located in the right side of the abdominal
cavity below the diaphragm & above the right kidney & intestines. The liver helps the
body in hundreds of ways:
All of the blood coming from the stomach & intestines passes through the liver through
a large vein called the portal vein. The liver turns nutrients from the food we eat &
chemicals from the medicines we take into forms that the rest of our bodies can use.
The liver helps clean the bloodstream of harmful substances & poisons.
The liver makes bile, which contains chemicals to help us digest the food we eat.

72. Testing & Dx for Cirrhosis of the liver in
Children
The first step in treating your child is
forming an accurate & complete
diagnosis. Your child’s doctor will usually
make a Dx of cirrhosis based on a
combination of symptoms, medical
history, physical exam, & blood tests.
In most cases, our doctors will order a
liver biopsy to confirm the diagnosis, &

73. Treatments for Cirrhosis of the liver in
Children
At Boston Children’s Hospital, we take a
multidisciplinary approach to the care of
children with cirrhosis. Our
Center for Childhood Liver Disease is one
of the few centers with a dedicated team
of specialists who are board-certified in
pediatric hepatology & transplant.
Cirrhosis has no cure; once the liver starts

74. Portal Hypertension Liver Disease in
Children
Contact the Center for Childhood Liver
Disease
1-617-355-5837
While liver disease is rare in children, portal
hypertension is even less common. For this
reason, few centers are able to adequately
manage the care of children with this
serious condition. Over the next few pages

75. Portal Hypertension Liver Disease Symptoms & Causes
Contact the Center for Childhood Liver Disease
1-617-355-5837
What is the liver, & what does it do?
The liver is the body’s second largest organ, located in the right side
of the abdominal cavity below the diaphragm & above the right
kidney & intestines. The liver helps the body in hundreds of ways.
All of the blood coming from the stomach & intestines passes
through the liver through a large vein called the portal vein. The
liver turns nutrients from the food we eat & chemicals from the
medicines we take into forms that the rest of our bodies can use.
The liver helps clean the bloodstream of harmful substances &
poisons.
The liver makes bile, which contains chemicals to help us digest the
food we eat.

76. Testing & Dx for Portal Hypertension
Liver Disease in Children
Contact the Center for Childhood Liver
Disease
1-617-355-5837
The first step in treating your child is
forming an accurate & complete
diagnosis. Because portal hypertension
can cause a variety of complications, your
child’s doctor will likely already be on the

77. Treatments for Portal Hypertension Liver
Disease in Children
Contact the Center for Childhood Liver
Disease
1-617-355-5837
As there is no simple cure for portal
hypertension, our
Center for Childhood Liver Disease takes
a multidisciplinary approach to preventing
the condition from becoming worse while

78. Surgery – porto-systemic shunts
sclerotherapy with banding is effective control
bleeding esophageal varices in majority of
children. surgical intervention :
Failure of at least 2 sessions of banding/
sclerotherapy
gastric/ectopic varices not responding to
endoscopic Rx
Hypersplenism
Lack of access to endoscopic Rx
biliary obstruction due to choledochal varices
Selected patients with Budd-Chiari syn
30 different operations were reported. Occlusion
of the portal system in the extrahepatic portal
hypertension, for example, may affect the portal
vein alone or may involve either the splenic or the
superior mesenteric veins. At least 20 percent of

79.  What tests are required before the TIPS& DSRS
procedures?
 Evaluation of medical history
 A physical examination
 Blood tests
 Galactose LFT
 Angiogram
 Ultrasound
 Endoscopy
 Before either the TIPS or DSRS procedure, your physician
may ask you to have other pre-operative tests, which
may include an electrocardiogram (also called an EKG),
chest X-ray or additional blood tests. If your physician
thinks you will need additional blood products (such as
plasma), they will be ordered at this time.
 More about the TIPS procedure
During the TIPS procedure, a radiologist makes a tunnel
through the liver with a needle, connecting the portal
vein (the vein that carries blood from the digestive organs
to the liver) to one of the hepatic veins (the three veins

80. FU after TIPS or DSRS
 After 10 d: evaluate progress by Lab work
 6 weeks& again 3 mo: do USG to check shunt. Do
angiogram only if it indicates a problem
 6 mo after TIPS or DSRS: do an USG to see shunt. Lab
work& a galactose LFT also done
 12 mo: USG. angiogram to check pressure within veins
across the shunt. Lab work& a galactose LFT done
 If the shunt is working, every 6 mo after the first year
Other treatment
 Liver transplant
 removes the varices when a TIPS or a surgical shunt is not

81. Clinical features
PH mainly starts as splenomegaly at 8 y age
Encephalopathy& ascites may be present
Growth failure (malabsorption, protein losing
enteropathy) is well-noted
In PV occlusion: <5 y age with ac. GI hge.,
hemorrhoids seen in 2/3
In Budd-Chiari syn, intractable ascites with
hepatomegaly is the usual initial presentation.
Jaundice is variable

82. InvestigationsInvestigations
Hypersplenism : anemia, low WBC, platelet.
Plasma procoagulant& anti- may be reduced
in PV occlusion or Budd-Chiari syn
LFT are abnormal in cirrhosis, but normal in
PVO. Albumin is low in ac. variceal hge
USG: large collateral veins, portal cavernoma,
splenomegaly. Doppler for direction, velocity&
waveform characteristics of PV flow. In Budd-
Chiari syn, Doppler of HV& IVC can be Dx.
Ascites is a notable feature of BCS& cirrhosis
can be definitely identified& quantified on USG

83. GI endoscopy
Useful in Dx& Rx of varices
grading of esophageal varices. Smaller
Portal congestive gastropathy is characterized
by mucosal hyperemia with dilated
submucosal veins
CT angiography& MRI
increasingly used in Dx of BCS& to identify
liver lesions in PH (focal nodular hyperplasia)
MR angiography is non-invasive. It delineates
porto-mesenteric venous anatomy

84. Angiography
Inferior vena cavography with pressure
measurements is valuable in patients with
Budd-Chiari syndrome in whom hepatic
venography can be used to assess hepatic
venous patency. Balloon dilatation can be
undertaken of inferior vena caval membrane
or short segment narrowing of the hepatic
veins, which can prove therapeutic.

85. signs of cirrhosis. As noted before, these signs are not specific to cirrhosis, nor
do they occur in all cases of cirrhosis:
Spider angiomata or spider nevi, spider telangiectasias: Vascular lesions
consisting of a central arteriole (the smallest branches of an artery, terminating
in capillaries) surrounded by many smaller vessels commonly found on trunk,
face,& upper limbs, due to an increase in estradiol to free testosterone ratio.[3]
These occur in about one third of cases.[4]
Verify this finding by compressing it
with a glass slide,& observe the pulsation of the central arteriole as blood fills
the central arteriole, then travels to the peripheral tips with blanching. Spider
angiomas may also be seen in pregnancy (high estrogen state), severe
malnutrition, or occasionally in healthy individuals. Greater number& size of
spider angioma are associated with more severe liver cirrhosis& risk of bleeding
from varices (permanent abnormal dilation& lengthening of a vein).[5][6]
Palmar erythema: Exaggerations of normal speckled mottling of the palm, due
to altered sex hormone metabolism.[7]
Palmar erythema affects mainly the
thenar& hypothenar prominences while sparing the central palm. It is also seen
in pregnancy, rheumatoid arthritis, hyperthyroidism,& blood malignancies.
Nail changes:
– Muehrcke's nails: Paired horizontal bands separated by normal color due to
hypoalbuminemia,[8]
from inadequate production of albumin, which is solely made by the
liver. Muehrcke's nails are seen in other conditions associated with low serum albumin,
such as malnutrition& nephrotic syndrome (related to the kidney).
– Terry's nails: Proximal two-thirds of the nail plate appears white& distal one-third red,

86. Rx for PHT
 Diet, drugs, endoscopic therapy, surgery or radiology
 Rx are based on severity of the symptoms& liver
functioning.
 First level of Rx
For variceal bleeding, endoscopic therapy or medications.
Dietary& lifestyle changes are also important.
 Endoscopic Rx: either sclerotherapy or banding.
Sclerotherapy: a solution is injected into the bleeding
varices to stop or control the risk of bleeding. Banding: a
rubber bands to block the blood supply to each varix.
 Medications: beta blockers or nitrates may be prescribed
alone or in combination with endoscopic therapy to

87. Treatment
The survival of the children with portal
hypertension depends almost entirely on the
etiology. Recent reports show that
oesophageal varices in childhood are well
controlled with either injection sclerotherapy
or porto-systemic shunting& both methods
have their advocates. Patients with portal vein
obstruction& normal liver histology can be
expected to live normal lives providing the
oesophageal varices are under control.

88. A)Treatment of the acute bleed
Acute variceal bleeding, particularly in young
infants, can pose problems in management. A
delay in immediate management could prove
fatal for a child. Medical measures include
blood transfusion& the intravenous infusion of
vasopressin (0.2 – 0.4 units/1.73 m / min)
which may arrest the bleeding. Vasopressin or
its precursor, glypressin may be used alone or
in combination with nitrates to reduce the
portal venous pressure. Unfortunately, these
agents have side-effects related to systemic

89. B) Inj. sclerotherapy for long-term Rx.
Injection sclerotherapy was suggested for the
treatment of oesophageal varices in children
because of failures& complications of primary
surgery. Portosystemic shunt thrombosis&
rebleeding, the hazards of splenectomy in
children& long term risks of encephalopathy
all encouraged an alternative therapy.
Controlled trials in adult patients confirmed
that early endoscopic sclerotherapy after the
onset of bleeding significantly reduced the risk
of rebleeding& may prolong survival in the
cirrhotic. Injections are performed through a

90. C) Variceal banding.
This technique, which involves application of
an elastic band to a variceal column, is done
through flexible upper gastro-intestinal
endoscopes. The strangulated varix,
subsequently, thromboses& sloughs. Usually
upto three bands are applied at each session.
Multi-band devices allow the application of
several bands without the need for reloading.
Treatment is performed initially at 1 to 2
weekly intervals, extending to monthly
intervals once the larger varices are treated.

91. D) Transjugular intrahepatic port-systemic
shunt (TIPS)
The indications of TIPS in children include
uncontrolled variceal bleeding especially the
ones who are awaiting liver transplantation.
Some patients of Budd-Chiari syndrome or
intractable ascitis may also benefit by this
procedure. Portal vein thrombosis, bacterial
sepsis& coagulopathy are contraindications to
TIPS.
This intervention involves insertion of an