From:  Pediatric cirrhosis: special consideration for its diagnosis and management

 Cirrhosis complications in children.

CategoryComplicationTreatmentWhen to admitPearls and pitfalls
Hydrostatic pressure-related complicationsTreatable by portal system decompressionAscitesSodium restriction

  • De novo ascites

  • Fever

  • Respiratory distress

  • Diuretic refractory

  • Only using furosemide

  • Aggressive fluid restriction in the absence of hyponatremia

Spironolactone 3–6 mg/kg
Furosemide 1 mg/kg
Spontaneous bacterial peritonitis3rd generation cephalosporins

  • Always

  • Waiting for cultures to treat

Gastroesophageal varicesEndoscopic band ligation

  • Variceal bleed

  • Avoid beta-blockers*

  • EVL if > 8–10 kg

  • Sclerotherapy if < 8 kg

  • Always treat hemorrhage with antibiotics

Octreotide 1–5 µg/kg/h
Proton pump inhibitors BID
3rd generation cephalosporins
Hepatorenal syndromeTerlipressin + albumin

  • Always

  • A watchful “volume repletion” helps rule out pre-renal AKI

Liver transplantation
Not treatable by portal system decompressionHepatic encephalopathyLactulose 5–30 mL TID-QID

  • Always grade III–IV

  • Any progression

  • New onset

  • Avoid sedatives

  • Temporary protein restriction (0.5–1 g/kg) may be useful in grades II–IV

  • NH3 helps guide treatment

Rifaximin 10–15 mg/kg/day BID
Porto-pulmonary hypertensionEndothelin receptor antagonists (bosentan)

  • Dyspnea

  • Thoracodynia

  • Signs of heart failure

  • Hemoptysis

  • Screen with 3D-cardiac ultrasound

Phosphodiesterase-5 inhibitors (sildenafil)
Prostacyclin analogues (epoprostenol)
Hepatopulmonary syndromeLiver transplantation

  • Worsening hypoxemia

  • Screen with pulse oximetry in upright position breathing room air for 5 min

Not pressure-related complicationsMalnutritionMCT and essential fatty acids supplementation

  • Consider if BMI < p5

  • Hepatic encephalopathy

  • Need for nutritional therapy intensification

  • Weight is not reliable as oedema and ascites overestimate nutritional status

  • Sarcopenia is related to worse outcomes

  • High doses of ADEK vitamins are needed

Vitamin A: 5,000–10,000 IU/day
Vitamin D: 2,000–5,000 IU/day
Vitamin E: 20–100 IU/kg
Vitamin K: 2–10 mg/day
Zinc: 1–2 mg/kg/day
Hepatocellular carcinoma (HCC)Surgical resection

  • For treatment, either surgical or medical

  • Metabolically related cirrhosis is at higher risk

  • HCC may develop in the absence of cirrhosis

Immunotherapy (PD-1/PD-L1 or CTLA-4)
Pembrolizumab (pediatric dose to be determined)
Liver transplantation
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*: Some centers adopted the use of carvedilol as primary prophylaxis, though not much literature exists to formally adopt this practice, and its use is left to the clinician’s judgment. BID: twice daily; EVL: endoscopic variceal ligation; AKI: acute kidney injury; TID: thrice daily; QID: four times a day; NH3: ammonia; MCT: medium-chain triglycerides; BMI: body mass index; ADEK vitamins: vitamins A, D, E, K; PD-1: programmed cell death protein 1; PD-L1: programmed death-ligand 1; CTLA-4: cytotoxic T-lymphocyte-associated protein 4.