Last updated:
April 1, 2026
Biliary atresia is a progressive fibro-inflammatory obliteration of the lumen of the biliary tree. It causes conjugated hyperbilirubinaemia, since there are defects in the biliary tract, resulting in cholestasis. The conjugated bilirubin is reabsorbed into the bloodstream, causing a direct hyperbilirubinaemia.
It has a slight female preponderance.
- Signs and symptoms
- A neonate who was normal on delivery and discharge
- Develops jaundice at 2-3 weeks of life
- Dark urine
- Acholic (clay-colored) stools
- Failure to thrive within a few months
- Signs and symptoms of portal hypertension (ascites, splenomegaly, etc.) within a few months
- Investigations
- LFTs
- Elevated ALP and GGT
- Abdominal ultrasound: This is the best initial test. It is done to rule out a choledochal cyst and extrinsic compression
- “Triangular cord sign” along the porta hepatis
- HIDA scan:
- Normal uptake
- Decreased excretion
- Percutaneous liver biopsy
- Intraoperative cholangiogram: This is the most accurate diagnostic test
- LFTs
- Treatment
- Kasai procedure (hepatoportoenterostomy)
- It should be performed early, within 8 weeks of age
- Transplantation
- Post-operative nutritional supplementation
- A formula with medium-chain triglycerides is recommended
- Monitor fat-soluble vitamins
- Kasai procedure (hepatoportoenterostomy)
Reference Intervals
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Biochemistry
| ACTH | P: <80 ng/L |
| ALT | P: 5–35 U/L |
| Albumin | P: 35–50 g/L |
| Aldosterone | P: 100–500 pmol/L |
| Alk. phosphatase | P: 30–130 U/L |
| α-Amylase | P: 0–180 IU/dL |
| α-Fetoprotein | S: <10 kU/L |
| Angiotensin II | P: 5–35 pmol/L |
| ADH | P: 0.9–4.6 pmol/L |
| AST | P: 5–35 U/L |
| Bicarbonate | P: 24–30 mmol/L |
| Bilirubin | P: 3–17 μmol/L |
| BNP | P: <50 ng/L |
| CRP | P: <10 mg/L |
| Calcitonin | P: <0.1 mcg/L |
| Calcium (ionized) | P: 1.0–1.25 mmol/L |
| Calcium (total) | P: 2.12–2.60 mmol/L |
| Chloride | P: 95–105 mmol/L |
| Cholesterol | P: <5.0 mmol/L |
| VLDL | P: 0.128–0.645 mmol/L |
| LDL | P: <2.0 mmol/L |
| HDL | P: 0.9–1.93 mmol/L |
| Cortisol AM | P: 450–700 nmol/L |
| Cortisol Midnight | P: 80–280 nmol/L |
| CK ♂ | P: 25–195 U/L |
| CK ♀ | P: 25–170 U/L |
| Creatinine | P: 70–100 μmol/L |
| Ferritin | P: 12–200 mcg/L |
| Folate | S: 2.1 mcg/L |
| FSH | P: 2–8 U/L ♂; >25 menopause |
| GGT ♂ | P: 11–51 U/L |
| GGT ♀ | P: 7–33 U/L |
| Glucose (fasting) | P: 3.5–5.5 mmol/L |
| Growth hormone | P: <20 mu/L |
| HbA1C (DCCT) | B: 4–6% |
| HbA1C (IFCC) | B: 20–42 mmol/mol |
| Iron ♂ | S: 14–31 μmol/L |
| Iron ♀ | S: 11–30 μmol/L |
| Lactate (venous) | P: 0.6–2.4 mmol/L |
| Lactate (arterial) | P: 0.6–1.8 mmol/L |
| LDH | P: 70–250 U/L |
| LH | P: 3–16 U/L |
| Magnesium | P: 0.75–1.05 mmol/L |
| Osmolality | P: 278–305 mosmol/kg |
| PTH | P: 0.8–8.5 pmol/L |
| Potassium | P: 3.5–5.3 mmol/L |
| Prolactin ♂ | P: <450 U/L |
| Prolactin ♀ | P: <600 U/L |
| PSA | P: 0–4 mcg/mL |
| Protein (total) | P: 60–80 g/L |
| Red cell folate | B: 0.36–1.44 μmol/L |
| Renin (erect) | P: 2.8–4.5 pmol/mL/h |
| Renin (recumbent) | P: 1.1–2.7 pmol/mL/h |
| Sodium | P: 135–145 mmol/L |
| TBG | P: 7–17 mg/L |
| TSH | P: 0.5–4.2 mU/L |
| T4 | P: 70–140 nmol/L |
| Free T4 | P: 9–22 pmol/L |
| TIBC | S: 54–75 μmol/L |
| Triglycerides | P: 0.50–2.3 mmol/L |
| T3 | P: 1.2–3.0 nmol/L |
| Troponin T | P: <0.1 mcg/L |
| Urate ♂ | P: 210–480 μmol/L |
| Urate ♀ | P: 150–390 μmol/L |
| Urea | P: 2.5–6.7 mmol/L |
| Vitamin B12 | S: 0.13–0.68 nmol/L |
| Vitamin D | S: 50 nmol/L |
Arterial Blood Gases
| pH | 7.35–7.45 |
| PaCO₂ | 4.7–6.0 kPa |
| PaO₂ | >10.6 kPa |
| Base excess | ±2 mmol/L |
Urine
| Cortisol (free) | <280 nmol/24h |
| Hydroxyindole acetic acid | 16–73 μmol/24h |
| Hydroxymethylmandelic acid | 16–48 μmol/24h |
| Metanephrines | 0.03–0.69 μmol/mmol cr. |
| Osmolality | 350–1000 mosmol/kg |
| 17-Oxogenic steroids ♂ | 28–30 μmol/24h |
| 17-Oxogenic steroids ♀ | 21–66 μmol/24h |
| 17-Oxosteroids ♂ | 17–76 μmol/24h |
| 17-Oxosteroids ♀ | 14–59 μmol/24h |
| Phosphate (inorganic) | 15–50 mmol/24h |
| Potassium | 14–120 mmol/24h |
| Protein | <150 mg/24h |
| Protein/creatinine ratio | <3 mg/mmol |
| Sodium | 100–250 mmol/24h |
Haematology
| WCC | 4.0–11.0 ×10⁹/L |
| RBC ♂ | 4.5–6.5 ×10¹²/L |
| RBC ♀ | 3.9–5.6 ×10¹²/L |
| Hb ♂ | 130–180 g/L |
| Hb ♀ | 115–160 g/L |
| PCV ♂ | 0.4–0.54 L/L |
| PCV ♀ | 0.37–0.47 L/L |
| MCV | 76–96 fL |
| MCH | 27–32 pg |
| MCHC | 300–360 g/L |
| RDW | 11.6–14.6% |
| Neutrophils | 2.0–7.5 ×10⁹/L (40–75%) |
| Lymphocytes | 1.0–4.5 ×10⁹/L (20–45%) |
| Eosinophils | 0.04–0.44 ×10⁹/L (1–6%) |
| Basophils | 0–0.10 ×10⁹/L (0–1%) |
| Monocytes | 0.2–0.8 ×10⁹/L (2–10%) |
| Platelets | 150–400 ×10⁹/L |
| Reticulocytes | 0.8–2.0% / 25–100 ×10⁹/L |
| Prothrombin time | 10–14 s |
| APTT | 35–45 s |
Paediatric
| Pulse Rate (bpm) | |
|---|---|
| Neonate | 140–160 |
| Infant <1yr | 120–140 |
| 1–5 years | 110–130 |
| 5–12 years | 80–120 |
| >12 years | 70–100 |
| Respiratory Rate (tachypnoea) | |
| 0–2 months | ≥60/min |
| 2–12 months | ≥50/min |
| 1–5 years | ≥40/min |
| >5 years | ≥30/min |
| Blood Pressure (mmHg) | |
| Term | 65/45 |
| 1 year | 75/50 |
| 4 years | 85/60 |
| 8 years | 95/65 |
| 10 years | 100/70 |
| Weight Formulas | |
| 3–12 months | (a + 9)/2 kg |
| 1–6 years | 2a + 8 kg |
| >6 years | (7a − 5)/2 kg |
| Haemoglobin (g/dL) | |
| Term newborn | 13–20 |
| 1 month | 11–18 |
| 2 months | 10–15 |
| 1–2 years | 10–13 |
| >2 years | 11–14 |
| MUAC (6 months–5 years) | |
| Obese | >17.5 cm |
| Normal | 13.5–17.4 cm |
| At risk | 12.5–13.4 cm |
| Moderate malnutrition | 11.5–12.4 cm |
| Severe malnutrition | <11.5 cm |
| Developmental Milestones | |
| Social smile | 1.5 months |
| Head control | 4 months |
| Sits unsupported | 7 months |
| Crawls | 10 months |
| Stands unsupported | 10–12 months |
| Walks | 12–13 months |
| Talks | 18 months |
| CSF WBC (/mm³) | |
| Term newborn | 0–25 |
| >2 weeks | 0–5 |
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