Table 3.
Clinical and biological symptoms and signs encountered in patients with congenital portosystemic shunts. Signs and symptoms not in order of frequency of presentation. ADHD = attention deficit hyperactivity disorder; LOC = loss of consciousness.
| Clinical and Biological Symptoms & Signs Encountered in Patients with Congenital Portosystemic Shunts [References] | ||||||
|---|---|---|---|---|---|---|
| Hepatic [1,3,4,5,9,12,15,16,17,33,34,35,36,37,38,39] | Gastro- Intestinal [9,55,56,57,58,59,60,61] |
Cardio- Pulmonary [1,5,10,18,40,41,42,43,44] |
Endocrine/Metabolic [1,3,13,19,20,21,22,29,33,62,63,64,65,66,67,68,69,70,71,72,73,74] | Renal [23,75,76,77,78,79,80] | Neurocognitive [1,5,9,12,25,26,33,37,45,46,47,48,49,50,51,52,53] | Other [3,5,33,56,81,82,83,84,85,86] |
| Abnormal hepatic vasculature on antenatal ultrasound |
Abdominal pain | Hepatopulmonary syndrome | Hyperinsulinemic hypoglycemia |
Proteinuria | Mild cognitive deficits | Brain abscesses (when associated with intrapulmonary shunts) |
| Tumors: | Gastrointestinal bleeding | Pulmonary artery hypertension | Hyperandrogenism | Hematuria | ADHD | Coagulation abnormalities |
| - Nodular regenerative hyperplasia | High-output cardiac failure | Precocious puberty | Post-prandial LOC | Cutaneous and visceral hemangioma |
||
| - Focal nodular hyperplasia | Amenorrhea | Parkinson-like | ||||
| Adenoma | Hypothyroidism | Hepatic myelopathy | ||||
| - Hepatoblastoma | Fetal growth retardation | Portosystemic encephalopathy |
||||
| - Hepatocellular carcinoma | Tall stature/overgrowth | Learning difficulties | ||||
| Hemangioma | Hyperammonemia | Unexplained mental retardation |
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| Hypoplastic left liver | Elevated serum bile acids | |||||
| Steatosis | Increased galactose on newborn screen | |||||
| Portal hypertension | ||||||