Faeco-oral transmission. Global distribution.
Incubation 4/52, can be asymptomatic in young children. Non-specific fever, malaise, possibly RUQ pain before jaundice appears, usually with rapid relief in symptoms. Up to 1% however have fulminant disease with hepatic failure.
Post exposure prophylaxis for hepatitis A with hepatitis A vaccine reduces secondary infection rate from 13% to 2.8% (NNT=18).
See also viral hepatitis.
Plus the newer D (faeco-oral as A, particularly severe in pregnant women) and E (a co-factor in Hepatitis B infection).
But a range of other viruses can cause hepatitis eg EBV, Varicella.
= hepatolenticular degeneration. Autosomal recessive condition with copper accumulation due to impairment of biliary excretion. Leads to cirrhosis, via a stage indistinguishable from chronic active hepatitis, plus neurological disease. Caused by mutations of the ATP7B gene that codes for a copper transporting ATPase – over 300 mutations known, varying geographically.
Usually presents in late teens but has been described as young as 3yrs. Neurological presentation tends to be older (by 5 years) although they usually have subclinical liver disease. Hepatic disease varies from elevated aminotransferases, through chronic liver disease to fulminant hepatic failure (often with Coombs negative haemolytic anaemia), about 5% of presentations.
Basal ganglia involvement leads to movement disorders viz:
Other neurological signs are:
It can also cause:
Can be tricky given multisystem disorder and limited sensitivity/specificity of tests. Heterozygotes may also have borderline results. If typical presentation then diagnosis can be made on basis of:
May require extensive tests of copper metabolism especially with severe hepatic presentation, where up to 50% can have normal ceruloplasmin (an acute phase reactant) eg
In fulminant hepatic failure the following features may suggest diagnosis:
Broad spectrum antibiotics usually added in. Some suggest N-acetyl cysteine even without Paracetamol ingestion (Gastroenterology. 2009 Sep;137(3):856-64).
Transplant: indicated when bilirubin >150, hypoalbuminaemia, failing nutrition.
ie raised blood bilirubin.
First decide if conjugated or unconjugated. Conjugated suggests a biliary problem, ie obstruction to bile flow, usually associated with raised gamma glucuronyl transferase (gamma GT). Unconjugated (as measured in split bilirubin) implies haemolysis or else hepatocellular damage (transaminases also go up ie AST, ALT). Unconjugated can also go up in conjugated disease, presumably competition for conjugation sites, or else because of secondary or mixed hepatocellular damage.
History – maternal if baby, immunisations, transfusions, outdoor activities, pets, holidays, drugs, tattoos. Family history (haemolytic conditions, in particular).
Look for signs of liver failure, lymphadenopathy, eyes (Wilsons – best with slit lamp), dysmorphic (Alagille’s).
Consider:
So do:
If PT>15, give IV vitamin K (0.3mg/kg, max 10mg) and check daily. If resistant then refer.
Damage is from sodium hydroxide generated by electric current, not from leakage!
Should be recognised as a medical emergency, deaths have occurred.
Haematemesis, haemoptysis and respiratory difficulties can manifest up to 28 days after ingestion of the battery, so easy to miss or ignore. Given the age of the children involved, they are unlikely to tell you! Catastrophic aortooesophageal fistula possible, as in the case of Hugh McMahon in Lanarkshire. Earliest perforation has been at 12 hours – median is 5 days. [Am J Emerg Medicine 2019]
Removal of the battery alone may be insufficient action to prevent further damage, with further symptoms manifesting later; patients need expert input, and careful monitoring and follow-up. One further incident described the death of a child from late complications after they had been treated and sent home.
US Poisons centre recommends use of honey or sucralfate if ingestion within the last 12 hours.
Rome III classification (now Rome IV?) of functional GI disorders – has child section. Colic, rumination, cyclical vomiting, diarrhoea, dyspepsia, abdo migraine, IBS, abdominal pain, constipation.
Functional abdominal pain (FAP) must include all of the following:
Episodic or continuous abdominal pain
Insufficient criteria for other functional GI disorders
No evidence of an inflammatory, anatomic, metabolic, or neoplastic process that explains the subject’s symptoms
Must include childhood functional abdominal pain, and at least 25% of the time have 1 or more of the following:
Some loss of daily functioning
Additional somatic symptoms such as headache, limb pain, or difficulty sleeping
That loss of daily function is an optional criterion is because it would exclude motivated children who continued activity despite the pain and children whose parents insisted that they continue activities. However, it is recognized that there is a subgroup of children in whom loss of daily functioning and/or accompanying somatic symptoms form an important component of their symptom complex. This group is now referred to as having FAPS.
See testing.
It is still debatable whether H pylori can cause symptoms in the absence of peptic ulcer disease. Young children may of course not give a typical ulcer history, but clues would be epigastric, pain before meals or helped by eating or antacids. Not helpful when functional abdominal pain or reflux disease.
A meta-analysis of 45 studies concluded that H pylori infection is not associated with abdominal pain. In a German study of 1221 children social and familial factors (single-parent household, family history of PUD, or functional pain) were significantly associated with abdominal pain, but not with the H pylori status of the child, as assessed by the 13C-Urea Breath Test.
In a study of 695 schoolchildren between 10 and 12 years old there was no association between H pylori and recurrent abdominal pain, in fact there was an inverse relationship after adjustment for selected possible confounders!
Although there have been studies that seem to show that symptoms improve with treatment, these have been of poor quality and the natural course of illness is to improve anyway.
Now that a link has been established between helicobacter and cancer (esp stomach, via chronic gastritis, also non-Hodgkins lymphoma) in adults, benefits and risks of treatment when it has been discovered should be discussed.
Who Should Be Tested? See Helicobacter.
Refractory iron-deficiency anaemia is not considered a good reason for testing anymore.
Gold standard is Gastric biopsies (antrum and corpus) for histopathology during endoscopy. Initial diagnosis of H. pylori infection can be based on either positive histopathology + positive rapid urease test, or a positive culture.
Stool antigen test is pretty reliable. Could simply indicate asymptomatic carriage though. Once you know about it, of course, you need to consider eradication given cancer risk.
You should wait at least 2 weeks after stopping proton pump inhibitor (PPI) therapy and 4 weeks after stopping antibiotics before doing any of these tests.
Detection of antibodies against H. pylori (whether in blood, urine, saliva) are not reliable for use in the clinical setting.
[ESPGHAN and NASPGHAN. Evidence-based guidelines J Pediatr Gastroenterol Nutr. 2016]
Children with congenital heart disease – Consider stopping domperidone therapy or discuss with parents/carers and ensure that cardiac monitoring is regularly performed. Consider offering an alternative treatment where appropriate.
Other children with established reflux or nausea and vomiting – Take no immediate action in patients already established on domperidone. Consider reducing the dose (where appropriate) to 250microgram/kg three times a day at the next convenient review. Consider routine cardiac monitoring where there are concerns (e.g. cardiovascular instability,
concomitant CYP3A4 inhibitors prescribed).
In new patients, always give a proper trial of feed thickeners before considering pharmacological intervention – at least two weeks. In more serious cases, and after the introduction of thickeners then consider the benefits and risks of medical anti-reflux/anti-acid secretion treatment.
If domperidone is to be used, give an initial maximum of
250micrograms/kg three times a day. Where reflux or nausea is refractory to
this then give increased doses to a maximum of 400micrograms/kg (max
20mg) three times a day and recommend regular cardiac monitoring.
Patient Information Leaflet entitled “Domperidone for gastrooesophageal
reflux” available from www.medicinesforchildren.org.uk