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Diagnosis of neonatal cholestasis; outcome in a regional centre.
  1. S Tamhne1,
  2. C Charlton1,
  3. D O'Neill2
  1. 1Child Health, Nottingham University Hospitals NHS Trust, Queen's Medical Centre Campus, Nottingham, UK
  2. 2Paediatric Pathology, Nottingham University Hospitals NHS Trust, Nottingham, UK

Abstract

Aims Extrahepatic biliary atresia (EHBA), fatal without surgery, has a prevalence of 1 in 15000 births and an incidence of 50 cases per year. A Kasai operation before 8 weeks gives the best chance of survival with native liver. Quality clinicopathological care comprises early recognition of cholestatic jaundice, with prompt diagnosis and referral of cases of EHBA (or other causes of surgically correctable biliary obstruction) to super-regional centres for specialised surgery. This requires close cooperation between gastroenterologist and pathologist. Our aim was to assess the quality of our clinical and pathological contribution to this pathway.

Methods We identified all infants over the last 12 years who had underdone liver biopsy for cholestatic jaundice. Every case had been discussed at clinicopathological conference. We examined the clinical and pathological records to establish initial diagnoses and subsequent outcome.

Results *P<0.05 compared to Pre SMOFOf 31 cases with cholestatic jaundice and liver biopsy, 5 cases of EHBA and 1 case of choledochal cyst were referred (together with histological slides) to super-regional centres, where the diagnoses were confirmed. Age at biopsy of the cases of EHBA were: 27, 48, 30, 36 and 136 days. Four children underwent Kasai procedure before 8 weeks. The fifth child had presented at 19 weeks and was referred on promptly; after counselling, the parents elected for primary liver transplantation. The histological slides were forwarded (without the patient) for second opinion in 4 cases; the specialist centre concurred with the local differential diagnoses and referral of these patients was not considered necessary. No cases of EHBA or choledochal cyst were missed. Of those not referred, it was possible to reach specific diagnoses in a subset of cases as follows: TPN hepatopathy (4); alpha-1 antitrypsin deficiency (3); Alagille syndrome (2); non-syndromic paucity of intrahepatic bile ducts (1); hypoadrenalism (1); hypopituitarism (1); CMV hepatopathy (1); cystinosis (1). The clinical features resolved in 14 cases.

Conclusions A close working relationship between the pathologist and the gastroenterologist at a regional centre, in partnership with the pathologists and hepatologists at super-regional centres, delivered effective care.

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