PAEDIATRIC RESEARCH SOCIETY ABSTRACTS

12–84 μg/g). There was no evidence of significant iron deposi-tion, nor did he have evidence of fatty liver.

Further autoantibody profiling (Institute of Liver Studies, King’s College London) for antibodies implicated in cryptogenic auto-immune hepatitis demonstrated negative anti-liver cytosol type 1 (anti-LC1) and anti SLA/LP antibodies. On the basis of these results, a diagnosis of seronegative autoimmune hepatitis was made. He was commenced on 40 mg prednisolone, with a subse-quent improvement in his liver function. This improvement was not sustained, which necessitated the introduction of azathioprine and finally mycophenolate mofetil before normalisation of LFTs.Conclusions: It has been proposed that a GFD is ‘protective’ against the development of autoimmune disease, but this and other cases described in the literature demonstrate that autoim-mune hepatitis can occur after GFD has been commenced – in our case, the ALT changes were seen a year after gluten had been discontinued and having previously been normal. By using screening LFTs, we may well have avoided this patient develop-ing cirrhosis, liver failure and subsequent liver transplant.

Diabetic dwarfism: is it history?

H Dunne, V AlexanderTayside Children’s Hospital, Dundee

Case history: A 14-year-old boy was diagnosed with type 1 dia-betes aged 7. He presented in severe diabetic ketoacidosis (DKA) and subsequently had very poor glycaemic control, with eight admissions in DKA and escalating insulin requirements. His HbA1c was consistently between 10 and 14%.

At age 13 years, during a further admission with DKA, he was found to have abdominal distension and hepatomegaly. His growth had also slowed significantly from a height on the 90th centile at the age of 9 years to the 10th centile on this admission. He was prepubertal, and his weight had dropped from the 90th centile to the 75th in the same time. His HbA1c was 11.2%. Liver function tests were deranged: AST 122, ALT 101, GGT 472, ALP 242, cholesterol 3.3, triglycerides 4.05, coagulation normal. Liver ultrasound scan showed a grossly enlarged liver with diffuse echo-genicity consistent with hepatic steatosis. Extensive hepatic work-up revealed no obvious cause of the underlying liver disease. He received inpatient insulin therapy and achieved normoglycaemia with standard doses of insulin. As he had poorly controlled diabe-tes, decreased height velocity with short stature, delayed puberty and hepatomegaly, a diagnosis of Mauriac syndrome was made.

There have been significant problems in reviewing this boy since diagnosis despite intensive multidisciplinary input. This culminated in a child protection network meeting in December, and a referral has been made to the Children’s Reporter. Mau-riac syndrome is a condition that occurs as the result of chronic poorly controlled diabetes. It leads to an enlarged liver due to glycogen deposition, short stature and delayed puberty. Interest-ingly, the initial hepatic ultrasound suggested hepatic steatosis – a condition more commonly associated with obesity/diabetes.Conclusions: There is currently very little in the literature on Mauriac syndrome as it is thought of as a historical condition in the advent of more flexible insulin regimens and improved multidisciplinary input. Theories vary on the pathogenesis: some believe it is due to growth hormone resistance, others that it is an exaggeration of the expected metabolic consequences of poorly controlled diabetes. Improved insulin delivery usually results in normalisation of growth but can produce a rapid deterioration of retinopathy and nephropathy if too aggressive.

Acute abducens nerve palsy

S Sudhakaran, I Abu-ArafehStirling Royal Infirmary, Stirling

Case history: A10-year-old boy presented with a 1-week his-tory of headache, vomiting on several occasions, and a sudden onset of double vision and head tilt to the right side. His past history was significant for recurrent ear infections and a recent episode of ear infection treated with antibiotics 3 weeks prior to presentation.

On examination, he had obvious right-sided torticollis and a bilateral erythematous tympanic membrane. Right lateral gaze palsy was evident, while the rest of the CNS examinations were within normal limits. Blood investigations showed a raised C-reactive protein of 116. Right abducens nerve palsy was confirmed on visual field assessment by Hess screen chart. An urgent CT scan showed a normal brain structure, and there was no evidence of any mass lesions. Lumbar puncture attempts were unsuccessful.

A non-contrast magnetic resonance imaging (MRI) scan of the head and neck confirmed normal brain structure and showed prominent bilateral middle ear effusions, opacification of the right petrous apical air cells and an incidental finding of thora-cocervical syrinx. Contrast MRI of the head and spine confirmed the above finding and also indicated dural involvement adjacent to the right petrous tip. It also revealed a distended thoracocervi-cal syrinx and a probable cavernous haemangioma.

Treatment was initiated with intravenous ceftriaxone, and was continued for 2 weeks. Due to the uncertain aetiology, aci-clovir was also started but was stopped after 5 days. The boy

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was regularly reviewed by the ENT and ophthalmology teams, and they agreed with the above management. On follow-up examination, he still had persistence of right gaze palsy but complete resolution of his other symptoms. Repeat MRI showed an improvement in his petrous bone opacification. The final diagnosis was that of acute abducens nerve palsy secondary to petrous apicitis, a complication of otitis media.

© 2007 Published by Elsevier Ltd.