The online version of this article (doi:10.1186/1752-1947-6-200) contains supplementary material, which is available to authorized users.
The authors declare that they have no competing interests.
CIE was the primary physician of the child and initiated the writing of the case. NB extracted the clinical data from the medical record. ARS performed the slit-lamp examination while FEAL made the provisional diagnosis of WD and was a major contributor to writing the case. All authors read and approved the final manuscript.
Wilson disease is rarely reported among African children. This report describes the second case report of a Nigerian child with Wilson disease in three decades.
An eight-year-old African boy presented with generalized oedema and ascites and proteinuria. Over the next three weeks he developed conjugated hyperbilirubinaemia, severe coagulopathy and prominent extrapyramidal features consisting of rigidity, tremors at rest and in action, shuffling gait, slurred speech and emotional lability. Slit-lamp examination of his eyes revealed Kayser-Fleischer rings and sunflower cataracts. His serum caeruloplasmin level was 5mg/dL. Using the scoring system proposed by the 8th International Meeting of Wilson Disease and Menkes Disease, a diagnosis of Wilson disease was made.
Wilson disease does occur in African children, although the diagnosis is rarely made. A diagnosis of Wilson disease should be entertained in the evaluation of African children presenting with liver dysfunction and/or extrapyramidal neurological features.
Authors’ original file for figure 113256_2012_2146_MOESM1_ESM.pdf
Cisse A, Morel Y, Coulibaly M, Souare IS, Cisse AF, Nabe A, Camara LM, Cisse B, Camara S, Bangoura SA, Sylla A, Dia H, Da Silva L: Wilson’s disease: study of 6 neurologic forms observed at the University Hospital in Conakry. Med Trop (Mars). 2004, 64 (1): 53-57.
Dumas M, Girard PL, Jacquin-Cotton L, Konate S: 1st case of Wilson’s disease in Senegal. Bull Soc Med Afr Noire Lang Fr. 1970, 15 (1): 96-99. PubMed
Morgan HG, Stewart WK, Lowe KG, Stowers JM, Johnstone JH: Wilson’s disease and the Fanconi syndrome. Q J Med. 1962, 31: 361-384. PubMed
Marcellini M, Di Ciommo V, Callea F, Devito R, Comparcola D, Sartorelli MR, Carelli G, Nobili V: Treatment of Wilson’s disease with zinc from the time of diagnosis in pediatric patients: a single-hospital, 10-year follow-up study. J Lab Clin Med. 2005, 145 (3): 139-143. 10.1016/j.lab.2005.01.007. CrossRefPubMed
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