Abstract

Four infants with prolonged cholestasis, of differing aetiologies, developed metabolic bone disease during infancy. Radiologically three distinct patterns of skeletal abnormality were found: classical vitamin D deficiency rickets; axial skeleton demineralization with rib fractures and rickets; and in one patient, long term defective remodelling of tubular bones. Variations in the age of onset of rickets may be related to the baby's gestational maturity at birth, while its severity is probably more closely linked to the interval before vitamin therapy was commenced. In such infants increased supplements of prophylactic vitamin D are recommended.

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