Grave familial jaundice of the newly born / Sir Humphry Rolleston.

  • Rolleston, Humphry Davy, Sir, 1862-1944.
Date:
[1920?]
Catalogue details

Licence: In copyright

Credit: Grave familial jaundice of the newly born / Sir Humphry Rolleston. Source: Wellcome Collection.

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    ducts in the recorded cases except in one of McGibbon’s cases, described in detail by Buchan and Cowrie, in which there was obstruction but not obliteration of the biliary papilla. Clinical Features.—Afebrile jaundice usually appears a few hours to a day or two after birth, and increases; but, as in other forms of jaundice, may show curious variations. The infants may be born icteric; Smith describes a jaundiced stillborn child, and noticed in his three cases that the liquor amnii and membranes were bile-stained. The stools are not acholic; the urine may or may not contain bile. Haemorrhages are not a feature of the disease. In a few instances enlargement of the liver and spleen has been present. The infant becomes drowsy, and usually death occurs within the first 14 days, often during the first week, and commonly with convulsions. Recovery after prolonged drowsiness appears to be more likely to occur in the earlier infants of the family; in nearly all the reported cases of this familial jaundice the recovery has been complete; one of Morris’s cases, a girl, survived for 4| years, and was jaundiced practically all the time. Prognosis.—This is bad; out of 130 collected cases 100, or 77 per cent., proved fatal. It is possible that prophylactic treatment of the pregnant mother (vide p. 7) may improve the outlook in the future. Spiller has recorded four cases of spastic diplegia following recovery from severe jaundice in the newly-born, but none of these was stated to be of the grave familial form. In connection with this and with the selective staining (“ kernicterus ”) of the cerebral nuclei in grave familial and in physiological jaundice of the newly- born, it is interesting to recall Wilson’s comparison with the selective action of a poison, probably non- microbic and possibly a lipoid, causing bilateral degeneration of the lenticular nucleus in the disease progressive lenticular degeneration, which is constantly