Scientific Memoirs by
It generally begins towards the end of the first year, but in some children the
enlargement of the liver has been detected as early as the third month, and in
others it does not begin until the end of the second year.
The onset is insidious, unmarked by symptoms likely to attract the attention
of the parents. In most cases the disease is not discovered until the liver has
attained a considerable size. For example, a child was brought to the Medical
College Dispensary by its grandfather, an intelligent man, who stated that up to
a month previous it was quite well. It then had an attack of fever for which
it was taken to a medical practitioner, who found the liver to be greatly enlarged.
It is evident that this must have existed for a considerable time, although the
child appeared to the parents to be in good health. This child died three months
after the disease was detected, which is about the average duration. The symp-
toms are : painless enlargement of the liver ; the child becomes languid and fretful,
having at first a voracious appetite, but after a time refusing food. Throughout
the whole course of the disease constant thirst is present. Fever may be present,
though usually absent, except in those cases in which the spleen is enlarged.
The hands and feet have a burning sensation even when no rise of temperature
can be detected by the thermometer. Enlargement of the spleen takes place in
most cases, either simultaneously with that of the liver or later. The bowels are
constipated, the stools white and clayey, especially towards the termination of
the case. The liver enlargement goes on, and the organ reaches an enormous
size ; for example, in case 5 it filled the whole of the upper part of the abdomen,
the right lobe reached within half an inch of the crest of the ilium, the left lobe
filled the left hypochondrium. Many children die without exhibiting any other
symptoms, the immediate cause of death being fever or some other intercurrent
affection, but in most cases they survive to a later period, and, in these, the liver
diminishes in size, contracting gradually, and jaundice appears, at first slight, but
becoming daily deeper. Oedema, beginning in the feet and gradually extending
upwards to the abdomen, in which sometimes a considerable quantity of fluid
collects, comes on towards the end. Death is generally preceded by symptoms
of cholmia. I have not atempted to give a f ullaccount of the symptoms, but
merely to indicate the principal features of the disease. Of the five cases upon
which the following description of the morbid anatomy of the disease is based,
t he fifth died in the stage of enlargement without signs of jaundice or oedema ; in
the other four, the livers, though larger than normal, had contracted ; jaundice and
general oedema were present. I shall consider case 5 separately, after describ-
ing the changes in the advanced stage as shown by the other four specimens,
In the first place it is to be noted that none of the other organs exhibited
any definite alterations from the normal, except the kidneys, which in the first four
cases showed marked degeneration and shedding of the epithelium of the tubules.
In case 5 the kidneys were perfectly healthy. It is probable that these organs
are not affected until the destruction of the hepatic cells is extreme.
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