AMYLOIDOSIS

a セ i B y l o i d diseaseis known also as lardaceousor waxy disease. The term "amyloid" is not apt, as the substancecontainsnitrogen and is more allied chemically to albumin or gelatin than to starch. It has been termed lardacein-it is not a simple degeneration product and affected organs are enlarged.It follows prolongedsuppurationin conditions of chronic sepsis, syphilis, tuberculosis of bones, joints and lungs and empyema;but it also occurs in syphilis without suppurationand in the cachexiaattendingsomeforms of malignantdisease. How theseconditionsact is not known. It is seen most often in the spleen,kidneys, liver, intestines, stomach and panacreas and, with diminishing frequency, in the suprarenals, lymph glands. thyreoid gland, aorta, ovariesand uterus. Amyloid tumoursof the larynx are rare. They may be a local manifestationof amyloidosis or may replace preexisting tumour cells. Such tumours may be mistaken for malignant growths or syphilitic gummata. Amyloid disease may be seen in the conjunctiva, generally, but not always, following trachoma. Adamiik regards the material as a depositbetweenthe connectivetissuecells and fibres and not a product of cell degeneration. In general the tissuesinvadedare firstly the walls of the blood vessels,especially the inner and middle coats with the muscular fibres; secondly, connective tissues and lastly, if at all, the glandcells of an organ. The organsaffectedareof firm consistence.The amyloid substance is firm, colourless and translucent, resisting putrefaction and chemical action. It is insoluble in dilute acids and very slightly soluble in gastric juice at body temperature. With some colouring agents it gives special reactions. With iodinea rich brownish-redor claretcolour is formed, changed to dark purple on the addition of dilute sulphuricacid. Methyl violet gives a red or bluishpink tint, the surrounding healthy tissue being stainedblue. When the spleenis affectedit is large and hard, with a typical "sago spleen" appearance. An affected liver becomes greatly enlarged, but painlessand not tender. There is no jaundiceand if ascitesand anasarcaexist, they are attributedto coexisting kidney disease. A slight amount of amyloid diseasemay be seen in chronic nephritis 'apart from profusesuppurationor cachexia. E. G. Bannick, J. :1!. Berkmanand D. G. Beaver have described three unusual cases of diffuse amvloidoais.! The first case was one of lymphoウ 。 イ セ ッ ャ ャ ャ 。 of Hodgkin's type with healed duodenal ulcer and pseudomembranousentero-colitis. The spleen,kidneys, liver, suprarenals,ileum and colon were the sites of amyloidosis. The lymphosarcoma was probably the basis of the amyloidosis. The diagnosis of the amyloidosis was correctly made after various forms of nephritis and nephrosiswere excluded. Bence-Jones proteinuria was also excluded, but the result of the congo red test for amyloid diseasewas positive and hepatic function tests indicated extensiveliver implication. In the second case autopsy revealed acute エ オ 「 ・ イ 」 オ ャ ッ セ ウ degenerationof the kidneys and healedtuberculosis of the hilar glandsof the lungs with atherosclerosis and cholelithiasis. There was amyloidosisaffecting the spleen, liver, suprarenalglands, myocardium, lymph glands, kidneys, pancreas,thyreoid gland, thymus,lungs,stomachandotherorgans. Therewas hypertrophy of the liver and spleen with ascites, hvdrothorax and redema of the lungs. What 、 セ エ ・ イ ュ ゥ ョ ・ 、 the amyloidosiswas not certainly ascertained.No congo イ セ 、 test was employedduring life; at the autopsy it gave its characteristicreaction. The methyl violet test was only partly successful; it did not disclosethe usualdegreeof metachromatic reaction given by amyloid substance. Iodine alone or followed by sulphuric acid did not give a typical reaction. In sectionsit failed entirely and in the fresh state the gross iodine reaction gave a black or dark brown colour, but not the typical reddish brown. The diagnosisof amyloidosiswas not made. Therewas no evidenceof suppuration.The terminal illness was characterizedby renal, suprarenaland hepatic insufficiency. The diffuse hyaline amyloidlike substancedeveloped without apparent cause. Other hyaline substanceshave a markedaffinity for congo red, and in this casethere was no chemically perfect amyloid substancebut some allied or intermediatebody. The third caserevealedcarcinomaof the stomach,evidently of long standing. It was of a medullary type of carcinoma simplex, with peculiar acidophilic staining of the cytoplasm. It probablywas concernedwith the pathogenesisof the amyloidosis. Therewas alsohypoplasiaof the heart with calcareousdepositson the mitral valve, hydrothorax and slight cedema ofthe lower extremities. Amyloidosis involved the liver, spleen,kidneys and suprarenals. There was no suppuration. Diffuse amyloidosisand amyloid nephrosiswere tentatively diagnosed. Lipoid nephrosis, the nephrotic stage of chronic glomerulo-nephritisand cardiac dropsy were excluded. No congo red test could be carried out during life. At the autopsy typical staining reactionswere elicited. Bannick and his colleagues state that diffuse amyloidosiscan usually be diagnosed,if suspected. The congo red test is valuablein the diagnosis. It had previously been noted by H. Bennhold that patientswith extensiveamyloidosis,particularly of the liver, almost all gave positive tests with congo