Biomedical subjects
Publications and source records attributed to F S Buck.
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The liver is almost universally involved in systemic amyloidosis. Patterns of topographic distribution of amyloid within the liver lobule have been recognized, but the reliability of using these for classification of amyloid type is in question. We examined 286 livers from cases of systemic amyloidosis obtained from autopsies at Los Angeles County-University of Southern California Medical Center, classifying them as AL or AA type by means of the potassium permanganate Congo red-staining method along with a specific anti-AA antiserum. Prior publications have asserted that deposition of secondary (AA) amyloidosis is limited to the vessels in the portal tract, constituting a "vascular" pattern, and that in primary (AL) amyloidosis the deposits exhibit a "sinusoidal" pattern in that they are seen along hepatic sinusoids as well as in portal vessels. We confirmed that AL amyloid involves the portal vessels as frequently as AA amyloid and that deposition occurred significantly more frequently in the portal stroma, the central vein, and the "sinusoidal" areas. However, we also found a "sinusoidal" pattern in 29 of 78 cases of secondary (AA) amyloidosis; in 14 of these, more than half of the sinusoidal spaces were replaced by amyloid deposits. We also noted that in 23 of the 29 AA amyloidosis cases with "sinusoidal" involvement, a "sago" pattern of distribution of amyloid in the spleen was present. No consistent association of a specific chronic inflammatory disease with "sago" spleen and "sinusoidal" deposits could be documented. We conclude that topographic distribution of amyloid within the liver lobule is not a reliable method of distinguishing AA from AL amyloidosis and that specific staining methods must be used if the physician is to be able to attempt modern therapeutic modalities.
We examined the ethnic heritage of 467 patients with amyloidosis and related it to the type of amyloid (secondary versus other types) found among 52,370 autopsies at Los Angeles County-University of Southern California Medical Center. Classification of amyloidosis by type was accomplished by using the potassium permanganate Congo red staining method and a specific anti-AA antiserum, supplemented by the anatomical distribution of the amyloid in some instances. We discovered a statistically significant increase in amyloidosis among patients with hispanic surnames as compared with other Caucasians. The overall rate for Hispanics in our total autopsy population was 2.3% as compared with 0.6% for other Caucasians (P less than or equal to 0.001). The increase was mostly among those whose amyloid was negative to tests for secondary (AA) amyloidosis and not anatomically compatible with senile cardiac (senile systemic) amyloidosis. Hispanics accounted for 76% of these cases as compared with 18.5% for Caucasians (P less than or equal to 0.001). Our findings, along with previously published reports, suggest that the frequency of amyloidosis may vary significantly in different ethnic groups.
We report a case of heterotopic liver located in the right adrenal gland and connected to the liver through a vascular membranous pedicle. Features of chronic active hepatitis with cirrhosis were present in the heterotopic site, mirroring the abnormal histopathology in the liver.
An unusual patient with blue rubber bleb nevus syndrome is presented. At birth a single abdominal wall hemangioma was noted, and over a 21-year follow-up the full syndrome developed, including many cutaneous and intestinal lesions and anemia. Our long-term follow-up of this patient allows an understanding of the natural history of this condition, especially regarding recurrence of lesions after excision.