No abstract

A B S T R A C T MNlost p)atiellts witlh stable cirrhosis of the alcoholic have "target" red cells; however, a minoritv have "spur" cells and severe hemolytic anemia. These two syndromes were studied in 27 patients with target cells and 17 patients with spur cells, all of whom had advanced cirrhosis. The cholesterol and phospholipi(d content of red cell membranes effectively distinguished target cells from spur cells. Target cells alone were rich in lecithin, and both the cholesterol/phospholipid and cholesterol/lecithin mole ratios were greater in spur cells. The cholesterol/phospholipid mole ratio of both types of red cells correlated closely with the free cholesterol saturation of serum lipoproteins, as defined by the anmount of free clholesterol relative to phospholipid and protein in these lipoproteins. Lecithin: cholesterol acyltransferase (LCAT) activity was decreased in most patients with target cells and spur cells; however, the relationship between this activity and the lipid abnormiialities observed was weak. Serum bile acid levels also correlated poorly witlh serum and cell lipids. However, in patients with target cells the amount of cholic and (leoxycholic acids in serumi was approximately equal to the amount of chenodeoxycholic acid, whereas in patients with spur cells chenodeoxycholic acid (the precursor of lithocholic acid) pre(lominatedl.

Serologic tests for antineutrophil antibodies were used to determine if autoantibodies cause neutropenia. The serums of five patients with idiopathic neutropeniaopsonized normal neutrophils, causing them to be ingested by rabbit macrophages or else to activate glucose oxidation rates of other normal neutrophils by at least twice the rate of controls. Some of the serums inhibited the ability of normal neutrophils to ingest by 62 to 56 per cent. At splenectomy in two of the patients splenic macrophages contained ingested neutrophils, suggesting that the opsonic activity of the serum demonstrated in vitro had pathogenetic importance. In two adults, and possibly in an infant, corticosteroids raised the neutrophil count, although antibody activity remained in the serum of the adults. The findings indicate that autoantibodies are the basis of some cases of idiopathic neutropenia, and that they act by promoting the clearance of neutrophils by mononuclear phagocytes.

Caucasians with prevalence estimates of 2 to 8 cases per The prevalence of homozygous hereditary hemochro-1,000 population. [4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21] Approximately 70% of affected persons matosis (HHC) is estimated at 1:250 in Caucasian adults.possess HLA-A3 alloantigen. 5,[22][23][24][25][26][27][28] Simon et al. hypothesize Little is known about ethnic subpopulations that might that the genetic mutation leading to iron overload originally be at increased risk for this disease. HLA data have sugoccurred in the Celtic peoples and that the HLA marker for gested a Celtic origin for HHC. Screening for HHC was HHC might constitute a genetic tracer of the migratory patoffered to all employees of the Massachusetts Polaroid tern of these peoples. 29 More recently, a non-HLA-linked Corporation. Participants with a transferrin saturation form of iron overload has been described in South African of ú55% or ú45% and an elevated serum ferritin concenblacks. 30 tration on two screenings were referred for liver biopsy.Most studies have dealt with populations from ethnically The diagnosis of HHC was based on histological criteria, homogeneous areas (Table 1). [9][10][11][12][13][14][15][16][17][18][19][20][21]31 Detailed comparisons of quantitative hepatic iron determination, hepatic iron inethnic and racial backgrounds have not been reported. The dex, and the phlebotomy requirement for iron depletion.current study examines the prevalence of HHC in a healthy Participants completed a questionnaire regarding their working population of heterogeneous ethnic and racial backethnic background. Two thousand two hundred ninetyground. We sought to identify ethnic and racial subpopulafour employees were screened, and 5 cases of HHC were tions that might be at high risk for inheriting this disease. tion of HHC with Celtic background (P Å .012). The esti-Polaroid study of prostate-specific antigen values in male employees mated cost of screening per patient identified was over age 50 years, and an additional 1,331 nonduplicate serum sam-$18,041. Polaroid Corporation has a high representation ples were collected through a corporation-wide informational and of employees of British-Irish ancestry. Our data suggest promotional campaign offering free screening for HHC. This included that they are at high risk for developing HHC. A signifi-a telephone hotline outlining the signs and symptoms of HHC and cant association of HHC with Celtic ancestry was found the benefits of early detection. Demographic data for the total Polarin this subpopulation, supporting the concept of a Celtic oid population were made available for comparison ( HLA-A and -B phenotyping of T-lymphocytes was performed on TS, transferrin saturation; HII, hepatic iron index; SF, serum ferritin.probands in anticipation of family screening. 43,44 From the 1 Division of Gastroenterology, Faulkner Hospital, Tufts University School of Medicine, Boston, MA; 2 Medical Department, Polaroid Corporation, Cambridge, MA;Protocol. The initial sc...