ABO, secretor, and Lewis carbohydrate histo-blood groups are associated with autoimmune neutropenia of early childhood in Danish patients

BACKGROUND: Autoimmune neutropenia of early childhood (AIN) is caused by autoantibodies directed against antigens on the neutrophil membrane. The ABO, secretor, and Lewis histo‐blood group systems control the expression of carbohydrate antigens and have previously been linked to autoimmune diseases. We aimed to investigate the association between genotypes and the risk of AIN in Danish patients. STUDY DESIGN AND METHODS: One hundred fifty‐four antibody‐positive AIN patients were included. Controls (n = 400) were healthy unrelated Danish blood donors. Molecular determination of ABO, secretor (FUT2), and Lewis (FUT3) genotypes were determined using real‐time polymerase chain reaction (qPCR) or Sanger sequencing to infer the prevalence of Lewis antigens (Le(a) and Le(b)) and secretor (SeSe or Sese) or nonsecretor (sese) phenotypes. RESULTS: Blood type O was more common in controls (46.8%) than in AIN patients (36.4%) (OR = 0.65; p = 0.028). Secretors of H Le(b) antigens were less frequent among AIN patients (25.2%) than controls (35.0%) (OR = 0.62; p = 0.037). DISCUSSION: ABO blood group antigens and the secretion of these antigens are associated with a diagnosis of AIN. The mechanism underlying the association between autoimmunity and interaction among ABO, secretor, and Lewis genotypes has not yet been elucidated, but several studies indicate a connection to the gut microbiota

The social situation of motherless children in rural and urban areas of Guinea-Bissau

With the increasing prevalence of HIV infection and the high maternal mortality, orphans are a rapidly growing problem in Africa. However, few studies describe the social conditions of these children. Our study focuses on motherless children in urban and rural areas of Guinea-Bissau. A rural and an urban cohort of children (128 and 192, respectively) that had been followed by demographic surveillance since 1990 were identified and the relatives of these children interviewed. A control cohort of 808 individuals was also identified. Although orphan children remained disadvantaged, there were few differences between surviving motherless and control children in nutritional status, use of health care services, school attendance, quality of housing, and clothing. Motherless children moved more frequently and were more likely to live in small families, often with an older grandmother. The traditional extended family system appears to be capable of handling motherless children in a non-discriminatory fashion. However, the AIDS epidemic will continue to stress the extended family system and social services to the limit.Guinea-Bissau Extended family system Mothers Orphanages Social conditions HIV/AIDS

The Prognostic Value of YKL-40 Concentrations in Nonmyeloablative Conditioning Allogeneic Hematopoietic Cell Transplantation

Increased plasma concentrations of YKL-40, also called chitinase-3-like-1 protein (CHI3L1), have been correlated with disease severity in a variety of malignant and inflammatory diseases. The objective of the current study was to assess pretransplant recipient and donor CHI3L1 polymorphisms and plasma YKL-40 concentrations as prognostic biomarkers in a cohort of 149 patients treated with hematopoietic cell transplantation (HCT) after nonmyeloablative conditioning for hematologic malignancies. Recipients with pretransplant YKL-40 concentrations above the age-adjusted 95th percentile (high) had higher relapse-related mortality (33% versus 18%, P = .04; hazard ratio (HR) = 4.41, P = .01), lower progression-free survival (38% versus 64%, P < .01; HR = 2.84, P = .01), and overall survival (42% versus 69%, P = .01; HR = 3.09, P = .01). Recipients transplanted with donors with high YKL-40 concentrations had an increased probability and risk of grade 2-4 acute graft-versus-host disease (aGVHD) (93% versus 62%, P < .01; HR = 2.25, P = .02). CHI3L1 polymorphisms were associated with plasma YKL-40 concentrations, but not with clinical outcomes. In conclusion, our study suggests that plasma YKL-40 could function as a biomarker for relapse risk and treatment-related toxicity, and possibly as a tool complementing clinical risk scores such as the HCT comorbidity index