Veno-occlusive liver disease: a case report

A case of veno-occlusive liver disease (VOLD) in a 12-years old Ethiopian boy is described The salient clinical features and gross and microscopic examination of biopsy material are reviewed. Veno-occlusive disease which occurs in the West Indies, East and West Africa, and India is an acute, subacute or chronic condition that affects the central and sublobular hepatic veins. In the West Indies (1) it is related to the consumption of bush tea made from plants that contain toxic pyrrolizidine alkaloids, such as Crotalaria and Senecio (2). Hepatotoxic compounds in Crotalaria, Senecio, Heliotropium and other composite plants can also enter the diet through the contamination of cereals with weed seeds. For example 28 of 67 patients died with veno-occlusive disease in central India after consuming a local cereal, gondli contaminated with the seeds of Crotalaria (3). Heliotropium Popovii has been implicated in outbreaks in villages in northwestern Afghanistan, with high mortality (4). The primary pathological change of hepatic veno-occlusive disease is sub-endothelial edema followed by intimal growth of connective tissue, with narrowing and occlusion of the central and sub-lobular hepatic veins. Atrophy or necrosis of liver cells, with consequent fibrosis leads to gross changes similar to those seen in cardiac cirrhosis, portal hypertension results. The present report, the first of it kind in Ethiopia describes a case of veno-occlusive liver disease in a 12-year old Ethiopian boy.     

Incidence and Clinical Characteristics of Ocular Involvement in Mucous Membrane Pemphigoid

Purpose: To describe the risk of developing ocular mucous membrane pemphigoid (MMP) or a new extraocular site of MMP, and to identify risk factors for new involvement.

Methods: Retrospective chart review of 162 biopsy-proven MMP patients.

Results: At presentation, 109 of 162 MMP patients (67.3%) had ocular involvement and 53 patients did not. Of the 53 patients without ocular involvement at presentation followed up to 22 years, the risk of developing ocular MMP was 0.014 per person-year (PY, 95% confidence interval [CI]: 0.005/PY, 0.034/PY). The risk of developing any new location of extraocular MMP was 0.020/PY (95% CI: 0.007/PY, 0.043/PY). Smoking was a risk factor for developing an additional extraocular MMP location (hazard ratio [HR] = 4.09, p = 0.04).

Conclusions: Patients presenting with extraocular MMP are at risk for developing ocular MMP, and all MMP patients are at risk for developing secondary extraocular MMP locations, although the rates were low.     

Unmet service needs of persons with traumatic brain injury

OBJECTIVES: Assess unmet needs of persons with traumatic brain injury (TBI) 1 year after hospital discharge; compare perceived need with needs based on deficits (unrecognized need); determine major barriers to services; evaluate association of needs with satisfaction with life. PARTICIPANTS: Representative sample of 1830 community-dwelling persons with TBI aged 15 years and older. MEASURES: Perceived and unrecognized unmet needs, barriers to receiving services, and satisfaction with life as a function of met service needs. RESULTS: 35.2% of participants reported at least 1 unmet need, 51.5% had unrecognized needs, 47% reported at least 1 barrier to receiving help. Receipt of services significantly increased satisfaction with life. CONCLUSIONS: Many persons experiencing TBI report having unmet service needs 1 year after hospital discharge.     

Tumor angiogenesis is accompanied by a decreased inflammatory response of tumor-associated endothelium

We previously showed that endothelial cells (EC) from the vasculature of human solid tumors have a decreased expression of intercellular adhesion molecule-1 (ICAM-1) and ICAM-2 as compared with normal tissue EC. This effect is explained by EC exposure to angiogenic factors. It is known that upregulation of endothelial adhesion molecules (EAM) is a sign of EC activation in inflammatory responses. We therefore tested the effect of angiogenic factors on upregulation of EAM on tumor EC and human umbilical vein EC (HUVEC) by proinflammatory cytokines. Incubation of tumor-derived EC in tumor necrosis factor alpha (TNF alpha) did result in expression levels of only 20% of the level of similarly treated normal tissue-derived EC. Pretreatment of HUVEC with 10 ng/ml basic fibroblast growth factor (bFGF) for 3 days, before TNF alpha- or interleukin-1 alpha (IL-1 alpha) stimulation, resulted in ICAM-1 levels of only 30% to 60% of cells without pretreatment. Also, the induction of vascular EC adhesion molecule-1 (VCAM-1) and E- selectin by TNF alpha was significantly inhibited by prior exposure to bFGF. Vascular endothelial growth factor had similar but less prominent effects. The effect of transforming growth factor-beta and IL-8 was studied as well. The functional relevance of the finding of a decreased EC inflammatory response was confirmed by adhesion assays. Our results show that tumor angiogenesis induces EC anergy. This may serve as a tumor-protecting mechanism by impairing the development of an efficient leukocyte infiltrate in tumors.     

Abnormal neutrophil phenotype and neutrophil FcRIII deficiency corrected by bone marrow transplantation

BACKGROUND : Neutrophils from a patient in first remission of acute myeloid leukemia were found to lack NA1 and NA2 alloantigens. This NA null phenotype was converted to the normal phenotype of NA1, NB2 by the transplantation of bone marrow from an HLA-identical sibling. To investigate the inherited or acquired nature of this rare phenotype, a combination of conventional neutrophil serology and recently developed restriction fragment length polymorphism (RFLP) and polymerase chain reaction (PCR) assays was used. STUDY DESIGN AND METHODS : Diagnosis, remission, and posttransplant patient peripheral blood samples were used for neutrophil phenotyping by granulocyte agglutination and immunofluorescence tests. The presence and dose of the gene for neutrophil Fc gamma RIIIb (Fc gamma RIIIB) were tested for with RFLP and Southern analysis and PCR-based RFLP tests. Plasma levels of circulating soluble Fc gamma RIII (sFc gamma RIII) were measured with radioimmunoassay. The sibling bone marrow donor and the patient's parents were also studied. RESULTS : RFLP analysis of DNA obtained from the patient at the time of diagnosis showed that she lacked the Fc gamma RIIIB gene for neutrophil Fc gamma RIII (i.e., Fc gamma RIIIb), but that, in DNA prepared from posttransplant samples, the Fc gamma RIIIB gene was present. Quantitation of plasma levels of soluble FcRIII (sFcRIII) demonstrated a complete absence of sFcRIII in the patient's pretransplant plasma. However, 20 units of sFcRIII were detected in the patient's plasma by 160 days after graft. Hair samples from the patient provided sufficient nonhematopoietic, genomic DNA to confirm that her genotype was NA0NA0. DNA prepared from lymphocytes of both parents and the sibling marrow donor was used to quantitate their Fc gamma RIIIB gene dose. The mother and brother had only one Fc gamma RIIIB gene each, while the father apparently had a normal complement of two Fc gamma RIIIB genes. CONCLUSION : In this case, an inherited absence of Fc gamma RIIIB gene in a patient with acute myeloid leukemia was unintentionally corrected by the transplantation of bone marrow from a sibling donor who himself carried only one Fc gamma RIIIB gene.