Familial haemophagocytic lymphohistiocytosis (FHL) is an inherited immune deficiency with defective cytotoxicity of natural killer cells and cytotoxic T lymphocytes. A highly stimulated, but ineffective response leads to severe hyperinflammation. Clinical laboratory features are characteristic, unspecific; thus awareness FHL important for early diagnosis. rapidly fatal without treatment. Standa...

INTRODUCTION: The etiology of posterior tibial tendon dysfunction (PTTD) is unclear although it has been suggested that it may be related to a preexisting flexible flatfoot. Obesity, diabetes, and hypertension may increase the risk of PTTD according to epidemiological studies. It is uncertain how tendonopathy can severely affect the PT tendon, while adjacent tendons, flexor digitorum longus (FD...

BACKGROUND Pulmonary hypertension (PH) is a severe disease with a poor prognosis. Different forms of PH are characterized by pronounced vascular remodeling, resulting in increased vascular resistance and subsequent right heart failure. The molecular pathways triggering the remodeling process are poorly understood. We hypothesized that underlying key factors can be identified at the onset of the...

BACKGROUND Flexor hallucis longus (FHL) and flexor digitorum longus (FDL) tendon transfers are frequently used to restore the function of a deficient tibialis posterior tendon in stage II adult acquired flatfoot deformity (AAFD). Either transfer causes some loss in toe flexion force, although the decision to tenodese the cut tendon to restore associated function remains controversial. This stud...

Borrelia burgdorferi has developed efficient mechanisms for evading the innate immune response during mammalian infection and has been shown to be resistant to the complement-mediated bactericidal activity of human serum. It is well recognized that B. burgdorferi expresses multiple lipoproteins on its surface that bind the human complement inhibitors factor H and factor H-like protein 1 (FH/FHL...

The etiologic agent of Lyme disease, Borrelia burgdorferi, is capable of circumventing the immune defense of a variety of potential vertebrate hosts. Previous work has shown that interaction of host-derived complement regulators, factor H and factor H-like protein 1 (FHL-1), with up to five complement regulator-acquiring surface proteins (CRASPs) expressed by resistant B. burgdorferi sensu lato...

Familial hemophagocytic lymphohistiocytosis (FHL) is a rare autosomal recessive disorder characterized by hyperactive phagocytes and defects in natural killer cell function. It has been shown previously that mutations in the perforin 1 gene (PRF1) and in UNC13D are associated with FHL2 and FHL3, respectively, indicating genetic heterogeneity. We performed genome-wide homozygosity mapping in a l...

A skin mucus lectin exhibiting a homodimeric structure and an S-S bond between subunits of ~40 kDa was purified from flathead Platycephalus indicus (Scorpaeniformes). This lectin, named FHL (FlatHead Lectin), exhibited mannose-specific activity in a Ca(2+)-dependent manner. Although FHL showed no homology to any previously reported lectins, it did exhibit ~20% identity to previously discovered ...