IIlizarov external fixator for bilateral severe flexion deformity of the knee in haemophilia: case report

Introduction  

Deformity correction by Illizarov method in haemophilic patients is a rare phenomenon.     

Crystal identification of synovial fluid aspiration by polarized light microscopy. An online test suggesting that our traditional rheumatologic competence needs renewed attention and training

Testing a reading exercise for identification of several typical crystal such as the negatively birefringent needle-shaped crystals that are under polarized light microscopy is the gold standard for diagnosing gout. The objective of this study was to assess current performance of crystal identification by professionals involved in examining synovial fluid in routine care. Rheumatologists, trainees, lab technicians, and other physicians with an interest in crystal arthritis completed an online test. The test consisted of 30 images: 8 monosodium urate (MSU) crystals, 5 calcium pyrophosphate (CPP), 4 cholesterol, 2 depot methylprednisolone, 2 calcium oxalate, 2 rice bodies, 1 hydroxyapatite, 1 liquid lipid, 1 fibrin, 1 Charcot-Leyden, and 5 different artifacts. Of the 22 non-MSU slides, a subset of 8 was pre-designated that were thought to be clinically important to be identified as non-MSU. The primary outcome was defined as the correct identification of all eight MSU slides plus the identification of all eight pre-defined non-MSU slides as non-MSU. The online test was completed by 110 participants. The primary outcome was achieved by 39%. Correct identification of all MSU images was achieved by 81%, correct identification of all 8 pre-defined non-MSU, CPP images, and all 22 non-MSU images as non-MSU by 68, 68, and 23%, respectively. MSU crystals were well identified, but incorrect identification of non-MSU crystals occurred frequently. This study suggests that there is room for improvement regarding crystal identification of particularly CPP and other non-MSU crystals even in this highly motivated group.     

Serological study for sexually transmitted diseases in patients attending STD clinics in Calcutta

Total 457 patients attending different STD clinics of Calcutta were studied for serological tests for STD. TPHA positivity was maximum (18.60%), followed by chlamydia infection (15.97%), VDRL reactivity (8.98%), HIV infection (6.35%), and HBs Ag (3.72%). Total 37.20% samples were positive for one or more infection. Out of these, one, two, three and four tests positivity was seen in 65.29%, 25.88%, 8.24% and 0.59% respectively. The age group which had maximum infection (20.13%) was 15-30 years.     

Interplay of IKK/NF-kappaB signaling in macrophages and myofibers promotes muscle degeneration in Duchenne muscular dystrophy

Duchenne muscular dystrophy (DMD) is a lethal X-linked disorder associated with dystrophin deficiency that results in chronic inflammation and severe skeletal muscle degeneration. In DMD mouse models and patients, we find that IkappaB kinase/NF-kappaB (IKK/NF-kappaB) signaling is persistently elevated in immune cells and regenerative muscle fibers. Ablation of 1 allele of the p65 subunit of NF-kappaB was sufficient to improve pathology in mdx mice, a model of DMD. In addition, conditional deletion of IKKbeta in mdx mice elucidated that NF-kappaB functions in activated macrophages to promote inflammation and muscle necrosis and in skeletal muscle fibers to limit regeneration through the inhibition of muscle progenitor cells. Furthermore, specific pharmacological inhibition of IKK resulted in improved pathology and muscle function in mdx mice. Collectively, these results underscore the critical role of NF-kappaB in the progression of muscular dystrophy and suggest the IKK/NF-kappaB signaling pathway as a potential therapeutic target for DMD.     

Modulation of amyloid fibrillation of bovine β-lactoglobulin by selective methionine oxidation

Deposition of oxidation-modified proteins during normal aging and oxidative stress are directly associated with systemic amyloidoses. Methionine (Met) is believed to be one of the most readily oxidisable amino acid residues of protein. Bovine beta-lactoglobulin (β-lg), a model globular whey protein, has been presented as a subsequent paradigm for studies on protein aggregation and amyloid formation. Herein, we investigated the effect of t-butyl hydroperoxide (tBHP)-induced oxidation on structure, compactness and fibrillation propensity of β-lg at physiological pH. Notably, whey protein modification, specifically Met residues, plays an important role in the dairy industry during milk processing and lowering nutritional value and ultimately affecting their technological properties. Several bio-physical studies revealed enhanced structural flexibility and aggregation propensity of oxidised β-lg in a temperature dependent manner. A molecular docking study is used to predict possible interactions with tBHP and infers selective oxidation of methionine residues at 7, 24 and 107 positions. From our studies, it can be corroborated that specific orientations of Met residues directs the formation of a partially unfolded state susceptible to fibrillation with possible different cytotoxic effects. Our studies have greater implications in deciphering the underlying mechanism of different whey proteins encountering oxidative stress. Our findings are also important to elucidate the understanding of oxidation induced amyloid fibrillation of protein which may constitute a new route to pave the way for a modulatory role of oxidatively stressed proteins in neurological disorders.

This work reports selective methionine oxidation of β-lactoglobulin by tBHP reduces its thermal stability and enhances fibrillation propensity.