Prevention Of Acute Attacks Research Articles

Current Gout Management Guidelines

Only 20 % of patients have hyperuricemia implemented in gout as a clinically defined disease. In Madrid, at the Congress of the European League Against Rheumatism (EULAR) in 2013, it was concluded that gout is two different diseases, one of which is metabolic and manifests as hyperuricemia (also called “chemical gout”), and the other the gout component is actually a typical inflammation of the joints of the favorite localization due to the deposition of sodium monourate crystals in the tissues. That is, gout is always hyperuricemia, but hyperuricemia is not always gout.The main principles of gout management are the treatment of acute gout attack with colchicine, non-steroidal anti-inflammatory drugs, glucocorticosteroids, and combinations thereof; further prevention of acute attack with minimal doses of the aforementioned drugs; long-term lifesaving therapy. A non-purine selective xanthine oxidase inhibitor with potent hypouricemic action and evidence-based febuxostat is thought to be 2–3 times more effective than allopurinol.

Evaluation of familial mediterranean fever patients: a single center experience

Familial Mediterranean Fever (FMF) is an autosomal recessive autoinflammatory disease due to mutations in MEFV, and characterized by recurrent acute attacks of fever and serosal inflamation. The disease mainly affects populations from the Mediterranean basin, especially Arabs, Turks, Jews, and Armenians. The diagnosis of the disease relies on clinical criteria, family history, and ethnic considerations, and genetic analysis of known mutations. Standart therapy for the prevention of acute attacks and also disease-related amiloidosis is colchicine. Valid therapeutic alternatives are anti-IL-1 agents in unresponder or noncompliant patients.

Acute Intermittent Porphyria—A Diagnostic Dilemma

Porphyrias are metabolic diseases resulting from a partial deficiency of an enzyme in the heme biosynthetic pathway. AIP (deficiency of hydroxymethylbilane synthase) causes acute attacks due to secondary accumulation of heme precursors. It presents with abdominal pain, tachycardia, hypertension, hyponatremia, seizures, motor neuropathy, etc. Screening is done with qualitative urinary PBG and ALA. Urinary and fecal fractionated porphyrins and DNA testing provided the confirmation. Acute attacks are treated with IV hemin and glucose. Prevention of acute attacks is with smoking cessation and avoidance of inciting agents. A 11-year-old undernourished girl, on multiple antiepileptics, for 4 years (started for refractory seizures), presented with complaints of recurrent generalized abdominal pain with weight loss since the past 1 year, with constipation and poor oral acceptance since 10 days. Examination revealed tender epigastrium, decreased air entry on left side of chest, generalized muscle wasting, and paresthesias. Investigations revealed Hb 9 g/dl; TLC 8200/cu mm (77/ 20/01/02); platelets 1,84,000/cu mm; PS-normocytic normochromic; Na 131 meq/L; K 4 meq/L; Ca 8.9 mg/dl; PO4 3.3 md/dl; ALP 255 U/L; blood urea 49 mg/dl; serum creatinine 0.9 mg/dl; bilirubin 0.5 mg/dl; SGOT 54 U/L; SGPT 21 U/L; blood culture—no growth; HIV-NR; urine examination— normal; serum amylase 112U/L; serum lipase 71U/ L; lipid profile, carbamazepine level, and CXR—normal; PPD test –NR; GA for AFB—negative; USG abdomen, CECT abdomen, and brain—normal; CECT thorax-opacities and necrotic patches on left lower zone consistent with pulmonary tuberculosis; EEGpartial epilepsy with focus at right temporal lobe; serum lead level—normal; Urine for porphobilinogen, 5-ALA, and total porphyrins—positive; and NCV— Severe axonal motor and sensory neuropathy in all four limbs. A diagnosis of AIP with pulmonary tuberculosis with partial seizure with undernutrition was made. Antiepileptics were tapered gradually and gabapentine with modified ATT was started and the child responded very well. AIP should be kept in the differentials of chronic abdominal pain and patients with psychological symptoms.

Evaluation of the Mean Platelet Volume and Red Cell Distribution Width in FMF: Are They Related to Subclinical Inflammation or Not?

In this paper we want to demonstrate whether higher than normal levels of RDW, and lower than normal levels of MPV can be used as indicators of subclinical inflammation and tools for treatment decision in FMF or not. The participants in this study included 89 patients with FMF during attack-free periods and 30 healthy controls. The RDW and platelet counts were significantly higher, while the MPV was significantly lower in the patients with FMF group than healthy control group (P < 0.001; P = 0.005; P < 0.001, resp.). In the attack-free FMF group, a negative correlation was found between the MPV and RDW values (P < 0.001, r = −0.40). The positive correlation was found between the RDW and ESR (r = 0.23, P = 0.028). And the negative correlation was found between the MPV and CRP (r = −0.216, P = 0.042). Consequently, our results suggest that low MPV and high RDW levels may provide additional information about subclinical inflammation in FMF patients. But other strong predisposing factors affecting subclinical inflammation in FMF should be considered. Further studies with large numbers of patients are needed. Treatment of FMF should include not only prevention of acute attacks but also decreasing of the subclinical inflammation.

Clinical and pathological aspects of filarial lymphedema and its management.

Lymphatic filariasis, transmitted by mosquitoes is the commonest cause of lymphedema in endemic countries. Among 120 million infected people in 83 countries, up to 16 million have lymphedema. Microfilariae ingested by mosquitoes grow into infective larvae. These larvae entering humans after infected mosquito bites grow in the lymphatics to adult worms that cause damage to lymphatics resulting in dilatation of lymph vessels. This earliest pathology is demonstrated in adults as well as in children, by ultrasonography, lymphoscintigraphy and histopathology studies. Once established, this damage was thought to be irreversible. This lymphatic damage predisposes to bacterial infection that causes recurrent acute attacks of dermato-lymphangio-adenitis in the affected limbs. Bacteria, mainly streptococci gain entry into the lymphatics through 'entry lesions' in skin, like interdigital fungal infections, injuries, eczema or similar causes that disrupt integrity of skin. Attacks of dermato-lymphangio-adenitis aggravates lymphatic damage causing lymphedema, which gets worse with repeated acute attacks. Elephantiasis is a late manifestation of lymphatic filariasis, which apart from limbs may involve genitalia or breasts. Lymphedema management includes use of antifilarial drugs in early stages, treatment and prevention of acute attacks through 'limb-hygiene', antibiotics and antifungals where indicated, and physical measures to reduce the swelling. In selected cases surgery is helpful.

Evidence for humoral autoimmunity in neuromyelitis optica

Neuromyelitis optica (NMO) is an idiopathic central nervous system (CNS) demyelinating syndrome that may be distinguished from typical multiple sclerosis (MS). Although the cause of the disorder is not known, several lines of evidence suggest that the fundamental immunological process is driven by humoral mechanisms. These observations include the frequent coexistence of systemic autoimmune disease or positive serum autoantibodies with NMO, immunopathologic studies that demonstrate prominent complement activation and immunoglobulin deposition and the discovery of the serum autoantibody NMO-IgG, a potential NMO biomarker that targets aquaporin-4. Furthermore, clinical experience suggests that plasmapheresis and immunosuppressive therapies are beneficial for treatment and prevention of acute attacks but that standard MS immunomodulatory drugs may not alter the course of NMO. This evidence is reviewed in the context of its implications for future laboratory and clinical research in NMO.

Tumor necrosis factor-alpha and interleukin-1beta levels in recurrent and persistent otitis media with effusion.

Based on interleukin (IL)-1beta and tumor necrosis factor (TNF)-alpha levels in effusions, our goals were to specify either recurrent or persistent otitis media with effusion (OME) is a mid stage in the development of chronic disease and to identify the factors that have an influence on cytokine levels. Samples from groups with recurrent (n = 15) and persistent (n = 39) OME were assayed for IL-1beta and TNF-alpha. Children were also grouped with respect to age, sex, quality of effusion, and the presence of pharyngeal adenoid tissue. Tertiary referral center. In recurrent and persistent OME groups, IL-1beta was higher than TNF-alpha (P < 0.01). IL-beta was higher in recurrent OME than in persistent OME (P < 0.05). Recurrent OME seems to be closer to the chronic stage of the disease relative to persistent OME in terms of higher IL-1beta levels. Each exacerbation of acute disease in recurrent otitis media is likely to be mediated by IL-1beta. We were able to clarify that recurrent OME is a stage that occurs before chronic OME. Therefore, the prevention of acute attacks in recurrent disease would also impede long-term damage to the middle ear.

Mutation Screening by Denaturing Gradient Gel Electrophoresis in North American Patients with Acute Intermittent Porphyria

Acute intermittent porphyria (AIP) is a dominantly inherited disorder of heme metabolism caused by a 50% decrease in the activity of porphobilinogen deaminase (EC 4.3.1.8.). The estimated prevalence of the disease is 1:1000 to 1.5:100 000, making it the most common of the neurologic porphyrias (1). Although its penetrance is only 10–20% (2), acute attacks of AIP are disabling and can be life-threatening. Prevention of acute attacks is the most important principle in managing the disease; however, it requires prior identification of individuals at risk. Because AIP has a genetic basis, molecular analysis for this disease is ultimately the most reliable way to identify gene carriers. More than 130 mutations in the porphobilinogen deaminase gene have been characterized to date (3). The majority have been restricted to a single family, or at most a few families, and no mutation accounts for more than a small percentage of all cases. With the exception of selected geographic areas where single mutations predominate (4)(5), testing for known mutations in a patient who has not previously been investigated is likely to be negative. Molecular analysis therefore requires an efficient strategy for finding the causative mutation. Denaturing gradient gel electrophoresis (DGGE) has been used by several groups to screen the exons and flanking intronic segments of the porphobilinogen deaminase gene for variations in the wild-type sequence (6)(7)(8)(9)(10)(11)(12)(13). This work has been carried out in European laboratories, and the mutations that have been discovered reflect the molecular defects within that population. By contrast, relatively few mutations have been described …