China
Africa
Explore chapters and articles related to this topic
Munchausen’s syndrome and related factitious disorders
Published in David Enoch, Basant K. Puri, Hadrian Ball, Uncommon Psychiatric Syndromes, 2020
David Enoch, Basant K. Puri, Hadrian Ball
Wittkowski et al. (2017) reported an informative case report of a male child who presented with clinical signs from the age of 5 months onwards which were erroneously attributed, until the age of 3 years, to systemic autoinflammatory disease. A very large number of investigations were carried out, from blood tests and gene sequencing to bone marrow aspiration and skin biopsies; these all gave normal results. At the age of 3 years, the child was diagnosed as being a victim of MSBP, the perpetrator being his mother; the probable causes of some of the physical presenting signs were thought to be mechanical irritation (erythematous, bullous and necrotising skin lesions), addition of maternal blood (macrohaematuria) and the application of capsaicin (facial swelling).
Activation of Human Inflammatory Cells
Published in William S. Lynn, Inflammatory Cells and Lung Disease, 2019
Although the above observations clearly indicate that various inflammatory cells are capable of destroying normal tissue cells and do accumulate at the sites of tissue injury and that the products of these accumulating cells are capable of initiating and/or prolonging the observed tissue injury, it remains difficult to assess, both in human disease and in the toxin-produced responses cited above, whether the observed inflammatory response, in a given situation, is involved primarily in repair or in tissue destruction. It is clear, however, that if the destructive capability of the various inflammatory cells, including platelets and rbc is uncontrolled, tissue damage can ensue. Thus, studies on the mechanism which controls this inflammatory cell effector system are crucial if one is to understand or control their pathogenetic potential in the above “autoinflammatory” disease of man.
Unexplained fever
Published in A Sahib El-Radhi, James Carroll, Paediatric Symptom Sorter, 2017
A Sahib El-Radhi, James Carroll
Genetic causes of periodic fever syndromes have been identified in the past few years. The term autoinflammatory disease has been proposed to describe a group of disorders characterised by attacks of unprovoked systemic inflammation without significant levels of autoimmune or infective causes.
Evaluation of the relationship of lymphangiogenesis markers with disease pathogenesis in patients with Behçet’s uveitis
Published in Acta Clinica Belgica, 2022
Taner Özgürtaş, Çiğdem Yücel, Erdim Sertoğlu, Yıldız Hayran, Seda Çolak, Emre Tekgöz, Ahmet Omma, Ali Hakan Durukan
55 patients with Behçet’s uveitis according to the International Study Group criteria [30] and 30 age and sex-matched healty controls were included. Patients and controls were excluded if they had one of the following combined diseases/situations: 1) concomitant autoimmune or autoinflammatory disease; 2) acute or chronic infection; 3) malignancy; 4) systemic diseases such as diabetes mellitus and heart failure; 5) pregnancy or postpartum 6 months. Demographic features and clinical characteristics were recorded. Disease activity was assessed with Turkish version of BD Current Activity Form (BDCAF) [31]. The items of this activity include headache, oral ulcers, genital ulcers, erythema, skin pustules, arthralgia, arthritis, intestinal involvement, new eye involvement, new nervous system involvement, vascular involvement (venous thrombosis and/or arterial aneursyms). The BDCAF score was calculated by adding up the scores on each item and ranged from 0 to 12. The study protocol was approved by the local ethics committee. The research protocol complies with the 2000 Declaration of Helsinki and written informed consent was obtained from all participants.
Posterior Segment Ocular Parameters in Children with Familial Mediterranean Fever
Published in Ocular Immunology and Inflammation, 2021
ArifÜlkü Yener, AslıÇelebi Tayfur
Familial Mediterranean fever (FMF) is an autosomal recessive hereditary disease primarily seen in populations living in the Mediterranean region.1 This autoinflammatory disease, which predominantly affects Jews, Arabs, Armenians or Turks is characterized by recurrent attacks of fever, peritonitis, pleuritis, arthritis or erysipelas-like skin lesions.1 Although some reports suggest a slightly male predominance, FMF affects both sexes in a similar ratio. FMF symptoms are caused by an inflammatory reaction that affects serosal tissues such as pleura, peritoneum and synovium. These symptoms usually start before 10 years of age and development of the disease after the age of 30 is extremely rare.1 The severity and frequency of attacks mostly decline as the patients get older.
Co-occurrence of incontinentia pigmenti and down syndrome: examining patients’ potential susceptibility to autoimmune disease, autoinflammatory disease, cancer, and significant ocular disease
Published in Ophthalmic Genetics, 2021
David C. Gibson, Natario L. Couser, Kayla B. King
These characteristics of IP are widely accepted, but the possibility of increased disease susceptibility in IP has not been well established. A variety of diseases and genetic conditions have been reported in patients with IP. In many of these cases, patients with IP develop a concurrent autoimmune or autoinflammatory disease. Systemic lupus erythematosus (SLE), Behcet disease, Still’s disease with macrophage activation syndrome, myasthenia gravis, immune thrombocytopenic purpura, and acute disseminated encephalomyelitis have all been seen in patients with IP (2–6) (Table 1). It has been hypothesized that the link between IP and autoimmune or autoinflammatory disease is due to the dysregulation of NF-κB (4). As discussed, NF-κB is an important transcription factor regulated by the gene that is mutated in IP. The dysregulation of NF-κB in many autoimmune and autoinflammatory diseases like SLE, type 1 diabetes mellitus (DM), celiac disease, inflammatory bowel disease, multiple sclerosis, rheumatoid arthritis, and haploinsufficiency of A20 has been demonstrated in multiple human studies (16). This relationship between NF-κB, autoimmune disease, and autoinflammatory disease supports the concept of increased susceptibility to immune-related disorders in patients with IP.