An extremely rare condition manifested as monoclonal IMMUNOGLOBULIN M dysproteinemia without features of lymphoproliferative disease, but with chronic urticaria, fever of unknown origin, disabling bone pain, hyperostosis, and increased erythrocyte sedimentation rate.
Works containing information articles on subjects in every field of knowledge, usually arranged in alphabetical order, or a similar work limited to a special field or subject. (From The ALA Glossary of Library and Information Science, 1983)
A vascular reaction of the skin characterized by erythema and wheal formation due to localized increase of vascular permeability. The causative mechanism may be allergy, infection, or stress.
Conditions characterized by the presence of M protein (Monoclonal protein) in serum or urine without clinical manifestations of plasma cell dyscrasia.

Beneficial response to anakinra and thalidomide in Schnitzler's syndrome. (1/9)

BACKGROUND: Schnitzler's syndrome is an inflammatory disorder characterised by chronic urticarial rash and monoclonal gammopathy, accompanied by periodic fever, arthralgia or arthritis, and bone pain. The cause and treatment are still unknown. OBJECTIVE: To assess treatment with thalidomide and an interleukin 1 receptor antagonist, anakinra, in Schnitzler's syndrome. CASE REPORTS: Three patients with Schnitzler's syndrome are described, one with IgM gammopathy, two with IgG type. In one patient, thalidomide induced complete remission, but was stopped because of polyneuropathy. Anakinra 100 mg daily in all three patients led to disappearance of fever and skin lesions within 24 hours. After a follow up of 6-18 months, all patients are free of symptoms. CONCLUSION: Anakinra proved to be effective in three patients with Schnitzler's syndrome. This treatment is preferable to thalidomide, which induced a complete remission in one of our patients, as it has fewer side effects.  (+info)

Time-course analysis of serum hepcidin, iron and cytokines in a C282Y homozygous patient with Schnitzler's syndrome treated with IL-1 receptor antagonist. (2/9)

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Dysfunctional inflammasome in Schnitzler's syndrome. (3/9)

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Cryopyrin-associated periodic syndrome: an update on diagnosis and treatment response. (4/9)

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The Schnitzler syndrome. (5/9)

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Effects of the histone deacetylase inhibitor ITF2357 in autoinflammatory syndromes. (6/9)

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Increased angiogenesis and enhanced bone formation in patients with IgM monoclonal gammopathy and urticarial skin rash: new insight into the biology of Schnitzler syndrome. (7/9)

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Effect of in vitro and in vivo anakinra on cytokines production in Schnitzler syndrome. (8/9)

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Schnitzler Syndrome is a rare autoinflammatory disorder characterized by the recurrent occurrence of erythema (skin rash), often resembling chronic urticaria, and arthralgia or arthritis (joint pain or inflammation). It is typically associated with monoclonal gammopathy, usually of IgM type. Other common features may include fever, lymphadenopathy (swollen lymph nodes), hepatosplenomegaly (enlarged liver and spleen), bone pain, and fatigue. The exact cause of Schnitzler Syndrome is not known, but it is thought to be related to an abnormal immune response. Treatment typically involves the use of medications that suppress the immune system, such as steroids or biologic agents.

An encyclopedia is a comprehensive reference work containing articles on various topics, usually arranged in alphabetical order. In the context of medicine, a medical encyclopedia is a collection of articles that provide information about a wide range of medical topics, including diseases and conditions, treatments, tests, procedures, and anatomy and physiology. Medical encyclopedias may be published in print or electronic formats and are often used as a starting point for researching medical topics. They can provide reliable and accurate information on medical subjects, making them useful resources for healthcare professionals, students, and patients alike. Some well-known examples of medical encyclopedias include the Merck Manual and the Stedman's Medical Dictionary.

Urticaria, also known as hives, is an allergic reaction that appears on the skin. It is characterized by the rapid appearance of swollen, pale red bumps or plaques (wheals) on the skin, which are often accompanied by itching, stinging, or burning sensations. These wheals can vary in size and shape, and they may change location and appear in different places over a period of hours or days. Urticaria is usually caused by an allergic reaction to food, medication, or other substances, but it can also be triggered by physical factors such as heat, cold, pressure, or exercise. The condition is generally harmless, but severe cases of urticaria may indicate a more serious underlying medical issue and should be evaluated by a healthcare professional.

Monoclonal gammopathy of undetermined significance (MGUS) is a medical condition characterized by the presence of a monoclonal protein, or M-protein, in the blood or urine, but without any signs or symptoms of related disorders. The M-protein is produced by a single clone of plasma cells, which are a type of white blood cell found in the bone marrow.

In MGUS, the level of M-protein is typically low (less than 3 grams per deciliter), and there are no signs of damage to organs such as the bones, kidneys, or nervous system. However, people with MGUS have a higher risk of developing certain related conditions, such as multiple myeloma, amyloidosis, or lymphoplasmacytic lymphoma, compared to those without MGUP.

MGUS is usually detected through routine blood or urine tests and is typically asymptomatic. However, in some cases, people with MGUS may experience symptoms such as fatigue, bone pain, or recurrent infections. If these symptoms occur, further testing may be necessary to determine if MGUS has progressed to a more serious condition.

It's important to note that MGUS is not a cancer itself, but rather a potential precursor to certain types of cancer. Regular monitoring with blood or urine tests and physical examinations is recommended for people diagnosed with MGUS to monitor for any changes that may indicate progression to a more serious condition.

... or Schnitzler's syndrome is a rare disease characterised by onset around middle age of chronic hives ( ... AA amyloidosis has also been reported in people with Schnitzler syndrome. The life span in patients with Schnitzler syndrome ... Chu, Cong-Qiu (27 January 2022). "Schnitzler syndrome and Schnitzler-like syndromes". Chinese Medical Journal. 135 (10): 1190- ... Schnitzler syndrome is a late-onset autoinflammatory disorder. The typical onset is at around 55 years old, and the symptoms ...
... is a French dermatologist who described the eponymous Schnitzler's syndrome in 1972. Schnitzler's syndrome ... First description of Schnitzler's syndrome) Schnitzler, L; Schubert, B; Boasson, M; Gardais, J; Tourmen, A (1974). "Urticaire ... Sfriso, Paolo; Galozzi, Paola (2019). "15: The Schnitzler Syndrome". In Efthimiou, Petros (ed.). Auto-Inflammatory Syndromes: ... Schnitzler; B. Schubert; J. Sibilia; D. Lipsker (2013). Bieber, Thomas (ed.). "Schnitzler's syndrome: diagnosis, treatment, and ...
Schnitzler, L.; Schubert, B.; Sibilia, J.; Lipsker, D. (2013). "Schnitzler's syndrome: diagnosis, treatment, and follow-up". ... The presence of IgM or, rarely, IgG is one of the obligate criteria for a diagnosis of Schnitzler's syndrome. Immunodeficiency ... Gusdorf, L.; Lipsker, D. (17 July 2017). "Schnitzler Syndrome: a Review". Current Rheumatology Reports. 19 (8): 46. doi:10.1007 ... Waldenström, J. (1943). "Incipient myelomatisis or "essential" hyoerglobulinemis with fibrinogenopenia-a new syndrome?". Acta ...
It is used to treat Schnitzler's syndrome (off label in the US). Its response rate is such that it has been suggested that " ... Gusdorf L, Lipsker D (August 2017). "Schnitzler Syndrome: a Review". Current Rheumatology Reports. 19 (8): 46. doi:10.1007/ ... October 2014). "Long-term effectiveness and safety of interleukin-1 receptor antagonist (anakinra) in Schnitzler's syndrome: a ... allowing Anakinra to be commissioned as a first-line treatment for Schnitzler's syndrome and in cases where the first-line ...
"The two-syndrome concept as formulated by T. J. Crow was especially important in spurring research into the nature of negative ... Schnitzler, Alfons; Münchau, Alexander (1 September 2012). "The pathophysiology of echopraxia/echolalia: Relevance to Gilles De ... Although the vast majority of studies of the efficacy of antipsychotic treatment do not report effects on syndromes or symptoms ... Disturbances of thinking and speech, such as clanging or echolalia, may also be present in Tourette syndrome; other symptoms ...
"Orphanet: Rudiger syndrome". Retrieved August 2, 2010. Online Mendelian Inheritance in Man (OMIM): 268650 Schnitzler, L.; ... "EEC syndrome". However, this is not to be confused with the formal EEC syndrome associated with chromosome 7. It was ... Rudiger syndrome is a congenital disorder characterized by the association of severe growth retardation with abnormalities of ... The features ectrodactyly, ectodermal dysplasia and cleft palate have been described with Rudiger syndrome, giving it the ...
... described in one individual with Tourette syndrome (as of 2012[update]). Ganos C, Ogrzal T, Schnitzler A, Münchau A (September ... a new echo phenomenon in a person with Gilles de la Tourette syndrome" (PDF). Psychopathology. 41 (3): 201-2. doi:10.1159/ ... insights into the pathophysiology of symptoms in Tourette's syndrome". Ann Neurol. 33 (2): 212-5. doi:10.1002/ana.410330212. ... 2012). "The pathophysiology of echopraxia/echolalia: relevance to Gilles de la Tourette syndrome". Mov. Disord. 27 (10): 1222-9 ...
Ganos C, Ogrzal T, Schnitzler A, Münchau A (September 2012). "The pathophysiology of echopraxia/echolalia: relevance to Gilles ... de la Tourette syndrome". Mov. Disord. 27 (10): 1222-9. doi:10.1002/mds.25103. PMID 22807284. S2CID 22422642. Heyes C (Aug 5, ...
Ganos C, Ogrzal T, Schnitzler A, Münchau A (September 2012). "The pathophysiology of echopraxia/echolalia: relevance to Gilles ... It has long been recognized as a core feature of Tourette syndrome, and is considered a complex tic, but it also occurs in ... Echopraxia is a typical symptom of Tourette syndrome but causes are not well elucidated. One theoretical cause subject to ... de la Tourette syndrome". Mov. Disord. 27 (10): 1222-9. doi:10.1002/mds.25103. PMID 22807284. Realmuto GM, August GJ (December ...
Some of his favorite writers include Susan Cooper, Thomas Mann and Arthur Schnitzler. In 2014, Sedgwick was diagnosed with ... chronic fatigue syndrome, also known as myalgic encephalomyelitis. After his diagnosis, he moved to the French Alps and then to ...
... occurs in many cases of autism spectrum disorder and Tourette syndrome. It may also occur in several other ... List of language disorders Ganos C, Ogrzal T, Schnitzler A, Münchau A (September 2012). "The pathophysiology of echopraxia/ ... Echolalia can be an indicator of communication disorders in autism, but is neither unique to, nor synonymous with syndromes. ... Bashe, P. R. The OASIS Guide to Asperger Syndrome; Advice, Support, Insight, and Inspiration. Crown Publishers, 2001, p. 22. ...
"Imaging findings in 22 cases of Schnitzler syndrome: characteristic para-articular osteosclerosis, and the "hot knees" sign ... Osteosclerosing types of chronic osteomyelitis Hypervitaminosis D hyperparathyroidism Schnitzler syndrome Mastocytosis Skeletal ... Buschke-Ollendorff syndrome) Osteopathia striata with cranial sclerosis Mixed sclerosing bone dysplasia Progressive diaphyseal ... with renal tubular acidosis Infantile osteopetrosis with immunodeficiency IO with leukocyte adhesion deficiency syndrome (LAD- ...
Schnitzler's syndrome, Gleich's syndrome, or thyroid disease. The specific symptoms, triggers, and results of skin and blood ... "The overlap syndrome of urticaria and gastroesophageal reflux disease". PLOS ONE. Public Library of Science (PLoS). 13 (11): ... and conditions such as mastocytosis and mast cell activation syndrome. It's also important to rule out systemic diseases that ...
... father of Arthur Liliane Schnitzler, French dermatologist who first described Schnitzler syndrome Michoel Schnitzler, a ... personality and propagandist Schnitzler syndrome Schnitz Schnitzer Schnitzel This page lists people with the surname Schnitzler ... There have been several people named Schnitzler (Hebrew: שניצלר): Arthur Schnitzler (born 1862), an Austrian playwright and ... Schnitzler (1884, Köln - 1962, Basel), executive board member of IG Farben ∞ Lilly von Schnitzler (de) Ottilie Marie Edith von ...
Schnitzler syndrome, and chronic recurrent multifocal osteomyelitis. It is likely that these diseases are multifactorial, with ... The syndromes are diverse, but tend to cause episodes of fever, joint pains, skin rashes, abdominal pains and may lead to ... Periodic fever syndromes are a set of disorders characterized by recurrent episodes of systemic and organ-specific inflammation ... The most common genetic autoinflammatory syndrome is familial Mediterranean fever, which causes short episodes of fever, ...
Mutations in CHD3 cause a neurodevelopmental syndrome (Snijders Blok-Campeau syndrome) with macrocephaly and impaired speech ... Tong JK, Hassig CA, Schnitzler GR, Kingston RE, Schreiber SL (Oct 1998). "Chromatin deacetylation by an ATP-dependent ... Tong JK, Hassig CA, Schnitzler GR, Kingston RE, Schreiber SL (Oct 1998). "Chromatin deacetylation by an ATP-dependent ... November 2018). "CHD3 helicase domain mutations cause a neurodevelopmental syndrome with macrocephaly and impaired speech and ...
Kelly syndrome Schnitzler syndrome Schwartz-Jampel syndrome Schöpf-Schulz-Passarge syndrome Scimitar syndrome Scott syndrome ... syndrome Wende-Bauckus syndrome Werner syndrome Wernicke-Korsakoff syndrome West syndrome Westerhof syndrome Wet lung syndrome ... syndrome Shone's syndrome Short anagen syndrome Short bowel syndrome short limb syndrome Short man syndrome Short QT syndrome ... syndrome Radial tunnel syndrome Rage syndrome Raghib syndrome Raine syndrome Ramos-Arroyo syndrome Ramsay Hunt syndrome type 1 ...
Schnitzler syndrome MeSH C20.683.780.650 - multiple myeloma MeSH C20.683.780.750 - POEMS syndrome MeSH C20.683.780.925 - ... Guillain-Barré syndrome MeSH C20.111.258.750.400.500 - Miller Fisher syndrome MeSH C20.111.258.750.600 - hereditary sensory and ... Sézary syndrome MeSH C20.683.515.950 - tumor lysis syndrome MeSH C20.683.780.250 - cryoglobulinemia MeSH C20.683.780.490 - ... stiff-person syndrome MeSH C20.111.258.925 - uveomeningoencephalitic syndrome MeSH C20.111.258.962 - vasculitis, central ...
Crohn's disease or Blau syndrome) IL‑1β pathway dysregulation (PFAPA, Schnitzler syndrome, DIRA or DITRA) Impaired efficacy of ... Schnitzler syndrome, Crohn's disease, or DIRA) Persistent, affecting the skin (Interleukin-36-receptor antagonist deficiency ( ... This syndrome is caused by mutation in the gene which encodes subunit β type-8 of proteasome (PSMB8 gene). Due to this mutation ... In the case of Aicardi-Goutieres syndrome 7 (AGS7), the gain-of-function mutation in a sensor molecule in the RNA-sensing ...
Aicardi-Goutières syndrome Still's disease Schnitzler syndrome Hyperimmunoglobulin D syndrome Familial Mediterranean fever ... This syndrome is also known as the Prieur-Griscelli syndrome as it was first described by these authors in 1981. The age of ... The disease is caused in 60% of cases by a mutated gene called CIAS1 that is known to be involved in other syndromes that ... Hyperimmunoglobulin D syndrome in 50% of cases is associated with mevalonate kinase deficiency which can be measured in the ...
PMID 6580071 L. Houet, I. Möller, M. Engelhardt, G. Köhler, H. Schmidt, D. Herchenbach, M. Schnitzler, A. Schmitt-Graeff, A. A ... Phase I clinical trial of 13-cis-retinoic acid in myelodysplastic syndromes. In: Cancer Treat Rep. 1983 Nov;67(11), S. 981-986 ...
It can also be associated with Turcot syndrome. MLH1 has been shown to interact with: Bloom syndrome protein Exonuclease 1, ... Warusavitarne J, Schnitzler M (2007). "The role of chemotherapy in microsatellite unstable (MSI-H) colorectal cancer". ... FAQs on HNPCC from the National Institute of Health GeneReviews/NCBI/NIH/UW entry on Lynch syndrome MLH1+protein,+human at the ... MLH1-MLH3 heterodimer (MutL gamma) together with EXO1 and Sgs1 (ortholog of Bloom syndrome helicase) define a joint molecule ...
Her daughter Nele, who has Down's syndrome, is also an actress; she had a role in Dora or The Sexual Neuroses of Our Parents ... Peter Zadek 2002 Anatol by Arthur Schnitzler (as Gabriele, Burgtheater, Wien), director: Luc Bondy 2003 The Night of the Iguana ...
Mutations in this gene have been associated with a condition known as Sifrim-Hitz-Weiss syndrome. This condition is ... "Entrez Gene: CHD4 chromodomain helicase DNA binding protein 4". Tong JK, Hassig CA, Schnitzler GR, Kingston RE, Schreiber SL ( ... Tong JK, Hassig CA, Schnitzler GR, Kingston RE, Schreiber SL (October 1998). "Chromatin deacetylation by an ATP-dependent ... Tong JK, Hassig CA, Schnitzler GR, Kingston RE, Schreiber SL (October 1998). "Chromatin deacetylation by an ATP-dependent ...
White nose syndrome is a condition associated with the deaths of millions of bats in the Eastern United States and Canada. The ... Schnitzler, H.-U.; Kalko, E. K. V.; Kaipf, I.; Grinnell, A. D. (1994). "Fishing and Echolocation Behavior of the Greater ... "White-Nose Syndrome Confirmed in Illinois Bats: Illinois becomes 20th state in U.S. to confirm deadly disease in bats" (PDF). ... "White-Nose Syndrome - Background". Canadian Cooperative Wildlife Health Centre. Retrieved 3 June 2014. Daly, M. (14 November ...
Andreas Giedion Schnitzler syndrome - Liliane Schnitzler Seaver Cassidy syndrome - Laurie Seaver, Suzanne Cassidy Seligmann's ... Hakaru Hashimoto Havisham syndrome (a.k.a. Diogenes syndrome, Miss Havisham syndrome, and Plyushkin syndrome) - Miss Havisham, ... Havisham syndrome, Miss Havisham syndrome, Plyushkin syndrome)- Diogenes of Sinope (the particular usage, Diogenes syndrome, is ... syndrome - Moritz Simmonds Sipple's syndrome - John H. Sipple Sjögren syndrome - Henrik Sjögren Sjögren-Larsson syndrome - ...
Bartter's syndrome Gitelman syndrome Hypomagnesemia Hypocalcemia Konrad M, Schlingmann K, Gudermann T (2004). "Insights into ... Chubanov V, Waldegger S, Mederos y Schnitzler M, Vitzthum H, Sassen M, Seyberth H, Konrad M, Gudermann T (2004). "Disruption of ...
Schnitzler M, Hasskarl J, Egger M, Bertz H, Finke J (August 2009). "Successful treatment of severe acute intestinal graft- ... New cases of norcardial infection and cytomegalovirus syndrome". Multiple Sclerosis. 23 (6): 876-877. doi:10.1177/ ... which include serious opportunistic nocardial infections and cytomegalovirus syndrome. Alemtuzumab can also precipitate ...
Reflex cold urticaria Schnitzler syndrome Secondary cold contact urticaria Solar urticaria Systemic capillary leak syndrome ... Turner syndrome Ulnar-mammary syndrome Van Der Woude syndrome Von Hippel-Lindau syndrome Watson syndrome Werner syndrome (adult ... Freeman-Sheldon syndrome, Windmill-Vane-Hand syndrome) Wilson-Turner syndrome Wolf-Hirschhorn syndrome (4p- syndrome) X-linked ... Rombo syndrome Rothmund-Thomson syndrome (poikiloderma congenitale) Rud syndrome Say syndrome Scalp-ear-nipple syndrome (Finlay ...
Lion in the Streets (1990) uses a structure similar to Arthur Schnitzler's La Ronde to follow violent and cruel impulses from ... The success of this verbatim play inspired Thompson to create a play with 9 performers with Down Syndrome for the Fringe ...
Schnitzler syndrome or Schnitzlers syndrome is a rare disease characterised by onset around middle age of chronic hives ( ... AA amyloidosis has also been reported in people with Schnitzler syndrome. The life span in patients with Schnitzler syndrome ... Chu, Cong-Qiu (27 January 2022). "Schnitzler syndrome and Schnitzler-like syndromes". Chinese Medical Journal. 135 (10): 1190- ... Schnitzler syndrome is a late-onset autoinflammatory disorder. The typical onset is at around 55 years old, and the symptoms ...
Schnitzler syndrome is characterized by chronic, nonpruritic urticaria in association with recurrent fever, bone pain, ... encoded search term (Schnitzler Syndrome) and Schnitzler Syndrome What to Read Next on Medscape ... Schnitzler syndrome was first described in a 1972 case report by French dermatologist Liliane Schnitzler, [5] who further ... that are also found in cryopyrin-associated periodic syndrome (CAPS) has been found in a few Schnitzler syndrome patients. ...
Schnitzler syndrome is characterized by chronic, nonpruritic urticaria in association with recurrent fever, bone pain, ... encoded search term (Schnitzler Syndrome) and Schnitzler Syndrome What to Read Next on Medscape ... Schnitzler syndrome was first described in a 1972 case report by French dermatologist Liliane Schnitzler, [5] who further ... that are also found in cryopyrin-associated periodic syndrome (CAPS) has been found in a few Schnitzler syndrome patients. ...
Stories of Schnitzler syndrome - Tell your story and help others ...
An OverviewSchnitzler syndrome is a rare autoinflammatory disorder that is characterized by chronic urticarial rash and ... Clinical Features of Schnitzler Syndrome. The hallmark of Schnitzler syndrome is a chronic urticarial rash, which is often ... Diagnosis of Schnitzler Syndrome. The diagnosis of Schnitzler syndrome can be challenging, and it often requires the exclusion ... Pathophysiology of Schnitzler Syndrome. The pathophysiology of Schnitzler syndrome is not fully understood, but it is thought ...
Schnitzler Syndrome. Schnitzlers syndrome is an adult-onset autoinflammatory disease characterized by urticarial exanthema and ... Vanderschueren and Knockaert (2013) tested canakinumab in patients with Schnitzler syndrome. A patient with Schnitzler syndrome ... Cryopyrin-Associated Periodic Syndromes (CAPS). Cryopyrin‐Associated Periodic Syndromes (CAPS) refer to rare genetic syndromes ... Sweet syndrome; pyoderma gangrenosum; hidradenitis suppurativa; Schnitzler syndrome; Still disease". The precise dermatological ...
... also called autoinflammatory syndromes, are characterized by relapsing fever and additional manifestations such as skin rashes ... Schnitzler Syndrome / complications * Schnitzler Syndrome / genetics * Skin Diseases / etiology* * Stills Disease, Adult-Onset ... Hereditary periodic fever syndromes, also called autoinflammatory syndromes, are characterized by relapsing fever and ... Autoinflammatory syndromes in dermatology] Rev Med Suisse. 2012 Apr 4;8(335):756-8, 760-1. ...
Schnitzler syndrome (SchS) was first described in 1972 and to date 281 cases have been reported. SchS is an adult onset, ... Clinical symptoms and molecular investigations in 13 patients with Schnitzler syndrome identified at the single UK centre. *D ... Clinical symptoms and molecular investigations in 13 patients with Schnitzler syndrome identified at the single UK centre ... Clinical symptoms and molecular investigations in 13 patients with Schnitzler syndrome identified at the single UK centre. ...
Schnitzler Syndrome / diagnosis * Schnitzler Syndrome / drug therapy* Substances * Dermatologic Agents * Immunoglobulin A ...
Unraveling corticobasal syndrome and alien limb syndrome with structural brain imaging. Cortex 117, pp. 33 - 40 (2019) ... Schnitzler, A.; Südmeyer, M.; Oertel, W.; Kassubek, J.; Höglinger, G.; Schroeter, M. L.: Comparative analysis of machine ... learning algorithms for multi-syndrome classification of neurodegenerative syndromes. Alzheimers Research & Therapy 14 (1), 62 ... Multiclass prediction of different dementia syndromes based on multi-centric volumetric MRI imaging. NeuroImage: Clinical 37, ...
Unraveling corticobasal syndrome and alien limb syndrome with structural brain imaging. Cortex 117, pp. 33 - 40 (2019) ... Schnitzler, A.; Südmeyer, M.; Oertel, W.; Kassubek, J.; Höglinger, G.; Schroeter, M. L.: Comparative analysis of machine ... learning algorithms for multi-syndrome classification of neurodegenerative syndromes. Alzheimers Research & Therapy 14 (1), 62 ... Multiclass prediction of different dementia syndromes based on multi-centric volumetric MRI imaging. NeuroImage: Clinical 37, ...
Urticarial vasculitis (UV) and autoinflammatory syndromes, i.e., cryopyrin-associated periodic syndromes (CAPS) and Schnitzler ... syndrome (SchS), can mimic CSU-like rash but represent rare disorders with systemic symptoms including fever, headache, ...
Fleischer W, Theiss S, Slotta J, Holland C, Schnitzler A (2015) High-frequency voltage oscillations in cultured astrocytes. ... The Effects of Pharmacological Compounds on Beat Rate Variations in Human Long QT-Syndrome Cardiomyocytes. Stem Cell Rev. ... Response to autonomic stimulation is blunted in a murine model of Burgade Syndrome Heart Rhythm 12:S324-S371. ... Ajmaline blocks INa and IKr without eliciting differences between Brugada syndrome patient and control human pluripotent stem ...
Fleischer W, Theiss S, Slotta J, Holland C, Schnitzler A (2015) High-frequency voltage oscillations in cultured astrocytes. ... The Effects of Pharmacological Compounds on Beat Rate Variations in Human Long QT-Syndrome Cardiomyocytes. Stem Cell Rev. ... Response to autonomic stimulation is blunted in a murine model of Burgade Syndrome Heart Rhythm 12:S324-S371. ... Ajmaline blocks INa and IKr without eliciting differences between Brugada syndrome patient and control human pluripotent stem ...
Schnitzler syndrome in a patient with a family history of monoclonal gammopathy *Pathology mentor of a medical student. ... Molecular analysis of multi-focal tumors in a patient with Birt-Hogg-Dube syndrome. FASEB J 19(5, Suppl S, Part 2):A1533, 2005. ... Sweet syndrome following vemurafenib therapy for recurrent cholangiocarcinoma. J Cutan Pathol 41(3):326-8, 2014. e-Pub 2013. ... Trichorhinophalangeal Syndrome Type 1-Positive Cells in Breast Dermal Granulation Tissues and Scars: A Potential Diagnostic ...
a. Möller L, Huppertz HJ, Südmeyer M, Hilker R, Hattingen R, Egger K, Amtage F, Respondek G, Stamelou M, Schnitzler A, Oertel ... b. Huppertz HJ, Möller L, Südmeyer M, Hilker R, Hattingen E, Egger K, Amtage F, Respondek G, Stamelou M, Schnitzler A, ... Im Jahr 2016 hat die Arbeitsgruppe Atypische Parkinson Syndrome der die DPG folgende Projekte realisiert:. *Zwei in personam ... Die Mitglieder der DPG AG Atypische Parkinson Syndrome waren zahlreich vertreten (Höglinger GU, Gesine Respondek, Brit ...
Schnitzler syndrome: a rare cause of difficult to treat chronic urticaria Aslı GELİNCİK, Mustafa DEMİRTÜRK, Suna BÜYÜKÖZTÜRK, ...
Schnitzler syndrome: a rare cause of difficult to treat chronic urticaria Aslı GELİNCİK, Mustafa DEMİRTÜRK, Suna BÜYÜKÖZTÜRK, ...
Schnitzler syndrome and Schnitzler-like syndromes. Chu Cong-Qiu, et al. Chinese medical journal 2022 0 (10) 1190-1202 ... Exploratory Study of MYD88 L265P, Rare NLRP3 Variants, and Clonal Hematopoiesis Prevalence in Patients With Schnitzler Syndrome ...
Rett syndrome Rheumatoid arthritis Rhinosinusitis Schizophrenia Schnitzler syndrome Serious Trouble Short bowel syndrome ... Alagille syndrome Alcohol use disorder Alkaptonuria Alopecia areata Alport Syndrome Alzheimers disease Amyloid light chain ... WHIM syndrome Wilsons disease Wiskott-Aldrich syndrome (WAS) X-linked adrenoleukodystrophy (X-ALD) X-linked protoporphyria ... Bladder pain syndrome Blastic plasmacytoid dendritic cell neoplasm (BPDCN) Bleeding Bone and Cartilage Cancer Bone sarcoma ...
It has been approved in Europe to treat cryopyrin-associated periodic syndromes, gouty arthritis, and juvenile arthritis. The ... It is used to treat cryopyrin-associated periodic syndromes, familial mediterranean fever, juvenile arthritis, and mevalonate ... Myelodysplastic syndromes. D009190. D46. 2. 3. -. -. -. 4. Schnitzler syndrome. D019873. EFO_1001165. -. 3. -. -. -. 3. ... Cytokine release syndrome. D000080424. D89.83. -. -. 1. -. 1. 2. Atherosclerosis. D050197. EFO_0003914. I25.1. -. 1. 1. -. -. 2 ...
Schnitzler syndrome; 24th EADV Congress; 9-12 October 2015; Copenhagen, Denmark (invited speaker) ... Schnitzler syndrome; 26th EADV Congress; Geneva, 23-17 September 2017 (invited speaker) ... Stöllberger C, Vujic I, Wollmann E, Freudenthaler J, Finsterer J. Carvajal syndrome with oligodontia, hypoacusis, recurrent ...
Schnitzler Syndrome. Pruritus, Increased circulating IgM level, Skin rash, Arthritis. ORPHA:37748. ...
Muckle-Wells Syndrome (MWS); Scleroderma, Systemic; Anemia; Schnitzler Syndrome; Diabetes Mellitus, Type 1; Stills Disease, ... Macrophage Activation Syndrome; Cytokine Release Syndrome; Lymphohistiocytosis, Hemophagocytic. Details Hutrukin. Phase 1 ... Cryopyrin-Associated Periodic Syndromes. Regeneron Pharmaceuticals Inc. 2008-02-27. Pericarditis; Renal Insufficiency, Chronic ... Inflammation; Gout; Scleroderma, Diffuse; Urticaria; Cryopyrin-Associated Periodic Syndromes; Hearing Loss, Sensorineural; ...
Rett syndrome Rheumatoid arthritis Rhinosinusitis Schizophrenia Schnitzler syndrome Serious Trouble Short bowel syndrome ... Alagille syndrome Alcohol use disorder Alkaptonuria Alopecia areata Alport Syndrome Alzheimers disease Amyloid light chain ... WHIM syndrome Wilsons disease Wiskott-Aldrich syndrome (WAS) X-linked adrenoleukodystrophy (X-ALD) X-linked protoporphyria ... Bladder pain syndrome Blastic plasmacytoid dendritic cell neoplasm (BPDCN) Bleeding Bone and Cartilage Cancer Bone sarcoma ...
Autoinflammatory syndromes (AIS) are a group of related inflammatory disorders. They are characterized by unprovoked repeated ... Schnitzler syndrome. 5- Pyogenic Arthritis, Pyoderma gangrenosum and Acne (PAPA):. *Blau Syndrome ... LIST OF AUTOINFLAMMATORY SYNDROMES:. 1- Tumor necrosis factor receptor-associated periodic syndrome (TRAPS) or Familial ... 3- Hyperimmunoglobulin D syndrome (HIDS). 4- Cryopyrin-associated periodic syndrome (CAPS):. *Familial cold autoinflammatory ...
Other variants, including a chronic ataxic neuropathy known as Miller-Fisher syndrome (a variant of Guillain-Barré syndrome), ... POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes) also may be associated with ... Infiltration of the CNS by the malignant clone can cause a syndrome of confusion, memory loss, disorientation, and motor ... Ophthalmologic techniques to assess the severity of hyperviscosity syndrome and the effect of plasmapheresis in patients with ...
The Dubowitz syndrome: further observations.. Orrison WW, Schnitzler ER, Chun RW. Am J Med Genet 1980;7(2):155-70. doi: 10.1002 ... The C syndrome, also known as Opitz trigonocephaly syndrome, is a malformation syndrome characterized by trigonocephaly, severe ... The Dubowitz syndrome: further observations.. Orrison WW, Schnitzler ER, Chun RW. Am J Med Genet 1980;7(2):155-70. doi: 10.1002 ... C syndrome shows phenotypic overlap with Bohring-Opitz syndrome, or C-like syndrome (605039), a disorder with more severe ...
... of people with Schnitzler syndrome develop a lymphoproliferative disorder. Schnitzler syndrome or Schnitzlers syndrome is a ... Schnitzler syndrome - Schnitzler syndrome, which is often unrecognized, is a form of chronic urticaria associated with ... The diagnosis rests on clinical criteria, a Schnitzler Syndrome. The etiology of Schnitzler syndrome remains unknown. ... Schnitzler syndrome may be present in up to 1.5% of patients with a monoclonal IgM in their serum and likely under-recognized ...
  • Urticarial vasculitis (UV) and autoinflammatory syndromes, i.e., cryopyrin-associated periodic syndromes (CAPS) and Schnitzler syndrome (SchS), can mimic CSU-like rash but represent rare disorders with systemic symptoms including fever, headache, conjunctivitis, and arthralgia. (iasp-pain.org)
  • It is used to treat cryopyrin-associated periodic syndromes, familial mediterranean fever, juvenile arthritis, and mevalonate kinase deficiency in the USA. (pharmakb.com)
  • It has been approved in Europe to treat cryopyrin-associated periodic syndromes, gouty arthritis, and juvenile arthritis. (pharmakb.com)
  • The disease is named after the French dermatologist Liliane Schnitzler who first described this syndrome in 1972. (wikipedia.org)
  • It was first described by Dr. Liliane Schnitzler in 1972, and since then, the understanding of the disease has grown significantly. (ineedmedic.com)
  • Schnitzler syndrome (SchS) was first described in 1972 and to date 281 cases have been reported. (biomedcentral.com)
  • However, the mechanism leading to the increased IL-1 Se hela listan på ojrd.biomedcentral.com Schnitzler syndrome (SchS) is a late-onset autoinflammatory disease characterized by the association of a chronic urticarial rash and monoclonal gammopathy with signs and symptoms of systemic inflammation. (web.app)
  • Schnitzler syndrome or Schnitzler's syndrome is a rare disease characterised by onset around middle age of chronic hives (urticaria) and periodic fever, bone pain and joint pain (sometimes with joint inflammation), weight loss, malaise, fatigue, swollen lymph glands and enlarged spleen and liver. (wikipedia.org)
  • Other conditions which can cause periodic fevers, paraproteins or chronic hives that should be ruled out, include (and are not limited to) autoimmune or autoinflammatory disorders such as adult-onset Still's disease, angioedema, hematological disorders such as lymphoma or monoclonal gammopathy of undetermined significance (MGUS), other causes of hives, cryoglobulinemia, mastocytosis, chronic neonatal onset multisystem inflammatory disease or Muckle-Wells syndrome. (wikipedia.org)
  • Mutations in the NLRP3 gene (nucleotide-binding oligoisomerization domain [NOD]-like [NLR] family pyrin domain containing 3) that are also found in cryopyrin-associated periodic syndrome (CAPS) has been found in a few Schnitzler syndrome patients. (medscape.com)
  • Hereditary periodic fever syndromes, also called autoinflammatory syndromes, are characterized by relapsing fever and additional manifestations such as skin rashes, mucosal manifestations, or arthralgias. (nih.gov)
  • Schnitzler syndrome is a late-onset autoinflammatory disorder. (wikipedia.org)
  • Dec 4, 2020 Alberth and Schnitzler reported a 10% incidence of irreversible mydriasis following PKP, but IOP was normal in all patients. (web.app)
  • Because anakinra is so highly and rapidly effective for inducing complete remission of Schnitzler syndrome, it has been suggested that in patients who do not respond to anakinra, the diagnosis should be reconsidered. (wikipedia.org)
  • The diagnosis of Schnitzler syndrome can be challenging, and it often requires the exclusion of other causes of chronic urticarial rash. (ineedmedic.com)
  • The diagnosis rests on clinical criteria, a Schnitzler Syndrome. (web.app)
  • Still's disease is a syndrome, meaning that the diagnosis is based on excluding other causes (that may look like Still's) and having typical symptoms, findings, and lab results compatible with a Still's diagnosis. (stillsnow.com)
  • Schnitzler syndrome is an autoinflammatory disease characterized by chronic, nonpruritic urticaria in association with monoclonal gammopathy. (medscape.com)
  • 2] Background/Purpose: Schnitzler's syndrome is characterized by chronic urticaria, intermittent fever, arthralgia, bone pain, gammopathy and marked systemic inflammation. (web.app)
  • The striking response to IL-1 blockade suggests that Schnitzler's syndrome is an IL-1 mediated condition of the expanding spectrum of systemic autoinflammatory disorders. (web.app)
  • Se hela listan på ctajournal.biomedcentral.com Schnitzler's syndrome is characterized by recurrent urticarial rash and monoclonal gammopathy, associated with clinical and biological signs of inflammation and a long‐term risk of AA amyloidosis and overt lymphoproliferation. (web.app)
  • Schnitzler's syndrome: Increased levels of bone formation and angiogenesis factors are reduced after successful pefloxacin treatment. (allthingsmanifestation.com)
  • Schnitzler syndrome is a rare autoinflammatory disorder that is characterized by chronic urticarial rash and monoclonal gammopathy. (ineedmedic.com)
  • Schnitzler syndrome is also associated with monoclonal gammopathy, which is characterized by the presence of a monoclonal immunoglobulin in the blood. (ineedmedic.com)
  • The monoclonal gammopathy is usually of the IgM subtype, and it is often found in the serum and/or urine of patients with Schnitzler syndrome. (ineedmedic.com)
  • The presence of monoclonal gammopathy is a distinguishing feature of Schnitzler syndrome, and it is not seen in other forms of chronic urticaria. (ineedmedic.com)
  • It has been suggested that the monoclonal gammopathy seen in Schnitzler syndrome is the result of clonal expansion of B-cells, which are responsible for the production of immunoglobulins. (ineedmedic.com)
  • Rash of Schnitzler syndrome. (medscape.com)
  • The hallmark of Schnitzler syndrome is a chronic urticarial rash, which is often described as pruritic and recurrent. (ineedmedic.com)
  • Schnitzler syndrome is a rare disorder characterized by a chronic reddish rash that resembles hives (urticaria) and elevated levels of a specific protein in the blood (monoclonal IgM gammopathy). (web.app)
  • The etiology of Schnitzler syndrome remains unknown. (web.app)
  • Schnitzler syndrome is considered an autoinflammatory disorder and is generally treated with anakinra, which inhibits interleukin 1. (wikipedia.org)
  • Schnitzler syndrome is a sporadic disorder, meaning it is not inherited in a familial pattern. (ineedmedic.com)
  • Discrimination of COVID-19 from inflammation-induced cytokine storm syndromes by disease-related blood biomarkers. (immunaid.eu)
  • Schnitzler syndrome may be present in up to 1.5% of patients with a monoclonal IgM in their serum and likely under-recognized as a clinical syndrome. (web.app)
  • Identification of Critical Transcriptomic Signalling Pathways in Patients with H Syndrome and Rosai-Dorfman Disease, Journal of Clinical Immunology volume 41, pages 441-457 (2021). (immunaid.eu)
  • Infiltration of the CNS by the malignant clone can cause a syndrome of confusion, memory loss, disorientation, and motor abnormalities called the Bing-Neel syndrome. (medscape.com)
  • Schnitzler syndrome is an autoinflammatory disease for which the exact pathophysiology remains unclear but seems to involve the innate immune system. (medscape.com)
  • The pathophysiology of Schnitzler syndrome is not fully understood, but it is thought to be related to abnormal activation of the immune system. (ineedmedic.com)
  • Urticaria needs to be differentiated from other medical conditions where wheals, angioedema, or both can occur, e.g. anaphylaxis, autoinflammatory syndromes, urticarial vasculitis, orbradykinin-mediated angioedema including hereditary angioedema (HAE). (cyberderm.net)
  • Another possible mechanism for the pathogenesis of Schnitzler syndrome is the activation of the adaptive immune system. (ineedmedic.com)
  • Several genetic mutations have been found in Schnitzler syndrome patients, though not consistently. (medscape.com)
  • Novel somatic mutations in UBA1 as a cause of VEXAS syndrome Blood. (immunaid.eu)
  • Schnitzler syndrome - Schnitzler syndrome, which is often unrecognized, is a form of chronic urticaria associated with monoclonal … The autoinflammatory diseases: An overview View in Chinese …consideration of cyclic neutropenia, which may be of childhood or adult onset. (web.app)
  • Craniofrontonasal syndrome is an X-linked developmental disorder that shows paradoxically greater severity in heterozygous females than in hemizygous males. (nih.gov)
  • However, when the human body is frequently flooded with larg Down syndrome, or trisomy 21, is a genetic disorder and chromosomal condition characterized by a third copy of chromosome 21. (web.app)
  • However, approximately 10-15% of people with Schnitzler syndrome develop a lymphoproliferative disorder. (web.app)
  • 19 A central role for IL-1β is illustrated by the high efficacy of anti-IL-1β therapy in patients with Schnitzler syndrome… 2019-02-14 The Schnitzler syndrome is a rare and underdiagnosed entity which is considered today as being a paradigm of an acquired/late onset auto-inflammatory disease. (web.app)
  • The Schnitzler syndrome is a rare and underdiagnosed entity which is considered today as being a paradigm of an acquired/late onset auto-inflammatory disease. (web.app)
  • The life span in patients with Schnitzler syndrome has not been shown to differ much from the general population. (wikipedia.org)
  • Radiologic evaluation shows evidence of hyperostosis in 35% of Schnitzler syndrome patients. (medscape.com)
  • Patients with Schnitzler syndrome can also present with bone pain, which is often located in the long bones of the extremities. (ineedmedic.com)
  • In one study, a genetic variant in the NLRP3 gene, which is involved in the inflammasome pathway, was identified in a small number of patients with Schnitzler syndrome. (ineedmedic.com)
  • Interhemispheric motor networks are abnormal in patients with Gilles de la Tourette syndrome. (uniklinik-duesseldorf.de)
  • Imitation in patients with Gilles de la Tourette syndrome--a behavioral study. (uniklinik-duesseldorf.de)
  • AA amyloidosis has also been reported in people with Schnitzler syndrome. (wikipedia.org)
  • Altered pattern of motor cortical activation-inhibition during voluntary movements in Tourette syndrome. (uniklinik-duesseldorf.de)
  • Increased sensory feedback in Tourette syndrome. (dagstuhl.de)
  • Schnitzler syndrome is a rare disease, and the true incidence and prevalence are not well-established due to underdiagnosis and misdiagnosis. (ineedmedic.com)
  • Autoinflammatory syndromes (AIS) are a group of related inflammatory disorders. (biomedicalfacts.com)
  • Schnitzler syndrome is a rare disease, with an estimated prevalence of less than 1 per million individuals. (ineedmedic.com)
  • Schnitzler syndrome is a very rare immunological disease. (web.app)
  • Other variants, including a chronic ataxic neuropathy known as Miller-Fisher syndrome (a variant of Guillain-Barré syndrome), have been described. (medscape.com)
  • Courtesy of DermNet New Zealand (http://www.dermnetnz.org/assets/Uploads/systemic/schnitzler.jpg). (medscape.com)
  • Sjögren syndrome (SS) is a systemic long-term auto-immune condition which affects glands in the body's moisture-producing surfaces. (wikenigma.org)
  • Investigation of a nosocomial outbreak of severe acute respiratory syndrome (SARS) in Toronto, Canada. (cdc.gov)
  • GLI3-related Pallister-Hall syndrome (GLI3-PHS) is characterized by a spectrum of anomalies ranging from polydactyly, asymptomatic bifid epiglottis, and hypothalamic hamartoma at the mild end to laryngotracheal cleft with neonatal lethality at the severe end. (nih.gov)
  • Antibody response after severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) vaccination is impaired in kidney transplant recipients. (lww.com)
  • Foundations of the severe acute respiratory syndrome preparedness and response plan for healthcare facilities. (cdc.gov)
  • The long-term outlook (prognosis) for people with Schnitzler syndrome is generally good. (web.app)
  • For people with Down syndrome, family members, caregivers and professionals. (advocatehealth.com)
  • GERD is common in people with Down syndrome (DS). (advocatehealth.com)
  • It is more common in people with Down syndrome. (advocatehealth.com)
  • However, some cases of Schnitzler syndrome have been reported in families, suggesting a possible genetic predisposition. (ineedmedic.com)
  • However, cases of Schnitzler syndrome have been reported in individuals as young as 17 years and as old as 83 years. (ineedmedic.com)