Characteristics of Good's Syndrome in China: A Systematic Review.

CONCLUSIONS: GS is a rare association of thymoma and immunodeficiency with a poor prognosis. Astute clinical acumen and increased awareness of the clinical and immunological profile of GS are needed to increase early diagnosis, that would benefit improved therapeutic effects. PMID: 28639577 [PubMed - in process]
Source: Chinese Medical Journal - Category: General Medicine Authors: Tags: Chin Med J (Engl) Source Type: research

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ConclusionsOur systematic review update found that there is presently insufficient evidence on the effectiveness of training and support programs for peer leaders of disease-based support groups, highlighting the need for well-designed and rigorously conducted RCTs to examine the effects of training for peer leaders of support groups, especially in a rare disease context. The Scleroderma Patient-centered Intervention Network ’s trial of the Scleroderma Support group Leader EDucation Program will serve as such a trial.Systematic review registrationPROSPEROCRD42018096369
Source: Systematic Reviews - Category: International Medicine & Public Health Source Type: research
AbstractWe aimed to analyze 10-year experience of WAIHA patients at a single referral center in Turkey. Clinical data, survival outcome of sixty patients who were diagnosed with WAIHA were retrospectively analyzed. All the patients were direct antiglobulin test (DAT) positive. In 21 (30%) patients, IgG plus C3d DAT positivity was documented. 16 patients were secondary WAIHA and most common underlying causes were lymphoproliferative diseases (5 patients) and connective tissue disease (8 patients). Corticosteroids were first choice as a first line therapy with 54.5% CR and 40.2% PR rates. 43.3% of the patients relapsed after...
Source: Indian Journal of Hematology and Blood Transfusion - Category: Hematology Source Type: research
This report represents one of the possible presentations of EGPA according to the antineutrophil cytoplasmic antibody status – which in our case was negative, with a low prognostic Five-Factor Score – that was successfully treated with oral steroids and azathioprine as a steroid-sparing agent. Our objective was to add a case report to the scarce existing literature in order to learn more about therapeutic options for EGPA. This case report demonstrates that oral steroids, as induction treatment, and azathioprine, as maintenance treatment, are effective in elderly patients with EGPA without involvement of any ot...
Source: Case Reports in Dermatology - Category: Dermatology Source Type: research
Will Schuller would be confined to a wheelchair - if not worse - without medication for his rare autoimmune disease, LEMS. The drug was free, but a drug company hiked the price to $375K.
Source: the Mail online | Health - Category: Consumer Health News Source Type: news
Publication date: Available online 31 January 2019Source: Multiple Sclerosis and Related DisordersAuthor(s): Benjamin M. Greenberg, Chitra Krishnan, Lana HarderAbstractImportancePatients afflicted with rare diseases often have a delay in diagnosis and treatment. Understanding the prevalence and impact of delayed diagnosis in transverse myelitis could trigger directed educational initiatives to increase clinician awareness and improve care.ObjectiveTo determine if symptoms at onset or care provider initially approached was associated with time to diagnosis, treatment or outcome in patients with transverse myelitis.DesignThi...
Source: Multiple Sclerosis and Related Disorders - Category: Neurology Source Type: research
Exp Clin Endocrinol Diabetes DOI: 10.1055/a-0804-2715Addison’s disease – the traditional term for primary adrenal insufficiency (PAI) – is defined as the clinical manifestation of chronic glucocorticoid- and/or mineralocorticoid deficiency due to failure of the adrenal cortex which may result in an adrenal crisis with potentially life-threatening consequences. Even though efficient and safe pharmaceutical preparations for the substitution of endogenous gluco- and mineralocorticoids are established in therapy, the mortality in patients with PAI is still increased and the health-related quality of life (HRQ...
Source: Experimental and Clinical Endocrinology and Diabetes - Category: Endocrinology Authors: Tags: Article Source Type: research
Publication date: Available online 26 October 2018Source: Joint Bone SpineAuthor(s): François Chasset, Christophe Richez, Thierry Martin, Alexandre Belot, Anne-Sophie Korganow, Laurent ArnaudAbstractSeveral conditions have clinical and laboratory features that can mimic those present in Systemic Lupus Erythematosus (SLE). Some of these “SLE mimickers” are very common, such as rosacea which can be mistaken for the butterfly rash, while others such as Kikuchi disease, type-1 interferonopathies, Castleman's disease, prolidase deficiency, angioimmunoblastic T-cell lymphoma, Evans' syndrome in the context of ...
Source: Joint Bone Spine - Category: Orthopaedics Source Type: research
Purpose of review A comparative description of dysregulatory syndromes with mutations in signal transducer and activator of transcription (STAT) genes. Recent findings STAT 1, 3 and 5b loss of function (LOF) and gain of function (GOF) mutations are a heterogeneous group of genetic disorders that range from immunodeficiency (ID) to autoimmune disease (AID), depending on the underlying signalling pathway defect. Between them, there are clear overlapping and differences in clinical presentation and laboratory findings. Summary Dysregulatory syndromes due to LOF and GOF mutations in STAT1, 3 and 5b are a particular gro...
Source: Current Opinion in Pediatrics - Category: Pediatrics Tags: ALLERGY, IMMUNOLOGY AND RELATED DISORDERS: Edited by Jordan S. Orange Source Type: research
The U.S. Food and Drug Administration approved on Wednesday Catalyst Pharmaceuticals Inc's drug to treat a rare autoimmune disease.
Source: Reuters: Health - Category: Consumer Health News Tags: healthNews Source Type: news
ConclusionOur study shows a wide diversity of etiologies of LE in Morocco with essentially an acute mode of onset of symptoms.
Source: Brain and Behavior - Category: Neurology Authors: Tags: ORIGINAL RESEARCH Source Type: research
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