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161. PSMB8 Encoding the β5i Proteasome Subunit Is Mutated in Joint Contractures, Muscle Atrophy, Microcytic Anemia, and Panniculitis-Induced Lipodystrophy Syndrome. (Full text)

PSMB8 Encoding the β5i Proteasome Subunit Is Mutated in Joint Contractures, Muscle Atrophy, Microcytic Anemia, and Panniculitis-Induced Lipodystrophy Syndrome. We performed homozygosity mapping in two recently reported pedigrees from Portugal and Mexico with an autosomal-recessive autoinflammatory syndrome characterized by joint contractures, muscle atrophy, microcytic anemia, and panniculitis-induced lipodystrophy (JMP). This revealed only one homozygous region spanning 2.4 Mb (5818 SNPs

2010 American Journal of Human Genetics PubMed

162. An Autosomal Recessive Syndrome of Joint Contractures, Muscular Atrophy, Microcytic Anemia, and Panniculitis-Associated Lipodystrophy. (Full text)

An Autosomal Recessive Syndrome of Joint Contractures, Muscular Atrophy, Microcytic Anemia, and Panniculitis-Associated Lipodystrophy. Genetic lipodystrophies are rare disorders characterized by partial or complete loss of adipose tissue and predisposition to insulin resistance and its complications such as diabetes mellitus, hypertriglyceridemia, hepatic steatosis, acanthosis nigricans, and polycystic ovarian syndrome.The objective of the study was to report a novel autosomal recessive (...) nigricans. Two had mild hypertriglyceridemia and all had low levels of high-density lipoprotein cholesterol. Skin biopsy of an erythematous nodular skin lesion from one of the patients revealed evidence of panniculitis. The lipodystrophy initially affected the upper body but later became generalized involving abdomen and lower extremities as well.We conclude that these patients represent a novel autoinflammatory syndrome resulting in joint contractures, muscle atrophy, microcytic anemia

2010 Journal of Clinical Endocrinology and Metabolism PubMed

163. Alpha-1 antitrypsin deficiency

known as Wegener's granulomatosis) and necrotising panniculitis are infrequent complications but can prompt diagnosis. Plasma AAT levels, protein phenotyping (called Pi-typing), and protein genotyping may be necessary for diagnosis. Rare alleles may require gene sequencing. Intravenous AAT augmentation therapy benefits some patients. Definition Alpha-1 antitrypsin (AAT) deficiency is an autosomal codominant genetic disorder (i.e., 1 allele is inherited from each parent and each allele is expressed

2018 BMJ Best Practice

164. Aggressive subcutaneous panniculitis-like T-cell lymphoma with hemophagocytosis in two children (subcutaneous panniculitis-like T-cell lymphoma). (PubMed)

Aggressive subcutaneous panniculitis-like T-cell lymphoma with hemophagocytosis in two children (subcutaneous panniculitis-like T-cell lymphoma). Subcutaneous panniculitis-like T-cell lymphoma is an uncommon form of cutaneous lymphoma in the pediatric population. It is characterized histologically by subcutaneous infiltration of pleomorphic cytotoxic T cells, mimicking a lobular panniculitis. Although usually described as having an indolent clinical course, the condition may be complicated (...) by systemic involvement and hemophagocytic syndrome, resulting in a poorer prognosis. We present two pediatric patients with aggressive forms of subcutaneous panniculitis-like T-cell lymphoma complicated by hemophagocytic syndrome, and discuss the current literature.

2009 Journal of American Academy of Dermatology

165. Erythema nodosum

disease, auto-immune disease, malignancy, and pregnancy. They are also related to the ingestion of certain medications. Histopathology demonstrates a septal panniculitis without vasculitis. Miescher's radial granulomas are a diagnostical feature, but are not necessary for confirmation. Treatment consists of supportive care, including bed rest and leg elevation, as well as non-steroidal anti-inflammatory drugs (NSAIDs), potassium iodide, and intralesional or oral corticosteroids. Nodules may also (...) are associated with extravated blood, resulting in the appearance of purpura. These ecchymotic areas fade into variably pigmented patches before resolving. As new crops of nodules may form weeks after the initial presentation, lesions at varying stages of resolution may coexist. Microscopy of these lesions demonstrates a septal panniculitis without vasculitis. Bondi EE, Margolis DJ, Lazarus ZS. Panniculitis. In: Freedberg I, Eisen A, Wolff K, et al, eds. Fitzpatrick's dermatology in general medicine. 5th ed

2017 BMJ Best Practice

166. Lymphoma

? Lymphocyte rich ? Lymphocyte depleted Peripheral T-cell, unspecified Angioimmunoblastic (AITL. formerly AILD) Enteropathy associated T-cell Hepatosplenic T-cell Subcutaneous panniculitis-like Anaplastic large cell (CD30+) ALK+ Anaplastic large cell (CD30+) ALK- Extranodal NK/T-cell, nasal type Special Burkitt lymphoma Intermediate between DLBCL and BL Intermediate between DLBCL and Hodgkin lymphoma B lymphoblastic leukemia/lymphoma B prolymphocytic leukemia Lymphomas associated with HIV infection

2016 CPG Infobase

169. Primary Cutaneous Lymphomas: ESMO Clinical Practice Guidelines

studies (CT and/or FDG-PET scans) [5]. In PCLs with a predominantly subcutaneous presentation, such as subcutaneous panniculitis-like T cell lymphoma (SPTCL) and primary cutane- ous gamma/delta T cell lymphoma (PCGD-TCL), FDG-PET is essential to evaluate the extent of disease. In patients with typical lymphomatoid papulosis (LyP) or primary cutaneous CD4 þ small/medium T cell lymphoproliferative disorder (LPD), CT and FDG-PET scans are not required. Bone marrow biopsy and V C The Author(s) 2018 (...) disease with patches/?at plaques. In stage IB, Table 1. WHO-EORTC classi?cation for cutaneous lymphomas CutaneousTcelllymphoma Mycosis fungoides (MF) Variants of MF • Folliculotropic MF • Pagetoid reticulosis • Granulomatous slack skin Se ´zary syndrome Primary cutaneous CD30 þ lymphoproliferative disorders • Primary cutaneous anaplastic large cell lymphoma • Lymphomatoid papulosis Subcutaneous panniculitis-like T cell lymphoma Extranodal NK/T cell lymphoma, nasal-type Primary cutaneous peripheral T

2018 European Society for Medical Oncology

171. Childhood Non-Hodgkin Lymphoma Treatment (PDQ®): Patient Version

anaplastic large cell lymphoma, subcutaneous panniculitis-like T-cell lymphoma, gamma-delta T-cell lymphoma, and . Mycosis fungoides rarely occurs in children and adolescents. Past treatment for cancer and having a weakened immune system affect the risk of having childhood non-Hodgkin lymphoma. Anything that increases your risk of getting a disease is called a . Having a risk factor does not mean that you will get cancer; not having risk factors doesn’t mean that you will not get cancer. Talk with your (...) or cells from a . Treatment options for cutaneous T-cell lymphoma Treatment options for panniculitis-like in children may include: . High- . ( ). . . . Treatment options for may include: , , or both. In children, treatment options for may include: Steroids applied to the skin. Retinoid therapy. Radiation therapy. (light therapy using ). To Learn More About Childhood Non-Hodgkin Lymphoma For more information from the about childhood non-Hodgkin lymphoma, see the following: For more childhood information

2018 PDQ - NCI's Comprehensive Cancer Database

172. Genetics of Skin Cancer (PDQ®): Health Professional Version

by inflammatory conditions such as panniculitis and malignancies such as liposarcoma.[ ] These compartments give rise to their own malignancies but are also the region of immediate adjacent spread of localized skin cancers from other compartments. The boundaries of each skin compartment are used to define the staging of skin cancers. For example, an in situ melanoma is confined to the epidermis. Once the cancer crosses the basement membrane into the dermis, it is invasive. Internal malignancies also commonly

2018 PDQ - NCI's Comprehensive Cancer Database

173. International Consolidated Venous Ulcer Guideline

resulting in fibrosing panniculitis of leg with severe, chronic venous insufficiency). A (Geyer et al., 2004; Navarro et al., 2002; Volikova et al., 2009) CVI= 1.000, High SOR A.2.b.v. Medial lower leg site, with slower healing in ulcers associated with VU location in posterior ankle or back of calf region. A (McGuckin et al., 2002; Pappas et al., 1997; Szewczyk et al., 2009; Yim et al., 2014), CVI= 0.762, High SOR Growth of hair indicates adequate arterial supply and likely VU healing outcome

2015 Association for the Advancement of Wound Care

175. Adult Non-Hodgkin Lymphoma Treatment (PDQ®): Health Professional Version

neoplasms Precursor T-cell neoplasm: precursor T-acute lymphoblastic leukemia/LBL. Peripheral T-cell and NK-cell neoplasms. T-cell CLL/prolymphocytic leukemia. T-cell granular lymphocytic leukemia. Mycosis fungoides (including Sézary syndrome). Peripheral T-cell lymphoma, not otherwise characterized. Hepatosplenic gamma/delta T-cell lymphoma. Subcutaneous panniculitis-like T-cell lymphoma. Angioimmunoblastic T-cell lymphoma. Extranodal T-/NK-cell lymphoma, nasal type. Enteropathy-type intestinal T-cell (...) -cell, diffuse large cell, immunoblastic, and T-cell rich large B-cell lymphoma). Distinguish: Mediastinal large B-cell lymphoma. Follicular large cell lymphoma (grade 3). Anaplastic large cell lymphoma (CD30-positive). Extranodal NK-/T-cell lymphoma, nasal type/aggressive NK-cell leukemia/blastic NK-cell lymphoma. Lymphomatoid granulomatosis (angiocentric pulmonary B-cell lymphoma). Angioimmunoblastic T-cell lymphoma. Peripheral T-cell lymphoma, unspecified. Subcutaneous panniculitis-like T-cell

2018 PDQ - NCI's Comprehensive Cancer Database

176. Compression stockings as an effective treatment for erythema nodosum: Case series (Full text)

Compression stockings as an effective treatment for erythema nodosum: Case series Erythema nodosum (EN) is a septal panniculitis that is characterized clinically by tender, erythematous, subcutaneous nodules that are predominately localized on the pretibial lower legs. EN affects women more than men and can be idiopathic or secondary to another disease process such as infection or an immune response. Treatment options for erythema nodosum are suboptimal and often involve significant side

2017 International journal of women's dermatology PubMed

177. Inflammatory skin disorders and self-esteem (Full text)

inflammatory conditions panniculitis quality of life self-esteemmental healthinflammatory skinpsoriasisatopic dermatitiseczema 2017 09 01 2017 09 25 2018 6 7 6 0 2018 6 7 6 0 2018 6 7 6 1 epublish 29872672 10.1016/j.ijwd.2017.09.006 S2352-6475(17)30079-5 PMC5986113 JAMA Dermatol. 2015 Jul;151(7):743-52 25738422 J Pediatr. 2016 Feb;169:284-90.e5 26616249 J Cutan Med Surg. 2010 Nov-Dec;14(6):285-90 21084021 Arch Dermatol. 2005 Jan;141(1):19-26 15655138 J Invest Dermatol. 2017 Jan;137(1):26-30 27616422

2017 International journal of women's dermatology PubMed

178. Istodax for Peripheral T-Cell Lymphoma

subtypes are PTCL Not Otherwise Specified (PTCL NOS), anaplastic large cell lymphoma (ALCL) and angioimmunoblastic T cell lymphoma (AITL). ALCL can be further subclassified based on the presence of ALK expression, which confers a superior prognosis. Other PTCL subtypes include hepatosplenic T-cell lymphoma, enteropathy-associated T-cell lymphoma, subcutaneous panniculitis-like T cell lymphoma, rare subtypes of primary cutaneous T cell lymphoma (not to be confused with mycosis fungoides), and extranodal

2015 Canadian Agency for Drugs and Technologies in Health - Rapid Review

180. Relationship between abdominal trauma or surgery and mesenteric panniculitis (Full text)

Relationship between abdominal trauma or surgery and mesenteric panniculitis Mesenteric panniculitis is a rare disease characterized by chronic non-specific inflammation of mesenteric fat tissue. Several etiologic and/or associated factors have been reported in the literature so far. Although trauma or surgery is one of the potential etiologic factors for mesenteric panniculitis, to the best of our knowledge, no strong correlation has been shown in the literature until now.

2009 World journal of gastroenterology : WJG PubMed

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