The Chromones: Cromolyn Sodium and Nedocromil Sodium

Edwards, Alan M.; Holgate, Stephen T.

doi:10.1016/b978-0-323-05659-5.00090-5

Cited by 3 publications

(5 citation statements)

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Section: Discussionmentioning

confidence: 99%

“…Cromoglicic acid (also known as cromoglycate or cromolyn), which is marketed as a sodium salt (disodium cromoglycate, DSCG, or cromolyn sodium), belongs to the group of chromones and is a mast cell stabilizer that prevents the release of MC mediators [ 32 , 33 ]. Its mechanism of action remains poorly understood [ 32 , 33 ]. DSCG has minimal systemic absorption following ingestion and negligible fat solubility, suggesting an inability to enter the cells and an interaction with a cell surface receptor [ 32 , 33 ].…”

Section: Discussionmentioning

confidence: 99%

“…Its mechanism of action remains poorly understood [ 32 , 33 ]. DSCG has minimal systemic absorption following ingestion and negligible fat solubility, suggesting an inability to enter the cells and an interaction with a cell surface receptor [ 32 , 33 ]. In permeabilized mast cells, DSCG inhibits GTP-gamma-S-induced secretion by a mechanism not involving the nucleoside diphosphate kinase [ 34 ].…”

Section: Discussionmentioning

confidence: 99%

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Systemic mastocytosis with KIT V560G mutation presenting as recurrent episodes of vascular collapse: response to disodium cromoglycate and disease outcome

Conde-Fernandes¹,

Sampaio²,

Moreno³

et al. 2017

Allergy Asthma Clin Immunol

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BackgroundMastocytosis are rare diseases characterized by an accumulation of clonal mast cells (MCs) in one or multiple organs or tissues. Patients with systemic mastocytosis (SM), whose MCs frequently arbor the activating D816V KIT mutation, may have indolent to aggressive diseases, and they may experience MC mediator related symptoms. Indolent SM with recurrent anaphylaxis or vascular collapse in the absence of skin lesions, ISMs(−), is a specific subtype indolent SM (ISM), and this clonal MC activation disorder represents a significant fraction of all MC activation syndromes. The V560G KIT mutation is extremely rare in patients with SM and its biological and prognostic impact remains unknown.Case presentationA 15-year old boy was referred to our hospital because of repeated episodes of flushing, hypotension and syncope since the age of 3-years, preceded by skin lesions compatible with mastocytosis on histopathology that had disappeared in the late-early childhood. Diagnosis of ISM, more precisely the ISMs(−) variant, was confirmed based on the clinical manifestations together with increased baseline serum tryptase levels and the presence of morphologically atypical, mature appearing (CD117+high, FcεRI+) phenotypically aberrant (CD2+, CD25+) MCs, expressing activation-associated markers (CD63, CD69), in the bone marrow. Molecular genetic studies revealed the presence of the KIT V560G mutation in bone marrow MCs, but not in other bone marrow cells, whereas the screening for mutations in codon 816 of KIT was negative. The patient was treated with oral disodium cromoglycate and the disease had a favorable outcome after an eleven-year follow-up period, during which progressively lower serum tryptase levels together with the fully disappearance of all clinical manifestations was observed.ConclusionsTo the best of our knowledge this first report of a patient with ISM, whose bone marrow MCs carry the KIT V560G activating mutation, manifesting as recurrent spontaneous episodes of flushing and vascular collapse in the absence of skin lesions at the time of diagnosis, in whom disodium cromoglycate had led to long term clinical remission.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Systemic mastocytosis with KIT V560G mutation presenting as recurrent episodes of vascular collapse: response to disodium cromoglycate and disease outcome

Conde-Fernandes¹,

Sampaio²,

Moreno³

et al. 2017

Allergy Asthma Clin Immunol

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“…Cromolyn sodium is an MC stabilizer that has been proven to inhibit MC activation and MC release of mediators both in vitro and in vivo despite its limited systemic absorption following ingestion; this suggests an inability of the drug to enter cells and the need for a potential interaction with an as-yet-unidentified cell surface receptor to induce its biologic activity 43 ( Table 6). Side effects include headache, sleepiness, irritability, abdominal pain, diarrhea, and constipation, most of which are attenuated by progressive introduction of the drug 44 .…”

Section: Treatment Of Primary Mast Cell Activation Syndromesmentioning

confidence: 99%

Advances in the understanding and clinical management of mastocytosis and clonal mast cell activation syndromes

et al. 2016

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Clonal mast cell activation syndromes and indolent systemic mastocytosis without skin involvement are two emerging entities that sometimes might be clinically difficult to distinguish, and they involve a great challenge for the physician from both a diagnostic and a therapeutic point of view. Furthermore, final diagnosis of both entities requires a bone marrow study; it is recommended that this be done in reference centers. In this article, we address the current consensus and guidelines for the suspicion, diagnosis, classification, treatment, and management of these two entities.

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“…In vitro evidence suggests it diminishes the calcium flux necessary for degranulation following FcER1 cross-linking, and it is thus purported as a MC stabilizer. Its mechanism of action remains unclear, however, as it has minimal systemic absorption following ingestion, and negligible fat solubility plus extensive ionization at physiologic pH (which suggests an inability of the drug to enter cells, thus implicating an interaction with an as-yet-unidentified cell surface receptor for cromolyn to have biologic activity) [24]. Early case reports stressed its usefulness in treating mastocytotic bone disease while acknowledging the minimal absorption of the drug from the GI tract [25].…”

Section: Antimediator Therapymentioning

confidence: 99%

Mastocytosis: update on pharmacotherapy and future directions

Cardet

Akin

Lee

2013

Expert Opinion on Pharmacotherapy

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Introduction Mastocytosis is a disorder characterized by abnormal mast cell (MC) accumulation in skin and internal organs such as bone marrow. The disease follows a benign course in most patients with cutaneous and indolent systemic mastocytosis (SM); however, advanced variants associated with decreased life expectancy also exist. Pharmacotherapy of mastocytosis is aimed at the control of symptoms caused by MC mediator release, treatment of comorbidities and cytoreductive therapies in advanced variants. Areas covered This article will cover the general treatment principles of anti-MC mediator and cytoreductive therapies of mastocytosis. The literature discussed was retrieved with PubMed using the search terms ‘treatment of mastocytosis,’ ‘mastocytosis antimediator therapy’ and looking for important cross-references. Expert opinion Pharmacotherapy of mastocytosis should be individualized for each patient considering the category of disease, reduction of risk of anaphylaxis, constitutional symptoms and comorbidities including osteoporosis. Cytoreductive therapies are generally reserved for patients with aggressive mastocytosis (ASM), MC leukemia (MCL) and MC sarcoma (MCS); however, some patients with indolent disease and recurrent anaphylactic episodes not responsive to antimediator therapies may also be considered for cytoreduction on a case-by-case basis.

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The Chromones: Cromolyn Sodium and Nedocromil Sodium

Cited by 3 publications

References 78 publications

Systemic mastocytosis with KIT V560G mutation presenting as recurrent episodes of vascular collapse: response to disodium cromoglycate and disease outcome

Systemic mastocytosis with KIT V560G mutation presenting as recurrent episodes of vascular collapse: response to disodium cromoglycate and disease outcome

Advances in the understanding and clinical management of mastocytosis and clonal mast cell activation syndromes

Mastocytosis: update on pharmacotherapy and future directions

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