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Table 1.

Characteristics of index patients, their first-degree relatives, and healthy controls.

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Table 2.

Prevalence of MCAD in first-degree relatives and healthy subjects (A) and within the first-degree relatives group (B).

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Table 2 Expand

Table 3.

Odds ratios for the symptoms and findings collected in the questionnaire (independent variables) occurring in patients, compared with the healthy control group.

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Table 3 Expand

Table 4.

Odds ratios for the symptoms and findings collected in the questionnaire (independent variables) occurring in first-degree relatives, compared with the healthy control group.

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Table 4 Expand

Figure 1.

Pedigree of a family with a high familial loading of mast cell activation disease.

Filled symbols indicate family members with either systemic mastocytosis (SM), according to WHO criteria, or mast cell activation syndrome (MCAS); open symbols denote clinically healthy family members. Squares, males; circles, females; symbols with a diagonal line through, deceased. Mild, moderate and severe indicate the clinical intensity of the mast cell activation disease. The arrow indicates the index patient. Bold type: genetic alterations detected in the tyrosine kinase, KIT. GNNK(–/+): ratio of the GNNK(–) over the GNNK(+) isoform (i.e., the amount of PCR amplification product without and with the tetrapeptide sequence glycine-asparagine-asparagine-lysine [GNNK]).

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Figure 1 Expand