Fig. 2

A Quality assessment of 23 studies reporting on cases with PPPK1 with a history of malignancy (Y: yes. N: no. P: perhaps. NR: not relevant. (a) Group of patients without palmar lesions. (b) Sex. (c) ‘all available members were interviewed using a questionnaire including a pedigree drawing in order to link closely related families. The questionnaire was conducted to collect family data…and family history regarding the disease comorbidity’ [12]. (d) [affected and a number of unaffected individuals]’…were interviewed and examined by two physicians’ [31] (e) In the article by Mamai et al., the following information appears in the text ‘Person marked by asterisk are died by different types of cancers’ [26]. However, we could not find any asterisk symbols. (f) Familial cancer in the general Canadian population. (g) Family members without PPPK1. (h) Arsenic exposure (i) Statistical calculation of probability of two rare conditions in two family members). B Quality assessment of the 22 studies reporting on individuals with PPPK1 without history of malignancy (Y: yes. N: no. P: perhaps. NR: not relevant. (a) Only subjects from the cohort with disease-causing variants in AAGAB are entered in the figure as information on malignancy status was only available for this subgroup of subjects in the study. (b) ‘The data were entered into prepared questionnaires ’[46]).