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Table 1 Inclusion and exclusion criteria for patients with PFAPA

From: Tonsillectomy reduces the family impact of periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome and improves health-related quality of life in affected children

Inclusion criteria

Exclusion criteria

Fulfil the modified Marshall criteria of PFAPA [1]:

 I. Regularly recurring fevers with an early onset (< 5 years of age)

 II. Constitutional symptoms in the absence of upper respiratory infection with at least 1 of the following clinical signs:

  (a) Aphthous stomatitis

  (b) Cervical lymphadenitis

  (c) Pharyngitis

 III. Exclusion of cyclic neutropenia

 IV. Completely asymptomatic between episodes

 V. Normal growth and development

Duration of fever episodes less than 7 days

Age up to 7 years at the time of inclusion

In association with fever episodes had any of the following:

 Skin rash

 Arthritis

 Severe abdominal pain

 Diarrhoea

 Thoracic pain

 Spleen enlargement

 Sensorineural hearing impairment

Cold-induced symptoms

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  1. Inclusion criteria were based on the modified Marshall criteria by Thomas et al. [1]. To ensure a homogenous study population, limits to the length of fever episodes and age at inclusion, as well as exclusion criteria for atypical symptoms, were added
  2. PFAPA: Periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis