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Table 1 Case series reporting the outcome after tonsillectomy and adenotonsillectomy in children with the PFAPA syndrome

From: The role of tonsillectomy in the Periodic Fever, Aphthous stomatitis, Pharyngitis and cervical Adenitis syndrome; a literature review

 

Author/year (reference)

Number of children

Curative

Partly effective

Not effective

Observation post surgery (months, mean)

Adeno-tonsillectomya

Diagnosis set prior to surgery

Criteria for PFAPA diagnosisb

Quality

1

Abramson et al. 1989 [14]

4

4

15

3 (75%)

No

Marshall

Low

2

Padeh et al. 1999 [3]

3

3

Not clear

0

Yes

Marshall

Low

3

Dahn et al. 2000 [30]

5

5

3

5 (100%)

No

Adapted

Low

4

Galanakis et al. 2002 [31]

15

15

Not clear

0

No

Thomas

Low

5

Berlucchi et al. 2003 [32]

5

5

10

2 (40%)

Yes

Adapted

Low

6

Parikh et al. 2003 [33]

2

0

2

Not clear

0

No

Marshall

Low

7

Tasher et al. 2006 [5]

6

6

19

0

Yes

Thomas

Low

8

Wong et al. 2008 [34]

9

8

1

24

0

Yes

Marshall

Medium

9

Pignataro et al. 2009 [35]

9

5

4

26

0

Yes

Marshall

Medium

10

Fedrer et al. 2010 [36]

11

11

18

0

Yes

Thomas

Low

11

Peridis et al. 2010 [37]

9

8

1

12

2 (22%)

Yes

Thomas

Low

12

Wurster et al. 2011 [7]

12

6

3

3

Not clear

10 (83%)

Yes

Thomas

Low

13

Licameli et al. 2012 [38] c

102

99

3

43

102 (100%)

Yes

Marshall

High

14

Førsvoll et al. 2013 [29]

17

17

Not clear

7 (41%)

Yes

Thomas

Low

15

Krol et al. 2013 [39]

18

18

Not clear

0

Yes

Thomas

Low

16

Ter Haar et al. 2013 [40]

8

4

3

1

Not clear

Not clear

Yes

Marshall

Low

17

Valenzuela et al. 2013 [41]

9

9

10

0

Yes

Thomas

Low

18

Kubota et al. 2014 [42]

5

4

1

Not clear

0

Yes

Thomas

Low

19

Vigo et al. 2014 [43]

41

27

14

69

0

Yes

Marshall

High

20

Dytrych et al. 2015 [44]

10

10

19

0

Yes

Thomas

Low

21

Førsvoll et al. 2015 [45]

4

4

27

1 (25%)

Yes

Thomas

Medium

22

Lantto et al. 2015 [46]d

31

31

6

11 (35%)

No

Adapted

Low

23

Perko et al. 2015 [47]

28

26

2

Not clear

0

Yes

Thomas

Medium

24

Batu et al. 2016 [48]c

53

50

3

Not clear

0

Yes

Thomas

Medium

25

Dusser et al. 2016 [49]

4

3

1

Not clear

Not clear

Yes

Thomas

Low

26

Erdogan et al. 2016 [50]

75

74

1

24

Not clear

Yes

Thomas

High

27

Lantto et al. 2016 [22]d

58

56

2

107

0

No

Thomas

Medium

28

Rigante et al. 2016 [51]

2

1

1

Not clear

0

Yes

Thomas

Low

 

Total

555

509

14

32

143 (26%)

  
  1. PFAPA Periodic Fever, Aphthous stomatitis, Pharyngitis and cervical Adenitis
  2. a:Adenotonsillectomy performed instead of tonsillectomy alone
  3. b:PFAPA diagnosis was set according to criteria by Marshall [13], Thomas [4] or adapted criteria not clearly based on either of the two
  4. cand d:Possible overlap between study populations