. 2015 Mar;135(3):e730–e735. doi: 10.1542/peds.2014-2268

TABLE 1.

Summary of Clinical Investigations

	P1	P2	P3	P4	P5	P6	Foeldvari et al 1995⁵	Karatzios et al 2006⁶	Onal et al 2007⁷	Dowden et al 2009⁸	Sion-Sarid et al 2010⁹	Gousseff et al 2011³	Piastra et al 2012¹⁰	Perme et al 2013¹¹	Iwasa et al 2014¹²
Current age, y^a	9	5	11	8	8	9	9	6	7	6	8	5	9	11	11
Age at first episode	8 y	2y	5 y	3 y	4.5 y	3 y	3 y	5 y	5 m	6 y	5 m	5 y	6 y	8 y	10 y
Gender	M	F	F	F	M	F	F	F	M	M	M	F	F	M	F
Number of severe acute episodes^b	1	3	1	2	1	4	5	2	3	1	5	2	2	2	2
Mild episodes^c	1	No	Multiple with viral illness	1	2	2	0	0	0	0	0	2	0	0	0
Infectious trigger (NPA or throat swab)	Rhinovirus parainfluenza 3	Respiratory syncytial virus influenza B	Influenza A	Rhinovirus, parainfluenza, paronychia	Influenza A	Group A Streptococcus	Unknown	Influenza A (H3N2)	Unknown	Unknown	Unknown	Unknown	Unknown	Influenza A (H3N2 and H1N1)	Unknown
Prodrome	Lethargy, coryzal symptoms, abdominal and leg pain, thirst, vomiting	Coryzal Sx, vomiting, fever	Fever, lethargy, abdominal and leg pain, thirst, vomiting	Fever, coryzal symptoms, headache, periorbital swelling, cough, abdominal pain, vomiting	Fever, lethargy, sore throat, periorbital swelling, abdominal pain, vomiting, diarrhea	Cough, periorbital swelling	Cough, fever anorexia	Fever, abdominal pain, vomiting	Vomiting, diarrhea	Fever, vomiting	Abdominal pain, vomiting	Unknown	Fever, cough	Fever, coryzal symptoms, periorbital swelling	Fever, abdominal pain, diarrhea
Leak phase	3 Hs^d	3 Hs^d	3 Hs^d	3 Hs^d	3 Hs^d	3 Hs^d	3 Hs^d	3 Hs^d	3 Hs^d	3 Hs^d	3 Hs^d	3 Hs^d	3 Hs^d	3 Hs^d	3 Hs^d
Postleak diuresis	Yes, pulmonary edema	Yes, pulmonary edema	Yes	Yes, pulmonary edema	Yes, pulmonary edema requiring extracorporeal membrane oxygenation	Unknown	Unknown	Unknown	Yes	Unknown	Yes, pulmonary edema	Unknown	Yes, pulmonary edema	Unknown	Yes, pulmonary edema
Nonfatal complications	NA	Pericardial effusion	Compartment syndrome Rhabdomyolysis	Pleural effusion	Cardiac arrest, pericardial and pleural effusion, ascites	Compartment syndrome, Rhabdomyolysis, peroneal neuropathy	No	Compartment syndrome, Rhabdomyolysis	No	Compartment syndrome	Cerebral edema, acute renal failure	Unknown	Rhabdomyolysis	Rhabdomyolysis, renal failure	Compartment syndrome, fasciotomies, rhabdomyolysis, ascites, pleural and pericardial effusion, ARF requiring CHDF
Acute CRP (mg/L)	10	12	14.7	22	ND	Unknown	Unknown	8	Unknown	Unknown	Normal	Unknown	Normal	<3	32.4
Acute complements (C3, C4)	Low C3, C4	Normal	Low C3, C4	Low C3, C4	Low C3	Unknown	Low C3	Unknown	Normal	Unknown	Unknown	Unknown	Unknown	Unknown (baseline normal)	Unknown
Acute CH50	Normal	Normal	Low	ND	ND	Unknown	Unknown	Unknown	Unknown	Unknown	Unknown	Unknown		Unknown	Unknown
C1 esterase inhibitor	Normal	Normal	Normal	Normal	ND	Normal	Normal	Normal	Normal	Unknown	Unknown	Unknown	Normal	Normal	Unknown
Serum Tryptase	Normal	Normal	ND	Increased (17.6)	ND	Unknown	Unknown	Unknown	Unknown	Unknown	Unknown	Unknown	Normal	Unknown	Unknown
Monoclonal gammopathy	No	No	No	No	No	No	No	Unknown	No	Unknown	No	Unknown	Unknown	No	Unknown
Acute Igs (g/L)	Low IgG (4.96) and IgM (0.28)	Normal	Low IgG (4.61)	Low IgG (3.2) and IgM (0.36)	Low IgG (4.3)	Unknown	Low IgG	Unknown	Unknown	Unknown	Unknown	Unknown	Normal	Unknown (baseline normal)	Unknown
ANA titer	Negative	160 speckled	2560 speckled	Negative	Negative	Unknown	Negative	Unknown	Unknown	Unknown	Unknown	Unknown	Negative	Unknown	Unknown
Prophylactic therapy	SCIG	IVIG	No	SCIG	IVIG	Theophylline, verapamil	Unknown	Unknown	Theophylline, ginkgo biloba	No	Unknown	Unknown	No	No	Theophylline, terbutaline
Outcome^a	Alive	Alive	Alive	Alive	Alive	Alive	Alive	Alive	Alive	Dead	Alive	Alive	Alive	Alive	Alive

ND, not done.

^{^a}

At the time of publication.

^{^b}

Acute episodes presenting as shock requiring fluid resuscitation and/or PICU admission.

^{^c}

Acute episodes of swelling without shock.

^{^d}

Hypotension, hemoconcentration, hypoalbuminemia.