Implication of overexpression of dishevelled-associated activator of morphogenesis 1 (Daam-1) for the pathogenesis of human Idiopathic Pulmonary Arterial Hypertension (IPAH)

Table 1 Characteristics of autopsy cases

A. PAH Cases
Patient	Diagnosis	H-E Grade	Age (years)	Sex	Underlying conditions
1^a	APAH	II	4 months	Female	DORV and VSD
2^a	APAH	II	5 months	Female	Trisomy 18 and VSD
3^a	APAH	II	6 months	Female	DORV and VSD
4^a	APAH	II	7 months	Female	Trisomy 21, ASD, and VSD
5	IPAH	III	13	Female
6	IPAH	IV	16	Female
7^a	IPAH	IV	17	Female
8^a	IPAH	IV	19	Female
9^a	IPAH	III	21	Female
10	IPAH	IV	24	Male
11	APAH	VI	29	Male	Collagen disease
12	IPAH	IV	39	Female
13	APAH	IV	41	Male	Malignant lymphoma
14^a	IPAH	III	44	Female
15^a	IPAH	IV	51	Female
16^a	APAH	III	63	Male	Collagen disease
B. Age-matched controls
Patient	Age (years)	Sex	Underlying conditions
17	3	Female	Pneumonia
18^a	4	Male	Leukemia
19^a	11	Female	Biliary atresia
20	14	Female	Leukemia
21^a	14	Male	Leukemia
22^a	15	Male	Leukemia
23^a	16	Male	Hypertrophic cardiomyopathy
24	17	Male	Idiopathic myocarditis
25^a	18	Male	Ewing sarcoma
26^a	22	Female	Suicide
27^a	23	Female	Cerebral hemorrhage
28^a	29	Male	Chronic hepatitis
29	42	Male	Anterior mediastinal tumor
30	43	Female	Breast cancer
31^a	47	Female	Gastric cancer
32^a	56	Female	Subacute hepatitis
C. Summary
	No.	Median age	Males:females
PAH cases	16	20 y (4 m - 63 y)	4:12
IPAH	9	21 y (13 y - 51 y)	1:8
APAH	7	7 m (4 m - 63 y)	3:4
Age-matched controls	16	18 y (3 y - 56 y)	8:8

Cases are in ascending order of age
H-E Grade, Heath–Edwards grade ³⁶; DORV, double outlet right ventricle; VSD, ventricular septal defect; ASD, atrial septal defect
^a indicates that positive reactivity against both anti–Dvl-2 antibody and anti–Daam-1 antibody was confirmed in built-in control before further evaluation

ISSN: 1746-1596