Table 4.

Clinical characteristics of monogenic very-early-onset inflammatory bowel disease patients

Patient	Sex	Disease onset	Z-score of height	Z-score of body weight	Chief complaints	Others	Disease locations and behavior	Clinical and genetic diagnosis	Intestinal surgery	Treatment	Prognosis
1	M	< 1 mo	0.22	-1.18	Diarrhea, bloody stools	Pyoderma	L2, P, B2B3	CD, IL10RB mutation	Colostomy	Steroid, IFX, thalidomide, 6-MP/MTX/AZA	Remission
2	F	4 mo	-1.76	-0.96	Diarrhea, bloody stools	Pyoderma	L2,L4b, P, B2B3	CD, IL10RA mutation	Intestinal perforation repair, colostomy and J-POUCH	Steroid, mesalazine, IFX, MTX/CsA	Remission
3	M	1 mo+	-0.79	-2.21	Diarrhea, bloody stools	Elevation of ALT	L2, P, B2	CD, IL10RA mutation	None	None	Waiting HSCT
4	M	1 mo+	-3.11	-3.66	Diarrhea, bloody stools	None	L2, P, B1	IBDU, IL10RA mutation	None	Antibiotics	Give up
5	M	11 d	-4.92	-3.38	Diarrhea, bloody stools	NEC	L2,L4b, P, B1	CD, IL10RA mutation	None	Steroids, AZA	Give up
6	M	< 1 mo	0.46	-1.08	Diarrhea, bloody stools	Epilepsy	L2, P, B1	IBDU, CGD	None	None	Remission
7	M	< 1 mo	-0.84	-2.04	Persistent fever	Intestinal malrotation, elevation of ALT	L2, P, B1	IBDU, CGD	None	None	Dead
8	M	9 mo	-4.63	-2.95	Diarrhea, bloody stools	Hepatosplenomegaly	L2, B1	CD, CVID	Intestinal resection and anastomosis	Steroid, 6MP, mesalazine, thalidomide	Partial remission
9	M	5 yr 11 mo	-2.2	-1.57	Abdominal pain, diarrhea, bloody stools	None	L2, L4b	CD, XIAP deficiency	None	DXM, IFX, 6MP/AZA	Remission

6-MP: 6-mercaptopurine; ALT: Alanine aminotransferase; AZA: Azathioprine; CGD: Chronic granulomatous disease; CsA: Cyclosporin A; CVID: Common variable immunodeficiency; HSCT: Hematopoietic stem cell transplantation; IFX: Infliximab; IL10R: Interleukin 10 receptor; MTX: Methotrexate; XIAP: X-linked inhibitor of apoptosis protein.