Variable outcome in infantile-onset inflammatory bowel disease in an Asian cohort

Table 1 Phenotypic characteristics, disease behavior, therapy and outcome in six Asian children with infantile-onset inflammatory bowel disease

Patients’ initials	No. 1	No. 2	No. 3	No. 4	No. 5	No. 6
Sex	Male	Male	Male	Male	Female	Female
Ethnicity	Chinese	Chinese	Malay	Chinese	Indian	Malay
Consanguinity	No	No	No	No	No	No
Breast feeding (duration)	No	No	No	Yes (3 mo)	Yes (2 mo)	Yes (4 mo)
Age at onset	First week	12 mo	7 mo	2 mo	6 mo	4 mo
Disease phenotype	EC→UC	UC	CD	CD	CD	IBD-U
Major symptoms at presentation	Bloody diarrhea and PR bleeding	Bloody diarrhea, anemia	Oral ulcers, bloody diarrhea, abdominal pain	Bloody diarrhea, abdominal pain	Bloody diarrhea, growth faltering	Bloody diarrhea
Perianal disease	Nil	Nil	Abscess and fistula	Abscess and fistula	Nil	Abscess and fistula
Other medical or autoimmune conditions	Nil	Nil	Nil	Nil	Nil	Nil
Recurrent infections	Nil	Nil	Congenital CMV infection,	Nil	Nil	Nil
Disease location1	E4S1	E4	L3L4a	L3L4a	L3	L2
Disease behavior/severity1	S1	S1	B2B3p	B2B3p	B1	B1p
Histopathology	Dense lymphoplasmacytic and eosinophilic infiltration of the lamina propria involving the gastric mucosa, duodenum and colonic mucosa.	Colonic mucosa showed mild degenerative atypia, cryptitis and crypt abscesses. Lamina propria showed increase in neutrophilic and lymphoplasmacytic infiltration.	Lymphocytic infiltration of lamina propria. No granuloma or crypt abscess seen. The duodenum showed chronic inflammation.	Lymphocytic infiltration of lamina propria. No granuloma or crypt abscess seen	Mild inflammation in the lamina propria with lymphocytes and plasma cells. No granuloma or crypt abscess	First biopsy: transmural inflammation involving all layers of bowel wall, including the skeletal muscle with dense lymphoplasmacytic infiltration. No crypt abscess seen.
						Second biopsy: colonic mucosa inflammed but the architecture was preserved. Significant lymphoplasmacytic and neutrophilic infiltration mainly confined to the lamina propria
Other associated diseases	Nil	Nil	Developmental regression at 6 yr age	Nil	Nil	Nil
Mutational analysis	Not done	Not done	Not detected	Not detected	Not detected	Not detected
Medical therapy	CS, CsA, enteral nutrition	CS, Aza	CS, Aza, IFX × 24 doses	EN, CS, Aza, IFX × 14 doses	Aza, IFX × 3 doses	Nil
Surgery	Total colectomy at 18 mo	Nil	Ileostomy at 6 yr of age	Nil	Nil	Ileostomy; closure at 18 mo of age
Age at last follow up	21 yr	6 yr	9 yr	6 yr	13 yr	3 yr
Final clinical status	Alive, deafness due to aminoglycosides. in remission, off therapy for 18 yr	Alive, in remission; on Aza	Alive, persistent disease, on CS, Aza. Developmentally delayed.	Alive, persistent disease, on CS, Aza and IFX. Parents refused surgery	Alive, in remission; off therapy for 2 yr	Alive, in remission; no therapy

¹According to Levine et al[24]. Aza: Azathioprine; CD: Crohn’s disease; CMV: Cytomegalovirus; CS: Corticosteroid; CsA: Cyclosporin; EC: Eosinophilic colitis; EN: Enteral nutrition; IFX: Infliximab; PR: Per rectal; UC: Ulcerative colitis.