A Retrospective Study on the Clinical, Laboratory, and Nutritional Status of Pediatric Epidermolysis Bullosa in a Tertiary Referral Hospital in West Java, Indonesia

Figures & data

Table 1 Clinical Factors Affecting Nutritional Status

Variable	EBS	JEB	DEB		Total (n)
			RDEB	DDEB
Skin involvement (n=12)
Percentage of EB wound area (BSA)
<10%	0	0	1	0	1
10–20%	2	3	1	2	8
>20%	0	0	2	1	3
Percentage of infected EB wound area (BSA)
No infection	0	1	2	1	4
<10%	2	2	2	2	8
10–20%	0	0	0	0	0
>20%	0	0	0	0	0
Gastrointestinal involvement (n=12)
Oral blisters or sores	0	1	3	1	5
Microstomia	0	0	1	0	1
Ankyloglossia	0	0	1	0	1
Tooth decay*	1	1	2	2	6
Disphagia	0	0	0	0	0
Odinophagia	0	1	3	1	5
Heartburn	0	0	0	0	0
Epigastric pain	0	0	0	0	0
Constipation	0	0	1	0	1
Painful defecation	0	0	1	0	1
Extraintestinal involvement (n=12)
Hand deformity
Yes	0	0	1	0	1
No	2	3	3	3	11
Pain when changing dressing
Yes	2	3	4	3	12
No	0	0	0	0	0

Note: *Only assessed in patients who have had teeth eruption.

Abbreviations: BSA, body surface area; EB, epidermolysis bullosa; n, noun.

Table 2 Laboratory and Nutritional Status in Pediatric EB Patients

Patient ID	Diagnosis	Age	Laboratory Examination								Nutritional Status
			Hemoglobin		Ferritin		Albumin		Zinc
			Level(g/dl)	Category	Level (ng/mL)	Category	Level (g/dl)	Category	Level (μg/dl)	Category	Z-scores	Category
1	JEB	9 days	13	Normal	N/A	N/A	N/A	N/A	56	Low	<-1 SD >-2	Normal
2	JEB	11 months	9,7	Anemia	82,8	Normal	2,9	Hypoalbuminemia	N/A	N/A	<-3 SD	Severe malnutrition
3	EBS	1 years	12,6	Normal	23	Normal	4,3	Normal	76	Normal	<-2 SD >-3	Moderate malnutrition
4	DDEB	2 years	12,4	Normal	N/A	N/A	4,5	Normal	80	Normal	2 SD	At risk of obese
5	EBS	4 years	13,3	Normal	42	Normal	3,6	Normal	39	Low	<-2 SD >-3	Moderate malnutrition
6	RDEB	5 years	14,3	Normal	37	Normal	4,1	Normal	59	Low	<2 SD >1	At risk of obese
7	DDEB	15 years	7,8	Anemia	2	Low	3,9	Normal	72	Normal	<-3 SD	Severe malnutrition
8	DDEB	5 months	12,7	Normal	33	Normal	4,1	Normal	121	Normal	2 SD	At risk of obese
9	RDEB	4 years	9,2	Anemia	5	Low	2,9	Hypoalbuminemia	57	Low	<-3 SD	Severe malnutrition
10	RDEB	11 years	9,7	Anemia	N/A	N/A	2,4	Hypoalbuminemia	60	Low	<2 SD >1	Overweight
11	JEB	28 days	9,5	Anemia	1337,8	Normal	1,63	Hypoalbuminemia	N/A	N/A	<-3 SD	Severe malnutrition
12	RDEB	1 years	11	Anemia	11	Low	4,1	Normal	59	Low	<-2 SD >-3	Moderate malnutrition

Abbreviations: DEB, Dystrophic EB; DDEB, Dominant DEB; EBS, EB Simplex; JEB, Junctional EB; N/A, not available, not examined; RDEB, Recessive DEB; SD, standard deviation.