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Erschienen in:
06.03.2019 | Scientific Letter
Recurrent Infection Due to Phagocyte Dysfunction
Erschienen in: Indian Journal of Pediatrics | Ausgabe 9/2019
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To the Editor: We report 3 children diagnosed to have leucocyte adhesion deficiency-type 1 based on typical clinical and laboratory features. One was a 30-mo-old female infant, the second was a 5-mo-old male infant and the third was a 4-y-old male child. All had high leukocyte counts at presentation. All had delayed fall of umbilical cord; two had omphalitis; one each had ear lobule infection and periodontitis. The sociodemographic, clinical and laboratory features of the three children are depicted in Table 1. All had reduced expression of CD 18 on their neutrophils, of which one had absent expression and the other had markedly reduced expression and both expired.
Table 1
Sociodemographic, clinical and laboratory features of three children with LAD type-1
|
Sociodemographic/Clinical/ Laboratory features
|
Patient 1
|
Patient 2
|
Patient 3
|
|---|---|---|---|
|
Age at presentation
|
30 mo
|
5 mo
|
4 y
|
|
Gender
|
Female
|
Male
|
Male
|
|
Consanguinity
|
Third degree
|
Third degree
|
Non consanguineous
|
|
Previous sibling death due to infections
|
Yes
|
Yes
|
No
|
|
Delayed fall of umbilical cord
|
Yes, day 35
|
Yes, day 17
|
Yes, day 14
|
|
Omphalitis
|
Yes
|
Yes
|
No
|
|
Local infections without pus formation
|
Dry gangrene ear lobe
|
NA
|
Periodontitis
|
|
Organomegaly
|
Hepatosplenomegaly
|
Hepatosplenomegaly
|
Nil
|
|
Hb g/dl
|
6.4
|
8.3
|
8
|
|
Total leukocyte count at presentation
|
54,050 cells/cu.mm
|
131,000/cu.mm
|
69,770/cu.mm
|
|
Differential count
|
N-71%, L-26%, E-3%
|
N-88%, L-10%, E-2%
|
N-87%, L-10%, E-3%
|
|
Platelet count
|
150,000/cu.mm
|
470,000/cu.mm
|
419,000/cu.mm
|
|
Organisms isolated
|
Pseudomonas aeruginosa
|
None
|
None
|
|
CD 11/18 expression on flow cytometry
|
0%
|
0.8%
|
2%
|
|
Treatment
|
Packed red blood cell transfusion, intravenous antibiotics followed by oral co-trimoxazole and fluconazole prophylaxis
|
Intravenous antibiotics followed by oral co-trimoxazole and fluconazole prophylaxis
|
Intravenous antibiotics followed by oral co-trimoxazole prophylaxis
|
|
Outcome
|
Expired
|
Expired
|
Alive on prophylaxis
|