Patients and Methods
The study was conducted in Hacettepe Üniversity İhsan Dogramacı
Children’s Hospital, Ankara, Turkey. The hospital serves as a referral
tertiary hospital.
We assessed a retrospective medical record of 27,000 patients who
admitted to Pediatric Immunology department. To define selective IgM
deficient patients, The European Society for Immunodeficiencies (ESID)
Registry criteria have been used [3-5]. Patients who had repeatedly
(at least twice in a 6-month period) low serum IgM level according to
age referrals were evaluated in the study. Fifty-five patients were
found in this cohort (Figure 1). Thirteen patients who were diagnosed
with a primary immunodeficiency had also low IgM levels with normal IgG
and IgA levels. Forty-two patients fulfilling the diagnosis of sIgM
deficiency were evaluated over a five-year period (2013-2018).
As transient hypogamaglobulinemia may prolong to five years of
age[12], we determined the patients who got the diagnosis less than
five years of age. Eighteen of 42 patients had taken the diagnosis of
sIgMD before five years of age. Out of 18, in nine patients sIgM
deficiency continued after five years of age, in two patients IgM levels
normalized, and seven patients lost follow-up. Eventually, 33 patients
were fulfilling the disease criteria [13].
Cases of sIgMD were reviewed for the symptoms on admission, concurrent
conditions, and clinical course. Patients with genetic mutations,
chromosomal abnormalities and associated immunological disorders were
also recorded. Laboratory findings; complete blood counts, serum
immunoglobulin levels and lymphocyte subsets were noted. To evaluate
vaccination response antiHbs antibody and isohemaglutinins were used.
The patients with PID (n=13) and patients who got the diagnosis of sIgMD
at less than 5 years of age (n=9) were evaluated separately (Figure 1),
and their characteristics are given after the results of patients with
sIGMD.
The study was approved by the ethical committee of Hacettepe University
Medical School Hospital (GO 18/182-20).
Statistical analysis
All the data collected during the study were uploaded to SPSS
statistical program. Parametrical tests (Student t-test) were used to
analyze the variables which distributed normally. The variables that did
not have normal distribution were analyzed by nonparametric tests
(Mann-Whitney U test). Categorical and qualitative variables were
analyzed by Fisher Exact test and Chi-Square test. A p value
lower than 0.05 was considered to be statistically significant.