Assessment of weight and height of patients with primary immunodeficiency disorders and group of children with recurrent respiratory tract infections

Abstract

Background: Primary immunodeficiences (PIDs) are a group of chronic, serious disorders in which the immune response is insufficient. In consequence, it leads to an increased susceptibility to infections. Up to date, there are about 350 different disorders classified in that group. There are also patients suffering from recurrent respiratory tract infections (RRTI), however that group doesn't present any abnormalities in terms of conducted immunological tests. Many factors, including medical, can have an impact on physical development of a child. Data such as birth weight and length, also weight, height, BMI during admission to the hospital were collected from 195 patients' medical histories from their hospitalization at Clinical Immunology and Paediatrics Ward of J. Gromkowski Hospital in Wrocław. Investigated groups included patients with PIDs, RRTI and a control group of healthy children. Our purpose was to evaluate the physical growth of children with PID and children with RRTI by assessment of their height and weight. All of parameters were evaluated using centile charts, suitable best for the Polish population. Results: The lowest mean birth weight and height was found among the PIDs patients group. Children with PIDs during hospitalization had statistically relevant lower mean weight than the control group and almost 18% of them had their height situated below 3rd percentile. The statistically relevant differences have been found between them and RRTI group in terms of weight, height and nutritional status. The statistically significant difference was detected between the nutritional status of PID and control group. Conclusions: There is a higher percentage of PID patients with physical growth abnormalities in comparison to healthy children. Our findings indicate a need for further investigation of immune system irregularities and their influence on physical growth of children.

NF-κB-Inducing Kinase Maintains Mitochondrial Efficiency and Systemic Metabolic Homeostasis

“…Several genetic defects in the NF-κB signaling pathway are associated with PIDs [58–62], including recently described patients with loss-of-function mutations in NIK [10, 11]. Patients with PIDs have been observed to have growth defects, mainly in children [63, 64]. Risk for infection have also been linked to factors including body mass index and adipose deposition due to the immunomodulatory effects of the tissue, but less is understood of the metabolic dynamics in patients with PIDs [65, 66].…”

Basic Indicators of Physical Development in Children Aged 5-6 Years With Recurrent Respiratory Infections

“…Найчастіше в наукових публікаціях повідомляється, що суттєві й різноспрямовані -у бік зменшення або збільшення -відхилення фізичного розвитку дітей зумовлюють їхню вищу схильність до РРІ [8,10,14,15,28]. З іншого боку, відзначається наявність вираженого негативного впливу частих епізодів ГРІ на їхні антропометричні показники [11,18,19,29]. Саме наявність такого циклічного причинно-наслідкового зв'язку обґрунтовує актуальність подальшого поєднаного вивчення показників фізичного розвитку в дітей дошкільного віку, які виявляють схильність до РРІ.…”

“…До того ж Pieniawska-Śmiech K. та співавт. [18] наведено дані, відповідно до яких МТ, довжина тіла (ДТ) та індекс маси тіла (ІМТ) серед пацієнтів віком від 4 тижнів до 18 років з рекурентними респіраторними інфекціями (РРІ) не відрізнялися від аналогічних параметрів фізичного розвитку в осіб із групи контролю, які не мали в анамнезі частих епізодів ГРІ.…”

Diagnostic Challenges in Patients with Inborn Errors of Immunity with Different Manifestations of Immune Dysregulation

“…Disease manifestation appearance (i.e., Nijmegen breakage syndrome (NBS), Shwachman-Diamond syndrome, and DiGeorge syndrome), as well as subject growth during both in utero life and later, may suggest the diagnosis of IEI and provide an important diagnostic clue [ 6 ]. Severe and/or recurrent infections, consanguinity, or an unexplained death in one’s family are well-known signs of IEI; however, more attention should be paid to signs of immune dysregulation.…”