Malignancies and Lymphoproliferations in Children With Primary Immune Deficiency-A Single-center Experience.

Primary immune deficiencies (PIDs) are rare genetic disorders characterized by impaired immune function, leading to frequent infections and immune dysregulation. Studies have shown that individuals with PID are at an increased risk of developing malignancies and lymphoproliferative disorders compared with the general population. In this single-center study, we aimed to analyze the occurrence of malignancies and lymphoproliferations in children diagnosed with PID. We retrospectively analyzed the medical records of 550 pediatric patients diagnosed with PIDs at our center. Among them, 17 (3,0%) patients were identified with malignancy and/or benign lymphoproliferation. Eight of the 17 patients (47.0%) had immune dysregulatory diseases, whereas ataxia-telangiectasia was the second most common PID associated with malignancy and/or benign lymphoproliferation (n = 5, 29.4%). Lymphoma was the predominant malignancy (n = 11, 64.7%), and Epstein-Barr virus was identified as the most common viral agent associated with malignancy and/or benign lymphoproliferation in patients with PID (n = 8, 47.0%). Our study highlights the association between PID and malignancies/lymphoproliferations, with immune dysregulation syndromes being the most common subclass associated with malignancies/lymphoproliferations. Early diagnosis, multidisciplinary management, and regular surveillance are crucial in improving patient outcomes and saving lives.

The nutritional status of hospitalized children: Has this subject been overlooked?

BACKGROUND/AIMS: To determine the nutritional status of hospitalized children at the time of admission and to investigate the relationship between diagnosis and nutritional status. MATERIALS AND METHODS: Body weight, height, triceps skinfold thickness, and mid-arm circumference were measured on admission and percentages of weight-for-age, weight-for-height, body mass index, mid-arm circumference, and triceps skinfold thickness were calculated. The nutritional status was evaluated using the Waterlow, Gomez, and other anthropometric assessments. RESULTS: A total of 511 patients were included in the study with a mean age of 5.8±4.9 years. Malnutrition was determined in 52.7% of patients according to the Waterlow classification. Mild malnutrition was determined in 39%, moderate in 12%, and severe in 1.7%, with the characteristics of acute malnutrition in 23.9%, acute-chronic in 7.3%, and chronic in 21.5%. The highest rate of malnutrition was in the 0-2 years age group (62.3%). According to the Gomez classification, malnutrition rate was determined as 46.8%. The rates of malnutrition in malignant, gastrointestinal, and infectious diseases were 60%, 59.8%, and 54.5%, respectively. CONCLUSION: The prevalence of malnutrition in hospitalized children was noticeably high. The nutritional evaluation of all patients and an early start to nutritional support could provide a significant positive contribution.