Division of Gastroenterology, Hepatology, and Nutrition, Department of Pediatrics, Mersin University Faculty of Medicine, Mersin, Turkey.
Division of Gastroenterology, Hepatology, and Nutrition, Department of Pediatrics, Hacettepe University Faculty of Medicine, Ankara, Turkey.
Pediatr Int. 2022 Jan;64(1):e14996. doi: 10.1111/ped.14996. Epub 2021 Nov 23.
Nutritional status in primary immunodeficiencies (PID) is a major factor influencing immune defense. We aimed to evaluate the nutritional status of patients with PID.
Demographic findings and anthropometric measurements of 104 patients were recorded for this cross-sectional study.
Combined immunodeficiencies (n = 49), predominantly antibody deficiencies (n = 28) and phagocytic system disorders (n = 17), were the major disease groups. In total, 44 (42.3%) patients had at least one anthropometric measurement below -2 standard deviations. Chronic, acute, and mixed-type malnutrition were detected in 18.3%, 16.3%, and 7.7% of the patients, respectively. No significant difference was detected among groups regarding anthropometric measurements however higher malnutrition rates were observed in 'combined immune deficiency less profound than severe combined immuno deficiency' (52%), chronic granulomatous disease (66.6%), and X-linked agammaglobulinemia (50%) patients. Severe malnutrition was present in 22 (21.2%) of the patients, although it was not significant. It was more common in the phagocytic system disorder group. All patients in the severe combined immunodeficiency group had undergone hematopoietic stem cell transplantation and 50% of them had malnutrition. There was also no significant difference regarding age, sex, anthropometric indexes (Weight for age, lenght/height for age body mass index Z-scores), malnutrition types, and prevalence of malnutrition among three major disease groups. Only the hospitalization history inversely related to body mass index and weight for age Z-scores (P < 0.0001). In patients with malnutrition, daily caloric intake was at least 20% or more below the requirement.
Regardless of the type of immunodeficiency, nutritional status was poor in PID and hospitalization is the most important determinant of nutritional status. Even after hematopoietic stem cell transplantation, nutritional support should be continued.
原发性免疫缺陷病（PID）患者的营养状况是影响免疫防御的主要因素。本研究旨在评估 PID 患者的营养状况。
本横断面研究记录了 104 名患者的人口统计学发现和人体测量学测量结果。
联合免疫缺陷（n=49）、主要抗体缺陷（n=28）和吞噬系统疾病（n=17）是主要疾病类型。共有 44 名（42.3%）患者的至少一项人体测量值低于-2 个标准差。分别有 18.3%、16.3%和 7.7%的患者存在慢性、急性和混合性营养不良。然而，在“联合免疫缺陷但不严重联合免疫缺陷”（52%）、慢性肉芽肿病（66.6%）和 X 连锁无丙种球蛋白血症（50%）患者中，观察到更高的营养不良率，各组之间的人体测量值无显著差异。22 名（21.2%）患者存在严重营养不良，但无统计学意义。这种情况在吞噬系统疾病组更为常见。严重联合免疫缺陷组的所有患者均接受了造血干细胞移植，其中 50%的患者存在营养不良。在三个主要疾病组中，年龄、性别、人体测量指标（年龄体重 Z 评分、年龄身高 Z 评分、体重指数 Z 评分）、营养不良类型和营养不良患病率之间也无显著差异。只有住院史与体重指数和年龄体重 Z 评分呈负相关（P<0.0001）。在营养不良患者中，每日热量摄入至少低于需求的 20%。
无论免疫缺陷类型如何，PID 患者的营养状况都很差，住院是营养状况的最重要决定因素。即使在造血干细胞移植后，也应继续进行营养支持。
