[Clinical features of children with severe adenovirus pneumonia and hemophagocytic syndrome: an analysis of 30 cases].

OBJECTIVE: To study the clinical features of children with severe adenovirus pneumonia (SAP) and hemophagocytic syndrome (HPS). METHODS: A retrospective analysis was performed from the chart review data of 30 children with SAP and HPS who were admitted from January 2014 to June 2019. According to the prognosis, the children were divided into a good prognosis group (n=18) and a poor prognosis group (n=12). RESULTS: Among the 30 children with SAP and HPS, the ratio of male to female was 2:1. The median age of onset was 1 year and 3 months (range 3 months to 5 years), and the mean course of fever was 19±7 d. Of the 30 children, 28 (93%) experienced disease onset in January to June. High-throughput gene detection of serum pathogens showed that 16 (53%) children were positive for human adenovirus type 7 (HAdV-7), and the other 14 (47%) children were positive for HAdV antigen based on immunofluorescence assay for throat swab, with unknown type. Of all 30 children, 29 (97%) had respiratory complications, 24 (80%) had cardiovascular complications, 16 (53%) had gastrointestinal complications, and 9 (30%) had toxic encephalopathy. Eighteen children (60%) improved or recovered and 12 (40%) did not recover (3 died). Compared with the good prognosis group, the poor prognosis group had a significantly longer course from onset to diagnosis of HPS (P<0.05), significantly higher levels of fibrinogen and tumor necrosis factor-α (P<0.05), and a significantly lower level of interferon-γ (P<0.05). The mean follow-up time was 6±2 months; 11 (41%) children recovered, 1 (4%) experienced recurrence of HPS, and 15 (56%) had the sequela of post-infectious bronchiolitis obliterans (PIBO). CONCLUSIONS: HPS may be observed in children with SAP, and PIBO is the most common sequela of SAP.

Profiles of responses of immunological factors to different subtypes of Kawasaki disease.

BACKGROUND: The responses of immunological factors to different subtypes of Kawasaki disease (KD) remain poorly understood. METHODS: We recruited 388 patients with KD, 160 patients with infectious febrile disease and 85 normal children who served as control subjects. Both the levels and percentages of T lymphocyte subsets, natural killer cells (NK cells) and B cells were analyzed via flow cytometry. The levels of serum IgG, IgM, IgA and C3, C4 were assessed via velocity scatter turbidimetry. RESULTS: The most significant differences noted between the patients with infectious febrile disease and the normal children were the elevated levels of B cells, C3 and the ratio of CD4/CD8, and the decreased levels of CD8+ T cells and NK cells, as well as the moderate increase in the absolute value of the CD3+ cells. The decreased T cell levels and the elevated B cell levels were helpful in distinguishing typical KD from atypical KD; the elevated T cell levels, the elevated NK cell and B cell levels and the decreased B cell levels were helpful in predicting the effectiveness of IVIG; low C3 and C4 levels were linked with prodromal infections. CONCLUSIONS: Lymphocytes subsets and complement markers may be useful in differentiating among the different subtypes of KD and in helping clinicians understand the pathophysiology of KD.

[Changes in serum insulin-like growth factor-1 and insulin-like growth factor-binding protein-3, and their significance in children with left-to-right shunt congenital heart disease associated with heart failure].

OBJECTIVE: To investigate changes in serum insulin-like growth factor-1 (IGF-1) and insulin-like growth factor-binding protein-3 (IGFBP-3) and their significance in children with left-to-right shunt congenital heart disease (CHD) associated with heart failure (HF). METHODS: Twenty healthy children (control group), 20 children with HF, without basic heart disease (HF group), 20 children with left-to-right shunt CHD, without HF (CHD group), and 30 children with left-to-right shunt CHD associated with HF (CHD+HF group) were included in the study. These groups were compared in terms of serum IGF-1 and IGFBP-3 levels. According to the New York Heart Association (NYHA) Functional Classification, the CHD+HF group was further divided into NYHA-II, NYHA-III and NYHA-IV subgroups and the subgroups were compared in terms of serum IGF-1, IGFBP-3, and cardiac troponin I (cTnI) levels. The correlation of serum IGF-1 and IGFBP-3 levels with serum cTnI level in the CHD+HF group was analyzed. RESULTS: The CHD group showed decreased serum IGF-1 and IGFBP-3 levels compared with the control group (P<0.01). The CHD+HF group showed a significantly decreased serum IGF-1 level compared with the control group (P<0.01) and CHD group (P<0.05). The HF group had significantly increased serum IGF-1 and IGFBP-3 levels compared with other groups (P<0.01). The NYHA-II subgroup had the highest serum IGF-1 level and the NYHA-IV subgroup had the lowest serum IGF-1 level (P<0.01). In the CHD+HF group, serum IGF-1 and IGFBP-3 levels were negatively correlated with serum cTnI level (r=-0.692, P<0.05; r=-0.530, P<0.05). CONCLUSIONS: Serum IGF-1 level can be used as an objective condition evaluation indicator for CHD, and low serum IGF-1 level is a risk factor for HF. This also provides a clinical basis for treatment of HF using exogenous IGF-1.

Expression of nuclear factor -κBp65 in mononuclear cells in Kawasaki disease and its relation to coronary artery lesions.

OBJECTIVE: To assess the association of nuclear factor-kappa B (NF-κB) and complications of Kawasaki disease (KD) in Chinese children. METHODS: Based on color Doppler examination results, 86 affected children in the KD group were divided into two groups: 39 cases in coronary artery lesion group (CALs subgroup) and 47 cases in non-coronary artery lesion group (Non-CALs subgroup). Infection control group consisted of 65 cases of hospitalized infected children with fever, having same age as the affected children. Healthy control group consisted of 102 cases of healthy children of the same age, visiting the hospital for physical examination. Western blot was used to detect the expression of NF-кBp65 and IкBα proteins in periphery blood mononuclear cells (PBMC); reverse transcription polymerase chain reaction (RT-PCR) was used to detect the expression of TNF-α and MCP-1 mRNA. RESULTS: The value of NF-kBp65 (optical density) in the PBMC cell nuclei in the KD group was significantly higher than that in the two control groups (p < 0.01). The value of NF-κBp65 in the CALs subgroup was significantly higher than that in the Non-CALs subgroup (p < 0.05). The value of NF-κBp65 inhibitor IκBα in the KD group was significantly lower than that in the infection control group and the healthy control group (p < 0.01). There was a positive correlation between the ratio nucleus NF-κBP65/ IκBα and the severity degree of CALs(r = 0.536, p < 0.05). The value of TNF-α mRNA (O.D ratio) in the KD group was significantly higher than that in the two control groups (P < 0.01), and the value of TNF-α mRNA in the CALs subgroup was significantly higher than that in the Non-CALs subgroup (P < 0.05). The value of MCP-1 mRNA in the KD group was significantly higher than that in the two control groups (P < 0.01), and the value of MCP-1 mRNA in the CALs subgroup was significantly higher than that in the Non-CALs subgroup (P < 0.05). CONCLUSIONS: NF-κBp65 participates in the pathogenesis of vasculitis of KD in acute stage, and may aggravate the vasculitis in KD and plays a part in the formation of CALs.