Clinical Characteristics and Pulmonary Computerized Imaging Findings of Critically Ill Egyptian Patients with Multisystem Inflammatory Syndrome in Children.

Objectives. This study was carried out to delineate the patients' characteristics and the imaging findings and their relation to some biochemical markers of 31 critically ill patients with MIS-C. Design. A retrospective cross-sectional study including all critically ill MIS-C patients admitted to the PICU from June 23rd to July 22nd, 2020. Results. Eighteen males and 13 females, with a median age of 9 years (interquartile range 6-11) presented mainly with fever (100%) and hypotension (100%). Abnormalities in the chest computed tomography were detected in 22 cases (71%). Consolidation and architecture distortion were detected in 58.1% of patients; bilateral lesions and lower lobe infiltrates, each, was evident in 64.5% of patients, while the peripheral distribution of lesions was seen in 71% of the cases. Pleural thickening and effusion, each, was found in 51.6% of the patients. In this small case series, the presence of high ferritin was significantly associated with the bilaterality of the lesions. Elevated C-reactive protein was associated with the peripheral distribution of the lesions. Thrombocytopenia and hypoalbuminemia were significantly correlated with the CT disease stage and CT severity score respectively. Conclusions. Although a few children in this group of MIS-C patients presented with respiratory manifestations, yet, most of them demonstrated significant radiological lung involvement, which necessitates a longer-term follow-up.

Follow-up study of immune defects in patients with dysmorphic disorders.

BACKGROUND: In a previous study, we noted immunologic abnormalities in 46 (54.8%) of 84 individuals with dysmorphic disorders. OBJECTIVE: To reevaluate patients with dysmorphic disorders and immunologic abnormalities 2 to 3 years after an initial study to determine any changes in those abnormalities. METHODS: Information was gathered regarding significant infections during the previous 12 months. Blood samples were drawn for the immunologic tests that were previously performed (IgG, IgA, and IgM level determinations; complete blood cell count; and lymphocyte subset enumeration) and for determination of IgG subclasses and T-cell activation by CD69 expression. RESULTS: In the 21 patients available, 26 (63.4%) of the previously noted 41 low immunologic values were still present. In 5 patients, all previously noted immunologic abnormalities resolved. Of the 17 low values noted in 6 patients with Down syndrome, 12 (70.6%) were still present. Also, the 2 patients with Turner syndrome continued to have low IgA and IgM levels. Two patients had a low IgG4 level. A history of significant clinical infections within the previous 12 months was noted in 10 (58.8%) of 17 patients; 8 (47%) had current immune defects. There was a significantly lower T-cell response to staphylococcal enterotoxin B than in healthy controls. The T-lymphocyte activation response was low in 8 (38.1%) of the 21 patients. CONCLUSIONS: Our study revealed a high rate of immune defects in patients with dysmorphic disorders, both during the initial study and 2 to 3 years later, which may contribute to their increased susceptibility to infections. This association was most obvious in patients with Down syndrome and Turner syndrome. The findings should alert for early immunologic evaluation when such patients have infections.

Immune defects in subjects with dysmorphic disorders.

Patients with dysmorphic disorders seem to have frequent respiratory infections that may be attributed to associated anatomic or neurological abnormalities, but immune defects may contribute to their susceptibility to infections. We screened subjects with dysmorphic conditions for major hematologic, B-cell and T-cell defects. We studied 84 subjects with dysmorphic disorders: 29 with chromosomal disorders, 27 with single gene disorders, and 28 with unclassified dysmorphic disorders. They were evaluated by physical examination; medical history suggestive of possible immune deficiency; complete blood count; serum immunoglobulin G (IgG), IgA, and IgM levels; and lymphocyte subsets. Low laboratory values (less than fifth percentile for age) were detected in 54.8%; was highest in the chromosomal disorder group (79.3%) followed by the single gene disorder group (55.6%) and was lowest in the unclassified dysmorphic disorder group (28.6%). The most common low values were in the CD19 and CD16/56 lymphocyte subpopulations followed by IgG and IgA levels. None of the subjects had neutropenia or thrombocytopenia. History of significant recurrent infections was noted in five subjects, all of whom had abnormal laboratory values. The highest frequency of abnormal laboratory values was in Down syndrome followed by Turner syndrome and chromosome deletions. We concluded that patients with dysmorphic disorders, particularly those with chromosomal disorders, have a high frequency of various B-cell and T-cell defects that may be contributing to their susceptibility to infection. Studies are needed to further delineate the immunologic defects in that population and to investigate a possible genetic basis at the molecular level.