OBJECTIVES: To investigate the clinical manifestations and laboratory examination results of children with Kawasaki disease complicated by macrophage activation syndrome (KD-MAS), and to provide a basis for identifying early warning indicators for the early diagnosis and treatment of KD-MAS. METHODS: A retrospective study was performed on 27 children with KD-MAS (KD-MAS group) and 110 children with KD (KD group) who were admitted to Wuhan Children's Hospital, Tongji Medical College, Huazhong University of Science and Technology, from January 2014 to January 2022. Clinical and laboratory data were compared between the two groups. The receiver operating characteristic (ROC) curve was used to investigate the value of laboratory markers with statistical significance in the diagnosis of KD-MAS. RESULTS: Compared with the KD group, the KD-MAS group had significantly higher incidence rates of hepatomegaly, splenomegaly, incomplete KD, no response to intravenous immunoglobulin, coronary artery damage, multiple organ damage, and KD recurrence, as well as a significantly longer length of hospital stay (P<0.05). Compared with the KD group, the KD-MAS group had significantly lower levels of white blood cell count, absolute neutrophil count, hemoglobin, platelet count (PLT), erythrocyte sedimentation rate, serum albumin, serum sodium, prealbumin, and fibrinogen (FIB), a significantly lower incidence rate of non-exudative conjunctiva, and significantly higher levels of C-reactive protein, alanine aminotransferase, aspartate aminotransferase, lactate dehydrogenase (LDH), and serum ferritin (SF) (P<0.05). The ROC curve analysis showed that SF, PLT, FIB, and LDH had high value in the diagnosis of KD-MAS, with areas under the curve (AUC) of 0.989, 0.966, 0.932, and 0.897, respectively (P<0.001), and optimal cut-off values of 349.95 μg/L, 159×10⁹/L, 3.85 g/L, and 403.50 U/L, respectively. The combination of SF, PLT, FIB, and LDH had a larger AUC than PLT, FIB, and LDH alone in the diagnosis of KD-MAS (P<0.05), but there was no significant difference in the AUC between the combination of SF, PLT, FIB, and LDH and SF alone (P>0.05). CONCLUSIONS KD-MAS should be considered when children with KD have hepatosplenomegaly, no response to intravenous immunoglobulin, coronary artery damage, and KD recurrence during treatment. SF, PLT, FIB, and LDH are of high value in the diagnosis of KD-MAS, especially SF is of great significance in the diagnosis of KD-MAS.
Child ; Humans ; Immunoglobulins, Intravenous ; Macrophage Activation Syndrome/etiology* ; Mucocutaneous Lymph Node Syndrome/diagnosis* ; Retrospective Studies ; Blood Sedimentation ; Hepatomegaly

Objective: To analyze the clinical characteristics of children with multisystem inflammatory syndrome (MIS-C) associated with SARS-CoV-2 in China, and to improve the understanding of MIS-C among pediatricians. Methods: Case series study.Collect the clinical characteristics, auxiliary examinations, treatment decisions, and prognosis of 64 patients with MIS-C from 9 hospitals in China from December 2022 to June 2023. Results: Among the 64 MIS-C patients, 36 were boys and 28 were girls, with an onset age being 2.8 (0.3, 14.0) years. All patients suffered from fever, elevated inflammatory indicators, and multiple system involvement. Forty-three patients (67%) were involved in more than 3 systems simultaneously, including skin mucosa 60 cases (94%), blood system 52 cases (89%), circulatory system 54 cases (84%), digestive system 48 cases (75%), and nervous system 24 cases (37%). Common mucocutaneous lesions included rash 54 cases (84%) and conjunctival congestion and (or) lip flushing 45 cases (70%). Hematological abnormalities consisted of coagulation dysfunction 48 cases (75%), thrombocytopenia 9 cases (14%), and lymphopenia 8 cases (13%). Cardiovascular lesions mainly affected cardiac function, of which 11 patients (17%) were accompanied by hypotension or shock, and 7 patients (12%) had coronary artery dilatation.Thirty-six patients (56%) had gastrointestinal symptoms, 23 patients (36%) had neurological symptoms. Forty-five patients (70%) received the initial treatment of intravenous immunoglobulin in combination with glucocorticoids, 5 patients (8%) received the methylprednisolone pulse therapy and 2 patients (3%) treated with biological agents, 7 patients with coronary artery dilation all returned to normal within 6 months. Conclusions: MIS-C patients are mainly characterized by fever, high inflammatory response, and multiple organ damage. The preferred initial treatment is intravenous immunoglobulin combined with glucocorticoids. All patients have a good prognosis.
Male ; Child ; Female ; Humans ; Immunoglobulins, Intravenous/therapeutic use* ; Blood Coagulation ; COVID-19 ; China/epidemiology* ; Connective Tissue Diseases ; Coronary Aneurysm ; Fever ; Systemic Inflammatory Response Syndrome/therapy*

International guidelines regarding the role of intravenous immunoglobulin (IVIG) in the management of Rh- and ABO-mediated haemolytic disease of the newborn was drafted by an international panel of experts in the fields of hematology, neonatology, and blood transfusion and was published in British Journal of Haematology on March 16, 2022. The guidelines summarize the evidence-based practice of IVIG in Rh- and ABO-mediated haemolytic disease of the newborn and propose related recommendations. The guidelines recommend that IVIG should not be applied as a routine treatment regimen for Rh- and ABO-mediated haemolytic disease of the newborn in order to reduce exchange transfusion (ET), and the best time to apply IVIG remains unclear in the situations where hyperbilirubinaemia is severe (approaching or exceeding the ET threshold) or ET cannot be implemented. These guidelines are formulated with rigorous methods, but with the lower quality of evidence.
Infant, Newborn ; Female ; Humans ; Immunoglobulins, Intravenous/therapeutic use* ; Erythroblastosis, Fetal/drug therapy* ; Exchange Transfusion, Whole Blood ; Hematologic Diseases ; Hyperbilirubinemia

Adult ; Aged ; Aged, 80 and over ; Air Pollutants ; adverse effects ; Blood Chemical Analysis ; Coke ; adverse effects ; Environmental Exposure ; Female ; Humans ; Immunoglobulins ; metabolism ; Male ; Middle Aged ; Polycyclic Aromatic Hydrocarbons ; adverse effects ; Young Adult

PURPOSE: Kawasaki disease (KD) is a common, acute systemic vasculitis in children. Acute phase reactants (APRs) have been used to assist diagnosis, and to predict outcome in children with KD. However, it remains unknown on levels of APRs depending on duration of fever. We aimed to compare APR levels of children with KD who visited with < 5 days duration of fever and with ≥ 5 days. METHODS: Children (≤ 15 years) with complete KD who visited the emergency department were enrolled from March 2012 through February 2018. The children were divided into the early (fever < 5 days) and late (fever ≥ 5 days) presenters. The baseline characteristics, APR levels, such as platelet count, and outcomes were compared between the 2 groups. RESULTS: A total of 145 children with complete KD were enrolled. Median age was 27.0 (interquartile range [IQR], 12.0–46.5) months, and boys accounted for 60.0%. The early presenters (63 [43.4%]) had a younger age (17.0 [IQR, 7.0–45.0] vs. 32.5 [IQR, 14.0–48.0] months; P = 0.006), shorter duration of fever (3.0 [IQR, 2.0–4.0] vs. 6.0 [IQR, 5.0–7.0] days; P < 0.001), and a lower platelet count (336.7 ± 105.2 [× 10³/µL] vs. 381.6 ± 121.8 [× 10³/µL], P = 0.02) than the late presenters. The other APR levels, and frequency of resistance to intravenous immunoglobulin and coronary artery abnormalities showed no differences between the 2 groups. CONCLUSION: Children with KD who visited with < 5 days duration of fever had a lower platelet count compared to those with ≥ 5 days. No differences were found in the other APR levels and the outcomes. It may be necessary to consider the differences in APR levels depending on duration of fever when treating children with KD.
Acute-Phase Proteins ; Blood Platelets ; C-Reactive Protein ; Child ; Coronary Vessels ; Diagnosis ; Emergency Service, Hospital ; Fever ; Humans ; Immunoglobulins ; Leukocyte Count ; Mucocutaneous Lymph Node Syndrome ; Platelet Count ; Systemic Vasculitis

Adolescent ; Anti-N-Methyl-D-Aspartate Receptor Encephalitis ; blood ; cerebrospinal fluid ; drug therapy ; Autoantibodies ; blood ; cerebrospinal fluid ; Female ; Follow-Up Studies ; Humans ; Immunoglobulins, Intravenous ; therapeutic use ; Immunotherapy ; Methylprednisolone ; therapeutic use

A girl was diagnosed with intravenous immunoglobulin (IVIG)-resistant Kawasaki disease (KD) due to pyrexia and hemoptysis for eight days. The girl was a school-age child with major clinical manifestations of pyrexia, skin rash, enlargement of bilateral cervical lymph nodes, conjunctival hyperaemia, red and cracked lips and strawberry-like tongue, followed by swelling of both hands and feet. Laboratory examination showed significant increases in white blood cell count, platelet count, C-reactive protein, erythrocyte sedimentation rate and liver enzymes, a significant reduction in albumin, and the presence of aseptic pyuria. After the first course of IVIG treatment, the girl still had recurrent pyrexia, with hemoptysis on day 2 after admission, and lung CT showed uneven luminance and patchy shadow. The symptoms were quickly alleviated after the second course of IVIG treatment combined with methylprednisolone and aspirin treatment. KD is a febrile disease characterized by multiple systemic vasculitis in childhood and can involve various organ systems such as the heart, lungs, kidneys and the nervous system. Therefore, it is necessary to carefully monitor and recognize the rare symptoms of KD, and early recognition of pulmonary complications of KD can avoid delay in diagnosis, prevent the development of more serious complications, and help with early treatment and disease recovery.
Blood Sedimentation ; Child ; Female ; Fever ; Hemoptysis ; Humans ; Immunoglobulins, Intravenous ; Mucocutaneous Lymph Node Syndrome

BACKGROUND AND OBJECTIVES: Immunological variability in Kawasaki disease (KD) shows age-specific differences; however, specific differences in laboratory values have not been compared between infants and non-infants with KD. We compared age-adjusted Z-values (Z) of white and red blood cells in infants with KD with those in non-infants with KD. METHODS: This study retrospectively investigated 192 infants and 667 non-infants recruited between 2003 and 2015 at the Korea University Hospital. Laboratory values for infants with KD and non-infants with KD were analyzed and age-unadjusted raw values (R) and age-adjusted Z for blood cells counts were determined. RESULTS: Z in infants with KD during pre-intravenous immunoglobulin (IVIG), post-IVIG, and chronic phases showed increased lymphopenia and eosinophilia, low neutrophil:lymphocyte and neutrophil:eosinophil ratios, worse anemia, increased thrombocytosis, and reduced erythrocyte sedimentation rates compared with those in non-infants with KD. The optimal cut-off value for pre-IVIG Z-hemoglobin for prediction of KD in all patients was <−0.01 (area under the curve [AUC], 0.914; sensitivity/specificity, 0.999/0.886; p=0.04). The optimal cut-off value for pre-IVIG C-reactive protein (CRP) for prediction of KD in infants compared to that in febrile control infants was >40 mg/L (AUC, 0.811; sensitivity/specificity, 0.712/0.700; p=0.04). CONCLUSIONS: Laboratory characteristics enable differentiation between infants and non-infants with KD and contribute to a better understanding of changes in blood cell counts. Infants with incomplete KD can be more easily differentiated from infants with simple febrile illness using pre-IVIG Z-hemoglobin and pre-IVIG CRP values.
Anemia ; Blood Cell Count ; Blood Cells ; Blood Sedimentation ; C-Reactive Protein ; Eosinophilia ; Erythrocytes ; Humans ; Immunoglobulins ; Infant ; Korea ; Leukocyte Count ; Lymphopenia ; Mucocutaneous Lymph Node Syndrome ; Retrospective Studies ; Thrombocytosis

A 61-year-old man presented with a 3-year history of erythematous firm nodules on the hands and feet. Histopathological findings of the lesional skin revealed perivascular and diffuse neutrophilic infiltrations on the upper and mid-dermis. Increased and dilated blood vessels were observed in the upper dermis. Fibrinoid necrosis of the vessel walls was unremarkable, but endothelial swelling and scant red blood cell (RBC) extravasation were noted. Fibrosis and sclerosis of collagen fibers were noted on the deep dermis. Results of laboratory examinations, including complete blood count (CBC), routine chemistry, c-reactive protein (CRP), syphilis and human immunodeficiency virus (HIV) tests, and serum immunoglobulin electrophoresis, were all negative or within normal limit. A diagnosis of erythema elevatum diutinum was made based on the clinical and histological findings. The patient was treated with prednisolone, dapsone, colchicine, and intralesional injection of triamcinolone and showed slight improvement after treatment for 8 months.
Blood Cell Count ; Blood Vessels ; C-Reactive Protein ; Chemistry ; Colchicine ; Collagen ; Dapsone ; Dermis ; Diagnosis ; Electrophoresis ; Erythema ; Erythrocytes ; Fibrosis ; Foot ; Hand ; HIV ; Humans ; Immunoglobulins ; Injections, Intralesional ; Middle Aged ; Necrosis ; Neutrophils ; Prednisolone ; Sclerosis ; Skin ; Syphilis ; Triamcinolone

OBJECTIVE: To study the clinical and laboratory features of children with Kawasaki disease (KD) aged >5 years. METHODS: A retrospective analysis was performed for the clinical data of 250 elderly children (aged >5 years) who were diagnosed with KD or incomplete KD (divided into 5-9 years and >9 years groups) and 266 KD children aged 1-5 years. Clinical and laboratory features were compared between groups. RESULTS: The >9 years group had the lowest incidence rates of hand and foot swelling and fingertip or perianal desquamation (P<0.05). The 5-9 years group had the highest incidence rate of neck lymph node enlargement (P<0.05). The >9 years group had the longest course of fever (P<0.05). There were no significant differences among the three groups in the incidence rates of rash, bulbar conjunctival hyperaemia and the change in lips, the proportion of children with incomplete KD, and the proportion of children with no response to intravenous immunoglobulin (IVIG). The >9 years group had the lowest platelet count and albumin (P<0.05). The 5-9 years group had the highest percentage of neutrophils (P<0.05). There were no significant differences among the three groups in white blood cell count, C-reactive protein, erythrocyte sedimentation rate, and alanine aminotransferase (P>0.05). As for the degree of CAL, the 1-5 years group had the highest incidence of mild coronary dilation, and the >9 years group had the highest incidence rate of moderate coronary aneurysm (P<0.05). There was no significant difference in the incidence rate of large coronary aneurysm among the three groups (P>0.05). CONCLUSIONS KD children aged >5 years have atypical clinical manifestations, with a high incidence rate of neck lymph node enlargement, a high percentage of neutrophils, and a low level of albumin and platelet. The risk of moderate coronary aneurysm increases with age.
Blood Sedimentation ; Child ; Child, Preschool ; Coronary Aneurysm ; Humans ; Immunoglobulins, Intravenous ; Infant ; Mucocutaneous Lymph Node Syndrome ; Retrospective Studies