OBJECTIVES: To investigate the change in the distribution of memory B cell subsets in children with frequently relapsing nephrotic syndrome (FRNS) during the course of the disease. METHODS: A total of 35 children with primary nephrotic syndrome (PNS) who attended the Department of Pediatrics of the Affiliated Hospital of Xuzhou Medical University from October 2020 to October 2021 were enrolled as subjects in this prospective study. According to the response to glucocorticoid (GC) therapy and frequency of recurrence, the children were divided into two groups: FRNS (n=20) and non-FRNS (NFRNS; n=15). Fifteen children who underwent physical examination were enrolled as the control group. The change in memory B cells after GC therapy was compared between groups, and its correlation with clinical indicators was analyzed. RESULTS: Before treatment, the FRNS and NFRNS groups had significantly increased percentages of total B cells, total memory B cells, IgD⁺ memory B cells, and IgE⁺ memory B cells compared with the control group, and the FRNS group had significantly greater increases than the NFRNS group (P<0.05); the FRNS group had a significantly lower percentage of class-switched memory B cells than the NFRNS and control groups (P<0.05). After treatment, the FRNS and NFRNS groups had significant reductions in the percentages of total B cells, total memory B cells, IgM⁺IgD⁺ memory B cells, IgM⁺ memory B cells, IgE⁺ memory B cells, IgD⁺ memory B cells, and IgG⁺ memory B cells (P<0.05) and a significant increase in the percentage of class-switched memory B cells (P<0.05). The FRNS group had a significantly higher urinary protein quantification than the NFRNS and control groups (P<0.05) and a significantly lower level of albumin than the control group (P<0.05). In the FRNS group, urinary protein quantification was negatively correlated with the percentage of class-switched memory B cells and was positively correlated with the percentage of IgE⁺ memory B cells (P<0.05). CONCLUSIONS Abnormal distribution of memory B cell subsets may be observed in children with FRNS, and the percentages of IgE⁺ memory B cells and class-switched memory B cells can be used as positive and negative correlation factors for predicting recurrence after GC therapy in these children.
Child ; Humans ; B-Lymphocyte Subsets/metabolism* ; Immunoglobulin E ; Immunoglobulin M ; Nephrotic Syndrome/immunology* ; Prospective Studies ; Glucocorticoids/therapeutic use*

Acetaldehyde ; metabolism ; Alcoholics ; Alcoholism ; blood ; immunology ; Antibodies, Anti-Idiotypic ; Humans ; Immunoglobulin A ; immunology ; Immunoglobulin G ; immunology ; Immunoglobulin M ; immunology ; Protein Processing, Post-Translational

OBJECTIVETo investigate the relationship between TCM syndrome type and laboratory indexes in patients with systemic lupus erythematosus (SLE).

METHODSThree hundred and eighty-three SLE patients were differentiated into six syndrome types based on the "toxin" sydrome differentiation of TCM, Type 1, the heat-toxin flourishing type; Type 2, the stasis heat with toxin type; Type 3, the turbid toxin congested type; Type 4, the general deficiency with evil stay type; Type 5, the heat-toxin burning yin type; Type 6, the Gan-stagnant with toxin convergency type. The indexes, including complement C3 (C3), immunoglobulin A (IgA), immunoglobulin M (IgM), immunoglobulin G (IgG), alanine aminotransferase (ALT), 24 h urinary protein quantitation, white blood cell (WBC) and platelet (PLT) count, were determined, and the SLEDAI score was calculated.

RESULTSLevels of IgA and IgM in all patients were basically normal. Level of IgG was higher than the normal range in patients of type 1 and 2, being 18,713.81 mg/L and 23,131.54 mg/L respectively, showing significant difference between the Type 2 and Type 3 (P < 0.05); the lowest mean value of C3 presented in patients of type 1 (586.32 mg/L), and that in patients of type 4 was significantly different to that in patients of other types (P < 0.05); count of WBC and PLT was lower in patients of type 5 and significantly different to that in the other five types (P < 0.05); level of 24 h urinary protein quantitation was higher than normal in all the patients and the highest level (2.78 g/24 h) was found in patients of type 3; and the highest level of ALT (112.75 U/L) appeared in patients of type 6 as compared with that in patients of other types.

CONCLUSIONDifferent variations of laboratory indexes present in SLE patients of different syndrome types, there are certain relationship between the TCM syndrome types and the laboratory indexes, and these rules may provide reference for evaluating clinical therapeutic effects.

Complement C3 ; metabolism ; Diagnosis, Differential ; Female ; Humans ; Immunoglobulin A ; blood ; Immunoglobulin G ; blood ; Immunoglobulin M ; blood ; Lupus Erythematosus, Systemic ; blood ; diagnosis ; urine ; Male ; Medicine, Chinese Traditional ; Proteinuria ; urine ; Syndrome

PURPOSE: Kawasaki disease is a systemic vasculitis, and its etiology and pathogenesis are still not clear. Our study was undertaken to investigate the characteristics of the activation of B cells in the peripheral blood of Kawasaki disease (KD) patients and evidence of stimulation by superantigens. MATERIALS AND METHODS: Blood samples were obtained from three patients (2 males, 1 female) with KD, who were admitted to our Hospital, Seoul, Korea. The mean age was 1.2 years. Distribution of B cells was studied in the acute and subacute phases of KD patients. From the RNA of B cells, we obtained complementary DNA (cDNA) and performed polymerase chain reaction (PCR). To determine the oligoclonal expansion of immunoglobulin M (IgM) VH family, we cloned and sequenced the PCR products from each group and analyzed DNA. RESULTS: In the peripheral blood of acute phase patients, T cells were significantly decreased (p < 0.05), whereas B cells were significantly increased (p < 0.05). When the first PCR was done on the B cell chains, VH1 to VH6 were all found to be expressed. The number of micro gene clones obtained from 3 patients was 312, and they belonged to VH3, VH4 and VH5 family. M99686 germ line was most frequently used and the next most frequently used, were X92224/J, L21967 and L21964. A similar order was seen in patients. Among the clones, 20 sets of clones showed the same base sequence and this was frequent between VH2 and VH5. There was one set, which showed almost the same base sequence between different patients, and the homology was 99.5%. Twenty sets of clones that had the same base sequence showed high similarity to the germ line (94 - 100%). Among these, the clones that utilized the M99686 germ line were 4 sets which were most frequent. The 3-dimensional structure of one of these clones showed typical beta, sheet structure of immunoglobulin chains. CONCLUSION: The IgM transcripts expressed by the B cells in the peripheral blood of KD patients in the acute phase of the disease clearly showed an oligoclonal expansion, suggesting that KD is caused not by stimulation of a superantigen, but rather by a conventional antigen.
B-Lymphocytes/*metabolism ; Female ; Humans ; Immunoglobulin M/*metabolism ; Immunoglobulin Variable Region/*genetics ; Infant ; Male ; Mucocutaneous Lymph Node Syndrome/*genetics/*immunology ; Reverse Transcriptase Polymerase Chain Reaction ; Sequence Analysis, DNA

PURPOSE: Kawasaki disease is a systemic vasculitis, and its etiology and pathogenesis are still not clear. Our study was undertaken to investigate the characteristics of the activation of B cells in the peripheral blood of Kawasaki disease (KD) patients and evidence of stimulation by superantigens. MATERIALS AND METHODS: Blood samples were obtained from three patients (2 males, 1 female) with KD, who were admitted to our Hospital, Seoul, Korea. The mean age was 1.2 years. Distribution of B cells was studied in the acute and subacute phases of KD patients. From the RNA of B cells, we obtained complementary DNA (cDNA) and performed polymerase chain reaction (PCR). To determine the oligoclonal expansion of immunoglobulin M (IgM) VH family, we cloned and sequenced the PCR products from each group and analyzed DNA. RESULTS: In the peripheral blood of acute phase patients, T cells were significantly decreased (p < 0.05), whereas B cells were significantly increased (p < 0.05). When the first PCR was done on the B cell chains, VH1 to VH6 were all found to be expressed. The number of micro gene clones obtained from 3 patients was 312, and they belonged to VH3, VH4 and VH5 family. M99686 germ line was most frequently used and the next most frequently used, were X92224/J, L21967 and L21964. A similar order was seen in patients. Among the clones, 20 sets of clones showed the same base sequence and this was frequent between VH2 and VH5. There was one set, which showed almost the same base sequence between different patients, and the homology was 99.5%. Twenty sets of clones that had the same base sequence showed high similarity to the germ line (94 - 100%). Among these, the clones that utilized the M99686 germ line were 4 sets which were most frequent. The 3-dimensional structure of one of these clones showed typical beta, sheet structure of immunoglobulin chains. CONCLUSION: The IgM transcripts expressed by the B cells in the peripheral blood of KD patients in the acute phase of the disease clearly showed an oligoclonal expansion, suggesting that KD is caused not by stimulation of a superantigen, but rather by a conventional antigen.
B-Lymphocytes/*metabolism ; Female ; Humans ; Immunoglobulin M/*metabolism ; Immunoglobulin Variable Region/*genetics ; Infant ; Male ; Mucocutaneous Lymph Node Syndrome/*genetics/*immunology ; Reverse Transcriptase Polymerase Chain Reaction ; Sequence Analysis, DNA

PURPOSE: Behcet's disease (BD) is a disease of unknown etiology, which has multisystemic involvement. This multisystemic involvement might be the clue for an autoimmune pathogenesis. In order to evaluate an autoimmune pathogenesis, we examined immunoreactans depositions in the skin of BD patients. MATERIALS AND METHODS: The skin samples of 108 BD patients (28 perilesional skin, 44 positive pathergy test site, 22 negative pathergy test site, 14 normal skin) were examined for the depositions of immunoglobulin (Ig)M, IgG, IgA, complement 3 (C3), and fibrinogen (F) using direct immunofluorescence (DIF). The data were statistically compared to the DIF of 36 systemic lupus erythematosus (SLE) patients and 20 healthy controls using chi-square Fisher exact test. RESULTS: Highly significant immunoreactans depositions were obtained in BD (deposition rates: IgM 70.3%, IgG 0%, IgA 20.3%, C3 62.9%, F 83.3%). The comparison with SLE revealed no differences in IgM, IgA, and C3. However, IgG deposition was higher in SLE while F deposition was higher in BD. In both BD and SLE, the Ig depositions were highly significant when the data were compared with the healthy controls. CONCLUSION: The significant deposition of immunoreactans in BD, especially in the negative pathergy and the normal skin sites, were observed. This study is the first controlled study revealing positive Ig depositions in BD, and it is expected to help us to reconsider the autoimmune pathogenesis in BD.
Adolescent ; Adult ; Aged ; Behcet Syndrome/*metabolism ; Case-Control Studies ; Female ; Fluorescent Antibody Technique, Direct/*methods ; Humans ; Immunoglobulin A/metabolism ; Immunoglobulin G/metabolism ; Immunoglobulin M/metabolism ; Male ; Middle Aged ; Skin/metabolism/pathology ; Young Adult

PURPOSE: Behcet's disease (BD) is a disease of unknown etiology, which has multisystemic involvement. This multisystemic involvement might be the clue for an autoimmune pathogenesis. In order to evaluate an autoimmune pathogenesis, we examined immunoreactans depositions in the skin of BD patients. MATERIALS AND METHODS: The skin samples of 108 BD patients (28 perilesional skin, 44 positive pathergy test site, 22 negative pathergy test site, 14 normal skin) were examined for the depositions of immunoglobulin (Ig)M, IgG, IgA, complement 3 (C3), and fibrinogen (F) using direct immunofluorescence (DIF). The data were statistically compared to the DIF of 36 systemic lupus erythematosus (SLE) patients and 20 healthy controls using chi-square Fisher exact test. RESULTS: Highly significant immunoreactans depositions were obtained in BD (deposition rates: IgM 70.3%, IgG 0%, IgA 20.3%, C3 62.9%, F 83.3%). The comparison with SLE revealed no differences in IgM, IgA, and C3. However, IgG deposition was higher in SLE while F deposition was higher in BD. In both BD and SLE, the Ig depositions were highly significant when the data were compared with the healthy controls. CONCLUSION: The significant deposition of immunoreactans in BD, especially in the negative pathergy and the normal skin sites, were observed. This study is the first controlled study revealing positive Ig depositions in BD, and it is expected to help us to reconsider the autoimmune pathogenesis in BD.
Adolescent ; Adult ; Aged ; Behcet Syndrome/*metabolism ; Case-Control Studies ; Female ; Fluorescent Antibody Technique, Direct/*methods ; Humans ; Immunoglobulin A/metabolism ; Immunoglobulin G/metabolism ; Immunoglobulin M/metabolism ; Male ; Middle Aged ; Skin/metabolism/pathology ; Young Adult

OBJECTIVETo investigate the clinical pathologic characteristics of IgM nephropathy in children.

METHODSThe data of 34 children with IgM nephropathy from the First Affiliated Hospital of Zhengzhou University were retrospectively reviewed.

RESULTSOf the 34 cases of IgM nephropathy, nephrotic syndrome (NS) was clinically presented in 22 cases (64.7%). The renal pathological classification was as follows: minimal change disease (12 cases, 35.3%), minimal change disease with acute renal tubular injury (3 cases, 8.8%), minimal change glomerulonephritis (6 cases, 17.6%), minimal change glomerulernephritis with ischemic renal injury (1 case, 2.9%), mesangial proliferative glomerulonephritis (7 cases, 20.6%), focal segmental glomerulosclerosis (4 cases, 11.8%), focal proliferative glomerulernephritis (1 case, 2.9 %). Glomerular injury score, renal vascular injury score and total renal injury score increased with the increasing IgM deposition.

CONCLUSIONSThe majority of children with IgM nephropathy manifest clinically as nephrotic syndrome. The patterns of renal pathology may be varied in children with IgM nephropathy. IgM deposition in the mesenteric area is an important pathologic feature and is related to the degree of renal injury.

Child ; Child, Preschool ; Female ; Glomerulonephritis ; pathology ; Humans ; Immunoglobulin M ; metabolism ; Infant ; Kidney ; pathology ; Male ; Prognosis ; Retrospective Studies

OBJECTIVESTo explore the category, quantity and clinical significance of autoantibodies on bone marrow hematopoietic cells in patients with immunorelated cytopenia and evaluate the sensitivity of direct antiglobulin reaction (Coombs test ) of bone marrow mononuclear cells (BMMNC).

METHODSThe category and the positive rate of autoantibodies on bone marrow hematopoietic stem cells, nucleated erythrocytes, granulocytes in 32 patients with uncertain immunorelated cytopenia were investigated by using BMMNC-Coombs test and double immunofluorescence flow cytometry.

RESULTSThe positive rate of autoantibodies on bone marrow hematopoietic cells tested by flow cytometry was 90.63% which was higher than that by BMMNC-Coombs test (50.0%) (p < 0.05). In 29 positive cases, IgG autoantibody accounted for 6.90%, IgM13.8%, IgG+IgA 3.4%, IgG+IgM 31.0%, and IgG+IgM+IgA 44.8%. Of the 29 Patients, 25 (86.2%) with IgG autoantibody, 26 (89.7%) with IgM and 14 (48.3%) with IgA. The patients with IgG autoantibody alone had the lowest hemoglobin levels, and those with IgM autoantibody might have intravascular hemolytic findings. The response time of patients with IgG and IgG+IgM was shorter than that of the other patients. 91.3% of the patients had autoantibodies on bone marrow hematopoietic stem cells and showed pancytopenia, and 50% of the patients had autoantibodies on nucleated erythrocytes and granulocytes. Eleven of 13 patients with negative BMMNC-Coombs tests had autoantibodies on bone marrow hematopoietic stem cells detected by FACS. There was no significant difference of the quantities of the three categories of autoantibodies of nucleated erythrocytes and stem cells. The quantities of IgA on granulocytes were lower than that of IgG and IgM. There was no significant difference between IgG and IgM on granulocytes. The quantity of IgA on hematopoietic stem cells was significantly higher than that on nucleated erythrocytes or granulocytes.

CONCLUSIONSThe sensitivity of double immunofluorescence flow cytometry assay was higher than that of BMMNC-Coombs test for detecting autoantibodies. In immunorelated cytopenia patients, the predominant autoantibody was IgM which could cause intravascular hemolysis, and the second one was IgG which could cause severe anemia. Most immunorelated cytopenia patients had autoantibodies on hematopoietic stem cells and showed pancytopenia. IgA was more easily seen on the hematopoietic stem cells.

Adolescent ; Adult ; Aged ; Autoantibodies ; classification ; metabolism ; Autoimmune Diseases ; immunology ; Bone Marrow Cells ; immunology ; Child ; Coombs Test ; Female ; Flow Cytometry ; Humans ; Immunoglobulin A ; metabolism ; Immunoglobulin G ; metabolism ; Immunoglobulin M ; metabolism ; Male ; Middle Aged ; Pancytopenia ; immunology

In order to asses congenital cytomegalovirus (CMV) infection in Korea, five hundred and seventy five pregnant women (mean age 29.5 +/- 3.8 yrs., mean gestational age at test 37.5 +/- 6.7 weeks) visiting the prenatal clinic at Severance Hospital, Seoul, Korea were studied. CMV IgG antibody was present in 96% (552/575) and IgM antibody was present in 0.7% (4/575) of the pregnant women by the third trimester. Four of 445 cord sera were positive for CMV IgM antibody (0.9%). Urine samples from 514 newborns were tested for the evaluation of congenital CMV infection. Six (1.2%) of 514 newborns excreted CMV in their urine. All the congenitally infected infants had subclinical involvement at birth and during the 12 months of the follow-up period. These results indicate that Korean pregnant women were highly immunized against CMV by the third trimester. Furthermore this study suggests that the rate of congenital CMV infection is relatively as high as rates previously reported from other countries, although there is a very high prevalence of maternal immunity. The incidence of maternal primary infection during pregnancy seems to be rare and therefore most congenital infections in Korea might be following by maternal reactivation or reinfection.
Adult ; Cytomegalovirus Infections/congenital/*epidemiology/immunology/metabolism ; Female ; Follow-Up Studies ; Humans ; *Immunity, Maternally-Acquired ; Immunoglobulin G/blood ; Immunoglobulin M/blood ; Infant, Newborn ; Korea/epidemiology ; Pregnancy