Predictive value of peripheral blood lymphocyte subsets for children with intravenous immunoglobulin-resistant Kawasaki disease / 中国当代儿科杂志

OBJECTIVES@#Based on peripheral blood lymphocyte subsets and common laboratory test indexes, this study aimed to construct a predictive scoring system for intravenous immunoglobulin (IVIG)-resistant Kawasaki disease (KD).@*METHODS@#Children hospitalized in Tianjin Children's Hospital from January 2021 to March 2023 were included in the study (185 cases of IVIG-sensitive KD and 41 cases of IVIG -resistant KD). Forty-six healthy children matched for age and gender were selected as controls. The relative percentage and absolute counts of peripheral lymphocyte subsets were measured by flow cytometry. Multivariate logistic regression was used to identify the predictive factors for IVIG-resistant KD and to construct a predictive scoring system for predicting IVIG-resistant KD.@*RESULTS@#The multivariate logistic regression analysis showed that CD4+ T cell absolute count, natural killer cell absolute count, serum sodium level, globulin level, and total bilirubin level were identified as predictive factors for IVIG-resistant KD (P<0.05). The predictive scoring system based on these factors achieved a sensitivity of 70.7% and a specificity of 83.8% in predicting IVIG-resistant KD.@*CONCLUSIONS@#Peripheral blood lymphocyte subsets can serve as predictive indicators for IVIG-resistant KD in children. The introduction of this indicator and the establishment of a scoring system based on it can provide a higher accuracy in predicting IVIG-resistant KD in children.

Reiter's syndrome in children: a clinical analysis of 22 cases / 中华儿科杂志

<p><b>OBJECTIVE</b>To study the clinical features of Reiter's syndrome (RS) in children.</p><p><b>METHOD</b>Twenty-two patients with RS were referred to our department between August 2002 and September 2008. Their clinical features were analyzed retrospectively.</p><p><b>RESULT</b>Of the 22 patients, 19 were male, only 3 were female. Age ranged from 4 to 14 years, and the average was 10.7 years, most patients (20/22) were older than eight. Among their relatives, 2 had ankylosing spondylitis, 4 had undifferentiated spondyloarthropathy or presented with a history of inflammatory low back pain, and 2 had psoriasis. The season of onset of all patients was summer months from June to September every year. Ten had a history of diarrhea within 1 month preceding the symptoms of arthritis. Twenty-one had fever at the onset. Conjunctivitis occurred in 20 patients, only one was complicated with uveitis. Urethral symptoms occurred in 12 patients, and another 3 patients had abnormal results of urine analysis only. Synovitis occurred in all cases, most of whom had oligoarthritis, predominantly affecting large joints of the lower limbs in an asymmetric pattern with enthesitis occurred in 9. Balanitis circinata was common in male patients (10/19). Elevated inflammatory indicators such as white blood cell, neutrophil, platelet, erythrocyte sedimentation rate, C-reactive protein, immunoglobulins and serum complement C3 were common during the acute illness. All of the 22 cases were negative for rheumatoid factor and 16 (72.7%) were HLA-B27 positive. Nonsteroidal anti-inflammatory drugs and sulfasalazine were the mainstay of treatment. Cyclophosphamide was used in 14 patients (total doses 0.6 - 2.0 g), in 4 cases methotrexate was added. Corticosteroids were added in 4 patients and cyclosporine was given to the patient complicated with uveitis. Most patients achieved full remission within 6 months.</p><p><b>CONCLUSION</b>RS is common in children with clinical features different from those in adults and a relatively good prognosis.</p>

Macrophage activation syndrome in children with rheumatic disorders: a retrospective study on 6 patients / 中华儿科杂志

<p><b>OBJECTIVE</b>To study the clinical manifestations of rheumatic disorders with macrophage activation syndrome (MAS) in children.</p><p><b>METHODS</b>The authors characterized MAS by carrying out a retrospective study on patients who were identified during the past 12 years in Tianjin Children's Hospital.</p><p><b>RESULTS</b>Six cases (4 females, 2 males) were studied. Four had typical systemic onset juvenile idiopathic arthritis (SOJIA), two had systemic lupus erythematosus (SLE) with lupus nephritis. Clinical manifestations at diagnosis, which occurred in the lower activity state of these primary diseases, included high spiking fever (in 5 cases) or high fever (in 1), hepatosplenomegaly (in 6), lymphadenopathy (in 6), profound decrease of all 3 blood cell lines (in 6), significant injury of liver (in 6), diseminated intravascular coagulation (DIC)-like picture (in 2), and central nervous system dysfunction (in 3). Hypofibrinogenemia, elevated liver enzymes and hypertriglyceridemia were found consistently. The phagocytic histiocytes with plasmacytosis were found in 3 bone marrow smears (not done in others). MAS was presumed to have been precipitated by viral infections in 3 patients, two had evidences for herpes simplex virus infection and one for hepatitis A virus infection. The treatment regimen was tailored to each patient, as the clinical course was variable.</p><p><b>CONCLUSIONS</b>MAS may not only be most frequently seen in children with SOJIA, but also in those with other rheumatic diseases, and may be a syndrome that is more common than previously thought. Infection may be main trigger factor for MAS. The immunoapheresis combined with immunochemotherapy may be optimal for severe injury of the liver in patients with MAS.</p>

Clinical characteristics of children with enthesitis related arthritis / 中华儿科杂志

<p><b>OBJECTIVE</b>To study clinical characteristics of children with enthesitis related arthritis (ERA).</p><p><b>METHODS</b>Twelve patients fulfilling the international league of associations for rheumatology (ILAR) criteria for classification of juvenile idiopathic arthritis (JIA) and ERA were referred to our department between August and November, 2002. Their gender, age, family history, clinical manifestations, imaging data, laboratory data and treatment regimens were analyzed retrospectively.</p><p><b>RESULTS</b>Of the 12 patients, 11 were male, only one was female; their age ranged from 4 to 16 years, and the median age was 10.5 years. Ten (83%) of the patients were older than eight years. Among their first degree relatives, one had definite ankylosing spondylitis (AS), and 3 presented with a history of inflammatory low back pain. Enthesitis occurred in 9. Synovitis occurred in 11, most of which were oligoarthritis, predominantly affecting large joints of the lower limbs in an asymmetric pattern. Seven patients underwent CT scan, and only one had erosions of the sacroiliac joints to achieve a diagnosis of juvenile AS. Ten had fever at the onset, and one had a history of diarrhea preceding the symptoms of arthritis. Urethritis and balanitis circinata occurred in 3 cases fulfilling the classification criteria of Reiter's syndrome, with conjunctivitis in 2 and corneitis in one. Elevated inflammatory indicators such as white blood cell, neutrophil, platelet, erythrocyte sedimentation rate, C-reactive protein, immunoglobulins and serum complement C3 were common during the acute illness. Mild anemia was found in 4, and reactive hemophagocytosis in 4 bone marrow specimens. DNA of human parvovirus B19 was detected in sera of 2 cases. One had positive IgM antibody to coxsackie virus B. All 12 cases were rheumatoid factor negative and HLA-B27 positive. Nonsteroidal anti-inflammatory drugs and sulfasalazine were the mainstay of treatment. Corticosteroids were added in 3 cases as a "bridge" therapy due to the severe systemic inflammation. Methotrexate was used in 4 cases with refractory disease or with the hip involved, and in 2 of them cyclophosphamide was added.</p><p><b>CONCLUSION</b>The clinical characteristics of ERA can facilitate an early diagnosis so as to avoid joint damage and disabilities.</p>

Methylprednisolone and cyclophosphamide pulse therapy of severe systemic lupus erythematosus in children / 中华儿科杂志

<p><b>OBJECTIVE</b>To study the effect of methylprednisolone (MP) and cyclophosphamide (CPA) intermittent intravenous pulse therapy and the clinical prognosis in children with severe juvenile onset systemic lupus erythematosus (JOSLE).</p><p><b>METHODS</b>Thirty patients with JOSLE, diagnosed by clinical, laboratory or renal histological examinations, were enrolled in this study. Of the 30 patients, 27 were females and 3 were males, the mean age was (12 +/- 3) years, and 20 of the 22 patients who had undergone initial therapy had LN, and the clinical courses before being involved in the study were 3 to 12 months in nine patients. Twenty-three of the 30 patients had clinical manifestations of renal damages, of whom 4 patients were proven by initial renal biopsy to have WHO type IV, 2 had type II,1 had type V and 1 had type III, and 7 patients had one or more manifestations of central nervous system, including chorea, seizures, cerebrovascular accident (CVA) and organic brain syndrome (OBS), simultaneously, 9 patients had nervous system symptoms without the clinical manifestations of renal damages, 3 patients had lupus crisis, 7 patients did not have any manifestations of renal or neurological damages. According to the protocol of the therapy, the patients were divided into 3 groups: group A (n = 18) patients were treated with MP plus CPA intermittent intravenous pulse for children with lupus nephritis, and with or without neuropsychiatric lupus erythematosus (NPLE), group B (n = 7) with pulsed doses of MP, followed by prednisone and tripterygium wilfordii hook f(T(whf)) for patients without renal or central nerves system damage, and group C (n = 5) with prednisone alone for patients with LN determined by clinical and laboratory features. The effects of those regimes and the clinical prognosis were observed.</p><p><b>RESULTS</b>On short-term follow-up, the SLEDAI-2K (by weight of the renal damage) showed significant difference between group A and group B, but there was no significant difference at the 9th months of the therapy. The long-term follow-up lasted in average for (37.2 +/- 24.8) months. Nineteen patients were followed up for more than 18 months. At the end of follow-up, the mean age was 14 to 19 years. There was no difference on the effect of both group A and group B, and no frequent infections were seen, ANAs were negative and SLEDAI-2K = 0-point in two patients of each group 12 months after discontinuation of the therapy. Four patients in group C died within 18 months.</p><p><b>CONCLUSION</b>The immunosuppressive regimen MP + CPA in patients with severe JOSLE and MP + prednisone + T(whf) in patients without major organs damage were superior to the regimen of prednisone alone.</p>