ABSTRACT
Objective:To demonstrate the clinical significance of group A streptococcal infection (GAS) in patients with enthesitis related arthritis (ERA).Methods:A retrospective study was conducted on ERA (136) and PolyRF-/Oligo juvenile idiopathic arthritis (JIA) (272) patients in Beijing Children's Hospital from 2016 to 2018. Anti-streptococcal hemolysin "O" (ASO) was tested and documented in all patients. The infection rate of GAS was compared between patients with ERA and PolyRF-/Oligo JIA. Patients with ERA were divided to two groups according to the result of ASO (ASO positive and ASO negative). All the clinical data were documented and compared within the two groups. The statistical methods used mainly include t test, rank sum test, chi-square test, and Spearman correlation analysis.Results:The GAS infection rate of patients with ERA was higher than patients with PolyRF-/Oligo JIA (17.6% vs 9.5%, χ2=5.52, P=0.019). In ERA patients, clinical data were analyzed, and a statistical significant difference was observed in the presence of human leukocyte antigen (HLA)-B27 between ASO positive and ASO negative group [75.0%(18/24) vs 49.1%(55/112), χ2=5.329, P=0.021]. Statistical differences were found in Patrick's sign positive rate between the two groups [100%(24/24) vs 67.0%(75/112), χ2=10.61, P=0.001]. There was statistically significant difference between the two groups regarding the radiogr-aphic grading at the sacroiliac joint. More patients with positive ASO had grade Ⅲ damage at the sacroiliac joint compare to patients with negative ASO [68.2%(15/22) vs 28.4%(29/102), χ2=12.49, P<0.001]. The logarithmic of the ASO was slightly correlated with the radiographic grade of sacroiliac joint ( r=0.26, P=0.005). Conclusion:Patients with ERA are prone to be infected by GAS. It's probably related to HLA-B27 postivity for antigen presentation. Patients who were infected by GAS fre-quently have sacroiliac joint involvement, and tend to be more sever. This indicates that GAS may play an important role in the pathogenesis of sacroiliac joint destruction.
ABSTRACT
Objective To investigate the detection rate and possible factors of hyperhomocysteinemia(HHcy) in children with chronic kidney disease(CKD).Methods The clinical data of children with CKD between July 2012 and September 2016 in the Department of Pediatrics,Peking University First Hospital were retrospectively collected.The homocysteine(Hcy) level of patients were measured.The other data included the general information,diagnosis and laboratory test results.Results Seventy-six pediatric patients with CKD were enrolled including 49 boys and 27 girls.The average age of the patients was (9.9±3.4) years old.The main cause of the patients in the study was primary glomerulopathy(48.7%,37/76 cases),and the rest were congenital and inherited glomerular diseases(36.8%,28/76 cases),secondary glomerular diseases(9.2%,7/76 cases)and renal tubular diseases(5.3%,4/76 cases).Fifty patients (65.8%,50/76 cases) had normal level of Hcy which was 10.40(7.30,11.62) μmol/L.Twenty-six patients(34.2%,26/76) were detected with HHcy whose Hcy level was 17.93(16.76,24.11) μmol/L.The detection rate of HHcy in CKD stage 1,stage 2,stage 3,stage 4 and stage 5 was 13.9%(5/36 cases),22.2%(2/9 cases),50.0%(4/8 cases),57.1%(4/7 cases) and 68.8%(11/16 cases) respectively,and the detection rate increased with CKD stages and the difference was statistically significant (χ2=17.574,P<0.001).The level of Hcy was 10.05(7.04,12.47) μmol/L,11.75(10.78,16.44) μmol/L,13.73(10.09,18.23) μmol/L,15.81(11.12,20.71) μmol/L and 17.39(11.86,24.76) μmol/L in CKD stage 1,stage 2,stage 3,stage 4 and stage 5,respectively.The Kruskal-Wallis test revealed that the distribution of homocysteine in CKD stages had statistically significant difference(P=0.001).Multiple linear regression model showed that creatinine clearance was an independent predicator of HHcy.Conclusions In this study of the CKD patients,the detection rate of HHcy was high and increased with the progression of CKD.HHcy is mainly influenced by creatinine clearance in CKD.The level of Hcy should be monitored regularly in children with CKD and HHcy should be treated with proper measures.
ABSTRACT
SUMMARY We described 1 case of autoimmune lymphoproliferative syndrome ( ALPS) , first diagnosed in our hospital, and reviewed the recent literature. The 11-month old male patient presented with a histo-ry of splenomegaly and hepatomegaly since 1 month after birth. He suffered recurrent infectious diseases including cytomegalovirus infection, parvovirus B19 infection and chronic diarrhea disease. Besides, his symptoms included hemolytic anemia and thrombocytopenia. The laboratory abnormality indicated an ex-panded population of alpha/beta double-negative T cells (DNTs) (27. 18% of lymphocytes, 35. 16% of CD3 + T lymphocytes) in peripheral blood, and autoantibodies including antinuclear antibody, double-stranded DNA and rheumatic factor were positive. Hyper gamma globulinemia and positive direct Coombs tests were seen in the patient. His parents were both healthy and denied autoimmune diseases. We iden-tified a heterozygous point mutation in exon 3 of the FAS gene carrying c. 309 A>C, resulting in a single base pair substitution in exon 3 of FAS gene which changed the codon of Arg103 to Ser103 . Unfortunate-ly, we were unable to obtain the gene results of the child' s parents. The patient was treated with glu-cocorticoids in our hospital and with mycophenolatemofetil in other hospital. And we were informed that his anemia condition relieved through the telephone follow-up, but he still suffered recurrent infections, hepatomegaly and splenomegaly still existed. As we all know ALPS is characterized by defective lympho-cyte apoptosis, and thus cause lymphoproliferative disease and autoimmune disease, and increase the risk of lymphoma. It is more likely to be misdiagnosed as other diseases. ALPS should be suspected in the case of chronic lymphadenopathy, splenomegaly and autoimmune features. Flow cytometry approach is helpful for the diagnosis. Immunosuppressive drugs are the necessary treatment.
ABSTRACT
Homocysteine has been confirmed as the independent factor for cardiovascular disease.Hyperhomocysteinemia remains the prominent manifestation in the cases of chronic kidney disease, atypical hemolytic anemia,methylmalonic aciduria combined with kidney injuries.As the recognition of the kidney injuries caused by homocysteine remains obscure, this review tries to show the synthesis, metabolism of homocysteine and the possible mechanism of homocysteine-induced heart and kidney injuries.Besides, summarize the current treatment of lowering-hyperhomocysteinemia in order to arouse the pediatricians' attention of homocysteine which has endothelial cell toxicity and causes the wide lesions in great, middle and small vessels, and thus improve the outcomes of the patients.