Objective To explore the clinical features of systemic onset of juvenile idiopathic arthritis (SoJIA) with atlantoaxial subluxation as the initial manifestation. Methods The clinical data from one SoJIA patient with atlantoaxial subluxation as the initial manifestation were retrospectively analyzed. Results A 9-year-old boy presented the head and neck movement disorder as the ifrst symptom, developed fever on the third day, then rapidly progressed on 23rd day, with a sharp decline in red blood cell, platelet, and hemoglobin, blood coagulation dysfunction, and a large number of bilateral pulmonary exudation. Ultrasonography showed excess lfuid in abdomen, chest, and pericardium. According to the 2001 version of the revised International College of Rheumatology standard, the diagnosis of SoJIA combined macrophage activation syndrome was conifrmed. Then treated with hemoperfusion, ifltration, and methylprednisolone combined with cyclosporine A, the disease was in remission. Conclusion For children with spontaneous atlantoaxial subluxation accompanied by the systemic symptoms, with no obvious skeletal deformity or acute inlfammation etc., should be alerted to the consideration of systemic juvenile idiopathic arthritis.

Adenocarcinoma ; surgery ; Adenoma ; surgery ; Adult ; Aged ; Female ; Follow-Up Studies ; Gastrointestinal Stromal Tumors ; surgery ; Hemangioma ; surgery ; Humans ; Ileal Neoplasms ; surgery ; Jejunal Neoplasms ; surgery ; Laparoscopy ; methods ; Lymphoma ; surgery ; Male ; Middle Aged

Objective To investigate the mRNA and protein expression of somatostatin receptors in hepatocellular carcinoma HCCLM3 cell lines and to explore the mechanism by which somatostatin effects on hepatocellular carcinoma. Methods RT-PCR., immunocytochemistry and MTT were used to detect mRNA and protein expression of somatostatin receptors in hepatocellular carcinoma cells and evaluate the antiproliferative effect of somatostatin. Results The effect of somatostatin on the cellular proliferation was verified. Immunocytochemistry study revealed a mainly intracellular distribution of all SSTRs with unique patterns for each of them. mRNA expression of all 5 subtypes of somatostatin receptors was different, SSTR2 and SSTR1 mRNA expressions were significantly higher than SSTR3, SSTR4 and SSTR5 ( P

Renal replacement therapy(RRT)is the most effective treatment for patient with severe acute kid-ney injury(AKI).But the timing of initiation of RRT remains controversial.In recent years,there has been an ongoing trend in clinical practice to initiate RRT for AKI earlier especially for patient with AKI stage 2 and severe fluid accumu-lation.There is no single target can predict the timing of discontinuation of RRT,combined use biomarker and urine volume may be a better choice in future.

Objective To explore the clinical characteristics of pancreatic gland damage in children with Henoch-Sch(o)nlein purpura(HSP).Methods The serum and urine analysis of 95 examples were detected by the automatic biochemical analyzer,which were diagnosed as HSP from Aug.2009 to Jun.2010 in Department of Nephrology,Hunan Children's Hospital,and the clinical characteristics of them were analyzed.All children were treated with anti-infection and anti-allergic drugs.The pancreatic morphology of patients was observed by B ultrasonic,and the clinical features of skin,joints,digestive tract,and kidney damage were observed.And the relationship between damage of pancreas and damage of other systems was analyzed.Results There were 64 cases suffering from pancreatic gland damage in 95 HSP children:34 cases were male(53.1％),30 cases were female(46.9％),and there was no significant difference (x2 =0.56,P ＞ 0.05).The incidence of pancreatic gland damage of the patients with allergic purpura combined with abdominalgia was 82.8％ (53 cases),which was obviously higher than that in the patients without abdominalgia (11 cases,17.2％)(x2 =14.24,P ＜0.05).The incidence of pancreatic gland damage in mixed type of allergic purpura(61 cases,95.3％) was obviously higher than that in the patients which only possess the skin rash(3 cases,4.7％) (x2 =18.18,P ＜0.05).The pancreatic glands of the total 64 patients were detected by type B ultrasonic.None of them had been detected with pancreatic gland edema and deformation of structure.The mean hospital stay of the HSP patients with pancreatic gland damage was (10.80 ± 6.39) days (5-39 days),while mean hospital stay without pancreatic gland damage was (8.42 ± 3.51) days (4-13 days),and there was no significant difference between them (t =5.68,P ＞ 0.05).Conclusions HSP children usually were accompanied with pancreatic gland damage,and if they get abdominalgia and multi-system damage they are more likely to be accompanied with pancreatic gland damage,which should be paid attention to.

Objective To analyze the clinical and pathological characteristics of children in different stages and to investigate the clinical significance based on clinical diagnosis and staging criteria of acute kidney injury(AKI).Methods Based on the clinical diagnosis and staging criteria of AKI,165 AKI children admitted to the Department of Nephrology of Hunan Children's Hospital,between Oct.2004 and Oct.2011 were divided into 3 groups:stage 1,stage 2 and stage 3.Clinical characteristics,age,etiology,pathology and prognosis were compared among 3 groups of children with AKI.Results (1) A total of 165 patients(109 males and 56 females) were included in this study,average age of (6.26 ± 4.43) years,including 69 patients in stage 1,19 patients in stage 2,and 77 patients in stage 3.(2) The 3 groups of patients had an average age of(9.09 ± 3.69) years,(4.34 ± 3.90) years,and (4.22 ± 3.78) years,respectively,which showed significant differences (P ＜ 0.01).(3)Three most frequent causes of AKI were drugs (24.8％),acute glomerulonephritis (AGN) (22.4％) and septicemia (15.2 ％),which showed significant differences (P ＜ 0.01).(4) Renal histopathological examination was performed on 140 AKI children,3 most main types of pathology were acute tubular interstitial nephritis 56 cases (40.0％),endocapillary proliferative glomerulonephritis 33 cases (23.6％) and mesangial proliferative glomerulonephritis 18 cases(12.9％).In the stage 1 patients,glomerular disease was predominant(84.4％).In the stage 2 patients,glomerular disease(38.5％) and tubulointerstitial lesions(38.5％) were the major pathological types.In the stage 3 patients,tubulointerstitial damage (73.0％) was the major pathological type (P ＜0.01).The patients in 3 different groups showed significant differences in the ratio of tubulointerstitial disease and glomerular disease(P ＜0.01).(5)The median recovery time of serum creatinine to the baseline was 9 days(3-41 days) for stage 1 patients,11 days(3-25 days) for stage 2 patients,and 16 days(3-∝ days) for stage 3 patients,which showed a significant difference (P ＜ 0.05).(6)Of the 165 A KI patients,124 cases had hematuria,126 cases had varying degrees of proteinuria.There were significant differences in the hematuria incidence and the duration among the 3 groups(P ＜ 0.01).There was no difference in the proteinuria incidence among the 3 groups (P ＞ 0.05),while the stage 1 patients showed significant differences from the other 2 groups (P ＜ 0.01).Conclusions The patients are mostly seen in stage 1 and stage 3.The stage 1 AKI children are largely school-age children and acute glomerulone phritis is the main etiology.The stage 3 AKI children are mainly infants and the etiology of AKI is mainly drugs and septicemia,the pathological type is mainly acute tubulointerstitial nephritis,and the renal functional recovery is slow.

OBJECTIVERecent studies have shown that integrin linked kinase (ILK) plays an important role in the pathogenesis and development of some kidney diseases. This study aimed to investigate the relationship between ILK and renal glomerular damage in children with Henoch schonlein purpura nephritis (HSPN).

METHODSOne hundred and eighty eight HSPN children (aged 3 to 17 years) were assigned to five groups according to the classification of the International Study of Kidney Disease in Children (ISKDC): grade < or = IIa (n = 62), grade IIb (n = 42), grade IIIa (n = 29), grade IIIb (n = 40) and grade > or = IV (n = 15). Fifteen children with basement membrane nephropathy served as the control group. ILK expression on glomeruli was ascertained by immunohistochemical staining. The relationships of ILK expression on glomeruli with glomerular histopathologic lesions and urinary protein excretions were examined.

RESULTSThe positive areas of ILK expression on glomeruli in the control, grade < or = IIa, grade IIb, grade IIIa, grade IIIb and grade > or = IV groups were (3.35 + or - 1.01)%, (4.88 + or - 1.13)%, (9.64 + or - 1.36)%, (11.27 + or - 1.68)%, (17.42 + or -3.0)% and (20.62 + or - 2.32%), respectively. There were significant differences in the ILK expression between groups (p<0.01). ILK expression on glomeruli increased with increased urinary protein excretions. There were significant differences in the ILK expression in children with different urinary protein excretions (p<0.01).

CONCLUSIONSILK might be involved in the process of renal glomerular histopathologic damage and the production of proteinuria in children with HSPN.

Child ; Child, Preschool ; Female ; Humans ; Immunohistochemistry ; Kidney Glomerulus ; pathology ; Male ; Nephritis ; enzymology ; pathology ; Protein-Serine-Threonine Kinases ; analysis ; Purpura, Schoenlein-Henoch ; enzymology ; pathology

Objective:To explore the clinical immune manifestations and high risk factors of children with neuropsychiatric systemic lupus erythematosus (NPSLE), so as to find the basis for early clinical differential diagnosis, prevention and treatment of NPSLE.Methods:Ninety-four children with systemic lupus erythematosus (SLE) admitted to Hunan Children′s Hospital from January 1, 2015 to April 30, 2022 were retrospectively selected. Among them, 19 children with NPSLE were in the NPSLE group, and 75 children without NPSLE were in the SLE group. The disease activity, immune system, lymphocyte subsets, inflammatory factors and autoantibodies were compared between the two groups.Results:Among 94 children with SLE, the incidence of NPSLE was 20.21%(19/94). The most common symptoms of 19 children with NPSLE were headache, epileptic seizure and cerebrovascular disease. The Systemic Lupus Erythematosus Disease Activity Index (SLEDAI)-2000 score was (19.89±8.33)points. Most of them had a good prognosis through hormone intervention. Compared with the SLE group, NPSLE group had higher SLEDAI-2000 score, white blood cell count, urine protein, erythrocyte sedimentation rate (ESR), interferon (IFN)-α, positive rate of anti-Sm antibody and anticardiolipin antibody, lower platelet count, interleukin-2 (IL-2), C 3, C 4 level (all P<0.05), while there was no significant difference in positive rate of anti-dsDNA antibody and anti-SSA antibody between the two group (both P>0.05). Conclusions:Compared with SLE children, children with NPSLE are often complicated with many kinds of abnormal immune function, mainly abnormal humoral immune function and many kinds of positive antibody, with increased inflammatory factors.

Objective　To analyze the clinical indications,efficacy and safety of Chinese minimally invasive percutaneous nephrolithotomy (MPCNL) in treating upper urinary calculi based on our experience.Methods　From June 1992 to September 2010,a total of 10,452 patients (6060 males and 4392 females)with a mean age of (47.6 ± 13.7) years (7 months-93 years) received MPCNL in our center.The mean stone burden was (777.4 ± 740.3) mm2 (20 - 4 080 mm2 ).The data of stone burden,operative techniques,operating time,stone-free rate,major complication,hospital stay and stone composition were investigated.　Results　Of the 10 452 cases,11 801 procedures were performed on 10 876 (5493 left and 5383right) renal units,including 10 102 first stage procedures,1604 secondary procedures,86 third procedures and 9 fourth procedures.There were 11 830 tracts established,including 373 (3.15％ ) tracts of 14 F,7867 (66.50％) tracts of 16 F and 3590 (30.35％) tracts of 18 F.There were 1207 (10.20％),9174(77.55％) and 1449 (12.25％) punctures located in upper,middle and lower pole,respectively.956(8.79％) renal units were managed with multiple tracts,which including 2 tracts in 846 (7.78％) units,3tracts in 85 (0.78％) units,4 tracts in 18 (0.17％) units and 5 tracts in 7 (0.06％) units.Pneumatic lithotripsy was used in 8563 (72.56％) procedures,Holmium:YAG laser lithotripsy was used in 2981(25.26％)　procedures and Pneumatic lithotripsy + Holmium: YAG laser lithotripsy was used in 257(2.18％) procedures.762 (7.29％) cases needed ESWL to clean the stone after MPCNL.The average operating time was ( 101.3 ± 44.2) min ( 10 -240 min).The stone-free rate of MPCNL was 89.9％,which increased to 93％ by adjunctive ESWL.And the mean hospital stay was ( 13.2 ± 6.4) days (2 - 72 days).The major complications happened on 321 (3.07％) cases,including 294 (2.81％ ) cases of blood transfusion,12 (0.11％ ) cases of sepsis,2 (0.02％) cases of renal abscess,9 (0.09％) cases of pleura injury,2 (0.02％) cases of colon injury and 2 (0.02％) cases of death.53 (0.51％) cases needed selective renal arterial embolization to achieve hemostasis.The main stone compositions were analyzed in 4345 cases.Calcium oxalate,calcium phosphate,magnesium ammonium phosphate,uric acid,ammonium urate,carbapatite and cystin were 91.74％,90.33％,14.91％,17.77％,4.83％,8.47％ and 0.51％,respectively. Conclusions　MPCNL is an effective and safe treatment option for all kinds of upper urinary calculi in patients at all ages with a high stone free rate and low major complication rate.

OBJECTIVETo study clinical features of 3 children who presented with nephrotic syndrome (NS) associated with ichthyosis vulgaris (IV), and to detect relationship between NS associated with IV in patients and FLG gene or NPHS2 gene.

METHODClinical and kidney pathological data of the 3 patients were analyzed and progress of pathologic damage in the patient kidney was observed through repeated percutaneous renal biopsy. Using polymerase chain reaction-single strand conformation polymorphism and DNA sequencing, the diversity of the expression of NPHS2 gene in the 3 patients were analyzed, and FLG gene in the 3 patients and parts of their family members with IV was detected.

RESULT(1) The age of the 3 patients (patient 1 was a girl and patients 2 and 3 were boys) suffering from NS was 3 years and 8 months, 2 years and 6 months, and 5 years and 3 months, respectively. The age of onset of IV was 1 year and 6 months, 10 months, and 2 years and 6 months, respectively. All the 3 patients were resistant to steroid therapy. Despite multi-immunosuppressive therapy, no clinical response was achieved. The patients were followed up for 1.5 to 4.0 years. The patients displayed continuous proteinuria, renal function was normal, but their heights were lower than other children at the same age. (2) The older brother of patient 1 died of uremia. The other patients' family members did not have kidney disease. (3) Renal histopathology showed that the patients 1 and 2 had mild mesangial proliferative glomerulonephritis (MsPGN) and the patient 3 had minimal change disease (MCD). One and a half years after the first renal biopsy, the patients 1 and 2 underwent repeated renal biopsy. Renal histopathology showed that the 2 patients' disease developed to medium MsPGN. (4) None of the 3 patients had NPHS2 gene mutation. All the three patients had R501X and 2282del4 which are the common gene mutation type of the FLG, and all the patients were heterozygote. With the detection of the FLG gene of the part of the patients of the three families, the second patient's grandfather had the R501X homozygote mutation and the others were the R501X heterozygote mutation and 2282del4 heterozygote mutation.

CONCLUSIONThe 3 cases of NS associated with IV had no response to steroid and multi-immunosuppressive therapy, the renal damage observed by histopathology progressed fast. The children with NS associated with IV displayed R501X heterozygote mutation and 2282del4 heterozygote mutation of FLG gene, which suggested that the absence of response to steroid and multi-immunosuppressive therapy may be related to the FLG gene.

Child, Preschool ; DNA Mutational Analysis ; Female ; Humans ; Ichthyosis Vulgaris ; complications ; genetics ; Infant ; Intracellular Signaling Peptides and Proteins ; genetics ; Kidney ; pathology ; Male ; Membrane Proteins ; genetics ; Mutation ; Nephrotic Syndrome ; complications ; genetics ; Pedigree