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 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.

BACKGROUNDBone morphogenetic protein (BMP)-2, alkaline phosphatase (ALP), and collagen type I are known to play a critical role in the process of bone remodeling. However, the relationship between mechanical strain and the expression of BMP-2, ALP, and COL-I in osteoblasts was still unknown. The purpose of this study was to investigate the effects of different magnitudes of mechanical strain on osteoblast morphology and on the expression of BMP-2, ALP, and COL-I.

METHODSOsteoblast-like cells were flexed at four deformation rates (0, 6%, 12%, and 18% elongation). The expression of BMP-2 mRNA, ALP, and COL-I in osteoblast-like cells were determined by real-time quantitative reverse transcription polymerase chain reaction, respectively. The results were subjected to analysis of variance (ANOVA) using SPSS 13.0 statistical software.

RESULTSThe cells changed to fusiform and grew in the direction of the applied strain after the mechanical strain was loaded. Expression level of the BMP-2, ALP, and COL-I increased magnitude-dependently with mechanical loading in the experimental groups, and the 12% elongation group had the highest expression (P < 0.05).

CONCLUSIONMechanical strain can induce morphological change and a magnitude-dependent increase in the expression of BMP-2, ALP, and COL-I mRNA in osteoblast-like cells, which might influence bone remodeling in orthodontic treatment.

Alkaline Phosphatase ; metabolism ; Analysis of Variance ; Animals ; Bone Morphogenetic Protein 2 ; metabolism ; Cell Line ; Collagen ; metabolism ; Collagen Type I ; metabolism ; Mice ; Osteoblasts ; cytology ; metabolism

OBJECTIVETo investigate the status of vaccination against hepatitis B among postgraduate students of medical institutions of higher education in Guangzhou.

METHODSHBsAg and anti-HBs in the serum samples from 1139 postgraduate students were detected by ELISA. Data on hepatitis B vaccine inoculation were investigated by using a questionnaire. Statistical analyses were performed by using SAS software.

RESULTSThe HBsAg positive rate among the 1139 postgraduate students was 2.90 percent. The HBsAg positive rates in hepatitis B vaccine inoculated (1.15 percent) and non- inoculated (21.69 percent) postgraduate students were significantly different (x2=119.11, P<0.0001). The positive rates of HBsAb between the two groups were also significantly different (x2=62.05, P<0.0001). Among the hepatitis B vaccine inoculated students, 17.31 percent were negative for HBsAb. The positive rate of HBsAb among those inoculated the vaccine within the past 3 years was higher than that among those inoculated the vaccine earlier (0-3 years vs. 4-6 year, P=0.0089) (0-3 years vs. 7-9 years, P=0.0172) (0-3 years vs. >9 years, P=0.0474). The positive rate of HBsAb among the students who received hepatitis B vaccine booster dose was higher than that of the students who did not receive any booster dose (P=0.0093).

CONCLUSIONWith the increase of ages, the effect of vaccination for hepatitis B decreased. Male populations may be more susceptible to hepatitis B virus than female. It is necessary to monitor HBsAb levels for those who were inoculated with HBV vaccine more than 3 years ago to give booster dose in time to prevent HBV infection.

Adult ; China ; Female ; Hepatitis B ; immunology ; prevention & control ; virology ; Hepatitis B Antibodies ; blood ; Hepatitis B Vaccines ; administration & dosage ; immunology ; Hepatitis B virus ; immunology ; Humans ; Male ; Middle Aged ; Students, Medical ; Surveys and Questionnaires ; Vaccination ; Young Adult

OBJECTIVETo compare the therapeutic effects of prednisone combined with mycophenolate mofetil (MMF) versus cyclosporin A (CsA) in children with steroid-resistant nephrotic syndrome (SRNS).

METHODSThe clinical data of 164 SRNS children who were treated with prednisone combined with MMF or CsA between January 2004 and December 2013 were collected, and the clinical effect of prednisone combined with MMF (MMF group, 112 children) or CsA (CsA group, 52 children) was analyzed retrospectively.

RESULTSAt 1 month after treatment, the CsA group had a significantly higher remission rate than the MMF group (67.3% vs 42.9%; P<0.05). At 3 months after treatment, the CsA group also had a significantly higher remission rate than the MMF group (78.8% vs 63.3%; P<0.05). The 24-hour urinary protein excretion in both groups changed significantly with time (P<0.05) and differed significantly between the two groups (P<0.05). There were no serious adverse events in the two groups.

CONCLUSIONSPrednisone combined with MMF or CsA is effective and safe for the treatment of SRNS in children, and within 3 months of treatment, CsA has a better effect than MMF.

Adolescent ; Child ; Child, Preschool ; Cyclosporine ; administration & dosage ; Drug Therapy, Combination ; Female ; Humans ; Immunosuppressive Agents ; administration & dosage ; Infant ; Male ; Mycophenolic Acid ; administration & dosage ; analogs & derivatives ; Nephrotic Syndrome ; drug therapy ; Prednisone ; administration & dosage ; Retrospective Studies ; Treatment Outcome

OBJECTIVETo study the clinical characteristics of children with an initial onset of IgA nephropathy with nephrotic syndrome and compare them with children with primary nephrotic syndrome, in order to provide a theoretical basis for the differential diagnosis of the two diseases.

METHODSFifty children diagnosed with an initial onset of IgA nephropathy with nephrotic syndrome were included in this study. Seventy-two children diagnosed with an initial onset of primary nephrotic syndrome served as the control group. The clinical and laboratory examination characteristics were compared between the two groups.

RESULTSThe IgA nephropathy group had significantly higher incidence rates of gross haematuria, microscopic haematuria, hypertension, acute kidney injury, low serum high-density lipoprotein cholesterol, anemia, low serum complement C4, steroid resistance, and nephritis-type nephrotic syndrome and a significantly lower incidence of elevated serum IgE compared with the control group (P<0.05). There were significant differences in serum creatinine, serum uric acid, serum total cholesterol, serum high-density lipoprotein cholesterol, serum IgE, serum complement C4, and hemoglobin levels between the IgA nephropathy and the control groups (P<0.05). The thresholds of serum IgE (<131.2 IU/mL) and high-density lipoprotein cholesterol (<1.35 mmol/L) were reference parameters in the differential diagnosis of IgA nephropathy with nephrotic syndrome and primary nephrotic syndrome.

CONCLUSIONSChildren with IgA nephropathy presenting nephrotic syndrome manifest mainly as nephritis type and steroid-resistant type in the clinical classification. Cinical manifestations accompanied by serum levels of high-density lipoprotein cholesterol and IgE are helpful for differential diagnosis of IgA nephropathy presenting nephrotic syndrome and primary nephrotic syndrome.

Adolescent ; Child ; Child, Preschool ; Cholesterol, HDL ; blood ; Complement C4 ; analysis ; Female ; Glomerulonephritis, IGA ; blood ; complications ; Hematuria ; etiology ; Humans ; Immunoglobulin E ; blood ; Male ; Nephrotic Syndrome ; blood ; complications

OBJECTIVETo study the significance of trace immunoglobulin M (IgM) deposits in glomerular mesangium in children with minimal change primary nephrotic syndrome (PNS).

METHODSOne hundred and six children who were clinically diagnosed with PNS and pathologically diagnosed with minimal change disease (MCD) and trace deposition of IgM in renal tissues were enrolled as subjects. Eighty-one PNS children with MCD but no deposition of immune complexes were used as the control group. The clinical characteristics and efficacies of glucocorticoids and immunosuppressants were retrospectively analyzed in the two groups. All patients were given full-dose prednisone by oral administration, and patients with glucocorticoid resistance or frequent relapses were additionally given immunosuppressants.

RESULTSThe incidence of glucocorticoid resistance in the IgM deposit group was significantly higher than that in the control group (27.2% vs 12.3%; P<0.05). The incidence of frequent relapses in the IgM deposit group was also significantly higher than that in the control group (48.1% vs 10.4%; P<0.05). The complete remission rate for glucocorticoid-resistant patients treated with prednisone combined with mycophenolate mofetil (MMF) was 68% and 62% respectively in the IgM deposit and control groups (P>0.05). The relapse frequency in patients with frequent relapses was significantly reduced in both groups after treatment with prednisone and MMF in combination (P<0.05).

CONCLUSIONSTrace deposition of IgM in renal tissues may be an important factor for glucocorticoid resistance and frequent relapses in PNS children with MCD. Prednisone combined with MMF may be a better choice in the treatment of patients with glucocorticoid resistance or frequent relapses.

Adolescent ; Child ; Child, Preschool ; Drug Resistance ; Female ; Glomerular Mesangium ; immunology ; Glucocorticoids ; therapeutic use ; Humans ; Immunoglobulin M ; analysis ; Immunosuppressive Agents ; therapeutic use ; Infant ; Male ; Nephrosis, Lipoid ; drug therapy ; immunology ; Retrospective Studies

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

OBJECTIVETo explore the clinical characteristics and treatment methods for complicated atlantoaxial dislocation.

METHODSA retrospective evaluation was done to summarize and analyze the clinical characteristics and complicated factors of 54 patients with complicated atlantoaxial dislocation who could not to be treated effectively by using conventional therapy in our hospital from February 2005 to October 2008. According to different complicated factors, different treatment methods mainly including transoral atlantoaxial reduction plate-III (TARP-III) operation, decompression procedure with deep grinding guided by computer aided design-rapid prototyping (CAD-RP), screw placement technique with CAD-RP guide plate and extensile approach surgery were performed.

RESULTSThe average follow-up period was 24 months. Among 54 cases, 48 cases achieved immediate anatomic reduction completely and 6 cases almost achieved anatomical reduction. All the compressed spinal cords were decompressed sufficiently. The decompression rate was 86.0% and the improvement rate of nerve function was 77.8%. Two cases suffered postoperative intracranial infection.

CONCLUSIONSome cases of complicated atlantoaxial dislocation can be effectively treated by using TARP-III operation, decompression procedure with deep grinding guided by CAD-RP, individualized screw placement technique with CAD-RP guide plate and extensile approach surgery.

Adolescent ; Adult ; Aged ; Atlanto-Axial Joint ; abnormalities ; Child ; Child, Preschool ; Female ; Follow-Up Studies ; Humans ; Joint Dislocations ; diagnosis ; surgery ; Male ; Middle Aged ; Retrospective Studies ; Spinal Fusion ; methods ; Young Adult