Objective To study the clinical characteristics of children with anti-neutrophil cytoplasmic antibody(ANCA)-associated vasculitis(AAV).Methods A retrospective analysis was conducted on the clinical data of 25 children diagnosed with AAV at the Second Xiangya Hospital of Central South University from January 2010 to June 2022.Results Among the AAV children,there were 5 males and 20 females,with a median age of onset of 11.0 years.Involvement of the urinary system was seen in 18 cases(72%);respiratory system involvement in 10 cases(40%);skin involvement in 6 cases(24%);eye,ear,and nose involvement in 5 cases(20%);joint involvement in 4 cases(16%);digestive system involvement in 2 cases(8%).Eleven cases underwent kidney biopsy,with 5 cases(46%)showing focal type,2 cases(18%)showing crescentic type,2 cases(18%)showing mixed type,and 2 cases(18%)showing sclerotic type.Immune complex deposits were present in 5 cases(45%).Seven cases reached chronic kidney disease(CKD)stage Ⅴ,with 2 cases resulting in death.Two cases underwent kidney transplantation.At the end of the follow-up period,2 cases were at CKD stage Ⅱ,and 1 case was at CKD stage Ⅲ.Of the 16 cases of microscopic polyangiitis(MPA)group,13(81%)involved the urinary system.Of the 9 cases of granulomatosis with polyangiitis(GPA),6 cases(66%)had sinusitis.Serum creatinine and uric acid levels were higher in the MPA group than in the GPA group(P<0.05),while red blood cell count and glomerular filtration rate were lower in the MPA group(P<0.05).Conclusions AAV is more common in school-age female children,with MPA being the most common clinical subtype.The onset of AAV in children is mainly characterized by renal involvement,followed by respiratory system involvement.The renal pathology often presents as focal type with possible immune complex deposits.Children with MPA often have renal involvement,while those with GPA commonly have sinusitis.The prognosis of children with AAV is poor,often accompanied by renal insufficiency.

To investigate the effect of naringenin on ovariectomy-induced postmenopausal osteoporosis comprehensively and systemically, thirty-two virgin Sprague-Dawley rats about 3-month-old were used and randomly divided into 4 groups: sham control group (Sham), OVX control group (OVX), naringenin treatment group and 17β-estradiol (E2) treatment group. After 12 weeks treatment with different drugs, 24 h urine were collected, organs were weighed and the organ indies were computed. Uterine pathological changes were observed by making paraffin section. Biochemical parameters and bone turnover markers: serum osteocalcin (BGP) and urine deoxypyridinoline (DPD) were analyzed with automatic biochemical analyzer or ELISA assay. Bone mineral density (BMD) and bone mineral content (BMC) were analyzed by DEXA, bone biomechanical properties was measured by three point bending test and the trabecular bone microarchitecture was evaluated by Micro CT. From the results, we can see that: the gaining of weight and the increasing of bone turnover markers such as serum BGP and urinary DPD could be inhibited by naringenin. The treatment could also enhance the bone strength and prevent the deterioration of trabecular microarchitecture, increase the bone volume, trabecular number and thickness, and decrease the trabecular space. The effects mentioned above were not accompanied with stimulating effects on uterus. Long-term using of naringenin had no obvious influence on other organs and the liver and kidney functions. The study suggests that naringenin had obvious antiosteoporotic effect on ovariectomized rats and it had the potential value for the treatment of postmenopausal osteoporosis.
Amino Acids ; urine ; Animals ; Bone Density ; Disease Models, Animal ; Estradiol ; pharmacology ; Estrogen Antagonists ; pharmacology ; Female ; Flavanones ; pharmacology ; Osteocalcin ; blood ; Osteoporosis ; drug therapy ; Ovariectomy ; Rats ; Rats, Sprague-Dawley ; Uterus ; pathology

Objective To investigate the value of B-type ultrasound during and after operations in patients sustaining craniocerebral injury in Wenchuan earthquake. Methods The patients with suspected multiple intracranial hematomas due to intraoperative acute encephalocele were referred for B-type ultrasonic examination. After the operation, B-type ultrasonic examination was performed at the cranial defects or the site of cranial drilling by horizontal coronal or sweep scanning. Results Open cranial surgery was performed in 58 cases of craniocerebral injury resulting from the earthquake. Intraoperative B-type ultrasound was used in 24 cases, and intracranial hematomas or hemorrhage in different brain regions were found in 18 cases. Hematomas in the injured hemisphere were found in 2 cases, and ultrasound-guided hematoma clearance was performed in 14 cases. In 28 cases, postoperative B ultrasonic examination was carried out along with CT scanning, and the results demonstrated basic agreement between the two medalities. Conclusions Intraoperative B-type ultrasound may help accurately localize multiple intracranial hematomas and can be crucial to identify the causes of intraoperative acute encephalocele. Postoperative sweep scanning with B-type ultrasound at the cranial defect can accurately identify delayed hematoma to allow timely management and improve the operative SUCCESS.

OBJECTIVETo study the regulattory effect of Astragalus membranaceus on immune disturbance of the rats with IgA nephropathy.

METHODSRats IgA nephropathy (IgAN) model was duplicated by oral feeding of bovine serum albumin (BSA), subcutaneous injection of carbon tetrachloride (CCl4) and injection of lipopolysaccharide (LSP) into vena caudalis. The rats were divided into three groups randomly for the normal, IgAN model group and the group treated with Astragalus membranaceus (treatment group). The treatment group was given the Astragalus membranaceus granules via intragastric administratsion, the normal group and the IgAN model group were given the equal amount of aqua destillata by gastric perfusion. The rats were examined for albuminuria, hematuria and pathological changes of renal tissue and the distribution of TGF-beta and interleukin-5 in renal tissue was determined by immunohistochemistry and the IFN-gamma and IL-4 of cytokine of Th1 and Th2 types were detected in rats IgA nephropathy model by sandwich enzyme linked immunosorbent assay (ELISA).

RESULTS(1) The hematuria in rats with IgA nephropathy significantly increased compared with normal control group and Astragalus treatment group (P < 0.05). There was significant increase in albuminuria in rats with IgA nephropathy, compared with normal control group and astragalus treatment group (P < 0.01). (2) The pathological change of glomerular mesangium, renal tubules and renal interstitia became serious in rats IgA nephropathy model when compared with normal control group and astragalus treatment group. Immumofluorescence showed renal IgA density in rats IgA nephropathy model was significantly higher than that in the normal control group (P < 0.001) and astragalus treatment group (P < 0.001). (3) The result of immuno histochemistry showed that there was only weak expression of TGF-beta and interleukin 5 in normal renal tissue. The expression of TGF-beta and interleukin 5 in IgA nephropathy model was significantly stronger than those in normal control group (P < 0.05) and astragalus treatment group (P < 0.05). (4) The serum IL-4 levels were (33.74 +/- 7.52) pg/ml in rats IgA nephropathy model, significantly higher than that in normal control group (2.36 +/- 0.85) pg/ml and astragalus treatment group (3.24 +/- 1.13) pg/ml. The IFN-gamma level in serum of rats IgA nephropathy model was (18.79 +/- 3.80) pg/ml, which was significantly higher than that in normal control group (46.53 +/- 5.56) pg/ml and astragalus treatment group (41.28 +/- 2.95) pg/ml.

CONCLUSIONSThe astragalus could lower the level of hematuria and 24 hours-albuminuria of the IgAN model, and amelioratse the change of the renal pathology and reduce the deposit of IgA in glomerular mesangium. The possible mechanism of the effect is that astragalus could regulate the derangement of Th1, Th2, accordingly could improve the level of IL-4 and IFN-gamma in the serum and diminish the expression of cytokine Th2 TGF-beta1 and IL-5 of the renal tissue, and thereby could postpone the development of IgAN.

Animals ; Astragalus membranaceus ; chemistry ; immunology ; Cattle ; Drugs, Chinese Herbal ; pharmacology ; Glomerulonephritis, IGA ; immunology ; Interleukin-4 ; pharmacology ; Interleukin-5 ; pharmacology ; Kidney Tubules ; drug effects ; Rats ; Transforming Growth Factor beta ; immunology ; Transforming Growth Factor beta1 ; pharmacology

OBJECTIVETo investigate the effects of astragalus on tubulointerstitial lesions in rats with IgA nephropathy (IgAN) and to explore the possible mechanism.

METHODSTwenty-eight Sprague-Dawley rats were randomly assigned to three groups. The rat model of IgA nephropathy was induced by intragastric administration of bovine serum albumin and injections of LPS and CC14. Six weeks later, the rats with IgAN were randomly treated with oral astragalus (3 g/kg/d, for 6 weeks) or normal saline. Normal control rats which were not subjected to IgAN were treated with normal saline. The number of urinary erythrocytes and urinary protein and B-D-N-Acetyl glucosaminidase (NAG) contents were determined by Pan-automatic biochemistry analyzing meter. Expression of monocyte chemotactic protein-1 (MCP-1) and nuclear factor-kappa B (NF-kappaB) in tubulointerstitial tissues were analyzed by immunohistochemistry. A semiquantitative score was used to evaluate the degree of renal pathologic lesions.

RESULTSThe number of urinary erythrocytes (74.02+/-16.58 / microL vs 383.23+/-4.94 /microL) and urinary protein (13.88+/-4.94 vs 59.82+/-14.73 mg/L) and NAG contents (2.84+/-0.31 vs 5.24+/-0.80 U/L) in the astragalus-treated IgAN rats decreased remarkably compared with those in the IgAN rats without astragalus treatment (P<0.01). Expression of the NF-kappaB and MCP-1 in the renal tissues in the IgAN rats without astragalus treatment was significantly higher than that in the astragalus-treated IgAN rats and normal control rats (P<0.01). There were significant differences in the scores of renal pathologic lesions between the IgAN rats with or without astragalus treatment (6.03+/-0.46 vs 10.57+/-1.23; P<0.01).

CONCLUSIONSAstragalus can decrease the number of urinary erythrocytes and urinary protein and NAG contents, and relieves tubulointerstitial lesions, possibly through the down-regulation of NF-kappaB and MCP-1 expression in rats with IgAN.

Animals ; Astragalus Plant ; Chemokine CCL2 ; analysis ; Glomerulonephritis, IGA ; drug therapy ; metabolism ; pathology ; Immunohistochemistry ; Kidney Tubules ; pathology ; Rats ; Rats, Sprague-Dawley ; Transcription Factor RelA ; analysis

Objective To explore the electrophysiological results and rehabilitation outcome of two prelingually deafened pediatric cochlear implant patients with auditory neuropathy.Methods Preoperative audiological evaluation,intra-postoperative electrically evoked auditory brainstem response(EABR)and neural response telememetry(NRT)record for the two cases were conducted in Beijing Tongren Hospital.A one year follow-up was performed.Data collected before and at 6,12-month intervals after implantation were compared with that from control pediatric cochlear implant patients matched for the same duration of implant use as this two cases. Results The two children implanted had not had any postoperative medical or cochlear implant device complications. Intraoperative EABR and NRT were elicited in case 1 with unrepeatable waveforms. After 12 months of training,Case 1 had shown significant improvements in sound detection,speecn perception abilities and communication skills,which was better than the control group.and the eJectrophysiological results became normal.Case 2 had also benefited from cochlear implantation, even though no recognizable NRT was found until he returned 12 month after the operation.Conclusions The desynchronization of auditory path had been changed after the electrical stimulation ongoing 12 months for children with auditory neuropathy.The two children had not had any complications postoperatively,and eacn child had shown improved listening and communication skills. Cochlear implantation COUld help patients witn auditory neuropathy to improve their communication skill and go back to the main stream.

OBJECTIVETo investigate fibronectin synthesis in SD rat mesangial cells after transforming growth factor-beta1 (TGF-beta1) is silenced by the short interfering RNA (siRNA) expressed by reconstructed pGEFP-C1 vectors.

METHODSDepending upon the 538th - 556th (A) and 895th - 913th (B) nucleotides of rat TGF-beta1 gene, a nucleotide (A or B) was constructed into a small hairpin nucleotide which was separately (A or B) or together (A plus B) inserted into a pGEFP-C1 vector with three reconstructed pGEFP-C1 vectors separately expressing the siRNAs for A or/and B. TGF-beta1 and fibronectin were dynamically investigated for their interrelationship by ELISA in the supernatant and RT-PCR in their extracted total RNA.

RESULTSThe siRNA hairpin-like molecules were constructed according to the 538th - 556th nucleotides of rat TGF-beta1 gene were able to markedly silence the expression of TGF-beta1 mRNA (P < 0.01) and protein (P < 0.01) at 48 h. Lipfectamin 2000 transfection stimulated the peak secretion of fibronectin at 24 h in the control and the experimental group whose TGF-beta1 was not silenced, but the silence of TGF-beta1 in both experimental groups delayed the top values of fibronectin to 48 h (P < 0.01).

CONCLUSIONThe silence of TGF-beta1 by siRNA decreased the fibronectin expression, but the latter was possibly not completely TGF-dependent.

Animals ; Cells ; Cells, Cultured ; Fibronectins ; metabolism ; Mesangial Cells ; drug effects ; metabolism ; RNA Interference ; drug effects ; physiology ; RNA, Messenger ; metabolism ; RNA, Small Interfering ; pharmacology ; Rats ; Rats, Sprague-Dawley ; Transforming Growth Factor beta1 ; chemistry ; genetics

OBJECTIVETo investigate the role of mast cells in the development of renal interstitial fibrosis in children with Henoch-Schonlein purpura nephritis (HSPN) and possible mechanisms.

METHODSParaffin-embedded renal biopsy tissue sections from 20 children with HSPN were examined for the levels of tryptase-beta and transforming growth factor-beta1 (TGF-beta1) by immunohistochemical staining. Mast cells were counted by toluidine blue staining. Masson staining was used to assess the level of renal interstitial fibrosis and renal histopathological scores. Normal renal tissue sections from 5 nephrectomized children for nephroma were used as control group.

RESULTSThe percentages of positive tryptase-beta cellsand mast cells and the TGF-beta1 expression in the HSPN group were significantly higher than those in the control group (P < 0.05). The percentages of positive tryptase-beta cells and mast cells and the TGF-beta1 expression in renal tissue were positively correlated with the glomeruli histopathological score (r =0.940, 0.920, 0.937, respectively; P < 0.05) and were also positively correlated with the histopathological score of renal interstitium (r=0.903, 0.859, 0.948, respectively; P < 0.05). The level of renal interstitial fibrosis was positively correlated with the percentages of positive tryptase-beta cells and mast cells and the expression of TGF-beta1 (r =0.790, 0.766, 0.858, respectively; P < 0.05). There was a positive correlation between the percentages of positive tryptase-beta cells and mast cells (r =0.941, P < 0.05), between the percentage of positive tryptase-beta cells and the TGF-beta1 expression (r =0.897, P < 0.05) and between the percentage of positive mast cells and the TGF-beta1 expression (r=0.942, P < 0.05).

CONCLUSIONSTubulointerstitial mast cell infiltration is associated with the development of renal interstitial fibrosis in children with HSPN. Mast cells together with TGF-beta1 and mast cell-derived tryptase-beta may be involved in the development of the renal interstitial fibrosis in HSPN.

Adolescent ; Child ; Female ; Fibrosis ; Humans ; Kidney ; chemistry ; pathology ; Male ; Mast Cells ; physiology ; Nephritis ; pathology ; Purpura, Schoenlein-Henoch ; metabolism ; pathology ; Transforming Growth Factor beta1 ; analysis ; Tryptases ; analysis

OBJECTIVETo investigate the clinicopathologic characteristics of childhood renal diseases.

METHODSA retrospective analysis of 1316 renal biopsies performed over the past 20 years was performed.

RESULTSOf the 1316 patients, 383 (29.09% ) were diagnosed as nephrotic syndrome, 291 (22.00%) as acute nephritis syndrome, 224 (17.21%) as isolated hematuria, 209(15.87%) as purpura nephritis, and 96 (7.30% ) as hepatitis B virus-associated nephritis . Mesangial proliferation was the most common pathological change (756 cases; 57.45%), followed by IgA nephropathy (113 cases; 8.59%), endothelial capillary proliferation(112 cases; 8.51%), membranous nephropathy (66 cases; 5.02%), and various minor and minimal changes (59 cases; 4.48%). Alport syndrome, congenital nephrotic syndrome, thin basement membrane nephropathy, fibrillary glomerulopathy disease, and Fabry disease were confirmed by electronic microscopy. IgA, IgM and C1q nephropathy were definitely diagnosed using immune histochemistry or immunofluorescent. A diagnosis of primary glomerular disease was made in 69.53% of the cases (915 cases); secondary glomerular disease was noted in 26.14% (344 cases). Of the 915 cases of primary glomerular disease, 375 (41.0%) had nephrotic syndrome. Secondary glomerular disease due to purura nephritis was common (209/344; 60.8%).

CONCLUSIONSPrimiary glomerular disease predominates in children. Nephrotic syndrome is the most common clinical diagnosis. Mesangial proliferation is the most common pathological patterns in children with renal disease.

Adolescent ; Child ; Child, Preschool ; Female ; Humans ; Infant ; Infant, Newborn ; Kidney ; pathology ; Kidney Diseases ; pathology ; Kidney Glomerulus ; pathology ; Male ; Renal Insufficiency ; pathology ; Retrospective Studies

OBJECTIVE: To evaluate the clinical and pathological features of 94 children suffering from IgA nephropathy (IgAN) while estimating the prevalent situation in Hunan province. METHODS: To summarize the annual number of hospitalized children, those with kidney diseases, those accepted biopsy, and those confirmed as IgAN in both Xiangya Hospital and Second Xiangya Hospital undertaking kidney biopsy in Hunan province during 1995 and 2004. RESULTS: In the past 10 years, as the hospitalized population in both hospitals accrued to 9.98% each year. The rate of 7.5% was seen in those with kidney diseases. Among whom 56.3% accepted kidney biopsy and 94 of them were confirmed as IgAN. Hematuria was the main clinical presentation, seen in 71 cases, accounting to 76%, and even to 98% after excluding those with nephrotic syndrome and isolating proteinuria type of IgAN. Inflammation infiltration (91%), renal tubule degeneration (81%), and renal interstitial fibrosis (31%) were the major pathological features of 94 children, especially in nephrotic syndrome IgAN. CONCLUSION The number of children with IgAN synchronously accrues as hospitalized population, those with kidney diseases, and those by kidney biopsy. Hematuria is the major symptom. To routinely perform urine analysis and kidney biopsy in asymptomatic hematuria may improve the diagnosis. Inflammation infiltration, renal tubule degeneration, and renal interstitial fibrosis are the major pathological features in IgAN children, especially in nephrotic syndrome IgAN, probably relating to continuous proteinuria. Early control of proteinuria may delay or decrease renal tubule fibrosis.
Adolescent ; Biopsy, Needle ; Child ; Child, Preschool ; China ; epidemiology ; Female ; Glomerulonephritis, IGA ; complications ; epidemiology ; pathology ; Hematuria ; diagnosis ; etiology ; Hospitalization ; statistics & numerical data ; Humans ; Kidney ; pathology ; Male