Analysis of clinical diagnosis and treatment of 20 children with hypophosphatemic tickets / 中华实用儿科临床杂志

Objective To analyze the clinical diagnosis and treatment data of 20 children with hypophosphatemic rickets (HR) in order to improve the clinical diagnosis and treatment of HR.Methods The retrospective analysis of clinical data of 20 cases with HR who were hospitalized at Children's Hospital of Nanjing Medical University from May,2010 to April,2016 was performed to summarize the clinical characteristics.All patients were analyzed for the phosphate regulating gene with homologies to endopeptidase on the X chromosome(PHEX) gene by direct sequencing.If no mutations were detected,multiplex ligation-dependent probe amplification analysis was performed.Results All of the 20 cases with HR showed different degrees of growth retardation and typical X-ray rickets.After treatment,the clinical features were improved.Height standard deviation score (HSDS) was improved significantly with longer treatment time,and the difference was statistically significant(P =0.027).There was a correlation between the blood phosphorus fluctuation and secondary hyperparathyroidism(P ＜ 0.05).Nineteen cases had PHEX gene mutations.Truncating mutations was the most frequent mutation type,and 4 new mutations were found.Conclusions Clinical characteristics,laboratory test results and X-ray examination are important clinical index for the diagnosis of HR,and PHEX gene test can be used as an important auxiliary diagnostic tool.Early diagnosis and treatment can significantly improve the clinical manifestations of the patients.

Oxidative stress-dependent Ras-ERK activation involves in aldosterone-induced mesangial cell proliferation / 中华肾脏病杂志

Objective To investigate the role of oxidative stress-dependent Rasextracellular signal-regulated kinase (ERK1/2) signaling in aldosterone (ALDO)-induced mesangial cell proliferation. Methods The incorporation of 3H-thymidine (3H-TdR) and cell count were used as the measure of mesangial cell (MC) proliferation.Western blotting was used to detect the activation of Ki-RasA,c-Raf,MEK1/2,ERK1/2 and PI3K. Results Aldosterone significantly induced human mesangial cell proliferation,and anti-oxidant N-Acetylcysteine (NAC),catalase,and super oxide dismutase (SOD) significantly inhibited ALDO-induced mesangial cell proliferation (P＜0.01,respectively).Stimulation by ALDO for 3 h,Ki-RasA,c-Raf,MEK1/2,and ERK1/2 activity increased by 4.05-, 3.62-, 4.52-, and 3.40-fold compared with control group (P ＜0.01,respectively).NAC almost completely blocked ALDO-induced Ki-RasA,c-Raf,MEK1/2,and ERK1/2 activation (P＜0.01,respectively).Ki-RasA siRNA dose-dependently inhibited Ki-RasA expression, ALDO-induced Ki-RasA activation, and mesangial cell proliferation (P ＜0.01,respectively).c-Raf inhibitor GW5074 and MEK1/2 inhibitor PD98059 also reduced ALDO-induced mesangial cell proliferation by 65％ respectvely (P＜0.01).Ki-RasA siRNA had no effect on ALDO-induced PI3K phosphorylation.Combining LY294002 and PD98059 completely blocked ALDO-induced mesangial cell proliferation (P＜0.01). Conclusions ALDO-induced Ki-RasA-c-Raf-MEK-ERK signaling activation is dependent on reactive oxygen species (ROS) production,which mediates ALDO-induced mesangial cell proliferation.Inhibition of both ERK1/2 and PI3K signaling simultaneously completely blocks ALDO-induced mesangial cell proliferation.

Clinicopathological features and treatment of C1q nephropathy in children / 中华肾脏病杂志

Objective To investigate the clinicopathological characteristics and treatment of C1q nephropathy in children.Methods Data of 23 C1q nephropathy cases in Nanjing Children's Hospital during recent eight years were retrospectively reviewed. Results The incidence of C1q nephropathy was 4.78％ in primary glomerulonephritis proven by biopsy.Among 23 patients,15 were boys and 8 were girls.The mean age at onset was (5.0±3.4) years old with a range of 0.9-12.4 years.The clinical manifestations included nephrotic syndrome(NS) in 18 cases (78.3％),nephrotic-range proteinuria in 4 cases(17.4％) and microhematuria in 1 case.Two patients with NS and one patient with nephrotic-range proteinuria also presented microhematuria.One patient with NS who received oral herbal medicine for two weeks developed acute renal insufficiency at the same time of diagnosis.Three cases had a family history of kidney disease,among them two patients(presented nephrotic range proteinuria) were siblings,their father had proteinuria as well,and routine genetic examination confirmed familial Denys-Drash syndrome in association with C1q nephropathy.One NS patient's sister had nephrotic-range proteinuria too,but renal biopsy was not performed.No patient had hypertension.None of the patients had low C3 or C4 levels,and serological markers of systemic lupus erythematosus were absent.Light microscopy showedminimalchangedisease (MCD)in13cases (56.5％), mesangialproliferative glomerulonephritis(MsPGN) in 6(26.1％) and focal segmental glomerulosclerosis(FSGS) in 4(17.4％).Immunofluorescence displayed C1q co-deposits of IgG(78.3％),IgM(78.3％),IgA (34.8％) and C3 (47.8％),and a full-house pattern was found in 6 patients (26.1％).Electron microscopy revealed 4 out of 19 had mesangial deposits,except for 4 patients whose glomerulus could not be found.Children with either NS(18 cases) or nephrotic-range proteinuria(2 cases)received prednisone,among them,15 were steroid-resistant,4 were steroid-dependent,only 1 was steroid-sensitive.Those with steroid-resistant(15 cases) or steroid-dependent(3 cases) received further immunosuppression with cyclophosphamide(CTX) or cyclosporine A (CsA).One NS case of steroid-dependent received prednisone re-induction therapy.The siblings associated with DenysDrash syndrome and one case presented microhematuria were commenced on angiotensin-converting enzyme inhibitor(ACEI).Of the 19 patients with sufficient follow-up date,15 cases (78.9％)achieved complete remission,2 cases(10.5％) achieved partial remission,and 2 cases (10.5％) were ineffective.Median follow-up was 15 months.Remission of the NS occurred in 94.4％ (17/18)while nephrotic-range proteinuria was 50.0％(2/4).Remission of MCD was 100.0％,MsPGN was 83.4％(5/6),but FSGS was only 50.0％(2/4).Conclusions C1q nephropathy is rare,and often manifests as steroid-resistant or steroid-dependent NS and nephrotic-range proteinuria.The most common histological feature is MCD,and some as MsPGN or FSGS.A combination of prednisone and immunosuppressive agent is always effective for MCD and MsPGN,but FSGS always has a poor response.

Clinical study of early enteral nutrition on prevention of stress ulcer in patients with severe craniocerebral brain injury / 中国实用护理杂志

Objective To study the effect of early enteral nutrition on stress ulcer in patients with severe craniocerebral injury.Methods 134 patients with severe craniocerebral injury were randomly divided into two groups,the early enteral nutrition group (the EEN group,69 cases) was given nasogastric intubation feeding after hospitalization or within 24 hours postoperation,the delayed enteral nutrition group (the DEN group,65 cases) was given nasogastric intubation feeding 24 hours postoperation.The incidence of upper gastrointestinal bleeding in two groups were compared.Results The incidence of upper gastrointestinal bleeding in the EEN group was significantly lower than that of the DEN group.Conclusions Patients with severe brain injury should be given enteral nutrition as soon as possible to reduce the occurrence of stress ulcer and prevent upper gastrointestinal bleeding.

Analysis of clinicopathology and prognosis of childhood Henoch-Schonlein purpura nephritis with diffused endothelial cell proliferation / 中华肾脏病杂志

Objective To investigate the clinicopathological characteristics and prognosis of Henoch-Schonlein purpura nephritis with diffused endothelial cell proliferation (DEP-HSPN) in children. Methods Data of 8 DEP-HSPN cases in Nanjing Children's Hospital within recent ten years were retrospectively reviewed. The clinicopathological features, efficacy and prognosis were compared between DEP-HSPN cases and 48 cases of non-DEP-HSPN. Non-DEP-HSPN cases were divided into two groups according to the clinical classification or the pathological classification.Results (1) In DEP-HSPN, HSP developed nephritis within 4 to 15 days after the initial onset of purpuric rashes. Hematuria was present in all the 8 patients. The main clinical manifestation of DEP-HSPN was nephritic-nephrotic syndrome (4 cases), nephrotic level proteinuria (3 cases) and acute nephritic syndrome (1 case). Four cases had macrohematuria. Six cases had abdominal symptoms and two cases had arthritis. Pathology of all the cases showed grade Ⅲ-b lesion with diffused endocapillary proliferation and segmental necrotizing lesion of the capillary wall, always accompanied with intraglomerular inflammatory cell infiltration. Crescent was found in 4 cases. (2)Compared to non-DEP-HSPN grades Ⅲ, DEP-HSPN showed a shorter course of disease.Macrohematuria, heavy proteinuria, nephritic-nephrotic syndrome, and segmental necrotizing lesion of capillary wall were more common in DEP-HSPN. Compared to non-DEP-HSPN with nephrotic level proteinuria, DEP-HSPN had a lower rate of crescent. (3) Methylprednisolone pulse therapy in early stage, then prednisone combined with cyclophosphamide were used in the treatment of DEP-HSPN.After an average follow-up period of seven months, one patient showed complete remission, five showed persistent microhematuria, and two showed persistent microhematuria accompanied with minor proteinuria. No significant difference of prognosis was found between DEP-HSPN and nonDEP-HSPN. Conclusions DEP-HSPN has an acute onset. The main clinical manifestation of DEP-HSPN is nephritic-nephrotic syndrome and nephrotic level proteinuria, always accompanied with macrohematuria. Immunosuppressant treatment in the early stage of disease is effective for a short-term outcome.

ROS-EGFR-JNK-AP-1 signaling pathway involves in angiotensin Ⅱ-induced human mesangial cells proliferation / 中华肾脏病杂志

Objective To elucidate whether Ang Ⅱ indnces the proliferation of mesangial cells through ROS-EGFR-JNK-AP-1 signaling pathway. Methods The incorporation of 3H-thymidine (3H-TdR) and cell count were used to measure mesangial cell (MC) proliferation. ROS production was determined by DCFDA fluorescence. EGFR and JNK activation was assayed by Western blot. Results Ang Ⅱ significantly enhanced ROS production in mesangial cells, which was up-regulated by 2.26 folds of control group after incubation with Ang Ⅱ for 60 min. Ang Ⅱ induced EGFR phosphorylation in dose- and time-dependent manner, with the peak (3.96 folds increase) at 30 min. EGFR phosphorylation was significantly blocked by AT1R antagonist losartan, antioxidant NAC, and NADPH oxidase inhibitor apocynin and DPI. EGFR antagonist AG1478 significantly inhibited Ang Ⅱ-induced mcsangial cell proliferation. Losartan, NAC, apocynin, DPI, and AG1478 ahnost abolished Ang Ⅱ-induced JNK activation. Conclusions ROS-EGFR-JNK-AP-1 signaling pathway is involved in Ang Ⅱ-induced mesangial cell proliferation. Apocynin and AG 1478 may be used as new therapy.

The Experimental Study of Garlicin in Treating Pneumocystis carinii Pneumonia in Rats / 中国寄生虫学与寄生虫病杂志

Objective To assess the therapeutic effect of garlicin on Pneumocystis carinii pneumonia (PCP) of immuosuppressed rats. Methods The Wistar rats were injected intramuscularly continually with dexamethasone for eight weeks to establish the rat model of PCP. The rats were treated with garlicin, meanwhile control groups without treatment and with SMZ-TMP treatment were established respectively in PCP model. The efficacy was evaluated based on the lung weight, lung /body weight ratio and mean cyst number per 100 fields in lung print smear. Results The mean lung weight of the rats in garlicin is 1.73?0.17, lung/body weight ratio is 0.84?0.12, they are obvious lower than 2.31?0.35 and 1.25?0.35 of control (P

Determination of Trace Elements in Hair of Mice Infected with Trichinella spiralis / 中国寄生虫学与寄生虫病杂志

Mice were divided into 3 groups:heavy infection group with 80 mice each was fed with 400 muscle larvae of Trichinella spiralis,light infection group with 60 mice each was fed by 200 larvae,and uninfected control (60 mice). The content of Cu,Zn and Fe in the dorsal hair samples was measured in the week of 1,3,5,7,9,11,13 and 15 after infection. Results indicated that the content of Zn,Cu and Fe in the two experimental groups reduced considerably in comparison to the control(P

CHARACTERIZATION,ISOLATION AND CULTURE OF OSTEOCLASTS FROM NEONATAL RAT LONG BONES / 解剖学报

Osteoclasts were isolated mechanically from neonatal rat long bones and cultured on glass or devitalized human cortical bone slices. Both morphological and functional studies showed that the isolated cells were of typical osteoclasts, because; 1. they attached rapidly on the matrix and had pseudopodial activity when cultured on glass;2. they had multinuclei,most of them contained 3-10 nuclei;3. calci-tonin and clacitonin gene related peptide directly inhibited the cellular activity; 4. they showed high acid phosphatase activity; 5. resorption lacunae could be found when the cells cocultured with devitalized bone slices.