Comprehensive review of 18 cases with Abernethy malformation in China from 2001 to 2012 / 中华实用儿科临床杂志

Objective To acquire more knowledge about Abernethy malformation.Methods Eighteen cases with Abernethy malformation published previously in China from 2001 to 2012 were reviewed.The clinical,radiological and pathological manifestations and treatment were summarized.Results Of 13 cases with type Ⅰ Abernethy malformation (including 6 type Ⅰ a and 7 type Ⅰ b,5 male and 8 female),11 cases occurred by the age of 18 years.Their clinical manifestations included hematochezia in 4,hematemesis in 4,liver dysfunction in 5,liver cirrhosis in 2,hepatic encephalopathy in 2,hepatic nodule in 4,splenomegaly in 5,hypersplenism in 4,portal hypertension in 3 and other associated malformations in 3.Of 5 cases with type Ⅱ Abernethy malformation,2 cases occurred by the age of 18 years.Their clinical manifestations included liver dysfunction in 4,hematochezia in 1,liver cirrhosis in 1,hepatic encephalopathy in 1,hepatic nodule with focal nodular hyperplasia in 1.All 18 patients underwent imaging evaluation,multi-slice computed tomography(CT) angiography (MSCTA) in 16,including vascular ultrasound in 15,digital subtraction angiography (DSA) in 7,magnetic resonance angiography (MRA) in 1 and magnetic resonance imaging (MRI) in 1.The sites of drainage for portal vein system in 15 cases were documented,including the inferior vena cava (2/15 cases),internal iliac vein (5/15 cases),left renal vein (3/15 cases),azygos vein (2/15 cases),right atrium (2/15 cases) and pelvic venous plexus (1/15 case).And there was no record of specific draining site in other 3 cases.Ballooning degeneration of liver cells,liver cells nodular hyperplasia and fatty degeneration were detected in 2 cases of type Ⅰ Abernethy malformation.And liver focal nodular hyperplasia was demonstrated in 1 case of type Ⅱ Abernethy malformation.Four cases with hematochezia were misdiagnosed as ulcerative colitis,hemorrhoids or purpura.Two cases with clitoral hypertrophy were misdiagnosed as genital malformations.Due to crying,one 5-month-old baby was misdiagnosed as sepsis.Of these 18 cases of Abernethy malformation,most of the cases received conservative treatment.Surgical techniques such as splenectomy,surgical ligation of the veins of sigmoid and interventional embolization to occlude the shunt were used to treat splenomegaly,hematochezia,hematemesis or hepatic encephalopathy.Conclusions Abernethy malformation mainly occurs in children.Clinical presentation is nonspecific.Liver dysfunction,hepatic nodule,hematochezia,hematemesis,plenomegaly and hypersplenism are common manifestations.Compared with overseas reports,hepatic nodule,hepatic encephalopathy and concomitant abnormalities are rare,while hematochezia and hematemesis are more common.The diagnosis is made primarily according to imaging changes,and MSCTA is a useful tool to make a diagnosis.Individualized treatment of Abernethy malformation is determined by the type of deformity and the conditions of the patient.

Alteration of glomerular anionic sites and expression of renal heparanase in respiratory syncytial virus nephropathy in rats / 中华实用儿科临床杂志

Objective To observe the alteration of glomerular anionic sites and renal heparanase (Hpa)expression in respiratory syncytial virus(RSV) nephropathy in rats,and to investigate the role of Hpa in the pathogenesis of proteinuria in RSV nephropathy.Methods Twenty-five Sprague-Dawley (SD) rats were inoculated with 6 x 106plaque forming units(PFU) RSV and were sacrificed on days 4,8,14 and 28 post-inoculation(RSV4,RSV8,RSV14 and RSV28 group).Five normal SD rats served as normal control.The proteinuria and serum parameters were measured.Glomerular anionic sites were measured by histochemical electron microscopy.The expression of Hpa in kidney was determined by immunohistochemical staining.The relationship between the expression level of Hpa and the quantity of 24-hour urine protein was studied.Results After inoculation,the proteinuria increased,especially in RSV14 group.The 24-hour urine protein of RSV14 group,RSV8 group,RSV28 group,RSV4 group and normal group were (30.9860 ± 3.3464)mg,(15.3212 ± 1.2249) mg,(13.9193-2.1409) mg,(11.5857± 1.5705) mg,(3.9780 ± 0.6224) rag,respectively.The serum albumin of RSV14 group decreased.The anionic sites of glomerular basement membrane(GBM) decreased in RSV nephropathy.The number of anionic sites per 1000 nm GBM of RSV14 group,RSV8 group,RSV28 group,RSV4 group and normal control group were 12.0000 ± 1.5811,14.0000 ± 1.0000,14.6000 ± 1.1401,16.8000 ± 0.8366 and 21.2000 ± 1.3038,respectively.Hpa in glomeruli couldn't be detected in normal rats.Glomerular Hpa expression was up-regulated in RSV nephropathy.The expression of glomerular Hpa of RSV14 group,RSV8 group,RSV28 group and RSV4 group were 0.6622 ±0.1145,0.5511 ± 0.0257,0.3524 ± 0.0296 and 0.4521 ± 0.0087,respectively.The expression level of Hpa in RSV8 group and RSV14 group was higher than that in RSV4 group and RSV28 group.There was a linear positive correlation between the expression level of glomerular Hpa and the quantity of 24-hour urine protein (r =0.783,P ＜ 0.05).Conclusions The increased expression of glomerular Hpa in RSV nephropathy of rats leads to loss of the glomerular anionic sites and damage of the electrostatic barrier of GBM,which promote the proteinuria.

Mechanical ventilation in acute respiratory distress syndrome / 中国当代儿科杂志

The goal of mechanically ventilating patients with acute respiratory distress syndrome (ARDS) is to ensure adequate oxygenation and minimal ventilator-associated lung injury. Non-invasive ventilation should be cautiously used in patients with ARDS. Protective ARDS mechanical ventilation strategies with low tidal volumes can reduce mortality. Driving pressure is the most reasonable parameter to optimize tidal volume. Available evidence does not support the routine use of higher positive end expiratory pressure (PEEP) in patients with ARDS. The optimal level of PEEP may be titrated by the inflection point obtained from static pressure-volume curve. Promising therapies include prone position ventilation, high frequency oscillatory ventilation and extracorporeal membrane oxygenation as salvage treatment. While mechanically ventilating, it is also important for ARDS patients to maintain spontaneous breathing via assisted ventilation mode such as bilevel positive airway pressure, pressure support ventilation and neurally adjusted ventilation assist. Exogenous surfactant, inhaled nitric oxide, bronchodilators, airway pressure release ventilation and partial liquid ventilation are not recommended therapies.

Mechanism for promoting repair of renal ischemia reperfusion injury by mesenchymal stem cells / 中国当代儿科杂志

Preclinical studies have demonstrated that exogenous mesenchymal stem cells (MSCs) may ameliorate kidney damage and enhance repair of renal ischemia reperfusion injury (IRI). This review will focus on the mechanism for accelerating repair of renal IRI by MSCs. Several chemokine receptors such as CXCR4 and CD44 are related to MSCs trafficking to post-ischemic kidney. MSCs differentiate into tubular epithelial cells, which is not the predominant mechanism for repair of the damaged kidney. Instead, MSCs exert their therapeutic effect mainly through paracrine action via a variety of cytokines and microvesicles, and the paracrine actions of infused MSCs work to activate intrinsic kidney cells, promote angiogenesis, inhibit oxidative stress and reduce apoptosis, inflammation and renal fibrosis.

Isolation and analysis of differentially expressed genes from peanut in response to challenge with Ralstonia solanacearum

Background: Bacterial wilt caused by Ralstonia solanacearum is the most devastating disease in peanut. Planting resistant peanut cultivars is deemed as the sole economically viable means for effective control of the disease. To understand the molecular mechanism underlying resistance and facilitate breeding process, differences in gene expression between seeds of Rihua 1 (a Virginia type peanut variety resistant to bacterial wilt) inoculated with the bacterial pathogen suspension (10(9) cfu ml-1) and seeds of the same cultivar treated with water (control), were studied using the GenefishingTM technology. Results: A total of 25 differentially expressed genes were isolated. Expression of genes encoding cyclophilin and ADP-ribosylation factor, respectively, were further studied by real time RT-PCR, and full length cDNAs of both genes were obtained by rapid amplification of cDNA ends. Conclusions: The study provided candidate genes potentially useful for breeding peanut cultivars with both high yield and bacterial wilt resistance, although confirmation of their functions through transgenic studies is still needed.

Relationship between Expression of Respiratory Viruses from Urine in Children with Steroid Responsive Simple Nephrotic Syndrome and Its Episode / 实用儿科临床杂志

Objective To explore the relationship between respiratory viruses infection and the episode of steroid responsive simple nephrotic syndrome(SRSNS).Methods Thirty-eight children with SRSNS were selected,including the active stage group(n=28)and the remission group(n=10).The urine from 18 cases of nephritic nephrosis,16 cases of bronchiolitis,15 cases of secondary glomerular diseases and 15 cases of healthy children were served as controls.By using the method of reverse transcriptase-polymerase chain reaction(RT-PCR)and alkaline phosphoesterase-anti alkaline phosphoesterase enzyme-linked assay(APAAP),viral genes and antigens in urine were assayed,respectively.Results The positive rate of detecting viruses in the active stage group of SRSNS children was higher than that in the remission group and the other control groups(Pa

Viral Transactivation of Transcription in Steroid Responsive Simple Nephrotic Syndrome Mediated by Nuclear Factor-Kappa B/Inhibitor Protein-Kappa B Signal Pathway / 实用儿科临床杂志

Objective To investigate the role of nuclear factor-kappa B(NF-?B)/inhibitor protein-kappa B(I?B) signal pathway in viral transactivation of transcription in steroid responsive simple nephrotic syndrome(SRSNS).Methods Children with SRSNS(inclu-ding active stage and remissive stage) were examined,and were compared to children with nephritic nephrosis,secondary glomerular di-seases,bronchiolitis and healthy children.Electro-mobility shift assays,reverse transcription-polymerase chain reaction(RT-PCR) and enzyme-linked immunosorbent assay(ELISA) were used to detect the activity of NF-?B,the gene expression of respiratory tract viruses (including respiratory syncytial virus and influenza virus) in peripheral blood mononuclear cells(PBMCs) and the levels of viral antibody in plasma,respectively.The protein levels of I?B? and IL-8 were measured through Western blot and ELISA in SRSNS at active stage and healthy children.Results Compared with SRSNS at remissive stage and other groups,the activity of NF?B in SRSNS at active stage was much higher.And there was a positive linear correlation trend between the activity of NF-?B and the gene expression of respiratory tract viruses in SRSNS at active stage.With healthy children,the level of IL-8 in plasma from SRSNS at active stage was significantly increased.There was a positive correlation between the activity of NF?B and the level of IL-8(r=0.88 P

Heparanase expression of peripheral blood cells in steroid responsive nephrotic syndrome patients / 中华儿科杂志

<p><b>OBJECTIVE</b>The beta-D-endoglycosidase heparanase (Hpa) is HS-specific which leads to the degradation of heparan sulfate (HS). An increased permeability of the glomerular basement membrane (GBM) for proteinuria was suggested to relate to a decrease of HS side chains in the GBM. However, whether an up-regulated expression of Hpa exists in steroid responsive nephrotic syndrome (SRSNS) remains unknown. The purpose of this study was to evaluate the role of Hpa in the development of SRSNS and the correlation with the proteinuria.</p><p><b>METHODS</b>Forty-three children with SRSNS were selected and included the active stage group (n = 23), the restoration stage group (n = 10) and the remission stage group (n = 10). There were 23 nephritic nephrosis children, 15 purpura nephritis children and 15 healthy children as controls. By using the method of reverse transcriptase-PCR (RT-PCR), Hpa gene expression in the peripheral blood leukocytes (PBLs) was assayed.</p><p><b>RESULTS</b>(1) All patients with nephrotic syndrome exhibited higher levels of Hpa mRNA than those of the healthy group (P < 0.05). The highest expression was in the active stage group of SRSNS (1.27 +/- 0.36, P < 0.001), while there was no difference between the patients of nephritic nephrosis group (0.62 +/- 0.15) and purpura nephritis group (0.55 +/- 0.17) (P > 0.05). (2) In contrast with the healthy group, there was a significant difference in the active stage group of SRSNS (P < 0.001). So was the restoration stage group (P < 0.05), but there was no difference to the remission stage group (P > 0.05). (3) There was a positive correlation between the expression level of Hpa mRNA and the quantity of urinary protein (r(s) = 0.751, P < 0.001).</p><p><b>CONCLUSION</b>Up-regulated expression of Hpa mRNA may be important to the loss of glomerular negative charge in GBM and lead to proteinuria in SRSNS.</p>