Neonatal diabetes mellitus ( NDM ),which was often misdiagnosed as type 1 diabetes in the past,is a heterogenous single-gene genetic disease.Permanent neonatal diabetes( PNDM )is mainly associated with mutation in KCNJ11,ABCC8,and insulin associated gene instead of immunity.The most common manifestation includes diabetic ketoacidosis,intrauterine growth retardation and dehydration.Gene examination contributes to the classification of NDM and corresponding targeted therapy.Oral sulfonylurea may be used in treating patients with gene mutation of ATP-sensitive K+ channel.

To improve the quality of health care by improving the quality of residency training,the Accreditation Council for Graduate Medical Education of USA has renewed the standards and methods of residency training to improve health care,and issued the compe- tence-based training standards. The residents have to achieve six key competences when they finish the training. The University of Washington,School of Medicine,responds to the new re- quirements and standards by reforming its residency training pathway,enriching formal teach- ing/training activities,and improving its supervision and the evaluation. We should use for refer- ence reforming the residency training in China.

Objective To analyze the clinical and pathological features of Wegener granulomatosis (WG) in children in order to improve the understanding of this disease. Methods Nine cases of WG,including two cases from our hospital and seven cases throughout the country,were analyzed in aspects of their clinical characteristics,laboratory examinations,X-ray and treatments. Results Of the nine cases,six cases were male and three cases were female with average age of twelve years old. The average time from the initiation of clinical symptom to diagnosis was 8.3 months. Patients had multiple systems and organs involvements. Lung and kidney involvement were the most common. Six out of seven were positive in c-ANCA and anti-PR3 antibody. Biopsy was conducted in eight patients,and pathological manifestations involved parenchymal necrosis,granulomatosis,vasculitis and so on. Most patients improved significantly after being treated with glucocorticoids and cyclophosphamide. Conclusion WG is a syndrome which involves many organs without distinctive clinical manifestation. An ANCA test should be performed to make an early diagnosis.

Not only does autophagy play a vital role in maintaining kidney cell survival and homeostasis,but al-so it is involved in the pathophysiology of several kidney diseases. Recent studies have indicated that autophagy was ac-tivated in kidney cells during acute kidney injury and its regulatory mechanism was unclear. Autophagy activation in kidney may be associated with oxidative stress, endoplasmic reticulum stress, hypoxia inducible factor-1α, p53 and Bcl-2 family. The role of autophagy in acute kidney injury is still controversial. Most believe that it plays a protective role during acute kidney injury. Therefore autophagy will become a novel and potential target for the prevention and treatment of acute kidney injury.

This review focuses on the current literatures on the therapeutic potential of non-renal derived adult stem cell transplantation for kidney disease.Although the therapeutic mechanism remains debatable,some reports suggest that hematopoietic stem cell transplantation holds potential for treatment of renal diseases such as renal ischemia reperfusion injury,IgA nephropathy and lupus nephritis.Mesenchymal stem cell(MSC) are capable of homing to injured kidney and contribute to activate intrinsic kidney cells,promote angiogenesis,inhibit oxidative stress and reduce apoptosis,inflammation and renal fibrosis.These renoprotective effects are likely mediated by paracrine/autocrine mechanism via cytokine and microvesicles from MSC.Pluripotent stem cells may be induced from fibroblast,mesangial cells,renal tubular epithelial cell and urine epithelial cell.Induced pluripotent stem cell(iPSC) can differentiate into renal lineage cells.The derivation of kidney disease-specific iPSC opens new avenues for the resolution to limited donor availability in kidney transplantation and for treating kidney diseases such as diabetic nephropathy and autosomal dominant polycystic kidney disease.

ObjectiveTo determine the levels of 8-iso-prostaglandin F2α (PGF2α) in sera and lesions as well as antioxidant capacity in sera of psoriatic patients,and to assess their correlations with disease severity.MethodsSerum and skin tissue samples were collected from 15 healthy controlsand 50 patients with psoriasis vulgaris.Spectrophotometry was performed to determine the levels of total antioxidant capacity (T-AOC) and the activities of antioxidant enzymes such as superoxide dismutase (SOD),catalase (CAT) and glutathione peroxidase (GSH-Px) in serum samples.Enzyme-linked immunosorbent assay (ELISA) and immunohistochemical SP method were carried out to detect the expression level of 8-iso-PGF2α in the serum and tissue specimens respectively.ResultsThe psoriatic patients showed a significant decrease in the serum level of TAOC((12.78 ± 7.75) U/ml vs. (23.17 ± 8.81) U/ml,P＜ 0.01) as well as the activities of SOD((28.91 ±9.35) U/ml vs.(51.36 ± 7.92) U/ml,P＜ 0.01) and GSH-Px ((180.64 ± 47.70) U vs.(244.20 ± 66.68) U,P ＜ 0.01 ) compared with the healthy controls.The serum T-AOC level and SOD activity were lower in patients with severe psoriasis than those with mild or moderate psoriasis ((9.06 ± 5.30) U/ml vs. (15.27 ± 8.18) U/ml,(21.63 ± 5.28) U/ml vs. (33.76 ± 8.28) U/ml,both P＜ 0.01 ),while there was no significant difference in the activity of GSH-Px between patients with severe and mild or moderate psoriasis.The serum CAT activity was significantly higher in patients with mild or moderate psoriasis than in the healthy controls and patients with severe psoriasis ( (36.92 ± 11.31 ) U/ml vs.( 28.55 ± 8.51 ) U/ml and (24.15 ± 9.36 ) U/ml,P ＜ 0.05 and 0.01 ).Increased serum and lesional 8-iso-PGF2α levels were observed in psoriatic patients compared with the healthy controls ( (88.77 ± 25.27) ng/L vs.(38.34 ± 8.94) ng/L,0.0186 ± 0.0082 vs.0.0027 ± 0.0014,both P ＜ 0.01),as well as in patients with severe psoriasis compared with those with mild or moderate psoriasis(( 114.24 ±13.93) ng/L vs.(71.78 ± 14.35) ng/L,0.0279 ± 0.0027 vs.0.0125 ± 0.0030,both P＜ 0.01 ).The psoriasis area and severity index(PASI) score was negatively correlated with T-AOC level,SOD and CAT activities(r =-0.384,-0.573 and -0.444,all P ＜ 0.01 ),positively correlated with serum and lesional 8-iso-PGF2α levels (r =0.710,0.783,both P ＜ 0.01 ),and uncorrelated with GSH-Px activity.None of the parameters was correlated with the course of disease.ConclusionThe serum and lesional levels of 8-iso-PGF2α may be a more sensitive marker for oxidative damage and disease severity.

Objective To investigate the cerebral regularity of dynamic charge and the correlation with intracranial pressure(ICP),clinical seriousness and prognosis on acute stage of intracerebral hemorrhage(ICH) patients by transcranial Doppler(TCD).Methods 54 patients with first acute supratentorial ICH 24h after onset were studied.All patients were dynamic evaluated by beside TCD on 1st,7th,21st day.The correlation of TCD with ICP,MESSS were studied.Results On the 1st day the TCD abnormity of the acute phase of the ICH occurred mainly in the anterior circulation of cerebrum,in which the cardinal manifestation was high-resistance,low-flow velocity blood waveforms,especially on the bleeding side.Continuous TCD monitoring revealed the low velocity of the cerebral blood flow increased;meanwhile the high value of PI decreased gradually and the TCD monitoring was within the normal range three weeks after the attack.The ICP had negative correlation with the VmMCA,positive correlation with the PI value.MESSS scale had negative correlation with the VmMCA,positive correlation with the PI value.Conclusions The TCD examination can be used to monitor the cerebral hemodynamics of the patients suffering from intracerebral hemorrhage.The combination of fVEP and TCD facilitate the evaluation of the dynamic changes of ICP so as to instruct lowering the ICP.

Arthritis, Juvenile ; therapy ; Child ; Humans ; Macrophage Activation Syndrome ; therapy ; Plasmapheresis

Objective To improve the diagnosis and treatment of adenocarcinoma of bladder. Methods The data of 20 cases of adenocarcinoma of bladder from 1986 to 2004 were retrospectively analyzed. All patients underwent operation and the diagnosis was proven by histopathologic examination. Results Eighteen cases were primary adenocarcinoma of bladder,8 cases applied to radical cystectomy and 10 cases partial cystectomy. Two cases were urachal adenocarcinoma,and received extended partial cystectomy.Seventeen patients were followed up for 3 months to 5 years. The one-year survival rate was 52.9% (9/17),two-year survival rate 41.2% (7/17),and five-year survival rate 29.4% (5/17). Conclusion Radical total cystectomy is the best choice for primary adenocarcinoma of bladder and extended partial cystectomy for urachal adenocarcinoma. Radiotherapy and chemotherapy after operation can improve the treatment effect.

Objective To explore the effect of evidence-based medicine combined withcase based learning(CBL) in pediatric residency training.Methods Totally 52 residents were equally divided into two groups:experiment group and control group.Method of CBL combined with the evidencebased medicine (Taking two residents as one group,firstly teacher posed the questions and then residents searched for references when off duty and made evidence-based case presentation at fixed time when doing teaching ward around,after wards all personnel in the department made case discussion,finally teacher made the summary) was used in experimental group while traditional teaching method (teacher guided the residents to analyze the case by clinical experiences,residents took notes and sometimes asked questions,teacher directly gave the answers)was used in control group.The teaching effect is evaluated by questionnaire,theory test and clinical skills assessment.Statistical analysis was carried out on appraisal results with SPSS 16.0 software,test results and appraisal results were expressed as ((x-) ±s) and t test was performed,P ＜ 0.05 was taken as statistically significant.Results Residents in experimental group had more advantages in self-study ability,literature retrieval ability,physician teamwork spirit,clinical thinking,PowerPoint making and language expression after receiving CBL combined with evidence-based medicine.Theory examination showed that average score of experimental group was better than that of control group (75.46 ± 6.646) vs.(71.38 ±4.758),with statistically significantly differences (P =0.014),especially in subjective items.Clinical skills assessment showed that experimental group was better than control group in writing medical records and doing clinical thinking.Conclusions Teaching method of evidence-based medicine combined with CBL is significantly better than traditional indoctrination teaching in improving the abilities of self-study,cultivating clinical thinking,literature retrieval,language performance and other skills.This teaching method is more suitable for pediatric residency training.