Object To develop a method for the determination of palmatine hydrochloride and berberine hydrochloride in Chinese Mahonia Stem from different habitats Methods HPLC method was set up, using Intersil ODS 3 C 18 column, the mobile phase was acetontrile water sodium laurylsulfonate (470∶ 530∶1 g), the UV detection wavelength was 265 nm, with a flow rate of 1 0 mL/min at 40 ℃ Results A good linearity was obtained in the range of 4 368 52 416 ?g/mL(r=0 999 9) for palmatine hydrochloride and 4 532 54 384 ?g/mL (r=0 999 9) for berberine hydrochloride The average recovery of palmatine hydrochloride and berberine hydrochloride was 98 97% and 98 98%, respectively Conclusion The method is simple, rapid and with better reproducibility for the determination of palmatine hydrochloride and berberine hydrochloride in Chinese Mahonia Stem

Objective To analyze the etiology of diabetes ketoacidosis(DKA) in children with type 1 diabetes.Methods Totally 850 person-time of type 1 diabetes children in recent 20 years in our hospital were selected as studied subjects. Two hundred and twenty-five person-time of them were hospitalized because of DKA.Fifty-six cases (131 person-time) were due to recurrent DKA.These patients were classified into 2 groups according to onset time: group 1(diagnosed from 1982 to 1991) and group 2(diagnosed from 1992 to 2001).Results The analysis of recurrent DKA suggested that 71.8 % of them was due to infection, 20.4 % of them did not obey diabetic diet and 9.2 % of them discontinued insulin injection. The etiology of DKA showed no difference in two groups. The number of recurrent DKA in two groups was significantly different (P

Objective To analyze the clinical characteristics of children's Pseudo-Bartter syndrome(PBS) in order to enhance physician's understanding of the disease.Methods Nine children with PBS who were admitted into Beijing Children's Hospital from Nov.2008 to Sep.2013 were selected as research subjects.A retrospective study was carried out with the clinical data and the outcome of treatment.Results 1.Clinical characteristics:there were 9 cases in this group including 5 male and 4 female.The patients' age ranged from 4 months to 8 years 8 months.The most common cause of children's PBS was gastrointestinal symptoms(such as diarrhea and vomiting) induced by respiratory tract infection (7/9 cases).Six patients had no striking clinical manifestations,and hypokalemia was found in the treatment of primary disease.2.Laboratory tests:All of the children in this group had hypokalemia and metabolic alkalosis in varying degrees.The activation of renin,angiotensin and aldosterone system increased.3.Therapy:all children were treated by giving potassium supplemental treatment or indomethacin therapy [1 mg/(kg · d),3 times orally].After treatment,all cases achieved clinical improvement and normal blood electrolytes.All patients' blood electrolytes remained normal for 5 to 7 days after stopping treatment.Conclusions 1.In China,the most common cause of children's PBS is gastrointestinal symptoms(such as diarrhea and vomiting) induced by respiratory tract infection.2.Except for clinical manifestations related to causes,patients have no significant clinical manifestations.Hypokalemia can be found in the treatment of primary disease.3.The biochemical results show low blood potassium chloride with metabolic alkalosis.In PBS renin,angiotensin and aldosterone concentration in blood are all elevated.4.Treatment of children's PBS mainly includes etiological treatment and electrolyte supplement therapy.The treatment effectiveness is good after etiological treatment and potassium supplement treatment.In the condition of controlling etiology and potassium supplementation,electrolytes mas return to normal in 2-4 days.

Objective To analyze the clinical characteristics and the endocrine changes in children with craniopharyngioma,and to improve the pediatrician understanding of the disease.Methods The study subjects consisted of 14 children with craniopharyngioma admitted to the Department of Endocrinology,Beijing Children's Hospital Affiliated to Capital Medical University from Jan.2004 to Dec.2012.All the patients were followed up to analyze the clinical symptoms improvement,endocrine test results and medication,et al.Results The main clinical manifestations were headache (7/14 cases,50.0％),growth retardation(4/14 cases,28.6％),vomiting (4/14 cases,28.6％),polydipsia/ polyuria (3/14 cases,21.4％) and vision diminution (3/14 cases,21.4％).Three patients didn' t undergo the surgery,and 3 cases with diabetes insipidus and 2 cases with growth hormone deficiency,and 1 case with central hypothyroidism by laboratory test.The rest 11 children received surgery and all patients had changes in endocrine after it.Five cases got polydipsia and polyuria,other 5 cases had electrolyte disturbances,and 2 cases had epilepsy.Nine patients were followed up,and the follow-up duration ranged from 5 months to 10 years [(3.29 ± 3.52) years] after surgery.Seven patients got better and 2 patients got worse.Conclusions For clinical symptoms of increased intracranial pressure,changes in endocrine,the vision and visual field,the possibility of craniopharyngioma should be taken into account.Surgery is the main treatment,but it can lead to the damage of hypothalamus and pituitary gland.Changes in endocrine,electrolyte disturbances and epilepsy are the common complications.According to the level of endocrine,longterm hormone replacement therapy for some postoperative patients should be continued.

Biliary tract diseases have always been one of the important indications for liver transplantation. These biliary tract diseases include congenital atresia of biliary duct, biliary diseases with tendency of malignant transformation, malignant biliary diseases, primary biliary cirrhosis, and secondary biliary cirrhosis. The right time to consider liver transplantation for the above biliary diseases includes situations when transplantation is the only approach to treat the disease, the only effective approach to treat the disease, or one of the effective approaches to treat the disease. Currently there is still no commonly accepted pre-operative evaluation standard for biliary disease-related liver transplantation. However, it is important to obey the following principles. First, the diagnosis should be confirmed; secondary, the necessity or urgency of the liver transplantation should be clarified; and third, the patient and his/her family should understand the whole procedure of transplantation the risk and advantage of the surgery, and the potential life-long intake of immunosuppressors. This article will briefly review all these relavant issues based on the authors' personal experience and clinical cases.
Biliary Atresia ; surgery ; Biliary Tract Diseases ; surgery ; Humans ; Liver Cirrhosis, Biliary ; surgery ; Liver Transplantation ; Living Donors

BACKGROUND: To know the effect of droperidol and ondansetron on nausea and sedation in postoperative patients, we studied 120 gynecological patients receiving patient-controlled analgesia (PCA) with morphine and droperidol or ondansetron. METHODS: Subjects were randomly allocated to one of four groups according to PCA regimen, morphine 0.5 mg/cc alone (group M); morphine plus droperidol 0.034 mg/morphine 1 mg (group D); morphine plus ondansetron 0.132 mg/morphine 1 mg (group O1); morphine plus ondansetron 0.066 mg/morphine 1 mg (group O2). The PCA device, WalkMed was set at basal rate 2 ml/hr (1 mg/hr), bolus dose 1 ml (0.5 mg), lockout time 10min, 1 hour maximum dose 4 mg. The severity of nausea, sedation and pain were assessed at 1h, 4h, 8h, 12h, 24h, and 48h postoperatively. RESULTS: The occurrence of nausea was not different among groups. But there were statistical differences in the nausea severity (p<0.05). The group D and group O1 had lower nausea scores, and between them there was no difference. The scores for sedation were significantly lower in the group O1 compared with group M and group D (p<0.05). Overall pain scores were not different among groups. CONCLUSIONS: Ondansetron and droperidol are effective in reducing nausea. Ondansetron is superior to droperidol in avoiding excessive sedation.
Analgesia, Patient-Controlled* ; Droperidol* ; Humans ; Morphine ; Nausea ; Ondansetron* ; Passive Cutaneous Anaphylaxis ; Postoperative Nausea and Vomiting*

PURPOSE: With the process of neoangiogenesis being linked to the growth and metastasis of various tumors, anticancer therapeutics with a basis in the suppression of neoangiogenesis has recently been receiving attention. In this study, we tried to clarify the immunoreactivities of vascular endothelial growth factor (VEGF), major angiogenic inducer and thrombospondin-1 (TSP-1), major angiogenic inhibitor in human Wilms' tumor and its clinicopathological significance. MATERAILS AND METHODS: Utilizing immunohistochemical staining, we assessed the immunoreactivities of VEGF and TSP-1 in archival tissues of 29 Wilms' tumors and 25 normal kidneys. Also, we assessed the relationship between expression of each factor and clinicopathological parameters in 29 cases of Wilms' tumors. RESULTS: Immunoreactivities of VEGF and TSP-1 were detected mainly in the cytoplasm of the tubular cells in normal kidneys. In Wilms' tumors, whereas VEGF was detected in the cytoplasm of the tumor cells and peritumoral stromal tissues, but TSP-1 only in the peritumoral stromal tissues. Immunohistochemical expression patterns of each factor were divided into two groups according to the area of immunoreactivity (negative:<10%, positive: > OR =10%). VEGF immunoreactivity was detected in 25 (100%) normal kidneys and in 20 (69%) Wilms' tumors. However, TSP-1 immunoreactivity was detected in 24 (97%) normal kidneys and in 3 (10%) Wilms' tumors. Therefore, although no significant difference was observed between the expressions of VEGF and TSP-1 in normal kidney, the TSP-1 immunoreactivity was significantly lower than VEGF immunoreactivity in Wilms' tumors. A relatively higher rate of positive expression of TSP-1 was observed in the patients with no demonstrable lymph node metastasis. Also, as for the VEGF, maximal diameter of the tumor was larger in the positive expression group. However, it proved otherwise for TSP-1 as the negative expression group demonstrated tumors with larger maximal diameters. CONCLUSIONS: Our study demonstrated that the TSP-1 immunoreactivity was significantly lower than VEGF immunoreactivity in Wilms' tumors, and disease progression has a tendency to be found in the VEGF-positive cases and TSP-1 negative cases. We suggest that the growth and metastasis of Wilms' tumor may be influenced mainly by TSP-1 decrease rather than VEGF increase.
Cytoplasm ; Disease Progression ; Humans ; Kidney ; Lymph Nodes ; Neoplasm Metastasis ; Thrombospondin 1 ; Vascular Endothelial Growth Factor A* ; Wilms Tumor*

OBJECTIVETo analyze the reasons why anterior decompression and titanium mesh fusion for cervical spondylosis always show poor therapeutic effects, and to investigate the clinical effects of anterior revision surgery in these patients.

METHODSFrom January 2004 to December 2011, 16 patients underwent anterior decompression and titanium mesh fusion for cervical myelopathy were treated with anterior revision surgery. There were 7 males and 9 females with an average age of 61 years old (ranged from 46 to 75 years), including 11 cases with cervical spondylotic myelopathy, 2 cases with nerve root cervical spondylosis and 3 cases with mixed type cervical spondylosis. Average duration from the first operation to reoperation was 7 years(ranged from 4 to 12 years). In the first operation, titanium mesh segment located in C3-C5 (2 cases), C4-C6 (8 cases), C4-C7 (2 cases), C5-C7 (4 cases), and one of them, titanium mesh implantation in C4 and C5,6 intervertebral disk removal and cage fusion. After the first operation, symptom of 13 patients recurred after improvement or disappearance, 2 patients did not show obvious improvement, and 1 patient aggravated. Cervical spine radiography, CT scan and MRI were performed in all patients before re-operation. There were 12 patients with compression of the spinal cord or nerve root caused by degenerative changes in adjacent segments of fusion segments, 4 cases in upper segments, and 8 cases in lower segments; 3 patients with compression of the spinal cord or nerve root caused by vertebral posterior osteophyte of decompressed segments; 1 patient with compression of the spinal cord caused by incomplete anterior decompression. JOA, NDI and Odom classification were used to assess the clinical effects.

RESULTSAll anterior revision surgery were successful with a mean time of 110 min (80 to 150 min) and mean bleeding of 160 ml (30 to 200 ml). There was 30 ml clear drainage fluid in 1 patient suspected of cerebrospinal fluid leakage. But the 2nd day after operation, the tube was removed and the drainage opening was sutured, and the suture incision healed in grade A after 10 days. Other patients had no complications such as dysdipsia, hoarseness, and laryngeal edema, etc. All patients were followed up for 12 to 28 months with an average of 16 months. Two months after operation and at last follow-up, JOA scores and ODI index had obviously improved than preoperation (P < 0.01), and there was significant difference between postoperative 2 months and last follow-up (P < 0.01). At the final follow-up, improvement rate of JOA was (72.9 +/- 0.2)%. According to the standard of Odom, 12 cases got excellent results, 3 good, 1 fair.

CONCLUSIONAfter surgery of cervical decompression and bone graft fusion with titanium mesh, the patients need re-operation because of incomplete decompression, degenerative changes in adjacent segments or newly formed compression factors, and complications caused by implants. Anterior revision surgery can obtain good clinical effects.

Aged ; Cervical Vertebrae ; surgery ; Decompression, Surgical ; methods ; Female ; Humans ; Male ; Middle Aged ; Reoperation ; Spinal Fusion ; methods ; Spondylosis ; surgery ; Surgical Mesh ; Titanium

Objective To analyze the clinical characteristics of Neonatal Bartter syndrome in order to enhance understanding of the disease.Methods Eighteen children with Neonatal Bartter syndrome who were admitted into our hospital from November 2006 to July 2013 were selected as research subjects.A ret-rospective study was done on the clinical data and the outcome of treatment.Results ⑴Clinical character-istics This group included 13 males and 5 females.The onset age ranged from birth to 1 years 3 months (4.01 ±4.49years).Six cases got the disease after birth.Amniotic fluid which lead to premature birth and low birth weight may happened in cases.The most common clinical symptom was malnutrition (89%).⑵Laboratory tests and Renal ultrasound All of the children showed hypokalemia and metabolic alkalosis in some degree,renin and angiotensin increased.In some cases urinary calcium /creatinine ratio were in-creased and urinary specific gravity,showed a low proportion of urine.Some cases'renal ultrasound exami-nation revealed nephrocalcinosis.⑶ Therapy:All of the patients in this group were given intravenous and /or oral potassium chloride treatment.For drug treatment,10 cases were given single application of indom-ethacin [1 ~3mg/(kg·d),points 3 times per day oral]treatment.Conclusions The incidence of Neo-natal Bartter syndrome was early in infant onset as early as after birth,even the fetal period.Some patients may appear premature birth and /or low birth weigh because of amniotic fluid.The main clinical manifesta-tions include malnutrition,no weight increasing,retardation,serious dehydration and even life-threatening. The characteristic appearance such as the forehead,small jaw,eyes are the important characteristics of neo-natal Bartter syndrome.Patients often appears Hypokalemia,hyponatremia and Hypochloremia.Urinary po-tassium of this type increased.Most children's urinary sodium and chloride increased significantly.

Objective To screen the mutation of KATP channel mutations in Chinese pedigrees with infantile onset type 1 diabetes mellitus (T1DM) and neonatal diabetes mellitus.Methods A cohort of 12 children of infant onset T1DM and neonatal diabetes mellitus admitted into Beijing Children's Hospital between March 2004 and June 2013 were selected.PCR amplification and direct sequencing were used to analyze the 39 exons of ABCC8 gene and one exon of KCNJ11.And the mutational sites of the parents of the probands was sequenced in order to identify the inheritance.Results Analysis revealed ABCC8 mutation in 25％ (3/12 cases) of the patients,a case of transient neonatal diabetes (TNDM),a case of permanent neonatal diabetes mellitus (PNDM) and a case of infant onset T1DM.All positive patients showed a known heterozygosis mutation in the ABCC8 gene(R1182Q,c.3545G ＞ A,D209E,c.627C ＞ G,E208K c.622G ＞ A).The residue R1182Q,which was located at a position involved in joining transmembrane domain 2 to nucleotide binding domain 2,the mutations E208K and D209E were located in the intracellular region that links the transmembrane domain with the gatekeeper module.All the three mutations were located throughout the cytoplasm part of SUR1 protein.The TNDM successfully transferred from insulin to oral sulfonylureas therapy.Conclusions There is a complex genetic pathogenesis in neonatal and infant-onset diabetes.The KATP channel activating mutations is one of the main causes of neonatal diabetes mellitus and may cause T1DM in infants in China.Oral Glibenclamide therapy seems highly effective for some patients with the KATP channel activating mutations.