Objective To assess the safety, effectiveness and predictive factors of endoscopic balloon dilatation for the treatment of esophageal stricture and esophageal achalasia in children. Methods 28 patients with esophageal stricture and esophageal achalasia treated by endoscopic balloon dilatation from January 2012 to November 2014 were included. All the patients were divided into two groups, 22 in group A (esophageal stricture) and 6 in group B (esophageal achalasia). All procedures were performed under tracheal intubation and intravenous anesthesia using the 3rd grade controlled radial expansion (CRE) balloon with gastroscope. Outcomes, including success, complications and recurrence data were recorded, and predictors for outcomes were analyzed. Results A total of EBD 57 sessions (1 to 5 per patient, 2.00 ± 1.15) were performed on 28 patients in this study. 22 patients were diagnosed with esophageal stricture (78.57%) and 6 with esophageal achalasia (21.43%). The median age was 25 months (range 0 ~ 150), and female/ male ratio was 12/16. EBD was successful in all the 28 cases. The total success rate was 100.00%. Complications occurred in 6 patients during the dilatation, and no complication in 22 patients. Completely remission of symptoms was seen in 82.14% cases (n = 23), relief in 14.28% (n = 4), non-response in 3.57% (n = 1), and recurrence in 3.57% (n = 1). The stricture diameter before EBD was (6.28 ± 1.77) mm (range 3.0 ~ 10.0 mm), and it was (10.85 ± 2.51) (range 6 ~ 15 mm) after the last EBD. The difference was significant (P < 0.01). There was no significant difference in success, effectiveness, complications and recurrence among the two groups (P > 0.05). The effectiveness of EBD was significantly associated with the diameter and number of strictures (P < 0.05), more complications were seen in the patients with multiple and/or smaller strictures (P < 0.05). In group A, the longer interval between surgery and the first EBD was related to more dilatation in the patients with anastomotic esophageal strictures (P < 0.05). The age and the interval between symptom onset and the first EBD were not the predicting factors for treatment in group B (P > 0.05). Conclusions The results of this study indicated that EBD under general anesthesia was an effective primary treatment in children with esophageal stricture and esophageal achalasia. The diameter and number of stricture were the most important predictive factors for successful clinical outcomes, while the interval between surgery and the first EBD was the most risk factor for EBD sessions in the patients with anastomotic esophageal strictures.

ObjectiveTo explore the clinical characteristics, treatment and prognosis of severe liver damage in children.MethodsClinical data of 55 children with severe liver damage were retrospectively analyzed.Results In 55 children (31 boys and 24 girls) aged from 28 days to 12 years, forty-five children had acute liver injury mainly caused by infectious diseases (21 cases, 53.3%), blood tumor diseases (5 cases, 11.1%), hereditary metabolic diseases (4 cases, 8.9%), and unexplained diseases (10 cases, 22.2%), ten children had chronic liver injury with decompensated cir-rhosis. Most of severe liver damage in children was caused by antipyretic drugs, traditional Chinese medicine and cold medicine, including 31 cases of acute liver injury and 4 cases of chronic liver injury. In children with acute liver injury, clinical symptoms included gastrointestinal symptoms (32 cases, 71.1%), jaundice (26 cases, 57.8%), hemorrhage (9 cases, 20.0%), multiple organ dysfunction (13 cases, 28.9%) and hepatic encephalopathy (6 cases, 13.3%). In children with chronic liver damage, clinical symptoms included abdominal distension and ascites (10 cases), jaundice (9 cases), gastrointestinal bleeding (7 cases), hepatic encephalopathy (3 cases) and multiple organ dysfunction (1 case). In 55 chil-dren, 39 children were died and the total mortality was 70.91%. In 14 cases of multiple organs dysfunction syndromes, 13 cases (92.9%) were died. All three cases of hepatic encephalopathy were died.ConclusionsInfectious diseases are the leading cause of sever liver damage in children. The most common inciting factors are antipyretic drugs, traditional Chinese medicine and cold medicine. Children with severe liver damage have a high mortality. Rational use of medicine and the concept of the prevention first should been strengthened.

Objective In China primary hyperparathyroidism is not a kind of common disease as in the wesyrn countries.This article reports the current status in the diagnosis and treatment of primary hyperparathyroidism in the mainland of China. Methods We collected 730 cages of primary hyperparathyroidism diagnosed and treated in 7 top hospitals for endocrine surgery from 1965 to 2005.Results In this study.652(89.3%)cases were clinically symptomatic while 78(10.7%)cases were asymptomatic:442 cases were positive on 99mTc-MIBI scanning.Bilateral explorations were undertaken in 377 patients and unilateral or uni-gland exploration through the conventional incision in 204 cases.Minimally invasive parathyroidectomy in 143 cases.Endoscopically assisted 2 cm incision was taken in 6 cases for unilateral gland exploration.Pathologically 632(86.6%)cases were identified as adenoma,58(8.3%)cases were of hyperplasia and 40(5.5%)cases were of carcinoma.There were no major postoperative complications.While 20 patients suffering from recurrence or persistent postoperative hyperparathyroidism,the others are of normal or depressed serum level of calcium. Conclusions Preoperative localization is very helpful: Unilateral exploration for parathyroid adenoma is feasible; minimally invasive parathyroidectomy throush minimal incision is a kind of improving procedure for the localized parathyroid adenoma.

Objective To explore fecal bacteria transplantation for the treatment of severe gastrointestinal disease caused by food allergy. Method The therapeutic process of fecal bacteria transplantation for treatment of severe food allergy gastrointestinal disease was retrospectively analyzed, and the related literature was reviewed. Results A 2-year-old boy had onset of intestinal infection and diarrhea was persistent even though he had received adequate anti-infection therapy and supportive treatment. Finally, the patient received the treatment of fecal bacteria transplantation and the symptoms were then improved. No adverse reactions were observed in 2 months of follow-up. In foreign literature, fecal bacteria transplantation in children is mainly applied to clostridium difficile infection (CDI) and inflammatory bowel disease (IBD), with efficiency of 90%- 100% and 55.6% - 100%, respectively. While in the domestic literature, fecal bacteria transplantation in children is mainly used in CDI and antibiotic associated diarrhea, and the effective rate is 100%. No serious adverse reactions were found in all the researches. Conclusion Fecal transplantation is safe and effective in the treatment of children with severe gastrointestinal disease caused by food allergy, but its application in children is not yet mature and needs more in-depth researches.

Objective To learn about the etiology , clinical characteristics and prognosis of infants with cholestasis jaundice. Methods The clinical data of 175 cholestatic patients were retrospectively analyzed , then the prognosis was followed-up with telephone. Results After analyzing the etiology , we found that among 175 patients , there were 42 with biliary atresia , of which 19 infants died , 4 recovered well after liver transplanta-tion , 8 had liver cirrhosis waiting for transplantation , 5 recovered well after Kasai Portoenterostomy and 6 lost contact. There were 2 patients with Bile duct dysplasia and 2 with congenital cholangiectasis and they had posi-tive outcomes. And 29 patients with Cytomegalovirus infection also had positive outcome. There were 16 patients with Heredity metabolic diseases , among which 13 patients were with Citrin protein deficiency; 10 had positive outcomes; 2 lost contact and 1 died. There were 3 patients with tyrosinemia , of which one had positive outcome;one lost contact and another got liver cirohosis waiting for liver transplantation. Four patients with TPN-related cholestasis all had positive outcomes. There were still 80 cases with unkown etiology , but 79 had positive out-comes and 1 case lost. The clinical characteristics showed that the infants with cholestatic jaundice often accom-panied by stool color changed , liver and spleen enlargement and so on , and often complicated with pneumonia , hypoalbuminemia and coagulation dysfunction and so on. Conclusion There are many etiologies for infants with cholestatic jaundice. Early diagnosis and early treatment would benefit the prognosis.

Objective To explore the treatment of acquired benign tracheoesophageal fistula.Methods Clinical data of 13 cases of acquired benign tracheoesophageal fistula were retrospectively studied and the related literature was reviewed.The physical conditions,locations of fistula and risk factors of the patients were assessed before surgery.A specific surgical protocol was determined for each individual patient using either radical surgery or palliative surgery to repair the fistula.Ten patients underwent radical surgery,including suture closure of the esophageal or tracheal defects only in 7 patients and segmental tracheal reseetion in 3 patients.The remaining 3 patients underwent palliative operation.Results No major complication was observed except pulmonary infectin in 2 patients.No patient died during the perioperative period.All but one patient who treated with mediastinal and neck radiation therapy 4 years ago were able to resume oral food three months after operation.All patients were followed up for 8 months to 73 months[mean (39.6 ± 19.7) months]and no fistulas were occurred in the patients who received a radical surgery.The tracheoesophageal fistula orifices became smaller or closed fairly well in the patients who underwent a palliative surgery.Conclusion Surgery is the treatment of choice for acquired benign tracheoesophageal fistula.Airway and esophagal stent placement is not recommended.Adequate drainage of gastric juice is a crucial step in the management of the condition.

OBJECTIVETo explore the etiology and clinical characteristics of hypoxic hepatitis (HH) in children.

METHODClinical data of 7 patients with HH in Shenzhen Children's Hospital from January 2011 to March 2014 were retrospectively reviewed.

RESULTSeven cases diagnosed as HH, age from 4 months to 11 years, were admitted to pediatric intensive care unit (PICU), and accounted for 0.32% of patients in PICU during the same period. The primary causes of HH were respiratory failure and cardiac shock caused by severe hand-foot-and-mouth disease, fulminant myocarditis, infant muggy syndrome . Serologic tests for hepatitis B virus, hepatitis C virus, as well as serum antibody and DNA for Epstein-Barr virus and cytomegalovirus were all negative. There was an increase of alanine aminotransferase (ALT) (≥20 time supper limit of normal (ULN), the highest ALT was more than 130 times ULN in all the patients, which was decreased to 2 times ULN from peak within 10 days. There was a significant relationship between ALT and aspartate aminotransferase(AST)in 3 cases(r=1.000, 1.000, and 0.833, respectively, P<0.05), ALT and lactate dehydrogenase (LDH)in 2 cases(r=1.000 and 0.886, respectively, P<0.05), ALT and blood urea nitrogen(BUN)in 1 case(r=1.000, P<0.05), and ALT and creatine kinase(CK)in 1 case(r=0.964, P<0.05). The ALT, AST and LDH returned to normal soon after the primary diseases were controlled.

CONCLUSIONSevere heart failure, hypoxemia, shock, etc. are the leading primary diseases causing HH. The sharp increase in ALT, AST and LDH is the typical laboratory manifestion in HH after the onset, which may decline to normal shortly after the treatment, sometimes complicated with reversible change in BUN or CK.

Alanine Transaminase ; Animals ; Aspartate Aminotransferases ; Child ; Child, Preschool ; Creatine Kinase ; Heart Failure ; Hepatitis ; Herpesvirus 4, Human ; Humans ; Hypoxia ; Infant ; L-Lactate Dehydrogenase ; Respiratory Insufficiency ; Retrospective Studies

OBJECTIVETo summarize the clinical features, biochemical change and genetic mutations of a neonate with congenital bile acid synthesis disorder type 2.

METHODSClinical features, blood biochemical index, gene analysis and treatment of the patient were reviewed.

RESULTSThe patient presented with the symptoms of jaundice 3 days after birth but without skin itching. Pale stool was noted. Subsequently, he presented with hepatomegaly, blood coagulation disorders, left cochlear nerve damage, liver cirrhosis and remarkable growth retardation. Serum biochemistries showed that bilirubin and transaminase were elevated, while γ -GT and total bile acid was normal. Abdominal ultrasonography indicated decline of gallbladder contraction. Cholangiography showed normal extra- and intrahepatic bile ducts and patent biliary tract. Liver biopsy showed intrahepatic cholestasis. Gene testing has identified a homozygous mutation in AKR1D1 gene.

CONCLUSIONCongenital bile acid synthesis disorder should be suspected when a neonate has presented with jaundice, elevated bilirubin and transaminase, normal or reduced TBA and γ -GT. Genetic testing and urine mass spectrometry analysis can diagnose congenital bile acid synthesis disorder. Early therapy is crucial to patients with congenital bile acid synthesis disorder.

Objective To compare the differences of hospital stays,hospitalization costs and effectiveness via keyhole hematoma debridement (KHED) and internal medicine conservative treatment (IMCT) in treating 30-40 mL hypertensive intracerebral hemorrhage.Methods Fifty-eight patients with hypertensive intracerebral hemorrhage whose bleeding was 30-40 mL,admitted to our hospital from January 2014 to September 2015,were chosen in our study;according to the will of the patients and their family members,the patients were divided into KHED group (n=31) and IMCT group (n=27).The differences of hospital stays,hospitalization costs,neurological dysfunction rate at hospital and three months after discharge,and recovery results were compared between the two groups.Results The average hospital stays of KHED group were (7.4±2.3) d and those of IMCT group were (14.5±5.1) d,with significant difference (P=0.012);the hospitalization costs for the two groups were (36 296.28±5292.12)yuan,and (41 769.48±6342.83) yuan,with significant difference (P=0.027).Glasgow outcome scale of KHED group at discharge indicated 29 patients with good recovery and 2 with poor recovery;that of IMCT group indicated 20 with good recovery and 7 with poor recovery,including two with cerebral edema accepted craniotomy operation in second time.Follow-up for three months showed that the KHED group had basic activities of daily living in 16 patients,mild hemiplegia in 11 and severe hemiplegia in 4,and IMCT group had basic activities of daily living in 9 patietns,mild hemiplegia in 13 and severe hemiplegia in 5;significant differences were noted between the two groups (Z=2.499,P=0.001).Conclusion KHED in treatment of 30-40 mL hypertensive intracerebral hemorrhage can shorten hospitalization time,reduce cost,have better prognosis and better short-term and long-term effectiveness than IMCT.

The physiological functions of endogenous amyloid-β (Aβ), which plays important role in the pathology of Alzheimer's disease (AD), have not been paid enough attention. Here, we review the multiple physiological effects of Aβ, particularly in regulating synaptic transmission, and the possible mechanisms, in order to decipher the real characters of Aβ under both physiological and pathological conditions. Some worthy studies have shown that the deprivation of endogenous Aβ gives rise to synaptic dysfunction and cognitive deficiency, while the moderate elevation of this peptide enhances long term potentiation and leads to neuronal hyperexcitability. In this review, we provide a new view for understanding the role of Aβ in AD pathophysiology from the perspective of physiological meaning.
Humans ; Alzheimer Disease/pathology* ; Amyloid beta-Peptides/metabolism* ; Long-Term Potentiation ; Synaptic Transmission/physiology* ; Hippocampus