Objective To investigate the effects of hepatic pedicle exclusion and low central venous pressure (LCVP) on blood loss in right lobe tumor resection and evaluate its influence on hepatic and renal function. Methods Forty-eight patients with right lobe tumor admitted from December 2006 to June 2008 were randomly allocated to the LCVP group (23 cases) and routine hepatectomy, (control group 25 cases). During the parenchymal transection phase of surgery, CVP < 5 mm Hg ( 1 mm Hg = 0.133 kPa) and SBP ≥90 mm Hg were maintained in the LCVP group by drugs. However, no special management of CVP and SBP was done in control group. The parenchymal transection blood loss, postoperative hospital stay, postoperative hepatic and renal function changes between two groups were compared, and the incidence of comphcation was also observed. Results There were no significant difference in type of hepatectomy, time of vascular clamping, period of operation, postoperative complication rate, postoperative hepatic and renal functions between two groups. Parenchymal transection blood loss in the LCVP group was significantly lower than that in the control group (326.67 ± 109.13 ) ml vs (538.33 ± 177.07 ) ml, (P < 0.01 ). Postoperative hospital stay in the LCVP group was significantly shorter than that in the control group (8.52 ± 1.78) d vs (9.40±1.68) d, (P < 0.05). Conclusions Hepatic pedicle exclusion and LCVP during hepatectomy is safe. It can reduced blood loss during parenchymal transection and decrease postoperative hospital stay. It is no detrimental effect to hepatic or renal function.

A 37-year-old male patient presented with persistent generalized itching erythema and papules for more than 1 month.The patient had received surgical treatment for type B3 thymoma in stage Ⅳ.Five months prior to the presentation,he developed myasthenia gravis.He also complained of chronic diarrhea for two years.Physical examination revealed white patches in the oral mucosa as well as scaly erythema of varying size on the face,trunk and extremities.Lamellar scales could be seen after scratching,while removal of scales could not result in the appearance of bleeding points.There was scaly hyperkeratotic erythema of palms and soles.Histopathological examination revealed psoriasis-like epidermal hyperplasia and parakeratosis with multiple dyskeratocytes,and some of the dyskeratocytes were surrounded by lymphocytes.There was a perivascular infiltration with a small number of lymphocytes.Immunohistochemical study showed positive staining for CD3,CD4 (dermis),CD8 (epidermis) and CD45RO,but negative staining for CD20,CD68 and CD30.The patient was diagnosed as thymoma-associated graftversus-host-like disease,myasthenia gravis and mucosal candidiasis.After treatment with tacrolimus and prednisone,the condition was gradually relieved.

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.

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 clinical outcomes of arthroscopy-assisted Tightrope technique and clavicular hook plating for the treatment of acromioclavicular joint dislocation of Rockwood Type Ⅲ in aduhs.Methods The clinical data were reviewed of the 60 patients who had been treated for acromioclavicular joint dislocation of Rockwood Type Ⅲ from January 2012 to December 2015 at Department of Orthopedics,Jinhua Municipal Central Hospital.Their age ranged from 34 to 62 years (mean,40 years).Injury was at the left side in 28 cases and at the right in 32 ones.They were randomly assigned to receive treatment either using arthroscopy-assisted Tightrope technique (group A,30 cases) or using clavicular hook plate (group B,30 cases).The 2 groups were compared in terms of incision length,operation time,blood loss,visual analogue scale (VAS) and Constant-Murley shoulder function scores at postoperative 3 and 12 months.Results The 2 groups were compatible because there were no significant between-group differences in gender,age,injury laterality,Rockwood fracture typing or injury cause (P ＞ 0.05).All the patients were followed up for a mean of 10 months (from 6 to 12 months).Primary incision healing was achieved in both groups with no neural lesion.The incision length in group A (3.4 ± 0.6 cm) was significantly shorter than that in group B (8.8 ± 1.8 cm),and the blood loss in the former (40.0 ± 8.2 mL) significantly less than that in the latter (70.9 ± 9.8 mL) (P ＜ 0.05).The VAS scores and Constant-Murley shoulder function scores at postoperative 3 and 12 months in group A (respectively:2.2±1.1 and 1.1 ±0.9 points;85.5±2.1 and 98.0±3.1points) were significantly better than those in group B (respectively:3.2 ± 1.8 and 2.1 ± 1.2 points;75.0 ±4.1 and 85.1 ±2.9 points) (P ＜ 0.05).There were no obvious complications during the follow-up period in either group.Conclusions Although both arthroscopy-assisted Tightrope technique and clavicular hook plating can relieve shoulder pain in the adult patients with acromioclavicular joint dislocation of Rockwood Type Ⅲ,the former shows advantages of a smaller incision,less bleeding,lower VAS scores,and reduced pain and financial cost for the patients due to no trouble of implant removal.