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The evaluation of pancreatic function includes the evaluation of endocrine function and exocrine function.Insufficiency of pancreatic endocrine function can lead to pancreatic related diabetes.The pancreatic endocrine function is often evaluated by fasting blood glucose, two hours postprandial blood glucose, and glycosylated hemoglobin.Pancreatic exocrine dysfunction can lead to abdominal pain, abdominal distension, fatty diarrhea, malnutrition, weight loss and other manifestations.Pancreatic exocrine dysfunction is often atypical, and it is difficult to accurately evaluate pancreatic exocrine function, so the evaluation of pancreatic exocrine function is particularly important.There are indirect and direct methods to evaluate pancreatic exocrine function.At present, the most commonly used direct detection method is the detection of pancreatic function under intravenous anesthesia endoscope.Fecal elastase-1 is a commonly used indirect detection method at present.
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<p><b>OBJECTIVE</b>To summarize the clinical features, biochemical change and genetic mutations of a neonate with congenital bile acid synthesis disorder type 2.</p><p><b>METHODS</b>Clinical features, blood biochemical index, gene analysis and treatment of the patient were reviewed.</p><p><b>RESULTS</b>The 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.</p><p><b>CONCLUSION</b>Congenital 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.</p>
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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.
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Objective To investigate the effect of artifictal circadian rhythm of melatonin on the postoperative cellular immune function in patients undergoing gynecological operation Methods Eighteen ASA Ⅰ or Ⅱ patients,aged 25-50 yr,weighing 45-80 kg,scheduled for elective gynecological operation,were randomly divided into 3 groups (n =6 each):control group (group C),placebo control group (group P) and melatonin group (group M).In group M.melatonin 6 mg was given orally at 10 min before lights-out (21:00) on 1 day before operation,on the day of operation and on 1 day after operation,while placebo was given orally instead of melatonin in group P.The operation was performed under epidural anesthesia.Patient-controlled epidural analgesia with ropivacaine was used for postoperative analgesia.VAS score was maintained ＜ 5.Blood samples were collected from the peripheral vein at 1 day before operation (baseline),the end of operation and 1 day after operation to measure CD4+,CD8+ and CD3+ cell count by flow cytometry.The ratio of the number of CD4+ cells to the number of CD8+ cells was calculated.Results There were no significant differences in the number of CD4+,CD8+ and CD3 + cells and ratio of the number of CD4 + cells to the number of CD8 + cells between groups C,P and M (P ＞0.05).Conclusion Artificial circadian rhythm of melatonin exerts no influence on the postoperative cellular immune function in patients undergoing gynecological operation.
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This study examined endogenous cannabinoid (ECB)-anandamide (AEA) and its cannabinoid receptors (CBR) in mice liver with the development of schistosoma japonicum. Mice were infected with schistosoma by means of pasting the cercaria onto their abdomens. Liver fibrosis was pathologically confirmed nine weeks after the infection. High performance liquid chromatography (HPLC) was employed to determine the concentration of AEA in the plasma of mice. Immunofluorescence was used to detect the expression of CBR1 and CBR2 in liver tissue. Morphological examination showed typical pathological changes, with worm tubercles of schistosoma deposited in the liver tissue, fibrosis around the worm tubercles and infiltration or soakage of inflammatory cells. Also, CBR1 and CBR2 were present in hepatocytes and hepatic sinusoids of the two groups, but they were obviously enhanced in the schistosoma-infected mice. However, the average optical density of CBR1 in the negative control and fibrosis group was 13.28+/-7.32 and 30.55+/-7.78, and CBR2 were 28.13+/-6.42 and 52.29+/-4.24 (P<0.05). The levels of AEA in the fibrosis group were significantly increased as compared with those of the control group. The concentrations of AEA were (0.37+/-0.07) and (5.67+/-1.34) ng/mL (P<0.05). It is concluded that the expression of endocannabinoids AEA and its cannabinoid receptor CBR were significantly increased in schistosoma-infected mice. Endogenous endocannabinoids may be involved in the development of schistosoma-induced liver fibrosis.