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Gaucher disease (GD) is an autosomal recessive lysosomal disorder caused by a disturbance in the metabolism of glucocerebroside in the macrophages. Most of its manifestations - hepatosplenomegaly, anemia, thrombocytopenia, and bone pain - are amenable to a macrophage-target therapy such as enzyme replacement. However, there is increasing evidence that abnormalities of the liver persist despite the specific GD treatment. In this work, we adapted histomorphometry techniques to the study of hepatocytes in GD using liver tissue of treated patients, developing the first morphometrical method for canalicular quantification in immunohistochemistry-stained liver biopsies, and exploring histomorphometric characteristics of GD. This is the first histomorphometric technique developed for canalicular analysis on histological liver biopsy samples.
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Humans , Image Cytometry/methods , Gaucher Disease/therapy , Bile Canaliculi , Hepatocytes , Biopsy, Large-Core NeedleABSTRACT
@#AIM: To explore the effect of anastomosis surgery for lacrimal canaliculi laceration combined with circular tube implantation.<p>METHODS: A retrospective study was conducted on 59 cases(59 eyes)from June 2018 to December 2018 with lower lacrimal canalicular laceration. The nasal cut end of lacerated lacrimal canaliculi was found under the microscope, the circular tube was implanted as a support. The tube was removed 3mo after the surgery. All the patients were followed additional 3mo. The therapeutic effect and complications were observed.<p>RESULTS: All patients were successfully operated. 46 cases(46 eyes)were cured, 5 cases(5 eyes)were improved, 8 cases(8 eyes)had no effect. The overall successful rate was 86%(51/59). No serious complications and eyelid deformity were found.<p>CONCLUSION: The anastomosis surgery combined with circular tube implantation is an effective treatment for the lacrimal canaliculi laceration.
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OBJECTIVE@#To assess the value of Ploton silver staining and phalloidin-iFlour 488 staining in observation of the morphology of osteocyte dendrites of mice at different developmental stages.@*METHODS@#The humerus and femurs were harvested from mice at 0 (P0), 5 (P5), 15 (P15), 21 (P21), 28 (P28), and 35 days (P35) after birth to prepare cryo-sections and paraffin sections. HE staining of P35 mouse femur sections served as a reference for observing osteocytes in the trabecular bone and cortical bone. The humeral sections at different developmental stages were stained with Ploton silver staining to observe the morphology of osteocytes and canaliculi, and the canalicular lengths in the cortical and trabecular bones of the humerus of the mice in each developmental stage were recorded. The cryo-sections of the humerus from P10 and P15 mice were stained with phalloidin iFlour-488 to observe the morphology of osteocytes and measurement of the length of osteocyte dendrites in the cortical bone.@*RESULTS@#In the trabecular bone of the humerus of P0-P15 mice, Ploton silver staining only visualized the outline of the osteocytes, and the morphology of the canaliculi was poorly defined. In P21 or older mice, Ploton silver staining revealed the morphology of the trabecular bone osteocytes and the canaliculi, which were neatly arranged and whose lengths increased significantly with age (P21 @*CONCLUSIONS@#Mouse osteocyte dendrites elongate progressively and their arrangement gradually becomes regular with age. Ploton silver staining can clearly visualize the morphology of the osteocytes and the canaliculi in adult mice but not in mice in early stages of development. Phalloidin iFlour-488 staining for labeling the cytoskeleton can be applied for mouse osteocytes at all developmental stages and allows morphological observation of mouse osteocytes in early developmental stages.
Subject(s)
Animals , Mice , Bone and Bones , Dendrites , Osteocytes , Phalloidine , Silver StainingABSTRACT
@#AIM:To explore the clinical outcomes of RS lacrimal tube for lacrimal canaliculi rupture without nasal endoscope.<p>METHODS:A retrospective analysis from September 2017 to March 2018 were taken. According to admission time sequence. Totally 52 patients with lacrimal canaliculi rupture was divided into two groups. One group was treated with RS lacrimal tube, the other was treated with annular silicone tube. The treatment results were comparatively analyzed. <p>RESULTS:There were 15 eyes of nasal mucosa hemorrhage in annular silicone tube group. There was no eye with this in RS tube group, the difference between the two groups was significant(<i>P</i><0.001). In RS tube group, 26 eyes were cured, 3 eyes improved, 3 eyes failed. The effective rate was 91%. In annular silicone tube group, 15 eyes were cured, 3 eyes improved, 2 eyes failed. The effective rate was 90%, the clinical efficacy was not significantly different between groups(<i>P</i>=0.877). There were 2 eyes in RS tube group occurred fall off in 2wk to 2mo after operations; 1 eye of lacrimal puncture were removed from the lacrimal puncture without 2wk, and was anastomosed again. The other eye was removed from the lacrimal puncture within 1-2mo after the operation, the lacrimal canal flushing was improved during follow-up. There were 3 eyes in annular silicone group occurred fall off without 2wk, and were not anastomosed again. The lacrimal duct flushing was improved during follow-up. No statistical significance was found in the two groups(<i>P</i>=0.361). <p>CONCLUSION:Lacrimal canaliculi rupture should be timely given surgical treatment. RS lacrimal tube can not only achieve good operation effect and reduce the probability of complications, but also has the advantages of simple surgical methods, good histocompatibility and no influence on the appearance of patients.
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@#AIM: To explore the usefulness of RS-type lacrimal drainage tube versus double silicone tube for children's lacrimal canaliculi rupture.<p>METHODS: A retrospective analysis from August 2016 and July 2018 were taken. Totally 37 children(37 eyes)of lacrimal canaliculi rupture were enrolled in the study and divided into two groups according to admission time sequence: treatment group(20 eyes)and control group(17 eyes). The treatment group was treated with RS-type lacrimal drainage tube. The control group underwent the implantation of double silicone tube and their efficacy was analyzed.<p>RESULTS: It was significant difference that the epistaxis rate between the treatment group and the control group, which was 0% and 100%. The effective rate was 95% and 88%, respectively, for the treatment group and the control group, the difference between the two groups has no significant(<i>P</i>=0.584). And the postoperative complication rate was 5% in treatment group, 12% in control group respectively, there was no significant statistical difference between the two groups(<i>P</i>=0.584).<p>CONCLUSION: The implantation of the RS-type lacrimal drainage tube can effectively prevent epistaxis, is safe and simple, easy to operate and extubate easily with good clinical effect in the treatment of lacrimal canaliculus fracture in children. So it can be widely carried out in basic hospitals.
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The elevated NADH/ NAD + ratio in the plasma of the hepatocyte is a key pathophysiologic altera-tion for the development of neonatal intrahepatic cholestasis caused by Citrin deficiency (NICCD). The elevated ratio results in energy shortage in hepatocytes and impairs the function of the ATP - dependent canalicular transporters,in-cluding BSEP,MDR3,FIC1,Sterolin 1 / 2 and MRP2,leading to intrahepatic cholestasis in NICCD patients. On the other hand,the increased NADH/ NAD + ratio inhibits galactose metabolism and thus gives rise to secondary galactosemia, damaging the hepatocyte as well as extrahepatic organs including the lens of the eyes. The lactose - free and medium -chain triglyceride - enriched formulas can rapidly correct the energy shortage of the hepatocyte and alleviate secondary galactosemia,hence improving the clinical presentations effectively in NICCD patients.
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AIM: To explore the curative effect of different ways of silicone tube catheter for the treatment of children`s lacrimal canaliculi rupture.METHODS: A retrospective analysis from January 2014 to December 2015 were taken.According to admission time sequence,42 children with lacrimal canaliculi rupture was divided into two groups.One group was treated with single silicone tube insertion,the other was treated with annular silicone tuber insertion.The treatment results were comparatively analyzed.RESULTS: There were 4 eyes in single silicone tube group occurred fall off in 2wk to 1mo after operations because of improper nursing and the second operations were taken.There was no eye with this in annular silicone tuber groups,the difference between the two groups was significant(P<0.05).In single silicone tube group,16 eyes were cured,1 eye improved,3 eyes unchanged which were the 3 eyes with fall off.Those 3 eyes were 1 patient bitten by dog,2 bombed;the effective rate was 85%.In annular silicone tuber insertion,20 eyes were cured,2 eyes improved and the effective rate was 100%,the difference between the two groups was significant (P<0.05).CONCLUSION: Children`s lacrimal canaliculi rupture should be timely given surgical treatment,reasonable surgical treatment can achieve good operation effect.Annular silicone tube has good stability,fewer complications than single silicone tube,and clinical treatment effect is good.
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AIM: To discuss the clinical applications of methods to localize nasal cut ends and the effects of Z-plasty in the surgeries for lower eyelid longitudinal laceration combined with lower lacrimal canaliculi disruption. METHODS: From September, 2010 to October, 2013, a total of 37 patients ( 37 eyes ) with lower eyelid longitudinal laceration combined with lower lacrimal canaliculi disruption were operated for anastomosis of lacrimal canaliculi disruption and suture of lower eyelid longitudinal. Different methods to search for the nasal cut ends of lacerated lacrimal canaliculi, such as “under a microscope directly”, “guided by probing needle” and“pigtail curved probe”. Then, to repair lower eyelid longitudinal laceration with Z-plasty transposition flaps. Follow up was 3mo~2a after operation. RESULTS: All nasal cut ends could be found successfully on 37 patients;Lacrimal duct unobstructed in 31 patients (83. 8%), improved in 5 patients (13. 5%), invalid in 1 patient (2. 7%),the overall successful rate was 97. 3%; the eyelids repair was satisfactory, small scars, the appearance and function was normal. CONCLUSION: The nasal cut ends can be found successfully by “directly under a microscope”, “guided by probing needle” and“pigtail curved probe”;the effect of silicone drainage tube used as lacrimal canaliculi bracket is satisfactory; most patients gained excellent recovery for both appearance and function after Z-plasty.
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PURPOSE: In case of facial injury, a canalicular injury is common. Many methods and materials have been used for reconstruction of lacrimal canaliculi. Silicone tube is the most widely used material, generally with pigtail probe or stainless steel probe. But it has still many problems. The authors guess that reconstruction using Mini Monoka(R) will reduce those problems and will be more successful. METHODS: From July, 2003 to November, 2006 in 24 patients with canalicular injury, 11 cases were reconstructed with former silicone tube and 13 cases were treated with Mini Monoka(R). RESULTS: The mean follow-up period was 3 months. The results of reconstruction were classified into three groups(Normal, Fair, Poor). In 13 cases with Mini Monoka(R) stent, 10 cases were normal, 3 were fair, and no poor cases. But in 11 cases with bicanaliculus silicone stent, 6 cases were normal, 3 were fair, and 2 were poor. CONCLUSION: Compared with bicanaliculus silicone stent, Mini Monoka(R) stent is fixed well, has no aesthetic problems, makes less foreign body sensation. And the results were successful.
Subject(s)
Humans , Facial Injuries , Follow-Up Studies , Foreign Bodies , Sensation , Silicones , Stainless Steel , StentsABSTRACT
Bile canaliculi is closely related to the cytoskeleton; actin filament web, microtubules and cytokeratin intermediate filaments. To understand how cytoskeletal alteration affects bile canalicular structure, the investigators injected cytochalasin B and colchicine into mice intraperitoneally to inhibit the polymerization of actin filaments and microtubules respectively, and observed the structural changes of bile canaliculi and hepatocytes with transmission and scanning electron microscopes. Bile canaliculi were dilatated and microvilli were decreased in number and length after injection of cytochalasin B and colchicine. Some bile canaliculi branched irregularly after colchicine treatment. Actin filament web in the canalicular ectoplasm was disrupted leaving granular zone after cytochalasin B treatment, but was intact after colchicine treatment. Intermediate filament bundles located at angles to the canalicular membrane appeared after colchicine treatment. Intercellular junctions delimiting bile canaliculi were intact after colchicine treatment, however were disrupted after cytochalsin B treatment. Focal junctions resembling desmosome were formed between microvilli after colchicine treatment. In both cytochalasin B and colchicine treated groups, lumen of rough endoplasmic reticulum were dilated, Golgi apparatus became prominent, and lipid droplets were appeared in the cytoplasm. These results suggest that both intact actin filaments and microtubules are necessary to keep the structural integrity of bile canaliculi.