Idiopathic pulmonary fibrosis(IPF) is a chronic progressive fibrotic interstitial pneumonia with unknown causes. The incidence rate increases year by year and the prognosis is poor without cure.Recently, phosphatidyli...Idiopathic pulmonary fibrosis(IPF) is a chronic progressive fibrotic interstitial pneumonia with unknown causes. The incidence rate increases year by year and the prognosis is poor without cure.Recently, phosphatidylinositol 3-kinase(PI3 K)/protein kinase B(PKB/AKT) signaling pathway can be considered as a master regulator for IPF. The contribution of the PI3 K/AKT in fibrotic processes is increasingly prominent, with PI3 K/AKT inhibitors currently under clinical evaluation in IPF. Therefore,PI3 K/AKT represents a critical signaling node during fibrogenesis with potential implications for the development of novel anti-fibrotic strategies. This review epitomizes the progress that is being made in understanding the complex interpretation of the cause of IPF, and demonstrates that PI3 K/AKT can directly participate to the greatest extent in the formation of IPF or cooperate with other pathways to promote the development of fibrosis. We further summarize promising PI3 K/AKT inhibitors with IPF treatment benefits, including inhibitors in clinical trials and pre-clinical studies and natural products, and discuss how these inhibitors mitigate fibrotic progression to explore possible potential agents, which will help to develop effective treatment strategies for IPF in the near future.展开更多
Idiopathic granulomatous mastitis is a rare chronic inflammatory lesion of the breast that can clinically and radiographically mimic breast carcinoma. The most common clinical presentation is an unilateral, discrete b...Idiopathic granulomatous mastitis is a rare chronic inflammatory lesion of the breast that can clinically and radiographically mimic breast carcinoma. The most common clinical presentation is an unilateral, discrete breast mass, nipple retraction and even a sinus formation often associated with an inflammation of the overlying skin. The etiology of idiopathic granulomatous mastitis is still obscure. Its treatment remains controversial. The cause may be the autoimmune process, infection, a chemical reaction associated with oral contraceptive pills, or even lactation. Various factors, including hormonal imbalance, autoimmunity, unknown microbiological agents, smoking and α 1-antitrypsin deficiency have been suggested to play a role in disease aetiology. In this review, causing factors in the aetiology of idiopathic granulomatous mastitis are reviewed in detail.展开更多
AIM: To review the literature on idiopathic sclerosing encapsulating peritonitis(SEP), also known as abdominal cocoon syndrome. METHODS: The Pub Med, MEDLINE, Google Scholar, and Google databases were searched using s...AIM: To review the literature on idiopathic sclerosing encapsulating peritonitis(SEP), also known as abdominal cocoon syndrome. METHODS: The Pub Med, MEDLINE, Google Scholar, and Google databases were searched using specific key words to identify articles related to idiopathic SEP. These key words were "sclerosing encapsulating peritonitis," "idiopathic sclerosing encapsulating peritonitis," "abdominal cocoon," and "abdominal cocoon syndrome." The search included letters tothe editor, case reports, review articles, original articles, and meeting presentations published in the English-language literature from January 2000 to May 2014. Articles or abstracts containing adequate information about age, sex, symptom duration, initial diagnosis, radiological tools, and surgical approaches were included in the study. Papers with missing or inadequate data were excluded. RESULTS: The literature search yielded 73 articles on idiopathic(primary) SEP published in 23 countries. The four countries that published the greatest number of articles were India(n = 21), Turkey(n = 14), China(n = 8) and Nigeria(n = 3). The four countries that reported the greatest number of cases were China(n = 104; 53.88%), India(n = 35; 18.13%), Turkey(n = 17; 8.80%) and Nigeria(n = 5; 2.59%). The present study included 193 patients. Data on age could be obtained for 184 patients(range: 7-87 years; mean ± SD, 34.7 ± 19.2 years), but were unavailable for nine patients. Of the 184 patients, 122 were male and 62 were female; sex data could not be accessed in the remaining nine patients. Of the 149 patients whose preoperative diagnosis information could be obtained, 65(43.6%) underwent operations for abdominal cocoon, while the majority of the remaining patients underwent operations for a presumed diagnosis of intestinal obstruction and/or abdominal mass. Management information could be retrieved for 115 patients. Of these, 68 underwent excision + adhesiolysis(one laparoscopic); 24 underwent prophylactic appendectomy in addition to excision + adhesiol展开更多
Although there are numerous causes of acute panc-reatitis, an etiology cannot always be found. Two potential etiologies, microlithiasis and sphincter of Oddi dysfunction, are discussed in this review. Gallbladder micr...Although there are numerous causes of acute panc-reatitis, an etiology cannot always be found. Two potential etiologies, microlithiasis and sphincter of Oddi dysfunction, are discussed in this review. Gallbladder microlithiasis, missed on transcutaneous ultrasound, is reported as the cause of idiopathic acute pancreatitis in a wide frequency range of 6%-80%. The best diagnostic technique for gallbladder microlithiasis is endoscopic ultrasound although biliary crystal analysis and empiric cholecystectomy remain as reasonable options. In contrast, in patients who are post-cholecystectomy, bile duct microlithiasis does not appear to have a role in the pathogenesis of acute pancreatitis. Sphincter of Oddi dysfunction is present in 30%-65% of patients with idiopathic acute recurrent pancreatitis in whom other diagnoses have been excluded. It is unclear if this sphincter dysfunction was the original etiology of the first episode of pancreatitis although it appears to have a causative role in recurring episodes since sphincter ablation decreases the frequency of recurrent attacks. Unfortunately, this conclusion is primarily based on small retrospective case series; larger prospective studies of the outcome of pancreatic sphincterotomy for SOD-associated acute pancreatitis are sorely needed. Another problem with this diagnosis and its treatment is the concern over potential procedure related complications from endoscopic retrograde cholangiopancreatography (ERCP), manometry and pancreatic sphincterotomy. For these reasons, patients should have recurrent acute pancreatitis, not a single episode, and have a careful informed consent before assessment of the sphincter of Oddi is undertaken.展开更多
Sclerosing encapsulating peritonitis (SEP) is a rare cause of intestinal obstruction that is characterized by a thick grayish-white fibrotic membrane encasing the small bowel. SEP can be classified as idiopathic,also ...Sclerosing encapsulating peritonitis (SEP) is a rare cause of intestinal obstruction that is characterized by a thick grayish-white fibrotic membrane encasing the small bowel. SEP can be classified as idiopathic,also known as abdominal cocoon,or secondary. It is difficult to make a definite pre-operative diagnosis. We experienced five cases of abdominal cocoon,and the case files were reviewed retrospectively for the clinical presentation,operative findings and outcome. All the patients presented with acute,subacute and chronic intestinal obstruction. Computed tomography (CT) showed characteristic findings of small bowel loops congregated to the center of the abdomen encased by a soft-tissue density mantle in four cases. Four cases had an uneventful post-operative period,one case received second adhesiolysis due to persistent ileus. The imaging techniques may facilitate pre-operative diagnosis. Surgery is important in the management of SEP.展开更多
Long-term gonadotropin-releasing hormone agonist（Gn RHa） administration before in vitro fertilization（IVF）/intracytoplasmic sperm injection（ICSI） in infertile women with endometriosis or adenomyosis significantl...Long-term gonadotropin-releasing hormone agonist（Gn RHa） administration before in vitro fertilization（IVF）/intracytoplasmic sperm injection（ICSI） in infertile women with endometriosis or adenomyosis significantly enhanced the chances of pregnancy in both fresh and frozen embryo transfer cycles. We hypothesized that long-term Gn RHa treatment might also be beneficial for the idiopathic repeated implantation failure（RIF） patients. In the 21 patients receiving Gn RHa and hormone replacement therapy（G-HRT） protocols for frozen embryo transfer, their data were compared with those of the 56 of frozen/fresh cycles they had previously undergone（previous protocols）. Comparison showed that the finial results were significantly better with G-HRT protocols than with their previous protocols, with pregnancy rate, clinical pregnancy rate, implantation rate and on-going pregnancy rate being 70%, 60%, 40% and 38% respectively with G-HRT protocols, against 17%, 11%, 6.3% and 5% with previous protocols. The results showed that hormonally controlled endometrial preparation with prior Gn RHa suppression could be used for patients who had experienced repeated failures of IVF treatment despite having morphologically optimal embryos, and the treatment may help increase the receptivity of the endometrium in these patients.展开更多
Background Verapamil-sensitive, idiopathic left ventricular tachycardia （ILVT） with right bundle branch block configuration and left-axis deviation is known to be due to re-entry mechanism but the exact nature of re...Background Verapamil-sensitive, idiopathic left ventricular tachycardia （ILVT） with right bundle branch block configuration and left-axis deviation is known to be due to re-entry mechanism but the exact nature of reentrant circuit in ILVT is not fully elucidated. Radiofrequency （RF） ablation was applied during ventricular tachycardia （VT） and termination of the VT or abolishing the inducibility of the tachycardia was used as an endpoint for successful RF. In this study, the left posterior fascicular block in surface electrocardiogram （ECG） was used as a new endpoint of ablation to cure ILVT. Methods Electrophysiological studies and radiofrequency ablation were performed in 39 consecutive patients [30 men, 9 women; age ranging from 10 to 64 years, mean （29± 16） years] with verapamil-sensitive ILVT and structurally normal hearts. VT could be terminated by the intravenous adininistration of verapamil in all patients. The target site was the midseptum of LV where the earliest Purkinje potentials were recorded during VT. RF current was applied to the target site with or without late diastolic potential （LDP） during sinus rhythm in 37 patients and during VT in 2 patients to meet the ablation endpoint： the left posterior fascicuiar block in the surface ECG. Results Thirty-seven patients with ILVT had been treated by RF ablation during sinus rhythm and two had been treated during VT. All of them met the endpoint of the left posterior fascicuiar block. Thirty-eight cases were symptom-free without medications during the follow-up period （range from 3 to 95 months, median 17 months）. One patient developed a clinical recurrence and the left posterior fascicuiar block in surface ECG disappeared. The patient received another treatment. The endpoint was met and the procedure was successful. Conclusions The left posterior fascicuiar block in surface ECG used as an endpoint of RF ablation to treat ILVT is effective. It is important especially in those patients whose VT can not be induced or the indu展开更多
Granulomatous lobular mastitis(GLM) is a rare and chronic benign inflammatory disease of the breast. Difficulties exist in the management of GLM for many front-line surgeons and medical specialists who care for patien...Granulomatous lobular mastitis(GLM) is a rare and chronic benign inflammatory disease of the breast. Difficulties exist in the management of GLM for many front-line surgeons and medical specialists who care for patients with inflammatory disorders of the breast. This consensus is summarized to establish evidence-based recommendations for the management of GLM. Literature was reviewed using PubMed from January 1, 1971 to July 31, 2020. Sixty-six international experienced multidisciplinary experts from 11 countries or regions were invited to review the evidence.Levels of evidence were determined using the American College of Physicians grading system, and recommendations were discussed until consensus. Experts discussed and concluded 30 recommendations on historical definitions,etiology and predisposing factors, diagnosis criteria, treatment, clinical stages, relapse and recurrence of GLM. GLM was recommended as a widely accepted definition. In addition, this consensus introduced a new clinical stages and management algorithm for GLM to provide individual treatment strategies. In conclusion, diagnosis of GLM depends on a combination of history, clinical manifestations, imaging examinations, laboratory examinations and pathology.The approach to treatment of GLM should be applied according to the different clinical stage of GLM. This evidencebased consensus would be valuable to assist front-line surgeons and medical specialists in the optimal management of GLM.展开更多
基金supported by the National Natural Science Foundation of China(No.82003873)the Postdoctoral Science Foundation of China(No.2020M681899)the Zhejiang Provincial Natural Science Foundation of China(No.LR21H310001)。
文摘Idiopathic pulmonary fibrosis(IPF) is a chronic progressive fibrotic interstitial pneumonia with unknown causes. The incidence rate increases year by year and the prognosis is poor without cure.Recently, phosphatidylinositol 3-kinase(PI3 K)/protein kinase B(PKB/AKT) signaling pathway can be considered as a master regulator for IPF. The contribution of the PI3 K/AKT in fibrotic processes is increasingly prominent, with PI3 K/AKT inhibitors currently under clinical evaluation in IPF. Therefore,PI3 K/AKT represents a critical signaling node during fibrogenesis with potential implications for the development of novel anti-fibrotic strategies. This review epitomizes the progress that is being made in understanding the complex interpretation of the cause of IPF, and demonstrates that PI3 K/AKT can directly participate to the greatest extent in the formation of IPF or cooperate with other pathways to promote the development of fibrosis. We further summarize promising PI3 K/AKT inhibitors with IPF treatment benefits, including inhibitors in clinical trials and pre-clinical studies and natural products, and discuss how these inhibitors mitigate fibrotic progression to explore possible potential agents, which will help to develop effective treatment strategies for IPF in the near future.
文摘Idiopathic granulomatous mastitis is a rare chronic inflammatory lesion of the breast that can clinically and radiographically mimic breast carcinoma. The most common clinical presentation is an unilateral, discrete breast mass, nipple retraction and even a sinus formation often associated with an inflammation of the overlying skin. The etiology of idiopathic granulomatous mastitis is still obscure. Its treatment remains controversial. The cause may be the autoimmune process, infection, a chemical reaction associated with oral contraceptive pills, or even lactation. Various factors, including hormonal imbalance, autoimmunity, unknown microbiological agents, smoking and α 1-antitrypsin deficiency have been suggested to play a role in disease aetiology. In this review, causing factors in the aetiology of idiopathic granulomatous mastitis are reviewed in detail.
文摘AIM: To review the literature on idiopathic sclerosing encapsulating peritonitis(SEP), also known as abdominal cocoon syndrome. METHODS: The Pub Med, MEDLINE, Google Scholar, and Google databases were searched using specific key words to identify articles related to idiopathic SEP. These key words were "sclerosing encapsulating peritonitis," "idiopathic sclerosing encapsulating peritonitis," "abdominal cocoon," and "abdominal cocoon syndrome." The search included letters tothe editor, case reports, review articles, original articles, and meeting presentations published in the English-language literature from January 2000 to May 2014. Articles or abstracts containing adequate information about age, sex, symptom duration, initial diagnosis, radiological tools, and surgical approaches were included in the study. Papers with missing or inadequate data were excluded. RESULTS: The literature search yielded 73 articles on idiopathic(primary) SEP published in 23 countries. The four countries that published the greatest number of articles were India(n = 21), Turkey(n = 14), China(n = 8) and Nigeria(n = 3). The four countries that reported the greatest number of cases were China(n = 104; 53.88%), India(n = 35; 18.13%), Turkey(n = 17; 8.80%) and Nigeria(n = 5; 2.59%). The present study included 193 patients. Data on age could be obtained for 184 patients(range: 7-87 years; mean ± SD, 34.7 ± 19.2 years), but were unavailable for nine patients. Of the 184 patients, 122 were male and 62 were female; sex data could not be accessed in the remaining nine patients. Of the 149 patients whose preoperative diagnosis information could be obtained, 65(43.6%) underwent operations for abdominal cocoon, while the majority of the remaining patients underwent operations for a presumed diagnosis of intestinal obstruction and/or abdominal mass. Management information could be retrieved for 115 patients. Of these, 68 underwent excision + adhesiolysis(one laparoscopic); 24 underwent prophylactic appendectomy in addition to excision + adhesiol
文摘Although there are numerous causes of acute panc-reatitis, an etiology cannot always be found. Two potential etiologies, microlithiasis and sphincter of Oddi dysfunction, are discussed in this review. Gallbladder microlithiasis, missed on transcutaneous ultrasound, is reported as the cause of idiopathic acute pancreatitis in a wide frequency range of 6%-80%. The best diagnostic technique for gallbladder microlithiasis is endoscopic ultrasound although biliary crystal analysis and empiric cholecystectomy remain as reasonable options. In contrast, in patients who are post-cholecystectomy, bile duct microlithiasis does not appear to have a role in the pathogenesis of acute pancreatitis. Sphincter of Oddi dysfunction is present in 30%-65% of patients with idiopathic acute recurrent pancreatitis in whom other diagnoses have been excluded. It is unclear if this sphincter dysfunction was the original etiology of the first episode of pancreatitis although it appears to have a causative role in recurring episodes since sphincter ablation decreases the frequency of recurrent attacks. Unfortunately, this conclusion is primarily based on small retrospective case series; larger prospective studies of the outcome of pancreatic sphincterotomy for SOD-associated acute pancreatitis are sorely needed. Another problem with this diagnosis and its treatment is the concern over potential procedure related complications from endoscopic retrograde cholangiopancreatography (ERCP), manometry and pancreatic sphincterotomy. For these reasons, patients should have recurrent acute pancreatitis, not a single episode, and have a careful informed consent before assessment of the sphincter of Oddi is undertaken.
文摘Sclerosing encapsulating peritonitis (SEP) is a rare cause of intestinal obstruction that is characterized by a thick grayish-white fibrotic membrane encasing the small bowel. SEP can be classified as idiopathic,also known as abdominal cocoon,or secondary. It is difficult to make a definite pre-operative diagnosis. We experienced five cases of abdominal cocoon,and the case files were reviewed retrospectively for the clinical presentation,operative findings and outcome. All the patients presented with acute,subacute and chronic intestinal obstruction. Computed tomography (CT) showed characteristic findings of small bowel loops congregated to the center of the abdomen encased by a soft-tissue density mantle in four cases. Four cases had an uneventful post-operative period,one case received second adhesiolysis due to persistent ileus. The imaging techniques may facilitate pre-operative diagnosis. Surgery is important in the management of SEP.
基金supported by grants from the National Natural Science Foundation of China(No.81100401 and No.81470063)Guangdong Natural Science Foundation of China(No.2014A030313129)the Doctoral Fund of the Ministry of Education of China(No.20110171120096)
文摘Long-term gonadotropin-releasing hormone agonist（Gn RHa） administration before in vitro fertilization（IVF）/intracytoplasmic sperm injection（ICSI） in infertile women with endometriosis or adenomyosis significantly enhanced the chances of pregnancy in both fresh and frozen embryo transfer cycles. We hypothesized that long-term Gn RHa treatment might also be beneficial for the idiopathic repeated implantation failure（RIF） patients. In the 21 patients receiving Gn RHa and hormone replacement therapy（G-HRT） protocols for frozen embryo transfer, their data were compared with those of the 56 of frozen/fresh cycles they had previously undergone（previous protocols）. Comparison showed that the finial results were significantly better with G-HRT protocols than with their previous protocols, with pregnancy rate, clinical pregnancy rate, implantation rate and on-going pregnancy rate being 70%, 60%, 40% and 38% respectively with G-HRT protocols, against 17%, 11%, 6.3% and 5% with previous protocols. The results showed that hormonally controlled endometrial preparation with prior Gn RHa suppression could be used for patients who had experienced repeated failures of IVF treatment despite having morphologically optimal embryos, and the treatment may help increase the receptivity of the endometrium in these patients.
文摘Background Verapamil-sensitive, idiopathic left ventricular tachycardia （ILVT） with right bundle branch block configuration and left-axis deviation is known to be due to re-entry mechanism but the exact nature of reentrant circuit in ILVT is not fully elucidated. Radiofrequency （RF） ablation was applied during ventricular tachycardia （VT） and termination of the VT or abolishing the inducibility of the tachycardia was used as an endpoint for successful RF. In this study, the left posterior fascicular block in surface electrocardiogram （ECG） was used as a new endpoint of ablation to cure ILVT. Methods Electrophysiological studies and radiofrequency ablation were performed in 39 consecutive patients [30 men, 9 women; age ranging from 10 to 64 years, mean （29± 16） years] with verapamil-sensitive ILVT and structurally normal hearts. VT could be terminated by the intravenous adininistration of verapamil in all patients. The target site was the midseptum of LV where the earliest Purkinje potentials were recorded during VT. RF current was applied to the target site with or without late diastolic potential （LDP） during sinus rhythm in 37 patients and during VT in 2 patients to meet the ablation endpoint： the left posterior fascicuiar block in the surface ECG. Results Thirty-seven patients with ILVT had been treated by RF ablation during sinus rhythm and two had been treated during VT. All of them met the endpoint of the left posterior fascicuiar block. Thirty-eight cases were symptom-free without medications during the follow-up period （range from 3 to 95 months, median 17 months）. One patient developed a clinical recurrence and the left posterior fascicuiar block in surface ECG disappeared. The patient received another treatment. The endpoint was met and the procedure was successful. Conclusions The left posterior fascicuiar block in surface ECG used as an endpoint of RF ablation to treat ILVT is effective. It is important especially in those patients whose VT can not be induced or the indu
基金supported by Improving the Ability of Diagnosis and Treatment of Difficult Diseases (ZLYNXM202009)。
文摘Granulomatous lobular mastitis(GLM) is a rare and chronic benign inflammatory disease of the breast. Difficulties exist in the management of GLM for many front-line surgeons and medical specialists who care for patients with inflammatory disorders of the breast. This consensus is summarized to establish evidence-based recommendations for the management of GLM. Literature was reviewed using PubMed from January 1, 1971 to July 31, 2020. Sixty-six international experienced multidisciplinary experts from 11 countries or regions were invited to review the evidence.Levels of evidence were determined using the American College of Physicians grading system, and recommendations were discussed until consensus. Experts discussed and concluded 30 recommendations on historical definitions,etiology and predisposing factors, diagnosis criteria, treatment, clinical stages, relapse and recurrence of GLM. GLM was recommended as a widely accepted definition. In addition, this consensus introduced a new clinical stages and management algorithm for GLM to provide individual treatment strategies. In conclusion, diagnosis of GLM depends on a combination of history, clinical manifestations, imaging examinations, laboratory examinations and pathology.The approach to treatment of GLM should be applied according to the different clinical stage of GLM. This evidencebased consensus would be valuable to assist front-line surgeons and medical specialists in the optimal management of GLM.
