The natural decline and death course of hepatic cystic echinococcsis / 中华消化外科杂志

Hepatic cystic echinococcosis (HCE) is a global public health problem. At present, the research of echinococcosis mainly focuses on etiology, epidemiology, immunology, imaging and treatment programs, while ignoring the systematic study of its natural decline and death course. The popularization of modern imaging examination methods enable researchers to obtain the clinical data of HCE in different stages, with different imaging features and pathological states. Based on CT imaging features and clinical practice, the authors discuss the natural decline and death course of HCE, in order to provide new ideas for its diagnosis and treatment.

Paying attention to the natural course of disease for a development of gene therapy of inherited retinal diseases / 中华实验眼科杂志

Most inherited retinal diseases (IRDs) severely impair vision and lack effective treatments.With the approval of Luxturna, the world's first gene therapy drug for IRDs in 2017 by the U. S.FDA, gene therapy has brought new hope for the treatment of the disease.With an early onset and a relatively small number of patients, the understanding of the natural course of IRDs is limited in the past.The research on gene therapy of IRDs is mainly based on the in-depth understanding of the pathogenesis and natural course of disease, and the selection of the optimal treatment window for the implementation of gene therapy is the premise of successful treatment.At the same time, the main vector for gene therapy is recombinant virus vector, and its tissue-immunogenicity, tumorigenicity, safety of its integration with host cells and effectiveness determine the outcome of therapy, so the evaluation technology of IRDs gene therapy needs to be established.Gene therapy for ophthalmic diseases also involves the consideration of laws and regulations, ethics, product process, races and regional environment, disease progression, gene mutation types, patient benefit and risk ratio, and other factors.Therefore, it is of great significance to take full account of the differences in IRDs population, especially the particularity of children patients, and actively carry out the study on the natural course of IRDs in China for the scientific and normative development of clinical trials of gene therapy, the effective establishment of endpoint and outcome indicators for clinical studies of gene therapy, and the compliance with international norms of ethics.

Research progress of myopic retinoschisis / 国际眼科杂志(Guoji Yanke Zazhi)

@#Myopic retinoschisis(MRS)is a major complication of pathological myopia. The pathogenesis of MRS is not yet fully understood and it can be the result of a number of different factors. The posterior vitreous cortex and the internal limiting membrane(ILM)are thought to play a role in the formation of the retinal splitting. In addition, retinal arteriole traction has been associated with axial length extension and the pathogenesis of MRS. The diagnosis of MRS is done by using optical coherence tomography(OCT), B-mode ultrasound and ultra-wide field fundus autofluorescence(UWF-FAF). The main treatment methods of MRS are pars plana vitrectomy(PPV)and the macular buckling technique(MB). This article reviews the pathogenesis, the course and the diagnostic methods of MRS, as well as, the treatment progress.

Establishment of rat model of retinal branch vein occlusion by photochemical method and correlational research / 国际眼科杂志(Guoji Yanke Zazhi)

AIM:To investigate the natural course and adverse event of branch retinal vein occlusion (BRVO) rat model induced by laser photochemical method. METHODS: Thirty SD (Sprague Dawley) rats were administrated Bangladesh via tail vein. Then 532nm laser (80mW, 100μ m and 100ms) was performed on retinal vein secondary bifurcation of bitamporal optic disk for 50 spots. Electroretinogram (ERG), fundus fluorescein angiography ( FFA), optical coherence tomography (OCT) and fundus (fluorescein) photograph were applied on 1, 3, 5, 7, 10, 14 and 21d after BRVO model constructed. Two rats were sacrificed, respectively, on 1, 5 and 21d after photocoagulation to carry on HE (Hematoxylin - Eosin stain) and VEGF - α (vascular endothelial growth factor - α) immumohistochemical staining. RESULTS: There were three rats died, three rats with severe retinal detachment for excessive bleeding,one rat with retinal sunken, and one rat with cataract. FFA and fundus ( fluorescein) photograph showed that the successful BRVO rat model was 73% (22/30). It was found that the near-end photocoagulation vein became coarse, far - end became diminution on 1d and the photocoagulation vein total recanalization was on 3-7d. ERG showed the amplification of b wave (dark -adaptation 3.0 response) decreased to 0.694士0.042 of control eyes and on 5-7d decreased to rock bottom about 0.487士0.064 of control eyes. Then it increased Aii the time to 0.708士0.0465 of control eyes on 21d. OCT and HE staining found that retinal ganglion cells and outer nuclear layer became edema on 1d in vivo and in vitro.It was observed that the thickness of retina on photocoagulation vein (0μ m or 250μ m) decreased from 5d and there were 3-4 layer cells in ONL on 21d. The expression of VEGF-α at injured site were significantly more than control eyes on 1d and there were no significant difference on 5d;But the expression of VEGF-α were slightly less than control eyes on 21d. CONCLUSION: Photochemical method was a feasible method to establish BRVO rat model. The evolution and development of the BRVO model could partly mimic human BRVO phenomenon. At the same time, it should be improved to increase the successful model rate.

Prognostic Significance of IL-10 in Childhood Chronic Immune Thrombocytopenic Purpura / 임상소아혈액종양

BACKGROUND: The proposal of the present study was to assess the cytokine imbalances such as serum levels of IL-2, IL-10 and TGF-β1 in childhood chronic ITP during the thrombocytopenic and spontaneous remission phases. The findings of this study are anticipated to offer new insights into the pathogenesis of childhood chronic ITP and the prognostic factors involved in spontaneous remission.METHODS: Ten children with chronic ITP, 6 thrombocytopenic and 4 recovered cases, were enrolled. Five healthy children and eight healthy adults were included in this study as controls. The serum levels of IL-2, IL-10 and TGF-β1 were measured by ELISA technique.RESULTS: During this study, four patients achieved spontaneous remission. The serum level of IL-10 was higher in the patients that recovered spontaneously compared to the patients with persistent chronic thrombocytopenia. The serum level of IL-2 was not able to be detected as the levels were too low for analysis. TGF-β1 showed no significant differences among the groups in this study.CONCLUSION: These data suggest that increased serum level of IL-10 could have prognostic significance in the natural course and long-term outcome of childhood chronic ITP.

Prognostic Significance of IL-10 in Childhood Chronic Immune Thrombocytopenic Purpura / 임상소아혈액종양

BACKGROUND: The proposal of the present study was to assess the cytokine imbalances such as serum levels of IL-2, IL-10 and TGF-β1 in childhood chronic ITP during the thrombocytopenic and spontaneous remission phases. The findings of this study are anticipated to offer new insights into the pathogenesis of childhood chronic ITP and the prognostic factors involved in spontaneous remission. METHODS: Ten children with chronic ITP, 6 thrombocytopenic and 4 recovered cases, were enrolled. Five healthy children and eight healthy adults were included in this study as controls. The serum levels of IL-2, IL-10 and TGF-β1 were measured by ELISA technique. RESULTS: During this study, four patients achieved spontaneous remission. The serum level of IL-10 was higher in the patients that recovered spontaneously compared to the patients with persistent chronic thrombocytopenia. The serum level of IL-2 was not able to be detected as the levels were too low for analysis. TGF-β1 showed no significant differences among the groups in this study. CONCLUSION: These data suggest that increased serum level of IL-10 could have prognostic significance in the natural course and long-term outcome of childhood chronic ITP.

Current Understandings of Spontaneous Isolated Superior Mesenteric Artery Dissection

Spontaneous isolated superior mesenteric artery dissection (SISMAD) has been known as a rare vascular disease. However it is increasingly reported in these days with the development of advanced imaging technology. Underlying etiology, natural course or an optimal management strategy of SISMAD is not exactly known at the moment. During the past 10 years, we have had an interest in this rare vascular disease and collected clinical and image data in 100 or more patients with SISMAD. In this review article, I would like to describe my current understanding of SISMAD on the base of our recent publications in the major vascular surgery journals.

Long-Term Recurrence Rates of Peptic Ulcers without Helicobacter pylori

BACKGROUND/AIMS: The purpose of this study is to investigate the recurrence rate of peptic ulcer disease (PUD) over a long follow-up period with PUD patients without Helicobacter pylori. METHODS: We retrospectively reviewed patients diagnosed with PUD on endoscopy and divided them into two groups: a H. pylori-negative group (HP-negative group), and a group of patients with untreated H. pylori (HP noneradicated group). We compared the recurrence rates of PUD in these two groups and analyzed the factors that affected ulcer recurrence. RESULTS: Total of nine hospitals in Korea participated, and a total of 1,761 patients were retrospectively reviewed. The HP-negative group included 553 patients, and the HP noneradicated group included 372 patients. The 5-year cumulative probabilities of PUD recurrence were 36.4% in the HP-negative group and 43.8% in the HP noneradicated group (p=0.113). The factors that were found to affect recurrence in the HP-negative group were elder, male, and comorbid chronic kidney disease. CONCLUSIONS: The 5-year cumulative probability of PUD recurrence without H. pylori infection after a long-term follow-up was 36.4% and the factors that affected recurrence were elder, male, and comorbid chronic kidney disease.

The natural course of atopic dermatitis / 国际儿科学杂志

The natural course of atopic dermatitis (AD) has been a key subject with major concerns of physicians and patients.Tne pathogenesis of AD is complicated, which is associated with genetic, immunological,environmental and lifestyle factors, resulting in skin barrier impairment and immune system dysfunction.Therefore, there are individual differences in the age of onset, duration, aggravation, prognostic factors for remission and response to treatment.In recent years, with evidence of genetic research and a large amount of clinical retrospective studies,we have a more comprehensive understanding of the natural course of AD,which will help develop individualized treatment and evaluation strategies.

Adult liver hemangioma: natural course and treatment / 中华肝胆外科杂志

Objective To investigate the natural course and treatment of adult liver hemangioma (ALH).Methods The records of ultrasonography of 2422 patients with ALH were retrospectively reviewed.Results The patients were between 16 and 69 years of age (mean age 42,9± 11.4) with a male to female ratio of 1 ∶ 1.02 (1197/1225).The prevalence rate gradually increased with age after 30 years old.The highest prevalence rate occurred between 40-69 years.ALH was most commonly 1.1-3 cm in size,located in right liver with a solitary lesion.ALHs were bigger and were seen earlier in females than males.Concurrent illness was also more common in females than males.1427 patients had 3 to 8 repeat sonographies.At a follow-up of 42.7±9.5 months,1351 patients had no change in the ultrasonographic pattern or number of haemangiomas.Increasing in size of the lesions was demonstrated in 76 patients.154 patients received operation or interventional treatment.Conclusions The prevalence rate of ALH rose with age.Female sex hormones might accelerate ALH enlargement but they did not induce the formation of ALH.Most ALH remained stable in size and in patterns.Attention should be paid to rule out other illnesses in patients with ALH and with symptoms.

Pituitary Stalk Hemangioblastoma in a von Hippel-Lindau Patient : Clinical Course Follow-Up Over a 20-Year Period

Supratentorial hemangioblastomas (HBs) are rare, and pituitary stalk HBs are extremely uncommon; therefore, pituitary stalk evaluation is often overlooked. Herein, we report the development of pituitary stalk HB over a 20-year period and the importance of regular long-term follow up for patients with HBs.

Etiology, Pathogenesis and Natural Course of Chronic Pancreatitis / 대한내과학회지

The etiology of chronic pancreatitis varies greatly as demonstrated in the TIGAR-O classification. However, due to the lack of correlation and reproducibility between the etiologic factors and the presentation of the disease, it is presumed that individual sensitivity such as genetic predisposition would play an important role in its pathogenesis. Although our understanding on the pathophysiology of chronic pancreatitis is still far from being adequate, we are gaining more insights on its underlying mechanism through active researches related to pancreatic stellate cells, and molecular/genetic studies. Since the clinical manifestations of chronic pancreatitis show significant variation according to its cause, stages of the disease, and presence of local complications, controversy still remains regarding the natural course of chronic pancreatitis and warrants further study. At present, it is generally agreed that acute pancreatitis, recurrent acute pancreatitis, and chronic pancreatitis lie on the same spectrum of the disease.

Solid Pseudopapillary Tumor of the Pancreas with Hepatic Metastasis: Spontaneous Regression Over 10-Year Follow-Up Period

A solid-pseudopapillary tumor (SPT) of the pancreas is known as a low grade malignant tumor with a good prognosis; therefore, surgical intervention is necessary. A 14-year-old boy presented with a large pancreatic SPT and three hepatic metastases. The patient and his family refused surgery. Two serial follow-up CT scans over a period of 13 years demonstrated almost complete disappearance of the pancreatic tumor and three hepatic metastases without relevant treatment. Although there have been a few reports of spontaneous healing of SPT, there has been no report regarding spontaneous disappearance of SPT and distant metastasis. Herein, we report on the spontaneous regression of a large SPT and the disappearance of three hepatic metastases.

The Natural Course of the Intraductal Papillary Mucinous Neoplasm of Pancreas

Intraductal papillary mucinous neoplasm (IPMN) of the pancreas is characterized by cystic dilatation of the pancreatic duct, excessive mucin production, and papillary growth of the tumor. Occasionally, it forms a fistula into other organs. Because of the higher malignant potential of the main duct type IPMN compared to the branch duct type, patients with main duct type IPMN usually undergo surgical treatment and the natural history of the main duct type IPMN is not commonly observed in clinical practice. An 84-year-old woman was diagnosed with main duct type IPMN of pancreas, but she refused operative treatment. Seven years later she was admitted by chance for another disease, and then referred to our gastrointestinal department. We think this case can give us an insight of the natural course of the main duct type IPMN.

The Natural Course of the Intraductal Papillary Mucinous Neoplasm of Pancreas

Intraductal papillary mucinous neoplasm (IPMN) of the pancreas is characterized by cystic dilatation of the pancreatic duct, excessive mucin production, and papillary growth of the tumor. Occasionally, it forms a fistula into other organs. Because of the higher malignant potential of the main duct type IPMN compared to the branch duct type, patients with main duct type IPMN usually undergo surgical treatment and the natural history of the main duct type IPMN is not commonly observed in clinical practice. An 84-year-old woman was diagnosed with main duct type IPMN of pancreas, but she refused operative treatment. Seven years later she was admitted by chance for another disease, and then referred to our gastrointestinal department. We think this case can give us an insight of the natural course of the main duct type IPMN.

Comparison of the Natural Course and Intravitreal Triamcinolone Treatment for Macular Edema with Branch Retinal Vein Occlusion

PURPOSE: To evaluate the natural course of eyes with decreased visual acuity associated with macular edema secondary to branch retinal vein occlusion (BRVO) and to compare the visual outcome and macular thickness with those of eyes treated with intravitreal triamcinolone acetonide injection (IVTA). METHODS: We reviewed the medical records of patients with macular edema secondary to BRVO who were followed-up for 12 months. We evaluated the best corrected visual acuity (BCVA) and macular thickness of the patients who had no treatment for macular edema (natural course group) and compared them with those of patients who had been treated with IVTA (treatment group). RESULTS: A total of 29 eyes (29 patients) in the natural course group and 27 eyes (27 patients) in the treatment group were enrolled. The BCVA of the natural course group improved in a slow but steady manner and showed a statistically significant difference at 12 months. The treatment group did not show any improvement in BCVA throughout the follow-up period. Therefore, BCVA significantly improved in the natural course group after six months compared to that in the treatment group. Macular thickness showed a statistically significant reduction in three months in the natural course group and in one month in the treatment group. CONCLUSIONS: Although IVTA was effective in reducing macular edema in branch retinal vein occlusion at one month after administration, the natural course group had a superior outcome and more improved macular thickness after six months.

The natural history and prognostic factors of Graves' disease in Korean children and adolescents / 소아과

PURPOSE: Graves' disease is the most common cause of hyperthyroidism in children and adolescents. In this study, we investigated the natural course and the prognostic factors of Graves' disease in Korean children and adolescents. METHODS: One-hundred thirteen (88 girls and 25 boys) patients were included in this study. A retrospective analysis was made of all patients who were diagnosed with Graves' disease. The following parameters were recorded and analyzed: patient's sex, age at diagnosis, duration of disease, laboratory findings, symptoms and signs, and family history of autoimmune thyroid disease. RESULTS: All patients were initially treated with antithyroid drugs, either methimazole (93.8%) or propylthiouracil (6.2%). Antithyroid drugs had been discontinued in 75 (66.4%) of 113 patients. Of these 75 patients, 23 (20.4%) relapsed after 25.5+/-33.7 months. Thirteen (11.5%) of 23 patients, who experienced the first relapse, showed a second remission. However, 2 (1.8%) of 13 patients relapsed again. Euthyroid state could not be achieved by antithyroid drugs in 1 patient, and radioactive iodine therapy was performed. The older the patient at diagnosis, the greater the likelihood of remission (P=0.034). CONCLUSION: Age at diagnosis seems to be a prognostic factor in Korean children and adolescents with Graves' disease, and should be taken into account in treatment plan determination.

Diabetic Macular Edema Before and After Intravitreal Triamcinolone Injection

PURPOSE: To compare intravitreal triamcinolone acetonide (IVT) versus natural course in refractory diabetic macular edema. METHODS: In a prospective interventional case series, twenty five eyes with refractory DME which had been allocated to the sham group of a previous clinical trial underwent new examination and optical coherence tomography about 9 months after their first enrollment. Twenty eyes that met the inclusion criteria, visual acuity (VA) 200 micrometer, were treated by 4 mg IVT. Evaluations were repeated at 2 and 4 months post-injection to imitate the similar examination intervals after sham injection. Corrected visual acuity and macular thickness changes following IVT were compared to the corresponding changes after sham injection (the natural course). RESULTS: Visual acuity changes within and between each period were not statistically significant. Visual acuity decreased 0.08 & 0.09 logMAR by 2 months and 0.06 & 0.04 logMAR by 4 months after sham and IVT injections, respectively. The changes of macular thickness after IVT and sham intervention were not meaningful either. However, the difference between thickness changes by 4 months (52+/-50 micrometer increase after sham vs. 262+/-115 micrometer reduction after IVT) was significant (P=0.014). CONCLUSIONS: Concerning macular thickness, IVT has beneficial effect on refractory diabetic macular edema as opposed to observation. However, considering visual acuity, it does not induce significant difference in comparison to the natural course of the disease.

Diabetic Macular Edema Before and After Intravitreal Triamcinolone Injection

PURPOSE: To compare intravitreal triamcinolone acetonide (IVT) versus natural course in refractory diabetic macular edema. METHODS: In a prospective interventional case series, twenty five eyes with refractory DME which had been allocated to the sham group of a previous clinical trial underwent new examination and optical coherence tomography about 9 months after their first enrollment. Twenty eyes that met the inclusion criteria, visual acuity (VA) 200 micrometer, were treated by 4 mg IVT. Evaluations were repeated at 2 and 4 months post-injection to imitate the similar examination intervals after sham injection. Corrected visual acuity and macular thickness changes following IVT were compared to the corresponding changes after sham injection (the natural course). RESULTS: Visual acuity changes within and between each period were not statistically significant. Visual acuity decreased 0.08 & 0.09 logMAR by 2 months and 0.06 & 0.04 logMAR by 4 months after sham and IVT injections, respectively. The changes of macular thickness after IVT and sham intervention were not meaningful either. However, the difference between thickness changes by 4 months (52+/-50 micrometer increase after sham vs. 262+/-115 micrometer reduction after IVT) was significant (P=0.014). CONCLUSIONS: Concerning macular thickness, IVT has beneficial effect on refractory diabetic macular edema as opposed to observation. However, considering visual acuity, it does not induce significant difference in comparison to the natural course of the disease.

Stem Cell Transplantation in Retinitis Pigmentosa Patients

PURPOSE: This study was to evaluate the effect of stem cell transplantation into the sub-retinal space in retinitis pigmentosa (RP) patients for improving their visual acuity. RP is a progressive hereditary disease that leads to the blindness, and there is no reliable treatment for recover the disease until now. METHODS: The author transplanted mesenchymal stem cell extracted from umbilical cord blood into the sub-retina of four RP patients recommended by Korea RP association, and checked the changes of their visual acuity and electroretinograms. RESULTS: There was no obvious improvement in their visual acuity and elecroretinograms compared with preoperative state. Two patients, however, experienced slight improvements of their color sense and peripheral visual fields, and slight decrease of photophobia. Although there were no any obvious complications in relation to the stem cell transplantation, surgical procedures developed complications such as vitreous hemorrhage and retinal detachment. CONCLUSIONS: Mesenchymal stem cell derived from umbilical cord blood could not improve visual acuity and electroretinogram of RP patients.