ObjectiveTo observe the efficacy of adjuvant intravitreal injection of anti-vascular endothelial growth factor (VEGF) therapy for advanced Coats disease. MethodsThis study is a retrospective case series study. Fourteen patients (14 eyes), presenting Coats Stages 3B and 4 (8 and 6 eyes, respectively) were enrolled. All the patients were treated with adjuvant intravitreal anti-VEGF therapy. The intravitreal anti-VEGF injections varied from 1 to 7, with a median injections of 2.14. In 14 eyes, combined therapy was subretinal fluid drainage in 4 eyes, photocoagulation in 2 eyes, vitrectomy in 8 eyes. The follow-up period was ranged from 4 to 36 months, with a median follow-up of 18.8 months. Visual acuity and retinal reattachment were observed in follow up. ResultsAt last follow up, global suvival was 100.0% with no enucleation performed in any patient because of disease progression. Except for 2 children who were unable to cope with the visual acuity test, visual acuity was improved in 2 patients, stable in 8 patients, and decreased in 2 patients. 5 patients (35.7%) achieved in complete retinal reattachment, 3 patients (21.4%) were succeed in partial retinal reattachment, and the remain 6 patients(42.8%) failed in retinal reattachment. Two patients developed cataract after vitrectomy, and no other adverse reaction was observed during follow-up. ConclusionAnti-VEGF therapy combined with classic treatments in advanced Coats disease can keep or impove the visual acuity in most patients by reducing of subretinal exudation.
ObjectiveTo evaluate the clinical features and follow-up effect of treatment for retinal capillary hemangioma (RCH) of myopic disc.MethodsRetrospective case series studies. A total of 14 patients (15 eyes) diagnosed with juxtapapillary RCH (JRCH) at Peking Union Medical College Hospital and followed up for more than 12 months from January 2007 to December 2019 were included. Among these patients, there were 7 males (7 eyes) and 7 females (8 eyes) with the average age of 29.8 ±12.7 years. There were 6 isolated JRCH cases, all of which were monocular patients, including 1 case with a single peripheral RCH; 8 cases with von Hippel-Lindau disease, including 5 cases of binocular RCH. Out of 15 eyes, 6 eyes were merged with peripheral RCH. Three eyes were simply observed, and 12 eyes were treated with photodynamic therapy (PDT) alone or PDT combined with other treatments. The average follow-up time was 55.2 months. Its clinical characteristics and long-term treatment effects were analyzed.ResultsAt the first visit, among the 15 eyes, 3 eyes had visual acuity＜0.1, 5 eyes were 0.1-0.4, and 7 eyes were＞0.5. The endogenous and fixed exogenous JRCH were 12 (80%, 12/15) and 3 (20%, 3/15) eyes, respectively. The size of the tumor was 1/4 to 4 optic disc diameters (DD); the combined macular edema, epiretinal membrane, and macular hole were 11, 5, and 1 eyes, respectively. There were 3 eyes with tumor diameter less than 1 DD. The visual acuity at the first diagnosis was 0.6-1.0, and no treatment was given. The follow-up time was 12 months in 2 eyes and 120 months in 1 eye. At the last follow-up, his vision remained stable and the tumor did not enlarge. Six eyes with tumor diameter ranging from 1.0 to 2.5 DD, visual acuity was 0.06 to 0.6 at first diagnosis, accompanied by retinal exudation. 5 eyes were treated with PDT alone, and 1 eye was treated with PDT combined with anti-vascular endothelial growth factor drugs. The follow-up time was 12 to 84 months. At the last follow-up, the visual acuity improved or stabilized in 5 eyes and decreased in 1 eye. There were 6 eyes with peripheral RCH, including 4 eyes with retinal detachment, 2 eyes with epiretinal membrane, and 1 eye with vitreous hemorrhage. The visual acuity at the first visit was 0.02-0.6. All patients were treated with PDT combined with transpupillary thermotherapy, extrascleral drainage, and vitrectomy. The follow-up time was 12 to 132 months. At the last follow-up, all eyesight decreased.ConclusionsVisual decrease cause by JRCH often occurs at young adults. Bilateral JRCH are more common in patients with von Hippel-Lindau's disease. The tumor can cause retinal exudation, macular edema, epiretinal membrane and macular hole, resulting in gradual vision loss. PDT and/or combined anti-vascular endothelial growth factor drugs for medium sized JRCH with retinal exudation can maintain long-term vision stability. Patients with large JRCH or severe complications such as retinal detachment tend to have poor prognosis.
ObjectiveTo evaluate the efficacy of intravitreal injection (IVI) of expansile gas alone to treat idiopathic full-thickness macular hole (FTMH).MethodsThis is a prospective interventional case series. Twenty FTMH patients (26 eyes) who underwent IVI with expansile gas alone were enrolled in this study. There were 5 males (5 eyes) and 21 females (21 eyes), with the mean age of (59±12) years. All patients received the best corrected visual acuity (BCVA), slit lamp microscope, indirect ophthalmoscopy, fundus color photography and three-dimensional optical coherence tomography (OCT) examinations. The BCVA was measured using the international standard visual acuity chart, and the results were converted to the logarithm of the minimum angle of resolution visual acuity. The diameters of macular holes and the interface between vitreous and macular were observed by OCT (Topcon, OCT-2000). Based on the diameter, the holes were classified as small FTMH (equal or lesser than 250 μm), medium FTMH (more than 250 μm but equal or lesser than 400 μm) and large FTMH (more than 400 μm). The mean BCVA was 0.85±0.29. There were 7, 10 and 9 eyes with small, medium and large FTMH. There were 10 eyes with vitreous- macular traction (VMT). All the eyes received IVI of 0.2 ml C3F8 followed facedown positioning for 7-14 days. The follow-up ranged from 1 to 23 months. The BCVA, FTMH closure and complications were observed. If holes failed to close at 1 month after IVI, vitrectomy combined with internal limiting membrane (ILM) peeling and C3F8 tamponade would be performed for these eyes.ResultsFTMHs was able to close in 17/26 eyes (65.4%) had hole closure, failed to close in 9 /26 eyes (34.6%). All 10 eyes with VMT achieved vitreous-macula separation after IVI of gas. The eyes failed in the closure initially with IVI of gas alone, all succeed with hole closure after vitrectomy combined with ILM peeling and C3F8 tamponade. The closure rate of small (6 eyes), medium (8 eyes) and large FTMH (3 eyes) was 85.7%, 80.0% and 33.3% respectively. The diameter of FTMHs in holes-closure eyes and failed-closure eyes was (307.8±122.8), (431.6±128.4) μm respectively, the difference was significant (t=−2.407, P=0.024). VMT was found in 6 eyes and 4 eyes in holes-closure group and failed-closure group, respectively, the difference was significant (t=−2.196, P=0.038). The mean preoperative BCVA was 0.51±0.36. There was a significant difference between pre-and postoperative BCVA (t=4.758, P＜0.05). Two eyes developed local retinal detachment, which achieved hole closure and retinal reattachment after vitrectomy.ConclusionIVI of expansile gas alone is an effective way in treating FTMH with a diameter smaller than 400 μm and with VMT before surgery.
Objective To study the quantitative changes of ubiquitin l igase MAFbx mRNA and protein expression, muscle atrophy and muscle function following free muscle transplantation and to explore relationshi ps among them. Methods Thirty-six female SD rats, SPF grade, weighing (250 ± 25) g, were used. One hind l imb of the rat was randomly selected as experimental side to receive in situ free gracil is muscle transplantation, and the counterlateral hind l imb underwent no operation serving as control side. General condition of the rats was observed after operation. Muscle contractivecapacity and muscle wet weight maintenance rate of the experimental and the control side were detected 1, 2, 4, 10, 15, and 30 weeks after operation, and 6 rats were killed at each time point. Meanwhile, HE staining was performed to observe muscle fibre cross-sectional area, real-time quantitative PCR was appl ied to detect relative expression of MAFbx/Atrogin-1 mRNA, and Western blot test was used to observe MAFbx protein expression. Results All rats survived till the end of the experiment, all incisions healed well, and no dysfunction occurred in the experimental sides. The value of muscle contractive capacity, muscle wet weight maintenance rate, muscle’s maximal force of single contraction, and muscle’s maximal force of tetanic contraction in the experimental sides dramatically decreased in the first 4 weeks after operation and increased gradually over 4 to 30 weeks. The MAFbx mRNA expression of the experimental sides peaked and was seven times greater than the control sides 2 weeks after operation, then the value gradually decreased over 15 to 30 weeks after operation and was 1.1 to 1.5 times greater than the control sides, and significant difference was evident between the experimental sides and the control sides at each time point (P lt; 0.05). Significant difference was evident between the experimental sides and the control sides in terms of MAFbx protein expression of the muscle 1 to 15 weeks after operation according to the Western blot result (P lt; 0.05), and no significant difference was noted at 30 weeks (P gt; 0.05). The correlation coefficient between muscle wet weight maintenance rate and muscle’s maximal force of single contraction maintenance rate was 0.95, between muscle wet weight maintenance rate and muscle’s maximal force of tetanic contraction maintenance rate was 0.75, between muscle fibre cross-sectional area recovery rate and muscle’s maximal force of single contraction maintenance rate was 0.93, and between muscle fibre cross-sectional area recovery rate and muscle’s maximal force of tetanic contraction maintenance rate was 0.68 (P lt; 0.05). The correlation coefficient between MAFbx mRNA expression and the parameter of muscle wet weight maintenance rate, muscle fibre cross-sectional area recovery rate, muscle’s maximal force of single contraction maintenance rate, and muscle’s maximal force of tetanic contraction maintenance rate was — 0.62 (P lt; 0.05), — 0.45 (P gt; 0.05), — 0.72 (P lt; 0.05) and — 0.78 (P lt; 0.05), respectively; the correlation coefficient between MAFbx protein relative expression and the parameter of muscle wet weight maintenance rate, muscle fibre cross-sectional area recovery rate, muscle’s maximal force of single contraction maintenance rate, and muscle’s maximal force of tetanic contraction maintenance rate was — 0.95 (P lt; 0.05), — 0.82 (P lt; 0.05), — 0.89 (P lt; 0.05), and — 0.54 (P gt; 0.05), respectively. Conclusion Decrease of muscle function after transplantation correlates closely with muscle atrophy. The high expression of MAFbx mRNA and protein, especially their persistent increases from 4 to 15 weeks after nerve reinnervation, is a junction between the muscle atrophy and thedecrease of muscle function.
ObjectiveTo explore the clinical characters, diagnosis and treatment of Penicilliosis marneffei (PSM) in healthy hosts. MethodsThe clinical data of three cases of PSM involving the lung, bronchus and pleura in healthy hosts were retrospectively analyzed, and the case reports of Penicillium marneffei (PM) infection in healthy hosts were summarized by searching database for related articles, with "Penicilliosis marneffei" as key word both in English and Chinese literatures. ResultsThe main clinical manifestations of three PSM patients included fever, cough, lymphadenopathy, and elevated white blood cell counts.Series of chest computed tomography of three cases demonstrated atypical and extensive lung lesions.The fiberoptic bronchoscopy of the 2nd case showed several irregular nodules on the bronchial wall.The thoracoscopy of the 3rd case showed much pleural adhesions and small nodules on visceral and parietal pleura.Final diagnosis of PSM was confirmed by the fungal culture, and all patients received antifungal treatment.The symptoms relieved on medication but reoccurred on discontinuation.Literature review identified 36 cases of PSM in healthy hosts.The main clinical features were fever, cough/expectoration, skin rash/skin abscess and elevated white blood cell counts.The most common used antifungal drug was amphotericin B alone or combined with traconazole.The thoracic radiological imaging showed that roughly half of them had clearly thoracic lesions (20/36, 55.65%), and the pulmonary inflammatory infiltrate shadow was most common.Seventeen cases (47.22%) were misdiagnosed as tuberculosis. ConclusionsPulmonary involvement is common in healthy hosts with PSM and the thoracic radiological features are variable and unspecific, so PSM is easy to be misdiagnosed as tuberculosis.The PSM patients need a longer treatment than other fungal infections.