Several unusual manifestations such as white bile draining in common bile duct (14 cases) and casual massive bleeding (2 cases ) during and following hepatobiliary and pancreatic operations is reported. These manifestations were in fact signs of hepatic insufficiency. The manners of manifestations of hepatic insufficiency and their treatment are discussed, with a stress that liver-protective treatment and nutritional support are the fundamental modalities.
This study aimed to assess the therapeutic effect of mannital administration on acute hemorrhagic necrotizing pancreatitis (AHNP), 28 Wistar rats were randomily divided into therapeutic group and control group after induction of AHNP by retrograde intraductal injection of 5 percent sodium taurocholate. The rats of therapeutic group received intravenous 20% mannital (1g/kg) through tail vein, once in 12 hours, until the end of experiment; control group received saline (5.0 ml/kg) with the same way. Blood of all the rats were collected from heart and the rats were killed after 96 hours. Results: lipid peroxide (LPO) in pancreatic tissue, LPO in serum, lactate dehydrogenase (LDH), alpha-1-antitrypsin (α1-AT), glutamicoxalacetic transaminase (GOT), necrotizing square of pancreatic tissue in the therapeutic group were significantly less than those in control group (Ｐ＜0.05 or Ｐ＜0.01). The damage to pancrease, heart, liver, kidney in the therapeutic group were lighter than those of the control group and the mortality was lower (Ｐ＜0.05).Conclusions: Mannital can scavenge the oxygenderived free radicals and play a therapeutic role in AHNP.
ObjectiveTo observe and analyze the clinical characteristics and correlation between the eye and nervous system in children with infantile gangliosideosis.MethodsFrom November 2018 to January 2021, 3 children with infantile ganglion lipidosis diagnosed by genetic examination in the Department of Ophthalmology and Neurology, Beijing Children's Hospital of Capital Medical University, and through China National Knowledge Infrastructure and Wanfang database and The National Library of Medicine of the United States (PubMed) were searched, and 53 cases of Chinese infantile gangliosideosis diagnosed by gene, enzyme activity or pathological examination were selected and a total of 56 cases were included in the study. The searching time was from the establishment of the database to February 2021, and the search keywords are "gangliosideosis", "cherry-spot" macula and "Chinese". The demographic characteristics of 56 cases of children and other system manifestations were analyzed such as eyes, nervous system, skin, bones. According to the presence or absence of cherry-spot (CS) on the fundus examination, the children were divided into a fundus CS group (group A) and a fundus without CS group (group B), with 20 and 27 cases, respectively. The age of onset, gender, different types and neurological manifestations of the two groups of children were compared and analyzed. The non-parametric rank sum test was used for age comparison between groups; the χ2 test or Fisher's exact test were used for the comparison of gender, disease type and incidence between groups.ResultsAmong the 56 children, 27 were males and 29 were females; the median age of onset was 7.0 months. There were 33 and 23 cases of GM1 and GM2, respectively. Among 44 children with visual function examination records, 41 cases (93.2%, 41/44) were unable to follow the visual object. Of 47 children who underwent ocular fundus examination, 20 cases (42.6%, 20/47) had CS on the fundus. The main manifestations of the nervous system are neuromotor development regression or retardation (100%, 56/56), convulsions (58.1%, 25/43), and "startle" phenomena (89.7%, 26/29). Among 42 patients with brain magnetic resonance imaging examination records, 39 cases (92.9%) were abnormal. The incidence of "startle" and seizures in group A was higher than that in group B, and the difference was statistically significant (χ2=5.815, 6.182, P=0.021, 0.013).Conclusios Chinese infantile gangliosideosis is more common in GM1 type. Ocular visual impairment is the visual object as the main manifestation, the incidence of fundus CS is 42.6%, and the symptoms of neurological damage in children with CS are more severe.
Objective To observe the multimodal imaging features and explore the treatment of parafoveal exudative vascular anomaly complex (PEVAC). Methods A retrospective study. Six patients (6 eyes) with PEVAC diagnosed in Tianjin Eye Hospital were included in this study from July 2018 to December 2021. All patients were female with monocular disease. The age was (61.1±9.3) years. All patients showed a sudden painless decline in monocular vision with metamorphopsia. All patients underwent best corrected visual acuity (BCVA), color fundus photography, fundus fluorescein angiography (FFA), optical coherence tomography (OCT) and OCT angiography (OCTA). Indocyanine green angiography (ICGA) was performed in 4 eyes. In 6 eyes, 3 eyes were treated with intravitreal injection of anti-vascular endothelial growth factor drug; 5 eyes were treated with micropulse laser photocoagulation and/or local thermal laser photocoagulation; 1 eye was treated with photodynamic therapy. Five patients were followed up for (9.2±7.4) months, and 1 patient was lost. At follow-up, the same equipment and methods were used as at the initial diagnosis. The clinical manifestations, multimodal image features and treatment response were observed. Results Baseline BCVA of affected eyes were ranged from 0.1 to 0.5. PEVAC was isolated in 6 eyes, and the fundus showed isolated hemangioma-like leision, accompanied by small bleeding and hard exudation. There were 2 isolated hemangiomatous lesions adjacent to each other in 2 eyes. In the early stage of FFA, punctate high fluorescence lesions near the macular fovea were seen, and the leakage was enhanced in the late stage. There was no leakage in the early stage of ICGA, or slight leakage with late scouring. OCT showed an oval lesion with high reflection wall and uneven low reflection. The central macular thickness (CMT) was (431±76) μm. OCTA showed blood flow signals in PEVAC, 2 eyes in the superficial capillary plexus (SCP), and it was also observed in the deep capillary plexus (DCP), but the intensity of blood flow signal was slightly weaker than that in the SCP. The blood flow signal was visible only in DCP in 2 eyes. SCP and DCP showed similar intensity of blood flow signals in 2 eyes. After treatment, the bleeding was absorbed basically in 4 eyes, the hard exudation partially subsided, the CMT decreased, the intercortical cystic cavity of the fovea nerve decreased, the hemangiomatous lesions narrowed, and BCVA increased. In 1 eye, the macular sac was reduced and partially absorbed by hard exudation, which was later relapsed due to blood pressure fluctuation.ConclusionsThe majority of PEVAC patients had monocular onset. The fundus is characterized by solitary or structure with strong reflex walls, with or without retinal cysts, hard exudates, and subretinal fluid, and visible blood flow signals inside.
ObjectiveTo investigate the correlation between macular cherry red spot (CS) and severity of neurological manifestations in Chinese children with sialidosis (SD) type I. MethodsA evidence-based medical study. "China", "Sialidosis" and "Sialidoses" were used as Chinese and English search terms. The literature was searched in CNKI, Wanfang and PubMed. The cases were all from China and matched the diagnostic criteria. According to the presence or absence of CS in the fundus, the SD children were divided into a group with CS (+) and a group without CS (−), and the correlation between the occurrence of ocular CS and neurological manifestations was compared with meta-analysis by RevMan 5.3 software. ResultsSixty-eight studies were initially retrieved according to the search strategy, and 17 studies were finally included, and 5 studies with CS+ and CS− were meta-analyzed. Among the 43 patients, 28 were male and 15 were female, with a median age of 12 years. Visual impairment was observed in 37 cases (90.2%, 37/41, 2 cases not recorded), and CS was present in 24 cases (55.8%, 24/43). The most common neurological manifestation was myoclonus (97.7%, 42/43), followed by cerebellar ataxia (95.1%, 39/41, 2 cases not recorded) and seizures (91.4%, 32/35, 8 cases not recorded). Pathogenic NEU1 gene mutations were detected in 42 cases and one case was undocumented. The incidence of seizure in group CS+ (100%, 20/20) was higher than that in group CS− (80%, 12/15). Meta-analysis showed that there was no statistically significant difference between the incidence of myoclonus or ataxia [relative risk (RR)=1.13, 95% confidence interval (CI) 0.79-1.63, P=0.49] and seizure (RR=1.13, 95% CI 0.84-2.06, P=0.24) among the children in the CS+ and CS− groups. ConclusionsThe incidence of ocular CS in Chinese children with type I SD was 55.8%. There was no correlation with neurological manifestations, however the incidence of seizure was significantly higher in patients with CS than in others without CS.
ObjectiveTo compare the clinical efficacy and complications of intra-arterial chemotherapy (IAC) and intravenous chemotherapy (IVC) for unilateral advanced retinoblastoma (RB). MethodsA retrospective clinical study. From January 2020 to January 2021, 40 patients (40 eyes) unilateral group cT2 RB patients diagnosed at Baoding Children’s Hospital and Beijing Children’s Hospital were recruited in this study. There were 22 males (22 eyes) and 18 females (18 eyes). All were monocular. All the patients were assigned to two groups according to different treatment modalities they received: IVC group and IAC group. There were 26 eyes and 14 eyes, respectively. When the tumor invades the optic nerve, choroid, sclera, anterior chamber and iris, enucleation was performed. The globe salvage rate, tumor extraocular metastasis rate, solid tumor control rate, treatment-related complications and pathological high-risk factors after enucleation were observed. The globe salvage rate and solid tumor control rate were compared between the groups by chi square test. ResultsThe globe salvage rate of IAC group and IVC group were 88.5% (23/26) and 50.0% (7/14), respectively. Solid tumor control of IAC group and IVC group were 84.6% (22/26) and 42.9% (6/14), respectively. There were statistically significant differences in globe salvage rate and solid tumor control between the two groups (χ2=7.18, 7.56; P＜0.05). Compared with IVC group, IAC group had less systemic complications, mild ocular and periocular side effects. Among 26 cases in IAC group and 14 cases in IVC group, 3 and 7 cases underwent enucleation respectively. The results of pathological examination showed that there were 2 cases and 3 cases with pathological high-risk factors in the two groups, respectively. During the follow-up period, 2 cases in IAC group had extraocular metastasis, there was no extraocular metastasis in IVC group. ConclusionCompared with IVC, IAC has the advantages of high tumor control rate, high globe salvage rate, less and mild complications, however, there is still tumor recurrence.