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Objective:To summarize and analyze the clinical, laboratory and imaging characteristics of patients with Caroli syndrome (CS), so as to deepen the understanding of the disease and explore the possible methods for improving early diagnosis.Methods:From January 2008 to June 2021, the clinical data of 18 CS patients who were hospitalized in Peking Union Medical College Hospital and diagnosed by pathology or by clinical and imaging features were collected. The general data, clinical manifestations, laboratory examination (white blood cell count, hemoglobin, etc.), imaging features (ultrasonography, computed tomography (CT), magnetic resonance imaging (MRI)), and diagnosis and treatment of the patients were retrospectively analyzed. Descriptive method was used for statistical analysis.Results:The median age of 18 CS patients was 18 years old (ranged from 1 to 39 years old); there were 10 male and 8 female patients with a male-to-female ratio of 5∶4. The median time from symptom onset to diagnosis was 24 months (ranged from 1 month to 28 years). At the time of diagnosis, 15 patients had already had portal hypertension-related complications, and 8 patients had biliary infections. The common symptoms included abdominal distension (6 cases), fever with or without abdominal pain (5 cases), and loss of appetite (3 cases). The common abnormal laboratory findings included peripheral white blood cell count, hemoglobin level and platelet count below the normal reference value range, alanine aminotransferase and bilirubin level above the normal reference value range, and 4 patients had positive autoantibodies. Four patients were clearly diagnosed according to the pathology of liver biopsy, 14 patients were clearly diagnosed by imaging. Among which the diagnostic rate of abdominal ultrasonography for CS was 4/18, CT was 11/15, and MRI or magnetic resonance cholangiopancreatography was 12/16. The typical features of abdominal ultrasonography were liver cysts with splenomegaly, typical manifestation of CT was intrahepatic bile duct dilatation with " central dot sign", and MRI typically manifested as multiple cystic dilatations of intrahepatic bile ducts. Among the 18 patients with CS, 1 case underwent right hepatectomy, 3 cases were waiting for liver transplantation, and the other 14 patients chose symptomatic treatment due to economic reasons.Conclusions:The clinical manifestations and laboratory findings of CS patients lack specificity, and the diagnosis of most patients is delayed. The lack of understanding of the disease by clinical and imaging doctors may be one of the reasons affecting the early diagnosis of CS patients. The findings of splenomegaly and liver cysts by abdominal ultrasound may provide clues for the diagnosis of CS for clinical and imaging doctors.
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Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis characterized by the infiltration of bone and multiple organs by foamy lipid-laden histiocytes. ECD is easy to be misdiagnosed due to its complicated clinical manifestations. We report a patient who visited the hospital due to hypothalamic dysfunction, with central nervous system, pancreas, and lower limb bones involvements. Together with the evidence of clinical manifestations, imaging and pathology, this patient was diagnosed with ECD. After treatment with interferon-α, both the clinical symptoms and imaging manifestations of this patient were significantly improved.
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One 51 years old man was admitted to the rheumatology department with a history of prominent eyes, headache and blurred vision for half year. The main manifestations included retrobulbar inflammatory pseudotumor and retroperitoneal fibrosis. He was initially diagnosed as granulomatosis with polyangiitis. Prednisone and cyclophosphamide were administrated and effective. New mass of dura mater and osteosclerosis presented during follow up. Finally Erdheim Chester disease(ECD) was diagnosed by biopsy and pathological examination. Vemurafenib, a v-raf murine sarcoma viral oncogenes homolog B1 (BRAF) inhibitor, 480 mg was given twice a day. The patient′s condition is stable and still in follow-up. Although ECD is a rare histiocytosis, clinicians should pay attention to its manifestations and differential diagnoses.
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Objective:To observe the clinical effect of oxycodone hydrochloride sustained-release tablets in the treatment of pain in advanced malignant tumors.Methods:One hundred patients with advanced malignant tumor pain admitted to Hangzhou Cancer Hospital from June 2017 to August 2018 were selected.They were grouped according to the admission order.The observation group (50 cases) implemented oxycodone hydrochloride sustained-release tablets.The control group (50 cases) was treated with morphine sulfate sustained-release tablets.The onset time, analgesic effect, adverse reaction and KPS score were compared between the two groups.Results:The onset time [(0.79±0.22)h] of the observation group was significantly shorter than that of the control group [(2.56±1.02)h]( t=11.995, P<0.05). The total effective rate of analgesia in the observation group (98.00%) was higher than that in the control group(74.00%)( t=11.960, P<0.05). The incidence of adverse reactions in the observation group was 4.00%(2/50), which was lower than 30.00%(15/50) in the control group, (χ 2 =11.977, P<0.05). The KPS score after treatment in patients with advanced malignant tumor pain in the observation group[(79.45±1.23)points] was higher than that in the control group[(65.34±1.92)points] ( t=43.756, P<0.05). Conclusion:Oxycodone hydrochloride sustained-release tablets have significant effects in the treatment of advanced malignant tumor pain, and it is safe.
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Objective Mesenteric panniculitis is an idiopathic , uncommon disease involving the adipose tissue of mesentery .The etiology , diagnosis and treatment are still unnoticed .We thus reported a case series to improve the understanding of this rare disorder .Methods We retrospectively analyzed the clinical data of 12 patients with mesenteric panniculitis including manifestation , diagnosis, treatment and prognosis.Results We found a male predominance (M∶F 3∶1) with the median age of 58 years old at diagnosis.The most common symptom was abdominal pain (9/12), followed by abdominal distension (3/12) and weight loss (3/12).Physical examination was unremarkable in the majority of patients (8/12).C reactive protein (9/12) and erythrocyte sedimentation rate (10/12) were normal in majority of patients.CT findings were of much diagnostic value .All patients had small intestinal mesentery involvement and multi-nodular appearance with increased fat density .Pseudo-capsule sign ( 8/12 ) and fat halo sign (6/12) were common.Pathological diagnosis was obtained in 4 cases showing fat tissue inflammation with local necrosis and fibrosis .Six cases all received prednisone , 2 with combined cyclophosphamide , 1 with azathioprine, 1 with tripterygium wilfordii .Short-term clinical response was achieved in all cases , but two patients relapsed .Conclusions Mesenteric panniculitis occurs predominantly in middle-aged and elderly . Abdominal pain is the leading symptom .Inflammatory markers are often normal while computed tomography is the most important diagnostic tool .Surgery combined with cortical steroid and immunosuppressant agents is effective.
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Objective To analyze the characteristics of published radiology original articles in China,and compare radiology research inboard and abroad.Methods According to the Contents of Chinese Science Core Periodicals in 2012,there were 15 comprehensive radiological core periodicals in China.All of them were retrospectively surveyed,and original articles published between October 2011 and October 2012were evaluated.The following information was abstracted from each article:radiologic technique used,radiologic subspeciahy,type of research,sample size and study design.At the same time,all the original articles from two top radiology journals,Radiology and European Radiology (ER) at the same period were evaluated.Chi-square test was used to compare the composition percentage inboard and abroad.Results There were 2784 original articles published in the 15 Chinese top radiological periodicals in total The most commonly used radiologic techniques were CT (1093 of 2784,39.3％),MRI (824 of 2784,29.6％) and intervention (402 of 2784,14.4％).Radiology and ER totally published 560 original articles.MRI (265 of 560,47.3％),CT (170 of 560,30.4％) and mixed techniques (63 of 560,11.3％) were most used.In China,as much as 2572 (92.4％) articles were clinical research,115 (4.1％) was basic research,9 (0.3％) was clinical and basic research,and 83 (3.2％) was non-basic non-clinical research.The sample size of less than 10,between 10 to 50,and more than 50 was 374 (13.4％),1448 (52.0％) and 847 (30.4％) respectively.339 (12.2％) Chinese articles belonged to perspective research,and 1211 (43.5％) were retrospective.For Radiology and ER,515 (92.0％) articles were clinical research,39 (7.0％) was basic research,3(0.5％) was clinical and basic research,and 3(0.5％) was non-basic non-clinical.The sample size of less than I0,between 10 to 50,and more than 50 was 30 (5.4％),225 (40.2％) and 252 (45.0％) respectively.230 (41.1％) articles belonged to perspective research,and 202 (36.1％) ones were retrospective.Radiology and ER articles used MRI more than that published in Chinese radiology journals (x2 =666.690,P ＜ 0.01).Clinical research was the most commonly used research type both inboard and abroad (x2 =0.116,P ＞0.05).Articles of sample size more than 50 abroad were more than that of China (x2 =44.895,P ＜0.01).Perspective research was used more abroad (x2 =275.667,P ＜ 0.01),and the main research type in China was retrospective.Conclusion Chinese radiology workers might need to do more perspective and larger sample size clinical researches.