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Objective:To explore the relationship between the abnormal adipose tissue around the primary tumor of colon cancer and the prognosis.Methods:From January 2015 to December 2017, 448 patients with colon cancer in Peking University Cancer Hospital were retrospectively and consecutively collected. The scores were assigned to the severity, horizontal and vertical ranges of peritumoral adipose tissue (PAT) abnormalities, and the cumulative scores were calculated to establish the PAT grades from 1 to 3. We defined a score of 0 or 1 as PAT grade 1, a score of 2 as PAT grade 2, a score of 3 as PAT grade 3. The patients were followed up, and the overall survival (OS) and metastasis-free survival (MFS) were recorded. The Kaplan-Meier curve, log-rank test and Cox regression analysis were used to evaluate its impact on prognosis, and the hazard ratio (HR) and 95% confidence interval (CI) were calculated.Results:Among the 448 patients, patients with PAT grade 1, 2, and 3 accounted for 70.1% (314/448), 18.1% (81/448), and 11.8% (53/448), respectively. The Kaplan-Meier survival curve showed that patients with PAT grade 1 had the best OS, patients with grade 3 had the worst OS, and patients with grade 2 were in between, and the difference was statistically significant (χ 2=27.38, P<0.001). There were statistically significant differences between the grades in pairs ( P<0.05). There was no significant difference in MFS between different PAT grades (χ 2=2.85, P=0.240). The results of Cox regression analysis showed that PAT grade was an independent factor affecting the OS. Compared with PAT grade 1, the risk of death in patients with PAT grade 2 and 3 was significantly increased (HR 2.563, 95%CI 1.181-5.561; HR 2.269, 95%CI 1.005-5.121; P=0.034). PAT grade was not an independent factor of MFS ( P=0.253). Conclusion:The PAT grade established based on the degree and scope of abnormal PAT in colon cancer is an independent factor for poor prognosis of colon cancer.
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Objective:To analyze distribution characteristics of facial port-wine stains and brain imaging features in children with Sturge-Weber syndrome (SWS) .Methods:Clinical and imaging data were collected from 22 children with confirmed SWS at Department of Dermatology, Children′s Hospital Affiliated to Capital Institute of Pediatrics from July 2017 to August 2020, and retrospectively analyzed. The distribution characteristics of port-wine stains along the facial trigeminal nerve and brain imaging features were investigated in these children with SWS.Results:Among the 22 children, 10 were males and 12 were females. Their age ranged from 0.08 to 9.92 years, and the median age was 1.67 years. There were 13 cases of SWS type Ⅰ and 9 cases of SWS type Ⅱ. In terms of the port-wine stain type, 4 children aged from 0.50 to 2.17 years were diagnosed with pink port-wine stains; 14 children aged from 0.08 to 8.83 years were diagnosed with purple port-wine stains; 4 children aged from 4.92 to 9.92 years were diagnosed with thickened port-wine stains. Port-wine stains were distributed in the ophthalmic (V1) division of the trigeminal nerve in 22 patients, in the maxillary (V2) division in 20 patients, and in the mandibular (V3) division in 8 patients. There were 17 children with ocular abnormalities, aged from 0.08 to 9.92 years, including 11 with glaucoma, 5 with elevated intraocular pressure and 2 with visual impairment. Among the children with glaucoma, 7 developed glaucoma within 2 years of age, 8 suffered from unilateral glaucoma, and 3 from bilateral glaucoma, and glaucoma occurred on the same side as port-wine stains. Brain imaging abnormalities were observed in 12 children, and mainly included vascular malformations in the cerebral cortex involving the frontal, parietal, temporal and occipital lobes, as well as cerebral atrophy, punctate hemorrhage, calcification, sulcal widening, midline deviation and increased intraventricular choroid plexus vessels. Eleven children had symptoms of epilepsy and other neurological manifestations, including developmental delay, mental retardation and physical impairment.Conclusion:Port-wine stains distributed in the V1 and V2 divisions of the facial trigeminal nerve may indicate a higher risk of SWS, and ophthalmic and brain imaging screening as well as long-term follow-up should be performed.
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Objective To improve the understanding and diagnosis and treatment level of infant with Takayasu arteritis (TA) by analyzing the clinical features of 14 pediatric patients and reviewing related articles.Methods The clinical and follow-up data of infants with TA who were admitted to the Children's Hospital Affiliated to Capital Institute of Pediatrics between July 2016 and May 2019 were retrospectively analyzed.By reviewing related articles,the clinical features of this disease were summarized.Results The age of 14 patients (including 6 males and 8 females) were between 1 month and 23 days and 28 months.The most common clinical manifestations were fever in 10 cases (71.4％),hypertension in 9 cases (64.3％),weak or no pulse in 5 cases (35.7％).According to the clinical type of lesion vessels,11 cases (78.5％) were generalized type,3 cases (21.4％) were brachiocephalic artery type,and there was no thoracic abdominal aorta or single pulmonary artery type in this group.Among 14 infants with TA,12 cases had common carotid artery,carotid artery,subclavian artery,coronary artery and its branches (anterior descending branch,circumflex branch) involved (85.7％);11 cases had renal artery involved (78.6％);9 cases had radial artery involved (64.2％);8 cases had abdominal aorta involved (57.1％);6 cases had descending aorta involved (42.9％);6 cases had thoracic aorta involved (42.9％);6 cases had superior mesenteric artery involved (42.9％);5 cases had femoral artery involved (35.7％);5 cases had pulmonary artery involved (35.7％);and 4 cases had brachial artery involved (28.6％).In those 14 patients,11 cases were misdiagnosed,and 3 cases had unclear diagnosis,with misdiagnosis duration of 18 days to 2 months.In misdiagnosed cases,8 cases were misdiagnosed as atypical Kawasaki disease.Among those 14 cases,the ranges of most lesions were gradually decreased,and the slightly involved vessels even completely returned to normal state after treatment in 7 cases.The vascular imaging showed no significant exacerbation or imnprovement in 4 cases.Nine cases developed hypertension,the blood pressure of whom could be controlled within normal range with hypotensive drugs which could not be interrupted.Physical examination found weak or no pulse in 5 cases who were not improved.Among 14 patients,7 cases showed normal development,while the height and body mass of another 7 cases were the 25th percentile below those of normal children of the same age.All 14 patients were followed up for 2-22 months and received regular treatment without recurrence.Conclusions TA patients aged less than 3 years tend to have more blood vessels involved,be in serious condition and have higher rate of misdiagnosis.The disease can be controlled quickly after treatment,but vascular diseases may be developed easily.Some patients have a poor prognosis.
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Objective@#To improve the understanding and diagnosis and treatment level of infant with Takayasu arteritis (TA) by analyzing the clinical features of 14 pediatric patients and reviewing related articles.@*Methods@#The clinical and follow-up data of infants with TA who were admitted to the Children′s Hospital Affiliated to Capital Institute of Pediatrics between July 2016 and May 2019 were retrospectively analyzed.By reviewing related articles, the clinical features of this disease were summarized.@*Results@#The age of 14 patients (including 6 males and 8 females) were between 1 month and 23 days and 28 months.The most common clinical manifestations were fever in 10 cases (71.4%), hypertension in 9 cases (64.3%), weak or no pulse in 5 cases (35.7%). According to the clinical type of lesion vessels, 11 cases (78.5%) were generalized type, 3 cases (21.4%) were brachiocephalic artery type, and there was no thoracic abdominal aorta or single pulmonary artery type in this group.Among 14 infants with TA, 12 cases had common carotid artery, carotid artery, subclavian artery, coronary artery and its branches (anterior descending branch, circumflex branch) involved (85.7%); 11 cases had renal artery involved (78.6%); 9 cases had radial artery involved (64.2%); 8 cases had abdominal aorta involved (57.1%); 6 cases had descending aorta involved (42.9%); 6 cases had thoracic aorta involved (42.9%); 6 cases had superior mesenteric artery involved (42.9%); 5 cases had femoral artery involved (35.7%); 5 cases had pulmonary artery involved (35.7%); and 4 cases had brachial artery involved (28.6%). In those 14 patients, 11 cases were misdiagnosed, and 3 cases had unclear diagnosis, with misdiagnosis duration of 18 days to 2 months.In misdiagnosed cases, 8 cases were misdiagnosed as atypical Kawasaki disease.Among those 14 cases, the ranges of most lesions were gradually decreased, and the slightly involved vessels even completely returned to normal state after treatment in 7 cases.The vascular imaging showed no significant exacerbation or improvement in 4 cases.Nine cases developed hypertension, the blood pressure of whom could be controlled within normal range with hypotensive drugs which could not be interrupted.Physical examination found weak or no pulse in 5 cases who were not improved.Among 14 patients, 7 cases showed normal development, while the height and body mass of another 7 cases were the 25th percentile below those of normal children of the same age.All 14 patients were followed up for 2-22 months and received regular treatment without recurrence.@*Conclusions@#TA patients aged less than 3 years tend to have more blood vessels involved, be in serious condition and have higher rate of misdiagnosis.The disease can be controlled quickly after treatment, but vascular diseases may be developed easily.Some patients have a poor prognosis.
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BACKGROUND@#Endobronchial ultrasound guided transbronchial needle aspiration (EBUS-TBNA) is well known as an important technique for diagnosis and staging of lung cancer. But a standard protocol to deal with patients who have a negative pathology result still needs to be defined. Herein, we describe the subsequent procedures of these patients in a single center.@*METHODS@#A total of 1,412 patients with clinical suspected lung cancer and mediastinal metastasis who underwent EBUS-TBNA were collected between September 2010 and December 2016. Among them, 51 patients with nonspecific pathology result were included and retrospectively analyzed.@*RESULTS@#The 51 patients were stratified into five groups by clinical characterize and follow-up procedures: (1) Diagnosed by other bronchoscopy procedures group (9 cases). Abnormalities of tracheobronchial tree were found during visual examination in the majority of patients (8 cases). Biopsy, endobronchial brushing, bronchoalveolar lavage, and transbronchial lung biopsy (TBLB) were used to get a specific diagnosis. (2) EBUS-TBNA re-biopsy group (11 cases). Patients in this group had normal mucosal appearance and airway lumen. Re-biopsy were performed on patients in this group. (3) Surgery group (6 cases). Patients underwent surgery after negative result of EBUS-TBNA. Five of them were confirmed with non-nodal metastasis after surgery. (4) Underwent other pathology diagnosis group (15 cases). patients in this group had other metastasis sites besides midiastinal lymph node. Computed tomography (CT)-guided fine-needle aspiration and lymph node biopsy were performed. (5) Follow-up group (10 cases). None invasive procedure was used in this group. The median follow up time was 38 months. One patient was diagnosed lymphoma during the follow up.@*CONCLUSIONS@#Diagnostic procedures should be chosen based on the clinical character in EBUS-TBNA negative patients with suspected lung cancer. Long time follow-up is very important in patients whose diagnosis is apparently unknown.
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Female , Humans , Male , Middle Aged , Endoscopic Ultrasound-Guided Fine Needle Aspiration , Lung Neoplasms , Pathology , Lymphatic Metastasis , Mediastinum , Retrospective StudiesABSTRACT
Objective@#To summarize the clinical manifestations and determine the molecular etiology for two collagen type Ⅵ-related myopathy pedigrees.@*Methods@#Two spontaneous collagen type Ⅵ-related myopathy patients were admitted to Department of Neurology, Children′s Hospital, Capital Institute of Pediatrics in October 2017. Clinical data of probands and their family members were collected and their genomic DNA was obtained for genetic testing. Next generation sequencing was performed and the variants were verified by the Sanger sequencing in the family members.@*Results@#Target region sequencing indicated that the proband of family 1 has carried a heterozygous variant of COL6A3 gene, c.6229G>C(p.Gly2077Arg), and it was de novo variant confirmed by Sanger-sequencing in the family.The patient 1, a 2-year-three-month old boy, was admitted due to motor retardation at birth. He was defined as early severe Ullrich congenital muscular dystrophy. He never achieved independent ambulation, he had onset of symptoms was found at birth, including diffuse muscle weakness, striking distal joint hyperlaxity, proximal contractures, calcaneal protrusion, kyphosis, and hip dislocation. Serum CK level was elevated slightly and EMG showed neurogenic changes. The patient 2, a 7-year-old girl with a limp for 4 years, carried one de novo variant of COL6A3 gene,c.5169_5177del (p.Glu1724_Leu1726del). This variant results in the deletion of amino acids (1724 to 1726) in α3 chain of collagen Ⅵ, which may disturb the function of this protein.She was diagnosed as Bethlem myopathy with a mild phenotype. She had delayed motor milestones and presented with walking on tiptoe, hypotonia, and ithylordosis. The contracture of proximal joints was not very obvious. Serum CK level was normal and EMG showed myogenic changes.Muscle biopsy revealed muscular dystrophy and muscle magnetic resonance imaging of patient 2 showed vastus lateral is a "sandwich" sign. Immunofluorescence staining for COL6A3 chain in the cultured skin fibroblasts from patients 2 showed decreased deposition compared with control.@*Conclusions@#These two patients were diagnosed as spontaneous collagen type Ⅵ-related myopathy and carried different variants of COL6A3 gene. Different in pathogenetic variants could cause different genetic features and different phenotypes. Collagen type Ⅵ- related myopathy patients have various clinical manifestations. Typical phenotypes include muscular dystrophies, proximal contractures, and distal hyperlaxity. Muscle MRI shows diffuse fatty infiltration of gluteus maximus and thigh muscle. The histological staining showed the low level expression of COL6A3 chain. The seventy of phenotype was related to the genotype.
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BACKGROUND@#Endobronchial ultrasound guided transbronchial needle aspiration (EBUS-TBNA) has emerged as an innovative technique for diagnosis and staging of lung cancer. But whether the procedure can provide enough tissue for the detection of gene mutations is still to be defined. Here we evaluated the efficacy of lung cancer diagnosis and gene analysis using samples obtain via EBUS-TBNA.@*METHODS@#Patients with suspected lung cancer and mediastinal lesions were referred for EBUS-TBNA. Diagnosis and sub-classifications were made by pathologists. Samples with non-squamous non small cell lung cancer sub type were tested for the EGFR and/or ALK mutations.@*RESULTS@#A total of 377 patients were included in this study. The median needle passes were 2.07. Lung cancer was diagnosed in 213 patients. The diagnosis accuracy for malignancy was 92%. Epidermal growth factor receptor (EGFR) mutations, anaplasticlymphoma kinase (ALK) fusion genes and double genes analysis were successfully preformed in 84 (90%), 105 (95%) and 79 (90%) patients. The number of needle passes and the diameters of lymph node were not associated with the efficacy of gene testing in univariate analysis. However, samples of adenocarcinoma sub type showed a tendency associated with higher genotyping efficacy.@*CONCLUSIONS@#Tissue samples obtained through EBUS-TBNA are sufficient for pathological diagnosis and genetic analysis of lung cancer. The pathology type of sample affected genotyping efficacy.
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Adult , Female , Humans , Male , Carcinoma, Non-Small-Cell Lung , Diagnosis , Genetics , Pathology , Endoscopic Ultrasound-Guided Fine Needle Aspiration , Feasibility Studies , Genotyping Techniques , Lung Neoplasms , Diagnosis , Genetics , PathologyABSTRACT
Objective To improve the imaging diagnostic performance by analyzing US and CT findings of pancreatoblastoma in children. Methods From January 2000 to December 2016, 9 patients with pancreatoblastoma proven pathologically were enrolled in the study. The clinical and imaging features in 9 cases were analyzed retrospectively, the tumor parameters compared with intraoperative and postoperative pathology were location, size, texture, capsule, calcification, adjacent organs invasion and lymph node metastasis. The parameter of tumor size was evaluated by intraclass correlation coefficient. Results The tumor diameter ranged from 30-95 mm (median diameter 40 mm). Six tumors located in the head of the pancreas, 3 tumors located in the body and tail of the pancreas. One tumor was pure cystic, 2 tumors were solid and cystic mixed, 5 tumors were solid. Calcification was seen in 5 cases. Capsule was seen in 5 cases. Blood vessels, biliary tract and bowel around tumors were infiltrated by tumors in 3 cases proven by the pathology. Lymphadenectasis was seen in 2 cases, in which 1 case was metastasis. There were no significant differences in location, nature, calcification on US and CT. The largest diameter measured by CT and US was consistent with the tumor''s actual size, ICC=0.90.Capsule was shown by US in 5 cases and CT in 3 cases. CT showed the morphology of calcification in 5 cases, lymphadenectasis in 2 cases, abdominal effusion in 2 cases, cavernous transformation of the portal vein with the extensive collaterals in 1 case, which were not shown by US. Both US and CT dit not show the surrounding tissue infiltrated by tumors in 3 cases,but showed biliary system expansion. Conclusions The typical pancreatoblastoma manifested as encapsulated solid tumor originated in the pancreas with necrosis and calcification. US examination took a slight advantage on displaying capsule of tumor than CT. CT was more clear and intuitive in showing calcification and relationship between tumor with surrounding tissues and lymph node, and no operator dependent.
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Objective To investigate the CT features of hepatic focal nodular hyperplasia (FNH) in children.Methods Thirteen patients with FNH,which was confirmed by postoperative pathology,were enrolled retrospectively.Plain and contrast CT were performed on all patients before operation.The CT imaging features of FNH including size,shape,density,style of contrast were analyzed retrospectively and compared with pathology.Results There were 13 patients with 16 lesions,8 lesions were found in the right lobe,5 lesions in the left lobe and 3 lesions involving both lobes.The tumor size ranged from 5.5 cm to 11.5 cm (media size 7.5 cm) in diameter.Histologically,2 cases were typical type,11 cases were atypical type.The lesions were regular morphology in 12 cases and 1 case with capsule.On plain CT,the lesions were isodensity (n=1) or slightly low-density (n=12).In 2 typical type lesions,there were slit-like,stellate-shaped low density central scars.Arterial phase demonstrated that 12 cases were significantly enhanced and 1 case showed mild enhancement.The central scar was not enhanced.In 12 cases,thickened and torturous arteries were seen.The enhancement was reduced at the portal venous phase in all the lesions,with 10 cases showing slightly higher density,2cases isodensity and 1 case low-density.Two cases showed mild enhancement of the central scar.The enhancement of the solid portion in all lesions decreased at the delay phase,with 12 cases showing isodensity and 1 case slightly low density.Two cases with central scar showed delayed enhancement with slightly higher density.Conclusion The CT features of FNH in children are diversified but distinctive which are related with postoperative pathological findings.Combining with clinical symptoms and CT features can be helpful for the early diagnosis of FNH in children.
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Primary liver tumors in children are not rare, and the incidence of malignant liver tumors in children ranks third of all malignant abdominal tumors in children, with the features of a high rate of malignancy, a high incidence of congenital tumors, and a specific age of onset. Liver tumors in children often have no specific clinical manifestations, with an insidious onset, a large volume of tumor lesion, various pathological types, and complicated locations, which makes them more difficult to treat compared with liver tumors in adults. This article reviews the imaging diagnosis of liver tumors in children and points out that with the rapid development of imaging medicine, imaging examination provides an important reference for clarifying the location of liver tumors, identifying benign or malignant tumors, and determining tumor stage.
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BACKGROUND:There are stil some controversies about lateral compression rotational y unstable pelvic ring injuries and fixed mode. OBJECTIVE:To evaluate the pelvic stability of locking plate fixation pelvic reconstruction repairing lateral compression rotational y unstable pelvic ring injuries. METHODS:We retrospectively analyzed 15 patients with unstable lateral compression rotational y unstable pelvic ring injuries treated with reconstruction locking plates fixation at Suzhou Xiangcheng People’s Hospital between November 2011 and November 2014. The Matta standard was used to evaluate the quality of fracture reduction. Clinical efficacy was evaluated by Majeed score. RESULTS AND CONCLUSION:Fifteen patients were fol owed up for 12-44 months (mean 24 months). According to the standard of Matta, 11 cases were excel ent, 2 cases were good, 2 cases were average, 0 case poor, and the excel ent and good rate was 87%. According to Majeed functional assessment, clinical function outcomes were graded as 9 cases excel ent, 3 cases good, 1 case average, 1 case poor;excel ent and good rate was 80%. There were no patient loss of reduction and internal fixation failure among these 15 patients in the final fol ow-up. No iatrogenic neurovascular injury occurred. Incision superficial infection was detected in 2 patients with a Morel-Laval ee lesion after subjecting to double-tube continuous negative pressure drainage, and healed after wound management. A large area of infection and skin necrosis did not occur. These results confirm that pelvic universal reconstruction locking plates in repair of lateral compression rotational y unstable pelvic ring injuries can maintain a strong and effective fixation. We should pay much attention to the cases of pelvic fractures combined with soft tissue injury around the pelvis.
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<p><b>BACKGROUND</b>The high blood homocysteine (Hcy) levels found in patients with hyperhomocysteinemia (HHcy) have been implicated in an increased risk of cardiovascular disease morbidity and mortality in end-stage renal disease (ESRD). This study investigated the association of HHcy with progression of IgA nephropathy.</p><p><b>METHODS</b>We analyzed 108 participants newly diagnosed with IgA nephropathy between August 2005 and August 2007 in the Department of Nephrology, Chinese People's Liberation Army General Hospital. The association between clinicopathological factors and the Hcy levels were analyzed by Logistic regression and those with ESRD risk were analyzed by Cox regression.</p><p><b>RESULTS</b>Patients were aged (35.71 ± 10.73) years and included 45.71% women and 12.04% patients with HHcy. In multivariate Logistic regression analysis, HHcy was associated with arterial lesions (OR 2.60; 95% CI 1.55 ± 4.34; P < 0.001) even when age, body mass index, estimated glomerular filtration rate, mean arterial pressure, and initial proteinuria were taken into account. Mean follow-up was (67.37 ± 16.21) months. HHcy was also associated with worse ESRD-free survival (HR 4.71; 95% CI 1.45 to 15.31; P = 0.010).</p><p><b>CONCLUSION</b>HHcy is associated with the risk of intrarenal arterial lesions and may be useful for estimating the prognosis of IgA nephropathy.</p>
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Adult , Female , Humans , Male , Middle Aged , Body Mass Index , Glomerular Filtration Rate , Physiology , Glomerulonephritis, IGA , Hyperhomocysteinemia , Logistic Models , Retrospective StudiesABSTRACT
<p><b>BACKGROUND</b>Anticoagulation treatments are an important aspect of hemodialysis; however, few reports have addressed these treatments. This investigation intends to increase the understanding of the current status and improvements of hemodialysis-related anticoagulation treatments in China.</p><p><b>METHODS</b>In this study, an epidemiological investigation was conducted that examined 842 patients in 2007 and 1 175 patients in 2012 who underwent hemodialysis anticoagulation treatments in seven blood purification centers in northern Chinese cities.</p><p><b>RESULTS</b>Heparin was the most commonly used anticoagulant, although the percentage of use of low-molecular-weight heparin (LMWH) increased from 26.5% in 2007 to 42.1% in 2012. In 2007, there were no significant differences in anticoagulant selection among either patients with various primary diseases or patients with hemorrhage, thrombosis, thrombocytopenia, or a low hemoglobin level. However, compared with patients with other diseases, significantly lower doses of LMWH were administered to patients with hypertension (55.5 U/kg vs. 67.3 U/kg, P < 0.05) or diabetes (58.5 U/kg vs. 67.3 U/kg, P < 0.05), and patients with hemorrhage received lower doses of heparin than the other patients (61.6 U/kg vs. 71.8 U/kg, P < 0.01). In 2012, patients with diabetic nephropathy (51.5% vs. 36.5%, P < 0.01), hemorrhage (43.4% vs. 31.7%, P < 0.01), or a hemoglobin level below 90 g/L (57.2% vs. 37.1%, P < 0.01) experienced significantly higher doses of LMWH administration; patients with hemorrhage received significantly reduced LMWH dosages (50.4 U/kg vs. 57.8 U/kg, P < 0.05), and patients with thrombosis received significantly higher doses of heparin (73.8 U/kg vs. 62.1 U/kg, P < 0.01) or LMWH (57.8 U/kg vs. 52.6 U/kg, P < 0.05). Antiplatelet drugs were administered to 20.4% of the examined patients in 2007 and 20.7% in 2012. In 2012, patients with hypertension (25.9% vs. 18.5%, P < 0.01) and thrombosis (36.6% vs. 16.1%, P < 0.01) had a higher rate of using antiplatelet drugs than patients with other primary diseases and complications. Patients receiving antiplatelet drugs also received higher doses of heparin than patients without using antiplatelet drugs (74.4 U/kg vs. 65.9 U/kg, P < 0.01). However, the use of the drugs was not correlated with thrombocytopenia. The rate at which coagulation indices were determined increased from 45.7% in 2007 to 64% in 2012.</p><p><b>CONCLUSION</b>These findings suggested that hemodialysisrelated anticoagulation treatments in China have gradually become more standardized and individualized.</p>
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Adult , Aged , Female , Humans , Male , Middle Aged , Anticoagulants , Therapeutic Uses , China , Cities , Heparin, Low-Molecular-Weight , Therapeutic Uses , Renal Dialysis , MethodsABSTRACT
Objective Pulmonary sequestration (PS) is a rare disease,and its clinical symptoms are usually related to subsequent pulmonary infections.We analyzed the clinical characteristics of PS complicated by aspergillosis,and reviewed related literature,so as to disclose the association between these two diseases.Methods Sixty-nine patients with surgery-confirmed PS in Peking Union Medical College Hospital between January 1990 and December 2013 were retrospectively analyzed,including seven cases complicated by aspergillosis.Clinical manifestation,imaging and surgery of these patients were analyzed.Literature focusing on PS complicated by aspergillosis in pubmed data base was reviewed.Results In these seven patients,four cases were male,and three cases were female.Age at diagnosis ranged from 29 to 58 years old.The interval from onset to definite diagnosis ranged from two weeks to 20 years.Clinical symptoms included productive cough in seven cases,hemoptysis in three cases,chest pain in two cases,and fever in one case.All cases were intralobar PS with four in the left lower lobe and three in the right lower lobe.Consolidation in chest CT was noted in six cases.Cavitation was positive in three cases.Surgery of lung lobe resection was performed in all patients.Aberrant arteries were found during operation with the origin from aortic artery in four cases,phrenic artery in two cases,and intercostal artery in one case.Aspergillosis was diagnosed by pathology in six cases and by lung tissue culture in one case.Conclusions PS complicated by aspergillosis is extremely rare,but the trend of an increase in recent years has been noted.Strict and cautious examinations for microorganisms and pathology will help find relatively insidious aspergillosis.
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Objective To investigate the CT features of hepatic mesenchymal hamartoma (HMH)in children.Methods Nine patients with HMH confirmed by postoperative pathology were enrolled,including 4 were males and 5 were females.Their age ranged from 3 days to 9 years 5 months (the median age was 7 months).All patients admitted due to palpable abdominal mass without jaundice.All patients were examed by contrast-enhanced CT before the operation.Results All the 9 cases showed solitary hepatic mass,among which 6 were in the right lobe,2 were in the left lobe and 1 involved in both lobes.The tumor size ranged from 7.0 to 22.5 cm (mean size was 13.5 cm) in diameter.The CT manifestations of HMH was related to the proportion and distribution of component in the masses.The masses were cystic (n =1),cystic-solid mixed (n =6) and solid (n =3).After contrast administration,the solid component and the septa of the mass showed enhancement while cystic component was not enhanced.Calcification was seen inside the tumor in one case.Conclusions The CT features of HMH in children are multitudinous which are related to postoperative pathological findings.With the clinical history,it is easy to distinguish HMH from the other hepatic tumors.
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Objective To set up a new method,which is sensitive,low cost,rapid and suitable for clinical application for FTO gene rs9930506 variant genotyping basing on high resolution melting (HRM) platform,and to preliminarily put into practice in susceptibility analysis for metabolic syndrome (MS) in Beijing.Methods Unlabelled probe with C3-spacer block specific for rs9930506 variant has been designed according to the Refseq from GenBank.With LC-Green plus dye pre-mixed,we scanned the signal for the genotype analysis after PCR amplification and HRM reaction.Restriction fragment length polymorphism (RFLP) and PCR-sequencing methods were designed as 2 control genotyping methods for the evaluation of accuracy and convenience.Afterwards,the HRM-based method was put into practice in metabolic syndrome patients (n =500) and control groups (n =500) for rs9930506 genotyping,and primarily study the association between rs9930506 and MS.Results All the 3 methods could genotype rs9930506 appropriately,although the 2 control methods seemed to be a little time-inefficient.The call rate of HRM-method was 100％ and sampling accuracy reached 99.3％ according to sequencing results.In the MS group,AA,AG and GG genotypes were found in 290,185 and 25 cases,respectively.And in the control group,those were found in 344,138 and 18 cases.No genotype distribution difference was detected between control group and HapMap-CHB data (P =0.520 ).The genotype distributions were all in Hardy-Weinberg equilibrium in each group.AA genotype of rs9930506 seemed to reduce the risk for MS( OR =0.626,95％CI =0.483-0.812).Conclusions The AA genotype of rs9930506 variant in FTO might be a protective factor for MS in Beijing population.The susceptibility related genotyping in clinical samples could be more rapid,precise and inexpensive with the development of HRM in genotyping.
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ObjectiveTo compare the CT features of retroperitoneal ganglioneuroblastoma (GNB) and neuroblastoma (NB) in children,and summarize the differentiations between them.Methods From November 2007 to November 2011,19 patients with GNB and 21 patients with NB proven pathologically were enrolled in the study.The CT data of GNB and NB were analyzed retrospectively.These tumor parameters in CT included lesion location,size,shape,margin,calcification,across central line,style of contrast enhancement,degree of contrast enhancement,presence of small vessels in or around the tumor arranged in clumps or in line,tumor embolus,adjacent organs invasion or metastasis,lymph node metastasis,and relationship between tumor and surrounding great vessels.These parameters of both groups were compared by x2 and t test.ResultsIn GNB group,11 patients were located in adrenal gland and 8 patients in the retroperitoneal sympathetic chain; however,in NB group,18 patients were located in adrenal gland and 3 patients in the retroperitoneal sympathetic chain. The difference were statistical significances in the incidence rate of regular morphology,clear border,vessel encased by tumor,vessels displaced,presence of small vessels in /around the tumor arranged in clumps or in line,adjacent organs invasion or viscera metastasis,lymph node metastasis between NB (6/21,7/21,14/21,7/21,11/21,10/21,and 11/21,respectively) and GNB ( 12/19,13/19,6/19,13/19,4/19,3/19 and 4/19,respectively) ( P ＜ 0.05 ).However,there was no statistical significance in size,calcification,across central line,contrast enhancement type,contrast enhancement degree between them ( P ＞ 0.05 ).ConclusionsNB are more likely located at adrenal gland and presented a higher incidence rate of presence of small vessels in or around the tumor which arranged in clumps or in line,vessel encased by tumor,adjacent organs invasion or metastasis and lymph node metastasis.Regular morphology,clear border and vessels displaced are more frequently observed in GNB.
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Objective To investigate the difference and treatment strategy of malignancy-associated dermatomyositis and other para-neoplastic neurological syndromes (PNS).Methods The clinical characteristics of a patient with malignancy-associated dermatomyositis and poly radiculoneuropathy was reported and the relevant literature was reviewed.Results Patients with dermatomyositis had increased risk of malignancies,and should routinely screened.Dermatomyositis and polyradiculoneuropathy was clinically similar,but could rarely be seen in the same malignant patient.Malignancy -associated dermatomyositis and PNS had similar pathogenesis.The treatment strategy of both was similar.Malignancy specific treatment should be initiated and immune suppressive agents should be prescribed concurrently.Conclusion Rheumatolgists should aware the association between dermatomyositis and potential underlying malignancies.Multiple para-neoplastic syndromes could be seen in the same patient,but the diagnosis should be considered as one.
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Objective To investigate the characteristics and proper use of anticoagulants in hemodialysis (HD). Methods Thirty-one HD patients were enrolled in the study. Unfractionated heparins (UFH), dalteparin sodium or argatroban were used for HD anticoagulation respectively. Blood specimens were taken from the arterial line at the beginning (0 h) and at the end of HD (4 h), and from the arterial (2a) and the venous (2v) line respectively at 2 h of the HD session. Glass bead activated clotting time (gbACT), clot rate (CR) and platelet function (PF) were examined by Sonoclot analyzer. Prothrombin fragment 1+2 (PF1+2) and granule membrane protein-140 (GMP-140) were assayed by ELISA. Meanwhile, blood was taken from 8 healthy volunteers to examine the above parameters as control. Results (1) Compared with the control group, CR, PF1+2, PF, GMP-140 were increased significantly in all the patients (P<0.05). (2) UFH group:Compared with the 0 h point, gbACT of other time points increased significantly (P<0.05), CR, PF, and PF1+2 decreased significantly (P<0.05). Compared with the control group, gbACT increased (P<0.05) and CR decreased (P<0.05) significantly at the end of the sessions. (3) Daheparin sodium group: Compared with the 0 h point, gbACT of 2a point increased significantly (P<0.05), CR and PF1+2 of 2a, 2v and 4 h points decreased significantly (P<0.05), meanwhile, the extents of increased gbACT and decreased CR from the arterial line were greater than those from the venous line. Compared with the control group, gbACT increased significantly at the end of HD session (P<0.05), but CR was not significantly different. (4) Argatroban group: There were no significant differences of gbACT between 0 h and other time points. CR of 2a, 2v points decreased obviously than that of 0 h point, and CR of 2v decreased more significantly. CR of 2a point was not different from the control group, while CR of 4 h point was greater as compared to control group. During the monitoring, PF1 +2 tended to increase. Conclusions With intensive anticoagulant effect, UFH may induce the risk of hemorrhage not only during but also after the dialysis sessions. Dalteparin sodium, a good anticoagulant, is stir related with the risk of hemorrhage during HD. Argatroban is an ideal anticoagulant for patients with the risk of hemorrhage.
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Objective To explore the clinicopathological features of IgA nephrolpathy associated with malignant hypertension (IgAN-MHT) and to analyze their correlation with renal vascular lesions. Methods Twenty-nine patients of IgAN-MHT were screened from 2000 biopsy-proven eases with primary IgA nephropathy (IgAN) in our department from April 1997 to May 2007. Data of clinicopathology and follow-up of these 29 patients were collected. Semi- quantitative analysis was performed to evaluate the pathological changes. Inner lumen, outer lumen, intimal thickness, tunica media-to-internal lumen ratio of 436 arterioles, 124 interlobular arteries and 5 arcuate arteries were measured. The primary endpeint was the composite of a doubling of serum creatinine level and ESRD. Correlations of renal vascular lesions with clinical manifestation, pathological change and prognosis were examined by Spearman and Cox methods. Results 1.5% of all the IgAN patients presented malignant hypertension. The common clinical features were renal failure (100%), hyperurieacidemia (62.7%) and hypertriglyceridemia (51.7%). The average amount of urine protein excretion was 2.8 g/d. The common pathological changes were moderate mesangial proliferation, severe global sclerosis, severe interstitial inflammation and severe interstitial- tubular fibrosis. The small arteries (arcuate arteries and interlobular arteries) and arterioles (afferent arterioles) were both involved in IgAN-MHT. The characteristic lesions of intrarenal arteries included vascular occlusion, media thickening, proliferative endarteritis (onionskin lesion, musculomucoid intimal hyperplasia), hyaline arteriosclerosis, but mainly vascular occlusion (86.2%). The arteriole lesion was negatively correlated with age and total protein level; vascular occlusion was positively correlated with uric acid level. The average foUow-up period was 21.1 months. Forteen patients reached the endpoint. The arteriole lesion was the main independent risk factor for the progression of IgAN-MHT (RR=10.21, 95%CI=1.16～89.67). Conclusions The main clinical feature of IgAN-MHT is renal failure. The main histological feature of intrarenal vascular lesions is occludes arterioles. Arteriole lesion is the main independent risk factor for the progression of IgAN-MHT.