Pleuropulmonary blastoma (PPB) is a kind of rare malignancy which often occurs in children and is related to dysplasia. It always involves in pleura and lung. PPB is misdiagnosed easily because of its special pathophysiological characters and complex biological behavior. Early detection and correct treatment are very important for thoracic surgeon to cure PPB. This paper reviews the advance of pathophysiological characters, molecular genetic characters, clinical manifestation, clinical diagnosis and differential diagnosis, and treatment and prognosis of PPB.
Objective To investigate the clinical manifestation and radiological features of unknownetiology ostearthritic disease in Shigeng village of Chongzhou city in Sichuan province so as to lay a basis for further study. Methods The data were analyzed retrospectively. The epidemiology investigation, physical examination, movement functional assessment, lab test and X-ray examination were performed on 116 villagers of 3 years and more than. Quantitative analysis on roentgenography was conducted. Results Sixty-four villagers had unknown ostearthritic disease，the prevalence was 55.2%；17 patients were limp, the prevalence of limp was 26.6%，onset age mainly focused under 40 years. Main manifestation of osteoarthritic disease was fixed pain of knee joint or hip joint, and bilateral pain was significantly more than single pain, but local fever and swelling were not obvious. Mild or moderate pain account for 73.5% of patients, and pain was aggravated after physical labor or movement and could be relieved after rest. Joint pain had no influence on joint movement function for long time, leading to a limp due to the joint functional disorders at late stage. Physical examination illustrated the flexion contracture of the hip and limitation of internal and external rotation. The Harris scores were 86.5±17.1(left hip) and 86.3±17.2. 46 (right hip), and the excellent or good result was achieved in 46 patients, fair and poor results in 18 patients. The average HSS scores were 88.4±9.3(left knee) and 88.2±11.0(right knee). The excellent or good result was achieved in 61 patients, fair and poor results in 3 patients. The X-ray films showed degenerative disorders of hip joint such as narrowing of the joint space (47.6%)，articular surface sclerosis and deformation (30.2%), shallow and increased density acetabulum, increased obliquity and insuffi cient coverage of the femoral head by the acetabulum, and increased femoral neck-shaft angles. Radiological features of knee joint were bony spur or bony bridge，osteoporosis，incomplete and thickening articular surface with sclerosis and deformation，degenerative disorders. Conclusion Main clinical manifestation of unknown osteoarthritic disease in Chongzhou city was fixed pain of bilateral knee joint or combined with hip joint pain at early stage，and functional limitation in hip joint at late stage. Main radiological feature was hip joint degeneration. To effectively control the osteoarthritic disease ofthis area, much more etiology researches should be done.
Objective To investigate early clinical manifestations of osteogenic sarcoma to help establishment of an early diagnosis of the disease.Methods A total of 92 patients with osteogenic sarcoma in the extremities were admitted to our hospital from April 1984 to October 2002. Of the 92 patients, 71 (42 males and 29 females; averaged age 17.4 years, range 666 years; illness course 1-28 weeks) had a complete record of their medical history and examination. From their first medical visits, we obtained their clinical symptoms, physical sings, diagnoses, and duration of the delayed diagnoses. The patients were pathologically confirmed as having osteogenic sarcoma in the extremities, with the lesions located in the distal femur in 38 patients, proximal tibia in 22, proximal femur in 3, proximal fibula in 3, proximal humerus in 2, distal tibia in 2, and distalradius in 1. Results Of the 71 patients, 70 had a local pain and/or a palpable mass, 37 had a persistent pain with no difference between day and night, 23 had an intermittent pain, and 11 had a nocturnal pain. Of the 71 patients, 42 had an initial pain related to trauma, and 3 of the 42 patients had a pathologic fracture. The patients with the local mass had a delayed diagnosis of osteogenic sarcoma with a delayed duration of 1-14 weeks, averaged 4 weeks; however, the patients without the local mass had a delayed diagnosis of this disease, with a delayed duration of 3-30 weeks averaged 14 weeks. In the patients undergoing an X-ray examination at the first medical visit, the duration of the delayed diagnoses was 1-20 weeks, averaged 8 weeks, but in the patients without an X-ray examination at first, the duration was 4-30 weeks, averaged 16 weeks. Conclusion Intermittent and persistent pains and local masses are the most characteristic clinical manifestations in the early stage of osteogenic sarcoma. A history of trauma often helps to make a diagnosis of the disease. Carefulclinical examination and observation should be given to adolescent patients whohave a recurrent pain around the joint.
Objective To investigate the clinical manifestations, CT and prognosis of pontine hemorrhage. Methods Analyze the summarized clinical data of 21 patients with pontine hemorrhage retrospectively. Results The period of 46-70 years old was vulnerable to pontine hemorrhage,and hypertension was the major risky factor of it. The death rate of pontine hemorrhage inceases when the bleeding beyond 5 ml. Nine of the 21 patients survived. Conclution The prognosis was highly related to the bleeding amount, the position of bleeding and the complication.
Objective To investigate the clinical characteristics， diagnosis， and treatment of gastric stromal tumor. Methods Clinical data of 217 patients with gastric stromal tumor from October 2007 to July 2011 were analyzed. Results The main clinical manifestation were abdominal pain， abdominal distension， bloody stools， abdominal mass， and so on. The tumour located at cardiac part， fundus of stomach， body of stomach， and pylorus part was 24 cases （11.0%）， 103 cases （47.5%）， 59 cases （27.2%）， and 31 cases （14.3%）， respectively. All the 217 patients underwent endoscopic or surgical resection and diagnosed by pathology and immunohistochemistry. The patients of high-low risk， low risk， intermediate risk， and high risk was 56 cases （25.8%）， 67 cases （30.9%）， 41 cases （18.9%）， and 53 cases （24.4%）， respectively. One hundred and forty patients were followed-up for 7-52 months （average 35 months）. Thirty-five patients of high risk were investigated about the drug treatment after the first operation：19 cases were treated by using imatinib （tumor progressed in 2 cases） and 16 patients were not （tumor progressed in 9 patients）. The rate of progression of patients treated by imatinib was significantly lower than another group （χ2=8.426， P=0.004）. In 11 patients with tumor progressed， tumor recurrnce in 4 cases， tumor recurrence with diffused abdominal cavity metastasis in 1 case， tumor metastasized to humerus in 1 case， metastasized to liver and abdominal cavity in 1 case， and metastasized to liver in 4 cases. Conclusions Gastric stormal tumor is lack of specific clinical manifestations. Complete excision of the tumor is the main therapy method， and imatinib can improve prognosis.
ObjectiveTo summarize clinical electrophysiological features and efficacy of some of Anti-epileptic drugs(AEDs) of Juvenile myoclonic epilepsy (JME). MethodsClinical electrophysiological information of 101 outpatients with JME observed at Xuanwu Hospital from Jul. 2001 to Sep. 2014 was retrospectively analyzed, including the seizure types, trigger factors, electroencephalogram. We followed some of these patients and compared the efficacy between different AEDs. Result According to different seizure types, there are four subtypes: Myoclonus (MJ) only 11.88%, MJ+generalized tonic-clonic seizure(GTCS) 75.24%, MJ+GTCS+Absence(Abs) 11.88%, MJ+Abs 1.00%. Patients with typical ictal generalized poly-spike and waves (PSW) or spike and waves (SW) or spikes account for 96.80%. And 75.00% of patients have no MJ and 91.80% have no GTCS with valproic acid monotherapy. 65.00% and 88.24% of patients were seizure free of MJ and GTCS recpectively. But the difference of efficacy between these two drugs have no statistically significance. Sleep deprivation was the primary trigger factors, accounting for 16.83%. ConclusionJME has clinical heterogeinety, clinicians should fully understand the whole condition of JME individual, including their clinical manifestation, EEG features, reaction to AEDs, trigger factors, habitual patterns and so on, in order to help making individualized therapy.
ObjectiveTo explore the features of images by CT,MRI,and MRV for early cranial venous sinus thrombosis (CVST) to provide the diagnostic evidence for choosing an optimal imaging examination. MethodsThe clinical data (imaging features of CT,MRI,and MRV) of 46 patients with CVST diagnosed between January 2009 and January 2013 were retrospectively analyzed. ResultsBrain CT showed the direct signs of CVST in 15 cases (32.6%),and indirect signs of CVST in 8(17.4%).MRI showed the signs of CVST in 17 cases (68.0%);the diagnostic positive rate of MR venography (MRV) combined with MRI was 87.5%,84.6% of which was in line with that of digital subtraction angiography. ConclusionCT can be used as screening tool for those highly suspect CVST cases.MRI combined with MRV show great diagnostic value for CVST.
ObjectiveTo analyze the clinical manifestations and laboratory test results of 357 cases of first-diagnosed systemic lupus erythematosus (SLE), and provide a reference for SLE diagnosis. MethodsA total of 357 SLE patients on their first diagnosis were collected from 2008 to 2012 in our hospital, and the clinical manifestations and laboratory findings of these patients were recorded and analyzed. ResultsAll the patients had different clinical manifestations and serious multi-system damages. In these patients, there were 59.1% (211 cases) involved with arthritis, followed by rash and kidney damage. The complement declined in 75.9% of the patients according to the results of immunological tests. In the ENA spectrum inspection, anti-SSA antibody had the highest positive rate (51.3%), followed by anti-u1RNP antibody (44.0%), anti-dsDNA antibody and anti-nucleosome antibody. The first onset of most clinical manifestations was rash (43.4%), followed by arthritis and lower extremity edema; a few patients had headache or numbness as the first symptom. ConclusionSLE patients have diverse clinical manifestations, serious multi-system damages and outstanding heterogeneity, which is inconsistent with foreign reports.
ObjectiveTo summarize the clinical pathologic features of primary tonsil tumor, in order to provide reference for its diagnosis and treatment. MethodsWe retrospectively analyzed the clinical data from January 2002 to April 2012, and found 810 cases of malignant tumor and 113 cases of benign tumor. The pathologic patterns and clinical manifestations of these cases were recorded for analysis. ResultsMost tonsil tumors were unilateral, and the malignant types were much more than the benign. The most common malignant tonsil tumors were lymphoma (610 cases), followed by squamous cell carcinoma (172 cases); inverted papilloma was the most common benign tumor (87 cases). Paresthesia in the pharynx was the most common clinical manifestation that existed in almost all cases. ConclusionTonsil tumor pathological type is very important for its diagnosis, treatment and prognosis. Laying emphasis on pathological diagnosis and differential diagnosis could avoid missed diagnosis, misdiagnosis and improper treatment, which is particularly important for clinical physicians of the otolaryngology, head and neck surgery.
ObjectiveTo investigate the clinical and imaging features of patients with pulmonary lymphangioleiomyomatosis (PLAM),to improve the knowledge of this disease. MethodsEighteen patients with PLAM admitted into Zhongshan Hospital between 2003 and 2013 were retrospectively analyzed. Relevant literatures were reviewed. ResultsAll the 18 cases were female and had dyspnea on exertion. Three of them suffered from chylothorax,6 from pneumothorax and 5 from hemoptysis. The high resolution CT (HRCT) findings in all the 18 cases showed multiple small cysts with thin wall diffusely distributing in the bilateral lung. PLAM was confirmed by biopsy in 14 cases. Pulmonary function tests in 4 cases showed obstructive ventilatory dysfunction. Nine cases (50%) were misdiagnosed as other diseases. All the patients were given symptomatic and supportive treatment. Literatures review showed that as a rare diffuse lung disease,PLAM is more common in women of childbearing age,mainly presenting with dyspnea,hemoptysis,pneumothorax and chylothorax. Diagnosis of the disease relies on medical history,HRCT features and lung tissue biopsy pathology. HRCT examination has diagnostic value for PLAM. Although anti-estrogen treatment has certain effects,rapamycin may be a promising drug for PLAM therapy. ConclusionPatients with PLAM have characteristic clinical and radiographic manifestations. Early diagnosis and effective treatment are keys to the management of patients with PLAM.