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Lymphangioleiomyomatosis: An Analysis of Cases Reported In Chinese Literature from 1981 to 2009

Objective To summarize the clinical features of lymphangioleiomyomatosis ( LAM) in Chinese population. Methods The first case of LAM in China was reported in 1981. All cases of LAM reported in Chinese literature from January 1981 to December 2009 were reviewed. The cases for analysis were included with a diagnosis of LAM confirmed or probable according to the guidelines of European Respiratory Society, and with individual clinical data available. Results A total of 111 cases of LAM werepooled for analysis. All cases were female. The most frequent onset symptomof LAMwas dyspnea ( 51. 4% ) .The main clinical manifestations included dyspnea ( 93. 4% ) , pneumothorax ( 48. 6% ) , cough ( 45. 7% ) ,hemoptysis ( 35. 1% ) , and chylothorax ( 33. 3% ) . 11 cases were associated with tuberous sclerosis complex.An obstructive with or without restrictive abnormality in pulmonary function testing was observed in 60 /67 cases. Diffusion impairment was also common ( 45 /46) . Of the 109 patients, computerized tomography of chest showed thin-walled air-filled cysts throughout both lungs in 104 cases, multiple bullae in 3 cases, andhoneycomb changes in 2 cases. 102 cases were diagnosed based on pathological findings. Misdiagnosis was found in 44 cases, the median time of misdiagnosis was 24 months. Conclusion Awareness of LAMshould be raised when an adult female who presents with an unexplained dyspnea, especially combined with repeatedpneumothorax or chylothorax.

Clinical Analysis of 39 Patients with Idiopathic Pulmonary Arterial Hypertension

【Abstract】 Objective To improve the knowledge of idiopathic pulmonary arterial hypertension ( IPAH) to elevate the levels of early diagnosis and treatment. Methods The clinical data of 39 IPAH patients admitted in Beijing Anzhen Hospital from October 1997 to June 2010 were reviewed. Results Of the 39 IPAH patients, 14 cases were male and 25 cases were female, with an average age of ( 29. 7 ±16. 4)years old. Main clinical manifestations were exertional dyspnea/breathlessness ( 90. 9% ) , chest tightness( 72. 7%) , chest pain ( 30. 7% ) , cough ( 41. 0% ) , fatigue ( 48. 7% ) , syncope ( 35. 9% ) , cyanosis( 28. 2% ) , edema of lower extremity ( 43. 6%) , etc. As revealed through echocardiography, 39 cases had a mean systolic pulmonary arterial pressure ( SPAP) of ( 88. 8 ±24. 2) mmHg, with right ventricle enlargementin 37 cases and normal in 2 cases. Pulmonary angiography showed central pulmonary arterial dilatation with pruning of the peripheral blood vessels in 36 cases and normal in 3 cases. Right heart catheterization and acute vasodilator testing was performed with iloprost in 15 patients, systolic pulmonary arterial pressure was( 77. 6 ±27. 8 ) mm Hg, and positive rate was 20. 0% . 24 cases were misdiagnosed at admission, and misdiagnosis rate was 61. 5% . The average time of misdiagnosis was ( 26. 0 ±24. 5) months. 20 cases were treated with general medical therapy and 1 case was managed with lung transplantation before April 2008.Then 13 cases were given pulmonary arterial hypertension-targeted therapies, including sidenafil, iloprost or bosentan. Two patients died in hospital with a mortality rate of 5. 1% . Conclusions IPAH is uncommon and often occurs in young and middle-aged women. The symptoms are nonspecific and easily misdiagnosed.Echocardiography and pulmonary angiography are helpful in diagnosis. Right heart catheterization and acute vasodilator testing should be carried out if available. The patients should be early treated with pulmonary arterial hypertension-targeted therapies. Lung transplantation may be an option for end-stage cases.

The Advance in Diagnosis and Treatment on Large Cell Neuroendocrine Lung Cancer

Large cell neuroendocrine lung cancer（LCNEC） is the rare subtype of nonsmall cell lung cancer. Because of its low incidence rate and the special biological behaviour, it is hard to define in pathology. And we also know little about its epidemiological feature and the purposeful therapy view of LCNEC, and the therapeutic effect is unsatisfactory. This article will review and introduce the advance of research, clinical diagnosis and therapeutic of the LCNEC.

he Clinicopathologic Features, Diagnosis and Treatment of Papillary Cystic and Solid Tumor of Pancreas

ObjectiveTo study the clinicopathologic features, diagnosis and treatment of papillary cystic and solid tumor of the pancreas (PCSTP).MethodsOne case with PCSTP in our hospital and a review of 60 others from the literatures of the People’s Republic of China, a total of 61 cases were analyzed retrospectively.ResultsThe patients (57 women, 4 men) were of mean age 24.6 (range 9~59) years. The main manifestations included abdominal mass (n=52), pain (n=22) and discomfort (n=10). They were distributed in the head (n=29), neck and body (n=2), body (n=1), body and tail (n=5), tail (n=16) and capsule (n=2) of the pancreas. The other 6 cases occurred outside the pancreas. All the patients underwent surgical therapy. The tumors were identified by postoperative histopathologic examination, 7 of which were malignant (11.5%). The total 1, 3, 5year survival rate was 100%, 96.1% and 86.5% respectively.ConclusionIt is difficult to correctly diagnose the PCSTP before operation because PCSTP is often lack of typical clinical manifestations. The correct diagnosis should depend on histopathologic examination.Complete removal of the PCSTP is the most perfect treatment. PCSTP has a good prognosis.

Suggestions for Using FEV1/FVC Ratio below 70% after Inhaling Bronchodilator as Diagnostic Criteria of COPD

DIAGNOSIS AND TREATMENT OF INSULINOMA (ANALYSIS OF 25 CASES)

Diagnosis and surgical procedures were studied in this paper. Twentyfive cases of insulinoma were treated surgically from 1970 to 1997, in which 10 patients had Whipple′s triad so that the early diagnosis could be made. The sites of tumours were found in 10 out of 12 undergoing. Selective celiac angiongraphy with the accuracy rate of 83%, but B-ultrasonography and CT gave only 10% (4 out of 25) and 40%(8 out of 20) respectively. In this series, 5 cases underwent bodytail pancreatectomy and 20 cases enucleation of insulinoma. Blood glucose levels were tested during operation in 15 cases, and they all reached the normal level one hour after the tumours were completely removed. The authors consider that Whipple′s triad is the main basis in diagnosis of insulinoma. The selective celiac angiongraphy play an important role in detecting and locating the tumour. If diagnosis is made, surgical operation should be taken. The blood glucose monitoring during operation is the hallmark for complete resection of insulinomas.

Diagnosis and Treatment of Primary Small Intestinal Tumor

【Abstract】Objective To investigate the diagnosis and treatment of primary small intestinal tumor (PSIT). MethodsEighty-eight cases of PSIT were analyzed in our hospital retrospectively. Results Thirty-six cases were diagnosed before operation, 52 cases were misdiagnosed and the percentage amounted to 59.1%. The major clinical manifestations of PSIT were abdominal pain (66 cases, 75.0%), obstruction (56 cases, 63.6%), abdominal mass (51 cases, 58.0%) and stool with blood (48 cases, 54.5%).The major pathological types of PSIT were adenocarcinoma (55.7%), sarcoma of smooth muscle (25.0%), lymphoma (13.6%), fatty sarcoma (5.7%). The tumors of 36 cases located in jejunum (40.9%), 34 cases in ileum (38.6%) and 18 cases in duodenum (20.5%).Conclusion It is difficult to diagnose PSIT before operation. The following advice could help to get a correct diagnosis: ① Surgeons should be alert when meeting a patient with the above symptoms, and then collect and analyze the clinical data comprehensively. ②Patients over 40 years with complaints of inexplicable abdominal pain, weight loss, anemia, dyspepsia and abdominal mass should be examined appropriately by gastrointestinal X-ray, gastrointestinal endoscopy, ultrasonography, CT and angiography of superior mesenteric artery. ③When the diagnosis can’t be confirmed, an exploratory operation should be performed immediately to avoid the delay of treatment. ④The treatment of PSIT includes radical resection of small intestine and chemotherapy according to the pathological results of tumor.

Reinterpretation of 2017 Global Strategy for the Diagnosis, Management and Prevention of Chronic Obstructive Pulmonary Disease

The Global Strategy for the Diagnosis, Management and Prevention of Chronic Obstructive Pulmonary Disease is produced based on important studies published in last five years and published online. So far it is the most influential evidence-based strategy for the diagnosis, treatment and prevention of chronic obstructive pulmonary disease to guide clinical professionals.

THE DIAGNOSIS AND SURGICAL TREATMENT OF MULTIPLE PRIMARY COLORECTAL CARCINOMA

【Abstract】Objective To analyze the clinical features of multiple primary colorectal carcinoma(MPCC). Methods Data in 21 patients with MPCC during the past 10 years in our hospital were analyzed retrospectively. Results The incidence of synchronous and metachronous carcinoma was 1.1% and 1.2% respectively. The sites and pathologic stages of tumors showed no significant difference compared with single colorectal carcinoma. 47.6% of the cases accompanied with colorectal adenoma. 77.8% of the MPCC could be found during operation. Patients with carcinoma involved rectum had relatively poor survival. Conclusion The full-course colonoscopy, careful intraoperative exploration and regular postoperative colonoscopic follow-up are essential in improving the diagnosis and prognosis of patients with MPCC.

Diagnosis and Treatment on Primary Gastric Lymphoma

ObjectiveTo summarize the experience of diagnosis and treatment on primary gastric lymphoma. MethodsThirtyseven patients, proved by pathology, were included in the study. ResultsAmong clinical presentation, the upper abdominal pain, intestinal bleeding, and weight loss were common. Only 4 cases were diagnosed as PGL in 33 cases with the examination of Xray barium meal, 88.5% ( 23 of 26 cases) were missdiagnosed as gastric ulcer under gastroscopy. All cases underwent operation, among them 33 had been performed a radical operation. The survival period was over 5 years in 12 of 25 patients who have been followed up. ConclusionThe multiple biopsy sampling from submucosal layer via gastroscope may improve diagnostic rate on primary gastric lymphoma. Operative removal of the tumor should be the first choice of treatment. Additional chemotherapy after the surgery increases the fiveyear survival rate.