Tuberous sclerosis complex combined with primary lymphedema: A case report.

BACKGROUND: Tuberous sclerosis complex (TSC) and primary lymphedema (PLE) are both rare diseases, and it is even rarer for both to occur in the same patient. In this work, we have provided a detailed description of a patient's clinical presentation, imaging findings, and treatment. And a retrospective analysis was conducted on 14 published relevant case reports. CASE SUMMARY: A 16-year-old male came to our hospital for treatment due to right lower limb swelling. This swelling is already present from birth. The patient's memory had been progressively declining. Seizures had occurred 1 year prior at an unknown frequency. The patient was diagnosed with TSC combined with PLE through multimodal imaging examination: Computed tomography, magnetic resonance imaging, and lymphoscintigraphy. The patient underwent liposuction. The swelling of the patient's right lower limb significantly improved after surgery. Epilepsy did not occur.after taking antiepileptic drugs and sirolimus. CONCLUSION: TSC with PLE is a rare and systemic disease. Imaging can detect lesions of this disease, which are important for diagnosis and treatment.

Lymphatic plastic bronchitis and primary chylothorax: A study based on computed tomography lymphangiography.

BACKGROUND: This study presents an evaluation of the computed tomography lymphangiography (CTL) features of lymphatic plastic bronchitis (PB) and primary chylothorax to improve the diagnostic accuracy for these two diseases. AIM: To improve the diagnosis of lymphatic PB or primary chylothorax, a retrospective analysis of the clinical features and CTL characteristics of 71 patients diagnosed with lymphatic PB or primary chylothorax was performed. METHODS: The clinical and CTL data of 71 patients (20 with lymphatic PB, 41 with primary chylothorax, and 10 with lymphatic PB with primary chylothorax) were collected retrospectively. CTL was performed in all patients. The clinical manifestations, CTL findings, and conventional chest CT findings of the three groups of patients were compared. The chi-square test or Fisher's exact test was used to compare the differences among the three groups. A difference was considered to be statistically significant when P < 0.05. RESULTS: (1) The percentages of abnormal contrast medium deposits on CTL in the three groups were as follows: Thoracic duct outlet in 14 (70.0%), 33 (80.5%) and 8 (80.0%) patients; peritracheal region in 18 (90.0%), 15 (36.6%) and 8 (80.0%) patients; pleura in 6 (30.0%), 33 (80.5%) and 9 (90.0%) patients; pericardium in 6 (30.0%), 6 (14.6%) and 4 (40.0%) patients; and hilum in 16 (80.0%), 11 (26.8%) and 7 (70.0%) patients; and (2) the abnormalities on conventional chest CT in the three groups were as follows: Ground-glass opacity in 19 (95.0%), 18 (43.9%) and 8 (80.0%) patients; atelectasis in 4 (20.0%), 26 (63.4%) and 7 (70.0%) patients; interlobular septal thickening in 12 (60.0%), 11 (26.8%) and 3 (30.0%) patients; bronchovascular bundle thickening in 14 (70.0%), 6 (14.6%) and 4 (40.0%) patients; localized mediastinal changes in 14 (70.0%), 14 (34.1%), and 7 (70.0%) patients; diffuse mediastinal changes in 6 (30.0%), 5 (12.2%), and 3 (30.0%) patients; cystic lesions in the axilla in 2 (10.0%), 6 (14.6%), and 2 (20.0%) patients; and cystic lesions in the chest wall in 0 (0%), 2 (4.9%), and 2 (4.9%) patients. CONCLUSION: CTL is well suited to clarify the characteristics of lymphatic PB and primary chylothorax. This method is an excellent tool for diagnosing these two diseases.

Endoscopic classification and pathological features of primary intestinal lymphangiectasia.

BACKGROUND: The appearance of the intestinal mucosa during endoscopy varies among patients with primary intestinal lymphangiectasia (PIL). AIM: To classify the endoscopic features of the intestinal mucosa in PIL under endoscopy, combine the patients' imaging and pathological characteristics of the patients, and explain their causes. METHODS: We retrospectively analyzed the endoscopic images of 123 patients with PIL who were treated at the hospital between January 1, 2007 and December 31, 2018. We compared and analyzed all endoscopic images, classified them into four types according to the endoscopic features of the intestinal mucosa, and analyzed the post-lymphographic computed tomography (PLCT) and pathological characteristics of each type. RESULTS: According to the endoscopic features of PIL in 123 patients observed during endoscopy, they were classified into four types: nodular-type, granular-type, vesicular-type, and edematous-type. PLCT showed diffuse thickening of the small intestinal wall, and no contrast agent was seen in the small intestinal wall and mesentery in the patients with nodular and granular types. Contrast agent was scattered in the small intestinal wall and mesentery in the patients with vesicular and edematous types. Analysis of the small intestinal mucosal pathology revealed that nodular-type and granular-type lymphangiectasia involved the small intestine mucosa in four layers, whereas ectasia of the vesicular- and edematous-type lymphatic vessels largely involved the lamina propria mucosae, submucosae, and muscular layers. CONCLUSION: Endoscopic classification, combined with the patients' clinical manifestations and pathological examination results, is significant and very useful to clinicians when scoping patients with suspected PIL.

[Predictive Value of Pancreatic Steatosis for Severity of Coronary Atherosclerosis in Patients with Type 2 Diabetes Mellitus].

Objective To investigate the association of pancreatic steatosis with coronary atherosclerosis in patients with type 2 diabetes mellitus (T2DM). Methods Patients with T2DM who underwent coronary computed tomography angiography(CCTA)in our center due to chest pain were enrolled from January 2016 to February 2019. According to the CCTA findings,patients were divided into normal group,mild-to-moderate coronary atherosclerosis group and severe coronary atherosclerosis group. CT attenuation of pancreas and spleen was measured on abdominal non-enhanced CT,and the CT attenuation indexes including the difference between pancreatic and splenic attenuation (P-S) and the ratio of pancreas-to-spleen attenuation (P/S) were calculated. Analysis of variance or Kruskal-Wallis rank test were used to assess differences among each group. Logistic regression analysis was used to analyze the risk factors of severe coronary stenosis. The accuracy of P/S in predicting severe coronary artery stenosis was assessed by receiver operator characteristic (ROC) curve analysis. Results A total of 173 consecutive T2DM patients were enrolled. These patients included 27 patients with normal coronary artery (15.6%),124 patients with mild to moderate stenosis (71.7%),and 22 patients with severe stenosis (12.7%). There were significant differences in CT attenuation of pancreas (Z=11.543,P=0.003),P-S (Z=11.152,P=0.004) and P/S (Z=11.327,P=0.004) among normal coronary artery group,mild and moderate stenosis group,and severe stenosis group. The CT attenuation of pancreatic head,body,and tail significantly differed in patients with coronary artery stenosis (Z=14.737,P=0.001). After adjusting for confounding factors,multiple Logistic regression showed that P/S (OR=0.062,95%CI=0.008-0.487,P=0.008) was still significantly associated with the severe coronary artery stenosis. The area under the ROC curve of P/S for the diagnosis of severe coronary artery stenosis was 0.701,and the optimal cutoff point was 0.660. Conclusion CT attenuation of pancreas and CT attenuation indexes are associated with the severity of coronary stenosis in T2DM patients,suggesting that pancreatic steatosis may be used as one of the indicators for predicting severe coronary artery stenosis.

Relationship between Radiological Stages and Prognoses of Pneumocystis Pneumonia in Non-AIDS Immunocompromised Patients.

BACKGROUND: Although radiological features of pneumocystis pneumonia (PCP) in non-Acquired Immune Deficiency Syndrome (AIDS) immunocompromised patients have been reported by other authors, there were no studies on the radiological stages of PCP previously. This study aimed to elucidate the radiological stages and prognoses of PCP in non-AIDS immunocompromised patients. METHODS: Retrospective analysis of radiological manifestations and prognoses of 105 non-AIDS PCP immunocompromised patients from August 2009 to April 2016 was conducted. Chest radiograph was divided into three stages: early stage (normal or nearly normal chest radiograph), mid stage (bilateral pulmonary infiltrates), and late stage (bilateral pulmonary consolidations); chest high-resolution computed tomography (HRCT) was also divided into three stages: early stage (bilateral diffuse ground-glass opacity [GGO]), mid stage (bilateral diffuse GGO and patchy consolidations), and late stage (bilateral diffuse consolidations). RESULTS: The case fatality rate (CFR) of all patients was 34.3% (36/105), all of them took routine chest X-ray (CXR), and 84 underwent chest CT examinations. According to the CXR most near the beginning of anti-PCP therapy, 18 cases were at early stage and CFR was 0 (0/18, P< 0.01), 50 cases were at mid stage and CFR was 28.0% (14/50, P> 0.05), and 37 cases were at late stage and CFR was 59.5% (22/37, P< 0.01). According to the chest HRCT most near the beginning of anti-PCP therapy, 40 cases were at early stage and CFR was 20.0% (8/40, P> 0.05), 34 cases were at mid stage and CFR was 47.1% (16/34, P> 0.05), and 10 cases were at late stage and CFR was 80.0% (8/10, P< 0.05); barotrauma, including pneumothorax, pneumomediastinum, and pneumohypoderma, was found in 18 cases and the CFR was 77.8% (14/18, P< 0.01). CONCLUSIONS: Based on the radiological manifestations, the course of PCP in non-AIDS immunocompromised patients can be divided into three stages: early stage, mid stage, and late stage. The prognoses of patients treated at early stage are good, and those at late stage are poor. Furthermore, the CFR of patients with barotrauma is high.

[Retrospective analysis of relationship between radiological features and prognoses of pneumocystis pneumonia in non-AIDS immunocompromised patients].

OBJECTIVE: To elucidate the relationship between the radiological features and prognoses of pneumocystis pneumonia (PCP) in non-acquired immunodeficiency syndrome (non-AIDS) immunocompromised patients. METHODS: Retrospective analyses were performed for the radiological features and prognoses of 36 non-AIDS immunocompromised PCP patients hospitalized at Peking University First Hospital from April 2006 to April 2010. There were 24 males and 12 females with an average age of (51.7 ± 18.9) years. RESULTS: All patients underwent chest photographic examinations and chest computed tomography (CT) was performed on 29 of them. The overall case fatality rate (CFR) of 36 patients was 33.3% (12/36). At the start of anti-PCP treatment, there were normal chest radiography (n = 7), bilateral diffuse infiltration (n = 21), bilateral diffuse consolidation (n = 8), aerothorax, mediastinal emphysema or pneumohypoderma (n = 6) and numbers of deceased patients (n = 0, 8, 5 and 5 respectively). The CFRs of the patients with normal chest radiography and aerothorax, mediastinal emphysema or pneumohypoderma were both statistically significant as compared with the overall rate (P < 0.05). At the start of anti-PCP treatment, there were bilateral diffuse ground glass opacities (GGO) (n = 29), "mosaic sign" (n = 19), air bronchogram (n = 19), pulmonary consolidation (n = 17), bilateral pleural effusion (n = 7), thickened lobular septa (n = 6), cystic lesions (n = 5), aerothorax, mediastinal emphysema or pneumohypoderma (n = 4) and numbers of deceased patients (n = 8, 5, 5, 5, 2, 2, 2 and 4 respectively). The CFRs of the patients with aerothorax, mediastinal emphysema or pneumohypoderma were statistically significant as compared with the overall rate (P < 0.05). CONCLUSIONS: The major radiological features of PCP in non-AIDS immunocompromised patients are bilateral diffuse infiltration. And bilateral diffuse GGO on chest CT scans are usually accompanied with "mosaic" sign, air bronchogram or pulmonary consolidation. The prognoses are excellent for the patients with normal chest radiograph and poor for those with aerothorax, mediastinal emphysema or pneumohypoderma.

[Acute fibrinous and organizing pneumonia: a case report and review of the literature].

OBJECTIVE: To better understand the clinical and pathological characteristics of acute fibrinous and organizing pneumonia (AFOP). METHODS: A case diagnosed with AFOP was reported and the related literature was reviewed. RESULTS: A 73 year-old man presenting with fever, cough with small amount of white sputum and gradually worsening dyspnea was admitted to this hospital. Chest CT scan showed bilateral multiple nodules and patchy infiltrates. Treatment including anti-bacterial and anti-fungal drugs was initiated, but no improvement was observed. The dyspnea deteriorated and repeated chest CT showed an increase of the nodules and the patchy infiltrates. Ultrasound guided percutaneous lung biopsy was performed and the pathological examination revealed slightly widened alveolar septa, lymphocyte and plasma cell infiltration and the presence of intra-alveolar fibrin in the form of fibrin "balls" (organization) within the alveolar spaces. No neutrophil and eosinophil infiltration was detected. The finding was consistent with AFOP. Corticosteroid therapy was started and the patient showed significant clinical and radiological improvement after a course of treatment. The patient was discharged and followed in the outpatient clinic. The chest CT became nearly normal after treatment with corticosteroids for 1.5 months. CONCLUSIONS: The main clinical manifestations of AFOP were similar to those of acute lung injury. Diagnosis was made by lung biopsy. The optimal treatment for AFOP had not been established. Therapy with corticosteroids could be attempted, but relapse may occur during the period of reducing the dosage of corticosteroids.

[The clinical and pathological subtypes of Castleman's disease and their relationship with complications: a large series analysis from a single center].

OBJECTIVES: To investigate the clinical and pathological subtypes of Castleman's disease (CD) and their relationship with complications. METHODS: The clinical complications of 53 patients with CD and the relationship of these complications with clinical and pathological subtypes were analyzed retrospectively. RESULTS: Among 53 CD patients, 32 (60.4%) were classified as uni-centric type and 21 (39.6%) multicentric type. Histopathological examination showed that 37 cases (69.8%) were hyaline vascular variants (HV), 9 (17.0%) plasmacytic variants (PC), and 7 (13.2%) mixed cellular variants (Mix). Complications were identified in 32 (60.4%) patients, including the involvements of skin, internal organs and hematopoietic system. Some complications were closely associated with the clinical subtype of CD: the majority of complications in the 32 uni-centric CDs were paraneoplastic pemphigus (PNP) and bronchiolitis obliterans (BO), and those in 21 multi-centric CDs were the involvements of kidney and hematopoietic system. The complications were different among the three kinds of histopathological subtypes: PNP and BO were the predominant complications of HV variants, while the internal organ and hematopoietic system involvements were those of PC and Mix variants. The clinical and histopathological classification of CD patients with PNP were different obviously from other subtypes of CDs. In Kaplan-Meier survival analysis, the survival rate of those with complications was significantly lower than those without complication (P = 0.028). CONCLUSION: The clinical complications of CDs are related to their clinical and histopathological subtypes. CD patients with PNP should be considered as a unique entity to tailor the therapy. The presence of clinical complications is an independent prognostic factor in CD patients.

Invasive pulmonary aspergillosis in patients with antineutrophil cytoplasmic antibody associated vasculitis.

BACKGROUND AND AIMS: Invasive pulmonary aspergillosis (IPA) has been reported as a severe opportunistic infection in immunocompromised patients without neutropenia or cancer. Patients with antineutrophil cytoplasmic antibody associated vasculitis (AAV) with immunosuppressive treatment are susceptible to IPA, but only few cases were reported in the literature. We retrospectively analyze the clinical characteristics of our patients with IPA in AAV. METHODS: Hospitalized patients with AAV who developed IPA were selected. Their clinical data were retrospectively reviewed and possible risk factors for development of IPA were investigated. RESULTS: Seven of 157 patients with AAV were identified to have IPA. Two patients were classified as Wegener granulomatosis and 5 as microscopic polyangiitis with a mean age at 68.6 +/- 10.9 years. After immunosuppressive therapy, 7 patients developed IPA within 2 approximately 13 weeks. They had 1 or more risk factors increasing susceptibility to Aspergillus. Pre-existing chronic respiratory diseases were found in 5 patients. Despite intensive antifungal therapy, only 3 patients survived. The patients who died were older, with more severe lung injury and lower hemoglobin level. CONCLUSIONS: AAV patients with immunosuppressive therapy are susceptible to Aspergillus infection. Monitoring and prophylactic antifungal therapy should be recommended for patients at high risk.

[Idiopathic pulmonary hemosiderosis in adults: report of two cases and literature review].

OBJECTIVE: To describe the clinical, radiological and pathological characteristics of idiopathic pulmonary haemosiderosis (IPH) in adults and to evaluate the methods of diagnosis and treatment. METHODS: Two patients were successfully diagnosed and treated in our hospital and the literature on the subject was reviewed. RESULTS: Two adult patients (19 and 34 years old) diagnosed in our hospital had 5 and 10 years of history of hemoptysis respectively, and chest CT showed bilateral diffuse alveolar opacities over mid and lower zones. Tests of antinuclear antibodies (ANAs), rheumatoid factor (RF), antineutrophilic cytopasmic antibodies (ANCA) and Anti-glomerular basement membrane (anti-GBM) antibody were negative. Haemosiderin-laden macrophages were found in bronchoalveolar lavage fluid (BALF) whose color was yellow. Microscopic examination of the lung tissue specimens obtained by transbronchial lung biopsy (TBLB) revealed hemorrhage and numerous hemosiderin-laden macrophages in the alveoli and no vasculitis or capillaritis were seen. These findings were consistent with a diagnosis of IPH. Steroid therapy had good effects. CONCLUSION: IPH is a diagnosis of exclusion of other causes of diffuse alveolar hemorrhage (DAH). IPH adults have relatively good drug responses and relatively good prognoses.

[The diagnostic value of serum latex agglutination test for pulmonary cryptococcosis].

OBJECTIVE: To evaluate the diagnostic value of serum cryptococcal capsular polysaccharide antigen latex agglutination test (LA) for pulmonary cryptococcosis. METHODS: Serum and lung biopsy specimens of 27 patients, who were suspected of having pulmonary cryptococcosis based on clinical presentations and imaging features, were collected from July 2000 to July 2007 in the First Hospital of Peking University. LA test and histopathological examination were performed in all the patients. RESULTS: Histopathological examination confirmed pulmonary cryptococcosis in 9 cases, all of whom were positive for serum LA test. The other 18 cases were confirmed to have no cryptococcosis and all of them were negative for serum LA test. Therefore, the sensitivity and specificity of LA test were both 100%. CONCLUSION: The diagnostic value of serum LA test for pulmonary cryptococcosis is high.

[An analysis of the clinical and radiological features of bronchioloalveolar carcinoma].

OBJECTIVE: To describe the clinical and radiological features of bronchioloalveolar carcinoma. METHODS: Data of 1 050 inpatients of lung cancer, including 50 cases of pathology-proven bronchioloalveolar carcinoma, diagnosed in our hospital between 1993 and 2003, were retrospectively reviewed. The clinical information of all the patients and the HRCT findings of 31 cases of bronchioloalveolar carcinoma were analyzed. RESULTS: There was a female predominance (60%) in the 50 patients with bronchioloalveolar carcinoma. Cough was the most common presenting symptom (20/50). Twenty-four patients sought medical attention because of abnormal chest X-rays, most of which were of nodular type at the early stage. Twenty patients were completely asymptomatic at presentation. Out of the 31 cases with HRCT, eight were of the miliary type. "Bubble-like lucency" and calcifications inside nodules were present in 7 cases respectively out of the 11 cases of the nodular type. There were 9 cases each with low attenuation consolidation, pseudocavities, reticular shadowing and ground-glass opacities. The signs of "crazy paving" and "CT angiogram sign" (distinct vasculature at the background of low-attenuated consolidation at the peak of contrast scanning) were found in 2 cases. CONCLUSIONS: Bronchioloalveolar carcinoma accounted for 4.76% of lung carcinoma in our series, with a female predominance. High prevalence of asymptomatic patients at presentation and unusual long course should prompt regular chest X-ray examination. The characteristics of HRCT findings are very helpful in its diagnosis and differential diagnosis.

[Treatment of paraneoplastic pemphigus with Castleman's disease].

OBJECTIVE: To discuss the clinical findings and treatment of paraneoplastic pemphigus (PNP) with Castleman's disease. METHODS: To investigate the clinical, histopathologic and CT findings of 8 cases paraneoplastic pemphigus with Castleman's disease. RESULTS: All of 8 patients were diagnosed PNP first and were found Castleman's tumor incidently during routine examination. All 8 cases showed severe erosion or ulcer of the oral mucosa with various skin lesions. Histopathologically, there were intraepidermal acantholytic vesicle, basal cell liquefaction, necrotic keratinocytes in the epidermis and lymphocyte infiltration in the upper dermis. CT scan appeared solitary mass in these patients. Some of them were attacked by bronchiolitis obliterans. All 8 patients were failed by use of predisone. Obvious relief of PNP and pulmonary lesion occurred after tumor was rescted. CONCLUSIONS: Paraneoplastic pemphigus with Castleman's disease is a rare disease. The key step is to find and resect the tumor in abdomen. CT scan should be used to detect the tumor in patients with PNP, especially, when predisone was failed in treatment.

[Histologic features and pathologic diagnosis in usual interstitial pneumonia].

OBJECTIVE: To study the pathologic features, differential diagnosis and role of open lung biopsies (OLB) in usual interstitial pneumonia (UIP). METHOD: The authors reviewed the pathologic, clinical and radiologic features of five cases of UIP (one autopsy case and four OLB cases), with follow-up information. RESULTS: The typical histologic features were a non-uniform distribution of alveolar inflammation, fibroblastic foci, interstitial fibrosis and honeycomb change. There also was associated metaplasia of bronchiolar epithelium, type II pneumocyte hyperplasia and accumulation of alveolar macrophages. CONCLUSIONS: Characteristically, UIP exhibits temporal heterogeneity under low-power light microscopy, which includes changes in both the early and end stages. Open lung biopsy is an important diagnostic adjunct for suitable patients with atypical radiologic features on computerized tomography. Correlation between clinical, radiologic and pathologic findings is also essential for a correct diagnosis.

[A clinicopathological study of Castleman's disease].

OBJECTIVE: To study the pathogenesis and the differential diagnosis of Castleman's disease. METHODS: Histopathology, immunohistochemical staining and clinical courses of 26 cases of Castleman's disease (CD) were studied with follow-up study of 16 cases. RESULTS: The present study included 6 cases of multicentric type, 20 cases of localized type in the clinical aspects and 19 cases with hyaline vascular type, 4 cases with plasma cell type, 3 cases with mixed type in the histologic aspect. The Multicentric type presented systemic lymphadenopathy, anemia, hyperglobulinemia, hepatosplenomegaly, skin changes, and lung disorder and kidney disfunction, of which 1 case died of respiratory and renal insufficiency. 13 of the 20 localized cases were of the hyaline vascular type, and with good prognosis. 7 of the 20 cases showed paraneoplastic pemphigus associated with hyperglobulinemia (4/7) and lung disease (5/7). The pathologic features composed of proliferation of the mantle zone B cell, follicular dendritic cell, plasma cell and small vessels. In immunohistochemical staining, kappa and lambda light chains were detected in each CD case. CONCLUSIONS: Many diseases are similar to CD clinicopathologically. It is important to make differential diagnosis through pathological study. Castleman's disease is a lymphoproliferative disorder. The pathogenesis of this multicentric disorder may be associated with autoimmune disease.