POEMS syndrome is a rare disease caused by monoclonal plasma cell proliferative disorder.The typical signs include peripheral neuropathy,organ enlargement,endocrine disease,M proteinemia,and skin changes.In clinical practice,the atypical,complex,and changeable clinical manifestations of this syndrome can easily lead to misdiagnosis and missed diagnosis.A case of POEMS syndrome with peripheral edema and ascites as the main manifestations is reported in this paper.
Humans ; Ascites/etiology* ; POEMS Syndrome/diagnosis* ; Edema/diagnosis* ; Skin

Objective: To summarize the clinical features, management and outcome of superior vena cava syndrome (SVCS) associated with mediastinal malignancy in children. Methods: Clinical data of 42 children of SVSC associated with mediastinal malignancy in Shanghai Children's Medical Center from January 2015 to December 2021 were collected and analyzed retrospectively. The clinical manifestations, pathological diagnosis, disease diagnosis process, and prognosis were summarized. Results: Among 42 children of SVCS associated with mediastinal malignancy, there were 31 males and 11 females. The age at diagnosis was 8.5 (1.9, 14.9) years. Cough and wheezing (33 cases, 79%), orthopnea (19 cases, 45%) and facial edema (18 cases, 43%) occurred most commonly. T-cell lymphoblastic lymphoma (T-LBL) was the most frequent pathological diagnosis (25 cases, 60%), followed by T-cell acute lymphoblastic leukemia (T-ALL) (7 cases, 17%), anaplastic large cell lymphoma (4 cases, 10%) and diffuse large B-cell lymphoma (2 cases, 5%), peripheral T-lymphoma, Hodgkin lymphoma, Ewing's sarcoma and germ cell tumor (1 case each). Pathological diagnosis was confirmed by bone marrow aspiration or thoracentesis in 14 cases, peripheral lymph node biopsy in 6 cases, and mediastinal biopsy in 22 cases. Twenty-seven cases (64%) had local anesthesia. Respiratory complications due to mediastinal mass developed in 3 of 15 cases who received general anesthesia. Of the 42 cases, 27 cases had sustained remission, 1 case survived with second-line therapy after recurrence, and 14 cases died (2 cases died of perioperative complications and 12 cases died of recurrence or progression of primary disease). The follow-up time was 36.7 (1.2, 76.1) months for 27 cases in continuous complete remission. The 3-year overall survival (OS) and events free survival (EFS) rates of 42 children were 59% (95%CI 44%-79%) and 58% (95%CI 44%-77%) respectively. Conclusions: SVCS associated with mediastinal malignancy in children is a life-threatening tumor emergency with high mortality. The most common primary disease is T-LBL. The most common clinical symptoms and signs are cough, wheezing, orthopnea and facial edema. Clinical management should be based on the premise of stable critical condition and confirm the pathological diagnosis through minimal invasive operation.
Child ; China ; Cough ; Edema ; Female ; Humans ; Male ; Mediastinal Neoplasms/diagnosis* ; Precursor T-Cell Lymphoblastic Leukemia-Lymphoma ; Respiratory Sounds ; Retrospective Studies ; Superior Vena Cava Syndrome/therapy*

To investigate the computed tomography (CT) characteristics and differential diagnosis of high altitude pulmonary edema (HAPE) and COVID-19, CT findings of 52 cases of HAPE confirmed in Medical Station of Sanshili Barracks, PLA 950 Hospital from May 1, 2020 to May 30, 2020 were collected retrospectively. The size, number, location, distribution, density and morphology of the pulmonary lesions of these CT data were analyzed and compared with some already existed COVID-19 CT images which come from two files, "Radiological diagnosis of COVID-19: expert recommendation from the Chinese Society of Radiology (First edition)" and "A rapid advice guideline for the diagnosis and treatment of 2019 novel corona-virus (2019-nCoV) infected pneumonia (standard version)". The simple or multiple ground-glass opacity (GGO) lesions are located both in the HAPE and COVID-19 at the early stage, but only the thickening of interlobular septa, called "crazy paving pattern" belongs to COVID-19. At the next period, some increased cloudy shadows are located in HAPE, while lesions of COVID-19 are more likely to develop parallel to the direction of the pleura, and some of the lesions show the bronchial inflation. At the most serious stage, both the shadows in HAPE and COVID-19 become white, but the lesions of HAPE in the right lung are more serious than that of left lung. In summary, some cloudy shadows are the feature of HAPE CT image, and "crazy paving pattern" and "pleural parallel sign" belong to the COVID-19 CT, which can be used for differential diagnosis.
Altitude ; COVID-19/diagnostic imaging* ; China ; Diagnosis, Differential ; Humans ; Lung/diagnostic imaging* ; Pulmonary Edema/diagnostic imaging* ; Retrospective Studies ; Tomography, X-Ray Computed

PURPOSE: To report a case of Epstein-Barr virus-related corneal endotheliitis accompanied by secondary glaucoma.CASE SUMMARY: A 73-year-old male presented with blurred vision in his right eye. In the ophthalmic evaluation, there were dispersed keratic precipitates overlying corneal edema. The anterior chamber showed trace ~1+ graded inflammation and an endothelial density decrease. His best-corrected visual acuity and intraocular pressure in the right eye were 0.2 and 34 mmHg, respectively. Paracentesis was performed on the anterior chamber of the right eye to confirm the diagnosis under the suspicion of corneal endotheliitis with trabeculectomy for the intraocular pressure control. Epstein-Barr virus was confirmed using a multiplex polymerase chain reaction (PCR), and oral and eye drops of Acyclovir were used to treat the patient. There was no evidence of a recurrence over 2 years and his intraocular pressure was 12 mmHg and best-corrected visual acuity was maintained at 0.5.CONCLUSIONS: A case of Epstein-Barr virus-related corneal endotheliitis was diagnosed using PCR of the aqueous humor. The patient was treated with an oral antiviral agent and eyedrops without a recurrence.
Acyclovir ; Aged ; Anterior Chamber ; Aqueous Humor ; Corneal Edema ; Diagnosis ; Glaucoma ; Herpesvirus 4, Human ; Humans ; Inflammation ; Intraocular Pressure ; Male ; Multiplex Polymerase Chain Reaction ; Ophthalmic Solutions ; Paracentesis ; Polymerase Chain Reaction ; Recurrence ; Trabeculectomy ; Visual Acuity

Systemic lupus erythematosus (SLE) is a chronic autoimmune disorder of an unknown origin. The role of leptospirosis as a triggering factor for SLE is unknown. This paper reports an uncommon case of SLE following a leptospira infection. A 29-year-old female was referred due to fevers, myalgia, and facial edema with rash. Laboratory investigations revealed a hepatic dysfunction, significantly raised lactate dehydrogenase with marked leukopenia and thrombocytopenia. A diagnosis of leptospirosis was confirmed. The patient was treated with antibiotic therapy for leptospirosis. She developed dyspnea after one week. The echocardiogram revealed global hypokinesia with a decreased ejection fraction. A positivity of antinuclear, anti-DNA, and anti-Smith antibodies, together with clinical and laboratory improvement by steroid therapy, led to the diagnosis of SLE. This case highlights the presence of concurrent SLE and leptospirosis. As the symptoms of SLE are similar to leptospirosis, accurate diagnosis through high suspicion is essential for appropriate treatment.
Adult ; Antibodies ; Diagnosis ; Dyspnea ; Edema ; Exanthema ; Female ; Fever ; Humans ; Hypokinesia ; L-Lactate Dehydrogenase ; Leptospira ; Leptospirosis* ; Leukopenia ; Lupus Erythematosus, Systemic* ; Myalgia ; Myocarditis ; Thrombocytopenia

Patients undergoing thoracic surgery show various lesions such as chronic obstructive lung diseases, pleural adhesion, pneumonia, acute respiratory distress syndrome, atelectasis, pleural effusion, pulmonary edema, and pneumothorax throughout preoperative, operative, and recovery periods. Therefore, lung ultrasonography has potential for perioperative use in thoracic surgery. Benefits of lung ultrasonography over conventional chest X-ray are convincing. First, ultrasonography has higher sensitivity than X-ray in various lesions. Second, it can be performed at bed side to obtain diagnosis immediately. Third, it does not expose patients to radiologic hazard. If anesthesiologists can obtain necessary skills and perform lung ultrasonography as a routine evaluation process for patients, territory of anesthesia would become broader and patients would obtain more benefit.
Anesthesia ; Diagnosis ; Humans ; Lung Diseases, Obstructive ; Lung* ; Pleural Effusion ; Pneumonia ; Pneumothorax ; Pulmonary Atelectasis ; Pulmonary Edema ; Respiratory Distress Syndrome, Adult ; Thoracic Surgery* ; Thorax ; Ultrasonography*

BACKGROUND: The purpose of this study was to determine prognostic factors that can affect the fetal survival immediate after fetoscopic laser ablation. METHODS: The study population consisted of consecutive twin pregnant women who underwent fetoscopic laser ablation with the diagnosis of twin to twin transfusion syndrome (TTTS) from 2011 to 2018 in Seoul National University Hospital. Fetal survival immediate after procedure was defined as survival to 48 hours after procedure and neonatal survival was defined as survival to 28 days of life. Clinical characteristics and ultrasound findings were compared according to the fetal survival immediate after procedure. RESULTS: A total of 57 pregnant women with TTTS were included, and the overall fetal survival immediate after procedure was 71.1% (81/114) after fetoscopic laser ablation. Fetuses who survived immediate after procedure had higher gestational age at procedure and lower frequency of abnormal Doppler studies than those did not survive. However, the frequency of hydrops was not different between cases with fetal survival and those with fetal death. The earlier gestational age at procedure and the presence of abnormal Doppler studies were significant risk factors for fetal death even after adjustment. CONCLUSION: Based on this data, the fetal survival immediate after procedure (fetoscopic laser treatment) in TTTS can be affected by the gestational age at procedure and the presence of abnormal Doppler studies.
Diagnosis ; Edema ; Female ; Fetal Death ; Fetofetal Transfusion* ; Fetus ; Gestational Age ; Humans ; Laser Therapy* ; Pregnancy ; Pregnant Women ; Risk Factors ; Seoul ; Twins* ; Ultrasonography

Transfusion-associated circulatory overload (TACO) is recently becoming more important than transfusion-related acute lung injury (TRALI) in terms of the number of patients with definite diagnosis as well as its prognosis. In order to diagnose TACO, it is helpful to recognize early the symptoms suspicious of transfusion reaction through electronic medical record system and computer network, and this will be of help for obtaining samples for brain natriuretic peptide (BNP) measurement before and after the onset of transfusion reaction. We report a case in which a transfusion reaction was diagnosed as TACO. A 62-year-old woman was admitted to the emergency room due to bleeding tendency. Two fresh frozen plasma units and one unit of leukocyte-reduced red blood cells were transfused. Blood pressure increased during transfusion, and the chest X-ray showed findings suggestive of newly developed pulmonary edema. N-terminal prohormone of BNP (NT-proBNP) test was carried out using the specimens in refrigerated storage. Compared with the NT-proBNP level measured 12 hours before the transfusion, that measured 6 hours after the transfusion was markedly increased (>48 fold of pre-transfusion level). As a result, this case was diagnosed with TACO.
Acute Lung Injury ; Blood Pressure ; Diagnosis ; Electronic Health Records ; Emergency Service, Hospital ; Erythrocytes ; Female ; Hemorrhage ; Humans ; Middle Aged ; Natriuretic Peptide, Brain ; Plasma ; Prognosis ; Pulmonary Edema ; Thorax ; Transfusion Reaction

OBJECTIVE: To determine the frequency of chromosomal abnormalities and outcome of pregnancy for fetuses with increased nuchal translucency (NT). METHODS: Between July 2014 and February 2018, 247 fetuses with increased NT (>95th centile)were analyzed by chromosome microarray analysis (CMA). The fetuses were divided into ones with isolated increased NT (168 cases), increased NT with cystic hygroma (20 cases), increased NT with edema (12 cases) or increased NT with other abnormalities (47 cases). All couples were followed up by telephone calls. RESULTS: The rate of chromosomal abnormalities was 31.6% (78/247), which included 66 cases with chromosomal aneuploidies and 12 with copy number variants (CNVs). CNVs accounted for 31.4% (11/35) of total abnormalities among fetuses with isolated increased NT, whilst only 2.3% (1/43) of the total abnormalities among fetuses with non-isolated increased NT. Three fetuses with a normal CMA result had mental and physical retardation. Two of them were diagnosed with single gene disorders by whole exome sequencing. CONCLUSION CMA can detect more chromosomal microdeletion/microduplications among fetuses with isolated increased NT. Furthermore, fetuses with increased NT and anegative CMA result during pregnancy cannot exclude all adverse outcomes.
Aneuploidy ; Chromosome Aberrations ; Chromosomes ; DNA Copy Number Variations ; Edema ; Female ; Fetus ; Humans ; Lymphangioma, Cystic ; Microarray Analysis ; Nuchal Translucency Measurement ; Pregnancy ; Pregnancy Outcome ; Prenatal Diagnosis ; Ultrasonography, Prenatal

STUDY DESIGN: Case report.OBJECTIVES: To report a case of osteoid osteoma arising in the sacrum in a 29-year-old male patient.SUMMARY OF LITERATURE REVIEW: Osteoid osteoma is a benign osteoblastic tumor that usually arises in the long bones. Osteoid osteoma involving the sacrum is extremely rare.MATERIALS AND METHODS: A 29-year-old male patient presented with pain localized in his sacral area for 10 months. His pain was worse at night, relieved by non-steroidal anti-inflammatory drugs, and independent of physical activity. Bone scintigraphy showed increased uptake in the second sacral vertebra (S2). Computed tomography revealed a nidus located in the S2 spinous process. Magnetic resonance imaging showed bone and soft tissue edema around the nidus.RESULTS: En bloc excision including the nidus revealed a diagnosis of osteoid osteoma and provided immediate relief of the patient's long-lasting sacral pain.CONCLUSIONS: When a young patient presents with localized sacral pain that is worse at night, relieved by non-steroidal anti-inflammatory drugs, independent of physical activity, and lasts longer than expected, proper imaging studies should be performed to rule out osteoid osteoma. Although less invasive treatment modalities have been introduced, classical en bloc excision is currently the gold standard for managing osteoid osteoma.
Adult ; Diagnosis ; Edema ; Humans ; Magnetic Resonance Imaging ; Male ; Motor Activity ; Osteoblasts ; Osteoma, Osteoid ; Radionuclide Imaging ; Sacrum ; Spine