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*

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

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

Hyperammonemia can be caused by several genetic inborn errors of metabolism including urea cycle defects, organic acidemias, fatty acid oxidation defects, and certain disorders of amino acid metabolism. High levels of ammonia are extremely neurotoxic, leading to astrocyte swelling, brain edema, coma, severe disability, and even death. Thus, emergency treatment for hyperammonemia must be initiated before a precise diagnosis is established. In neonates with hyperammonemia caused by an inborn error of metabolism, a few studies have suggested that peritoneal dialysis, intermittent hemodialysis, and continuous renal replacement therapy (RRT) are effective modalities for decreasing the plasma level of ammonia. In this review, we discuss the current literature related to the use of RRT for treating neonates with hyperammonemia caused by an inborn error of metabolism, including optimal prescriptions, prognosis, and outcomes. We also review the literature on new technologies and instrumentation for RRT in neonates
Ammonia ; Astrocytes ; Brain Edema ; Coma ; Diagnosis ; Edema ; Emergency Treatment ; Humans ; Hyperammonemia ; Infant, Newborn ; Metabolism ; Metabolism, Inborn Errors ; Peritoneal Dialysis ; Plasma ; Prescriptions ; Prognosis ; Renal Dialysis ; Renal Replacement Therapy ; Urea

An 8-year-old Shih Tzu, a 5-year-old Maltese, and a 10-year-old Maltese presented with conjunctival hyperemia and peripheral corneal edema. Severe conjunctival thickening with varying degrees of corneal extension was observed. Cytological examination showed many large lymphocytes with malignant changes in the conjunctiva which was consistent with findings in fine-needle aspiration samples taken from regional lymph nodes. They were diagnosed as having Stage V multicentric lymphoma. When conjunctival thickening is observed in canine patients with multicentric lymphoma, conjunctival metastasis with infiltration of neoplastic lymphoid cells should be included in the differential diagnosis.
Animals ; Biopsy, Fine-Needle ; Child ; Child, Preschool ; Conjunctiva ; Cornea ; Corneal Edema ; Diagnosis, Differential ; Dogs ; Humans ; Hyperemia ; Lymph Nodes ; Lymphocytes ; Lymphoma ; Neoplasm Metastasis ; Uvea

Subarachnoid hemorrhage (SAH) usually occurs due to aneurysmal rupture of intracranial arteries and its typical computed tomography (CT) findings are increased attenuation of cisterns and subarachnoid spaces. However, several CT findings mimicking SAH are feasible in diverse conditions. They are so-called as pseudo-SAH, and this report is a case of pseudo-SAH which is misdiagnosed as aneurysm rupture accompanied by bilateral chronic subdural hematoma (cSDH). A 42-year-old male with severe headache visited our institute. Non-contrast brain CT images showed increased attenuation on basal cistern, and cSDH on both fronto-temporo-parietal convexity with midline shifting. Trans-femoral cerebral angiography was done and we confirmed small aneurysm at right M1 portion of middle cerebral artery. Under diagnosis of SAH, we planned an operation in order to clip aneurysmal neck and remove cSDH. cSDH was removed as planned, however, there was no SAH and we also couldn't find the rupture point of aneurysm. Serial follow-up CT showed mild cumulative cSDH recurrence, but the patient was tolerant and had no neurologic deficit during hospitalization. We have checked the patient via out-patient department for 6 months, there are no significant changes in volume and density of cSDH and the patient also have no neurologic complications.
Adult ; Aneurysm ; Arteries ; Brain ; Brain Edema ; Cerebral Angiography ; Diagnosis ; Follow-Up Studies ; Headache ; Hematoma, Subdural, Chronic ; Hemorrhage ; Hospitalization ; Humans ; Intracranial Hypertension ; Male ; Middle Cerebral Artery ; Neck ; Neurologic Manifestations ; Outpatients ; Recurrence ; Rupture ; Subarachnoid Hemorrhage ; Subarachnoid Space

PURPOSE: Respiratory syncytial virus (RSV) infection can cause various neurological complications. This study aimed to investigate the RSV-associated neurologic manifestations that present with seizures. METHODS: We retrospectively reviewed the medical records of patients aged less than 15 years with laboratory-confirmed RSV infections and seizures between January 2011 and December 2016 in a regional hospital in South Korea. RESULTS: During this period, 1,193 patients with laboratory-confirmed RSV infection were identified. Of these, 35 (35 of 1,193, 2.93%; boys, 19; girls, 16; mean age: 20.8±16.6 months) presented with seizure. Febrile seizure was the most common diagnosis (27 of 35, 77.1%); simple febrile seizures in 13 patients (13 of 27, 48.1%) and complex febrile seizures in 14 (14 of 27, 51.9%). Afebrile seizures without meningitis or encephalopathy were observed in 5 patients (5 of 35, 14.3%), seizures with meningitis in 2 (2 of 35, 5.7%), and seizure with encephalopathy in 1 (1 of 35, 2.9%) patient. Lower respiratory symptoms were not observed in 8 patients. In a patient with encephalopathy, brain diffusion-weighted magnetic resonance imaging revealed transient changes in white matter, suggesting cytotoxic edema as the mechanism underlying encephalopathy. Most patients recovered with general management, and progression to epilepsy was noted in only 1 patient. CONCLUSION: Although febrile seizures are the most common type of seizure associated with RSV infection, the proportion of patients with complex febrile seizures was higher than that of those with general febrile seizures. Transient cytotoxic edema may be a pathogenic mechanism in RSV-related encephalopathy with seizures.
Brain ; Brain Diseases ; Child ; Diagnosis ; Edema ; Epilepsy ; Female ; Humans ; Korea ; Magnetic Resonance Imaging ; Medical Records ; Meningitis ; Neurologic Manifestations ; Respiratory Syncytial Viruses ; Retrospective Studies ; Seizures ; Seizures, Febrile ; White Matter

A 10-year-old intact female Jindo dog was presented with a 1-week history of conjunctival redness and ocular discharge in the left eye. There was an absence of menace response, dazzle reflex, and direct pupillary light reflex. Slit-lamp biomicroscopy revealed corneal edema, ciliary flush, and aqueous flare. Intraocular pressure was 68 mmHg. Based on the information available, a diagnosis of glaucoma and uveitis was made. Subsequent histopathologic examination showed the glaucoma was produced by the effects of goniodysgenesis, posterior synechia, and pigment dispersion in the trabecular meshwork. This is the first report of primary glaucoma caused by goniodysgenesis in Jindo dogs.
Animals ; Aqueous Humor ; Child ; Corneal Edema ; Diagnosis ; Dogs ; Female ; Glaucoma ; Humans ; Intraocular Pressure ; Reflex ; Trabecular Meshwork ; Uveitis

Choroid plexus tumors are uncommon brain tumors that primarily occur in children. Most of these tumors originate from the intraventricular area, and the most common clinicalpresentation is increased intracranial pressure. Dissemination through the cerebrospinal fluid space is the inevitable natural course of the disease. Here, we present 2 rare cases of adult choroid plexus carcinoma (CPC), each with distinct clinical presentation and progression. The first case was a 40-year-old male who presented with multiple intraventricular masses. After surgical biopsy, radiation and intrathecal chemotherapy failed to elicit any response. The patient progressed with spinal cord dissemination and expired 1 year later. The second case presented with visual disturbance, and brain MRI revealed a large ovoid juxtaventricular mass with peritumoral edema. This 49-year-old female patient underwent craniotomy for what was thought to be a high-grade glioma; however, the mass was connected to the choroid plexus at the operative field. Her pathology specimen was diagnosed as CPC, and adjuvant systemic chemotherapy was administered. She has now been free of recurrence for 10 months. The description of the presentation and progression of these rare adult-onset CPC provides insight for the diagnosis and treatment of other rare instances of choroid plexus tumors.
Adult ; Biopsy ; Brain ; Brain Neoplasms ; Cerebrospinal Fluid ; Child ; Choroid Plexus Neoplasms ; Choroid Plexus ; Choroid ; Craniotomy ; Diagnosis ; Drug Therapy ; Edema ; Female ; Fourth Ventricle ; Glioma ; Humans ; Intracranial Pressure ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Pathology ; Recurrence ; Spinal Cord