OpenMed/multicare-articles huggingface dataset:
Case report: Ruxolitinib as first-line therapy for secondary hemophagocytic lymphohistiocytosis in patients with AIDS
Authors: Xiang Liu, Xueling Zhu, Xiaotang Zhou, Yirui Xie, Dairong Xiang, Zhikai Wan, Ying Huang, Biao Zhu
Journal: Front Immunol (2022)
Keywords: acquired immune deficiency syndrome, cytokines, hemophagocytic lymphohistiocytosis, inflammation, ruxolitinib, therapeutics
MeSH terms: Acquired Immunodeficiency Syndrome, HIV Infections / complications, HIV Infections / drug therapy, Lymphohistiocytosis, Hemophagocytic / diagnosis, Lymphohistiocytosis, Hemophagocytic / drug therapy, Lymphohistiocytosis, Hemophagocytic / etiology, Case Reports, Research Support, Non-U.S. Gov't, Cytokines / metabolism, Cytotoxins / therapeutic use, Ferritins, Humans, Male, Middle Aged, ruxolitinib, Cytokines, Ferritins, Cytotoxins

Background:
Hemophagocytic lymphohistiocytosis (HLH) is a fatal immunological syndrome resulting from excessive production of inflammatory cytokines. The conventional therapies for HLH, which are based on cytotoxic agents, are not always efficacious and safe, especially in patients with severe immunodeficiency. Ruxolitinib, a strong inhibitor of Janus kinase (JAK) 1/2, has already been evaluated as salvage and first-line therapy for HLH. Despite its promising efficacy and tolerability in the treatment of secondary HLH, the efficacy and safety of ruxolitinib in HLH patients with HIV infection remain to be investigated.
Case presentation:
Two men (ages: 45 and 58 years) both presented at our hospital with a high fever. They were found to be HIV-positive with severe immunodeficiency and opportunistic infections. Their laboratory tests showed severe pancytopenia, hypofibrinogenemia, hypertriglyceridemia, and increased levels of inflammatory factors and ferritin. Hemophagocytosis was found in the bone marrow, and abdominal computed tomography or ultrasonography showed splenomegaly. Both patients were diagnosed with infection-induced HLH due to severe immunodeficiency. Given they were both highly immunocompromised, we chose ruxolitinib as a first-line treatment alternative to cytotoxic chemotherapy. Rapid remission of clinical symptoms and normalization of laboratory parameters were achieved after ruxolitinib therapy. Neither patient had any associated adverse drug reactions or other laboratory abnormalities. Both patients were eventually discharged and ruxolitinib was discontinued as their disease alleviated, and they did not show signs of relapse during the 3- and 5-month of follow-up examinations.
Conclusion:
We described two cases of AIDS-related secondary HLH treated with ruxolitinib. Our cases highlight the feasibility of using ruxolitinib as a first-line therapy in patients with HIV infection and secondary HLH. Nevertheless, the safety and efficacy of this novel treatment need to be evaluated in large clinical trials in the future.
Cardiac hydatid cyst: A rare presentation of echinococcal infection
Authors: Ata Firouzi, Mohsen Neshati Pir Borj, Alireza Alizadeh Ghavidel
Journal: J Cardiovasc Thorac Res (2019)
Keywords: cardiac echinococcosis, echinococcal infection, hydatid cyst
MeSH terms: Case Reports

A 57-year-old man presented with atypical chest pain. Transthoracic echocardiography was performed and revealed a very large and well defined intra-myocardial multicystic mass in the posterolateral and basal inferoseptal segments of left ventricle suggestive of hydatid cyst. Although the echocardiographic diagnosis was straightforward, serologic test (hydatid cyst antibody) with enzyme-linked immunosorbent assay (ELISA) was performed which was positive for echinococcal infection. Other works up showed no involvement of other organ system. Albendazol was started for him and he referred to cardiac surgeon for resection of cystic mass.
Precautionary Measures: Performing ERCP on a Patient With Juxtapapillary Duodenal Diverticula (JPDD)-Related Biliary Stone After COVID-19 Lockdown Restriction Lifted in Wuhan, China
Authors: Qian Chen, Min Yang, Guang-Quan Liao, Yu-Er Zhao, Dao-Yuan Yue, Yue Yuan, Bin Cheng, Hua Qin
Journal: Front Med (Lausanne) (2020)
Keywords: covid-19, ercp (endoscopic retrograde cholangiopancreatography), healthcare workers (hcw), personal protection equipment (ppe), post-coronavirus outbreak
MeSH terms: Case Reports

On April 8, 2020, after nearly 3 months of battling against the outbreak of COVID-19, Wuhan, where the pandemic began, began easing lockdown restrictions. However, given that asymptomatic carriers could continue to lead to transmission of COVID-19 during the very early stages, the endoscopists have taken precautions and conduct risk assessments to perform endoscopic intervention in this transition stage. Here, we have reported an urgent ERCP in a patient with acute pancreatitis secondary to JPDD-related biliary stone. Based on our experiences, the objective is to provide practical suggestions for the safe resumption of ERCP procedures in the setting of the COVID-19 pandemic with specific focus on patient risk assessment, personal protection equipment (PPE), and dress code modalities, all of which have been implemented in our hospital to reduce the risk of viral transmission.
Enalapril Influence on Arterial Stiffness in Rheumatoid Arthritis Women: A Randomized Clinical Trial
Authors: Felipe Perez-Vazquez, Magnus Bäck, Efrain Chavarria-Avila, Eduardo Gomez-Bañuelos, Carlos G Ramos-Becerra, Óscar Pizano-Martínez, Mario Salazar-Páramo, Fernando Grover-Páez, Arnulfo H Nava-Zavala, Ernesto G Cardona-Muñoz, David Cardona-Müller, Sergio Duran-Barragán, Valeria N Mera-Riofrio, Natalia Prado-Bachega, Monica Vazquez-Del Mercado
Journal: Front Med (Lausanne) (2020)
Keywords: acei, cavi, arterial stiffness, enalapril, rheumatoid arthritis
MeSH terms: Case Reports

Introduction: Cardiovascular parameters disruption can be found in patients at early stages of rheumatoid arthritis (RA). The primary endpoint of this study was the reduction of arterial stiffness in RA patients without traditional cardiovascular risk factors or previous comorbidities, measured by cardio-ankle vascular index (CAVI) through the enalapril intervention. The secondary endpoints were the enalapril influence on carotid femoral pulse wave velocity (cfPWV), carotid intima media thickness (cIMT), carotid artery distensibility (cDistensibility), Young's incremental elastic modulus (Einc)]. Materials and Methods: Fifty-three patients were enrolled in a clinical, randomized, closed-label trial. The subjects were randomly assigned into two groups: One receiving 5 mg of enalapril (27) or placebo (26), both twice a day. The drug was acquired at Victory Enterprises®. The placebo was kindly provided by the Universidad de Guadalajara (UdeG), as well as the blinding into two groups: A and B. Enalapril and placebo were packed into bottles without labeling. Clinical assessment included a structured questionnaire to gather demographic and clinical variables as well as determination of CAVI, cfPWV, cIMT, carotid artery distensibility and Einc. The whole set of evaluations were analyzed at the baseline and at the end of 12 weeks of intervention. Results: The CAVI measurement at baseline was 7.1 ± 1.4 and increased up to 7.5 ± 1.2 at the end of 12 weeks. Meanwhile, the enalapril group was as follows: 7.4 ± 1.2 and at the of intervention, reduced to 7.1 ± 0.9. A reduction in delta CAVI of 0.21 in the enalapril intervention group was found. In contrast, an increase of 0.39 was observed in the placebo group. The delta CAVI reduction was not influenced by age or peripheral systolic blood pressure (pSBP). Discussion: Enalapril seems to be effective in CAVI reduction in RA patients. The effect of enalapril intervention on arterial stiffness translated to the clinical context might be interpreted as a reduction of 6.4 years of arterial aging. Trial Registration: The protocol was approved by the Institutional Review Board with the register CI-0117 from UdeG, and 0211/18 from Hospital Civil "Dr. Juan I. Menchaca", Secretaría de Salud Jalisco: DGSP/DDI/D.INV.28/18 and retrospectively registered at ClinicalTrials.gov Protocol Registration and Results System: NCT03667131.
A Rare Case of MRSA Pericarditis with Expanding, Purulent Pericardial Effusion Leading to Uremic Kidney Failure from a Right, Necrotic Toe
Authors: Justin Brilliant, Diep Edwards, Ritu Yadav, Jana Lovell, Lena Mathews
Journal: Case Rep Cardiol (2022)
MeSH terms: Case Reports

Purulent pericarditis is an extremely rare entity with only a few reported cases so far. This condition deserves prompt diagnosis because of its significant mortality rate if left untreated. A 76-year-old man with a past medical history of coronary artery disease (CAD) with percutaneous coronary intervention (PCI) to the left anterior descending artery (LAD) and right circumflex artery (RCA), ischemic cardiomyopathy with moderately reduced ejection fraction (EF 45-50%), peripheral artery disease (PAD), COVID-19 pneumonia complicated by fibrotic lung disease (on 3 liters of home oxygen), type-2 diabetes mellitus (T2DM), hypertension (HTN), hyperlipidemia (HLD), and chronic kidney disease (CKD) stage III presented with complaints of pleuritic chest pain and shortness of breath. On hospital day 1, he was afebrile and hemodynamically stable with physical exam remarkable for bibasilar crackles and dry gangrene of his right first toe. He developed progressive altered mental status, hypotension, oliguric renal failure, and respiratory distress on hospital day 6. On exam at this time, he had an elevated jugular venous distension (JVD) of 12-14 cm water, pericardial friction rub with decreased heart sounds, and orthopnea; all were consistent with cardiac tamponade clinically. An electrocardiogram (EKG) showed new ST elevations in leads I, II, and aVL with ST depression in aVR and V1 with only mild elevation in troponin I to 0.07 ng/mL. A transthoracic echocardiogram (TTE) was done on hospital day 7 and showed a moderate sized pericardial effusion with inferior vena cava (IVC) enlargement but no atrial collapse, ventricular collapse, IVC collapse, or respiratory variation in the mitral and tricuspid inflow velocities. Blood cultures grew methicillin-resistant Staphylococcus aureus (MRSA) on hospital day 6, and he was started on intravenous (IV) vancomycin. The differential diagnosis for his enlarging pericardial effusion included purulent pericarditis, uremic pericarditis, or hemorrhagic effusion. He had urgent diagnostic and therapeutic pericardiocentesis with removal of 350 milliliters of fluid. The pericardial fluid was cloudy, tan-brown with a gram stain showing gram-positive cocci in clusters and cultures growing MRSA, which confirmed the diagnosis of purulent pericarditis secondary to MRSA infection. After the pericardiocentesis, his blood pressure, respiratory distress, and renal failure improved. The source of the bacteremia was from osteomyelitis of his gangrenous, right toe with bone biopsy growing both MRSA and Streptococcus anginosus. He underwent toe amputation for definitive source control. He was discharged on hospital day 24 with a plan to complete 6 weeks of IV vancomycin.
8q22.2q22.3 Microdeletion Syndrome Associated with Hearing Loss and Intractable Epilepsy
Authors: Alejandra Rincon, Paola Paez-Rojas, Fernando Suárez-Obando
Journal: Case Rep Genet (2019)
MeSH terms: Case Reports

8q22.2q22.3 microdeletion syndrome has been described in only seven patients. We present a new case from Colombia. The characteristics of this condition are developmental delay, microcephaly, seizures, and typical facial dysmorphism. We discuss the clinical phenotype of the patient presenting relevant findings like hearing loss and severe epilepsy and the possible relations between the phenotype and the genes involved in the microdeletion. We describe a female with developmental delay, microcephaly, epilepsy, severe short stature, impaired speech, facial dysmorphism, and congenital deafness. A minimal/maximal deletion of 5.238 Mb and 5.374Mb, respectively, at 8q22.2q22.3 was diagnosed using a genome-wide array. The clinical phenotype is similar to the others seven patients previously reported; however, the severity of epilepsy and the concomitant hearing loss is remarkable, characteristics previously observed independently in only two patients. The KCNS2 gene is located in the deleted regions (8q22.2). Therefore it is a possible candidate for explaining the complex neurologic phenotype.
SAPHO Syndrome Mimicking Infectious Spondylodiscitis and Bone Metastasis
Authors: S Biuden, K Maatallah, H Riahi, H Ferjani, M D Kaffel, W Hamdi
Journal: Case Rep Rheumatol (2021)
MeSH terms: Case Reports

The acronym SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) includes diseases with similar osteoarticular manifestations and skin conditions. Making this diagnosis is not always obvious, especially when the clinical presentation does not fit the typical pattern of the disease or it occurs in a particular field. We described three cases where the diagnosis was difficult. A 46 year-old woman presented with cervical pain. The cervical X-ray showed the aspect of an ivory C5 vertebra. The patient had, however, preserved general condition, no signs of underlying neoplasia, nor other joint complaints. Blood analysis was normal. Tomography did not find any suspect lesion but showed sclerosis and hyperostosis of the manubrium. Scintigraphy showed the characteristic "bullhead" appearance. A 61-year-old woman had thoracic and lumbar pain. MRI showed spondylodiscitis in D3-D4, D4-D5, D5-D6, D6-D7, and L1-L2 with paraspinal soft tissue involvement, simulating infectious spondylodiscitis. Infectious investigations and discovertebral biopsy performed twice were negative. SAPHO syndrome was then suspected. Bone scintigraphy showed uptake in the chondrosternal articulations and D4 to D7 vertebrae. The diagnosis of SAPHO was established. The third case was a 46-year-old man with a lung adenocarcinoma. Staging for metastatic disease, a TAP tomography was performed and showed osteosclerosis of D8 to D12 and intra-articular bridges in the sacroiliac joints. MRI and scintigraphy eliminated malignancy and confirmed the diagnosis of SAPHO. In our cases, imaging findings could facilitate differentiating SAPHO syndrome from other diseases.
Mutation in the Prothrombin Gene G20210A as a Cause of Cerebral Venous Thrombosis
Authors: Jorge A Arroyave, Jairo Quiñones
Journal: Case Rep Med (2012)
MeSH terms: Case Reports

Introduction. Cerebral venous sinus thrombosis (CVST) is a rare form of cerebrovascular disease, which may manifest clinically by a wide variety of signs and symptoms. It has been associated with multiple risk factors including genetic or acquired blood disorders, infections, and trauma. Case Report. Man of 17 years who presented with 10 days of intense global headache with nausea and vomiting and subsequent onset of mild hemiparesis and hypoesthesia in right hemibody. Studies show venous thrombosis of the superior longitudinal sinus. It was identified a gene mutation in prothrombin G20210A as a probable cause of the thrombosis. Conclusions. Substitution of guanine for adenine at nucleotide 20210 in the coding region of the prothrombin gene is the second most common primary thrombophilia. Multiple cases of CVST have been associated with this mutation. In the presence of CVST must be considered the primary studies for thrombophilia gene mutations, including prothrombin G20210A.
Sinobronchial allergic mycosis syndrome in an elderly male
Authors: Eisuke Mochizuki, Shun Matsuura, Tsutomu Kubota, Yasutaka Mochizuka, Kyohei Oishi, Hyogo Naoi, Masahiro Uehara, Shinichiro Mikura, Miyuki Nagaoka, Masaru Tsukui, Naoki Koshimizu, Ichirota Nameki
Journal: Allergy Asthma Clin Immunol (2019)
MeSH terms: Case Reports

Background:
Allergic bronchopulmonary aspergillosis (ABPA) and allergic fungal rhinosinusitis (AFRS) are characterized by hyper-responsiveness of the respiratory tract and the nasal cavity and paranasal sinuses, respectively to Aspergillus species and AFRS causes chronic rhinosinusitis. Herein, we report the first case of sinobronchial allergic mycosis (SAM) syndrome, defined as ABPA with concomitant AFRS, caused by Aspergillus fumigatus patient > 80 years.
Case presentation:
An 82-year-old male with interstitial pneumonia who returned for follow-up exhibited high-attenuation mucus plug in the right intermediate bronchial trunk, infiltration in the right lung field, and right pleural effusion on regular chest computed tomography (CT). We found unilateral central bronchiectasis in the right upper lobe. Similarly, CT scan of the paranasal sinuses revealed high-attenuation mucus plugs in left ethmoid sinuses. Biopsy specimens from the plugs in the right intermediate bronchial trunk and the left ethmoid sinuses revealed allergic mucin with layers of mucus eosinophils, eosinophil-predominant mixed inflammatory cell infiltrate and Aspergillus hyphae. The patient fulfilled all the major criteria for ABPA and AFRS, and was diagnosed with SAM syndrome. CT scan of the lung and paranasal sinuses revealed apparent amelioration after oral steroid therapy.
Conclusion:
Despite mostly reported in relatively young patients, SAM syndrome can occur in elderly individuals as well.
Orbital Pseudotumor as an Extrahepatic Complication of Chronic HCV Infection
Authors: Owen Huang, Tina Shah, Divya Sundarapandiyan, Matthew J Akiyama
Journal: Case Rep Infect Dis (2018)
MeSH terms: Case Reports

We present a patient who was admitted for eye swelling, pain, and discharge, with CT orbits with contrast demonstrating inflammation and enlargement of the lacrimal glands and surrounding tissue. He was found to have an HCV infection for unknown duration upon further investigation. All other workup (autoimmune, rheumatic, and infectious) was unrevealing. The patient was diagnosed with orbital pseudotumor as an extrahepatic complication of chronic HCV infection, and he was managed with prednisone which dramatically decreased his eye swelling and pain. Steroid treatment may serve as a bridge to suppress symptoms of extrahepatic manifestations, especially in ocular cases, while HCV-infected patients await treatment to eradicate their HCV infection.
Introduction of a Less Invasive Revision Rhinoplasty Using Closed Nasal Chondrotome
Authors: Amir Manafi, Zahra Sadat Hamedi, Farzad Manfai
Journal: World J Plast Surg (2019)
Keywords: closed nasal chondrotome, dorsal spur, revision rhinoplasty
MeSH terms: Case Reports

Rhinoplasty is the most common aesthetic surgical procedure in Iran. Dorsal bony and cartilaginous structures of the nose play an important role in nasal esthetics and function. Manipulation of nasal dorsum is one of the cardinal procedures during rhinoplasty operation. Some cases of primary rhinoplasty lead to various post-operative nasal imperfections. One of the most common imperfections is dorsal nasal spur which can cause dorsal irregularities. The accurate rate of imperfections relates to some factors like the surgeon's experience and his/her precision, and appropriate post-operative patient care. Alomost 15%of revision or secondary surgical manipulations are acceptable for an experienced plastic surgeon. Most of the revision rhinoplasties are due to minor deformities in nasal dorsum like cartilaginous spur or mild focal depression. We have introduced an innovative device "Closed Nasal Chondrotome" that can ease the procedure for treating of minor nasal dorsal deformities. we propose the use of closed nasal chondrotome for mild dorsal spurs and have presented the effectiveness of this device in one patient. This simple but very effective instrument can be an alternative for a revision rhinoplasty procedure in the operating room to an outpatient procedure with local anesthesia. This method has been used in one patient with the satisfactory result, permitting corrections of minor cartilaginous excess deformities, with a less invasive procedure.
Accuracy of hepatitis B disease surveillance, Gannan prefecture, Gansu province, China; 2017
Authors: Pinggui Wang, Lijie Zhang, Jian He, Guomin Zhang, Yvan Ma, Weimin Lv, Xiaoshu Zhang, Jin An
Journal: PLoS One (2022)
MeSH terms: Hepatitis B / diagnosis, Hepatitis B / epidemiology, Hepatitis B, Chronic / diagnosis, Hepatitis B, Chronic / epidemiology, Review, Research Support, Non-U.S. Gov't, Alanine Transaminase, China / epidemiology, Hepatitis B Antibodies, Hepatitis B Surface Antigens, Humans, Immunoglobulin M, Surveys and Questionnaires, Hepatitis B Antibodies, Hepatitis B Surface Antigens, Immunoglobulin M, Alanine Transaminase

Hepatitis B is a major global public health threat. According to China's National Notifiable Disease Reporting System (NNDRS), Gannan Tibetan Autonomous Prefecture (Gannan) had the highest incidence of hepatitis B in Gansu Province during 2004 to 2016. We evaluated NNDRS hepatitis B case reports from Gannan to determine accuracy of diagnosis and to understand factors associated with inaccuracy. We reviewed medical records with hepatitis B diagnosis hospitalized in seven county hospitals in Gannan between January 1, 2016 and July 31, 2017. Using national "Classification and Diagnostic Procedures for Hepatitis B," we independently reclassified the diagnoses. We determined the positive predictive value (PPV) of reported hepatitis B cases. We investigate clinicians' understanding of the diagnostic and reporting criteria for hepatitis B by questionnaire. We reviewed and re-categorized 400 inpatients reported. Sixteen cases had been reported as acute hepatitis B, but on re-categorization, none were acute hepatitis B cases. PPVs for chronic hepatitis B and unclassified hepatitis B cases were 66% and 15% respectively; 327 (82%) of the reported hepatitis B cases were inaccurately classified; 261 were carriers, 59 were reported previously, and 7 did not have hepatitis B. The actual incidence of hepatitis B in Gannan in 2016 was estimated to be 19/100,000, significantly below the reported incidence of 106/100,000. Among reported cases, 81% had been tested for Alanine aminotransferase, 52% for hepatitis C antibody, 80% with liver ultrasound, 32% for hepatitis A antibody, and 7% for hepatitis B virus (HBV) DNA. Not all cases were tested for anti-HBc IgM or hepatitis E antibody or had a liver biopsy. In the knowledge test, 56% of clinicians accurately diagnosed three simulated cases of acute hepatitis B, and 17% correctly diagnosed two simulated cases chronic hepatitis B; 22% knew that "a client with only HBsAg positivity need not be reported." The falsely high incidence in Gannan was due to diagnostic and reporting inaccuracies. We recommend that clinicians and laboratorians receive additional training in hepatitis B diagnostic criteria and reporting standards, including appropriate use of IgM anti-HBc tests. Hepatitis B surveillance data should be periodically reviewed and evaluated for accuracy.