OpenMed/multicare-articles huggingface dataset:
Complete Duplex of the Left Ureter with Lower Moiety Hydronephrosis Secondary to Ureteral Stone in Adult
Authors: Masresha S Dino, Alemayehu Tegegn Tefera, Kaleab Hebtemichael Gebreselassie, Sena Sefera Akkasa, Ferid Ousman Mummed
Journal: Case Rep Urol (2022)
MeSH terms: Case Reports

Ureteral duplication is a common embryologic abnormality of the kidney with an incidence rate of 0.8% in adults. However, complete duplex ureters opening independently into the urinary bladder are rarely present. We report a 35-year-old female who presented with left flank pain in the last three years. Abdominal CT scan showed left complete duplicated system with lower moiety hydroureteronephrosis and obstructed midureteral stone. The patient underwent left retroperitoneal exploration with complete excision of the hydronephrotic sac. The postoperative course was uneventful without complications. In conclusion, a complete duplex kidney with lower moiety hydronephrosis caused by mid ureteric stone is rare. The renal duplication system should be diagnosed and followed with image guidance periodically as the late diagnosis may have poor outcomes with loss of the kidney or part of it.
Liver Transplantation for Acute Liver Failure Attributed to Leptospirosis: A Report of Two Cases
Authors: Thibault Lebreton, Fréderic Aubrun, Jean-Yves Mabrut, Laurent Heyer, Camille Perrin
Journal: Case Rep Crit Care (2019)
MeSH terms: Case Reports

Background:
Leptospirosis is a zoonosis caused by pathogenic spirochetes of the genus Leptospira. Although it may be limited to nonspecific fever, leptospirosis may also be responsible for neurological symptoms or fulminant diseases such as Weil's disease. Diagnosis is challenging due to the difficulty in isolating the organism and the delays required for performing the serological test.
Case presentation:
Two cases of leptospirosis are presented here. The clinical picture differed from a real Weil's disease in the first case and from a neuro-leptospirosis in the second. However, both patients underwent liver transplantation because of the severity of the associated acute liver failure. Unfortunately, one of the cases had a fatal outcome.
Conclusion:
Antibiotic treatment for leptospirosis should not be delayed by the lack of a positive serology test for this potentially lethal disease. In the context of a history of exposure to risk factors for leptospirosis, a negative serology must be repeated 7 days to 2 weeks following the first test. Although not always present, acute liver injury may, in rare cases, require liver transplantation.
Percutaneous Management of Bioprosthetic Mitral Valve Dehiscence with Combined Valve-in-Valve Replacement and Paravalvular Leak Closure
Authors: Hani Alturkmani, Jack Xu, Adib Chaus, Srikanth Vallurupalli, Gaurav Dhar
Journal: Methodist Debakey Cardiovasc J (2022)
Keywords: mitral valve endocarditis, paravalvular leak, valve in valve, valvular dehiscence
MeSH terms: Heart Valve Prosthesis Implantation / adverse effects, Heart Valve Prosthesis Implantation / methods, Heart Valve Prosthesis / adverse effects, Mitral Valve Insufficiency / diagnostic imaging, Mitral Valve Insufficiency / etiology, Mitral Valve Insufficiency / surgery, Case Reports, Cardiac Catheterization / adverse effects, Humans, Mitral Valve / diagnostic imaging, Mitral Valve / surgery, Prosthesis Failure, Treatment Outcome

This case report describes a patient with bioprosthetic mitral valve dehiscence that resulted in severe paravalvular regurgitation and cardiogenic shock. Due to prohibitive surgical risk, valve-in-valve transcatheter mitral valve replacement was attempted but did not reduce the severity of the prosthetic paravalvular leak (PVL) severity. Subsequent percutaneous PVL closure with a ventricular septal defect occluder successfully reduced the PVL severity and led to significant clinical improvement.
Case Report: Resolution of Lichen Planus Pemphigoides as an unexpected outcome of SARS-CoV-2 infection
Authors: Valentina Ruffo di Calabria, Alice Verdelli, Lavinia Quintarelli, Alberto Corrà, Elena Biancamaria Mariotti, Cristina Aimo, Elena Del Bianco, Beatrice Bianchi, Vincenza Maio, Daniela Massi, Marzia Caproni
Journal: Front Immunol (2023)
Keywords: covid-19, lichen planus pemphigoides, sars-cov-2, autoimmunity, molecular mechanisms, viral infections
MeSH terms: Autoimmune Diseases, COVID-19, Lichen Planus, Case Reports, Humans, Pandemics, SARS-CoV-2

It is well known that viral infections play a relevant role in inducing or protecting from autoimmune diseases, thus representing a major environmental factor in the disruption of the immune system in genetically susceptible individuals. Since the beginning of the Covid-19 pandemic a great number of clinical and epidemiological studies have demonstrated that SARS-CoV-2 infection is no exception to the rule by interfering on many different levels in the normal functioning of our immune system. Even though a growing number of case series and case reports has been cited in the literature linking the infection to the new onset of autoimmune diseases, to date very little has been reported concerning a possible correlation between the virus and the clinical resolution of any kind of autoimmune pathology. Here we describe an interesting case of abrupt and unexpected resolution of Lichen planus pemphigoides mucocutaneous lesions in a fully vaccinated patient after a mildly symptomatic SARS-CoV-2 respiratory infection and we speculate on the possible underlying mechanisms correlating the two events.
Permanent Pacemaker Implantation in a Patient with Takotsubo Cardiomyopathy and Complete Atrioventricular Block
Authors: Toshihiro Terui, Masumi Iwai-Takano, Tomoyuki Watanabe
Journal: Case Rep Cardiol (2021)
MeSH terms: Case Reports

This case report presents a patient with Takotsubo cardiomyopathy (TCM) and complete atrioventricular (AV) block who was treated with permanent pacemaker implantation. A 78-year-old woman with a history of hypertension presented with a 6-month history of palpitations. On initial evaluation, her heart rate was 40 beats/minute. Electrocardiography revealed a complete AV block and T-wave inversion in these leads: I, II, aVL, aVF, and V3-6. Echocardiography showed akinesis from the midventricle to the apex and hyperkinesis on the basal segments. The patient was diagnosed with TCM and complete AV block. Because improvement of TCM may subsequently improve the AV node dysfunction associated with TCM, the patient was admitted for treatment of heart failure without pacemaker implantation. The left ventricular (LV) abnormal wall motion improved gradually; however, the AV block persisted intermittently. On hospital day 14, a pause of 5-6 seconds without LV contraction was observed, and permanent pacemaker implantation was performed. On day 92, echocardiography revealed normal LV wall motion. However, electrocardiography revealed that the pacemaker rhythm with atrial sensing and ventricular pacing remained. Although specific degree of damage that may result from AV block associated with TCM is unknown, some of these patients require pacemaker implantation, despite improvement of abnormality in LV wall motion.
Intraoral presentation of multiple malignant peripheral nerve sheath tumors associated with neurofibromatosis-1
Authors: Mahija Janardhanan, S Rakesh, Rb Vinod Kumar
Journal: J Oral Maxillofac Pathol (2011)
Keywords: malignant peripheral nerve sheath tumor, neurofibramotosis-1, oral
MeSH terms: Case Reports

Neurofibromatosis-1 (NF-1) is a relatively common autosomal dominant disease characterized by multiple cutaneous fibromatoses and café au lait spots. It is associated with the mutation of NF-1 gene, a tumor suppressor gene located on chromosome 17q11.2. Hence, it can be considered as a familial cancer predisposition syndrome in which the affected individuals are at increased risk of developing malignancies. Intraoral neurofibromas associated with NF-1 are quite common, but the occurrence of malignant peripheral nerve sheath tumor (MPNST) in the oral cavity is very rare. Oral MPNST can occur either de novo or by malignant transformation of neurofibromas or very rarely can represent a metastatic lesion. Here, we present a case of MPNST involving the maxillary region, in a patient with NF-1. Since MPNST often creates a diagnostic dilemma, histopathologic criteria for the diagnosis of MPNST are also discussed.
The Electrophysiological Features in X-Linked Charcot-Marie-Tooth Disease With Transient Central Nervous System Deficits
Authors: Qingxian Wen, Longqiao Cao, Cun Yang, Yanchen Xie
Journal: Front Neurol (2018)
Keywords: gjb1, x-linked charcot-marie-tooth disease, electrophysiological, transient, white matter

Background: Electrophysiological examination plays an important role in the diagnosis of X-linked Charcot-Marie-Tooth disease (CMTX1) with transient central nervous system deficits. However, the electrophysiological features are seldom reported. Methods: We reviewed and analyzed published reports to determine the electrophysiological features of CMTX1 patients with transient central nervous system deficits. Results: A total of 21 CMTX1 patients with transient central nervous system deficits were found in 17 published case reports/series. The age of onset ranged from 0.5 to 18 years (mean 12.02 ± 0.78 years). All patients were male. Recurrent episodes of central nervous system deficits were reported in all 21 cases and resolved in periods ranging from several minutes to 3 days. All 20 patients who had MRIs at presentation had bilaterally symmetrical abnormal T2/Diffusion signals in the white matter without enhancement of gadolinium. All subsequent MRIs showed improvement or were within normal limits. The median motor nerve conduction velocity (MNCV), motor latencies, and compound muscle action potential (CMAP) amplitude were the most commonly measurable electrophysiological parameters (85.7%). All cases that had MNCV at presentation had slower and significantly decreased MNCV compared with the normal value (34.1 ± 1.10 m/s vs. 46.8±2.05 m/s, P < 0.0001; 95% CI, -17.4 to -7.92). The average variations of MNCV in median nerve, ulnar nerve, peroneal nerve, and tibial nerve were 22.0 ± 5.96%, 27.6 ± 11.9%, 25.9 ± 4.36%, and 27.3 ± 4.30%, respectively. All cases with measured sensory nerve conduction velocity (SNCV) at presentation had slower and significantly decreased SNCV compared with the normal value (35.3 ± 1.33 m/s vs. 47.7 ± 2.40 m/s, P < 0.001; 95% CI -18.2 to -6.46). The average variations of SNCV in median nerve, ulnar nerve, and sural nerve were 19.9 ± 8.24%, 25.2 ± 7.75%, and 23.2 ± 3.95%, respectively. Conclusion: This case series serves as a reminder that electrophysiological examination should be included in the diagnosis of recurrent and episodic neurological deficit with white matter lesions. Median MNCV is a sensitive and valuable parameter to support the diagnosis of CMTX1 with transient central nervous system deficits.
Clinical features and management of posttraumatic subperiosteal hematoma of the orbit
Authors: Usha R Kim, Vipul Arora, Akash D Shah, Urvashi Solanki
Journal: Indian J Ophthalmol (2011)
MeSH terms: Hematoma / etiology, Orbit / injuries, Orbital Diseases / etiology, Periosteum, Case Reports, Accidental Falls, Adolescent, Child, Eye Movements, Female, Hematoma / diagnostic imaging, Hematoma / physiopathology, Humans, Male, Orbit / diagnostic imaging, Orbital Diseases / diagnostic imaging, Orbital Diseases / physiopathology, Tomography, X-Ray Computed, Visual Acuity

Traumatic subperiosteal hematoma (SpH) usually presents late, after the initial trauma. It is generally seen in young males. Computed tomography is the best mode of imaging and helps to rule out orbital fracture or associated subdural hematoma. We present the clinical features and management of four patients seen at the orbit clinic with SpH. Management is based on time of presentation, visual acuity and any communicating bleed. The prognosis of traumatic SpH is excellent if treated with an individualized patient approach.
Case Report: An Adult Patient With Deficiency of Adenosine Deaminase 2 Resembled Unilateral Frosted Branch Angiitis
Authors: Yufeng Xu, Yi Shan, Yin Hu, Jing Cao, Yijie Wang, Lixia Lou, Panpan Ye
Journal: Front Med (Lausanne) (2021)
Keywords: autoinflammatory and autoimmune diseases, blood coagulation factor xii, deficiency of adenosine deaminase 2, frosted branch angiitis, retina vascular disorder
MeSH terms: Case Reports

Purpose: Deficiency of adenosine deaminase 2 (DADA2) is a rare autosomal recessive systemic autoinflammatory disorder. We describe a rare case of an adult patient with DADA2 who presented with unilateral frosted branch angiitis (FBA) combined with branch retinal vein occlusion and panuveitis. Method: This paper is a clinical case report. Results: A 31-year-old male patient complained of blurred vision in his right eye for 2 days. His fundus examination showed FBA combined with branch retinal vein occlusion and panuveitis. He had a medical history of intermittent and recurrent fever, skin rash and aphthous ulcer for 5 years, and lacunar infarction for 1 month. Laboratory examinations showed hypogammaglobulinemia and mild prolonged activated partial thromboplastin time (APTT). Brain magnetic resonance imaging (MRI) revealed old lacunar infarction in the right basal ganglia and the lateral ventricle and fresh lacunar infarction in the right pons, respectively. The perivascular sheathing of FBA and macular edema were resolved after steroid administration and treatment of intravitreal anti-VEGF injection. During the period of follow-up, the patient subsequently suffered from recurrence of strokes, abnormality of coagulation function, sudden hearing loss of the left ear, and diplopia. His gene sequencing results demonstrated several deletion mutations in ADA2, and the diagnosis of DADA2 was eventually confirmed. Conclusions: FBA represents a very rare ocular feature of DADA2 and may in some cases be the presenting manifestation. Therefore, ophthalmologists need to be aware of this rare autoinflammatory disease.
Robotic right colectomy for hemorrhagic right colon cancer: a case report and review of the literature of minimally invasive urgent colectomy
Authors: Emanuele Felli, Francesco Brunetti, Mara Disabato, Chady Salloum, Daniel Azoulay, Nicola De'angelis
Journal: World J Emerg Surg (2014)
Keywords: emergency surgery, hemorrhagic colon cancer, laparoscopic surgery, minimally invasive surgery, review, robotic surgery
MeSH terms: Review

Right colon cancer rarely presents as an emergency, in which bowel occlusion and massive bleeding are the most common clinical presentations. Although there are no definite guidelines, the first line treatment for massive right colon cancer bleeding should ideally stop the bleeding using endoscopy or interventional radiology, subsequently allowing proper tumor staging and planning of a definite treatment strategy. Minimally invasive approaches for right and left colectomy have progressively increased and are widely performed in elective settings, with laparoscopy chosen in the majority of cases. Conversely, in emergent and urgent surgeries, minimally invasive techniques are rarely performed. We report a case of an 86-year-old woman who was successfully treated for massive rectal bleeding in an urgent setting by robotic surgery (da Vinci Intuitive Surgical System®). At admission, the patient had severe anemia (Hb 6 g/dL) and hemodynamic stability. A computer tomography scanner with contrast enhancement showed a right colon cancer with active bleeding; no distant metastases were found. A colonoscopy did not show any other bowel lesion, while a constant bleeding from the right pre-stenotic colon mass was temporarily arrested by endoscopic argon coagulation. A robotic right colectomy in urgent setting (within 24 hours from admission) was indicated. A three-armed robot was used with docking in the right side of the patient and a fourth trocar for the assistant surgeon. Because of the patient's poor nutritional status, a double-barreled ileocolostomy was performed. The post-operative period was uneventful. As the neoplasia was a pT3N0 adenocarcinoma, surveillance was decided after a multidisciplinary meeting, and restoration of the intestinal continuity was performed 3 months later, once good nutritional status was achieved. In addition, we reviewed the current literature on minimally invasive colectomy performed for colon carcinoma in emergent or urgent setting. No study on robotic approach was found. Seven studies evaluating the role of laparoscopic colectomy concluded that this technique is a safe and feasible option associated with lower blood loss and shorter hospital stay. It may require longer operative time, but morbidity and mortality rates appeared comparable to open colectomy. However, the surgeon's experience and the right selection of candidate patients cannot be understated.
Case Report: Acute Fulminant Cerebral Edema With Perivascular Abnormalities Related to Kawasaki Disease
Authors: Kenichi Maeda, Pin Fee Chong, Satoshi Akamine, Fumiya Yamashita, Yuya Morooka, Harushi Mori, Sooyoung Lee, Yumi Mizuno, Ryutaro Kira
Journal: Front Pediatr (2021)
Keywords: kawasaki disease, acute brain swelling, acute encephalitis, acute encephalopathy, acute fulminant cerebral edema, case report, perivascular lesions, vasogenic edema
MeSH terms: Case Reports

Introduction: Kawasaki disease (KD) is an acute systemic vasculitis in children, but 0.4% of patients with KD exhibit central nervous system involvement. Acute encephalitis and encephalopathy accompanied with KD have been reported to be mostly self-limiting complications. Case Presentation: A 2-year-old girl developed recurrent vomiting, a cluster of generalized seizures, and decreased consciousness on day 12 after the onset of KD. Magnetic resonance imaging (MRI) T2-weighted images on day 13 showed high signal intensities in bilaterally symmetrical and subcortical white matter and thalamus, and linear radial hyperintensities parallel to the cerebral vessels of the periventricular white matter. Diffuse white matter hyperintensity on the apparent diffusion coefficient map suggested vasogenic edema. Subsequently, lethal cerebral edema rapidly progressed in 8 hrs after the MRI examination. Conclusion: To our knowledge, acute fulminant cerebral edema in patients with KD has not been previously reported. We should be aware of the possibility of severe encephalitis related to KD. Furthermore, diffuse white matter vasogenic edema with perivascular abnormalities on MRI may be an alerm, potentially leading to fatal cerebral edema.
Use of Video Laryngoscope in Sedated Spontaneously Breathing Patients with Predicted Difficult Tracheal Intubation and Impossibility of Using Fibreoptic Bronchoscopy
Authors: Alba Piroli, Ida Marsili, Franco Marinangeli, Silvia Costanzi, Luca Gentili, Antonella Paladini
Journal: Case Rep Anesthesiol (2021)
MeSH terms: Case Reports

Intubation with a flexible fibrobronchoscope in an awake patient is frequently considered the technique of choice in patients with predicted difficult intubation. There are, however, situations in which the use of the fibrobronchoscope is not applicable, particularly due to problems attributable to the patient or to limited use of the instrument. In such situations, the video laryngoscope can be a useful alternative, as long as it is associated with adequate sedation of the patient. In fact, it ensures excellent viewing of the glottis, allowing for successful orotracheal intubation to be performed even in case of difficult airways, while keeping the patient spontaneously breathing throughout the procedure. From the data present in the literature, this technique seems to ensure a success rate and a safety profile similar to those obtained with the fibrobronchoscope, moreover, with greater ease of use by the anaesthesiologist. The main purpose of this work is to provide a valid and safe alternative to intubation with a fibrobronchoscope while awake in those patients with anticipated difficult airway management and in whom, for different reasons, fibrobronchoscope cannot be used.
Pembrolizumab-Induced Psoriasis in Metastatic Melanoma: Activity and Safety of Apremilast, a Case Report
Authors: Maria Anna Siciliano, Stefano Dastoli, Maria d'Apolito, Nicoletta Staropoli, Pierfrancesco Tassone, Pierosandro Tagliaferri, Vito Barbieri
Journal: Front Oncol (2020)
Keywords: apremilast, pembrolizumab, checkpoint inhibitors, cutaneous immune-related adverse events, immune-related adverse events, immunotherapy, melanoma, psoriasis
MeSH terms: Case Reports

Background:
Immune checkpoint inhibitors targeting cytotoxic T lymphocyte-associated antigen 4 (CTLA-4), programmed death-1 receptor (PD-1), and programmed death-1 receptor and its ligand (PD-L1) increased the survival of patients affected by metastatic malignant melanoma. Due to their mechanism of action, these drugs are associated with a unique toxicity profile. Indeed, immune-related adverse events (irAEs) present a wide clinical spectrum representing the Achilles' heel of immunotherapy. Overall, cutaneous toxicities are among the most common irAEs. Immunomodulatory drugs are used for the management of irAEs and can theoretically lead to tumor escape.
Case presentation:
We report the case of a 75-year-old man with metastatic melanoma receiving the anti-PD1 Pembrolizumab therapy. After 10 treatment cycles, the patient came to our clinic with itchy psoriatic manifestations widespread >30% of the body surface [12.3 Psoriasis Area and Severity Index (PASI) score] that negatively impacted on the patient's quality of life and compliance with immunotherapy. Additionally, he had no positive personal history of psoriasis. Given the severity of the cutaneous manifestations, in a multidisciplinary approach, Apremilast (an oral small molecule PDE4 inhibitor) was started. Furthermore, Pembrolizumab was interrupted for 4 weeks until the improvement of skin lesions and the disappearance of itching. Immunosuppressive methylprednisolone therapy was initiated with a dose of 16 mg/die; then, this initial dose was progressively reduced until discontinuation. After 10 months, the patient had a good general clinical condition with psoriasis complete remission. Moreover, positron emission tomography (PET) and computed tomography (CT) scans showed complete response by immune Response Evaluation Criteria in Solid Tumors (iRECIST).
Conclusion:
To the best of our knowledge, this is the first report on the safety and efficacy of Apremilast for the treatment of immunotherapy-induced psoriasis in metastatic melanoma.