OpenMed/multicare-articles huggingface dataset:
Macrophage Activation Syndrome: A Report of Two Cases and a Literature Review
Authors: Asaad Alkoht, Ibrahem Hanafi, Basheer Khalil
Journal: Case Rep Rheumatol (2017)
MeSH terms: Case Reports

Macrophage activation syndrome (MAS) is a severe, potentially fatal condition that may complicate autoimmune diseases, and it belongs to hemophagocytic lymphohistiocytosis (HLH) disorders. MAS occurs in adults and children. However, it is rare in juvenile systemic lupus erythematosus (jSLE), and it is extremely rare to be the initial presentation of jSLE. Here, we report two patients with juvenile SLE who initially presented with MAS. One of the two patients is 4 years old. This is the youngest reported patient to our knowledge.
Corrigendum: Giant, Bleeding, and Ulcerating Proliferating Trichilemmal Cyst, With Delayed Treatment Due to Coronavirus Outbreak: A Case Report and Review of the Literature
Authors: Cecilie Mullerup Kiel, Preben Homøe
Journal: Front Surg (2022)
Keywords: case report, corona, lipoma, pilar tumour, proliferating trichilemmal cyst, trichilemmal cyst
MeSH terms: Published Erratum

[This corrects the article DOI: 10.3389/fsurg.2021.680160.].
Pharmacological Hypotension as a Cause of Delirious Mania in a Patient with Bipolar Disorder
Authors: Manuel Glauco Carbone, Mario Miniati, Marly Simoncini, Beniamino Tripodi, Claudia Tagliarini, Claudia Carmassi, Liliana Dell'Osso
Journal: Case Rep Psychiatry (2017)
MeSH terms: Case Reports

Delirious mania is a severe but often underrecognized syndrome characterized by rapid onset of delirium, mania, and psychosis, not associated with a prior toxicity, physical illness, or mental disorder. We discuss the case of a delirious mania potentially triggered and maintained by a systemic hypotension induced by antihypertensive drugs. Symptoms recovered completely after the discontinuation of antihypertensive medications and the normalization of blood pressure levels.
Torticollis as Presentation for Atypical Kawasaki Disease Complicated by Giant Coronary Artery Aneurysms
Authors: Tracey Dyer, Paul Dancey, John Martin, Suryakant Shah
Journal: Case Rep Pediatr (2018)
MeSH terms: Case Reports

Kawasaki disease (KD) is an acute systemic vasculitis of childhood. The diagnosis can be made in a patient who presents with a prolonged high fever and meeting at least four of five criteria including polymorphous rash, mucosal changes, extremity changes (including swelling and/or palmar and plantar erythema), bilateral nonsuppurative conjunctivitis, and unilateral cervical lymphadenopathy. Atypical KD refers to patients who have not met the full criteria and in whom atypical features may be present. We discuss a case of a 6-year-old male who presented to the Emergency Department with torticollis. A series of investigations for elevated inflammatory markers revealed dilated coronary artery aneurysms on echocardiogram, and thus he was diagnosed with atypical KD. His only other criteria were bilateral nonsuppurative conjunctivitis and a prior brief febrile illness. He was treated with high-dose intravenous immune globulin (IVIG) and low-dose aspirin. Low-molecular-weight heparin and atenolol were added due to the presence of giant aneurysms.
Pediatric nasal lobular capillary hemangioma
Authors: Jordan M Virbalas, John P Bent, Sanjay R Parikh
Journal: Case Rep Med (2012)

Background. LCH is a benign vascular growth of the skin and mucous membranes commonly affecting the head and neck. Since it was first described in the nineteenth century, this entity has been variously known as "human botryomycosis" and "pyogenic granuloma." The shifting nomenclature reflects an evolving understanding of the underlying pathogenesis. We review the histopathology of and current epidemiological data pertaining to LCH which suggests that the development of these lesions may involve a hyperactive inflammatory response influenced by endocrine factors. We report two new cases of pediatric lobular capillary hemangioma (LCH) of the nasal cavity and review current theories regarding the etiology, diagnosis, and treatment of nasal LCH. Methods. Retrospective case series. Case Series. Two adolescent females presented with symptoms of recurrent epistaxis, nasal obstruction, and epiphora. Both patients underwent computed tomography imaging and biopsy of their intranasal mass. The tumors were excised using image-guided transnasal endoscopic technique. Seven other cases of nasal LCH have been reported to date in the pediatric population. Conclusion. Nasal LCH is a rare cause of an intranasal mass and is associated with unilateral epistaxis, nasal obstruction, and epiphora. We advocate for image-guided endoscopic excision of LCH in the adolescent population.
Standard treatment-refractory cytomegalovirus encephalitis unmasked by immune reconstitution inflammatory syndrome and successfully treated with virus-specific hyperimmune globulin
Authors: Natalia Maximova, Annalisa Marcuzzi, Irene Del Rizzo, Davide Zanon, Alessandra Maestro, Egidio Barbi, Roberto Sala
Journal: Clin Transl Immunology (2020)
Keywords: cmv‐hyperimmune globulin, il‐6, cerebrospinal fluid cmv antibodies, cytomegalovirus (cmv)‐related encephalitis, immune reconstitution inflammatory syndrome (iris)
MeSH terms: Case Reports

Objectives:
Cytomegalovirus (CMV)-related encephalitis is a rare but potentially life-threatening complication of CMV infection in immunocompromised patients. The high mortality rate is associated with deficient immune system reconstitution after hematopoietic stem cell transplant (HSCT) and poor bioavailability of antiviral drugs in cerebrospinal fluid (CSF). CMV-related central nervous system (CNS) infection may occur with aspecific symptoms, without evidence of either blood viral load or magnetic resonance imaging (MRI) signs of encephalitis.
Methods:
Here, we describe a 10-year-old girl who underwent an allogeneic HSCT and subsequently developed CMV encephalitis. Because of the absence of CMV antigen in the blood, the diagnosis of encephalitis was proposed only after a delay, following the onset of immune reconstitution inflammatory syndrome (IRIS). Two months of combined dual antiviral therapy with ganciclovir and foscarnet proved ineffective against CMV and caused significant bone marrow and renal toxicity. To avoid further toxicity, the girl was given daily treatment with CMV-hyperimmune globulins alone.
Results:
After three weeks, the CSF viral load dropped significantly and was undetectable within three more weeks. In the meantime, the renal impairment resolved, and there was a complete bone marrow recovery.
Conclusion:
We suggest that this patient succeeded in achieving CMV CSF clearance with high dose of CMV-hyperimmune globulin, given alone, because of the ability of immunoglobulins to penetrate the blood-brain barrier (BBB).
Treating Heart Inflammation With Interleukin-1 Blockade in a Case of Erdheim-Chester Disease
Authors: Alessandro Tomelleri, Giulio Cavalli, Giacomo De Luca, Corrado Campochiaro, Teresa D'Aliberti, Moreno Tresoldi, Lorenzo Dagna
Journal: Front Immunol (2018)
Keywords: erdheim–chester disease, anakinra, cytokines, inflammation, interleukin-1, pericarditis
MeSH terms: Erdheim-Chester Disease / complications, Interleukin 1 Receptor Antagonist Protein / therapeutic use, Interleukin-1 / antagonists & inhibitors, Myocarditis / drug therapy, Myocarditis / etiology, Case Reports, Echocardiography / methods, Erdheim-Chester Disease / diagnosis, Humans, Interleukin-1 / metabolism, Male, Middle Aged, Myocarditis / diagnosis, Pericardial Effusion / diagnosis, Pericardial Effusion / drug therapy, Pericardial Effusion / etiology, Pericarditis / diagnosis, Pericarditis / drug therapy, Pericarditis / etiology, Radionuclide Imaging, Receptors, Interleukin-1 / antagonists & inhibitors, Tomography, X-Ray Computed / methods, Treatment Outcome, Interleukin 1 Receptor Antagonist Protein, Interleukin-1, Receptors, Interleukin-1

Pericarditis is an inflammatory heart disease, which may be idiopathic or secondary to autoimmune or auto-inflammatory diseases and often leads to severe or life-threatening complications. Colchicine and non-steroidal anti-inflammatory drugs represent the mainstay of treatment, whereas use of corticosteroids is associated with recurrence of disease flares. While effective and safe anti-inflammatory therapies remain an unmet clinical need, emerging clinical and experimental evidence points at a promising role of inhibition of the pro-inflammatory cytokine interleukin-1 (IL-1). We thus evaluated treatment with the IL-1 receptor antagonist anakinra in a case of extremely severe pericarditis with cardiac tamponade and heart failure secondary to Erdheim-Chester disease (ECD), a rare clonal disorder of macrophages characterized by rampant inflammation and multiorgan involvement. A 62-year-old man was admitted to the Emergency Department with severe pericardial effusion requiring the creation of a pleuro-pericardial window. A whole-body contrast-enhanced computed tomography pointed at a diagnosis of ECD with involvement of the heart and pericardium and of the retroperitoneal space. Over the following days, an echocardiography revealed a closure of the pleuro-pericardial window and a relapse of the pericardial effusion. Treatment with anakinra, the recombinant form of the naturally occurring IL-1 receptor antagonist, was started at a standard subcutaneous dose of 100 mg/day. After 2 days, we observed a dramatic clinical improvement, an abrupt reduction of the inflammatory markers, and a reabsorption of the pericardial effusion. Anakinra was maintained as monotherapy, and the patient remained asymptomatic in the absence of disease flares for the following year. Recent studies point at inhibition of IL-1 activity as an attractive treatment option for patients with refractory idiopathic recurrent pericarditis. Anakinra treatment may also have a role in patients with pericarditis in the setting of systemic inflammatory disorders, such as ECD.
A Case of Neuropsychiatric Systemic Lupus Erythematosus With Hair Loss as the First Diagnostic Symptom
Authors: Xun He, Xi-Ling Duan, Jing-Song Liu
Journal: Front Psychiatry (2022)
Keywords: case report, diagnosis, hair loss, neuropsychiatric systemic lupus erythematosus, symptom
MeSH terms: Case Reports

Neuropsychiatric systemic lupus erythematosus is a serious complication of systemic lupus erythematosus. A 33-year-old female patient had repeated hair loss for more than 3 years. A dermatologic examination showed several pieces of irregularly shaped hair loss patterns in the center of the patient's scalp. The systemic treatment included oral hydroxychloroquine, aspirin enteric-coated tablets and prednisone, and intrathecal injection of dexamethasone and methotrexate. The local treatment included intralesional injection of triamcinolone acetonide and lidocaine in the lesion area, 0.1% tacrolimus ointment for external use. After 2-month treatment, hair regrew in a non-scarring patchy alopecia region with no further hair loss.
Coats' Disease-Related Macular Edema Treated with Combined Aflibercept and Laser Photocoagulation
Authors: Wen-Shi Shieh, Gaurav K Shah, Kevin J Blinder
Journal: Case Rep Ophthalmol Med (2017)
MeSH terms: Case Reports

Purpose:
To describe the clinical response of refractory macular edema associated with Coats' disease following treatment with aflibercept and laser photocoagulation.
Methods:
Case report.
Results:
A 17-year-old female presented with decreased vision of the left eye. Ophthalmic exam demonstrated intraretinal hemorrhages and exudation with associated edema centrally. Angiographic evaluation revealed central leaking microaneurysms and peripheral capillary dropout. These findings and a systemic work-up that yielded an incidental Factor V Leiden mutation lead to a diagnosis of Coats' disease. Initial treatment consisted of laser photocoagulation and intravitreal bevacizumab but with poor response. Switching to intravitreal aflibercept resulted in resolution of the refractory macular edema and improvement of visual acuity to 20/25 in the left eye.
Conclusion:
We describe a case of refractory macular edema which responded more favorably to intravitreal aflibercept compared with bevacizumab when combined with laser photocoagulation in a patient with Coats' disease.
Syncope caused by huge hiatal hernia
Authors: Gabriel Vanerio
Journal: Case Rep Cardiol (2011)
MeSH terms: Case Reports

A 84-year-old white female had a brief loss of consciousness while playing bridge. A few minutes before the episode she had eaten pizza and significant amount of carbonated soft drinks. After recovery, her friends noticed that she was alert, but pale and sweating. Upon arrival at the emergency room, sitting blood pressure was 160/60 mmHg with a normal sinus rhythm. A chest X-Ray was performed, which was essential to make the diagnosis. The X-Ray showed a large retrocardiac opacity with air and liquid level compatible with a giant hiatus hernia. After a copious snack the hiatal hernia compressed the left atrium, decreasing the left cardiac output, elucidating the mechanism of the syncopal episode. In patients presenting with swallow syncope (particularly after a copious meal, validating the importance of a careful history), a chest X-Ray should be always be performed.
Case Report: Successful Treatment of a Child With COVID-19 Reinfection-Induced Fulminant Myocarditis by Cytokine-Adsorbing oXiris® Hemofilter Continuous Veno-Venous Hemofiltration and Extracorporeal Membrane Oxygenation
Authors: Phuc H Phan, Dung T Nguyen, Nam H Dao, Ha T T Nguyen, An V Vu, Son T Hoang, Lam V Nguyen, Tung V Cao, Dien M Tran
Journal: Front Pediatr (2022)
Keywords: covid-19, ecmo, case report, fulminant myocarditis, oxiris® hemofilter
MeSH terms: Case Reports

Background:
Indirect cardiomyocyte damage-related hyperinflammatory response is one of the key mechanisms in COVID-19-induced fulminant myocarditis. In addition to the clinical benefit of using cytokines absorption hemofiltration, the effectiveness of instituting veno-arterial extracorporeal membrane oxygenation (VA-ECMO) support for cardiac compromise has been reported. However, current literature enunciates a paucity of available data on the effectiveness of these novel modalities.
Case presentation:
We reported a 9-year-old boy with recurrent COVID-19 infection-causing fulminant myocarditis, who was treated successfully by using novel modalities of oXiris ® hemofilter continuous venovenous hemofiltration (CVVH) and VA-ECMO. The patient made a full recovery without any sequelae.
Conclusion:
We conclude that the novel highly-absorptive hemofilter CVVH and VA-ECMO may be effective treatment modalities in managing SARS-CoV-2-induced fulminant myocarditis. Our report highlights the need for further well-designed investigations to confirm this extrapolation.
Acute Antibody-Mediated Rejection in Presence of MICA-DSA and Successful Renal Re-Transplant with Negative-MICA Virtual Crossmatch
Authors: Yingzi Ming, Juan Hu, Qizhi Luo, Xiang Ding, Weiguang Luo, Quan Zhuang, Yizhou Zou
Journal: PLoS One (2015)
MeSH terms: Graft Rejection / blood, Histocompatibility Antigens Class I / blood, Histocompatibility Testing, Isoantibodies / blood, Kidney Transplantation, Case Reports, Clinical Trial, Research Support, Non-U.S. Gov't, Adult, Female, Graft Rejection / immunology, Histocompatibility Antigens Class I / immunology, Humans, Isoantibodies / immunology, Male, Middle Aged, Histocompatibility Antigens Class I, Isoantibodies, MHC class I-related chain A

The presence of donor-specific alloantibodies (DSAs) against the MICA antigen results in high risk for antibody-mediated rejection (AMR) of a transplanted kidney, especially in patients receiving a re-transplant. We describe the incidence of acute C4d+ AMR in a patient who had received a first kidney transplant with a zero HLA antigen mismatch. Retrospective analysis of post-transplant T and B cell crossmatches were negative, but a high level of MICA alloantibody was detected in sera collected both before and after transplant. The DSA against the first allograft mismatched MICA*018 was in the recipient. Flow cytometry and cytotoxicity tests with five samples of freshly isolated human umbilical vein endothelial cells demonstrated the alloantibody nature of patient's MICA-DSA. Prior to the second transplant, a MICA virtual crossmatch and T and B cell crossmatches were used to identify a suitable donor. The patient received a second kidney transplant, and allograft was functioning well at one-year follow-up. Our study indicates that MICA virtual crossmatch is important in selection of a kidney donor if the recipient has been sensitized with MICA antigens.
Hypopituitarism as consequence of late neonatal infection by Group B streptococcus: a case report
Authors: Amanda Santana Ferreira, Ana Lourdes Lima Araújo Fernandes, Guilherme Guaragna-Filho
Journal: Pan Afr Med J (2015)
Keywords: group b streptococcus, hypopituitarism, meningitis, streptococcus agalactiae
MeSH terms: Hypopituitarism / etiology, Meningitis, Bacterial / complications, Streptococcal Infections / complications, Case Reports, Female, Humans, Hypopituitarism / microbiology, Infant, Newborn, Infectious Disease Transmission, Vertical, Male, Meningitis, Bacterial / microbiology, Pregnancy, Pregnancy Complications, Infectious / microbiology, Streptococcal Infections / microbiology, Streptococcal Infections / transmission, Streptococcus agalactiae / isolation & purification

Hypopituitarism is a condition characterized by dysfunction of the pituitary gland hormone production. The insults of the perinatal period, which includes the late infection by Group B Streptococcus, consists in a rare etiology of this condition. We present the case of a 39-days-old infant with meningitis caused by Streptococcus Group B, which showed, among other consequences, hypopituitarism.
Glandular Odontogenic Cyst in Dentigerous Relationship: An Uncommon Case Report
Authors: Jean Carlos Barbosa Ferreira, Eneida Franco Vêncio, Rodrigo Tavares de Sá, Giovanni Gasperini
Journal: Case Rep Dent (2019)
MeSH terms: Case Reports

Glandular odontogenic cyst (GOC) is an uncommon cyst of the jaw. Less than 200 cases are reported in the literature, and only 22 cases are associated with an unerupted tooth (dentigerous relationship). Although it is an asymptomatic lesion, it can be destructive and has high recurrence rates. The diagnosis can be especially challenging due to the lack of distinct diagnostic clinic-radiological criteria and nonspecific microscopic features, mimicking benign and malignant lesions. Conservative surgical treatment has been the choice for most surgeons, but marginal or partial jaw resection has been reported. This report describes a rare case of GOC in a dentigerous relationship, which was treated with enucleation and peripheral osteotomy.
Idiopathic retinal vasculitis, arteriolar macroaneurysms and neuroretinitis: clinical course and treatment
Authors: Alexander Rouvas, Eleni Nikita, Nikos Markomichelakis, Panagiotis Theodossiadis, Nikolaos Pharmakakis
Journal: J Ophthalmic Inflamm Infect (2013)

Background:
The purpose of the study is to describe the clinical course and treatment of idiopathic retinitis, vasculitis, aneurysms and neuroretinitis. The study utilized non-randomized, retrospective and interventional case series. The eight eyes of six patients were analysed. Testing included wide fluorescein angiography, indocyanine green angiography and systemic evaluation. Treatment involved observation, panretinal laser photocoagulation (PRP) for peripheral retinal ischemia, grid laser for macular oedema and focal laser on the macroaneurysms. The main outcome measures were initial visual acuity (VA), initial stage at diagnosis, clinical course, surgical intervention, final VA, final stage and complications of disease.
Results:
Five out of eight eyes with retinal ischemia in more than two quadrants that were treated with PRP and grid laser for macular oedema maintained excellent VA and demonstrated no progression of retinal ischemia during follow-up. The two eyes which exhibited retinal ischemia in less than two quadrants and macular oedema were treated with grid laser and focal laser on the macroaneurysms, but did not undergo PRP. VA improved by two lines of the Snellen chart, and there was no progression of retinal ischemia during the 3 and 4 years of follow-up. One eye with neither retinal ischemia nor macular oedema was not treated, and the clinical picture remained stable during the follow-up.
Conclusion:
Early PRP may be considered in the presence of angiographic evidence of peripheral retinal non-perfusion. However, treatment could be withheld until the patient develops retinal ischemia in more than two quadrants.
Recurrent urinary bladder paraganglioma
Authors: Ali A Al-Zahrani
Journal: Adv Urol (2010)
MeSH terms: Case Reports

A 39-year-old male presented with recurrent attacks of painless haematuria. The patient has a history of partial cystectomy for bladder paraganglioma 10 years prior to the presentation. Imaging study and cystoscopic examination revealed a small anterior wall bladder tumor. The histological examination of the lesion confirmed that it was a urinary paraganglioma. Partial cystectomy was performed to this recurrent lesion. This case report stresses the importance of life-long follow-up of these lesions.
Recurrent primary hepatic VIPoma treated with a combination of surgical resection and loco-regional therapy
Authors: Wafa Dahmani, Sahar Nasr, Kais Maalel, Yasser Becheikh, Ahmed Tibaoui, Zainab Lajmi, Sihem Hmissa, Hanene Jaziri, Nour Elleuch, Aida Ben Slama, Wafa Ben Ameur, Mahdi Ksiaa, Ali Jmaa
Journal: Future Sci OA (2023)
Keywords: vipoma, hepatic resection, neuroendocrine tumor, transarterial chemoembolization
MeSH terms: Case Reports

Vasoactive intestinal peptide (VIP) secreting tumors (VIPomas) are insidious functional neuroendocrine tumors originating mainly from pancreatic islet cells. Hepatic localization is considered exceedingly rare as only few cases have been reported in the literature. Diagnostic and therapeutic management of this tumor is still not clearly codified and therefore represents a real challenge for clinicians. Herein we report a unique case of a primary hepatic VIPoma recurrence in a female patient 22 years after curative resection. The patient had two sessions of transarterial chemoembolization. Complete symptomatic improvement was achieved since the first day after the first session. This case highlights that long-term follow-up for patients with hepatic VIPoma is mandatory as recurrence could occur several years after curative surgical treatment.
Cl II Malocclusion Treatment, Using the Modified Twin Block Appliance Coordinated with Fixed Orthodontics in a Postmenarche Patient
Authors: Amin Aminian, Shahriar Sarvareh Azimzadeh, Elina Rahmanian
Journal: Case Rep Dent (2017)
MeSH terms: Case Reports

Functional appliances have been used for treatment of Class II patients for a long time. The main objective of therapy with functional appliances is to induce supplementary lengthening of the mandible by stimulating increased growth at the condylar cartilage. The Twin Block appliance is one of the most commonly used functional appliances. The aim of this paper is to present a case report of mandibular deficiency treatment with Twin Block appliance in a female patient whose sexual maturation (one and a half years after menarche) and cervical vertebral maturation stage indicated the end of the growth peak. The treatment started with bonding 0.022 in MBT prescription brackets on the upper arch in order to align upper teeth and create a symmetric overjet. When reaching alignment, a modified Twin Block was given to the patient for 8 months. Final coordination was achieved with fixed appliances in both arches. At the end of the treatment, profile of the patient improved, crowding was relieved, and Cl I relationship with normal overjet and overbite was achieved.