keyword
https://read.qxmd.com/read/38387093/treatment-of-congenital-langerhans-cell-histiocytosis-with-cobimetinib
#21
Ghita Benjelloun, Charlotte Roquet-Gravy, Liliane Marot, Léo-Paul Secco, Pierre-Paul Roquet-Gravy, Marie Baeck, Audrey Bulinckx
We report a case of congenital multisystem Langerhans cell histiocytosis with cutaneous and hematopoietic involvement. After the failure of first-line (vinblastine and prednisolone) and second-line (vincristine and cytarabine) therapies, treatment with cobimetinib, a mitogen-activated protein kinase (MEK) inhibitor, led to the remission of disease and a sustained response after 11 months of ongoing treatment. Protein kinase inhibitors targeting BRAF or MEK could represent a promising future therapeutic option, also in children with LCH...
February 22, 2024: Pediatric Dermatology
https://read.qxmd.com/read/38381107/-panhypopituitarism-diabetes-insipidus-and-bone-pain-is-there-a-systemic-disease-behind-it
#22
JOURNAL ARTICLE
Martina Bertschinger, Lisa Sze, Irene Bosma, Corina Dommann-Scherrer, Jeroen Goede
A 27-year-old man presented due to unilateral leg pain. He had a history of diabetes insipidus and panhypopituitarism. Laboratory analysis revealed hormonal undersupply. MRI showed a large contrast medium-absorbing mass in the pituitary gland extending into the hypothalamus. FDG-PET/CT examination revealed a hypermetabolic soft tissue lesion around the left femoral shaft. After biopsy of the lesion, a diagnosis of multisystemic Langerhans cell histiocytosis was made.
January 2024: Praxis
https://read.qxmd.com/read/38377389/adult-presentation-of-cutaneous-langerhans-cell-histiocytosis-18f-fdg-pet-ct-detection-of-systemic-disease
#23
JOURNAL ARTICLE
Elizabeth Robinson, Ayah Nawwar, Julie Searle, Iain Lyburn
Langerhans cell histiocytosis (LCH) is a rare hematological disease, more common in pediatric populations. A 26-year-old man with biopsy-proven cutaneous LCH underwent 18F-FDG PET/CT systemic staging. 18F-FDG PET/CT highlighted multiple sites of avid cutaneous disease within the natal cleft. Additional characteristic cystic lung disease was seen on the CT component and focal uptake in the pituitary stalk/hypothalamic region noted, a common but occult site of disease in this patient. Because of the rarity of adult-onset cutaneous LCH, guidance on management and staging is lacking...
February 8, 2024: Clinical Nuclear Medicine
https://read.qxmd.com/read/38377089/metagenomic-sequencing-expedites-diagnosis-of-disseminated-bcg-in-an-infant-with-brafv600e-mutation
#24
JOURNAL ARTICLE
Que Yang, Baojing Wu, Wenxia Wang, Ni Tan, Huarong Huang
INTRODUCTION: Disseminated bacillus Calmette-Guérin (BCG) disease is a rare but serious BCG complication in children. Early diagnosis and timely interventions are essential to improve prognosis. However, its manifestations can closely mimic those of Langerhans cell histiocytosis (LCH), which usually leads to a high rate of misdiagnoses. Herein we report the first case of successful application of biopsy tissue metagenomic next-generation sequencing (mNGS) in the differential diagnosis of disseminated BCG disease and LCH...
January 31, 2024: Journal of Infection in Developing Countries
https://read.qxmd.com/read/38374584/orbital-indeterminate-cell-histiocytosis
#25
JOURNAL ARTICLE
Angela J Oh, Connie M Sears, Akhila A Vadivelan, Brigitte N Gomperts, Noah Federman, Jonathan Said, Kelsey A Roelofs
An 8-year-old female presented to the oculoplastics clinic with 3 months of left upper eyelid fullness and edema. Examination showed a mass in the left anterior superior orbit with erythema. Imaging demonstrated a well-circumscribed superolateral orbital mass that was T1 hypointense and T2 hypo-to-iso intense with contrast enhancement. An incisional biopsy was performed via an upper lid crease incision. Histopathology showed aggregates of histiocytic cells with fibrosis and infiltration of eosinophils...
February 19, 2024: Orbit
https://read.qxmd.com/read/38373712/langerhans-cell-histiocytosis-involving-the-temporal-bone-with-destruction-and-subsequent-reossification-of-the-bony-labyrinth-boundaries
#26
JOURNAL ARTICLE
Katja Döring, Philipp Ivanyi, Heinrich Lanfermann, Athanasia Warnecke, Anja Giesemann
PURPOSE:  With an incidence between 1-9/100 000 per year, Langerhans cell histiocytosis (LCH) is a rather rare disease from the hemato-oncologic disease spectrum (Hayes et al. 2009). The tumorlike disease with proliferation of histiocytic cells may manifest as localized to one organ or disseminated with infiltration of a wide variety of organs. Approximately 25-30 % of these cases show involvement of the temporal bone (Ni et al. 2017). CASE DESCRIPTION:  With vertigo persisting for three years, chronic mastoiditis, and acute progressive hearing loss bilaterally (r > l) for three weeks, a 41-year-old woman presented at an emergency department...
February 19, 2024: RöFo: Fortschritte Auf Dem Gebiete der Röntgenstrahlen und der Nuklearmedizin
https://read.qxmd.com/read/38370392/this-mite-surprise-you-scabies-masquerading-as-langerhans-cell-histiocytosis-a-case-report
#27
Sara Al Janahi, Raghda Al Maashari, Tausif Saleem
INTRODUCTION: Scabies is a common parasitic infestation caused by the mite Sarcoptes scabiei. Scabies can mimic other entities clinically, resulting in misdiagnosis. The presence of a mite in the stratum corneum on biopsy specimens is diagnostic of scabies. However, there are instances when mites are not visible, and immunohistochemical (IHC) staining may be misleading. An example is when IHC demonstrates Cluster of Differentiation 1a and S100 positivity. The main differential diagnosis for this finding is Langerhans cell histiocytosis, a group of idiopathic disorders of bone marrow-derived Langerhans cells, with manifestations ranging from isolated to life-threatening multisystem disease...
2024: Case Reports in Dermatology
https://read.qxmd.com/read/38368348/langerhans-cell-histiocytosis-of-the-skull-in-23-children
#28
JOURNAL ARTICLE
Min Wei, Wenbin Jiang, Rui Wang, Bo Xiao, Qijia Zhan
OBJECTIVE: To explore the clinical features, diagnosis, treatment and prognosis of Langerhans cell histiocytosis (LCH) of the skull in children. METHODS: This study retrospectively summarized the clinical manifestations, treatment methods and follow-up status of children with skull LCH who were admitted to the Department of Neurosurgery of Shanghai Children's Hospital from January 2014 to June 2021. RESULTS: A total of 23 patients confirmed by histology as LCH received hospitalization treatment, including 14 males and 9 females, aged (5...
February 17, 2024: European Journal of Medical Research
https://read.qxmd.com/read/38366656/pm2-5-vegetation-density-and-childhood-cancer-a-case-control-registry-based-study-from-texas-1995-2011
#29
JOURNAL ARTICLE
Lindsay A Williams, David Haynes, Jeannette M Sample, Zhanni Lu, Ali Hossaini, Laura A McGuinn, Thanh T Hoang, Philip J Lupo, Michael E Scheurer
BACKGROUND: Air pollution is positively associated with some childhood cancers while greenness is inversely associated with some adult cancers. The interplay between air pollution and greenness in childhood cancer etiology is unclear. We estimated the association between early life air pollution and greenness exposure and childhood cancer in Texas (1995-2011). METHODS: We included 6,101 cancer cases and 109,762 controls (aged 0-16 years). We linked residential birth address to census tract annual average particulate matter ≤2...
February 15, 2024: Journal of the National Cancer Institute
https://read.qxmd.com/read/38361317/cochlear-implantation-after-head-and-neck-radiation-a-case-series-systematic-review-and-meta-analysis
#30
JOURNAL ARTICLE
Jumah G Ahmad, Benjamin D Lovin, Anna Lee, Marc-Elie Nader, Paul W Gidley
OBJECTIVE: To determine if cochlear implant (CI) is safe and effective in patients with radiation therapy (XRT)-induced sensorineural hearing loss and to discuss considerations in this population through a retrospective cohort review, systematic review, and meta-analysis. DATABASES REVIEWED: PubMed, Cochrane Library, and Embase. METHODS: We retrospectively reviewed all CI cases after head and neck (HN) XRT at our institution, noting intraoperative findings, postoperative complications, and hearing outcomes...
February 16, 2024: Otology & Neurotology
https://read.qxmd.com/read/38353855/chronic-health-conditions-after-childhood-langerhans-cell-histiocytosis-results-from-the-swiss-childhood-cancer-survivor-study
#31
JOURNAL ARTICLE
Tomáš Sláma, Luzius Mader, Maša Žarković, Reta Malär, Alexandra Schifferli, Nicolas X von der Weid, Claudia E Kuehni, Christina Schindera
PURPOSE: Langerhans cell histiocytosis (LCH) is a rare disease characterized by dysregulated proliferation of myeloid marrow progenitors and subsequent organ infiltration. While LCH is associated with a favorable prognosis, some survivors may develop chronic health conditions (CHC) because of the disease. In this study, we aimed to assess the spectrum and prevalence of CHC among LCH survivors compared with siblings and identify factors associated with the development of CHC. METHODS: The Swiss Childhood Cancer Survivor Study sent questionnaires to all ≥ 5-year LCH survivors registered in the Swiss Childhood Cancer Registry and diagnosed between 1976 and 2015...
February 14, 2024: Journal of Cancer Survivorship: Research and Practice
https://read.qxmd.com/read/38350091/coexistence-of-langerhans-cell-histiocytosis-and-ganglioneuroblastoma-revealed-by-18f-fdg-pet-ct-in-a-pediatric-patient
#32
JOURNAL ARTICLE
Yanfeng Xu, Xia Lu, Wei Wang, Jigang Yang
Coexistence of Langerhans cell histiocytosis and ganglioneuroblastoma is rare and seldom reported in the literature. A 3-year-old girl with Langerhans cell histiocytosis underwent 18F-FDG PET/CT imaging for staging, which demonstrated significant 18F-FDG accumulation in the mandibles. Unexpectedly, a mild hypermetabolic soft mass was detected in the upper retroperitoneum. Results of surgical pathology of the abdominal mass were consistent with ganglioneuroblastoma.
February 2, 2024: Clinical Nuclear Medicine
https://read.qxmd.com/read/38345277/langerhans-cell-histiocytosis-oral-lesions-in-pediatric-patients
#33
JOURNAL ARTICLE
Verónica H Pavan, Virginia Fernández de Preliasco, Melisa Ienco, Carolina Benchuya
UNLABELLED: Langerhans cell histiocytosis (LCH) is a disease with unknown etiology. It presents as single-system (affecting a single organ or tissue) or as multisystem (with or without risk organ involvement). The oral cavity may be involved or be the site of the first manifestation. AIM: To describe, group, and determine the frequency of oral lesions in pediatric patients with LCH, and to relate these lesions to age and the different disease subtypes. MATERIALS AND METHOD: Clinical and radiographic examinations were used to evaluate 95 patients diagnosed with LCH, aged 0 to 16 years, who were referred to the Department of Comprehensive Pediatric Dentistry at the School of Dentistry, University of Buenos Aires...
December 31, 2023: Acta Odontológica Latinoamericana: AOL
https://read.qxmd.com/read/38342891/identification-and-characterization-of-stromal-like-cells-with-cd207-low-cd1a-low-phenotype-derived-from-histiocytic-lesions-a-perspective-in-vitro-model-for-drug-testing
#34
JOURNAL ARTICLE
Agnieszka Śmieszek, Klaudia Marcinkowska, Zofia Małas, Mateusz Sikora, Martyna Kępska, Beata A Nowakowska, Marta Deperas, Marta Smyk, Carlos Rodriguez-Galindo, Anna Raciborska
BACKGROUND: Histiocytoses are rare disorders manifested by increased proliferation of pathogenic myeloid cells sharing histological features with macrophages or dendritic cells and accumulating in various organs, i.a., bone and skin. Pre-clinical in vitro models that could be used to determine molecular pathways of the disease are limited, hence research on histiocytoses is challenging. The current study compares cytophysiological features of progenitor, stromal-like cells derived from histiocytic lesions (sl-pHCs) of three pediatric patients with different histiocytoses types and outcomes...
February 12, 2024: BMC Cancer
https://read.qxmd.com/read/38315057/safety-and-clinical-contribution-of-computed-tomography-guided-biopsy-for-cervical-spine-lesion
#35
JOURNAL ARTICLE
Daisuke Okamoto, Yasuhiro Ushijima, Nobuhiro Fujita, Keisuke Ishimatsu, Ryo Murayama, Masahiro Itoyama, Kousei Ishigami
INTRODUCTION: The purpose is to clarify the safety and clinical contribution of computed tomography (CT)-guided percutaneous needle-biopsy for patients with cervical spine lesion. MATERIAL AND METHODS: Between June 2015 and August 2022, CT-guided percutaneous needle biopsies were performed for 15 cervical spine lesions of 15 patients (8 male, 7 female; 2-81 years old). The technical success, clinical contribution, and safety were evaluated. Technical success was defined as the completion of the biopsy procedure...
February 5, 2024: Minimally Invasive Therapy & Allied Technologies: MITAT
https://read.qxmd.com/read/38313001/pulmonary-langerhans-cell-histiocytosis-an-unusual-differential-for-a-solitary-lung-nodule
#36
Tessabella M Magliochetti Cammarata, Sushan Gupta, Sai Sri Harsha Rallabhandi, Vishesh Paul
BACKGROUND: Pulmonary Langerhans cell histiocytosis (LCH) commonly presents as bilateral reticulonodular and cystic lung changes on chest imaging. Isolated lung nodule presentation is rare. Case Presentation . Our patient was an elderly male and an active smoker, who was referred to the pulmonology clinic for an incidental 19 mm lung nodule seen on a chest CT scan. A CT-guided transthoracic needle biopsy was performed to rule out malignancy. The biopsy sample showed marked inflammatory infiltrate with abundant eosinophils and epithelioid histiocyte-like cells suggestive of Langerhans cell histiocytosis...
2024: Case Reports in Pulmonology
https://read.qxmd.com/read/38301859/ocular-and-orbital-tumors-in-childhood
#37
JOURNAL ARTICLE
Kathryn Bentivegna, Nicholas J Saba, Roman Shinder, Jane M Grant-Kels
Pediatric tumors of the eye and orbit can be benign or malignant as well as congenital or acquired and are usually distinctively different than those seen in adults. Although most of these neoplasms are benign (eg, dermoid cyst, chalazion, molluscum), their location near and within a vital organ can result in serious dermatologic and ophthalmologic sequelae. Lesions discussed include vascular lesions, retinoblastomas (the most common primary pediatric intraocular malignancy), rhabdomyosarcoma (the most common primary pediatric orbital malignancy), Langerhans cell histiocytosis, and metastatic lesions to the orbit (neuroblastoma, Ewing sarcoma)...
February 1, 2024: Clinics in Dermatology
https://read.qxmd.com/read/38288964/isolated-eyelid-neonatal-langerhans-cell-histiocytosis
#38
JOURNAL ARTICLE
Abdelrahman M Elhusseiny, Jamal O Azhari, Tom Kornhauser, David A Kilgore, David K Wilson, Kevin J Bielamowicz, Kandi A Stallings-Archer, John D Pemberton
Langerhans cell histiocytosis (LCH) is a condition characterized by clonal proliferation of the phagocytic cells derived from the bone marrow. In this article, we present an exceedingly rare case of congenital/neonatal LCH in a 3-week-old girl who initially presented with an isolated swelling of the eyelid, initially misdiagnosed as a chalazion. Subsequently, a biopsy was performed, and histopathological evaluation confirmed the diagnosis of LCH. A staging work-up revealed no evidence of multisystem involvement, and thus, local steroid injection was performed as the initial treatment for the residual lesion...
January 30, 2024: Orbit
https://read.qxmd.com/read/38284911/an-unusual-manifestation-of-langerhans-cell-histiocytosis-in-the-gastrointestinal-tract
#39
JOURNAL ARTICLE
Laura Rayón Moreno, Alberto Pérez Garvin, Bruno Bernardo, Beatriz Merino
Langerhans cell histiocytosis (LCH) is a monoclonal proliferative disease that can affect multiple organs. It is a rare disorder, and children are the most commonly affected. Its classification depends on whether the disease is localized (usually bone or skin) or systemic. We present the case of a 49-year-old woman with a previous diagnosis of LCH in 2018 with only cutaneous involvement, managed with topical corticosteroids. After developing hypertransaminasemia, a PET-CT scan was performed, showing dissemination of the disease with bone, hepatosplenic and gynecological involvement...
January 29, 2024: Revista Española de Enfermedades Digestivas
https://read.qxmd.com/read/38283438/vanishing-lung-syndrome-in-a-young-male-with-chronic-marijuana-use-a-case-report
#40
Gabriel Velez Oquendo, Nivedha Balaji, Aleksandra Ignatowicz, Hisham Qutob
Vanishing lung syndrome (VLS) also known as type I bullae disease or idiopathic bullous disease is characterized by giant emphysematous bullae that commonly develop in the upper lobes, occupying at least one-third of a hemithorax. It is a progressive and irreversible condition that involves pulmonary parenchymal destruction and alveolar dilation. It is commonly associated with middle-aged tobacco smokers, habitual marijuana users, and those with alpha-1-antitrypsin deficiency. This case involves an incarcerated male in his 30s with chronic marijuana smoking who presented with a three-month history of right-sided chest pain accompanied by cough, hemoptysis, fever, and weight loss...
December 2023: Curēus
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