A 3-year-old boy presented with repeated episodes of corneal erosions and ulceration and absence of tear secretion since birth. Examination under anesthesia revealed a dry ocular surface, with corneal scarring and vascularization. Computed tomography of the orbit showed complete absence of lacrimal glands bilaterally. Minor salivary gland transplantation was performed in the right eye, which was more severely affected. This procedure has been used to manage severe dry eyes but not previously for congenital alacrimia. On long-term follow-up, 5 years after surgery, we observed considerable improvement in the photophobia, corneal transparency, and neovascularization.
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http://dx.doi.org/10.1016/j.jaapos.2024.103903 | DOI Listing |
J Peripher Nerv Syst
March 2025
Referral Center for Familial Amyloid Polyneuropathy and Other Rare Peripheral Neuropathies (CERAMIC) and Department of Neurology, Bicêtre University Hospital, AP-HP, Le Kremlin Bicêtre, France.
Objective: To assess the effectiveness of labial minor salivary gland biopsy (LSGB) alone or in combination with punch skin biopsy (SB) for the detection of amyloid deposits in hereditary transthyretin amyloidosis with polyneuropathy (ATTRv-PN).
Methods: In this single-center retrospective study, Congo red staining of minimal invasive LSGB (4 mm) and SB (3 mm) was assessed in ATTRv-PN patients consecutively evaluated between 2012 and 2023.
Results: Histopathological data of 171 ATTRv-PN, including 49 early-onset p.
J Clin Med
December 2024
Radiation Oncology Department, Osakidetza, Donostia University Hospital, 20014 San Sebastian, Spain.
(1) : Salivary gland tumors (SGTs) are a rare and diverse group of neoplasms arising in the parotid, submandibular, sublingual, and minor salivary glands distributed throughout the upper aerodigestive tract. Given the rarity and complexity of MSGTs, understanding their epidemiology across diverse populations is crucial for improving diagnostic and therapeutic strategies. (2) : A retrospective analysis involving 45 patients diagnosed with malignant salivary gland tumors and treated with curative intention between 1 July 2016 and 1 July 2021 in a tertiary academic hospital was performed.
View Article and Find Full Text PDFSupport Care Cancer
January 2025
Department of Dentistry and Oral Health, Faculty of Health, Aarhus University, Aarhus, Denmark.
Purpose: This systematic review aimed to assess the updated literature for the prevention of salivary gland hypofunction and xerostomia induced by non-surgical cancer therapies.
Methods: Electronic databases of MEDLINE/PubMed, EMBASE, and Cochrane Library were searched for randomized controlled trials (RCT) that investigated interventions to prevent salivary gland hypofunction and/or xerostomia. Literature search began from the 2010 systematic review publications from the Multinational Association of Supportive Care in Cancer/International Society of Oral Oncology (MASCC/ISOO) up to February 2024.
Int J Surg Pathol
January 2025
Department of Oral Diagnosis, Piracicaba School of Dentistry, State University of Campinas (UNICAMP), Piracicaba, São Paulo, Brazil.
Adv Rheumatol
January 2025
Department of Ophthalmology, Otolaryngology, Head and Neck Surgery, Ribeirão Preto Medical School, University of São Paulo, São Paulo, Brazil.
Background: Endoplasmic reticulum stress (ERS) and the unfolded protein response (UPR) are adaptive mechanisms for conditions of high protein demand, marked by an accumulation of misfolded proteins in the endoplasmic reticulum (ER). Rheumatic autoimmune diseases (RAD) are known to be associated with chronic inflammation and an ERS state. However, the activation of UPR signaling pathways is not completely understood in Sjögren's disease (SD).
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