Objective: To document the response of two patients with severe recurrent laryngeal papillomatosis following treatment with intralesional cidofovir in conjunction with carbon-dioxide laser evaporation.
Setting: Tertiary referral centre.
Methods: Retrospective review of treatment of two patients followed up over a 12-month period.
Intervention: Microlaryngoscopy and carbon-dioxide laser evaporation of lesions followed by intralesional injection of cidofovir.
Outcome Measures: Photodocumentation and descriptive statistical representation of intervals between endoscopic treatment. The disease was staged according to severity on endoscopy.
Results: Initially, both patients showed a marked improvement of disease. However, the disease relapsed to a significant extent. Overall, there was no demonstrable change in the frequency of required endoscopies despite subjective improvement of the airway.
Conclusion: Cidofovir may be of some benefit in the management of recurrent respiratory papillomatosis, but further studies are still required.
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http://dx.doi.org/10.2310/7070.2002.34526 | DOI Listing |
Eur J Pediatr
January 2025
Pediatric Hematology and Oncology, Liv Hospital, Gaziantep, Turkey.
Unlabelled: Spondyloenchondrodysplasia (SPENCD) is a rare genetic disorder characterized with skeletal dysplasia, immune dysregulation, and neurological impairment. Patients diagnosed with SPENCD at a single pediatric hematology center were included in the study. The patients' clinical characteristics, symptoms at presentation, imaging and laboratory results, and genetic analysis results were collected retrospectively from their files.
View Article and Find Full Text PDFThe bronchopleural fistula (BPF) is a pathological passageway between the bronchus and the pleural cavity. Diagnosing and localising BPF can be challenging, and the traditional retrograde methylene blue (MB) perfusion method may fail to identify multifocal BPFs. This article reports a novel method for locating multifocal BPFs in patients undergoing concurrent empyema debridement.
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Division of Clinical and Metabolic Genetics, Department of Paediatrics, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada
Biallelic pathogenic variants in cause a fatal autosomal recessive multisystem disorder characterized by recurrent autoinflammation, hypomyelination, progressive neurodegeneration, microcephaly, failure to thrive, liver dysfunction, respiratory chain defects and accumulation of glycogen in skeletal muscle. No missense variants in have been reported to date.We report a 6-year-old boy with microcephaly, global developmental delays, lower limb spasticity with hyperreflexia, epilepsy, abnormal brain MRI, failure to thrive, recurrent fevers and transaminitis.
View Article and Find Full Text PDFPathophysiology
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Division of Anatomical Pathology, Department of Pathology, College of Medicine, University of Saskatchewan, Royal University Hospital, 103 Hospital Drive, Saskatoon, SK S7N 0W8, Canada.
: Cause of death analysis is fundamental to forensic pathology. We present the case of a 9½-year-old girl with a genetically confirmed diagnosis of Dravet syndrome who died in her sleep with no evidence of motor seizure. She also had a lifelong history of recurrent pneumonias and, along with her family, had tested positive for COVID-19 10 days before death.
View Article and Find Full Text PDFFetal Pediatr Pathol
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Lauren V. Ackerman Laboratory of Surgical Pathology, Department of Pathology and Immunology, St. Louis, MO, USA.
, a gram-negative bacillus, has varied clinical manifestations with septicemia as the most lethal. PA infection is usually regarded as opportunistic and often nosocomial. We present a case of a "healthy" pediatric patient presenting with upper respiratory symptoms who rapidly deteriorated.
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