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Pycnodysostosis is a rare lysosomal storage disorder characterised by short stature, craniofacial dysmorphisms, dental anomalies, and increased bone fragility due to osteoclast dysfunction caused by cathepsin K gene mutations. This case report describes a 43-year-old female pycnodysostosis patient with recurrent subtrochanteric fractures and delayed bone healing following multiple surgical interventions, including femoral osteotomy and bone grafting. Despite these efforts, bony union was not achieved. The patient underwent 39 sessions of hyperbaric oxygen therapy (HBOT), administered at 243.2 kPa for 120 minutes daily, five days per week. Post-treatment radiographs revealed significant fracture healing, with improvements continuing one month after therapy. Visual analogue pain scores decreased from 4 to 1, and quality of life (SF-36) improved. HBOT enhances tissue oxygenation, stimulating osteogenesis, neovascularization, and immune responses, while optimising osteoclast function, making it a promising treatment for pycnodysostosis-related fracture complications. Although ideal, a controlled trial of HBOT in this rare disorder is probably unachievable. Nevertheless, this report highlights HBOT as a potentially useful adjunctive treatment for enhancing healing of refractory fractures in pycnodysostosis patients.
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http://dx.doi.org/10.28920/dhm55.2.191-194 | DOI Listing |
Cytopathology
September 2025
Department of Cardiothoracic and Vascular Surgery, Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Puducherry, India.
Mediastinal masses often present acutely as medical emergencies, necessitating prompt and accurate diagnosis. Imaging-guided fine needle aspiration cytology (FNAC) plays a pivotal role in rapidly identifying rare mediastinal tumours and differentiating them from other potential aetiologies, enabling timely intervention. Primary mediastinal germ cell tumours (PMGCTs) constitute approximately 15% of adult mediastinal neoplasms.
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Division of Pediatric Surgery, Federico II University Hospital, Naples, Italy.
Paratesticular rhabdomyosarcoma (RMS) is a rare pediatric malignancy often misdiagnosed as benign conditions like epididymitis. We report a 4-year-old boy with paratesticular RMS and retroperitoneal metastasis, initially mistaken for epididymitis. The diagnostic delay resulted in disease progression, necessitating radical inguinal orchiectomy, hemiscrotectomy, and complete multimodal therapy.
View Article and Find Full Text PDFEur J Case Rep Intern Med
August 2025
Medical Subspecialities Department, Rheumatology Section, King Fahad Medical City, Riyadh, Saudi Arabia.
Unlabelled: Concurrent presentation of pulmonary nocardiosis and granulomatosis with polyangiitis (GPA) is exceptionally rare and diagnostically challenging, given the overlapping clinical and radiological features. We report a 54-year-old female with fever, cough, weight loss, and arthralgia. Chest imaging showed multiple pulmonary nodules; serology revealed positive anti-neutrophil cytoplasmic antibodies -proteinase 3, and lung biopsy demonstrated necrotizing granulomatous inflammation with Nocardia species.
View Article and Find Full Text PDFEur J Case Rep Intern Med
July 2025
Department of Medicine, John A. Burns School of Medicine, University of Hawaii, Honolulu, USA.
Background: Thrombotic thrombocytopenic purpura (TTP) is a life-threatening hematologic emergency caused by ADAMTS13 deficiency, leading to microvascular thrombosis, haemolytic anaemia, thrombocytopenia, and end-organ damage. Neurological symptoms occur in up to 90% of cases and are frequently misdiagnosed as stroke. Prompt recognition and treatment reduce the mortality rate from over 90% to 10-20%.
View Article and Find Full Text PDFEur J Case Rep Intern Med
August 2025
Department of Internal Medicine, Western Michigan University Homer Stryker M.D. School of Medicine, Kalamazoo, USA.
Unlabelled: Autoimmune haemolytic anaemia (AIHA) is caused by antibody-mediated destruction of red blood cells. There are two broad categories of AIHA: warm and cold, both categorized by the thermal reactivity of the autoantibodies. Cold agglutinin disease (CAD) occurs at temperatures below normal body temperature and primarily involves IgM antibodies.
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