From 1989 to 1994 we have been following in our Department 22 patients (12 males, 10 females, age range 1-13.5 years) affected by sJCA who underwent glucocorticoid (GC) treatment. These patients were randomised to receive for 6 months two different GC schedules: group A (12) received 6 methylprednisolone (6MP) i.v. for 3 days with 5 mg/kg/d and for an additional 3 days at 2.5 mg/kg/d followed by po prednisone (1 mg/kg/d); group B (10) received po prednisone (1 mg/kg/d). A remarkable decrease of disease activity and fever score and a prompt improvement of joint involvement were observed. A CPR serum concentration decrease associated to a consistent increase of Hb over baseline values were disclosed in group A. GC cumulative daily requirement was significantly lower in group A. Four patients from group B but only one from group A developed a cushingoid appearance: no differences of BMI were found in the two groups. Our study shows that in sJCA patients i.v. GC mini pulses are effective on early clinical and biological parameters of inflammation and requires a lower cumulative daily dosage than conventional oral GC treatment.
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http://dx.doi.org/10.3109/03009749609082663 | DOI Listing |
Innate Immun
January 2025
Department of Respiratory and Critical Medicine, the First Affiliated Hospital of Soochow University, Suzhou, China.
The application of biological therapy and glucocorticoids in Auto-immune diseases (AID) patients will cause immunocompromised host (ICH) prone to infection. And monocytes play a key role in both innate and adaptive immune responses. We aimed to investigate the changes of circulating monocyte subsets in AID or AID-ICH patients with pulmonary infection.
View Article and Find Full Text PDFRev Med Suisse
January 2025
Service d'oto-rhino-laryngologie et de chirurgie cervico-faciale, Centre hospitalier universitaire vaudois, 1011 Lausanne.
This article highlights recent advancements in otorhinolaryngology. It reviews innovations in managing sudden sensory-neural hearing loss through low-dose glucocorticoid treatments and explores pediatric audiology breakthroughs, particularly gene therapy for DFNB9 deafness. In speech pathology and oncology, a new minimally invasive robotic surgical approach enhances organ preservation for head and neck cancers.
View Article and Find Full Text PDFFront Immunol
January 2025
Department of Rheumatology & Allergology, Japanese Red Cross Medical Center, Tokyo, Japan.
Patients with A20 haploinsufficiency (HA20) presenting with central nervous system (CNS) symptoms are rare, and available reports are limited. Here, we describe a patient with HA20, previously followed up as Behçet disease, who presented with CNS symptoms in adulthood. A 38-year-old Japanese male who had been followed up for incomplete Behçet disease at another hospital since 28 years of age presented to our hospital with acute-onset diplopia and persistent hiccups that were severe enough to cause vomiting.
View Article and Find Full Text PDFFront Med (Lausanne)
January 2025
Department of Hematology, The Second Affiliated Hospital of Nanchang University, Nanchang, China.
Congenital thrombotic thrombocytopenic purpura (cTTP) is a thrombotic microangiopathy (TMA) characterized by severe hereditary ADAMTS13 (a disintegrin and metalloproteinase with thrombospondin type 1 motifs 13) deficiency caused by mutations. This rare autosomal recessive genetic disorder is often misdiagnosed as immune thrombocytopenia (ITP) or hemolytic uremic syndrome (HUS). Here, we report a 21-year-old male cTTP patient with a compound heterozygous mutation.
View Article and Find Full Text PDFActa Endocrinol (Buchar)
January 2025
Dokuz Eylül University, Faculty of Medicine, Division of Pediatric Endocrinology.
Context: Osteonecrosis (ON) is bone death caused by inadequate blood supply and its optimal management remains uncertain.
Objective: We describe the outcomes of BP (pamidronate) treatment in our patients.
Design: Data regarding clinical, laboratory, magnetic resonance imaging (MRI) studies, and bone mineral density measurements (BMD) were recorded before and one year after treatment (reevaluation).
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