Idiopathic pulmonary hemosiderosis (IPH) is an extremely rare cause of massive pulmonary hemorrhage in children. During the acute phase, death due to massive alveolar hemorrhage and subsequent severe respiratory failure. We report two cases of IPH children who developed hypoxemic respiratory failure and massive pulmonary hemorrhage. One case of a 10-year-old boy was treated with methylprednisolone pulse therapy (10mg/kg/d) for the first three days and followed by systemic steroid therapy, he successfully decannulated 10days later and discharged with a favorable quality of life. Another case of a 4year-old female child with Down's syndrome diagnosed as IPH for over one year and treated with oral corticosteroids for maintenance therapy. She sudden suffered severe hypoxemia with rapid falls in the hemoglobin level. We applied methylprednisolone pulse therapy (10mg/kg/d) for three days and other supportive therapies, the girl survived through complicated with oxygen dependence. We suggest that methylprednisolone pulse therapy provides a chance of recovery and survival for patients with IPH at the acute phase, even if accompanied by severe pulmonary hemorrhage.
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http://dx.doi.org/10.1016/j.ajem.2017.07.094 | DOI Listing |
Lupus Sci Med
January 2025
Department of Medicine, Dvision of Rheumatology, NYU Grossman School of Medicine, New York City, New York, USA.
Objective: Traditional initial treatment regimens for lupus nephritis (LN) used oral glucocorticoids (GC) in starting doses up to 1.0 mg/kg/day prednisone equivalent with or without a preceding intravenous methylprednisolone pulse. More recent management guidelines recommend lower starting oral GC doses following intravenous pulse therapy.
View Article and Find Full Text PDFClin Rheumatol
January 2025
Department of Rheumatology and Immunology, Peking University People's Hospital, 11 Xizhimen South Street, Beijing, 100044, China.
Objective: To investigate the dose effect of methylprednisolone (MP) on peripheral lymphocyte profiles in patients with systemic lupus erythematosus (SLE). This study investigated the impact of varied MP doses on peripheral lymphocyte subtypes in SLE patients.
Methods: We conducted a prospective study involving 51 SLE patients, categorized into four groups (40 mg/day, 80 mg/day, 500 mg/day, and 1000 mg/day) based on the administered MP dosage during hospitalization.
Arthritis Res Ther
January 2025
Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, the Ministry of Education Key Laboratory, Chinese Academy of Medical Sciences & Peking Union Medical College, National Clinical Research Center for Dermatologic and Immunologic Diseases, Beijing, China.
Objective: Severe gastrointestinal lesions are associated with a poor prognosis in eosinophilic granulomatosis with polyangiitis (EGPA). The goal of this study was to develop an effective predictive model for gastrointestinal lesions and to examine clinical patterns, associated factors, treatment, and outcomes of gastrointestinal lesions in EGPA.
Methods: We retrospectively enrolled 165 EGPA patients.
J Nephrol
January 2025
Department of Pediatrics, Clinical Center of Pediatric Nephrology of Henan Province, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, 450052, China.
Background: One of the most common secondary glomerular diseases in children is IgA vasculitis-associated nephritis (IgAVN). Determining the best treatment for IgAVN based on current guidelines is controversial. The purpose of this study was to evaluate the efficacy of methylprednisolone pulse therapy in Chinese children with moderate and severe IgAVN.
View Article and Find Full Text PDFFront Immunol
December 2024
Department of Neurology, Shandong Provincial Hospital, Shandong University, Jinan, Shandong, China.
Paraneoplastic cerebellar degeneration (PCD) with anti-Yo antibodies represents a rare immune-mediated paraneoplastic neurological syndrome. Its diagnosis and management remain clinically challenging. Here, we present a case of PCD with confirmed anti-Yo antibodies, validated through anti-cerebellar degeneration protein 2 (CDR2) and anti-CDR2-like antibodies detection, which demonstrated a favorable response to ofatumumab therapy.
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