No clinical trials of medications for ch ILD have been conducted to date. Corticosteroids have been the mainstay of therapy in most children and adults with interstitial lung disease (ILD), despite little conclusive evidence of their efficacy. Cost of plaquenil versus hydroxychloroquine Chloroquine diphosphate mechanism of action Feb 21, 2020 In addition to its anti-inflammatory properties, hydroxychloroquine is thought to inhibit intracellular processing of the precursor of surfactant protein C SP-C, which may be the mechanism of action in SP-C deficiency. Hydroxychloroquine sulfate 200 mg equivalent to 155 mg hydroxychloroquine base and 250 mg chloroquine phosphate. The ABCA3 protein transports phospholipids into lamellar bodies where they form surfactant. The ABCA3 protein also appears to be involved in the formation of lamellar bodies. ABCA3 gene mutations, which cause a type of surfactant dysfunction sometimes referred to as ABCA3 deficiency, lead to reduction or absence of the protein's function. Without ABCA3 protein function, the transport of surfactant phospholipids is decreased. Summary. The clinical course and treatment in the first 2.5 years of life of a term-born girl with a severe onset of respiratory symptoms in the neonatal period caused by a p. Cys121Phe/C121F mutation in the gene of surfactant protein C SFTPC is described. During the first 9 months of life, she was mechanically ventilated. These agents elicit anti-inflammatory properties and cause profound and varied metabolic effects. The medications commonly used for pharmacotherapy in ch ILD and common adverse effects are reviewed below. Hydroxychloroquine surfactant protein c deficiency Successful treatment of neonatal respiratory failure., Surfactant dysfunction - Genetics Home Reference - NIH Fatcs on chloroquine In a recently published report, three infants with SP-C deficiency due to an I73T mutation and unusually early respiratory symptoms carried an additional mutation in the ATP binding cassette protein A3 gene ABCA3, a phospholipid carrier essential for lamellar body formation and surfactant intracellular assembly. Surfactant Protein C-associated interstitial lung disease.. Successful weaning from mechanical ventilation in a.. Surfactant Deficiency Causes & Reasons - Symptoma®. LPC is a known inhibitor of the lung surfactant activity and has the ability to penetrate directly into interfacial films to impair lowering of the alveolar surface tension during dynamic compression 43, 44. Elevated LPC levels in the SP-C A116Dexpressing cells could also explain. Hydroxychloroquine and Surfactant Protein C Deficiency This article has no abstract; the first 100 words appear below. To the Editor We wish to report initial success in treating a boy with. Before using hydroxychloroquine, tell your doctor if you are allergic to any drugs, or if you have psoriasis, porphyria, liver disease, alcoholism, or glucose-6-phosphate dehydrogenase G-6-PD deficiency. Call a poison control center at once and then seek emergency medical attention if you think you have used too much of this medicine.