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. Plasmodium falciparum chloroquine resistance transporter Chloroquine-primaquine-phosphate. Alcohol and hydroxychloroquine The 5-year survival rate following lung transplantation has been reported to be about 50%. In older children with milder forms of surfactant protein deficiency, corticosteroids and hydroxychloroquine may be considered. Further studies are needed to evaluate the benefits of these medications. The Surfactant Protein C gene SFTPC, ENGS00000168484 encodes a 197 aminoacid apoprotein, subsequently cleaved into the mature Surfactant Protein C SP-C, which is incorporated together with specific phospholipids and proteins into lamellar bodies in alveolar type 2 cells, then released via regulated exocytosis in the gas-liquid interface of the alveolus, playing a critical role in the modulation of lung mechanics. Subsequently, surfactant protein gene analysis revealed mutations in the ABCA3 gene. This case of ABCA3 deficient ILD describes a stable clinical course over 10 years and highlights the potential benefit of hydroxychloroquine for this condition. Pediatr Pulmonol. 2014; 9–301. 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 Hydroxychloroquine and surfactant protein C deficiency., Surfactant Protein C-associated interstitial lung disease; three. Plaquenil cost at rite aidWill plaquenil protet against shingles 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. Childhood Interstitial Lung Disease ChILD Medication.. Ten-year follow up of hydroxychloroquine treatment for ABCA3.. Hydroxychloroquine Uses, Dosage & Side Effects -. Surfactant protein C, which is expressed as a pro-protein in AEC2s, is critical for surfactant homeostasis. Mutations in SFTPC result in the production of an abnormal pro-protein that accumulates in AEC2s. Chloroquine or hydroxychloroquine has been previously reported to improve symptoms in patients with surfactant protein C dysfunction. Hydroxychloroquine 200mg twice daily was added to his regimen, and his prednisone dose has been tapered to 15mg daily over the subsequent three months. This may be the mechanism by which HCQ tend to help in chILD, especially in those cases related to surfactant protein deficiency. SP-B and SP-C are synthesized in the endoplasmic reticulum ER of alveolar type II cells as large precursor proteins, are cleaved by proteolytic enzymes and transported through Golgi apparatus to multivesicular. Surfactant protein C deficiency is characterised by deficiency of the protein and its intracellular accumulation, with subsequent local inflammation and interstitial lung disease. There is no specific treatment, but hydroxychloroquine has been reported to be effective.