RESUMEN
Sarcoidosis is a sterile non-necrotizing granulomatous disease without known causes that can involve multiple organs with a predilection for the lung and thoracic lymph nodes. Worldwide it is estimated to affect 2-160/100,000 people and has a mortality rate over 5 years of approximately 7%. For sarcoidosis patients, the cause of death is due to sarcoid in 60% of the cases, of which up to 80% are from advanced cardiopulmonary failure (pulmonary hypertension and respiratory microbial infections) in all races except in Japan were greater than 70% of the sarcoidosis deaths are due to cardiac sarcoidosis. Scadding stages for pulmonary sarcoidosis associates with clinical outcomes. Stages I and II have radiographic remission in approximately 30%-80% of cases. Stage III only has a 10%-40% chance of resolution, while stage IV has no change of resolution. Up to 40% of pulmonary sarcoidosis patients progress to stage IV disease with lung parenchyma fibroplasia, bronchiectasis with hilar retraction and fibrocystic disease. These patients are at highest risk for the development of precapillary pulmonary hypertension, which may occur in up to 70% of these patients. Sarcoid patients with pre-capillary pulmonary hypertension can respond to targeted pulmonary arterial hypertension medications. Stage IV fibrocytic sarcoidosis with significant pulmonary physiologic impairment, >20% fibrosis on HRCT or pre-capillary pulmonary hypertension have the highest risk of mortality, which can be >40% at 5-years. First line treatment for patients who are symptomatic (cough and dyspnea) with parenchymal infiltrates and abnormal pulmonary function testing (PFT) is oral glucocorticoids, such as prednisone with a typical starting dose of 20-40 mg daily for 2 weeks to 2 months. Prednisone can be tapered over 6-18 months if symptoms, spirometry, PFTs, and radiographs improve. Prolonged prednisone may be required to stabilize disease. Patients requiring prolonged prednisone ≥10 mg/day or those with adverse effects due to glucocorticoids may be prescribed second and third line treatements. Second and third line treatments include immunosuppressive agents (e.g., methotrexate and azathioprine) and anti-tumor necrosis factor (TNF) medication; respectively. Effective treatments for advanced fibrocystic pulmonary disease are being explored. Despite different treatments, relapse rates range from 13% to 75% depending on the stage of sarcoid, number of organs involved, socioeconomic status, and geography. CONCLUSION: The mortality rate for sarcoidosis over a 5 year follow up is approximately 7%. Unfortunately, 10%-40% of patients with sarcoidosis develop progressive pulmonary disease, and >60% of deaths resulting from sarcoidosis are due to advance cardiopulmonary disease. Oral glucocorticoids are the first line treatment, while methotrexate and azathioprine are considered second and anti-TNF agents are third line treatments that are used solely or as glucocorticoid sparing agents for symptomatic extrapulmonary or pulmonary sarcoidosis with infiltrates on chest radiographs and abnormal PFT. Relapse rates have ranged from 13% to 75% depending on the population studied.
RESUMEN
A pancreatic pseudocyst is a complication of abdominal trauma in pediatric patients. Octreotide acetate is an effective adjunct therapy used in combination with traditional surgical approaches. We describe a 19-month-old boy with a pancreatic pseudocyst secondary to blunt abdominal trauma who was successfully managed with octreotide acetate in combination with percutaneous drainage and the placement of a pancreatic stent. Octreotide acetate 1 µg/kg/hour was administered as a continuous intravenous infusion for 24 hours, followed by 2.5 µg/kg/dose every 12 hours subcutaneously for 11 days. The patient was discharged after the pseudocyst had resolved and oral feeding was restored. He had no recurrence of the pseudocyst. The published literature regarding octreotide acetate therapy for pediatric pancreatic pseudocysts is limited. Previously reported cases demonstrated successful resolution of pancreatic pseudocysts with varying doses of intravenous and subcutaneous octreotide acetate within 23-30 days; however, with our patient's regimen, along with surgical interventions, the pseudocyst resolved within 11 days. In addition, our patient's regimen involved higher doses of octreotide acetate given more frequently than those reported in the literature. This case report illustrates that use of higher octreotide acetate dosages may be a potential adjunct therapy to surgical interventions for the management of pancreatic pseudocysts in children.