Hemophagocytic lymphohistiocytosis (HLH) has been recognized as an inflammatory endpoint for a variety of conditions including autoimmune diseases, malignancies and infections. It can be further classified as primary and secondary HLH. Primary HLH is also known as familial HLH. It usually presents in childhood and can be associated with gene mutations. Secondary HLH is also known as acquired HLH and usually presents in adulthood. In comparison to children, it is difficult to diagnose adults with HLH since it occurs with a variety of different diseases and most of the literature on HLH is derived from a pediatric population.In this case series, we report two cases of HLH that illustrate the difficulty of making this diagnosis and a brief review of the literature on its pathophysiology, clinical presentation, diagnosis, and a subsequent therapeutic approach.
The lungs are a common site of metastatic spread of an osteosarcoma. An affiliated simultaneous bilateral spontaneous pneumothorax (SBSP) is a rare clinical sequela of this malignancy. In this case report, we present the clinical circumstances of a young teenager who presented to our clinical setting following a diagnosis of osteosarcoma. We also illustrate the postulated pathophysiology, the tools for diagnosis and a subsequent management for this rare clinical entity.
Bilateral adrenal hemorrhage (BAH) is a rare but potentially fatal entity that carries a mortality rate of 15%. Most cases are associated with sepsis, antiphospholipid syndrome, the use of anticoagulants, as well as trauma and surgery. In this case report, we present a case of BAH in a previously healthy man with a recent history of corticosteroid use. Our case emphasizes the ambiguous clinical presentation of BAH, which poses a challenge in the establishment of a correct diagnosis. We also illustrate the pathophysiology, diagnosis, and subsequent therapeutic approach to this rare clinical entity.
Introduction: This study compares the effectiveness and cost of trans-catheter verses surgical closure of secundum atrial septum defect (ASD). ASD accounts for 10% of congenital cardiac defects. Trans-catheter closure of secundum ASD is increasingly used as the primary intervention. Surgical repair is advised in a proportion of secundum type defects which are unsuitable for device closure.
Methods: We reviewed the clinical course of 176 patients who underwent closure of isolated secundum ASD. The patients were assigned to either the device or surgical group depending upon the treatment they received. Successful closure was assessed immediately after the procedure. The following outcomes were studied: mortality, morbidity, hospital stay, and costs.
Results: Ninety five patients were in the surgical group and 81 patients were in the group undergoing device closure. The median age was 14.0 years (range 1.1-61.0) for surgical group and 24.0 years (range 0.5-68.0) for the device group. The mortality in both groups was 0. The procedure success rate was 100% for the surgical group and 96.3% for the device group. The complication rate was 13.7% for surgical group and 7.4% for the device group. The mean length of hospital stay was 5.0 ± 2.7 days for surgical group and 3.0 ± 0.4 days for device group. The procedure cost for surgery was found to be 12.3% lower than that of trans-catheter closure.
Conclusion: Successful closure is achieved by both methods. Trans-catheter closure results in lower rate of complication and hospital stay but the cost of the procedure tends to be higher than surgery.
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