Fadi Safi

Kaposiform Lymphangiomatosis, a Newly Characterized Vascular Anomaly Presenting with Hemoptysis in an Adult Woman

Disorders of the pulmonary lymphatic system include macro- and microcystic lymphatic malformations, primary or secondary lymphangiectasias, generalized lymphatic anomalies, diffuse pulmonary lymphangiomatosis, and combinations of lymphatic and other tissue anomalies, including lymphangioleiomyomatosis (LAM). We report a case of a patient with a newly defined entity classified as kaposiform lymphangiomatosis (KLA). This 50-year-old nonsmoking Hispanic woman presented with a 20-year history of cough, hemoptysis, chyloptysis, and pleuritic chest pain. Laboratory evaluation demonstrated a low normal platelet count, elevated d-Dimer, low normal fibrinogen, and elevated fibrin split products. Chest computerized tomography imaging showed enlarged hypodense lymph nodes in the mediastinum and hila, and peribronchovascular thickening, without evidence of cystic parenchymal lesions. Magnetic resonance imaging of the chest showed cystic mediastinal lymph nodes with heterogeneously increased T2 and decreased T1 signal intensity. Fiberoptic bronchoscopy revealed hyperemic mucosa with granular appearance suggestive of a submucosal infiltrative process. Pathological specimens revealed dilated, malformed lymphatic channels within the pleura, pulmonary septa, and bronchovascular bundles, and foci of perilymphatic and intralymphatic spindle cells which reacted with the Prospero homeobox protein 1 (PROX-1) immunostain. The morphology and immunohistochemistry results were consistent with a diagnosis of KLA. This newly recognized clinical-pathological entity among intrathoracic lymphatic anomalies is distinguished from generalized lymphatic anomaly and diffuse pulmonary lymphangiomatosis in part by characteristic hematological abnormalities and hemorrhagic complications, including hemoptysis, as experienced by our patient.

Fecal Transplant for Treatment of Toxic Megacolon Associated With Clostridium Difficile Colitis in a Patient With Duchenne Muscular Dystrophy.

Clostridium difficile (C diff) colitis infection is the most common cause of nosocomial infectious diarrhea and the prevalence is increasing worldwide. Toxic megacolon is a severe complication of C diff colitis associated with high mortality. Gastrointestinal (GI) comorbidity and impaired smooth muscle contraction are risk factors for the development of C diff-associated toxic megacolon. We present a case of fulminant C diff colitis with toxic megacolon in a patient with Duchenne muscular dystrophy (DMD) in the intensive care unit. C diff colitis was diagnosed by clinical presentation and positive C diff DNA amplification test (polymerase chain reaction). The impairment of GI tract due to DMD predisposes these patients to severe C diff infection and toxic megacolon, as observed in this case report. For the same reason, the recovery of GI function in these patients can be prolonged. While surgery was conducted for relieving the pressure from toxic megacolon, fecal microbiota transplantation through colonoscopy resulted in successful resolution of the C diff symptoms, although the recovery is prolonged due to DMD.

Posterior Reversible Encephalopathy Syndrome Secondary to CSF Leak and Intracranial Hypotension: A Case Report and Literature Review

Posterior Reversible Encephalopathy Syndrome (PRES) is a clinical neuroradiological condition characterized by insidious onset of neurological symptoms associated with radiological findings indicating posterior leukoencephalopathy. PRES secondary to cerebrospinal fluid (CSF) leak leading to intracranial hypotension is not well recognized etiology of this condition. Herein, we report a case of PRES that occurred in the setting of CSF leak due to inadvertent dural puncture. Patient underwent suturing of the dural defect. Subsequently, his symptoms resolved and a repeated brain MRI showed resolution of brain lesions. The pathophysiology and mechanistic model for developing PRES in the setting of intracranial hypotension were discussed. We further highlighted the importance of tight blood pressure control in patients with CSF leak and suspected intracranial hypotension because they are more vulnerable to develop PRES with normal or slightly elevated bleed pressure values.

Micafungin-induced thrombotic thrombocytopenic purpura: a case report and review of the literature.

Thrombotic thrombocytopenic purpura (TTP) is a life-threatening multisystem disorder characterized by microangiopathic hemolytic anemia and thrombocytopenia accompanied by microvascular thrombosis that causes variable degrees of tissue ischemia and infarction leading to organ dysfunction. Drug-associated thrombotic thrombocytopenic purpura and hemolytic uremic syndrome has been recognized for several years. The most commonly implicated drugs are mitomycin C, cyclosporine, quinine, clopidogrel, and ticlopidine. Recent advances have suggested that like in idiopathic TTP, the most likely pathogenesis for drug-induced TTP is either an immune-mediated phenomenon involving the ADAMTS13 metalloprotease or direct endothelial toxicity. In this communication, we report a case of micafungin-induced TTP. Micafungin is a new antifungal drug of the Echinocandins group. Whether micafungin induces autoantibodies against ADAMTS13 or not, this needs further evaluation, but TTP should be recognized as a possible complication of micafungin. Clinicians should be alert to this adverse effect of micafungin and monitor platelet counts in patients receiving this drug.

Acute Amiodarone-induced Lung Toxicity

CLINICAL PRESENTATION A 62-year-old man was admitted to the hospital for abdominal surgery for biliary leak. On the postoperative period, he developed atrial fibrillation with rapid ventricular response that responded to intravenous amiodarone bolus (150 mg) followed by amiodarone drip at 1 mg/hr for 48 hours. One day later, patient developed progressive shortness of breath with hypoxemic respiratory failure, requiring noninvasive positive pressure ventilation and oxygen. Chest radiograph showed evidence of new onset bilateral alveolar-interstitial infiltrate and minimal pleural effusion. Computerized tomography angiogram of the chest revealed multifocal pulmonary ground-glass opacities and foci of consolidation (Figure 1, computerized tomography chest axial image 72 hours after amiodarone). The differential diagnosis includes atypical infection, congestive heart failure and acute amiodarone-induced lung toxicity. Cardiac examination was normal. Echocardiography and Doppler studies showed normal ejection fraction with no evidence of diastolic dysfunction. Patient underwent flexible fiberoptic bronchoscopy with bronchoalveolar lavage of the right upper lobe that showed no significant secretions or airway abnormality; however, the cell count and differential of the lavage showed the presence of 43% of neutrophils and 46% of macrophages, 70% of which were foamy macrophages (Figure 2, normal macrophages on left and foamy macrophage on right). Serum KL-6 level was not performed. Respiratory cultures showed no growth. Amiodarone-induced pulmonary toxicity occurs in approximately 5% of treated patients. It includes acute respiratory distress syndrome, chronic interstitial pneumonia, organizing pneumonia and solitary pulmonary mass. A characteristic finding in all patients exposed to amiodarone is the presence of numerous foamy macrophages in the air spaces, which are filled with amiodarone-phospholipid complexes because of the inhibition of phospholipase. Risk factors for amiodarone-induced lung toxicity includes daily dose greater than 400 mg/d, increased patient age, preexisting lung disease, thoracic or nonthoracic surgery and pulmonary angiography. Treatment is to discontinue amiodarone and institute systemic glucocorticoid therapy with slow taper over a 2to 6-month period.

Hypertriglyceridemia-Induced Pancreatitis in Psychiatric Patients: A Case Report and Review of Literature.

Hypertriglyceridemia is a known cause of 2%-7% of cases of acute pancreatitis. Although there are numerous potential causes, the use of atypical antipsychotics has been linked to elevated triglycerides and pancreatitis. Here, we present the case of a 42-year-old male patient with a diagnosis of schizoaffective disorder who presented to our hospital with acute pancreatitis due to hypertriglyceridemia, which was exacerbated after he was started on quetiapine.

Hemophagocytic Lymphohistiocytosis in the Elderly

Hemophagocytic lymphohistiocytosis (HLH) is a rare disease of massive, dysregulated cytokine release and secondary multiorgan failure and is associated with high mortality. Primary HLH occurs predominately in infants and young children with a genetic predisposition. Acquired HLH is less well characterized and usually occurs in younger adults in the setting of severe inflammation triggered by infection or malignancy. Little is known about the disease in elderly. We report 3 patients >50 years old who presented with multiorgan failure and shock without an identifiable source and were ultimately diagnosed with acquired HLH. We performed a literature review of HLH in adults >50 years of age and identified an additional 68 cases. Mean age was 62 years, with male predominance. Most cases were triggered by infection (49%) followed by malignancy (27%). Nineteen patients were treated with the HLH-94 protocol, 11 received corticosteroids and the remainder received non-HLH specific interventions. Overall mortality was 62%.

Endotracheal Tube Sizes and the Development of Ventilation-Associated Events: Does Gender Play a Role?

Hermann Simo* Aahd Kubbara William Barnett Kevin Qin Wail Alamoudi Madiha Fida Nawaf Almashal Asif Iqbal Amala Ambati Ajit Ramadugu Christopher Marino Faisal Khateeb Fadi Safi and Ragheb Assaly University of Toledo Medical Center, Toledo, OH IO N , R E S E A R C H , A N D Q U A L IT Y IM P R O V E M E N T PURPOSE: Ventilator-associated events (VAEs) are surveillance paradigms established by the CDC. They denote three categories of increasing progression in mechanically ventilated patients from a ventilator-associated condition (VAC), to an infection-related ventilator-associated complication (IVAC), and finally to a ventilator-associated pneumonia (VAP). In a recent study, Gacouin et al., incidentally found that risk of late-onset VAP was higher in patients with a tube size greater than or equal to 7.5 mm. While there has been association found between ventilator associated pneumonia and endotracheal tube (ETT) size, there has not been a study done looking at CDC defined Ventilator Associated Events occurence and ETT size. We wanted to determine if there is an association between endotracheal tube size and the development of VAEs and whether that association is gender base.

Assessment of ventilator-associated events using the geometric distribution

&NA; Hospital‐acquired rare events, such as nosocomial infections and other low‐occurring incidents, in the clinical environment are difficult to monitor using traditional quality indicators. Using the Centers for Disease Control and Prevention definition of ventilator‐associated events, we demonstrate an alternative method for assessment of rare events.

Effects of Steroidal Therapy on the Incidence of Ventilator-Associated Events in a Northwestern Ohio Hospital

C R IT IC A L C A R E PURPOSE: Corticosteroids are commonly administered in critically ill patients for a variety of reasons. They were estimated to be used in about 26% of critically ill patients. Controversies have always surrounded their outcomes in the ICU. While they have been shown to increase death in Intensive Care Unit acquired pneumonia, they have been proposed to prevent hospital acquired pneumonia in intubated patients with multiple trauma. In the Society of Critical Care Medicine guidelines, it was recommended to consider hydrocortisone treatment for patients with septic shock with no mortality benefit. Herein, we present a sample of an ongoing analysis of the use of hydrocortisone and the risk of Ventilator Associated Events compared to other steroids in our university hospital. Ventilator Asscoiated Events (VAE) defined by the CDC National Healthcare Safety Network developed in 2013.