Survival Against the Odds-Hemophagocytic Lymphohistiocytosis Amidst the Shadows of Disseminated Histoplasmosis: A Case Report and Literature Review.

Hemophagocytic lymphohistiocytosis (HLH) secondary to Histoplasma capsulatum is rare, impacting <1% globally, with a mortality rate of up to 31%. Herein, we present a rare case of HLH secondary to H capsulatum, affecting a 57-year-old female with rheumatoid arthritis. Extensive investigations were unrevealing and despite broad-spectrum antibiotics, her condition worsened, leading to respiratory failure requiring extracorporeal membrane oxygenation (ECMO) support, shock requiring multiple vasopressors, and acute kidney injury (AKI) requiring hemodialysis. Diagnosis confirmed disseminated histoplasmosis (DHP), prompting Amphotericin B and methylprednisolone treatment, resulting in significant improvement and discharge with posaconazole therapy. Secondary HLH, primarily arising from severe infections like DHP, is discussed. Limited research exists on this condition in human immunodeficiency virus (HIV)-seronegative individuals. Diagnosis involves HLH-2004 and HScore criteria. Managing histoplasmosis-associated HLH remains challenging due to multiorgan failure risks and treatment complexities and needs further research.

Recurrent Bioprosthetic Valve Serratia marcescens Endocarditis in Intravenous Drug Users.

BACKGROUND We report 2 cases of recurrent right-sided endocarditis in 2 young patients known to be intravenous (i.v.) drug users. We highlight the importance of early diagnosis and management, especially in recurrent infection, which has a higher mortality rate and poor prognostic outcome despite antibiotic treatment. CASE REPORT A 30-year-old woman with a medical history of active i.v. drug use and tricuspid valve replacement owing to Serratia marcescens endocarditis 2 months prior to presentation was admitted to the Intensive Care Unit for septic shock. The patient did not respond to i.v. fluids and required vasopressors. Blood cultures returned positive for S. marcescens again. The antibiotic regimen consisted of meropenem and vancomycin. The patient underwent redo sternotomy, explant of old tricuspid valve bioprosthesis, debridement of tricuspid valve annulus, and bioprosthetic valve replacement. She continued antibiotic treatment during hospital admission for 6 weeks. In another similar case, a 30-year-old woman, also an i.v. drug user, was admitted to the hospital for tricuspid bioprosthetic valve S. marcescens endocarditis after tricuspid valve replacement 5 months prior to her presentation with S. marcescens endocarditis. Her antibiotic regimen consisted of meropenem and vancomycin. She was eventually transferred to a tertiary cardiovascular surgery center for further case management. CONCLUSIONS In the setting of recurrent bioprosthetic valve S. marcescens endocarditis, it is suggested that treatment should be more focused on source control, including cessation of i.v. drug abuse and providing appropriate antibiotic treatment to prevent recurrence because, in the case of recurrence, morbidity and mortality risk can increase significantly.

Nasal myiasis in myasthenic crisis, a case report and literature review.

We are reporting a unique case of drosophila larva nasal myiasis (NM) in a 72-year-old male patient admitted to the ICU with diffuse muscle weakness and respiratory failure due to myasthenia gravis crisis and septic shock due to pseudomonas pneumonia. The myiasis was noticed on the third day of admission two days following traumatic insertion of a nasogastric tube. The patient underwent nasal endoscopic mechanical extraction and lidocaine nasal spray with saline nasal flushes. To our knowledge, this is the first case report of NM in a myasthenia gravis (MG) patient. Chronic muscle weakness in MG patients might play a role in having NM as these patients are less likely to be able to protect themselves from flies. Managing NM in our patient was challenging due to the potential neurologic side effects of most of treatment options mentioned in the literature.

Limbic Encephalitis as a Presenting Complication for Small Cell Lung Cancer.

Limbic encephalitis (LE) is a rare neurological paraneoplastic complication that occurs secondary to malignant tumors. It is commonly presented as refractory seizures that are resistant to most anti-epileptics. We are presenting a unique case of small cell lung cancer complicated with LE. The challenging part of our case is that the patient had a history of seizure disorder in the past, and she was treated initially as an anti-epileptic treatment failure. A 68-year-old patient with a history of epilepsy was admitted to the ICU with resistant status epilepticus (SE), and respiratory failure secondary to pneumonia. Further workup revealed that the patient has small cell lung carcinoma. An extensive workup done to investigate resistant seizures revealed that she had a rare type of paraneoplastic autoantibodies (Anti-Hu) in the cerebrospinal fluid, which supported the diagnosis of the paraneoplastic autoimmune LE. High dose steroids helped to decrease the seizures episodes, but the family decided to proceed with palliative measures only at the end. Diagnosing LE requires ruling out other common causes of SE. Treatment options include treating underlying cancer as well as means of immunosuppression or antibody removal by tacrolimus and cyclophosphamide and even intravenous immunoglobulin (IVIG) or plasma exchange. It is important to consider LE in the differential diagnosis when managing patients with resistant SE in the ICU, even if the brain imaging and cerebrospinal fluid (CSF) analysis were within normal limits.

A Case of Congenital Lymphedema Complicated by Chronic Chylous Effusions and Recurrent Pericardial Effusion Requiring Pericardial Window.

We are presenting a case of primary lymphedema (PL) complicated with a repeated need for thoracentesis and pericardiocentesis. Our patient is a 24-year-old male with primary lymphedema that is manifested in the left hand and right lower limb. The patient presented to the emergency department (ED) for recurrent right lower lobe cellulitis that had failed repeated attempts with outpatient antibiotic therapy. The patient was admitted to the intensive care unit due to signs of cardiac tamponade that were discovered on the physical examination. Pericardial tamponade was confirmed by echocardiography. The patient underwent thoracentesis and multiple pericardiocenteses and required a pericardial window. Pericardial and pleural fluids appeared milky and biochemical analysis was consistent with chylopericardium and chylothorax.

Acute Hemothorax Causing Hemorrhagic Shock Following Small-bore Thoracocentesis in a Patient on Clopidogrel: A Case Report and Literature Review.

Clopidogrel is one of the most commonly prescribed thienopyridines used postcoronary stenting for acute coronary syndrome (ACS). There have been several concerns regarding performing small-bore thoracocentesis on patients on clopidogrel in our practice. We present an 85-year-old male with a history of diabetes, atrial fibrillation, diastolic heart failure, chronic kidney disease (CKD) stage three, who recently had a non-ST elevation myocardial infarction (NSTEMI) requiring a drug-eluting stent (DES) to the left anterior descending (LAD) artery, and was on dual anti-platelet therapy (DAPT). He was admitted with worsening shortness of breath and found to have bilateral pleural effusions. He required several small-bore, ultrasound-guided thoracocentesis on the right side while on clopidogrel. Intensivists or residents did all the procedures. The patient went into a hemorrhagic shock following his third small-bore thoracocentesis, requiring pressors, blood and platelet transfusions, and a surgical intercostal (IC) drain insertion. He eventually became clinically stable. An IC arteriogram within 24 h ruled out IC artery injury. Healthcare professionals perceive small-bore thoracocentesis as a safe procedure done on medical, surgical, intensive care, and interventional radiology (IR) units. The overall consensus is that it is safe to perform it on patients taking clopidogrel. We emphasize through this case report the bleeding risks associated with performing such procedures on patients while on clopidogrel and considering holding clopidogrel when feasible or bridging with an intravenous anti-platelet drug.

A series of two cases of rare tumors: Solitary fibrous tumor of the pleura.

Solitary fibrous tumors of the pleura (SFTPs) are rare neoplasms of mesenchymal origin most commonly arising in the pleura and have a benign biological behavior in the majority of cases. Most patients with SFTPs are asymptomatic; however, symptoms are mostly related to the mass effect within the thoracic cavity. Chest computed tomography (CT) scanning is the radiographic test of choice, but findings lack specificity. Surgical resection is the treatment of choice for most patients. Long-term survival after resection of benign SFTPs is excellent. In this case series, we present two females, one presented with shortness of breath (SOB) and nonproductive cough and the other one was referred because of abnormal x-ray findings. The histological as well as the immunohistochemical examination revealed the mass to be a solitary fibrous tumor of the pleura.

Kounis syndrome secondary to gadolinium contrast agent.

Cases of coronary artery spasm secondary to contrast agent use are rarely reported. Herein, we report the case of a 53-year-old woman who developed chest pain, dyspnea, and bradycardia and quickly become unresponsive after magnetic resonance imaging of the brain. A heart monitor showed ST elevation, and an electrocardiogram showed ST elevations in leads II, III, aVF, V3, and V4 and ST segment depression in lead I. Urgent left heart catheterization revealed no evidence of obstructive coronary artery disease or pulmonary embolism. A few days later, she was discharged from the hospital with no symptoms. A type I variant of Kounis syndrome was diagnosed.

Pulmonary alveolar microlithiasis diagnosed with radiography, CT, and bone scintigraphy.

Pulmonary alveolar microlithiasis is rare disease characterized by accumulation of calcium phosphate microlithis in the alveoli. The pathogenesis relates to mutation in the gene SLC34A2 (solute carrier family 34 member 2) located on chromosome 4p15.2, which produces a defective sodium-phosphate cotransporter in alveolar epithelial type-2 cells, making these cells unable to clear phosphorus released during recycling of surfactant [1].

Massive hemoptysis due to recurrence of bronchial to pulmonary vascular malformation: A case report.

Bronchial dieulafoy lesions are quite rare with relatively few case reports in the literature. Symptoms may vary but the lesion is often associated with hemoptysis and may present as massive hemoptysis. We present a case of a 69-year-old male with a recurrent episode of hemoptysis three years after treatment for a bronchial dieulafoy lesion. The bronchoscopy done three years prior during an initial episode of hemoptysis showed a visible dieulafoy deep within a subsegmental branch of the right lower lobe. This case is unique because there are no other reports within the literature of a delayed recurrence several years after previous treatment of a bronchial dieulafoy lesion, which in our case was due to bronchial to pulmonary vascular malformation. Bronchial arteriography revealed a bronchial artery to pulmonary artery vascular malformation, which was successfully treated with coil embolization.

Rasburicase induced severe hemolysis and methemoglobinemia in a Caucasian patient complicated by acute renal failure and ARDS.

Rasburicase is a recombinant urate-oxidase enzyme and is a very important medication for tumor lysis syndrome. Methemoglobinemia and hemolysis are known side effects of rasburicase that result from oxidative stress caused by hydrogen peroxide, a byproduct generated during the breakdown of uric acid to allantoin. Patients with G6PD deficiency have a decreased tolerance to oxidative stress and are therefore at a greater risk of hemolysis and methemoglobinemia with rasburicase. Our patient is a 56-year-old Caucasian male with a recent diagnosis of grade 2-3a non-Hodgkin's lymphoma who presented to our emergency department with shortness of breath and dark discoloration of urine. Patient was discharged 36 hours ago from our hospital after he was given a first course of R-CHOP regimen and a dose of rasburicase. On further evaluation, patient was found to have severe anemia with hemolytic picture, hyperkalemia and acute kidney injury. He also had a discrepancy of the transcutaneous saturation (75%) and the saturation in an arterial blood gas value (99%). His methemoglobin level was found to be 11.9%. We were aware that methylene blue is a contraindication in patients with G6PD deficiency but considering patient being Caucasian and low risk for it and his deteriorating respiratory condition, it was decided to offer the treatment and patient received 1 dose of methylene blue which failed to improve his methemoglobinemia. He was also given vitamin C and 8 units of packed red blood cell throughout his stay in the hospital. Patient's hospital course was complicated by ARDS needed to be on mechanical ventilation support for 4 days and acute renal failure secondary to pigment nephropathy and acute tubular necrosis which required a hemodialysis support. Even if rasburicase induced methemoglobinemia and hemolysis are not very common complications, clinicians who prescribe and follow patients should detect this serious complication early and manage it accordingly. Our case can be used as a reminder that patients should be followed closely and given the right instructions on discharge to treat these complications which are associated with severe consequences. It is also vital to assume a diagnosis of G6PD deficiency until proven otherwise in a patient who presents with rasburicase induced hemolysis and avoid administration of methylene blue even if the patient is from a low risk ethnicity for G6PD as in our patient.

Pulmonary vein thrombosis secondary to tuberculosis in a non-HIV infected patient.

Our patient is a 67-year-old male with a past medical history significant for hypertension and hyperlipidemia came to a hospital with hemoptysis. He was also having cough and shortness of breath for the last 1 month. He said that his hemoptysis was about 1 cup per day mixed with yellowish sputum. He noticed around 20 pounds of weight loss in the last 1 month. He also complained of night sweats but had no fever. He had no history of travel outside the USA. He has never been incarcerated before, but he endorsed that his son has been to Jail before and he visited him twice a year in patient's home. But he also said that his son has never been diagnosed with TB. He smoked 1.5 packs per day for the last 50 years and quit smoking 2 months ago. His medication include hydrochlorothiazide, lisinopril, gabapentin, aspirin and trazodone. On examination, vital signs were within the normal range except a hearty rate of 106 beats/minute. He had slightly pale conjunctiva, non-icteric sclera and had wet tongue and buccal mucosa. There was decreased air entry with crepitations in the right side of the posterior chest but no wheezes or rales. No peripheral lymphadenopathy, no peripheral edema or sign of fluid collection in the abdomen. Chest x ray showed multiple cavitary lesion in the right upper lobe area. CT scan of the chest with PE protocol showed pulmonary venous partial thrombosis in the right upper lobe. Multiple cavitary lesions with hilar and mediastinal lymphadenopathy. There are also smaller nodular lesions in the left chest too. Small right pleural effusion with multiple calcified granulomata in the left upper lobe. QuantiFERON gold test was found to be positive. Sputum AFB smear was found to be strongly positive and it is sensitive to rifampin. Echocardiography showed no valvular lesions with preserved ejection fraction (>65%) and normal right ventricular size and normal right ventricular systolic pressure. Liver enzymes and renal function tests were found within the normal limit. HIV test was negative. Patient was started with intensive phase anti-tuberculosis treatment with rifampin, isoniazid, ethambutol, pyrazinamide with vitamin B6. He was also started with anticoagulation with heparin and warfarin considering the tuberculosis being the cause of the pulmonary vein thrombosis. Patient was also given supportive treatment and he made a gradual improvement and was discharged with anti-tuberculosis treatment and warfarin. Patient needed to be placed on a higher dose of warfarin as it was difficult to keep him therapeutic with lower doses. He was also advised to follow with infectious disease and anticoagulation clinic. Patient was found to have a significant increase in liver enzymes and bilirubin on follow up and the anti-TB medications were stopped to be restarted one by one with a follow up of his liver enzymes and liver function tests. He was also continued with warfarin.

Urinothorax Caused by Xanthogranulomatous Pyelonephritis.

Xanthogranulomatous pyelonephritis is a rare form of chronic pyelonephritis that generally afflicts middle-aged women with a history of recurrent urinary tract infections. Its pathogenesis generally involves calculus obstructive uropathy and its histopathology is characterized by replacement of the renal parenchyma with lipid filled macrophages. This often manifests as an enlarged, nonfunctioning kidney that may be complicated by abscess or fistula. This case details the first reported case of xanthogranulomatous pyelonephritis complicated by urinothorax, which resolved on follow-up chest X-ray after robot-assisted nephrectomy.

Atypical rapid onset Scleroderma Renal Crisis (SRC) complicated with diffuse alveolar hemorrhage and pleuro-pericardial effusions in a patient with recently diagnosed breast cancer and a positive anti-RNA polymerase III Ab.: A case report.

Scleroderma associated Pulmonary-Renal Syndrome is a rare but severe complication with a poor prognosis and high mortality. A high index of suspicion is needed for early recognition of this potential complication in patients with systemic sclerosis and institution of appropriate treatment. With more data showing an increased association between scleroderma and malignancy, a heightened vigilance should also be exercised in patients with malignancy and scleroderma-like presentation. We report of a case rapid onset systemic sclerosis complicated by acute renal failure and diffuse alveolar hemorrhage in a woman with stage IIB right breast cancer and elevated RNA Polymerase III IgG Ab. To our knowledge, this the first case of a patient with breast cancer associated with systemic sclerosis and pulmonary-renal syndrome.

Hypoxic respiratory failure due to hyperammonemic encephalopathy induced by concurrent use of valproic acid and topiramate, a case report and review of the literature.

Valproic acid (VPA) is widely used for the treatment of epilepsy, migraine, and a variety of psychiatric symptoms, including bipolar disorder, borderline personality disorder, and alcohol withdrawal. Valproate is associated with severe idiosyncratic adverse effects, the most notable being valproate-induced hyperammonemic encephalopathy (VHE). Topiramate is also a broad-spectrum anticonvulsant that is also extensively used for migraine prophylaxis, as a mood stabilizer, and for alcohol dependency. There is increased occurrence of VHE when valproate is used with other medications like phenytoin, phenobarbital, and topiramate. Our case report is on a young patient who was on valproic acid and topiramate and developed metabolic encephalopathy with hypoxic respiratory failure. We reviewed the causes and management of the hyperammonemic encephalopathy. We believe that clinicians should be aware of possible hyperammonemic encephalopathy in any patient who is taking valproic acid and presenting with impaired consciousness and cognitive decline. We also underline the importance of early recognition and high index of suspicion of encephalopathy related to hyperammonemia.

Candida krusei Empyema Thoracis: A Community-Acquired Infection Requiring a High Index of Suspicion.

Empyema thoracis is a serious condition characterized by the accumulation of purulent fluid in the pleural cavity, typically following a pneumonia, subdiaphragmatic abscess, or esophageal rupture. Fungal empyema thoracis is a rare form of this condition with especially high mortality, in which the most frequently isolated fungus is Candida spp. This article presents a 74-year-old female with Candida krusei pneumonia and a complicated hospital course, initially presenting with nausea, vomiting, and dysphagia. She was initially suspected to have community-acquired pneumonia and was started on azithromycin and ceftriaxone. Worsening respiratory function led to the diagnosis of hydropneumothorax. Pleural fluid and an independent sample of pus and pleural tissue grew Candida krusei, giving the diagnosis of fungal empyema. With further respiratory deterioration, the patient was intubated and switched to piperacillin/tazobactam and micafungin. Decortication with extensive pleural peel and removal of foul-smelling pus and food particles within the chest was performed. This further lead to confirmation of esophageal perforation, and she was started on voriconazole and meropenem. After developing septic shock, the patient was managed with phenylephrine and vasopressin. Finally, after improving she was weaned off pressors and extubated, followed by an esophagogastroduodenoscopy (EDG) with pneumatic balloon dilation and WallFlex stent placement. This patient's case demonstrated an example of empyema thoracis, which required a high index of suspicion since the presentation was with a community-acquired infection. Candida empyema thoracis may be a complication of operation, gastroesophageal fistula, and spontaneous esophageal rupture. On the other hand, the course of this patient's hospital stay progressed from esophageal perforation to Candida krusei pneumonia, empyema, and pneumothorax. Thus, community-acquired fungal empyema should be considered in patients with respiratory symptoms and suspected esophageal perforation; nevertheless, after a diagnosis of fungal empyema, esophageal perforation should also be ruled out in addition to other causes like pneumonia, subphrenic abscess, and hematogenous spread. Improved communication between clinicians and microbiologists can lead to early diagnosis and a reduction in the morbidity and mortality of this condition.

Two patients with daptomycin induced eosinophilic pneumonia with different presentations and treatment.

We present two cases of daptomycin-induced eosinophilic pneumonia with characteristic chest x ray, CT scan and bronchoalveolar lavage (BAL) findings. It is not a commonly seen complication of Daptomycin but when it happens, it could cause a diagnostic and treatment dilemma. Patients could present acutely with hypoxic respiratory failure or a less dramatic chronic presentation is also possible. Our two patients presented with acute hypoxic respiratory failure and presentation resembles that of an infectious etiology. Diagnosis is confirmed by bronchoscopy with BAL in one of them and the other was treated empirically as the clinical and radiologic presentation was typical. Treatment includes removal of the offending agent, daptomycin. Corticosteroids are used if symptoms are severe and can result in rapid clinical improvement. There is no agreed upon dose and duration of steroids and we suggest a long taper of steroids for patients who present with severe symptoms and a short course steroid for patients with milder symptoms.

Bronchopleural Fistula Resolution with Endobronchial Valve Placement and Liberation from Mechanical Ventilation in Acute Respiratory Distress Syndrome: A Case Series.

Patients who have acute respiratory distress syndrome (ARDS) with persistent air leaks have worse outcomes. Endobronchial valves (EBV) are frequently deployed after pulmonary resection in noncritically ill patients to reduce and eliminate bronchopleural fistulas (BPFs) with persistent air leak (PAL). Information regarding EBV placement in mechanically ventilated patients with ARDS and high volume persistent air leaks is rare and limited to case reports. We describe three cases where EBV placement facilitated endotracheal extubation in patients with severe respiratory failure on prolonged mechanical ventilation with BPFs. In each case, EBV placement led to immediate resolution of PAL. We believe endobronchial valve placement is a safe method treating persistent air leak with severe respiratory failure and may reduce days on mechanical ventilation.

Mechanical ventilation management by pulmonologists and surgeons in patients with adult respiratory distress syndrome.

BACKGROUND: Treatment of patients with acute respiratory distress syndrome (ARDS) is complex, and management by a specialist with expertise in pulmonary mechanics may improve outcomes. We compared mechanical ventilation management of patients with ARDS by pulmonologists and surgeons. METHODS: We retrospectively reviewed 97 patients with an ICD-9 diagnosis of ARDS at 2 community hospitals. We collected information on demographics and all necessary parameters to calculate the acute physiology, age, and chronic health evaluation (APACHE II) score. Main outcomes included mortality and total days spent in the intensive care unit (ICU) and on mechanical ventilation. All outcomes were adjusted for APACHE II score using multiple logistic regression. RESULTS: Mechanical ventilation was managed by a pulmonologist in 62 patients and by a surgeon in 35 patients. Mortality rate was 35.5% (n = 22) in the patients treated by pulmonologists and 45.7% (n = 16) in patients treated by surgeons (P = 0.32). This result was unaffected by adjustment for APACHE II score. However, those surviving spent fewer days in the ICU (median of 10 vs 16 days; P = 0.07) and fewer days on mechanical ventilation (median of 7 vs 15 days; P = 0.003) when treated by pulmonologists. These results were unaffected by adjustment for APACHE II score. CONCLUSIONS: We found that patients who survived with ARDS spent fewer days on mechanical ventilation, and there was a trend for spending fewer days in the ICU when mechanical ventilation is managed by a pulmonologist compared with a surgeon. There was a lower mortality rate in the pulmonologist group, although this did not reach statistical significance. A small sample size and the retrospective design limit our findings. Further study using a multicenter design to determine if a disease specific specialist improves efficiency of care is needed because if our findings are confirmed, it would translate into significant cost savings.