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2023 AMA Research Challenge

Mucosal Infection with Tsukamurella Species Following Nasal Septum Procedure: A Rare Case Report

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Introduction:
The main issue that has frequently plagued treatment of OSA is compliance and tolerance of continuous positive airway pressure (CPAP). This has spurred innovation in this field with the development of more tolerable nasal pieces and, most recently, hypoglossal nerve stimulators (HGNS). However, just like any other implantable device, there is a real risk of infection with implantation and subsequent need for removal. A published review in the American Journal of Otolaryngology on a search query of adverse events associated with HGNS implantation from the FDA Manufacturer and User Facility Device Experience (MAUDE) database yielded 180 reports containing a total of 196 adverse events. The most commonly reported adverse events to patients were infection (n = 50, 34.2%). Subsequently, a total of 83 adverse events, whether infection or device migration (42.3%) required re-operation. With the exciting innovation of new implantable devices being developed in medicine, our case stresses the importance to recognize unconventional sources of infection in the undifferentiated septic patient.

Case Description:
Our patient was a middle-aged gentleman with ankylosing spondylitis and OSA status post hypoglossal nerve stimulator (HGNS) implantation presenting with purulent drainage from the implant site on his chest wall, as well as subacute severe left knee pain and swelling. Following HGNS implantation five months prior to presentation, he had poor wound healing requiring multiple outpatient courses of antibiotics. During this time, his Enbrel for ankylosing spondylitis was held due to concerns for immune-compromised state and worsening infection, and his HGNS was never actually activated for use. On presentation there was valid concern for hematogenous spread to his left knee, and an MRI of the left knee was obtained that was not concerning for osteomyelitis. An arthrocentesis was performed showing an inflammatory fluid aspirate with negative grain stain and no growth on bacterial cultures. Wound cultures obtained from the implant site on the chest wall demonstrated MSSA, and he was transitioned from broad spectrum vancomycin and zosyn to IV cefazolin. The HGNS implant was subsequently removed with visible draining pus on the stimulator leads. He was ultimately discharged on a course of cefalexin for two weeks with Rheumatology follow up in the setting of suspected ankylosing spondylitis flare of his left knee.

Discussion:
We treated our patient similarly to a pacemaker lead infection with prompt removal of the device and a course of antibiotics geared towards bacteria that tend to grow on foreign devices. An additional complicating layer to our case was the presentation of a potential septic knee, which required swift evaluation and rule out from the interdisciplinary team. As HGNS products become more popular choices of treatment for OSA, we will be seeing more cases similar to ours in the community and will start to develop more informed and nuanced management. As hospitalists, it is our duty to view our patients on presentation as a whole - innate bodily systems and organs plus their foreign hardware.


Hypoglossal nerve stimulator (HGNS) implant infection: a feared
complication for a novel treatment of obstructive sleep apnea

Numerous studies have established a strong connection between the heart and brain, especially in response to stressful stimuli. One condition, Takotsubo cardiomyopathy (TC), is characterized by a temporarily weakened left ventricle apex resulting from excess catecholamines and microvascular dysfunction. Another different condition, posterior reversible encephalopathy syndrome (PRES), is associated with acute neurological changes and changes in the occipital lobe. These two conditions share similarities despite presenting different clinical features and requiring distinct diagnostic workups and treatments.

Case Description
A 49-year-old woman with a past medical history of hypertension and migraines presented after a witnessed seizure following a few hours of worsening headaches. She reported high stress, with her husband diagnosed with blood cancer and her dog recently passing away. Her blood pressure was mildly elevated at 156/92. Initially, she appeared to have right-sided muscle weakness, which resolved. A CT brain scan demonstrated occipital lobe changes, suggesting PRES. A CTA brain revealed no major vascular blockages, and an EEG revealed no epileptiform activity. The next day, she reported substernal chest pain and an ECG revealed new dynamic T-wave inversions in the anterior leads. Her troponins were elevated (peaking at 2222 ng/ml), and a TTE showed apical wall abnormalities. A subsequent cardiac catheterization revealed no coronary occlusion, suggesting TC. The patient improved clinically with conservative therapy during the hospitalization and was eventually discharged home.

Discussion
The "heart and brain connection" was previously identified in patients with acute neurologic insults. PRES is a rare neurologic entity; manifestations include headaches, focal neurologic deficits, seizures, vision loss, or encephalopathy. PRES is believed to involve cerebral blood flow alterations and brain autoregulation dysfunction. Imaging findings include vasogenic edema involving the posterior regions of the brain. It can be seen in hypertensive emergencies, renal failure, autoimmune diseases, and sometimes medication-induced flares. PRES is usually reversible. However, a recent small study showed a mortality rate of 4% and residual damage of 5.5%. TC, aka apical ballooning syndrome, previously known as stress-induced cardiomyopathy, has a 1–3% prevalence in postmenopausal females and 5–6% in those with ACS. It is usually triggered by underlying physical or emotional stress, as reported in 60–70% of cases. Studies have shown that 2% of ACS is due to TC. Hence, it is considered one of the ACS-mimicking diseases. Usually present with chest pain or new onset heart failure with characteristic echo findings of apical balloon shaping. The cardiac catheter is usually negative for any obstruction. 
Based on the available data, there is a possible association between PRES and TC. One study reported a prevalence of 2.7%, and around 14 cases were found in the literature, eight of which are in PubMed journals. It is noteworthy that 13 of the 14 cases involved postmenopausal women over the age of 45 years old. While these conditions can be serious, most patients have a good prognosis with appropriate treatment and support. Further research is needed to understand better the connection between these two conditions and their long-term implications. Treatment approaches may vary depending on the patient's circumstances, so seeking a multidisciplinary approach to care is important.

Conclusion
Healthcare providers should keep a high clinical index of suspicion for potential heart and neurological issues like PRES and TC in postmenopausal women with acute coronary syndrome. Neglecting or ignoring TC can lead to a worse prognosis. It is worth noting that neurological deficits associated with TC may indicate PRES. Further research must be conducted to understand and treat these serious illnesses.



A Rare Combination: A Case of a Patient with Takotsubo Cardiomyopathy and 
Posterior Reversible Encephalopathy Syndrome (PRES)

A venous air embolism occurs when gas enters a venous structure that is under negative pressure. This arises most frequently through central venous catheterization. We report the case of a man in his 70s who underwent computed tomography (CT) guided biopsy of a 14 millimeter nodule in the left upper lobe of the lung. This procedure was complicated by an anterior left-sided pneumothorax as well as pulmonary hemorrhage due to nicking of the left superior pulmonary vein. This nicking is believed to have introduced an air embolism, which then circulated to the heart as visualized on CT. The patient required bilevel positive airway pressure (BiPAP) for about twelve hours, as well as placement of a thoracostomy tube for three days. Following the event an echocardiogram and CT head were performed in order to track the air embolism, however the embolism was not visualized. The patient continued to have pleuritic chest pain and hemoptysis at the time of discharge, and was followed closely outpatient by pulmonology.

Introduction of air embolism during a lung biopsy - a case report

Background 
Diffuse large B cell lymphoma (DLBCL) is a common subtype of non-Hodgkin lymphoma. Rarely, it presents with lactic acidosis, a paraneoplastic phenomenon associated with high mortality. Lactic acidosis is caused by the Warburg effect, resulting in lactate production even in the presence of oxygen. Few cases of lactic acidosis in DLBCL have been reported. Prompt diagnosis and management are crucial. Treatment with R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone) chemo-immunotherapy is standard for DLBCL. We present a case of lactic acidosis in a 60-year-old female with DLBCL, emphasizing the importance of timely recognition and treatment of this condition in hematologic malignancies due to its poor prognosis. 

Case Presentation 
We present the case of a 60-year-old woman with a complex medical history, including obesity, hypothyroidism, chronic low back pain, adrenal insufficiency, anemia, thrombocytopenia, hyponatremia, and psychogenic polydipsia. She was admitted due to confusion, gait imbalance, dyspnea, leg edema, and hypotension. Elevated lactate levels indicated potential metabolic issues related to her comorbidities. Multiple specialties were consulted. Diagnostic procedures included laparoscopy, negative AAT stool results, liver and bone marrow biopsies, and discontinuation of antibiotics after negative cultures. Despite continuous monitoring, her lactate level remained elevated. Abnormal light chain ratio and albumin-predominant proteinuria suggested amyloidosis. Imaging revealed lung abnormalities, and a thoracentesis showed lymphocytic exudates. A biopsy of the abdominal fat pad confirmed diffuse large B cell lymphoma (DLBCL). Kidney function worsened, requiring pressure support. Treatment with Rituxan and Rasburicase, followed by R-CHOP chemotherapy, was initiated. This case underscores the challenges in managing a patient with multiple comorbidities, leading to metabolic abnormalities, lung findings, and a diagnosis of DLBCL. Prompt treatment initiation was vital. 

Discussion 
Lactic acidosis, an uncommon manifestation of hematologic malignancies, is associated with poor prognosis. Lactic acidosis occurs when there is an imbalance between lactic acid production and hepatic metabolism. It is classified into types A, B, and D based on the underlying causes. Type B lactic acidosis is linked to malignancies, including hematologic cancers, where tumor cells rely on glycolysis for energy production. The Warburg effect explains this phenomenon, where tumor cells preferentially metabolize glucose to lactate even in the presence of oxygen. Treatment involves addressing the underlying malignancy, while supportive measures like hemodialysis and thiamine supplementation may be used. Malignancy-associated lactic acidosis is a grave prognostic marker. 


Lactic Acidosis Unveiled: A Hidden Clue to Diffuse Large B Cell Lymphoma

Throughout the pandemic, many COVID-19 patients were treated with therapeutic anticoagulation due to the increased risk of thrombotic events. Spontaneous Lumbar Artery Rupture (SLAR) is an uncommon vascular phenomenon typically associated with trauma and even less frequently in the setting of anticoagulation therapy. While Enoxaparin can increase bleeding risk, fatal hemorrhage is a rare occurrence. In this case, we describe a unique presentation of a patient with COVID-19 and NSTEMI on therapeutic Enoxaparin who developed SLAR and widespread catastrophic hemorrhaging at multiple sites resulting in hemorrhagic shock, cardiac arrest, and ultimately death. 
A 64-year-old male with a history of type II diabetes, hypertension, and coronary artery disease status post coronary artery bypass grafting was admitted for Acute Hypoxic Respiratory Failure due to COVID-19 Pneumonia and found to have Non-ST Elevation Myocardial Infarction. Given his COVID-19 infection, cardiac catheterization was postponed and was instead treated medically with therapeutic dose Enoxaparin. On day six, he became intermittently hypotensive without any other vital sign changes, physical complaints, or worrisome changes on physical exam or Point of Care Ultrasound. Labs showed an unchanged Hemoglobin of 14.5 and his blood pressure stabilized with one liter of normal saline. Several hours later, his hypotension returned, now unresponsive to fluids requiring pressor initiation and transfer to ICU. He now complained of lower abdominal and back pain and had developed left flank and left lower abdominal ecchymoses. Shortly after transfer, the patient suffered four cardiac arrests and post-arrest blood gas showed a base deficit of -26 and hemoglobin of 5.0 prompting administration of Protamine. Post-arrest pelvic angiography showed a SLAR and coil embolization was performed. Post-procedure CTA revealed multiple areas of active hemorrhage and large hematoma development throughout the chest, mediastinum, abdomen, and pelvis. Over the next two days, he received massive blood transfusion and maximum doses of pressors before ultimately succumbing to multi-system organ failure.
A PubMed search revealed only two case reports of SLAR in the setting of therapeutic anticoagulation, no case reports of SLAR in setting of COVID-19, and one case series of four cases of localized hemorrhage in the setting of COVID-19 with concomitant therapeutic anticoagulation. This case illustrates the potential for hemorrhage in the setting of COVID-19 possibly due to endothelial dysfunction compounded by therapeutic anticoagulation. It also highlights an unusual presentation of an actively developing hemorrhagic event without obvious exam or vital sign changes.

A Rare Case of Atraumatic Spontaneous Lumbar Artery Rupture and Widespread Hemorrhage in the setting of COVID-19 and Therapeutic Anticoagulation

Background: Linezolid has gained increased use for multi-drug-resistant gram-positive bacteria in recent years. It inhibits bacterial as well as human mitochondrial protein synthesis by binding to specific ribosomes. Therefore, linezolid can cause rare, but potentially life-threatening lactic acidosis. To date, little is known about Linezolid-induced lactic acidosis (LILA). Here, we presented a case report of LILA who presented with the signs and symptoms of acute cholecystitis.

Case Presentation: A 55-year-old male with a history of obesity, tobacco use, and uncontrolled diabetes mellitus complicated by osteomyelitis of the left foot came to the emergency department with complaints of epigastric and left upper quadrant pain for 1 day associated with nausea and vomiting. Vitals signs were as follows: Temperature 35.5, heart rate 123, blood pressure 174/105, Oxygen saturation of 97% on room air. He was producing good urine output. Relevant labs are as following: WBC count 14.3 k/cumm, lactate level 7.3 mmol/L, pH 7.22, anion gap 18 mmol/L, creatinine 1.39 mg/dl. No other abnormalities were noted on the rest of the complete metabolic panel. On a CT scan, the patient had an enlarged gall bladder. Initially, the patient was diagnosed and treated for sepsis due to acute cholecystitis with broad-spectrum antibiotics and intravenous fluids per protocol. Despite additional 2-liter fluid bolus, lactate level continues to be elevated.
On review of his medication, the patient has a previous history of chronic diabetic left foot osteomyelitis and MSSA septicemia requiring hospitalization for 3 days. He was treated with oral linezolid due to his inability to tolerate any adhesives required for a PICC line. After 6 weeks of treatment with Linezolid, the patient developed sudden abdominal pain, nausea and vomiting. 
After investigating alternative differentials such as hypoperfusion, tissue necrosis or liver failure, Linezolid-induced lactic acidosis (LILA) was suspected. Linezolid was discontinued and transitioned to doxycycline. After 12 hours of discontinuation, his lactate level went down to 1.8 mmol/L and his pH improved to 7.32.

Discussion: Linezolid is likely associated with lactic acidosis with high mortality. Moreover, Patients can have divergent presentations. We should have high suspicion and check serum lactate regularly for the patients who are on Linezolid therapy. 


Linezolid-Induced Lactic acidosis mimics symptoms of Acute Cholecystitis: A Case Report

Background:
Prolonged walking can occur in various settings, ranging from amusement and national parks to marathon races. When performed in hot climates, prolonged walking can induce ExerciseInduced Purpura (EIP), a form of small-vessel vasculitis that primarily affects the lower extremities. Purpura in adults typically indicates an underlying platelet disorder or multi-system disease requiring urgent attention. We seek to raise awareness of EIP, a benign condition, among primary care health professionals.

Case Presentation:
A 55-year-old post-menopausal woman from Europe with a sedentary lifestyle traveled to Las Vegas for vacation. She noticed a purpuric rash with mild soreness and edema on her lower extremities, extending from the ankles to the mid-legs. There was no prior history of petechial rashes or bruisability. She drank a few glasses of wine daily and denied recent consumption of raw fish or meat. The only change was prolonged walking for four consecutive days with a daily step count of 20-30 thousand, as documented on a lifestyle device. Sneakers and ankle-socks
were worn throughout. She was afebrile and the physical exam was unremarkable apart from the lower-extremity, palpable petechial and purpuric rash. Areas covered by socks were spared. Based on her history of prolonged walking, coupled with the absence of systemic symptoms, a presumptive diagnosis of EIP was made. The patient recovered with supportive treatment.

Discussion:
Typically affecting healthy females in their 50s, EIP has been labeled in multiple contexts in the literature as Disney Rash, golfer’s rash/vasculitis, hikers’ dermatitis/purpura, jogger’s petechiae, sport-induced vasculitis, marathon runner’s vasculitis, and exercise-induced vasculitis (EIV). Initial cases of EIV are documented in the early 2000’s dermatology literature, exhibiting a common presentation of pruritic and burning lower extremity rash after prolonged walking in
warm climates. These clinical findings are often coupled with a histopathological analysis of small vessel leukocytoclastic vasculitis. Prior studies have suggested that the vasculitis is driven by cytokine release and circulating immune complex deposition. Potential EIV risk factors include chronic venous disease and lipedema due to an impairment in venous return and heat regulation. EIV is commonly misdiagnosed for stasis dermatitis, cellulitis, and systemic vasculitis. To avoid this, patients with bilateral lower extremity purpura after prolonged walking in warm weather should raise clinical suspicion for a diagnosis of benign EIV or EIP. Increased
awareness and knowledge of EIV/EIP is critical for preventing unnecessary diagnostic work-up, improper empirical treatment, or hospitalization.

Disney to Las Vegas: Benign Lower Extremity Purpura Following Prolonged Walking

Resistant hypertension is defined as uncontrolled blood pressure (BP) despite treatment with three or more medications at optimal doses. Hypertensive urgency is characterized by a severe elevation in BP without evidence of end organ damage. Ultimately, this can lead to hypertensive emergency manifesting as neurologic deficit, hypertensive retinopathy, intracranial hypertension, aortic dissection, myocardial ischemia, or hypertensive nephrosclerosis. 
A 55-year-old female with a medical history of longstanding hypertension, hypertensive cerebrovascular accident, CKD IV, hyperparathyroidism status post parathyroidectomy, and left ICA aneurysm presented to the emergency department after an office visit where her systolic BP was 260. She experienced intermittent headache, diaphoresis, and blurry vision for several months prior to presentation. Despite adherence to diet, exercise, and a regimen of losartan 50mg, carvedilol 25mg, isosorbide dinitrate/hydralazine 20-37.5mg, and nifedipine 90mg, her BP remained uncontrolled for years. Family history included accelerated hypertension, kidney disease, and stroke before age 50.
On exam, the patient was comfortable, but diaphoretic with BP 267/140, heart rate 68, respirations 16, and oxygen saturation of 99% on room air. Cardiac exam revealed S3 and S4. Extraocular movements were intact and fundoscopic exam was limited. Laboratory values revealed a creatinine 1.8mg/dL, 2+ urine protein, parathyroid hormone of 367.2 pg/mL and a calcium of 9.1mg/dL. Other values were within normal limits. Nicardipine infusion improved BP to 182/114, and she was restarted on her home regimen with continued improvement over the next several days.
Further work up for secondary hypertension revealed normal TSH and aldosterone. Abdominal imaging showed normal adrenal glands and moderate-to-severe left renal artery stenosis. Serum metanephrines were 1.2 times the upper limit of normal, and urine metanephrines were 1.4 times the upper limit of normal. 
Renal angiogram revealed proximal left renal artery stenosis and was less suggestive of fibromuscular dysplasia. A stent was placed in the left renal artery, resulting in a reduction of systolic pressure from 150-200 during hospitalization to below 130 at outpatient follow-up. 
A hypertension workup for etiologies was essential for this patient, and ultimately revealed several possibilities including MEN IIA, fibromuscular dysplasia, and renovascular hypertension. This underscores the importance of comprehensive assessment of resistant hypertension and hypertensive urgency to mitigate complications. In this patient with chronic, multi-organ injury due to hypertension, we were able to identify a reversible cause with potential benefits to morbidity. Identifying causes of hypertension can help avoid unnecessary pharmacotherapy and long-term sequelae associated with severely elevated blood pressure.

When to dig deeper: Unraveling Secondary Hypertension in the Context of Hypertensive Urgency

Kikuchi-Fujimoto disease is a form of benign necrotizing lymphadenitis also known as histiocytic necrotizing lymphadenitis. While Kikuchi disease is a globally prevalent disease, it is predominantly seen in the Asian population. Etiology can be traced back to dysregulated immune responses triggered by viral or autoimmune factors. Here we present a case report to emphasize the importance of considering Kikuchi disease and possible etiologies, highlighting the need for increased awareness and recognition of this rare condition. A 28-year-old female with a history of hypothyroidism, IBS, and GERD complained of sore throat, intermittent epigastric pain, mild heartburn, and fever and was taking PPIs without any improvement. Abdominal USG was negative for any pathology. She presented with bilateral enlarged cervical lymph nodes and worsening of symptoms 1.5 months later. Ciprofloxacin was prescribed which failed to improve the symptoms. CT scan of the neck showed an enlarged thyroid gland with diffuse lymphadenopathy. Labs showed elevated TSH, for which the levothyroxine dose was increased, despite that she still continued to have symptoms. LDH and CRP were normal. Rheumatology, oncology, and infectious disease specialists were called upon for further evaluation. Excisional Lymph node biopsy showed areas of extensive necrosis with karyorrhectic debris, surrounded by histiocytes and small lymphocytes, suggestive of necrotizing lymphadenitis, specifically consistent with Kikuchi disease. Immunophenotyping by flow cytometry demonstrated normal CD4+ and CD8+ T cell populations without any evidence of clonal lymphoid expansion. AFB and GMS stains were negative for infection. Hydroxychloroquine 200 mg twice daily was given, which resulted in the resolution of her symptoms. Kikuchi disease’s origins can be traced back to Japan, where it was initially documented. More prevalent in the young female Asian population under the age of 30. It presents as tender, enlarged nodes, primarily in the cervical region along with lymphadenopathy, fever, erythematous rash, joint pain, fatigue, arthritis, and hepatosplenomegaly. It becomes important to consider Kikuchi disease in patients presenting with symptoms that can be attributed to sore throat related to GERD, followed by the development of fever with cervical lymph node enlargement.

Unusual presentation of sore throat with bilateral lymphadenopathy in a hypothyroid patient: Kikuchi Fujimoto Disease, A case report.

Background: Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CADASIL) is a hereditary early-onset vascular disease causing recurrent ischemic subcortical infarcts. Common presentations include transient ischemic attacks (TIA) and strokes (74.36%), cognitive impairment (69.23%), psychiatric disturbance (25.64%),(25.64%) and migraines (15.38%). In the realm of medical mysteries, CADASIL stands as a perplexing and devastating hereditary disease. This case aims to highlight the unique presentation of CADASIL with facial palsy and the need for a multidisciplinary approach to management. 
Case: A 52-year-old male with a family history of strokes at a young age in his father and paternal grandfather, presented to a neurology clinic for left facial droop and drooling. Brain MRI showed extensive periventricular and subcortical white matter disease, including the external capsule and subcortical white matter of the temporal lobe. Findings were suggestive of small vessel vasculopathy. A cerebral angiogram showed that all large extra and intracranial vessels were patent without evidence of aneurysm formation. There was no obvious evidence of beading of the distal intracranial vessels. CSF studies were normal. The NOTCHNOTCH 3 mutation was sent to test for CADASIL, which came back positive. The patient was started on aspirin (aspirin 81 mg) and atorvastatin (20 mg) daily. The patient was counselled on the possibility of getting an ischemic or hemorrhagic stroke. Aspirin and atorvastatin were continued, a neuropsychological evaluation was ordered and CADASIL genetic counselling and testing was offered to him and his children. Over several years, the patient developed several strokes and seizures due to infarcts. He also developed Intraparenchymal haemorrhage complicated by dysphagia requiring a feeding tube. Due to his severe physical debility, he was discharged to a nursing home for rehabilitation, where he did not improve with therapy and remained bedbound. He was discharged home with his family.
Conclusion: CADASIL can present as a diagnostic challenge due to its common presentation with migraines, TIA, and strokes with or without risk factors. This unique presentation of CADASIL with facial palsy highlights the importance of emerging atypical presentations and the need for a detailed history of neuroimaging, family history, and personal history of neurovascular events. By accurately diagnosing the condition, patients and families can be counselled on the disease course and genetics. Management requires a multidisciplinary approach with neurology, genetic counselling, physical therapy, psychology, and psychiatry to improve the patient’s quality of life.


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Mucosal Infection with Tsukamurella Species Following Nasal Septum Procedure: A Rare Case Report

Next from 2023 AMA Research Challenge

Hypoglossal nerve stimulator (HGNS) implant infection: a feared complication for a novel treatment of obstructive sleep apnea