Metformin toxicity in the intensive care unit: A case series and review of the literature.

Metformin toxicity is a life-threatening condition with high morbidity and mortality. Toxicity predominantly occurs in the setting of acute renal dysfunction, as the drug is solely eliminated by the kidneys. While this risk is widely known to clinicians, diagnosing metformin toxicity is challenging because commercially available serum metformin levels require days to weeks to result. Therefore, the intensivist must rely on medical history, clinical presentation, and routine laboratory findings to make the preliminary diagnosis. Treatment of metformin toxicity includes supportive fluid hydration, vasopressors, and emergent hemodialysis (HD). We report three critically ill patients who had near-fatal severe metformin-induced lactic acidosis. Their metformin levels were markedly higher than the toxicity threshold reported by the Federal Drug Agency. These patients made a prompt and complete recovery after the initiation of HD. We also review the pathophysiology, clinical presentation, diagnosis, and treatment of metformin toxicity.

Fatal hemorrhagic bronchopneumonia caused by Bordetella bronchiseptica in an immunocompetent patient.

Introduction: Bordetella bronchiseptica is a rare cause of hemorrhagic bronchopneumonia. Important to the clinician is a clear understanding that the treatment of this rare organism differs greatly from the successful antibiotic treatment of the more common Bordetella species, pertussis and parapertussis. Case report: A 64-year-old female presented to the emergency department after experiencing one week of worsening hemoptysis. Upon admission, she was afebrile and all initial laboratory test results were normal. Bronchoalveolar hemorrhage suggested by radiographic imaging was confirmed by bronchoscopy. Bronchoalveolar lavage (BAL) cultures contained unspeciated Bordetella. Rapid worsening of the hemoptysis led to intubation and the decision to perform bronchial artery embolization. However, the intensity of the hemoptysis persisted. Septic shock ensued despite treatment with broad spectrum antibiotics including azithromycin, vancomycin, and cefepime. The microbiological speciation results finalized shortly after the patient's death. The identified organism was B. bronchiseptica. Conclusions: Although macrolide antibiotics are first line treatment for B. pertussis and parapertussis, macrolide antibiotics are generally not effective against B. bronchiseptica. Clinical suspicion of B. bronchiseptica infection should prompt consideration of alternative antibiotics known to be effective against this rare species, including carbapenems and fluoroquinolones. The use of these latter antibiotics may advisably be considered as an empirical treatment during the delay of microbiological speciation.

Extrinsic lipoid pneumonia due to chronic polyethylene glycol consumption: A case report.

Extrinsic lipoid pneumonia (ELP) results from the aspiration of lipid-containing substances. Tissue or cell histopathology after Oil-Red-O staining can confirm the diagnosis, which requires proper tissue handling and preparation during bronchoscopy. Here, we report a case of ELP in a quadriplegic patient with a long history of dysphagia and polyethylene glycol consumption. Computed tomography (CT) of the chest revealed multiple, progressively enlarging, fat-attenuated, nodular pulmonary lesions. Bronchoscopy with bronchoalveolar lavage (BAL) and a transbronchial forceps biopsy confirmed the diagnosis of lipoid pneumonia. We discuss the clinical, radiological, and pathological features of ELP and highlight the preparatory steps required for obtaining a successful diagnosis.

Selective IgG2-deficiency in yellow nail syndrome.

IgG2-deficiency increases susceptibility to recurrent pulmonary infections and the risk for bronchiectasis. Isolated IgG2-deficiency has not been previously described in Yellow Nail Syndrome (YNS).

Prolidase deficiency: A novel PEPD missense variant in exon 2.

Prolidase deficiency is an autosomal recessive disease that causes impaired collagen degradation. Altered collagen homeostasis results in the intracellular accumulation of imidodipeptides, which contain proline and hydroxyproline. The many clinical manifestations of prolidase deficiency include dysmorphic facial features, skeletal deformities, hepatosplenomegaly, necrotizing skin ulcers, and recurrent infections. Current clinical knowledge of this genetic disease relies upon few case reports due to its extreme rarity. Diagnosis is dependent on the detection of a pathologic gene variant. Additional diagnostic confirmation may be provided by urine amino acid quantification or reduced in vitro prolidase activity. We present a case of prolidase deficiency caused by a novel variant manifested by skeletal malformations and lifelong multisystemic infections. Genetic testing revealed a homozygous missense variant in the PEPD gene at nucleotide position 200, whereby adenine was replaced by guanine (c.200A > G). The corresponding amino acid change replaced glutamine with arginine at codon 67 (p.Gln67Arg). After boiling the urine sample for hydrolysis, quantitative urine amino acids demonstrated a markedly elevated proline level, confirming the diagnosis. We also provide a discussion of the pathophysiology, clinical manifestations, diagnostic testing, and clinical management of this disease.

Acquired Portosystemic Shunts in Cirrhosis and Portal Vein Thrombosis: A Case Report.

Acquired portosystemic shunts (PSS) are abnormal blood vessels that develop between the portal vein and systemic circulation as a result of portal hypertension. Recurrent hyperammonemic encephalopathy in our 62-year-old patient with cirrhosis and chronic portal vein thrombosis led to the discovery of an extremely rare and functioning portosystemic shunt (PSS). The PSS connected the inferior mesenteric and left renal veins. Such shunts are considered pathological structures and may require surgical intervention. The large PSS reported herein likely provided decompression of the portal hypertension. The concurrence of portal vein thrombosis clearly precluded any consideration of surgery. Therapeutic management in each instance of these shunts requires a full understanding of their origination, location, and physiologic implications.

Differential Diagnosis of Multiple Systemic Aneurysms.

Atherosclerosis and systemic hypertension are the most common pathogeneses of solitary acquired arterial aneurysms. The rare occurrence of multiple synchronous or metachronous arterial aneurysms requires considering alternative underlying causes. We present the unusual case of a male patient who sequentially developed multiple co-existing arterial aneurysms between the ages of 51 and 59. The sites of involvement included high-pressure systemic arteries and low-pressure pulmonary arteries. We discuss the broad differential diagnosis that includes heritable and non-inheritable etiologies. A keen clinical awareness of this broader array of arterial aneurysms is essential for accurate early diagnosis and proper management.

Congenital Porto-Azygous Shunt (Abernethy Malformation Type II) in an Elderly Patient: A Too-Often-Forgotten Occult Abnormality.

Congenital extrahepatic portosystemic shunts (CEPS) cause portal blood to circumvent the liver and its metabolism, allowing normally detoxified ammonia to accumulate in the systemic circulation. Hyperammonemia in the elderly often manifests clinically as toxic encephalopathy. We present a case of recurrent altered mental status in a 70-year-old patient that eluded diagnosis over several years. Hyperammonemia was the sole abnormality detected upon a thorough liver function evaluation prompted by the patient's history of remote liver disease. Enhanced computed tomography revealed an extrahepatic porto-azygous shunt arising from a hypoplastic portal vein. This case illustrates that, albeit rare, CEPS may express themselves for the first time in the elderly, a patient population that is frequently afflicted by many more common causes of altered mental status. CEPS should be considered in the differential diagnosis of inexplicable hyperammonemia in this age group.

Anti-3-Hydroxy-3-Methylglutaryl Coenzyme A Reductase (Anti-HMG CoA) Myopathy With Cardiac Involvement: Presentation, Diagnosis, and Management.

Immune-mediated necrotizing myopathy (IMNM) is categorized into three groups: anti-3-hydroxy-3-methylglutaryl coenzyme A reductase (HMGCR) IMNM, anti-signal recognition particle (SRP) IMNM, and seronegative IMNM. Cardiac involvement has been reported in a significant segment of patients with IMNM of the anti-SRP type. Emerging evidence now suggests that cardiac involvement is also implicated in the anti-HMGCR subgroup. In this report, we present a case of anti-HMGCR IMNM with cardiac involvement demonstrated by elevated troponin levels, a low ejection fraction of 40%, and regional wall motion abnormalities in the inferior, inferolateral, anteroseptal, inferoseptal, and anterolateral myocardial walls, as visualized on echocardiography. These findings markedly improved after treatment with intravenous immunoglobulin (IVIG) and prednisone. This case and other recent reports highlight the need for a cardiac workup in patients diagnosed with anti-HMGCR IMNM.

Massive empyema due to Lactococcus garvieae.

Introduction: Lactococcus garvieae, a zoonotic pathogen, may rarely infect humans through the consumption of fish. Documented manifestations of L. garvieae infection in humans include infective endocarditis, prosthetic joint infections, liver abscesses, peritoneal dialysis-associated peritonitis, osteomyelitis, meningitis, infective spondylodiscitis, acalculous cholecystitis, and urinary tract infection. Case report: An 87-year-old female was hospitalized for coffee-ground emesis secondary to acute gastritis after eating cooked fish. One week after her discharge, she developed new-onset confusion and was returned to the hospital. Chest computed tomography revealed total consolidation of the left lung and a multiloculated left pleural effusion. The patient required intubation and direct admission to the intensive care unit. Pleural fluid and blood cultures grew L. garvieae, which was susceptible to ceftriaxone, penicillin, and vancomycin. Despite intensive antibiotic therapy and supportive care for thirteen days, the patient remained in irreversible shock, and the family opted for comfort care. Conclusions: Heretofore unreported, this case demonstrates that L. garvieae can cause bronchopneumonia and empyema.

Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia (DIPNECH): A Case of Indolent Pulmonary Nodules Diagnosed with Robotic-Assisted Navigational Bronchoscopy.

Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is an atypical pulmonary disorder with limited understanding. Given the rare nature of this disease, it is essential to obtain adequate tissue pathology to confirm the diagnosis. This disease is mainly diagnosed in middle-aged, nonsmoking females, and it is now accepted as a precursor lesion to pulmonary carcinoid tumors. DIPNECH presents with characteristic radiographic and histologic findings, but its diagnosis, management, and prognosis are often underrecognized and poorly understood. Those with symptoms may present with shortness of breath, wheezing, and persistent cough and are often misdiagnosed with reactive airway disease. Pulmonary function testing may reveal airflow obstruction and air trapping. Imaging is characterized by multiple lung nodules, typically less than 5 mm in size, with a background mosaic attenuation on computed tomography imaging. Histologically, DIPNECH can be suspected based on the presence of hyperplastic neuroendocrine cells. DIPNECH is considered a precursor to invasive neuroendocrine tumor, and up to 50% of patients may have a well-differentiated neuroendocrine tumor at the time of presentation. Here, we present the case of a 46-year-old female with a history of ulcerative colitis on mesalamine who presented with a 6-month history of ongoing shortness of breath, chest tightness, wheezing, and cough. She was initially diagnosed with asthma before imaging later revealed as multiple pulmonary nodules with a diffuse mosaic pattern. Using robotic-assisted navigational bronchoscopy, she underwent sampling of a dominant 1.8 cm right middle lobe pulmonary nodule and pathology was consistent with low-grade neuroendocrine tumor.

Pathogenesis and therapy of arteriovenous malformations: A case report and narrative review.

Arteriovenous malformations (AVMs) are abnormal communications between arteries and veins that lack intervening capillary beds. They have been described in almost every organ in the body, emerging sporadically or as part of well-described syndromes. Hereditary hemorrhagic telangiectasia (HHT) is a rare, progressive, and lifelong disease characterized by AVMs and recurrent hemorrhaging. In the last 2 decades, significant advances have been made in understanding the pathogenesis of this condition. The accumulation of knowledge has led to a natural evolution of therapy, from open surgery to endovascular procedures, and now to a role for medications in certain AVMs. Here, we review a case of HHT and describe the most up-to-date clinical practice, including diagnosis of HHT, subtypes of HHT, and medical therapy.

Myocarditis in COVID-19 presenting with cardiogenic shock: a case series.

BACKGROUND: SARS-CoV2, also known as COVID-19, is a specific strain of coronavirus that is responsible for an ongoing global pandemic. COVID-19 primarily targets the respiratory system via droplet transmission, causing symptoms similar to influenza, including fever, cough, and shortness of breath. It is now known to impact other organ systems, causing significant cardiovascular and gastrointestinal illness, among others. CASE SUMMARY: We describe two cases of COVID-19 induced myocarditis presenting with cardiogenic shock. These cases highlight the importance of understanding the lethal cardiac complications of COVID-19 infection, as well as its presentation, diagnosis, pathophysiology, and potential treatment options. These two cases involve patients without underlying cardiovascular disease risk factors who experienced prolonged symptoms of COVID-19 infection. Both patients presented with cardiogenic shock more than one week after symptom onset and diagnosis. These cases demonstrate the late presentation of myocarditis and cardiogenic shock, treated with corticosteroids and inotropes, with subsequent recovery of cardiac function. DISCUSSION: The cases highlight the importance of recognizing late presentation viral myocarditis secondary to COVID-19 infection, even in patients without underlying cardiac disease.

Shortness of Breath in a 38-Year-Old Woman With Pulmonary and Hepatic Nodules.

CASE PRESENTATION: A 38-year-old African American woman with a history of menometrorrhagia on previous estrogen therapy and a previously biopsied benign thyroid nodule with recent interval enlargement presented with symptoms of shortness of breath on exertion, an intermittent nonproductive cough, and right upper quadrant abdominal pain for 1 year. She denied wheezing, hemoptysis, fevers, night sweats, or unintentional weight loss. Socially, the patient was a lifelong nonsmoker and denied alcohol or drug use. Travel history was not significant, and she had no contributory occupational, environmental, or animal exposures. Recent cancer screening that included Papanicolaou smear and mammography were negative for neoplasia. Vital signs were normal, and ambulatory pulse oximetry did not demonstrate evidence of oxygen desaturation. Physical examination demonstrated normal respiratory effort, diffuse vesicular breath sounds, and a soft abdomen without hepatomegaly or right upper quadrant tenderness.

Auditory Hallucinations as a Rare Presentation of Occipital Infarcts.

A stroke is a clinical syndrome characterized by a focal neurologic deficit that can be attributed to a vascular territory within the brain. The presenting features of an acute stroke depends on the area of the brain affected. Although unusual, the presenting feature may include psychosis with auditory and/or visual hallucinations. A 56-year-old female was admitted to the psychiatric unit after threatening her husband with a knife. She reported experiencing altered sensorium for one week with suicidal and homicidal command hallucinations. Given the acute onset, brain images were obtained to rule out an organic etiology. A brain MRI revealed an acute right occipital lobe infarct with hemorrhagic transformation. The patient's symptoms were self-limited, resolving without antipsychotic medications. Psychosis with auditory hallucinations is not commonly reported following stroke. Since histologic and functional alterations in the occipital lobe appear to play a significant role in psychosis of schizophrenics, it is likely that ischemia in the same area may cause similar changes. Familiarity with this rare presentation is important, as it prevents a delay in diagnosis, which may negatively impact the outcome.