The Case Files

The Case Files is an anecdotal collection of emergency medicine cases to enable physicians and researchers to find clinically important information on unusual conditions.

Case reports should focus on:

    • Unusual side effects or adverse interactions.
    • Unusual presentations of a disease.
    • Presentations of new and emerging diseases, including new street drugs.
    • Findings that shed new light on a disease or an adverse effect.

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Tuesday, July 17, 2018

​BY AN​UMEHA SINGH, MD

A 56-year-old man presented to the ED with shortness of breath and pleural effusions diagnosed by an outside treating physician. The workup included urinalysis, which showed proteinuria, and the exam was consistent with myxedema and pedal edema. He had a history of rheumatoid arthritis, leukocytoclastic vasculitis and Sjögren's syndrome, diabetes, and monoclonal gammopathy of unknown significance (MGUS).

The patient also had shortness of breath for a month and pleural effusions on outpatient chest x-ray. He had taken Humira, methotrexate (MTX), and steroids, but his arthritis and Sjögren's were currently well controlled without medications. His MGUS was monitored annually with no medical intervention. He felt excessive thirst, but had normal urine output. The patient appeared well, but would not stop taking sips of water in between every sentence of a conversation despite obvious myxedema.


He noted face and hand swelling that morning, which gave way to more significant pedal edema at the end of the day and progressively worsening exertional dyspnea. He noted low-grade fever and loss of appetite. His initial vitals were a blood pressure of 185/96 mm Hg, oxygen saturation of 97% on room air, heart rate of 106 bpm, respiratory rate of 20 bpm, and temperature of 100.1°F.

The differential diagnoses for this case included an infectious process such as viral or bacterial infiltrate and lung mass or atypical presentation of myeloma given the patient's history of MGUS, but cardiorenal and nephrotic syndrome and congestive heart failure were higher on our list of concerns. Other etiologies pertaining to the thyroid, syndrome of inappropriate antidiuretic hormone secretion, autoimmune activation of rheumatoid arthritis, and vasculitis were also considered.

This patient had numerous abnormal lab results. Anemia and thrombocytopenia per hemoglobin and hematocrit of 9 g/dL and 29%, respectively, platelets at 29, WBC of 12.1, high inflammatory markers with CRP of 23 and ESR at 45; cardiac creatine kinase and troponin were normal, but NTpBNP was 12,000. The chemistries were largely normal: a creatinine of 1.1 mg/dL and BUN of 25 mg/dL. The protein labs were low: serum protein was 5.1 g/L, albumin 2.8 g/dL, globulin 2.3 g/dL, prealbumin 20 mg/dL, and random urine protein of 289 mg/dL. The patient was admitted to the medicine service with cardiology and nephrology consults. Without the inflammatory markers and urinalysis, this may have been CHF, but the patient's complicated history demanded a more careful look.

The disruption of fluid volume control often results from overlapping renal and cardiac etiologies. Some would argue that CHF is as much a symptom as a diagnosis, and the renal part of the balance should not be ignored. The relationship between the cardiac and renal systems is particularly evident when disruption of homeostasis is evident and target treatment is considered. The first noted relationship of the heart and kidney in disease state was published in 1835 by Richard Bright. (J Nephropathol 2014;3[3]:99.) Much later, cardiorenal syndrome was classified by the Consensus Conference of the Acute Dialysis Quality Group in 2008, categorizing complex pathology into two main stems—cardiac and renal. (Eur Heart J 2010;31[6]:703; http://bit.ly/2NiENPA.) Renal disease is one of the highest risks of adverse cardiovascular disease, but the reverse pathway is also noted. This suggests that new presentation needs attention with bidirectional impact on the cardio-renal spectrum.​

Nephrotic Symdrome

Prognosis and progression of nephrotic syndrome varies greatly depending on whether the syndrome is primary or secondary. Many patients with focal glomerulosclerosis may experience frequent relapses and become steroid-dependent or -resistant. Idiopathic membranous nephropathy has an excellent prognosis with rare complications or incidences of renal failure. Up to 25 percent of adults with minimal-change nephropathy treated with steroids have frequent relapses, and up to 30 percent of these patients become steroid-dependent. (Clin J Am Soc Nephrol2007;2[3]:445, http://bit.ly/2MKRUIaClin J Am Soc Nephrol 2009;4[10]:1593, http://bit.ly/2tR1lPy.) Poor patient response to steroid therapy, hematuria, and hypertension predict a poor outcome.

Infection is a major factor in the mortality rate among patients with nephrotic syndrome. Management is focused on treating the underlying pathology. Studies have failed to prove the benefit of commonly used therapies such as angiotensin modifiers, which are frequently started in patients with NS because of their anti-proteinuria action. A Cochrane review showed that combining an alkylating agent with a corticosteroid has short- and long-term benefits for membranous nephropathy. (Cochrane Database Syst Rev 2014 Oct 16;[10]:CD004293; http://bit.ly/2KDhv8D.) Immunosuppressive therapy is not needed in most adults with idiopathic NS. Other immunosuppressive treatments should be considered if NS is steroid-resistant. Treating fluid edema with diuretics is indicated, but using 20% albumin has equivocal evidence of benefit reported in the literature.

Our patient was discharged seven days after he was diagnosed with nephrotic syndrome, MGUS, cardiomyopathy, and essential hypertension. He was discharged on carvedilol, alendronate, furosemide, and spironolactone. Cardiology, hematology/oncology, infectious disease, nephrology, pulmonary, and rheumatology were consulted. The patient was not a candidate for anti-TNF agents, MTX, and prednisone was recommended. The patient did not want to start steroids until bone marrow biopsy results were reviewed.

Unfortunately, the patient was readmitted in a week with symptomatic anemia, worsening pedal edema, and pneumonia. The patient was switched to torsemide, and was started on aspirin and statin. Vancomycin and high-dose Solu-Medrol was initiated. Two units of PRBC were transfused, and the patient was started on iron and folate supplements. His symptoms improved over 18 days following readmission.

Dr. Singh is an assistant professor of emergency medicine at Hartford Hospital, University of Connecticut. She and her emergency physician-husband, Daksh Rampal, MD, own Priority Urgent Care in Ellington, CT(www.priorityuc.com).

Tuesday, July 17, 2018

​BY GREGORY TAYLOR, DO, & ​​LAURIE WALLACE, DO

A 27-year-old man with a significant past medical history of depression, multiple suicide attempts, intravenous heroin abuse, and hypertension presented to the emergency department with cardiac arrest. The patient had hanged himself with a bungee cord in his family's basement, and had been hanging for approximately 15 minutes before EMS was called. The patient was unresponsive on EMS arrival, with an initial rhythm of pulseless electrical activity (PEA). Return of spontaneous circulation (ROSC) was achieved en route following five rounds of epinephrine and cardiopulmonary resuscitation (CPR). Estimated down time was 45 minutes.

The patient went back into PEA at the ED. The King airway was replaced with an endotracheal tube, and ROSC was achieved after two rounds of CPR. Vitals included a blood pressure of 60/42 mm Hg and heart rate of 113 bpm, and he was afebrile. The patient remained unresponsive, with a Glasgow coma score of 3 and fixed and dilated pupils.

He was ill-appearing and exhibiting mottling to all extremities, with a horizontal abrasion to the base of his neck consistent with a ligature mark from hanging. A central venous catheter was subsequently placed, and vasopressors were initiated. He went into sustained ventricular tachycardia 10 minutes later, followed by ventricular fibrillation, and was subsequently defibrillated. He was given 150 mg of amiodarone, followed by an amiodarone infusion. Repeat vitals revealed a blood pressure of 104/52 mm Hg, heart rate of 117 bpm, respiratory rate of 22 bpm, and 100% pulse ox on the ventilator. A CT of the head without IV contrast (Images 1 and 2) revealed blurring of the gray-white interface with findings consistent with diffuse cerebral edema related to hypoxic-ischemic injury. A CT of the neck without IV contrast was negative for any acute process.


Image 1.


Image 2.

The initial laboratory evaluation was notable for a creatinine of 1.48 mg/dL, aspartate aminotransferase of 848 U/L (10-37 U/L), alanine aminotransferase of 1041 U/L (9-47), ammonia of 191, lactic acid of 8, and a urine drug screen for alcohol, salicylates, and acetaminophen was negative. An arterial blood gas revealed a pH of 7.28, pCO2 of 50, pO2 of 505, and an HCO3 of 14. The patient had evidence of shock liver and diffuse cerebral edema secondary to hypoxic ischemic injury. He was admitted to the ICU, and remained unresponsive to noxious stimuli.

Low Survival Rate

A 27-year-old man with a significant past medical history of depression, multiple suicide attempts, intravenous heroin abuse, and hypertension presented to the emergency department with cardiac arrest. The patient had hanged himself with a bungee cord in his family's basement, and had been hanging for approximately 15 minutes before EMS was called. The patient was unresponsive on EMS arrival, with an initial rhythm of pulseless electrical activity (PEA). Return of spontaneous circulation (ROSC) was achieved en route following five rounds of epinephrine and cardiopulmonary resuscitation (CPR). Estimated down time was 45 minutes.

The patient went back into PEA at the ED. The King airway was replaced with an endotracheal tube, and ROSC was achieved after two rounds of CPR. Vitals included a blood pressure of 60/42 mm Hg and heart rate of 113 bpm, and he was afebrile. The patient remained unresponsive, with a Glasgow coma score of 3 and fixed and dilated pupils.

He was ill-appearing and exhibiting mottling to all extremities, with a horizontal abrasion to the base of his neck consistent with a ligature mark from hanging. A central venous catheter was subsequently placed, and vasopressors were initiated. He went into sustained ventricular tachycardia 10 minutes later, followed by ventricular fibrillation, and was subsequently defibrillated. He was given 150 mg of amiodarone, followed by an amiodarone infusion. Repeat vitals revealed a blood pressure of 104/52 mm Hg, heart rate of 117 bpm, respiratory rate of 22 bpm, and 100% pulse ox on the ventilator. A CT of the head without IV contrast (Images 1 and 2) revealed blurring of the gray-white interface with findings consistent with diffuse cerebral edema related to hypoxic-ischemic injury. A CT of the neck without IV contrast was negative for any acute process.

The initial laboratory evaluation was notable for a creatinine of 1.48 mg/dL, aspartate aminotransferase of 848 U/L (10-37 U/L), alanine aminotransferase of 1041 U/L (9-47), ammonia of 191, lactic acid of 8, and a urine drug screen for alcohol, salicylates, and acetaminophen was negative. An arterial blood gas revealed a pH of 7.28, pCO2 of 50, pO2 of 505, and an HCO3 of 14. The patient had evidence of shock liver and diffuse cerebral edema secondary to hypoxic ischemic injury. He was admitted to the ICU, and remained unresponsive to noxious stimuli.

The proposed mechanism for cardiac arrest involves compression of the jugular veins leading to a venous outflow obstruction, cerebral stagnation, hypoxia, and loss of consciousness. (Injury 2006;37[5]:435; http://bit.ly/2z54akX.) With the muscle tone of the neck now decreased, an external force, like a bungee cord, tightens further, leading to compression against the cervical vertebrae, carotid artery spasm, occlusion, decreased cerebral perfusion, brain injury, and possibly death. (Injury 2006;37[5]:435; http://bit.ly/2z54akX.) The airway structures require more force than the vascular structures to occlude, and are generally not thought to play a significant role in cardiac arrest.

A retrospective review, the largest to date on hanging arrest victims, was performed from 2000 to 2009, and included 52 patients who presented to a tertiary ED with an out-of-hospital cardiac arrest secondary to hanging. (Am J Emerg Med 2012;30[5]:690; http://bit.ly/2z5AVyl.) Cardiopulmonary resuscitation was performed on 60 percent of the patients. The initial cardiac rhythm in every case was either PEA or asystole. Thirteen (42%) of the patients who underwent resuscitation achieved ROSC, and five survived till hospital discharge. The conclusion drawn was that with the initial rhythm being nonshockable, the survival rate was only 9.6 percent, but the survival rate was 38.5 percent for the 13 who achieved ROSC.

The current literature has not shown a benefit to adopting a hypothermic protocol in these patients. The study authors utilized one on 10 of the 13 patients who achieved ROSC, with the majority dying from brain death and multiorgan failure. (Am J Emerg Med 2012;30[5]:690; http://bit.ly/2z5AVyl.) The few who survived had a poor neurologic outcome. Only one case has been reported of a patient treated with a hypothermic protocol post-hanging who made a complete neurologic recovery. The hanging time in that case, however, was only several minutes, and EMS arrived within five minutes, with ROSC being achieved soon after.

A GCS of 3, hanging time more than 30 minutes, CPR on scene, and a pH under 7.3 are associated with a poor outcome. (Am J Emerg Med 2004;22[3]:207; http://bit.ly/2KtyIlN.) The mortality rate in another study of 42 patients who attempted suicide and presented to the ED with a GCS of 3 in cardiac arrest was still 100 percent even with maximal intervention and treatment. (Resuscitation 2002;54[1]:27; http://bit.ly/2tP1L8V.) The authors concluded that the hypothermic protocol should be applied only to hanging cardiac arrest patients when the arrest time is short. (Am J Emerg Med 2012;30[5]:690; http://bit.ly/2z5AVyl.)

Our patient had no evidence of cortical or brainstem function or spontaneous movements by day two. The family elected to terminally wean, and he died shortly after.​

Dr. Taylor is completing his emergency medicine residency at Beaumont Hospital in Farmington Hills, MI, a teaching hospital of Michigan State University. He also serves as a captain and physician in the United States Air Force. Dr. Wallace is on staff at Beaumont Hospital as core faculty of the emergency medicine residency and the director of student clerkship.

Wednesday, July 11, 2018

​BY GREGORY TAYLOR, DO, & JACKLYN MCPARLANE​, DO

A 31-year-old woman with a significant medical history for one ectopic pregnancy and five spontaneous abortions presented with abdominal pain and vaginal bleeding. Her symptoms started abruptly two days earlier, and the pain was located in the right lower quadrant, which had become diffuse and she described as sharp. She was also experiencing nausea, vomiting, and vaginal bleeding.

She stated she had been spotting for five days and soaking up to four pads a day. Her last menstrual period was two months before, and a home pregnancy test was positive. Her presentation was complicated by no obstetrics/gynecology follow-up, no prenatal care, and no prior ultrasound.

The patient was afebrile. Her blood pressure was 89/70 mm Hg, her heart rate was 116 bpm, and her respiratory rate was 22 bpm. She was in distress, lying flat, not wanting to move, diaphoretic, and crying in pain. An abdominal exam revealed a moderately distended abdomen, diffuse tenderness with associated rebound, guarding, and percussive tenderness. A FAST exam revealed free fluid in the right upper quadrant, left upper quadrant, and posterior cul-de-sac. The history and physical exam was consistent with likely hemorrhagic shock secondary to a ruptured ectopic pregnancy.

The patient was typed and screened, and fluid resuscitation was initiated. Obstetrics and gynecology was emergently consulted. A repeat blood pressure after two liters of normal saline and one unit of packed RBCs was 125/91 mm Hg, and her heart rate came down to 86 bpm. Laboratory values were remarkable for a hemoglobin of 10, hematocrit of 29.9, and a serum-beta hCG of 12,692. A bedside emergent transvaginal ultrasound revealed no evidence of intrauterine pregnancy, but did reveal a complex mass measuring 1.8 x 3.0 x 2.3 cm with peripheral vascularity (Image A), in addition to moderate-large complex fluid in the posterior cul-de-sac and right adnexa. (Image B.) She was taken to the operating room for a diagnostic laparoscopy, where she underwent a right salpingectomy and evacuation of 1,400 cc of hemoperitoneum. (Image C.) Her hospital course was unremarkable. She remained hemodynamically stable, and was discharged home on post-operative day two.

case files-mcparlane.jpg

Image A. A bedside emergent transvaginal ultrasound revealed a complex mass measuring 1.8 x 3.0 x 2.3 cm with peripheral vascularity.

case files-mcparlane2.jpg

Image B. The transvaginal ultrasound also showed moderate-large complex fluid in the posterior cul-de-sac and right adnexa.​


Image C. The patient underwent a right salpingectomy and evacuation of 1,400 cc of hemoperitoneum.​

Ectopic Pregnancies

The Fallopian tube is the most common location of about 98 percent of all ectopic pregnancies; other locations like the cervix, ovary, abdomen, and Fallopian tube interstitium are rare. (Merck Manual, October 2017; https://mrkmnls.co/2HPzTWw.) Clinical research has shown that the structure containing the fetus ruptures between six and 16 weeks. The prevalence among patients who present to the ED with first-trimester vaginal bleeding or pelvic pain ranges from six to 16 percent. (UpToDate, October 2013; http://bit.ly/2laekXN.) An ectopic pregnancy can eventually lead to hemorrhage and shock, and becomes the leading cause of maternal mortality in the first trimester, accounting for up to 10 percent of all pregnancy-related deaths. (UpToDate, October 2013; http://bit.ly/2laekXN.)

Risk factors include previous ectopic pregnancy (15%), history of pelvic inflammatory disease, prior tubal surgery, intrauterine device, infertility, in vitro fertilization, multiple sexual partners, cigarette smoking, and prior induced abortion. It is important to note that more than 50 percent of patients are asymptomatic before tubal rupture and have no identifiable risk factors. (UpToDate, May 2, 2017; http://bit.ly/2JJD4oE.)

One retrospective study involving 2,026 pregnant patients who presented to the ED with abdominal pain and first-trimester vaginal bleeding diagnosed 18 percent (376 patients) with an ectopic pregnancy. Sixty-six percent of those 376 patients had abdominal pain and 76 percent had vaginal bleeding, making those the most common clinical presenting symptoms. (UpToDate, May 2, 2017; http://bit.ly/2JJD4oE.) Clinical research has shown no pathognomonic bleeding pattern for an ectopic pregnancy because vaginal bleeding varies from scant brown to spotting to hemorrhage. (UpToDate, May 2, 2017; http://bit.ly/2JJD4oE.)

Abdominal pain is usually described as a lower pelvic pain, but a patient may present with diffuse abdominal pain or even referred shoulder pain if intraperitoneal blood reaches the upper abdomen. No specific pain pattern is associated with ectopic pregnancy either; it ranges from acute to slow, and can be continuous or intermittent and often described as dull or sharp. (UpToDate, May 2, 2017; http://bit.ly/2JJD4oE.) Our case demonstrated that typical findings of a ruptured ectopic pregnancy include diffuse abdominal pain from intraperitoneal blood and potential hemorrhagic shock.

Treatment

The differential diagnosis of vaginal bleeding in pregnancy includes but is not limited to threatened miscarriage, spontaneous abortion, subchorionic hematoma, and cervical, vaginal, and uterine pathology. An ectopic pregnancy should be suspected in any girl or woman of reproductive age presenting with pelvic pain, vaginal bleeding, unexplained syncope, or even hemorrhagic shock. (Merck Manual, October 2017; https://mrkmnls.co/2HPzTWw.) A ruptured ectopic pregnancy is a surgical emergency, and a prompt diagnosis is essential to prevent maternal hemorrhage and death.

Lab testing includes a urine beta-hCG and, if positive, a quantitative serum beta-hCG and a transvaginal ultrasound. An ectopic pregnancy is exceedingly rare if an intrauterine pregnancy is identified on ultrasound. Though rare, sometimes a patient who has undergone in vitro fertilization will present with a heterotopic pregnancy (simultaneous intrauterine and ectopic pregnancy). A serum beta-hCG above the discriminatory zone, about 2,000, usually results in visualization of the intrauterine pregnancy. An ectopic pregnancy is a possibility if it does not. Patients may have a potential ectopic pregnancy or an early intrauterine pregnancy if the serum beta-hCG is below the discriminatory zone and a gestational sac is not visualized. These particular patients, if stable, are often discharged home with strict return precautions and a repeat serum beta-hCG in 48 hours because the serum beta-hCG does not usually double every 48 hours. Diagnosis of a rupture is based on findings of abdominal pain and echogenic fluid within the cul-de-sac or abdomen, indicating hemoperitoneum. A patient who is hemodynamically unstable requires immediate laparotomy; those who are stable often undergo laparoscopy. Surgical procedures include salpingectomy and salpingostomy, with salpingectomy being the most common surgical procedure when the Fallopian tube has ruptured. Methotrexate is sometimes used in patients who have an unruptured tubal pregnancy that is <3 cm in diameter, has no fetal heart tones, and preferably has a serum beta-hCG <5,000. Our patient was temporarily stabilized in the ED, went for a diagnostic laparoscopy and right salpingectomy, and was discharged on day two.

Dr. Taylor is an emergency medicine resident at Beaumont Hospital in Farmington Hills, MI, a teaching hospital of Michigan State University. Dr. McParlane is an emergency physician at Beaumont Hospital and a clinical professor at Michigan State University. She was previously the program director of the emergency medicine residency program.

Tuesday, June 26, 2018

​BY GREGORY TAYLOR, DO, & JACKLYN M​CPARLANE, DO​

A 33-year-old woman with a past medical history of sickle cell SS presented to the emergency department with chest pain, difficulty breathing, and a cough for two days. Her chest pain was diffuse, without radiation, and partially reproducible. Her cough was nonproductive, and she also reported fever and chills.

The patient noted this was different from her normal back and leg pain from past sickle cell crises. She was following up with a sickle cell specialist, and was compliant with her hydroxyurea treatment.

Her temperature was 102.8°F, blood pressure was 94/60 mm Hg, heart rate was 108 bpm, respiratory rate was 26 bpm, and oxygen saturation was 98% on 2 L nasal cannula. She appeared uncomfortable and in distress with slight diaphoresis. Respiratory exam revealed scattered rhonchi throughout her lung fields. Cardiovascular exam showed tachycardia with a regular rhythm but no murmurs, gallops, or rubs. ECG revealed sinus tachycardia.

A complete blood count showed a leukocytosis of 30.3 and hemoglobin of 7.1. Her reticulocyte count was elevated at 12%. All other labs were unremarkable. A chest x-ray showed bilateral mid- and lower lung airspace disease, small bilateral effusions, and a stable left subclavian port. (Fig. 1.)

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​Figure 1.

Acute chest syndrome may ultimately be the result of sickling within the microvasculature leading to pulmonary infarction, pulmonary embolism, viral pneumonia, or bacterial pneumonia. Acute chest syndrome may develop during a vaso-occlusive pain crisis or as an isolated event. It is the leading cause of morbidity and mortality in patients with sickle cell disease, with early recognition and treatment of paramount importance. None of the hemoglobinopathies is as catastrophic as acute chest syndrome. Roughly 50 percent of patients with sickle cell disease will have at least one episode of acute chest syndrome in their lifetime, with a recurrence rate of nearly 80 percent.(Blood 1994;84[2]:643.)

Two criteria must be met to make the diagnosis of acute chest syndrome: a new pulmonary infiltrate must be present on imaging, in addition to fever, tachypnea, chest pain, cough, wheezing/rales, intercostal retractions, use of accessory muscles of respiration, or hypoxia. (Blood 1994;84[2]:643.) Because pneumonia and the infiltrate of acute chest syndrome cannot be reliably distinguished from one another and because acute chest syndrome has infectious and noninfectious causes, patients should be placed on broad-spectrum antibiotics to account for the possibility of an underlying infection.

Among the infectious causes are Chlamydia pneumoniae, Mycoplasma pneumoniae, respiratory syncytial virus, Staphylococcus aureus, and Streptococcus. (N Engl J Med 2000;342[25]:1855.) Noninfectious causes include pulmonary fat embolism, fluid overload, hypoventilation, bone infarction, and in situ thrombosis. (N Engl J Med 2000;342[25]:1855.)

Pleuritic chest pain and shortness of breath have been shown to be the most common presenting complaint in adults with acute chest syndrome, while cough, tachypnea, fever, and abdominal pain are often seen in infants and children. It is important to realize that no pulmonary infiltrates may be evident on initial chest radiography. A study by Taylor, et al., found that 37 percent of 45 patients with acute chest syndrome had a normal initial ED examination, making high clinical suspicion important in patients who do not present with typical signs and symptoms of acute chest syndrome. (Postgrad Med J 2004;80[944]:346.) Acute chest syndrome does not develop for 48-72 hours following an episode of pain in some patients. These patients often had acute chest syndrome before or have atelectasis or pleural effusions on initial imaging.

The differential diagnosis for acute chest syndrome is broad, including but not limited to pulmonary embolism, acute coronary syndrome, pneumonia, acute sickle cell multiorgan failure syndrome, and aplastic crisis. Acute sickle cell multiorgan failure syndrome is considered a severe life-threatening complication of pain episodes, and is defined as an acute deterioration in function of two of three organ systems, including lung, liver, and kidney. (Am J Med 1994;96[2]:155.) These patients often present with fever, decrease in hemoglobin and platelets, encephalopathy, and rhabdomyolysis.

A patient with an aplastic crisis often presents with a viral prodrome and in shock, has an acute severe drop in hemoglobin, and has a reticulocyte count that is dramatically decreased or even zero. The treatment for these life-threatening and emergent conditions is an exchange transfusion.

All patients with suspected or impending acute chest should be admitted. Patients should receive oxygen if hypoxic, fluids if dehydrated, bronchodilators to improve peak expiratory flow rates, incentive spirometry, and pain control. Antibiotics should cover typical and atypical bacteria. Because acute chest syndrome and pneumonia cannot be clinically distinguished from one another, treatment for them must begin simultaneously. Hydroxyurea should be considered because it has been shown to decrease the incidence of pain episodes, acute chest syndrome, and the number of transfusions. Influenza and pneumonia vaccines also should be administered, and the patient should receive a simple or an exchange transfusion.

The transfusion of packed RBCs in these patients is life-saving. Patients are at extremely high risk of vaso-occlusive events secondary to the high concentration of hemoglobin S. A transfusion reduces the percentage, with a goal of less than 30%. The hemoglobin S percentage prior to the exchange transfusion was 83.3% and post-exchange transfusion revealed a hemoglobin S of 17.9% in our patient. Some of the top indications of a transfusion for life-threatening sickle cell disease includes stroke, acute chest syndrome, acute multiorgan failure syndrome, presence of neurologic symptoms, hypoxia unresponsive to supplemental oxygen, multilobar involvement, history of severe cardiopulmonary disease, and evidence of aplastic crisis. (Hematol Oncol Clin North Am 2005;19[5]:803.)

The disease process in our case was recognized promptly based on clinical presentation and supporting diagnostic workup, and our patient underwent appropriate treatment. Repeat chest x-ray on day eight revealed improved aeration of the lung bases and resolution of the airspace disease. (Fig. 2.) Our patient was started on broad-spectrum antibiotics, maintained on folic acid daily to avoid aplastic crisis, and continued hydroxyurea treatment. She was transferred to the ICU where she underwent Quinton catheter placement with a subsequent 6-unit exchange transfusion. Hemoglobin electrophoresis revealed a hemoglobin S of 83.3%. Hemoglobin electrophoresis post-exchange transfusion showed a hemoglobin S of 17.9%. The patient continued to improve, and was discharged home on day 14.​


​Figure 2.

Dr. Taylor is an emergency medicine resident at Beaumont Hospital in Farmington Hills, MI., a teaching hospital of Michigan State University. Dr. McParlane is an emergency physician at Beaumont Hospital and a clinical professor at Michigan State University. She was previously the program director of the emergency medicine residency program.

Tuesday, June 19, 2018

​​BY STUART ETENGOFF, DO, & ABDULLAH ​BOKHARI​, AB, DO

A 20-year-old Caucasian man presented via EMS with a chief complaint of withdrawal from ketamine and secondary complaints of abdominal pain, blood in his urine, and painful urination with urgency for two days.

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He said he had been using ketamine intravenously daily for the past five days, up to 35 grams over the past week. His last use was 24 hours prior to presentation to the ED. He stated that he had been using ketamine regularly for four years and that he has used it intravenously, orally, and intranasally.

He reported a history of ADHD and a family history of bipolar disorder and substance abuse. His mother had died two years earlier from an opiate overdose. He said he used ketamine daily, and had also used marijuana, cocaine, opiates, nitrous oxide, heroin, mushroom, tobacco, and occasionally alcohol.

The patient described his abdominal pain as sharp and crampy, radiating from the bladder upward with associated nausea for two days. He denied flank pain, recent trauma, any history of previous abdominal pain or urinary tract infection, fever, chills, discharge, diarrhea, constipation, chest pain, shortness of breath, and blood in stool. He also had no previous history of hematuria, kidney stones, or sexually transmitted diseases.

He appeared to be in mild to moderate discomfort and anxious, and was alert and oriented to person, place, and time. His blood pressure was 143/95 mm Hg, pulse 111 bpm, respiratory rate 18 bpm, temperature 96.6°F, and oxygen saturation of 97% on room air. His pupils were equal, round, and reactive to light and accommodation, and no nystagmus was noted.

The patient had no focal neurological deficits or acute respiratory distress, but was tachycardic. His lower extremities were negative for calf tenderness or pain, and multiple injection sites of various age noted. He had no signs of infection or cellulitis. His abdomen was soft, with minimal tenderness; no guarding or rigidity noted.

The patient's labs were a sodium 137 of mmol/L, potassium of 3.6 mmol/L, chloride of 98 mmol/L, glucose of 122 mg/dL, BUN of 12 mg/dL, creatinine of 0.9 mg/dL, GFR of 108 mL/min, and total CPK of 1769 U/L. His troponin was negative, calcium was 8.8 mg/dL, albumin 3.2 g/dL, total bilirubin 0.3 U/L, WBC 7.9 K/UL, RBC 4.9 M/UL, hemoglobin 14.9 g/dL, HCT 42.1%, platelet 396 K/UL, neutrophil 76.6%, and lymphocytes 14.1%. A standard urine drug screen was negative.

His urine analysis was cloudy and yellow, with 100 mg/dL of protein, trace ketones, no glucose, trace leukocyte esterase, and large blood, which showed microscopic hematuria with 50-100 RBC/HPF, 5-10 WBC/HPF, and trace bacteria. A urine culture was negative after 48 hours.


Urinalysis of patient with ketamine withdrawal.

The patient was treated symptomatically with IV rehydration and antianxiolytics, and he was observed in the ED for approximately four hours. He was subsequently discharged with directions to follow up with urology, and given information about substance abuse and rehab facilities in the area.

Ketamine-induced cystitis is a rare but increasing phenomenon. First described in 2007, it is usually seen in chronic users, recreational and prescribed. (Urology 2007;69[5]:810.) The number of case appears to be increasing because of ketamine's popularity recreationally as a club drug (especially in Asia) and increased off-label use for depression and chronic pain. Reports put its incidence in chronic ketamine users at up to 30 percent. (Urol Sci 2015;26[3]:153.) The studies have been limited, however, and the true incidence is difficult to report. The exact mechanism is unknown but thought to be caused by ketamine and its metabolites being excreted in the urine (85%), leading to inflammation. It is available in liquid, powder, tablet, or capsule form.

Complications to the urinary tract can include small painful bladder, urinary incontinence, upper urinary tract obstruction, ureteral hydronephrosis, reflux nephropathy, and papillary necrosis. (J Pharmacol Clin Toxicol 2017;5[7]:1094.)

Cystoscopy findings include inflammatory changes, neovascularization, ulceration, and a small, contracted bladder. Complications include renal impairment with raised serum urea and creatinine levels. Current treatment consists of cessation of ketamine. NSAIDs, acetaminophen, and local bladder anesthetics such as phenazopyridine can be used for symptomatic treatment. Pentosan polysulfate sodium, chondroitin sulfate, and hyaluronic acid have been reported as effective treatments. (Addict Res Ther 2017;1[1]: 1002.)

It appears that the majority of symptoms will improve by stopping ketamine use. The higher the dose and the longer the duration of usage increase the risks of long-term urinary and bladder problems.​

Dr. Etengoff is the director of emergency services at the McLaren Emergency Department in Clarkston, MI. Dr. Bokhari is the senior emergency medicine resident at the McLaren Oakland Emergency Department in Pontiac, MI.