Brother’s Stem Cells Make Remission Possible for Pediatric Leukemia Patient


How do you repay someone who has given you the gift of life? Eight-year-old Emma Duffin of Enfield, Connecticut, started by giving a kiss and cuddle to her brother, Alexander, who donated his bone marrow stem cells to Emma to reboot her immune system and send her rare form of leukemia into remission.

Emma’s journey to a stem cell transplant began in April 2014, when her usual energetic demeanor began to change. “Emma was very vibrant, very active, and she did not like to rest,” says her father, Brian Duffin. But suddenly his go-go daughter was exhausted all the time. She was diagnosed with strep throat, then foot-and-mouth disease, but neither medication nor time brought any improvement.

During yet another trip to the emergency room, a blood draw revealed Emma had an alarmingly low level of hemoglobin – a 3 instead of the normal range for a juvenile of 11 or higher.

“For an adult, such low levels would have been fatal,” Brian says. Emma was rushed to Connecticut Children’s Medical Center (CCMC), where she was diagnosed a few days later with acute undifferentiated leukemia.

Most leukemias fall into two types: acute lymphocytic leukemia (ALL) or acute myeloid leukemia (AML). Each type is treated with a distinct protocol. Acute undifferentiated leukemia is a subset of the disease that shows markers of both types.

“As a result, part of the leukemia is often resistant to one protocol or the other,” explains Steven Margossian, MD, PhD, a senior physician of pediatric hematology and oncology at Dana-Farber/Boston Children’s Cancer and Blood Disorders Center. “In this circumstance, the best approach is often a bone marrow stem cell transplant. The bone marrow is the factory where leukemia cells are made. By using strong chemotherapy to destroy the existing bone marrow cells, you can then replace those with normal, healthy cells from a donor as closely matched to the patient as possible.”

In Emma’s case, Alexander ended up being her perfect match.

Emma’s oncologist at CCMC trained under Dr. Margossian and referred the family to her mentor.

“When your doctor tells you the number one pediatric stem cell transplant hospital in the world is only 90 minutes from your house, you don’t question it,” says Allyson Duffin, Emma’s mom.

After Emma completed three rounds of chemotherapy at CCMC, the Duffin family traveled to Boston to prepare for “zero day”: the day Dr. Margossian would perform the stem cell transplant. Prior to the transplant, Emma had one last round of high-dose chemotherapy and radiation – a typical pre-transplant treatment called conditioning therapy – to wipe out any remaining malignant stem cells. Then, on zero day, Margossian’s team harvested and processed Alexander’s stem cells and prepared Emma for the infusion. Brian had the privilege of pushing the button that began the infusion, transferring this gift of life from his son to his daughter.

Emma remained at Boston Children’s Hospital for about a month until engraftment, or the point at which the new stem cells produce enough neutrophils, a specific kind of white blood cell, to provide protection against bacterial infection.

“She was still energetic during that time; she has a zest for life that is unquestionable,” Brian says.

On Halloween, Emma dressed as Elsa and enjoyed “reverse trick-or-treating,” as doctors and nurses brought candy to her on their rounds.

Still, the month of recovery had its challenges. The conditioning therapy often causes the onset of mucositis, an extremely painful inflammation of the mucous membranes that line the digestive tract. Emma’s case was especially severe; she had to be fed through a nasal tube.

“For 95 percent of stem cell patients, the pain of mucositis is what they remember the most about their transplant,” Margossian says.

Emma also endured graft-versus-host disease (GVHD), another expected side effect of a stem cell transplant in which the donor’s white blood cells (the “graft”) attack the host’s cells, which can cause skin rashes and irritate the digestive system and liver.

“Emma’s nurses were very proactive about it,” Allyson says. “They gave her Benadryl and used every lotion known to man to soothe her skin.”

After Emma was released from the hospital, she remained in quarantine at home for nine months, allowing her fragile immune system to gradually rebuild itself without unnecessary exposure to germs in indoor public places. She entertained plenty of visitors on her front porch, relished rides around town with her family, and enjoyed the occasional meal on the outside patio at her favorite restaurant.

When Emma’s quarantine restrictions were lifted on June 1, 2015, “she celebrated by doing anything and everything,” Brian says with a laugh. “She shopped, she visited friends, she ate inside at her favorite restaurant – and she was excited to go back to school.”

Today, Emma remains healthy. “You would never know she had been so sick,” Allyson says. Now 11, Emma is in a dance troupe and involved in acting. She plays trumpet in her school band. And yes, she and Alexander are back to the usual sibling antics.

“Kids often bounce back more easily, physically and psychologically, because they are more resilient,” Margossian said. “Emma is spunky, and her energetic attitude went a long way in positively influencing her recovery.”

Source:http://www.dana-farber.org

 

For all book lovers please visit my friend’s website.
URL: http://www.romancewithbooks.com

 

Advertisements

Temporary external implantable cardioverter‐defibrillator as a bridge to reimplantation after infected device extraction


Abstract

Patients with cardiac implantable electronic devices (CIED) and endovascular infection represent a difficult management group. The explantation of an implantable cardioverter‐defibrillator (ICD) system deprives the patient of the protection against life‐threatening ventricular tachyarrhythmias. In this study, we describe feasibility and clinical outcomes of bridging with temporary dual‐coil ICD lead and external ICD following the extraction of a CIED due to endovascular infection and compare the performance of this approach to other available options.

1 INTRODUCTION

The mainstay of treatment in patients with cardiac implantable electronic device (CIED)‐related infection is removal of all hardware.1 The benefit of providing backup defibrillation protection after explantation of a right ventricular shock lead varies according to patient’s risk of sudden cardiac death (SCD). In danger are patients in secondary SCD prevention, and those with earlier implantable cardioverter‐defibrillator (ICD) therapies, relative to primary prevention patients who have never received appropriate ICD therapies.2

2 CASE REPORT

A 64 year‐old woman was admitted due to CIED‐related infective endocarditis. Her past medical history was remarkable for non–ST‐segment elevation myocardial infarction in 2001. Subsequent coronary angiogram showed no significant intraluminal lesions. Due to sick sinus syndrome, she received a dual‐chamber permanent pacemaker in 2001. After cardiac arrest caused by ventricular fibrillation in 2002, she was implanted contralaterally with single‐chamber ICD for secondary prevention of sudden cardiac death. During 3 months before the index admission, patient had been hospitalized twice due to recurrent fever, pneumonia, and massive saddle pulmonary embolism. At that period, she presented with repetitive (11 episodes) ventricular tachycardia (VT) which triggered, in total, 33 antitachycardia pacing (ATP) sequences and 16 shocks. Two episodes of VT lasted for several minutes, a number of ICD therapies were ineffective, and VT was terminated with 5th shock and 9th shock, respectively. Lead‐dependent infective endocarditis was diagnosed based on fever, vegetations, and septic pulmonary embolism. Blood cultures were negative. After admission to tertiary referral center for transvenous lead extraction (TLE), a single‐step procedure of complete transvenous extraction of all hardware was performed with the use of mechanical systems (Byrd Polypropylene Dilator Sheath Set, Cook Medical™). The ICD lead was identified as the most challenging to extract and was hence extracted first; the whole DDD pacemaker was then extracted from the left side of chest. As a bridge to permanent ICD reimplantation, a temporary system was implanted, consisting of an active‐fixation dual‐coil DF4 ICD lead (St. Jude Medical Durata™ 7120Q‐65) inserted via percutaneous puncture of the left subclavian vein, anchored to the skin, and attached to an epicutaneous single‐chamber defibrillator (St. Jude Medical Ellipse™ VR) (Figure 1A‐B). “Active‐can” ICD was turned off, and the shock polarity was programmed from the right ventricular coil to the superior vena cava coil. Two weeks later, patient developed two episodes of VT, one of which was stopped by a first burst of ATP (Figure 2A), and the other did not respond to ATP sequence and was terminated with a 35 J shock (Figure 2B‐C). The ventricular arrhythmia was triggered by sinus bradycardia (Figure 2D); hence, later overdrive VVI pacing at 70 beats per minute fully suppressed the ventricular tachyarrhythmia for the remaining period of the endocarditis treatment (Figure 2E). Her antiarrhythmic treatment consisted of sotalol increased from 80 mg twice daily to three times daily. Chronic treatment with amiodarone was deferred due to the past medical history of amiodarone‐induced thyrotoxicosis. Antibiotic treatment consisted of vancomycin 1 g twice daily and ceftriaxone 2 g twice daily. Finally, 6 weeks after the TLE procedure, a new dual‐chamber ICD with single‐coil shock lead was implanted in the left pectoral region, and temporary ICD system was removed with simple traction. Evaluation at a 3 month follow‐up showed that the patient was in good condition.

A, ICD lead implanted via a percutaneous puncture of the left subclavian vein, sutured to the skin, and connected to ICD unit; B, Chest x‐ray after lead extraction and temporary dual‐coil ICD lead implantation
A, VT termination via ATP burst; B‐C, VT termination via 35 J shock; D, Bradycardia and ventricular ectopic beats (*—pacing spikes and ventricular fusion beats); E, Overdrive VVI pacing

3 DISCUSSION

Bridging patients with prior ICD therapies who require continuous ICD backup in the period between device explantation and reimplantation is particularly challenging.2 The options available to physicians include immobilization in an intensive care unit or telemetry ward, with continuous ECG monitoring where instant access to external defibrillation is provided; a wearable cardioverter‐defibrillator (WCD) 3; and a subcutaneous ICD (S‐ICD) as a permanent reimplantation device.4

A promising approach for patients in whom ICD implantation must be deferred, but in whom there is an urgent need to manage malignant tachyarrhythmias, is bridging with a temporary external ICD. Cooper et al reported a successful use of external ICD, programmed to burst ATP therapies only and connected to an active‐fixation pacemaker lead, in a patient who had both an ICD infection and a history of recurrent VT (responsive to single ATP therapy).5 Furthermore, in a similar setting, Dell’Era et al showed efficacious delivery of ATP and shock from a system consisting of a temporary dual‐coil active‐fixation DF4 lead, connected to an external ICD with passive‐can shock configuration.6 Our case demonstrates an additional method of controlling heart rhythm with VVI overdrive pacing, which has not been described in the aforementioned reports.

The noteworthy benefit of the temporary external ICD system over WCD is that it automatically starts treatment, whereas the latter is interactive and significantly depends on patient compliance. Moreover, an external ICD shares the same capacities as a traditional ICD, enabling bradycardia pacing, overdrive pacing, and ATP therapies. These are unavailable in the treatment choices involving “external” defibrillation, such as continuous ECG monitoring in an intensive care unit, WCD, and S‐ICD.

A limitation of the presented technique is a temporary ICD lead in the vascular system, which may impede complete infection elimination and contribute to increased risk of infection recurrence. Promising short‐term outcomes with no early infection recurrences were reported by Maciąg et al in a retrospective analysis of 34 pacemaker‐dependent patients. The patients were bridged with externalized active‐fixation pacing lead for 4‐26 days following TLE due to infection.7 Amraoui et al in a retrospective analysis of 80 consecutive pacemaker‐dependent patients bridged with externalized pacing lead for 4‐14 days reported excellent short‐ and long‐term effects with no early lead dislodgement and no infection recurrence at 1 year follow‐up.8 Of note, Perrin et al presented follow‐up outcomes in the group of 52 pacemaker‐dependent patients who were bridged with screwed‐in temporary lead for 11.1 ± 9.7 days and retrospectively followed up for a mean period of 25.2 months: Eight patients (15.4%) developed vegetations on their temporary lead; one temporary lead dislodged with sudden loss of capture; and one patient developed a CIED reinfection after 21 months of follow‐up.9 Undoubtedly, a prospective study would be required to fully assess the long‐term safety of temporary lead bridging. Importantly, in the above‐mentioned studies by Maciąg et al and Perrin et al, up to 20% of patients had an infected ICD system,7, 9 whereas in the study by Amraoui et al, ICD patients were excluded from analysis.8

The technique described above provides an important option for prolonged ICD backup. The presented case shows that a temporary external ICD is an efficacious bridge to permanent device reimplantation.

Appendiceal Pinworms


A 45-year-old man with a 1-day history of abdominal pain and loss of appetite presented to the emergency department. He did not have a fever. Physical examination revealed tenderness in the right lower quadrant of the abdomen. Rebound was present; Blumberg’s sign was negative. Laboratory results included a normal white-cell count, with 82% neutrophils. Abdominal ultrasonography was performed, but the results were inconclusive with regard to the presence or absence of appendiceal inflammation. An appendectomy was performed. The appendix was grossly normal, but pathological analysis revealed the presence of Enterobius vermicularis in the appendiceal lumen (Panels A and B, hematoxylin and eosin) and mild inflammation of the mucosa. E. vermicularis, known as pinworm, is a nematode that is most commonly found in young children, although adults can also be infected. The gravid female pinworm attaches to tissue near the cecum and migrates to the anus to deposit its eggs, which can cause perianal pruritus. Autoinfection and transmission occur through the ingestion of eggs from contaminated hands, bathroom fixtures, bedding, food, and other sources. It is difficult to determine definitively whether the presence of pinworm in the appendix was the cause of the patient’s presenting symptoms or was an incidental finding. After the appendectomy, the patient and his family were treated with mebendazole. At follow-up 6 months after surgery, the patient was asymptomatic.

Cutaneous Diphtheria


A 5-year-old, fully vaccinated girl was brought to the emergency department with pruritic lesions on both legs. She had recently returned from a trip to Sierra Leone. The lesions had appeared 3 weeks into her stay there and had increased in size and ulcerated. On presentation, she was afebrile, and an area over the medial aspect of the left lower leg was ulcerated and bleeding (Panel A). The C-reactive protein level was mildly elevated, and the white-cell count was normal. Empirical treatment with oral floxacillin was initiated, and a skin swab was sent for culture, which grew Staphylococcus aureus, group A streptococcus, and Corynebacterium diphtheriae. Gram’s staining showed predominantly gram-positive bacilli characteristic of corynebacterium (Panel B). An immunodiffusion test (Elek’s test) was positive, indicating that this diphtheria was toxin-producing. Early manifestations of cutaneous diphtheria can appear as nonhealing, well-demarcated ulcers. After the detection of diphtheria in this patient, the antibiotic agent was changed to clarithromycin. Contact tracing was undertaken, and chemoprophylaxis was given to household contacts. At follow-up 1 week after the change in antibiotics to clarithromycin, the skin lesions had fully healed.

When Exercise Can Kill — The Rare Risk of Rhabdomyolysis


Story at-a-glance

  • Muscle trauma due to overexercise can lead to rhabdomyolysis (rhabdo), a serious condition that can lead to kidney damage, renal failure and death
  • When muscle is damaged, it dumps myoglobin, an iron- and oxygen-binding muscle protein, into your bloodstream. Excessive myoglobin obstructs your kidney’s filtration system, which can lead to acute renal damage
  • Classic symptoms of rhabdo include muscle swelling, pain, muscle weakness and dark, scant urine output. Other possible symptoms include nausea, vomiting, fever, intense shivering, confusion and possibly fainting
  • Aside from overexercise, other circumstances that can trigger rhabdo include accidents and blunt trauma, prolonged immobilization, drug side effects and metabolic and genetic disorders
  • Rhabdo can happen with any intense, repetitive motion exercise. Spin classes have produced dozens of cases, typically among newcomers who work above capacity. But even professional athletes and military personnel are affected

By Dr. Mercola

A recent WebMD article1 addresses a rare but potentially fatal muscle disorder called rhabdomyolysis, or rhabdo for short. Estimates suggest it may affect 22 people out of 100,000, and muscle trauma due to overexertion is a common cause. Your skeletal muscles are under your volitional control, which is why they’re also known as voluntary muscles.

You engage them when walking and moving. When skeletal muscle is damaged through overexertion injury, the muscle starts dumping myoglobin, an iron- and oxygen-binding muscle protein, into your bloodstream. Excessive myoglobin obstructs your kidney’s filtration system, which can lead to acute renal damage.

Rhabdo causes kidney failure in up to 40 percent of cases, so early diagnosis and rapid medical intervention is crucial. Liberated potassium leading to hyperkalemia (high potassium in your blood), which can occur within hours after muscle injury, can also be life threatening.

Signs and Symptoms of Rhabdo

The following symptoms are considered “classic” rhabdo symptoms, although all may not necessarily be present in all cases:

  • Muscle swelling
  • Pain in the affected muscle group
  • Muscle weakness or trouble moving your limbs
  • Dark and scant urine output

Other possible symptoms include nausea, vomiting, fever, intense shivering, confusion, dehydration and possibly fainting. While it is clear that rhabdomyolysis occurs due to the breakdown of damaged muscle tissue, there are many situations or circumstances that can trigger it. Some of the most prominent examples include:2

Overexercising

Pushing yourself while exercising, such as running too far or lifting weights beyond your limit, can damage your muscles. While muscle soreness is normal following a workout, suspect rhabdo if the pain is extreme and seems disproportionate to your exertion. Another tipoff is if symptoms trend toward getting worse rather than better over the next couple of days. Untreated, rhabdo will progressively get worse.

Accidents and blunt trauma

Patients who survive major accidents typically develop extensive muscular damage. Nonaccidental injury can also cause muscle trauma that can lead to rhabdo.

Prolonged immobilization

Being bedridden for long periods of time, such as when you suffer a stroke, can put pressure on the muscles pressing against the bed, cutting off blood flow and causing tissue death.

Drug side effects

Cholesterol-lowering medications such as statins or fibrates usually produce muscle weakness as a side effect. Abuse of illicit drugs such as cocaine and heroin can cause weakness as well.

Metabolic disorders

Certain metabolic disorders can raise your risk of rhabdo. This includes problems with metabolism of lipids (fats), carbohydrates or purines, hypothyroidism, diabetic ketoacidosis and electrolyte imbalances.

Genetic disorders

Genetic conditions that can raise your risk includes carnitine deficiency, McArdle’s disease, lactate dehydrogenase deficiency and Duchenne muscular dystrophy.

High-Intensity Repetitive Movement Is a Major Risk Factor

According to Dr. Maureen Brogan, associate professor of medicine at New York Medical College and author of a 2017 paper3 on the condition, rhabdo can happen with any intense, repetitive motion exercise. Spin classes (high-intensity cycling), for example, have produced dozens of cases, typically among newbies who are just starting out and are working above capacity.

One New York City hospital reports seeing 29 rhabdo cases within a four-year span, 14 of which were related to high-intensity cycling.4 According to Brogan, “The high-intensity exercise associated with spin class comes with significant risks to newcomers.” She even goes so far as to call spinning-induced rhabo a “public health concern.” WebMD reports:5

“She says she came to see it as that after six patients came to her hospital’s ER, and all involved people trying a cycling class for the first time … When she searched medical literature, 42 of the 46 cases she found also involved people going to cycling class for the first time.

‘Those are the patients that were most at risk because they may not be conditioned and are using and engaging new muscle groups for the first time at an intense rate,’ she says. ‘So even if you were a different type of athlete like a runner, and then you switch to biking and use quadriceps and gluteus maximus muscles at an intense rate — that first time, you may be at risk of getting rhabdo’ …

[P]eople who stop cycling for some time and then go back at the same rate are at risk, too. ‘[Cycling] is great exercise if you are conditioned for it,’ she says. ‘But you burn 600 to 900 calories in one class. You wouldn’t go out and run 6 to 9 miles on your first day of running. If you did that, you wouldn’t be able to walk either.’”

Dehydration and Extreme Temperatures Raises Your Risk

Inadequate physical conditioning in combination with severe dehydration and/or extreme body temperatures raise your risk of rhabdo, regardless of the exercise you’re doing. Always make sure to stay well-hydrated before, during and after exercise, and take a break if you start feeling excessively hot. If you’re not conditioned to exercise in hot conditions, avoid starting a new type of exercise in a heated exercise room.

When starting a new exercise, even if you’re fit, listen to your body, start slow, take breaks, and work your way up to greater intensity over time. It’s important to recognize that you don’t have to be in poor physical condition for rhabdo to occur, or that you have to work out for an extended period of time. One of Brogan’s patients developed rhabdo after just 15 minutes of cycling.

In fact, many rhabdo patients exercise regularly and express surprise when getting their diagnosis. The key to remember is that there’s a fine line between exercising to capacity and overexerting yourself. Approximately 7 to 8 out of 100,000 military recruits, for example, are affected each year, and even professional athletes — especially marathoners and ultramarathoners — have suffered its consequences.

No One Is Immune to Rhabdo

Professional snowboarder Amy Purdy was hospitalized in 2016 after participating in a CrossFit class for the first time. While generally well-conditioned, she had not done pullups for a few months and the high-intensity, repetitive pullups done during class did her in. She told WebMD:

“It wasn’t until 72 hours later, I was back in my hometown with friends at a restaurant around 11 at night. I told them I worked out too hard a few days ago, and my arms wouldn’t straighten all the way. I then took my jacket off and instantly noticed swelling around my elbows on both arms. The doctors were convinced I didn’t have it because my arms were only slightly swollen. They decided to test me anyway.

That whole experience was one of that [sic] hardest experiences of my life! If you push your body to failure and keep going, you are at risk. Listen to your body. It knows best. And if you find yourself going on days with overly stiff and sore muscles and you notice swelling, get to the hospital ASAP. It may not be rhabdo, but a simple blood test can tell.”

Diagnosis and Treatment

Diagnosis usually begins with a review of your medical history and the events that led up to your medical visit. A variety of blood and urine tests can help diagnose rhabdo. The following tests are typically recommended:6

  • Complete blood count, including hemoglobin, hematocrit and platelets
  • Serum chemistries, including blood urea nitrogen, creatinine, glucose, calcium, potassium, phosphate, uric acid and liver function tests
  • Prothrombin time and activated partial thromboplastin time
  • Serum aldolase
  • Lactate dehydrogenase

In mild cases of rhabdo, simple lifestyle changes are typically sufficient for a full recovery. This includes:

Hydration: Keeping your body properly hydrated helps flush out toxins and ease the workload of your kidneys. Drink clean, filtered water until your urine turns to a light-colored yellow.

Reduce exercise: Cut back on your workout until your muscles recover and your urine normalizes. This can help lower the amount of toxins entering your kidneys until you get better.

Increase circulation: Improving blood circulation is vital to helping your muscles heal and lowering your risk of tissue death. Gentle full-body massages and gentle movements can be helpful. Certain foods can also help improve blood circulation. This includes oranges, goji berries, dark chocolate, sunflower seeds, garlic, ginger and cayenne pepper.

Eat a nutritious diet: Optimizing your diet will also increase your chances of a full recovery. By focusing on eating organic, whole foods that are rich in nutrients, you are nourishing your muscles to recover better and improve your overall well-being.

While protein is important for muscle recovery, avoid protein loading as excessive protein consumption can stimulate your mTOR (mammalian target of rapamycin) for growth rather than regeneration, which is not what you need. To avoid this, limit your intake to 0.5 gram of protein per pound of lean body mass, and focus instead on eating the highest quality protein you can get. This includes grass fed meats, raw seeds and nuts and pasture-raised eggs.

For more serious cases of rhabdo, additional measures may be required. An intravenous solution of special minerals may be used to counteract the potential harms caused by severe muscle damage. Electrolyte imbalances will need to be monitored and promptly treated as well, and in severe cases you may need hemofiltration to address kidney damage.

Rehabbing and Long-Term Prognosis

Overall, the prognosis of rhabdo is good as long as your blood, electrolytes and urine are closely monitored following muscle failure. The mortality rate for rhabdo is only 5 percent, but your risk can significantly increase if kidney failure occurs. If you take good care of yourself by moderating exercise, drinking enough water and getting enough rest, you will be on your way to a successful recovery. It can take time though. Purdy spent months in rehab before being able to lift even the lightest of weights.

While rhabdo is a serious condition, and can happen to anyone, you shouldn’t be scared away from exercise. The take-home message is to always listen to your body. Go easy when you first start something new or different from your regular fitness routine. Give your body some time to adapt and don’t go all-out during the initial sessions.

Also, if you just don’t feel right following a strenuous exercise, keep close watch on your symptoms. Check the color of your urine and pay attention to pain, swelling and weakness. If you’re trending downward when you know you should be recovering, seek medical attention. Blood tests can help diagnose the problem and with proper treatment, kidney damage can be avoided.

What Caused This Woman to Give Off Toxic Fumes?


There aren’t a lot of people whose deaths inspired episodes of “The X-Files.” But none of them deserved it as much as Gloria Ramirez. They called her “the toxic lady,” and to this day, we’re not sure what happened to her.

A Perilous Patient

Here’s what we know for sure. At 8:15 p.m. on February 19, 1994, 31-year-old cervical cancer patient Gloria Ramirez was admitted to Riverside General Hospital in Riverside, California. She was conscious, but suffering. Her breaths were shallow, her heart was beating rapidly, and her blood pressure was plummeting. They injected her with a suite of standard medications, and attempted to inject air into her lungs with a device known as an Ambu-bag (think a bellows, but shaped like a football). Two things seemed strange about her condition: her skin seemed to have a slightly oily sheen, and her breath had a smell like fruit and garlic. And then, they attempted to take her blood.

A nurse named Susan Kane was assigned that task and noticed that the room began to take on a strange, chemical smell as the syringe filled with blood. She gave the syringe to respiratory therapist Maureen Welch, who thought the blood smelled funny — not like the telltale scent of chemotherapy drugs, but more like ammonia. Welch then passed the syringe to medical resident Julie Gorchynski, who saw small, manila-colored particles floating in it. That’s when Kane, who’d been leaning over Ramirez to find the source of the smell, fainted.

Later, she said her face felt as though it was burning. Her fellow staff placed her limp body on a gurney and carried it out of the trauma center. Gorchynski felt the lightheadedness next, barely making it out of the room before she fainted as well. She also began showing other symptoms: her whole body began to shake and she would intermittently stop breathing. Welch fainted next, and when she woke up, she couldn’t control her limbs. Several other staff members at the hospital began exhibiting similar symptoms, and administrators declared a state of emergency as doctors, nurses, and patients were filed into the building’s parking lot. A skeleton crew remained behind to tend to Ramirez, who was eventually declared dead at 8:50 p.m. By the time the incident passed, 23 people had fallen ill and 5 would need hospitalization.

A Chemical Chain Reaction

Staff members continued to fall prey to whatever it was that happened to Gloria Ramirez even after she passed away. As you can probably imagine, the event sparked a massive investigation. But at the first pass, medical detectives came up with distinctly unsatisfying solutions ranging from a coincidental emission of poisonous sewer gas to a case of mass hysteria. Most frustrating of all, no sign of toxic chemicals was discovered in the hospital in the aftermath. But in Ramirez’s autopsy, doctors turned up elevated levels of dimethyl sulfone.

Another theory began to emerge — one that didn’t rely on blaming symptoms such as hepatitis, pancreatitis, and avascular necrosis to mass hysteria. If, hypothetically, Ramirez had been using a popular home remedy known as DMSO to alleviate her cancer symptoms, it may have combined with the oxygen in her oxygen mask to form the dimethyl sulfone. Investigators attempted exposing DMSO to oxygen to see if such a thing were possible, and were able to form large amounts of manila-colored dimethyl sulfone crystals in the process.

There’s just one problem: dimethyl sulfone isn’t toxic either. But it might have kicked off a chain reaction that resulted in a massive amount of toxic fumes. When one molecule of oxygen combines with DMSO, you get dimethyl sulfone. But when two molecules of oxygen combine with dimethyl sulfone, you get dimethyl sulfate, which really does cause serious problems. When absorbed into the body, it can cause convulsions, delirium, paralysis, coma, and delayed damage to various organs. The good news? Not much — except that people don’t really use DMSO to treat themselves anymore.

Rhinofacial Entomophthoromycosis


 

An immunocompetent 22-year-old man from southern China presented to the dermatology clinic with a 3-month history of progressive nasal and maxillofacial swelling. He reported that during the past 3 years he had had episodes of facial swelling for which he had received multiple treatments, including glucocorticoids and antibiotics. The physical examination revealed erythema, edema, and tenderness over the nasal dorsum and forehead, extending to the soft tissue around both eyes (Panel A), that correlated with soft-tissue thickening on computed tomography. Biopsy specimens were obtained from multiple sites, including the forehead and the nose. Histopathological examination of a specimen from inside the nose revealed granulomatous inflammation and fungal elements surrounded by eosinophilic material. A fungal culture grew Conidiobolus coronatus (Panel B), the identity of which was confirmed by polymerase-chain-reaction assay. Rhinofacial entomophthoromycosis was diagnosed. Conidiobolomycosis, a type of entomophthoromycosis, is a rare subcutaneous mycosis that can occur in immunocompetent persons in tropical and subtropical regions. It affects the upper respiratory mucosa and adjacent subcutaneous tissues. Infection probably results from the inhalation of fungal pathogens or from trauma. The patient received itraconazole, trimethoprim–sulfamethoxazole, and a 10% potassium iodide solution for 6 months, and the symptoms had fully resolved without relapse after 1 year of follow-up.

Source:http://www.nejm.org

A Woman Had 14 Worms in Her Eye And Doctors Didn’t Even Know It Was Possible


This species doesn’t usually attack humans.

A parasitic eye worm called Thelazia gulosa is pretty common in the northern US and southern Canada – at least in cows.

Now, for the first time, it’s been observed jumping over to humans, infecting the eye of an Oregon woman.

The 26-year old patient reported feeling an irritation in her left eye, before removing the first worm eight days later.

Over a 20-day period, 14 worms less than 13 millimetres (half an inch) in length had to be removed from her conjunctiva and the surface of her eyeball.

Typically, the eye worms are spread between cattle by flies that feed on the fluid that lubricates the eyeball.

“Cases of eye worm parasitic infections are rare in the USA, and this case turned out to be a species of the Thelazia that had never been reported in humans,” said case report lead author Richard Bradbury of the CDC’s Division of Parasitic Diseases and Malaria.

“Previously, it was thought that there were only two different species of these (Thelazia) eye worms that infected humans worldwide. Now, we have to add Thelazia gulosa, a third one to the list.”

The other two types of eye worms that infect humans are Thelazia callipaeda, found in Asia, and Thelazia californiensis, found across Asia and Europe.

Previously, only 10 cases of thelaziasis, the infection caused by Thelazia, had been reported in the US, and all had ended up being the result of Thelazia californiensis worms.

More commonly, thelaziasis occurs in animals such as cats, dogs and foxes, spread by different types of flies. If humans do end up infected, they tend to be the very young and the very old – possibly, the doctors noted, because they can’t as easily brush flies away from their faces.

Typically, patients infected with these parasitic eye worms report feeling similarly to the 26-year-old patient – eye irritation, and the sensation of a foreign body in the eye.

The doctors believe she may have contracted the worms while practising horseback riding in a region of Oregon where cattle farming is common.

“We immediately thought it could be Thelazia californiensis because that is the only species that was known to infect humans in the US,” Bradbury said.

“It was only after we looked more carefully that we realized some differences in anatomy that meant it could not be T. californiensis. We had to go back to papers published in German back in 1928 to help identify this worm as Thelazia gulosa.”

Thankfully, it’s pretty easy to get rid of these eye worms, since they stay on the surface of the eye and the conjunctiva, but that doesn’t mean the worms aren’t dangerous.

If the worms move across the surface of the eye, they can scar the cornea and even cause blindness, the doctors noted in the case report.

The worms were all removed manually – 6 by doctors, and the remaining 8 by the patient herself, over the space of several days.

Since the 14th worm left the patient’s eye, she has experienced no further symptoms.

The case report has been published in The American Journal of Tropical Medicine and Hygiene.

A parasitic eye worm called Thelazia gulosa is pretty common in the northern US and southern Canada – at least in cows.

Now, for the first time, it’s been observed jumping over to humans, infecting the eye of an Oregon woman.

The 26-year old patient reported feeling an irritation in her left eye, before removing the first worm eight days later.

Over a 20-day period, 14 worms less than 13 millimetres (half an inch) in length had to be removed from her conjunctiva and the surface of her eyeball.

Typically, the eye worms are spread between cattle by flies that feed on the fluid that lubricates the eyeball.

“Cases of eye worm parasitic infections are rare in the USA, and this case turned out to be a species of the Thelazia that had never been reported in humans,” said case report lead author Richard Bradbury of the CDC’s Division of Parasitic Diseases and Malaria.

“Previously, it was thought that there were only two different species of these (Thelazia) eye worms that infected humans worldwide. Now, we have to add Thelazia gulosa, a third one to the list.”

The other two types of eye worms that infect humans are Thelazia callipaeda, found in Asia, and Thelazia californiensis, found across Asia and Europe.

Previously, only 10 cases of thelaziasis, the infection caused by Thelazia, had been reported in the US, and all had ended up being the result of Thelazia californiensis worms.

More commonly, thelaziasis occurs in animals such as cats, dogs and foxes, spread by different types of flies. If humans do end up infected, they tend to be the very young and the very old – possibly, the doctors noted, because they can’t as easily brush flies away from their faces.

Typically, patients infected with these parasitic eye worms report feeling similarly to the 26-year-old patient – eye irritation, and the sensation of a foreign body in the eye.

The doctors believe she may have contracted the worms while practising horseback riding in a region of Oregon where cattle farming is common.

“We immediately thought it could be Thelazia californiensis because that is the only species that was known to infect humans in the US,” Bradbury said.

“It was only after we looked more carefully that we realized some differences in anatomy that meant it could not be T. californiensis. We had to go back to papers published in German back in 1928 to help identify this worm as Thelazia gulosa.”

Thankfully, it’s pretty easy to get rid of these eye worms, since they stay on the surface of the eye and the conjunctiva, but that doesn’t mean the worms aren’t dangerous.

If the worms move across the surface of the eye, they can scar the cornea and even cause blindness, the doctors noted in the case report.

The worms were all removed manually – 6 by doctors, and the remaining 8 by the patient herself, over the space of several days.

Since the 14th worm left the patient’s eye, she has experienced no further symptoms.

The case report has been published in The American Journal of Tropical Medicine and Hygiene.

A parasitic eye worm called Thelazia gulosa is pretty common in the northern US and southern Canada – at least in cows.

Now, for the first time, it’s been observed jumping over to humans, infecting the eye of an Oregon woman.

The 26-year old patient reported feeling an irritation in her left eye, before removing the first worm eight days later.

Over a 20-day period, 14 worms less than 13 millimetres (half an inch) in length had to be removed from her conjunctiva and the surface of her eyeball.

Typically, the eye worms are spread between cattle by flies that feed on the fluid that lubricates the eyeball.

“Cases of eye worm parasitic infections are rare in the USA, and this case turned out to be a species of the Thelazia that had never been reported in humans,” said case report lead author Richard Bradbury of the CDC’s Division of Parasitic Diseases and Malaria.

“Previously, it was thought that there were only two different species of these (Thelazia) eye worms that infected humans worldwide. Now, we have to add Thelazia gulosa, a third one to the list.”

The other two types of eye worms that infect humans are Thelazia callipaeda, found in Asia, and Thelazia californiensis, found across Asia and Europe.

Previously, only 10 cases of thelaziasis, the infection caused by Thelazia, had been reported in the US, and all had ended up being the result of Thelazia californiensis worms.

More commonly, thelaziasis occurs in animals such as cats, dogs and foxes, spread by different types of flies. If humans do end up infected, they tend to be the very young and the very old – possibly, the doctors noted, because they can’t as easily brush flies away from their faces.

Typically, patients infected with these parasitic eye worms report feeling similarly to the 26-year-old patient – eye irritation, and the sensation of a foreign body in the eye.

The doctors believe she may have contracted the worms while practising horseback riding in a region of Oregon where cattle farming is common.

“We immediately thought it could be Thelazia californiensis because that is the only species that was known to infect humans in the US,” Bradbury said.

“It was only after we looked more carefully that we realized some differences in anatomy that meant it could not be T. californiensis. We had to go back to papers published in German back in 1928 to help identify this worm as Thelazia gulosa.”

Thankfully, it’s pretty easy to get rid of these eye worms, since they stay on the surface of the eye and the conjunctiva, but that doesn’t mean the worms aren’t dangerous.

If the worms move across the surface of the eye, they can scar the cornea and even cause blindness, the doctors noted in the case report.

The worms were all removed manually – 6 by doctors, and the remaining 8 by the patient herself, over the space of several days.

Since the 14th worm left the patient’s eye, she has experienced no further symptoms.

The case report has been published in The American Journal of Tropical Medicine and Hygiene.

Herpetic Whitlow


nejmicm1711479_f1

A previously healthy 1-year-old girl was admitted to the hospital with a 4-day history of fever, along with erythema and swelling of the left third finger. Bacterial cellulitis was suspected, and intravenous cefazolin was initiated. However, over the next 36 hours, the fever persisted (with a maximum temperature of 39°C), the finger was noted to have visible vesicles, and the fingertip became pale (Panels A and B). Further history revealed that the child often sucked her fingers, and examination of the oral cavity was notable for gingival inflammation and tongue lesions (Panel C, arrow). Polymerase-chain-reaction assay of a specimen from an oral lesion was positive for herpes simplex virus type 1 (HSV-1). Primary HSV-1 infection in young children commonly causes gingivostomatitis and fever. Thumb and finger sucking can lead to digital HSV infection, known as herpetic whitlow. In this patient, cefazolin was discontinued and intravenous acyclovir was initiated. Within 2 days, the symptoms began to resolve, and treatment was switched to oral valacyclovir. The patient was discharged home and completed a 10-day course of antiviral therapy. Resolution of the skin lesion was confirmed at the outpatient clinic 9 days after discharge.

A Newborn with Thrombocytopenia, Cataracts, and Hepatosplenomegaly


http://www.nejm.org/doi/full/10.1056/NEJMcpc1706110?utm_medium=referral&utm_source=r360