A postacute COVID-19 multisystem inflammatory syndrome (MIS) has been recognized as a rare, yet severe, complication of SARS-CoV-2 infection. First characterized in children, MIS in adults (MIS-A) has now been reported, leading to the publication of a working case definition by the Centers for Disease Control and Prevention.

The goal of this cohort study was to describe the spectrum of MIS-A presentation after SARS-CoV-2 infection. We identified cases of MIS-A among all adults with laboratory-proven subacute or convalescent SARS-CoV-2 infection at a single tertiary care medical center and described their clinical characteristics and outcomes.

Discussion

The patients with MIS-A identified in our cohort have a broader distribution of organ involvement and lower illness severity compared with those in previously published series. Most patients who met the MIS-A criteria were not identified as such by the primary clinical team. This study had some limitations. Our data likely underestimate the incidence of MIS-A because many patients with COVID-19–related admissions did not have routine comprehensive clinical and laboratory assessments to screen for this syndrome. These data suggest that, although uncommon, MIS-A has a more heterogeneous clinical presentation than previously appreciated and is commonly underdiagnosed. Future investigations, including prospective enrollments, are necessary to improve the diagnostic and treatment approaches for patients with MIS-A.

It's not all in their minds. 

An unknown but growing number of the 4 million U.S. COVID-19 patients say they can't shake symptoms ranging from fatigue to serious respiratory or neurological problems, often for months after diagnosis. The ailments are all the more challenging because patients say they often face skeptical families, friends, employers and even doctors. 

Research is limited on these so-called "long haulers." New York City's Mount Sinai hospital appears to have the first post-COVID treatment center in the U.S. 

A study of 143 patients in Italy out this month in JAMA Network found 87% of patients who had recovered from COVID-19 reported at least one lingering symptom, notably fatigue and trouble breathing. 

Natalie Lambert, an Indiana University associate research professor, analyzed at least 1,100 responses to a poll about post-COVID-19 symptoms in the 81,000-member Survivor Corps Facebook group. More than half of the patients reported at least one of six symptoms, including the now-common fatigue and breathing problems.

The list also includes two â€“ inability to exercise or be active and difficulty concentrating â€“ the Centers for Disease Control and Prevention hasn't yet cited in its list of COVID-19 symptoms. 

Karyn Bishof appears to have most of them. 

On Saturday, the Boca Raton, Florida resident hit Day 133 of suffering with a staggering list of symptoms that includes: cough, chronic fatigue, memory issues, vision impairment, chest heaviness, drastic heart rate and oxygen changes, sore throat, hair loss, heart palpitations, reflux, nausea, dizziness, vertigo, rapid hot flashes, joint paint, full body itchiness, tremors, mild fever, dry mouth, excessive thirst, overheating with no fever, rash, sleep apnea, chest pain and tinnitus.

She started her own poll in the Survivor Corps group in June to see how many other so-called COVID-19 "long haulers" there were. More than 1,500 people said they, too, were still suffering and more than half said the symptoms lasted more than three months.

Diana Berrent, who created Survivors Corps in March while isolating at home with COVID-19, estimates more than half of the Facebook group members who no longer test positive still experience COVID-19 symptoms. 

Lambert said patients face even more skepticism with symptoms affecting the brain. Those include problems with memory, sleeping, irritability or sadness. Patients said their doctors often attribute sleeping problems to stress.

More than 40% of respondents in Bishof's poll reported their doctors hadn't listened to or believed them. 

Dr. Maja Artandi is not one of them.  

"We definitely see prolonged symptoms, sometimes a lingering cough and the most serious cases have long term chest pains and still feel they can't breathe well," said Artandi, medical director of an outpatient COVID-19 clinic at Stanford University hospital. "It just causes all kinds of inflammation and takes awhile to heal." 

Dr. George Abraham, chief of the department of medicine at St. Vincent Hospital in Worcester, Massachusetts, said the body's response to the virus's lingering inflammation will dictate how long it takes to resolve. 

"The virus itself is a receptor that binds to the human body," said Dr. Makesh Madhavan, a fellow in the cardiology division at Columbia University Medical Center. Because the body has so many receptors, where the virus connects and inflames may depend on which organs already are compromised or factors still not understood, he said.

Patients with preexisting cardiac diseases, diabetes or coronary artery disease are always at higher risk, he added. 

The lungs can nearly "drown in secretions" during the infection, which make them stiff.  It can take a long time for them to "start expanding and relaxing" again, said Abraham. 

And just how long any of this takes is one of coronavirus' biggest unknowns. 

"It’s only been about six months that COVID's been in the U.S. so the term 'long term' is relatively relative," Madavan said.  

"We have no idea if or when this will ever end"

Bishof, 30, first had COVID-19 symptoms March 15, tested positive for coronavirus March 23 and negative in May and June with positive antibodies. But the effects show no sign of abating.

"The damage COVID has caused is just continuing to spiral and I am afraid for my life," Bishof said. "I am afraid that there will not be resources for long haulers for a long time."

"Two days ago I passed out, was out for a few hours and when I woke up, my oxygen level was at 83," said Bishof. (Under 90 is considered low, requiring additional oxygen.)

Bishof, a firefighter and single mother, played soccer with her 11-year-old son and worked out five or six days a week before she tested positive for the virus. Now, she said she can't walk more than a block or two without taking days to just recover. She worries the damage to her lungs could keep her from continuing as a firefighter. 

"I can’t get help," Bishof said. "So many people are not believed by their doctors or are blatantly being told they have no idea how to treat them."

Like many long haulers, Bishof also wonders about contagion. 

"They say with no fever or symptoms for x amount of days you are no longer contagious, but what about us long haulers who constantly have symptoms or waves of flare ups?" Bishof said. "We have no idea if or when this will ever end."

"Doctors have told me this could last anywhere from six months to a year"

Kimberly Campbell, 39, of Pembroke Pines, Florida, has suffered from COVID-19 symptoms for five months. The mom of four started showing typical signs of the viral infection March 1 and was presumed positive for COVID-19 in mid-April when she tested negative for all other possible health complications.

"Doctors have told me this could last anywhere from six months to a year," she said. 

Campbell's children and husband showed signs of the virus early on when they developed coughs and intense fatigue. While her family recovered, Campbell continued to suffer from symptoms including a sore throat, constant headaches, pins and needles sensations, rapid heart rate, joint and body pain, temporary loss of vision and shortness of breath.

Campbell has slept on an ice pack for four months ever since her "COVID headaches" started. On June 7 she woke up with a "blotch" in her right eye and every 30 minutes, her vision worsened until she received anti-viral medication and steroids for both eyes.

"I thought I was losing my vision," Campbell said. "I have four little ones and to wake up and not be able to see is just earth shattering."

Campbell tested negative for the coronavirus June 18.

"Doctors said at this late stage, some people are testing positive again and some are testing negative," Campbell said. Although the virus is no longer active, some of her doctors believe she could be experiencing long-term effects. She was diagnosed with chronic fatigue syndrome after months of battling the virus.

Eight specialists couldn't come up with a conclusion about her case, so Campbell turned to Facebook, found a COVID-19 group and a page called "long haulers."

"I typed in some of my symptoms at the top of my search bar and hundreds of stories like mine came up," she said. "I cried for two hours after that."

"I realized how much I wanted to live"

Ryan Head, 44, of Engewood, Colorado had shortness of breath and fatigue symptoms that started March 15. He tested positive April 3 and the breathing problems became more intermittent. Head believed he was getting better when his lungs would clear up for a few days, until flaring up again for several days later.

"I've had days where I've felt great and had a ton of energy," Head said. "But I've also had days where I'm in bed the entire day."

Doctor's responses were varied, he said. Some physicians were receptive and concerned, while others sent him home with little advice to offer after testing. 

"What I feel is missing is doctors really wanting to find out what is actually going on," Head said, "I understand this is all new for everybody. Hopefully through spreading awareness as more people become long haulers, doctors will be more ready to help them."

Head said he hopes people will start taking coronavirus more seriously.

"It's not as simple as either having the flu for a couple weeks or being high risk and dying," he said. "There's so much about this virus that we don't understand and there are people living with long term effects that we are just now learning about. Wear masks, social distance and just look out for each other." 

"I felt odd because I wasn't getting better"

Diane Matikowski, 61, is a school nurse from Wallingford, Connecticut who volunteered at a rehabilitation facility after her school closed during the shut down.

She felt it was her duty as a nurse to help where she could but she was exposed to coronavirus by a patient at the rehab on March 30. A day later, she started showing symptoms and she tested positive on April 3.

"I knew I had it because I was exposed to a patient that had it and nurses weren't told to wear masks back then," Matikowski said. "My temperature was 100.6 and my doctor wouldn't send me for a test. So, there was a lot of feeling not validated. But I think doctors were frustrated too because they didn't know how enough about the coronavirus."

Matikowski said she was exhausted for more than three months. Her symptoms also included sore throat, loss of smell and taste, leg cramps and twitches, fevers, a rash, hair loss and memory issues.

"I would be watching something on TV and couldn't remember what happened in the last scene," she said. 

After about 80 days, depression and anxiety set in. Matikowski was re-tested on June 8 and received another positive result.

"Before finding the long-hauler Facebook group, I thought I was a freak," Matikowski said. "I felt like people didn’t believe me and I felt odd because I wasn't getting better. I just didn't know there were other people like me."'

Matikowski tested negative for COVID-19 July 10 and was briefly ecstatic. Her relief was short lived, however, when she realized she still felt sick.

Now, she said, "I'm fearful."  

"I try to focus on: what can I do to help myself?"

Joel Hough, 56, of Manassas, Virginia is an active outdoors man who used to enjoy biking, sailing and flying his glider plane. Now, he misses all of that. Since his symptoms started April 29, he's traded his three-hour bike rides for 30-minute walks five days a week on flat ground. Overexertion causes a sore throat and sometimes a fever.

He tested positive May 3. 

"I think the act of walking, swinging arms, the gentle impact on the pavement is more helpful," Hough said.

His symptoms began with a 100.7 fever, irregular heart rate, chills, headaches, a sore throat and a cough.

"The toughest thing for me is going to a doctor and being told there's nothing they can do," Hough said. "I didn't get any instructions really on what to do with myself besides go home, hope for the best."

Hough uses his CPAP breathing machine, takes multivitamins, practices breathing, uses a humidifier and does low-level exercise to feel better. 

"Coronavirus support groups have enlightened me," Hough said. "The doctor rolls their eyes when you say you're in a support group, but doctors tell me nothing. Too many of them are overburdened and just don't know enough."

"Some days feel like I don’t have one ounce of energy left in my body"

Firefighter Kimberly Talmadge, 53, of Hamden, Connecticut has been sick with symptoms since April 3, reporting exhaustion, shortness of breath and sleep apnea. She and her husband were exposed working at the fire department.

"We were both sick within a week of exposure," Talmadge said.

The couple reported most of the usual symptoms including fever, cough, dehydration, sore throat, shortness of breath, fatigue, nausea, loss of appetite and a lost sense of smell and taste. Talmadge lost 15 pounds and is still losing her hair at "an alarming rate." 

After a few weeks, the couple started feeling better. Talmadge's husband tested positive although he felt well enough to return to work, and the following weekend Talmadge was sick again. They thought living together might be keeping them sick, so she moved to college housing the fire department arranged. Two weeks later her husband got a negative result but it was another month before she did.  

She continues to have shortness of breath and intense fatigue.

"Some days feel like I don’t have one ounce of energy left in my body," Talmage said. "This is my 20th year on the job and I’m not sure if I’m going to make it back."

"This is not how they said COVID would be."

Lisa O'Brien, 42, of Roy, Utah is on day 137 of suffering from COVID-19.

It's "not how they said COVID would be," O'Brien said.

Her symptoms began March 11. She never had a fever, but along with lots of shortness of breath she now has arthritic pain, lung clots, vascular issues, internal tremors or buzzing, electrical zaps, sleep deprivation, phantom smells, nausea, loss of appetite and body aches.

"I contemplated sleeping in my car in the parking lot of the ER," O'Brien said. "I just feel so unsafe in my body."

Major brain fog came on Week 14, along with light headedness, varying blood pressure and heart rate fluctuations from 30 to 209 without activity. "I'm not sure which one is more terrifying," O'Brien said. "The spikes are getting higher and higher every week and they wake me up throughout the night every night, sometimes many times."

Doctors from Mount Sinai tell her they've seen many coronavirus patients like her, but they're unable to say why the lingering symptoms only affect some people. 

"There's no common factor among us," O'Brien said. "And they can't tell me if or when it will end."

"We can do this"

Rose Dougherty, 56, of Daytona Beach, Florida has been sick for 132 days after testing positive for COVID-19 on April 3. Her symptoms began March 16. She's had difficulty breathing, chest pain, a sore throat, loss of taste and smell, fatigue, burning eyes, loss of appetite, gastrointestinal symptoms, chills and brain fog.

"This virus has impacted my life in many ways," Dougherty said. "I would say the most prominent impact is my family. I have not been able to spend time with my grand kids like I was prior to the virus."

Dougherty used to love the beach, kayaking, going to the movies, shopping and playing at home with her 6-year-old granddaughter.

Being a long hauler with no end in sight is taking a toll on Dougherty's mental health. Yoga is usually her way of staying grounded. Lately, she has been unable to perform at the level she was before COVID-19 and even struggles taking a walk.

"My mind asks, 'Am I ever going to physically be able to do these things again?'" 

Doctors and other health professionals have offered their time and expertise freely, which Dougherty described as a "true gift." The long hauler community also has been supportive.

"I am getting to know the most wonderful, caring, supportive people in this country," Dougherty said. "This has helped tremendously knowing I am not alone, I am not crazy and we can do this."

This article originally appeared on USA TODAY: COVID-19 'long haulers' fight for months with lingering symptoms

Q)Very important in clinics/tests...you have a patient with angina. You need to DECREASE heart rate and cardiac contractility and block coronary vasospasm. Which drug, Verapamil or Nifedipine will do the work?

A) Verapamil will do it. Nifedipine, another Ca channel blocker, does not do this well.

Q) Very important in clinics/tests: What is the rate limiting committed step in de novo purine synthesis? Is it:
1) Ribose 5 phosphate > PRPP or
2) PRPP > 5 phosphoribosylamine?

A) PRPP > 5 phosphoribosylamine, CONFUSING...but this is because Ribose 5 phosphate > PRPP is the FIRST step, but not the rate limiting one because PRPP is also utilized in PYRIMIDINE synthesis and in base salvage.


Case on RBCs: If I present you with a mature RBC named George Bush, tell me, True or False:

Q) In the RBC, lactate is converted to pyruvate for use in gluconeogenesis.

A) False, recall that gluconeogenesis occurs only in the liver and kidneys.


Q) In RBCs there is a glycolysis where there are 2 ATP made. The two reduced NADH are then used to convert pyruvate into lactate. Is this true or false?

A) TRUE, some think it is acetyl CoA, but they are wrong.


Q) A mature RBC uses the pentose phosphate pathway for the formation of NADPH.  Why is this needed?

A) To maintain glutathione in a reduced state.


Q) What is the reduced glutathione used for in the RBC?

A) You need it to maintain the integrity of the cell membrane!


Q) What is the most common benign soft tissue tumor in adults.

A) LEIOMYOMA, do you know what a gross specimen looks like?



Q) The most common soft tissue SARCOMA. What do you say?

A) Malignant fibrous histiocytoma.

Q) As we just discussed, malignant fibrious histiocytoma is found where and in whom usually.

A) often in men, older, and involves the limb bones and retroperitoneum.


Q) True or False: Lipomas often will progress to liposarcomas, given enough years.

Also, where are they most often found?

A) False.

They are most often benign and found around the neck and torso!


Q) We discussed LIPOMAS (also known as uterine fibroids), are very common, but different from Leiomyomas. But what about leiomyoSARCOMAS? What are they?

A) They are malignant tumors of SMOOTH muscle origin. So, you will see lesions in the uterus, GI walls, and blood vessels.


Q) What exactly is a rhabdomyoma? Benign or Malignant?

A) Benign, they are benign tumors of skeletal or cardiac muscle. IT is the Second most frequent tumor of the heart. Myxomas are the most common here.


Q) What nucleotide cannot be a substrate of primase? (choices: ATP, TTP, UTP, GTP).

A) Think and recall that TTP has thymidine. Because primase makes RNA primers in DNA replication, only RIBOnucleotides can be used.


Q) You live in a house called "Sand and Fog". Again, your friend, Jennifer Connolly comes in and steps on a nail. You quickly give her tetanus immune globulin. Does this neutralize circulating toxin, toxoid, or fixed toxin on nerve tissue?

A) Cirulating toxin.


Q) Case: Your attending pulmonologist walks in and asks YOU if a flowmeter tracing depicts the relationship between flow rate during a Forced Vital Capacity (FVC) and LV (Lung Volume). An FVC starts at the point of total lung capacity (TLC) and ends at Residual Volume (RV). Is all this true or false?

A) True, KNOW also that a restrictive lung disease will DECREASE BOTH TLC and RV.


Q) T or F: You have a patient named Don Johnson who has partial seizures. He is refractory to phenytoin and carbamazepine. Your med student suggests ethosuximide. Is she correct?

A) NO! Ethosuximide works only for generalized absence seizures.


Q) Case: A previously healthy 7 year old girl suffers from a 2 week history of fever, fatigue, weight loss, muscle pain, and headache. He also has a heart murmur, petechiae, and splenomegaly. What disease does she have?

A) Endocarditis, with vegetations from Streptococcus or Staphylococcus infection.


Q) case: You are seeing a 19 year old primiparous woman with toxemia in her last trimester of pregnancy treated with MgSO4. She delivers full term a 2 kg infant with poor APGAR score. Labs have a persistent hematocrit of 80%, platelets of 110,000, glucose 40 mg/dL, Mg 2.5 mEq/L, and Calcium 10 mg/dL. Later this infant has a seizure. What is the cause?

A) Pt has polycythemia induced seizures. The Mg IMPLIES that she had PREGNANCY INDUCED HYPERTENSION. This results in nutritional deprivation and hypoxemia, and erythrocytosis. KNOW that a persistent hematocrit over 65% in a neonate baby results in HYPERVISCOSITY and seizures.


Q) Which bug more often causes congenital infections, Toxoplasma gondii, Mycobacterium tuberculosis, Trichomonas?

A) REMEMBER the TORCH! T=Toxoplasma...the others seldom are implicated.


Q) Case: A Turner's syndrome patient at infancy. (45, X,O). What lesion is predominant in the neck, heart and kidneys?

A) In the neck, you will see redundant skin folds. In the heart, you often will see coarctation of the aorta, HTN, bicuspid aortic valve, and sometimes horseshoe kidney.


Q) Failure to give vit K to a newborn patient will result in elevated prothrombin or thrombin time? Plus, what clotting factors are affected?

A) PROthrombin time, Factors II, VII, IX and X are affected.

Q) Your pregnant patient is 35 weeks. Which of the following should you NOT give to her (Pick from penicillin, phenytoin, heparin, and propranolol)?

A) Of these, propranolol is contraindicated at this 3rd trimester. Bradycardia and apnea can result. HOWEVER, phenytoin recall is contraindicated usually in the FIRST trimester. The other two are safe.

Q) Woman with no prenatal care delivers small for date baby. She told you she had multiple sexual partners during her pregnancy and before. The PE of the baby has hepatosplenomegaly, noted lymphadenopathy, and nasal discharge like the snuffles. What test do you think of getting to confirm the dx?

A) FTA-ABS for syphillis. Choose PENICILLIN for Rx.


Q) A 7 month old pt. comes in with a resting HR of 50. PE reveals NO rash, and NO cardiomegaly. But electrocardiogram reveals d-looped ventricles. FH is significant for SLE. What is causing the bradycardia?

A) Most likely, a congential complete heart block. Lyme disease can be ruled out because there is no tick bite, and cardiomyopathy can be ruled out because there is NO cardiomegaly on x-ray.


Q) What and where is the anterior recess of the ischiorectal fossa?

A) A fat filled space below the pelvic diaphragm, it is in between the inferior space of the of pelvic diaphragm and the superior fascia of the urogenital diaphragm.


Q) A 37 y.o. male patient of yours has GI symptoms and feels high strung a LOT for no apparent reason, sweating AND dry mouth. Does he have panic disorder or Generalized anxiety disorder?

A) Generalized anxiety disorder...rule out panic disorder because panic disorder is usually triggered by a known cause. Give anxiolytics for meds.


Q) Someone, a 27 year old male goes to the Southern-Eastern states for a camping trip. He gets Rocky Mountain Spotted Fever. Except for the rashes and fever, what is a typical medication you would use to treat? What is the MOA of the bug? What test is helpful?

A) Use either doxycycline or tetracycline combined with chloramphenicol. The MOA of the bug is a vasculitis resulting from endothelial invasion by Rickettsial buggies. The test of choice now is the indirect florescent antibody (IFA) test. OR you can use a Giemsa stain under light microscopy.


Q) Case: You see a 5 year old pt. with a history of a URI like symptoms that preceded a rash that started from his face and spread downward (there were no Koplick spots). Lymphadenopathy may OFTEN be present, particularly in the posterior auricular, posterior cervical, and suboccipital chains. What is the dx? What is the treatment?

A) This is Rubella. Treatment is supportive with Tylenol and Benadryl for the headaches and itching.


Q) A child patient of yours comes in. Your attending tells you this is NOT RUBELLA. He had a high fever for 3 days and the rash that followed started on the trunk and then spread from there but missed his face. The condition is an acute benign disease of childhood characterized by a history of a prodromal febrile illness lasting approximately 3 days, followed by defervescence and the appearance of a faint pink maculopapular rash. Bug please?

A) Roseola


Q) Case: Pt of yours comes in with crops of papular, vesicular, pustular lesions starting on the trunk and spreading to the extremities. Lesions are asynchronous (happening at different times). What is this?

A) Varicella


Q) This time, you see a young patient with ulcers on his tongue and oral mucosa. You also see a maculopapular vesicular rash on the hands and the feet surfaces (key finding). What disease is this?

A) Hand foot and mouth disease


Q) A 25 year old male patient of yours comes in with spironolactone overdose and HYPERKALEMIA. He gets muscle weakness and tetany. His potassium level is 7.4...no hemolysis. Which EKG change is NOT consistent with hyperkalemia? (pick between notched PR segment, ST depression, wide QRS complex, P wave loss, T wave elevation).

A) You WON'T see notched PR segments, but you WILL see all the others.


Q) Case: You see the same patient with Hyperkalemia. What are a few OTHER causes of this?

A) You'll see this in acute or chronic renal failure, especially in patients who are on dialysis.

Other causes include: 

Trauma, including crush injuries (rhabdomyolysis), or burns.

Ingestion of foods high in potassium (eg, bananas, oranges, high-protein diets, tomatoes, salt substitutes).

Meds - Potassium supplements, potassium-sparing diuretics, nonsteroidal anti-inflammatory drugs (NSAIDs), beta-blockers, digoxin, and digitalis glycoside.


Q) Case: Still looking at Hyperkalemia. We are dealing with a HYPERacute case of it. What med is better, Calcium gluconate or Kayexalate?

A) Calcium gluconate is better, its onset of action is as quick as 5 minutes while kayexalate may take 2-10 hours to take effect. HOwever, know that Calcium gluconate does not really affect TOTAL body K+ stores, but rather is CARDIOprotective

Q) Pt: A 6 year old child named Kill Bill presents with tachycardia at 230 beats per minute, no fever. The ECG shows a narrow complex tachycardia seen (no signs of atrial flutter). One dose of ADENOSINE makes the sinus rhythm normal with pre-excitation noted. There is NO cardiomegaly seen on radiograph. What is this? Could it be sinus tachycardia?

A) HARD HARD question. The pre excitation seen after conversion with adenosine is Wolff-Parkinson White syndrome. Sinus tachy is not likely because the patient is afebrile with no cardiomegaly.


Q) Case: Because this is so common, what is the difference in presentation between strabismus and amblyopia?

A) Strabismus is an eye that cannot align properly and amblyopia is the impairment of vision without detectable organic lesion of the eye.


Q) Case: A middle aged patient of yours tried to kill herself by injesting a bottle of antipsychotics with anticholinergic activity....can she acutely die from cardiac arrhymias?

A) YES


Q) Case: True or False: Besides mental slowness, iron toxicity can cause seizures.

A) True


Q) Case: Which one, (CCK, secretin, or bile acid levels in the plasma), determine the rate of bile secretion by hepatocytes?

A) Plasma levels of bile acids...tricky tricky. Stuff like secretin and parasympathetic innervation works at the LEVEL of the biliary ducts...NOT the hepatocytes.


Q) case: You encounter a 34 y.o. patient screaming in pain because he has a kidney stone. You find that the stone is a struvite or staghorn stone. What bug does he likely have? Is the stone calcium? What minerals are part of the stone? Is the urine acidic or alkaline?

A) He likely has a Proteus infection producing urease. The stone is NOT the most common Calcium stones. The minerals are M.A.P. or Magnesium, Ammonia, and Phosphate. The urine is ALKALINE (think ammonia).


Q) Case: Oh darn! Your patient has cystathionine synthetase deficiency. What disease is this associated with? What Amino Acid is elevated? How do the patients present clinically? What do they need to remove from their diet?

A) Homocystinuria is the dx. The amino acid elevated is methionine since its conversion is impossible. The patients present as a Marfan's body w/ scoliosis, dislocated eye lenses, mild mental retardation, thrombosis. The restriction of proteins like sulfhydryl groups leads to very low protein, foul tasting diets.


Q) A patient of yours has galactose 1 phosphate uridyl transferase deficiency. What enzyme is missing? What is the clinical presentation? What is the treatment?

A) This dx is the most common error of carbohydrate metabolism, galactosemia. Glycolysis is affected, and you see evidence of liver failure, direct hyperbilirubinemia, coag disorders, renal problems (acidosis, glycosuria), emesis, and sepsis. TREAT by eliminating all formulas and foods with galactose.


Q) What is the enzyme disease associated with ornithine transcarbamylase deficiency? How is it inherited? What toxic metabolite forms? MOA? Clinical presentation? Treatment?

A) This...OTCD...is a urea cycle defect inherited in an X-linked fashion. Ornithine couples with carbamylphosphate to make citrulline. If the enzyme is def., ornithine builds up and then urea cannot be made and excreted. AMMONIA builds up instead, and within only 24 hours, the newborn baby will become lethargic and have seizures. DIAGNOSIS by measuring the orotic acid levels in the urine. TREAT with a low fat diet and alternate pathways to excrete nitrogen via benzoic acid and phenylacetate.


Q) Case: Your patient has a respiratory disorder and is cyanotic. He comes in with a normal arterial oxygen tension (PaO2) and a LOW arterial oxygen saturation (SaO2). Your med student rushes to give oxygen therapy and the patient is STILL cyanotic. What does he have? (Pick either Right to left SHUNT, Methemoglobinemia, Respiratory Acidosis). Why?????????? How do you treat?

A) He has Methemoglobinemia. IRON needs to be in the ferrous form (+2) to be able to bind oxygen. In this dx, the IRON is in the ferric form (+3). So giving O2 does not help. You must give methylene blue which aids in the conversion.


Q) CASE: Please refer to the previous HY Concept 995...why is the answer not right to left shunt? (This is a crucial point)

A) Because, while O2 therapy has very little effect, BOTH oxygen tension (PaO2) AND oxygen saturation (SaO2) are LOW. Recall that in methemoglobinemia, the oxygen gas exhange is NOT affected in the lungs, so PaO2 is NORMAL there!


Q) Speaking of RBCs, a 14 month old male child presents with a hemoglobin of 7.6 and a hematocrit of 24%. The MCV is 65 and the adjusted reticulocyte count is 1.0. Is this ineffective erythropoiesis or not?

A) An ARC less than 2.0 is ineffective erythropoiesis for the anemia, an anemia with ARC more than 2.0 signals hemolysis or blood loss and decent erythropoiesis.


Q) Case: Everyone is going to have to do this procedure: Checking for the red reflex...what happens though if you see a reflection from a white mass within the eye giving the appearance of a white pupil? What diseases can cause this?

A) Congenital cataracts, Retinoblastoma, Glaucoma...RECALL if you see signs of a retinal hemorrhage, think SHAKEN BABY SYNDROME and protect the baby!


Q) Case: One of your patients comes in with blood streaked feces. He is an 19 month old. Fecal Occult blood test is positive. What diagnoses is MOST common here?

A) Anal fissure.


Q) The 19 month kid with the bloody stool.

"Why can't this be IBD?"
"Why can't this be Necrotizing enterocolitis?"
"Why can't this be a Mallory-Weiss tear?"
"What can't this be peptic ulcer disease?"
(So what do you say to each?)

A) Tell him that IBD (Chron's and Ulcerative Colitis) and necrotizing enterocolitis appears later in childhood, and a Mallory Weiss tear and PUD will produce dark MELENA instead!

1.
Case: You have a patient who needs to use pseudoephedrine as a nasal decongestant. He is an older gentleman with BPH, hypertension, hyperthyroidism, and coronary artery disease, and urinary incontinence. Which one of these symptoms are NOT made WORSE by the pseudoephedrine?

A) Urinary incontinence. Due to contraction, you may actually help the symptom of urinary incontinence.


2.
Q) First, tell me about the differences between extrinsic vs. intrinsic hemolytic anemia and extravascular vs. intravascular hemolysis. (People get these terribly confused at first).

A) Listen, EXTRINSIC hemolysis means something is wrong OUTSIDE the Red Blood Cell (RBC). INTRINSIC hemolysis means something is wrong INSIDE the RBC. Extravascular hemolysis occurs when MACROPHAGES eat up the RBCs and Intravascular hemolysis occurs when the hemolysis occurs by various mechanisms WITHIN the circulation.


3.
Q) What kind of hemolysis is PNH, or Paroxysmal nocturnal hemoglobinuria?

A) It is a stem cell disorder, acquired, by sensitivity of hematopoietic cells, which have a reduction of decay accelerating membrane factor, so they get destroyed by complement. So, PNH is an INTRINSIC, INTRAVASCULAR (they are NOT removed by macrophages) anemia!


4.
Q) Pt. comes in with WARM hemolytic anemia. What kind of hemolysis is this?

A) Its MOA is IgG/C3b deposited on RBCs with extravascular removal by MACROPHAGES, which have the Fc receptors for IgG and C3b! Thus, this is an EXTRINSIC and EXTRAVASCULAR hemolysis.


5.
Case: Pt with sickle cell anemia. Same question, what kind of anemia is this?

A) The sickled cells cannot escape the Billroth cords in the spleen. Thus, they are removed extravascularly by MACROPHAGES. Thus, this is an INTRINSIC hemolytic anemia with EXTRAVASCULAR hemolysis!

6.
Case: Middle aged man, smoker, received synthetic heart valve replacement.. later, anemia occurs. What kind of anemia?

A) Schistocytes (broken RBCs) are seen because they break apart after hitting the plastic heart valves. Thus, we see an EXTRINSIC (not inherent with RBC formation), INTRAVASCULAR (within the circulation) hemolysis.


7.
True or False: At a constant EDV (end diastolic volume), epinephrine RAISES stroke volume.

A) True.


8.
True or False: Vigorous exercise increases BOTH EDV and Stroke Volume!

A) TRUE!


9.
Case: Pt. on propanolol. Does Stroke Volume decrease at CONSTANT EDV? Or will it change too?

A) Yes, EDV remains constant.


10.
Case: An older female with a pacemaker that malfunctions and speeds up while the patient is at rest. How will EDV and SV change?

A) Here, they BOTH decrease.


11.
Q) In which case will there be FOLATE deficiency and not vit B12 deficiency? (Pick either Crohn's disease, Chronic pancreatitis, or Pregnancy)

A) PREGNANCY! The other two result in vitamin B12 def. Think of the MOA of vit B12 uptake! (recall R factor in saliva, intrinsic factor in stomach, etc. )


12.
Q) relating to the previous concept, will small intestine bacterial overgrowth affect vit B12 absorption? HOW?

A) YES, by breaking up the intrinsic factor and vit B12 complex. (Note, folate is not affected)


13.
Q) The posterior cerebral artery distributes to the OCCIPITAL CORTEX via the basilar artery...so will an embolism from the VERTEBRAL artery occlude such that someone's eyesight is lesioned? Yes or NO? The NBME stresses pictures of the brain, so be ready to identify all the main diseases and which blood vessels distribute to its different parts!

A) Yes. All is true here.

14.
Case: A man comes in with urinary stones...a history of them. Name THREE places which you should identify on radiograph or a diagram where a stone can likely get stuck along the ureter. Up to 10 percent of folks get stones!

A) LARGEST of them is at the ureteropelvic junction. Next is the area of the Pelvic brim. The last area is the ureterovesical junction, (area where the ureter passes through the bladder wall.


15.
Case: You see a 28 year old male with unknown reasons for lymphadenopathy, weight loss, and these weird raised skin lesions all over his chest. Which one, (Kaposi's sarcoma, a CD4 count of 220, or a positive antibody test for HIV), confirms AIDS?

A) Kaposi's sarcoma. The USA standards are that you need a CD4 count under 200, regardless of symptoms...BUT, if you see something like Kaposi's sarcoma, an AIDS defining illness, you can identify it as AIDS.


16.
Case: You see a person with bipolar disease on Lithium. He has Diabetes insipidus as a long term consequence from SIADH. True or False for each:

1..will he show HYPERnatremia?

2...intracellular compartment swelling?

3...increased plasma osmolality?

A1) YES, from loss of free water.

A2) NO (think of osmotic properties)

A3) YES...


17.
Cases: 4 different patients with gout...

First guy is on a drug that work by blocking the renal reabsorption of uric acid. Is it sulfinpydrazone, probeneicd, BOTH, or NEITHER?

A) BOTH, think MOA...

18.
Case: Next guy with ACUTE gout runs in limping. Will you give allopurinol or indomethacin for the ACUTE gout?

A) INDOMETHACIN, an NSAID which blocks prostaglandin synthesis.


19.
Case: Another guy with gout walks in. He needs meds for chronic gout. You give colchicine. What is the MOA against the gout?

A) It blocks leukocyte migration AND phagocytosis secondary inhibition of tubulin polymerization.


20.
Case: Another person waltzes in with chronic gout. He has a weak GI tract. Which med, probenecid or colchine, are you worried about giving?

A..colchine...it can cause serious GI side effects.


21.
Case: Which common bug, H. flu, S. pneumo, or Staph. aureus, LACKS IgA proteases which help a bug infect mucosal surfaces?

A) Staph aureus.


22.
Name two out of many enzymes that S. aureus makes which degrade human cells for colonization...what do they do?

A) Think about the Identifying traits like Catalase positive and Coagulase positive. These two enzymes of S. aureus work thus:
Coagulase clots plasma. And catalase converts cellular Hydrogen peroxide to water and oxygen, limiting the cellular killing of the bacteria.


23.
Case: A friendly friend comes into your office complaining of symptoms from an acoustic neuroma at the cerebellar-pontine angle. What symptoms is he likely to show? And what two nerves are likely to be affected?

A) Vertigo, Auditory stuff, and facial muscle paralysis are seen. CN VII and VIII are often lesioned.


24.
Case: An aneurysm appears in the superior mesenteric artery at the level of LV2. Which is compressed, the left or right renal vein? Which is longer? Important since you will know which kidney is in danger.

A) The LEFT renal vein, which passes ANTERIOR to the aorta. The left renal vein is LONGER.


25.
Q) What is different about the drainage of the right ovarian vein and the left ovarian vein? Is there anything?

A) The RIGHT ovarian vein drains directly into the Inferior vena cava while the LEFT drains into the left renal vein first before the IVC.

26.
Q) Tell us about the MOA of the degradation of cortisol? Where does it occur?

A) It occurs in the liver, converted to tetrahydrocortisone. It is then converted into glucuronic acid via CONJUGATION. Now it is water soluble, and is then urinated out into the toilet or potty.


27.
Case: An older patient comes in with cataracts. Can it be due to sorbitol production in the lens? What common dx is associated with excess sorbitol production?

A) Yes, it is often due to diabetes mellitus.


28.
Case: A patient of yours named Jennifer Connolly steps on a nail in a house called "House of Sand and Fog." She suffers paralysis from Clostridia. Is there an exotoxin associated? What is the MOA?

A) Yes...the MOA is that an inhibitory neurotransmitter called GLYCINE is blocked from release from the CNS, causing tetanic paralysis.


29.
Case: A cases of a patient with a murmur...a diagram shows a crescendo-decresendo, ejection type, diamond shaped figure between S1 and S2. What valve is lesioned?

A) This is AORTIC stenosis. Both pulmonary and aortic stenosis occurs during systole. The sound diagram is evident when the blood rushes out thru the narrow opening.


30.
Q) We just spoke of aortic stenosis and the sound diagram. What about MITRAL STENOSIS, in relation to S1 and S2?

A)Opening snap, cresendo, decresendo, diamond/wedge shaped...you will see a cresendo wedge leading up to S1.


31.
Q) T/F. The aortic valve and pulmonic valve opens during diastole.

A) False, they CLOSE during diastole.


32.
Case: You are treating a patient with mitral regurg...how does the sound/time graph look like?

A) Try to imagine the mech of action (MOA), then you will not forget...this is a pansystolic or holosystolic murmur, so the graph will look like a rectangle, the line with zero slope, where the blood rushes back into the atria with a CONSTANT velocity.


33.
Q) Will tricuspid regurg look like mitral regurg on a sound vs. time graph?

A) Yes, both have the same MOA...think about it logically. They are coupled as are the pulmonary and aortic valves.

34.
Case: Another patient comes in with aortic REGURG...how will this sound/time graph look like? Please review in a cardio text, as my explanations are not the best without pics.

A) Think about what is happening...during DIASTOLE, there is an insufficient aortic value, so there is regurg, so then there is a high pitched blowing murmur AFTER S2, when the aortic valve does not close right as the heart is trying to fill the ventricles. You will see a descending wedge/triangle after S2.


35.
True or False: An S4 heart sound is shown on a graph superimposed a cardiac cycle graph. Is it associated with atrial contraction OR ventricular contraction?

A) ATRIAL contraction or atrial systole...also seen with a hypertrophic ventricule...also maybe a heart attack.


36.
Case: You see a cardiac cycle graph. Point to the exact place where you may see an S3....what is the MOA?

A) Right after the mitral valve opens, you may see an S3 as you hear the blood slam into the walls of the ventricle during diastole (rapid ventricular filling).


37.
Case) (Hint, this is the most posterior chamber in the heart). A woman with rust colored sputum, difficulty swallowing, cough, and a hoarse voice comes in. What heart disease does she have that we recently discussed? What is the mech. of action?

A) This is MITRAL STENOSIS...greater pressure need to overcome the stenosis results in a hypertrophy of LEFT ATRIUM. As this is most posterior, enlargement compresses the esophagus (difficulty swallowing), the lungs (pulmonary edema and cough and hemoptysis), damage to the recurrent larygneal nerve (horseness of the voice).


38.
Case: You hear a murmur radiating to the carotid arteries in a 65 year old smoker. He has angina and dizziness/syncope on doing gymnastics, and weak pulses on extremities. What is the MOA? What is the heart disease?

A) This is AORTIC STENOSIS. This results in left ventricular hypertrophy...as the heart must push against more resistance out of the heart. As a result, we see angina (due to lessened coronary artery refill), syncope because his exercise demands more oxygen and because the stenosis lessens the flow, and weak pulses for the same reason.


39.
Q) Quick, are you retaining? I repeat the angina case presentation with aortic stenosis. Quickly, what does the sound-pressure vs. time graph between S1 and S2 look like?

A) Remember the diamond shaped ejection murmur...


40.
Q) You see another diagram of ONLY the "rectangle shaped" sound/pressure vs. time graph between S1 and S2. This you recall is MITRAL REGURG. What is the MOST COMMON CAUSE of this dx?

A) Rheumatic fever from Group A beta hemolytic strep. Is this bug bacitracin sensitive??? Yes, it is.


41.
Q) Are Strep viridans partially or completely clear on hemolysis on blood agar? Are they susceptible to optochin?

A) They are alpha hemolytic (partially clear)...not beta hemolytic (which is completely clear). They are NOT susceptible to optochin.


42.
A patient presents with tertiary syphillis. You are shown a sound/pressure vs time graph where there is a decresendo after S2 (a wedge or triangle with a negative slop). What dx and MOA of the heart disease is this?

A) This is commonly caused when the aortic valve closes INSUFFICIENTLY. The subsequent REGURG causes the syphillitic aortic aneurysm.


43.
A guy named Big MAC is very tall and has a heart defect from a chromosomal anomaly. He has Marfan's syndrome.. What other TWO common illnesses can cause this aortic valve insufficiency? (hint: M=Marfan's, A=?, C=?)

A) A=ankylosing spondylitis, and C=coarctation of the aorta.


44.
Q) T or F: Release of CCK results in contraction of the Sphincter of Oddi.

F) It results in its RELAXATION. It is the gallbladder that contracts.

45.
Q) True or False: CCK release will cause the secretin potentiation to release enzymes and BICARBONATE from the PANCREAS.

A) True.


46.
T or F: CCK is released by the presence of carbohydrates into the colon.

A) False, CCK is released by the presence of FATS and protein into the DUODENUM.


47.
T or F: CCK has no effect on the rate of gastric emptying.

A) False, CCK SLOWS the rate of gastric emptying by constricting the pyloric sphincter.


48.
Case: you are pimped by the cardiology attending and shown a graph of the Jugular Venous Pulse with three peaks (a, c, v). What heart sound (S1, S2, S3, S4) does peak v represent. What is happening physiologically?

A) S3, The increased JVP is caused by the blood pressure against the closed tricuspid valve.


49.
Q) Same as the previous concept...what does peak c stand for in the JVP graph? When does it occur?

A) c=Right ventricular contraction, as the tricuspid valve pushes back into the atrium. Occurs right AFTER S1, when the mitral valve closes and the aortic valve opens.


50.
Q) Which aortic pressure is HIGHER as measured the the left ventricle, the exact point when the aortic valve opens OR when the aortic value closes? When?

A) Surprise...! It occurs at S2, when the aortic valve closes!

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