WE'LL GO AHEAD AND GET STARTED, THANK YOU ALL VERY MUCH FOR COMING. IT IS MY VERY DEEP AND PROFOUND PLEASURE TO HAVE THE HONOR OF INTRODUCING DR. DAVID HELLMANN, HERE TODAY. SO THIS PATIENTS IN THE CLINIC SURFROM 1987. SO DAVE WAS THE VICE CHAIR OF THE DEPARTMENT OF MEDICINE WHEN I AND SEVERAL OTHER MISFITS WERE CHIEF RESIDENTS AT JOHNS HOPKINS. AND DAVE WAS THE VOICE OF CALM, SANITY, REASON, THROUGHOUT A YEAR THAT WAS NOT OTHERWISE GIVEN TO THOSE KINDS OF EMOTIONS AND CAN YOU SEE THAT IN 19 QUEACH, MANY OF US HAD MUCH MORE HAIR AND SMILED A LITTLE MORE BROADLY. NOW, DAVE, WENT ON TO SHOW THE THINGS HE HAD SHOWN AS A HOUSE OFFICER AND FELLOW AS HE BECAME A CHAIRMAN AT BAY VIEW, AND AMONG THOSE ARE THE FACT THAT HE ACTUALLY THINKS THAT SEEING PATIENT SYSTEM IMPORTANT. CAN YOU BELIEVE THAT? AND BELIEVED IT WAS IMPORTANT AT TED BODYICIDE AND UNCALIFORNIAING--BEDSIDE AND UNWILLING TO STAND ON SIMPLE TRADITION. HE'S BEEN ENTHUSIASTIC ABOUT TRYING TO BRING TECHNOLOGY AND UNDERSTANDING TECHNOLOGY RIGHT BACK TO THE BODY SIDE SO THIS IS ONE EXAMPLE OF THAT. DAVE AS CHAMPIONED THE IDEA OF TAKING AN ULTRASOUND TO THE BEDSIDE SO YOU COULD LOOK AT THE HEART FUNCTION, LOOK AT THE HEART SOUNDS, BRING THE KINDS OF THINGS THAT ALL OF US THINK ARE IMPORTANT IN OUR DAY-TO-DAY INTERPRETATION INTO HOW HE DOES THINGS. NOW, AS I WAS GETTING READY FOR THIS, I CONTACTED MY FORMER CHIEF RESIDENTS AND I SAID, YOU KNOW YOU DO YOU HAVE ANYTHING YOU WANT TO REMIND ME ABOUT, ABOUT DAVE, I CAN'T TELL YOU THE THINGS THAT I REMEMBER. SO ONE OF THEM, RONIN RUBENOFF, WHO WENT INTO RHEUMATOLOGY. HAD TWO RECOLLECTIONS I WILL SHARE WITH YOU. FIRST DAY OF ROOM FELLOWSHIP, JULY 1, 1987, MANY OF YOU WERE NOT BORN, FIRST CLINIC, FIRST PATIENT, RONIN WALKS IN AND HERE'S A MAN IN A WHEELCHAIR UNDIAGNOSED AND IMMOBILE. AND HE WAS REALLY STUMPED. AND AT THAT POINT, IN WALKS THE CONSUMMATE CLINICIAN DAVE HELLMANN, WHO IN A FEW MINUTES SHOWED HIM 16 THINGS HE MISS ON THE PHYSICAL EXAM, SORTED OUT THE DIAGNOSE AND I GUESS SHOWED HIM WHY SUBSPECIALTY TRAINING WAS SO IMPORTANT AND THIS MAN TURNED OUT TO HAVE LIFE THREATENING RHEUMATOID VASC LIGHT AND I GUESS WAS HOSPITALIZED FOR THREE WEEKS BUT AS A RESULT OF WHAT DAVE HAD HAD DIAGNOSED AND INSTRUCTED HIS LIFE WAS SAVED AND AS RONIN SAID, I KNEW HIINDEED FOUND AN ASTUTE CLINICIAN AS A MENTOR. THE OTHER THING WAS PERHAPS CLOSER TO MY HEART, AS SOMEONE WHO WAS GRATEFUL FOR DAVE'S JUST DAMN GOOD SENSE AND IT IS WELL CAPTURED HERE, ONE OF RONIN'S INTERNSOMETHING AGAINST THE WISHINGS OF THE ATTENDING PHYSICIAN, RESIDENTS HERE, TAKE NOTE, DON'T DO THAT AND AS RONIN, AND DAVE WERE TALKING DOWN THE HALL, THE ATTENDING REALLY LIT INTO HIM AND GAVE HYMN A FIVE MINUTE TONGUE LASHING ABOUT THE SINS OF HIS HAPLESS INTERN, RONIN DEFENDED HIM AS BEST HE COULD AND STAMMERED AND HAD TROUBLE ARTICULATING MUCH. AND THEN AFTER THE UNHAPPY ATTENDING LEFT, DAVE IN THE KIND OF GENEROSITY AND WISDOM THAT HAS CHARACTERIZED HIS CAREER TURNED TO RONINAND SAID, EVERYBODY IS, AND--IN CERT THINGS HERE ABOUT THEOHELLER, SOMETIMES. [LAUGHTER] AND RONIN FOUND THAT THOUGHT THAT EVERYBODY CAN BE A LITTLE DIFFICULT EVERY NOW AND THEN, QUITE COMFORTING IN MANY SETTINGS SINCE THEN. BUT THESE CAPTURE DAVE'S WISDOM AND HUMANITY AND HIS CLINICAL EXCELLENCE. NOT WILLING TO REST ON BOTH TRADITIONAL AND NONTRADITIONAL TECHNOLOGY, DAVE'S BEEN EXPERIMENTING WITH PYRAMID FORCES AND HE MAY SHARE SOME OF THAT WITH US HERE TODAY. SO IT JUST GIVES ME GREAT PLEASURE TO INTRODUCE YOU DR. DAVID HELLMANN THE ALIKI PERROTI, PROFESSOR OF MEDICINE AND JOHNS HOPKINS BAY VIEW. DAVE? [ APPLAUSE ] >> THANK YOU STEVE, I'M DELIGHTED TO BE HERE, I WAS TOLD THESE WERE STRAIGHT FORWARD CASE FIST I WANTED I COULD LOOK AT LAST YEARS AND LOW AND BEHOLD, FOUND THAT THIS WAS DONE BY LARRY TIERNEY, SOMEONE YOU NEVER WANT TO FOLLOW IN CENTER FIELD. AND EACH OF THE CASES WAS TURNED OUT TO BE AT LEAST FROM MY STANDPOINT VERY PECULIAR. SO, I'M EAGER TO PARTICIPATE IN THE THINKING. THEOALSO TOLD ME IT WOULD BE FINE TO USE WHATEVER SKILLS HIAS CHAIRMAN OF MEDICINE MY GREATEST SKILL IS GETTING OTHER PEOPLE TO WORK WORK AND SO, BE CAREFUL. I MAY ASK FOR YOUR HELP. >> HELLO, I HAVE THE FIRST CASE. THE FIRST CASE IS A 22 YEAR-OLD MAN WITH SICKLE CELL DISEASE PRECEPTED FOR OUTPATIENT AND HAS [LOW AUDIO ] --COMING OUT HERE, REPORTED [LOW AUDIO ] WHEN HE WAS SEEN AT FIRST SCHEDULED VISIT HE LATER SPIKED TO--]LOW AUDIO ] DURING THE DAY. [LAUGHTER] SORRY ABOUT THAT. I GUESS--YOU DIDN'T HEAR MOST OF THAT. PAST MEDICAL HISTORY CONSISTED OF HEMOGLOBIN ALOE GENERATED AIC STEM CELL PLANTS IN 2006. LEFT HIP OSTEONECKROSIS, HAD IROAN OVERLOAD AND WAS GOING PHLEBOTOMY AND HE HAD AN APPENDECTOMY AND COLLISTECTOMY. HE WAS CURRENTLY ON FOALIC ACID, HYDROXY UREA AND. >> SO MAYBE PAUSE HERE FOR A SECOND AND SO, HEARING ABOUT THIS PATIENT, YOUNG GUY WHO HAS SICKLE CELL WHO HAS TWO ATTEMPTS AT BONE MARROW TRANSPLANTATION, WORRY ABOUT ALL THE INFECTIONS THAT PEOPLE WITH SICKLE CELL ANEMIA GET, HE'S HAD HIGH FEVER AND HEADACHES, AND IRON OVERLOAD IN PARTICULAR, PREDISPOSES YOU TO A NUMBER OF INFECTIONS, VIBRIO BEING ONE OF THEM AND WITH SICKLE CELL ANEMIA, SALMONELLA INFECTION IS PARTICULARLY AN ISSUE. I WONDER WHAT DEGREES BEEN IMMUNO SUPPRESSED AT PREVIOUS ATTEMPTS AT BONE MARROW TRANSPLANTATION, THOUGH I HAVE TO THINK ABOUT UNUSUAL ORGANISMS. AND IN GENERAL, I TRY TO DIVIDE THE WORLD INTO TWO TEXTBOOKS OF MEDICINE, THOSE--THAT TEXTBOOK OF MEDICINE FOR PEOPLE WHO HAVE HIV AND THAT TEXTBOOK OF MEDICINE FOR PEOPLE WHO DON'T HAVE HIV. I'M PRESUMING AT THE MOMENT HE DOESN'T HAVE HIV, BUT THAT'S ALWAYS IN THE BACKGROUND OF MY THINKING. >> SO HIS SOCIAL HISTORY CONSISTED OF--HE WAS FROM PUERTO REEKEE, HE LIVES WITH HIS--PUERTO RICO, DOESN'T HAVE ANY DRUG USE, WORK INDEED A FRIED CHICKEN RESTAURANT AND WAS SEXUALLY ACTIVE WITH HIS GIRL, NO CONDOM, MONITOR O GBA MUSRELATIONSHIP, AND NO CAT OR DOG. >> WE HAVE TO THINK ABOUT IN THE CARIBBEAN, THINGS WE SEE THERE AND THINGS THAT COME TO MIND ARE CERTAINLY MALARIA. HE'S SEXUALLY ACTIVE AND NOT USING CONDOMS AND SO, ALWAYS HAVE TO WORRY IN A YOUNG PERSON, IS THIS INITIAL PRESENTATION OF HIV OR SEXUALLY TRANSMITTED DISEASE. AND CATS, IT TURNS OUT, YOU WANT TO KNOW WHETHER IT'S A NEW CAT OR AN OLD CAT BECAUSE CAT SCRATCH FEVER IS REALLY LIMITED TO KITTENS AND NOT CATS THAT YOU'VE HAD FOR A WHILE. >> THIS WAS AN ADULT CAT. >> [LAUGHTER] IS. >> ON PHYSICAL EXAM, THERE WAS [INDISCERNIBLE]--LATE OR IN THE DAY, BLOOD PRESSURE WAS 97/50 AND HE WAS BREATHING 97% ON [INDISCERNIBLE]. IN GENERAL HE WAS NONTOXIC AND APPEARED VERY COMFORTABLE, HIS EGNT SHOWED MILD [INDISCERNIBLE]. AND HIS FEIGN EXCITATORY WAS CLEAR, AND HE DID HAVE A RIGHT INTERIOR CERVICAL LYMPHNODE THAT WAS MEASURING 2-CENTIMETERS. LUNGS CLEAR, CARDIAC EXAM WAS NO MURMURS, AND HIS GI EXAM SHOWED ABDOMINAL SCARS, NO ORGANO MEANIA HE FELT. HE HAD A CLOSE INFECTION OF GENITALIA THAT SHOWED NO LESIONS OR DISCHARGE. HE DID HAVE 2-CENTIMETER CAR ON HIS RIGHT UPPER CHEST WALL. >> SO COUPLE OF THINGS HERE. ONE IS THE HEIGHT OF HIS FEVER, THERE ARE NOT THAT MANY THINGS THAT GIVE YOU FEVERS OF GREATER THAN 40. MALARIA CERTAINLY IS ONE OF THE THINGS THAT I THINK OF IN INFECTIOUS DISEASES. I'M NOT SURE WHEN THE HEART RATE WAS OBTAINED BUT IT'S A PRETTY SLOW HEART RATE FOR SUCH A HIGH FEVER. THERE'S CERTAIN INFECTIONS THAT ARE PARTICULARLY ASSOCIATED--ASSOCIATED WITH THAT GAP. HIS BLOOD PRESSURE IS LOW. BUT NOT KNOWING THE BASELINE, NOT SURE WHAT TO MAKE OF THAT, OR NOT SURPRISED THAT AS SOME MILD SCLERAL HOMOLYSIS THAT SICKLE CELL COULD PRODUCE. AND THEN HE HAS A PRETTY JUICY LYMPHNODE, 2-CENTIMETERS AND CERTAINLY WOULD MAKE YOU WOR THAT'S THIS IS PATH LOGIC LYMPHNODE. BY FAR I THINK THE THINGS I'M THINKING OF MOST ARE INFECTIONS. THERE ARE THINGS NONINFECTIOUS THAT GIVE YOU SUCH HIGH FEVERS, ADULTS, SILLS DISEASE, DRUG REACTIONS, PITUITARY AXLES, NONE OF THESE SEEM TO FIT. I WOULD BE INTERESTED ANDg27 AMAZED HOW OFTEN PERIPHERAL BLOOD SMEARS ARE HELPFUL IN SORTING OUT WHAT'S GOING ON. SO LOOKING AT HIS PERIPHERAL BLOOD SMEAR, LOOKING FOR PARASITES, IT COULD BE HELPFUL AS WELL AS, WE CLEARLY WANT TO GET BLOOD CULTURES. >> SO HERE ARE LABS ON ADMISSION. HE HAD A NORMAL BASIC METABOLIC PANEL AS YOU CAN SEE, THE EXTENDED PANEL DID SHOW SLIGHTLY ELEVATED LIVER ENZYMES WITH AN AST OF 71 AND ALT OF 89 AND HIS T-BELLY WAS 3.8 WITH A DIRECT OF 0.3. PHOS FOCUSED ONITATE WAS.IN EFFECT AND LDH WAS 467. CDC SHOWED HEMEEE GLOBIN 11.1 AND MACKRY OF 30.8 AND HIS COUNT WAS 900 PLATELETS WERE 275. YOU CAN SEE 75% NUTRIFILL AND 50% LIMPS. LYMPHS. >> HE HAS A VERY HIGH FEVER WITH MODERATE ELEVATED TRANSAMNAISS AND THE LDH IS NOT A SURPRISE HERE. SOMETIMES WE THINK OF IT WITH CERTAIN INFECTIONS, SUCH AS PC P, BUT CERTAINLY IT COULD BE EXPLAINED BY HOMOLYSIS. HIS WHITE COUNT IS NEITHER, REALLY HIGH NOR REALLY LOW. AND I THINK OF OF REALLY LOW WITH THINGS LIKE RICKETIAL INFECTIONS. I WOULD BE INTERESTED IN IMAGING HIS CHEST, LOOKING AT URINE AND PAN CULTURING. HE HAD A HEADACHE WHEN HE CAME IN AND IT WOULD BE INTERESTING TO KNOW IF THE HEADACHE PERSISTED, LISTERIA CAN OCCUR IN SUCH PATIENTS BUT REALLY HIGH FEVER ITSELF CAN GIVE HEADACHES AND SO, AT THIS POINT, I DON'T HAVE A FOCUS OF THE INFECTION, NOT POINTING TO THE ABDOMENfC‡M OR ANY PARTICULAR FOCUS YET AND SO, AT LEAST CONCERNED ABOUT WHAT'S HAPPENED TO HIS HEADACHE. >> SO HIS HEADACHE SPONTANEOUSLY RESOLVED AFTER THE FIRST DAY. >> OKAY. >> SO ADDITIONAL LABS WERE OBTAINED HIS PERCENTAGE WAS 5.5 4n4 TEST WAS PERFORMED AND HE HAD A TSHO OF 0.08. 2.1 FOR TPTR, 1049, CPR OF 18, NORMAL CO AGS, URINALYSIS WAS BLAND. HE HAD NAYS O FAIR IRKING--NASOPHARYNGEAL WASH WHICH WAS NEGATIVE FOR VIRUSES HEPATITIS E TIGHT PHS DONE ANDA AND B IMMUNE AND LAST HEPATITIS C ANTIBODY WAS NEGATIVE. BLOOD CULTURES WERE DRAWN AND WERE PENDING AT THIS TIME. CHEST X-RAY, WAS PERFORMED WHICH SHOWED NO INFILTRATES AND NO EFFUSION AND A LIVER ULTRASOUND WAS PERFORMED, SHOWED BORDER LINE ENLARGED CENTIMETERS BUT OTHERWISE NORMAL LEVEL. >> WOULD YOU MIND TELLING ME WHAT THE NORMAL IS FOR PRETTHREE? >> I DON'T REMEMBER THE RANGE. >> I'M STRUCK THAT TSH IS LOW, THREE TFOUR, I DON'T CARRY AROUND THE NORMALS IN MY HEAD BUT I THINK THAT'S PROBABLY NOT TOO FAR FROM YOUR NORMAL. >> YOU'RE CORRECT. >> AND SO, HYPER THYROID STORM CAN ALSO GIVE FEVER BUT I WOULD THINK THAT TSH IN THIS CASE, IS--IT'S A FREE TTHREE IS NOT THAT HIGH AND I DON'T MAKE TOO MUCH OF THIS TSH. FAIR TIN IS VERY HIGH, BUT TO BE EXPECTED IN SOMEBODY WHOSE REALLY SICK AND ADULT SILL'S DISEASE, FAIRA TINS OFTEN OVER 13,000--I'M SORRY, OVER 3000. THE C-REACTOR PROTEIN BEING ELEVATED IT WOULD BE STUNNED IF IT WEREN'T AND AT THIS LEVEL OF ELEVATION IS COMPATIBLE WITH THE INFECTION. AND WE STILL DON'T HAVE A LOCALIZED SOURCE WITH THE CHEST X-RAY NOT SHOWING INFILTRATIONS AND REMIND THAD CAT CAT SCANS CAN SOMETIMES SHOW INFILTRATES WITH THE NORMAL CHEST X-RAY. THE LIVER ULTRASOUND AGAIN, BORDER LINE ENLARGED. SO, WORRIED ABOUT SYSTEMIC DISORDERS. AGAIN, MOSTLY, INFECTIONS, I DON'T THINK THIS IS A MALIGNANCY, DON'T THINK THIS IS AUTOIMMUNE DISEASE, AND OF THE INFECTIONS, STILL WORRIED ABOUT THINGS LIKE MALARIA. THE LYMPHNODE AND HIGH DEGREE OF FEVER MAKES ME WONDER ABOUT SCAT SCRATCH FEVER. --CAT SCRATCH FEVER. SO I WOULD STILL LIKE TO LOOK AT PERIPHERAL BLOOD SMEAR AND GET THE RESULTS OF THE CULTURES. >> SO HE DID HAVE A INITIAL PROOF BLOOD SMEAR WHICH WAS UNREVEALING FROM DAY ONE. SO HIS HOSPITAL COURSE, INITIALLY WHEN HE WAS WORKED UP FOR THE FEVER, PERIPHERAL BLOOD WAS DRAWN AND NEGATIVE FOR PARASITES AND SO, HE WAS STARTED ON [INDISCERNIBLE] [LOW AUDIO ] HE CONTINUES TO SPIKE FEVERS TO 39.2, FOR THE NEXT FEW DAYS, AND IT WAS STOPPED AFTER THREE DAYS, WHEN THE BLOOD CULTURES WERE NEGATIVE BUT THE FEVER PERSISTED ON THE THIRD DAY. >> SO IT IS TYPHOID THAT GIVES YOU THIS GAP BETWEEN HIGH FEVER AND HEART RATE. BUT BY THIS TIME, SALMONELLA OR TYPHOID SHOULD HAVE BEEN IDENTIFIED. AND SO, IT RAISES THE QUESTION, WHAT CAN CAUSE REALLY HIGH FEVERS, IN SOMEBODY WHO DOESN'T HAVE AN OBVIOUS PYOGENIC INFECTION AND AMONG INFECTIONS I THINK OF SOME OCCULT, AN ABSCESS CAN DO THIS; SO, AND WHERE COULD SOMEBODY TICK AN ABSCESS AS A YOUNG PERSON WITH SICKLE CELL, COULD BE IN BONE, SOMETIMES IN KIDNEY, CAN BE IN LIVER AS WELL. I WOULD LIKE TO KNOW HIS HIV STATUS. AND AND THEN THERE ARE A NUMBER OF PARASIDIC OR PARASIDIC INFECTIONS THAT I WOULD STILL BE CONCERNED ABOUT WITH MALARIA. AND THEN RIQUESTIAL INFECTIONS MAY NOT WE SPONDY LIGHTIS TO SEPH TRAIKS OWN SO THOSE WOULD BE MY THOUGHTS. >> SO WE--HIS ELEVATED LIVER ENZYMES ACTUALLY INCREASED ON DAY THREE AND YOU CAN SEE THERE ARE AST WENT TO 118. ALT, 146, T-BELLY REMAINS STABLE AND LDH WAS MORE ELEVATED AT 713. HIS CBC DID SHOW A MARKED CHANGE WITH THE LEUKOCYTOSIS NOW, WITH 1008 EXPHND THERE WERE 20% ATYPICAL LYMPHOCYTES THAT WERE SEEN AND AT THAT POINT ANOTHER PERIPHERAL SMEAR WAS OBTAINED. >> SO THE THINGS THAT COME TO MIND SEEING THE CONTINUATION OF THE LIVER FUNCTION ABNORMALITYS AND THE A-TYPICAL LYMPHOCYTES ARE A VARIETY OF VIRUSES WHICH CAN PRODUCE HIGH FEVERS AND PRODUCE LIVER FUNCTION ABNORMALITYS, SO MONONUCLEOSIS, CBV VIRUS IN REALLY YOUNG PEOPLE, TEENAGERS IS A SORE THROAT AND FATIGUE, BUT IN--USUALLY SOMEWHAT OLDER PEOPLE IT CAN PRESENT AS JUST LIKE A HEPATITIS. CMB CAN CERTAINLY DO THAT AS WELL. AND I WOULD STILL BE INTERESTED IN THICK AND THIN SMEARS AND WOULD BE INTERESTED IN KNOWING HIV STATUS. >> SO THICK AND THIN SMEARS WERE OBTAINED AND THEY WERE NEGATIVE. >> ARE YOU SURE? >> [LAUGHTER] >> YES. AND PERIPHERAL BLOOD SMEARS SHOWED THE ATYPICAL LYMPHOCYTES AND YOU CAN SEE THE MORPHOLOGY AS THE NOMENCLATURE SAYS, VERY TYPICAL, AND THE MORPHOLOGY AT THE CYTOPLASM, ALSO SOME VACCULES AND GRANULES YOU COULD SEE. >> SO THIS DOES ME THINK MORE AGAIN ABOUT AN HERPES VIRUS AND INFECTION. EBV, CMB, I DIDN'T MENTION MALIGNANCY AND SOMETIMES MALIGNANCY CAN PRESENT SUDDENLY WITH HIGH FEVER. AND I'M NOT ENOUGH OF A MORPHOLOGYST TO KNOW IF THESE ARE PLASTIC BUT I WOULD BE INTERESTED IN RESULTS FOR CMB AND FOR EBV. >> OKAY. >> THE LAB RESULTS WERE OBTAINED, HIS HEPATITIS C ANTIBODY WAS POSITIVE, HOWEVER, HIS VIRAL LOAD WAS NEGATIVE, HEPATITIS SURFACE ANTIBODY, AGAIN HIS MEAN STATUS WAS CONFIRMED. HE HAD NO ACTIVE HEPATITIS B INFECTION, DIDN'T GET THE IDG POSITIVE, IGM WAS NEGATIVE AND HIS HIV SIRROLOGY WAS NEGATIVE WITH AN UNDETECTABLE VIRAL LOAD. TOX O PLASMA IGG SIRROLOGYS AND IGM WERE NEGATIVE AND HIS PC R FLOWER BLOOD WAS NOT REACTIVE. STOOL STUDIES WERE OBTAINED AND THEY WERE ALSO NEGATIVE. >> SO IT LOOKS LIKE HE HAD BEEN EXPOSED TO DEANINGY, IN THE PAST AND IT GIVES YOU BREAK BONE FEVER AND POST COME FROM THE CARIBBEAN OR LOUISIANA. THEY HAVE USUALLY MUCH MORE PAIN THAN THIS AND THE FACT THAT HIS IGM WAS NEGATIVE IT ARGUES AGAINST THIS. HEPATITIS C, USUALLY DOES NOT GIVE YOU FEVERS. SOY I STILL WOULD BE INTERESTED IN HIS EBV AND CMB. >> SO DIAGNOSTIC TEST WAS OBTAINED AND AS YOU GUESSED THE EBV PC R FOR BLOOD WAS 600, HOWEVER THE CMB SIRROLOGY SHOWED POSITIVE IGM. AND AT THE NIH WE CAN DO P-SERVE FOR BLOOD, SHOWED 10,000, 750 COPPERS PERM. SO DIAGNOSIS IS ACUTE MONONUCLEOSIS. >> SO THE CASE THAT REMINDS US ALL THAT WHEN PEOPLE HAVE HIGH FEVERS WITHOUT LOCALIZING SIGNS, TO THINK ABOUT SYSTEM TEMMIC VIRAL INFECTIONS AND THAT EBV AND CMB CAN PRODUCE A HYPE TIGHT ISOTOPE LIKE INFECTION. >> THANK YOU. >> THANKS. O I'LL SPEAK A LITTLE BIT ABOUT CMB MONONUCLEOISEIS FROM WHAT I LEARNED ABOUT THIS CASE. >> SO IT'S A PRIMARY INFECTION IN PERSON WHO IS ARE PREVIOUSLY SIRY NEGATIVE. AMONG THE HETEROGENEOUS ROW FILE NEGATIVE ARE EBV MONONUCLEOSIS INFECTIONS AND IT'S USUALLY DIAGNOSED BY CMB, AND ACUTE INFECTION. AND HISTORICALLY, TRANSFUSION HAS BEEN A POTENTIAL SOURCE, BUT, IN MODERN TIMES THAT'S NO LONGER SELF-MANIFESTED AS A TYPHOID LIKE SYNDROME WHICH IS WHY OUR PATIENT INITIALLY WAS THOUGHT COULD HAVE BEEN PILOT PROJECT FOR--TYPHOID. SO AZINE IN OUR CASE, THERE WAS MILD HEPATITIS AND MOST CASES HAVE LESS INTENSE ATYPICAL APOPTOSIS AND RARELY EXPLAINED. BUT THIS IS SOMETHING I LEARNED IS THAT CAN YOU GET, IMMUNOLOGICAL VARIANCES WITH THE CRYO[INDISCERNIBLE] AND FACTOR AND THE ANTINUCLEIC ACID IN THE BODIES. >> GREAT, THANK YOU. >> THANK YOU. [ APPLAUSE ] >> SO I'M ALSO ONE OF THE FELLOWS. >> NICE TO MEET YOU. >> NICE TO MEET YOU. >> I'M IN INFECTIOUS DISEASE, SO I WON'T TELL YOUo[[ ANY HINTS. >> A GREAT ONE I'M SURE. [LAUGHTER] >> SO THIS IS AN INTERESTING PATIENT I SAW LAST YEAR IN THE CLINIC, CAME IN OUTPATIENT, 22 YEAR-OLD WOMAN, PREVIOUSLY HEALTHY IN MAY OF 2007 AND HER PRESENTING SYMPTOMS WERE GENERALIZED ITCHING AND A RASH. THE RASH JUST TO GIVE YOU MORE DETAIL, THEY WERE HYPER PIGMENTED PATCHES ON THE ABDOMEN AT FIRST, NOT EATER CARRIAL AND THEY RESOLVED WITHOUT ANY INTERVENTION OVER A COUPLE OF MONTHS AND THEN CAME BACK AGAIN TO BOTHER HER IN THE WINTER BETWEEN 2007-2008. >> SO HYPER PIGMENTATION OCCURS FREQUENTLY AND THERE IS THAT--IN AREAS THAT HAVE HAD INFLAMMATION AND GENERALIZED PATHWAY GIVESRITIS IS A PF PF P A RIETIS, IS A COMMON CONDITION THAT ISN'T WITH URCARIA, AND I THINK OF IT AS BEING ENVIRONMENTAL INDUCED, DRUG INDUCED, INDUCED BY RENAL FAILURE, PEOPLE WITH RENAL FAILURE CAN HAVE TERRIBLE P A RITIS, SO I'M READY TO HERE MORE. >> SO, THIS WAS AN ITCHY RASH THAT RETURNED IN THE WINTER. IT WAS ORIGINALLY IN THE ABNORMALITIES ABDOMEN, REAPPEAR INDEED THE ABDOMEN AND SPREAD TO OTHER PARTS OF HER BODY, INCLUDES FACE AND EXTREMITIES SHE HAD NEW SYMPTOMS SHE HAD ADNUMBER OF PATIENTSATHY, LOSING HER HAIR, IT WAS FALLING OUT NOW AND SHE WAS STARTING TO WEAR A WIG AND SHE WAS HAVING INCREASED FATIGUE. SO HE ACTUALLY IS ORIGINALLY FROM GHANA AND BECAUSE THE DOCTORS WEREN'T SURE WHAT WAS GOING ON WITH HER, HER PARENTS TOOK HER BACK THERE TO SEE DOCTORS. IN OCTOBER OF 2009 SHE WAS THERE FOR THREE MONTHS. THEY DIAGNOSED HER WITH TYPHOID AS THE CAUSE OF HER SYMPTOMS AND TREATED HER WITH IV ANTIBIOTICS SHE MIGHT HAVE GOTTEN OTHER MEDICINE THEN BUT SHE WASN'T SURE WHAT SHE GOT. HE HAD MARKED IMPROVEMENT OF SKIN, HER HAIR LOSS AND HER ITCHING. BUT WITHIN THREE WEEKS OF COMING TO THE U.S., SHE GOT THE SYMPTOMS AGAIN. >> SO I THINK OF A YOUNG PERSON WITH SKIN LYMPHNODE INVOLVEMENT AND ALOPECIA, I'M THINKING OF CONDITIONS LIKE LUPUS AND WOULD HELP TO KNOW IF SHE'S AFRICAN-AFRICAN SINCE LUPUS IS 10 TYPES MORE COMMON IN BLACKS THAN IN WHITES. I DON'T THINK SHE HAD TYPHOID. HER CONDITION IS BACK, ALSO, PERHAPS ISM JADED BUT I'M ALWAYS INTERESTED IN KNOWING WHAT SOMEONE'S HIV STATUS IS. AND I'M TRYING TO THINK OF ANY TOXINS THAT COULD DO THIS. USUALLY--WELL, I WOULD BE INTERESTED IN KNOWING IF SHE HAS ANY UNUSUAL DIET, IF SHE'S TAKING ANY MEDICATIONS, BECAUSE THAT'S A COMMON CONFOUNDER. >> WE'LL GET TO HER SOCIAL HISTORY IN A SEC. HER PAST MEDICAL HISTORY, SHE DOES HAVE HEMOGLOBIN C-TRAIT, FROM AFRICK AIRON DEFICIENCY ANEMIA, VITAMIN D DEFICIENCY, NO HISTORY OF ALLERGIC DISEASES OR SIGNIFICANT INFECTION AND NO OTHER HOSPITAL RISATIONS OR SURGERY. HE IS A COLLEGE STUDENT AT PENN STATE, MAJORING IN BEHAVIORIAL SCIENCES, NOTHING UNUSUAL IN ACTIVITY$„Z$F)– I TOLD THAT YOU SHE WAS FROM GHANA, ORIGINALLY. NO ALCOHOL, TOBACCO OR DRUG USE. SHE WAS NOT SEXUALLY ACTIVE. >> IRON DEFICIENCIES IS PRETTY COMMON IN YOUNG MEN STRAIGHTING WOMEN, ALWAYS GIVE SOME THOUGHT TO SEALIAC DISEASE, I THINK SILLIAC DISEASE IS MORE COMMON IN WHITES THAN IN BLACKS, AND CAN GIVE, CAN BE ASSOCIATE WIDE RASHES, LYMPHOID OPERATING GLOBALLY AT SCHEALOPECIA, AND, YOU'VE TOLD ME ABOUT HER HABITS AND THERE ARE NO BAD HABITS. MANY DISEASES OF YOUNGER PEOPLE ARE ASSOCIATED WITH THOSE PEOPLE. SO, I'D BE, I THINK INTERESTED IN EXAMINING HER. DO HAVE YOU ANY INFORMATION ABOUT HER, WHETHER SHE'S--SO NO RECREATIONAL DRUG USE, IS SHE TAKE ANYTHING MEDICATIONS OTHER THAN IRON? >> SHE'S ON NO MEDICATIONS AT ALL. NOT EVEN ANY HERBALS OR SUPPLEMENTS. >> OKAY AND ANY UNUSUAL DIET? >> SHE EATS A NORMAL DIET, NOT VEGETARIAN OR ANYTHING. >> SHE'S NOT HERE, BUT ONE OF THE THINGS I TRY TO ENKUHLICATE IN EVERYBODY, ALL OF MY TRAINEES, IS, THE EXTRAORDINARY VALUE OF ASKING PEOPLE WHAT THEY THINK IS GOING ON. I THINK IT WAS MAYBE WHEN STEVE HOLLAND WAS ONE OF THE RESIDENTS WE HAD SOMEBODY IN THE EMERGENCY ROOM AND NO ONE COULD FIGURE IT OUT AND FINALLY SOMEONE ASKED HIM AND THEY SAID WHAT DO YOU THINK THEY HAVE AND HE SAID I THINK I HAVE TOX EDUCATIONALLIAL MIRROR IMAGE IN MARKED FOR IDENTIFICATION-TOX EDUCATIONALLIAL MITOSIS? AND SHE WAS RIGHT. TURNED OUT HE WAS A TRUCKER SO WE DON'T SEE COX EDUCATIONALLIAL MITOSIS IN METHESTIMATE THAD DAILY BASIS BUT HE WAS A TRUCK WHORE HAD BEEN DRIVING THROUGH CALIFORNIA AFTER SOME OF THE EARTHQUAKES AND HE THEN READ ABOUT ALL OF THESE PEOPLE GETTING FEVERS AND OTHER SYMPTOMS IN HIS TRUCKERS NEW YORK TIMES AND HE SHOWED THAD UP AND SAID, THIS IS WHAT I THINK I HAVE. SO I FOUND THAT PATIENTS WILL OFTEN MAKE YOU LOOK VERY SMART IF YOU JUST ASK THEM. >> I CAN TELL YOU SHE DOESN'T HAVE COX EDUCATIONALLIAL TO HELP YOU OUT. >> DID SHE KNOW WHAT SHE HAD. >> SHE THOUGHT SHE HAD ONE THING AND IT WAS THE WRONG DIAGNOSIS. >> OKAY. >> HER PHYSICAL EXAM AND I'LL SHOW YOU PICTURES SINCE WE LIKE PICTURES WITH A RASH, SHE HAS NORMAL VITALS, SHE DID HAVE ALLO PEERIA, WAS ALWAYS LOSING EYE BROW HAIR, EXTENSIVE LIMP FOR IDENTIFICATION NUMBER OF PATIENTSATHY, FOUR TO 6-CENTIMETERS AND THE CERVICAL AND INNER THIGH AREAS SHE HAD MILD EDEMA IN HER FACE, I TOLD YOU ABOUT THE RASHES, THERE ARE SOME HYPER CAR O TO THETIC PATCHES NOW, THE REST OF THE EXAM WAS PRETTY MUCH NORMAL SO THIS IS HER WHEN WE SAW HER IN CLINIC AND CAN YOU SEE HER FACE IS AFFECTED, SHE HAS A BIT OF EYE AND LIP SWELLING. HER FEET HAVE THE HYPER CARE O TO THETIC PATCHING, NAIL VS PITTING IN THEM AND THEN YOU CAN SEE THE OTHER KIND OF PATCHES THAT WERE FOUND THROUGHOUT HER BODY. >> SO THESE LESIONS DON'T--I'M STILL THINKING ABOUT LUPUS BECAUSE ALOPECIA, LIP ADNUMBER OF PATIENTSATHY IN A YOUNG BLACK WOMAN AND THE LESIONS CAN BE HYPER PIGMENTED. I DON'T SEE ANY OF THE INFLAMMATORY LESIONS AND IT'S UNUSUAL IN MY EXPERIENCE THAT THE LESIONS ARE OF SKIN LESIONS OF LUPUS ARE RICHIE--ARE ITCHY, BUT LOSS OF THE EYE BROW SYSTEM PART OF THE GENERALIZED HAIR LOSS AND THERE'S NOTHING ABOUT THIS LOSS OF THE LATERAL PORTION OF THE EYE BROWS IS THE QUEEN ANN SIGN, SIGN FOR HYPOTHYROIDISM BUT THAT DOESN'T FIT WITH THE REST. HYPER PIGMENTED LESIONS, SHOULD MAKE YOU THINK OF LEPROSY, I DON'T THINK THERE AS NUMEROUS LEPROSY, I DON'T KNOW IF IT OCCURS IN WHERE SHE'S FROM, THE LESIONS ARE SAID TO BE HYPER PIGMENTED AND ARE ANESTHETIC. AND LEPROSY ALSO GIVES YOU ENLARGED NERVES. THE OTHER STRIKING--I DON'T THINK THIS IS WHAT WE SEE, THE OTHER ISSUE IS THE LIMP ADNUMBER OF PATIENTSATHY. WE DON'T SEE AS MUCH SYPHILIS ANYMORE BUT I THINK THAT SYPHILIS IS THE GREAT IMITATOR AND WITH SKIN LESIONS, I WOULD BE WORRIED ABOUT SYPHILIS AND MAKE SURE THAT SHE GOT AN RPR AND AN FTA BECAUSE THAT COULD NICELY TIE TOGETHER THE ALOPECIA, THAT LIMP ADNUMBER OF PATIENTS AT SCHEKIN LESION FIST I HAD A LIFE LINE IT WOULD BE TO GET ONE OF MY DERMATOLOGY FRIENDS, I THINK PORFERIA CUE TANIOUS RETARDA IS WITH HYPER PIGMENTED LESIONS ON THE PALMS AND HANDS AND SUN EXPOSED AREAS SO LESS SO IN THE FEET AND PROFERIOR CUTANYA RETARD IS PEOPLE WHO HAVE HIV OR [INDISCERNIBLE], SO THERE'S SEROLOGIC TESTS WITH THE LIMP NUMBER OF PATIENTSATHY, ALSO SHOULD GIVE SOME CONSIDERATION TO SARKOID THIS, DOESN'T LOOK LIKE SARKOID LUPUS PERNIAL BUT SARKOID WOULD BE ANOTHER THING TO THINK OF IN A YOUNG WOMAN WITH EXTENSIVE LIMP NUMBER OF PATIENTSATHY. THIS DOESN'T RING ANY BELLS FOR A MALIGNANCY AT LEAST RIGHT NOW. >> SO THE RASH WAS VERY, VERY, ITCHY AND SHE HAD SOME ABNORMALITIES IN HER BLOOD. HER WHITE COUNT WAS LOW. 3.44. SHE HAD AN ABSOLUTE ESL COUNT OF 540. HER ANEMIA WAS MICROSIDIC ANEMIA, THAT GOES ALONG WITH HER PREVIOUS DIAGNOSIS OF IRON DEFICIENCY AND SHE WASN'T ON SUPPLEMENTS. THAT COUNT WAS HIGH, 412. HER IMMUNOGLOBULIN SHOWED A HIGH IGE AND THE NORMALS AROUND 90 SO SHE WAS 62,000. HER FARRA TIN WASOT LOW SIDE, SHE HAD A NORMAL TRIP TASE, HER NEGATIVE PRETTY MUCH ALL THESE INFECTIOUS THINGS THAT WERE SEBT OUT, ALL THESE THINGS THAT WERE SENT OUT WERE ALL NEGATIVE IN TERMS OF LUPUS AND HER OMP WAS NEGATIVE WITH THE ESNFL AND IGE. AND SHE HAD A NORMAL CAREIO TYPE. >> SO THE LEUKOPENIA BEGAN TO FIT FOR LUPUS BUT THEN DROPPED IN MY EYES TO THE ANA AND NEGATIVE ANA IS VERY GOOD AT EXCLUDING LUPUS. SO I THINK LUPUS IS OFF AS HIV AND HEPATITIS C. WE STILL, I THINK WOULD NEED TO BE WORRY BODY SYPHILIS. --WORRIED ABOUT SYPHILIS AND SUCH AN EXTRAORDINARY HIGH LEVEL OF--THE E-CENOFILL COUNT OF 540 DOESN'T MAKE ME--I WOULD THAT'S A MILD ELEVATION SO I DON'T FIND A GREAT CLUE THERE. SUCH ASTRONOMICAL LEVELS OF IGE, SO IT DOESN'T SOUND THE REST FOR JOB'S SYNDROME FORM OF IMMUNO DEFICIENCY AND THERE'S NOTHING ELSE GOING FOR THAT, BUT I WOULD--BE INTERESTED IF ANYBODY HERE HAS ANY SUGGESTIONS FOR SUCH A STRIKINGLY ELEVATED IGE BUT PERHAPS IN THE INTEREST OF TIME, COULD YOU TELL ME IF THE RPR OR FTE WAS DONE. >> RPR WAS NEGATIVE, SAID SHE WAS A VIRGIN. NEVER SEXUALLY ACTIVE AT ALL. BILLED AS A RULE OUT HYPER IGE WHEN SHE CAME AS WELL AS THEY THOUGHT SHE HAD ATOPIC DERMATITIS. WAS THE ORIGE NAME DIAGNOSIS CAN CALLS CAUSE THE ACENOFILL AND THE IGE. >> YEAH. >> AND SO I THINK THE QUESTION IS WHETHER HYPER IGE EXPLAINS ALL OF THIS AND I WOULD BE INTERESTED IN HER CHEST IMAGING AND THE OTHER IMAGES OF OF HER LYMPHNODES, WITH NO INFECTION, I'M WORRIED MORE ABOUT THE PUBLICITY OF SOME MALIGNANCY AND WANT TO KNOW IF THERE'S ANY IMAGING THAT'S GOING TO STOP US FROM EITHER LOOKING AT THE BONE MARROW OR LYMPHNODE. >> WELL LET ME JUST SEE IF THIS IS HER IMAGING, CAN YOU SEE SHE HAS EXTENSIVE IMP ADNUMBER OF PATIENTSATHY BOTH IN CHEST AND PELVIS, THE REST OF THE CT WAS UNREMARKABLE. SHE DID HAVE A KIN BIOPSY, SHOWED DIFFUSE INFILTRATES OF LYMPHCITEDS AND FOLLICULAR SEN ILLEGALSENOSE AND I GUESS NO INCREASE IN MASS CELLS, THEY WERE THINKING ABOUT CUE TANIOUS MASSIVE SIGNIFYITOSEIS. >> SO I THINK THE DIAGNOSTIC PROSEEDSURE WAS TO BIOPSY A LYMPHNODE AND THE MASTER SIGNIFYITOSEIS IS CERTAINLY SOMETHING TO CONSIDER, THE NEGATIVE TRANSCRIPT ACE WENT AGAINST THAT. AND SOUNDS LIKE SHE'S GOT LOTS OF SUPERFICIAL NODES AND SO I'D BE INTERESTED IF THAT WAS VIRUS, SO THAT WAS WHAT WAS PERFORMED SHE A HAD A LYMPHNODE BIOPSY AND REPEAT SKIN BI OPPOSITE SCHESHOWED AN ATYPICAL INFILTRATE, WITH RESERVE NODAL ARCHITECTURE AND WE DID CLONING STUDIES AND SHE ACTUALLY HAD TWO CLONAL POPULATIONS, AND SHE WAS DIAGNOSED WITH CUE TANIOUS T-CELL LYMPHOMA THIS IS THE DOCTOR'S PIBLGHTURES OF HER CLONES. THEY TOOK HER BIOPSY FROM HER SKIN AND ENDED UP CLONE TAG POPULATION AND SEEING IF THE CLONES MATCH. SO BOTH HER SKIN BI OPPOSITE SCHELYMPHNODE BIOPSY HAVE THE SAME. >> SO VERY INSTRUCTIVE, VERY INSTRUCTIVE CASE. SOMETIMES HYPER CALCEMIA CAN BE A CLUE TO SOMEONE HAVING A T-CELL LYMPHOMA. >> SHE HAD NORMAL CALCIUM IN OUR CASE. SO A BIT OF CUE TANIOUS T-CELL LIMB NOME ASHE WAS HERE BECAUSE I'M ANALOGY IMMUNOLOGY FELLOW AND SHE WAS COMING IN ON PROTOCOL FOR ATOPIC GERM AND RULE OUT IGE OR JOB'S SYNDROME. SO ECZEMA, AS WELL AS CTC, AND IT CAN CAUSE ICENOFILL AND IIGE LEVEL SO WHEN YOU THINK OF SOMEONE WHO COMES IN WITH ATOPIC DERM, YOU WOULD THINK OF A KID. 90% OF PATIENT WHO IS HAVE TTHEIR DIAGNOSED BEFORE AGE FIVE. SO BECAUSE SHE WAS 22 AND COME NOTHING WITH THIS NEW ONSET, POSSIBLE ATOPIC DERMATITIS THAT WAS SOMETHING UNUSUAL THE MEDIAN AGE OF DIAGNOSIS FOR CTCL IS IN THE MID50S. IT IS UNCOMMON IN YOUNG ADULTS SUCH AS HER BUT THERE'S A SPECIFIC HISTOLOGICAL VARIANT WHICH SHE DID HAVE. 66% CAN BE CLASSIFIED AS MYCOSIS FROM DEVOID EASE OR [INDISCERNIBLE] SYNDROME AND HE HAD A TYPE OF MITOSIS IN THE FINAL DIAGNOSIS. SHE'S ACTUALLY DOING WORSE AND SHE'S BEING EVALUATED FOR A STEM CELL TRANSPLANT CURRENTLY AT CORNELL. >> IT'S INTERESTING, THE LAST CASE I SAW WAS SOMEBODY IN MY OWN CLINIC HAD THIS INCREDIBLE PATHWAY GIVESRITIS, HE WAS OLDER AND HAD MULTIPLE BIOPSYS AND I LEARNED FROM NOT ONLY THAT CASE BUT TO THINK OF INTENSIVE PORRITEIS BUT HE MAY NEED MULTIPLE BIOPSIES BEFORE YOU ACTUALLY GET THE ANSWER. THANK YOU, VERY INSTRUCTIVE. [ APPLAUSE ] >> HELLO I'M SHANNON ROUGH ATOM SWATY, I WILL PRECEPT THE NEXT CASE THIS SAY PREVIOUSLY HEALTHY 28 YEAR-OLD GENTLEMAN FROM CYPRESS. HE CARRIES A CDHONE MUTATION WHICH IS A GENETIC MUTATION, IN THE HERON GENE AND CARRIES A HIGH RISK OF DEVELOPMENT OF GHASTIC CANCER. SOME OF HIS FAMILY MEMBERS WHO WERE ALSO POSITIVE FOR THIS GENE MUTATION HAVE DEVELOPED GHASTIC CANCER. HE WAS REFERRED@ PROPHYLACTIC GASTRECTOMY AND HE HAD AN UPPER ENDOSCOPY PERFORMED SIX MONTHS AGO THAT SHOWED NO SIGNS OF GASTRITIS OR MALIGNANCY. HE HAD A TONSILLECTOMY AS A CHILD AND TOLERATED SURGERY WITHOUT PROBLEM, SOCIAL HISTORY HAD EXTENSIVE TOBACCO USE AND WHAT HE DESCRIBED AS SOCIAL ALCOHOL USE, NO DRUG USE, WORKING AS A FARMER AND MEET DELIVERY MAN IN CYPRESS. HE WAS A HUNTER AND HAD A BARN FULL OF RABBITS AND DOGS. AND HE ALSO SERVED IN THE GREEK ARMY. HE WAS ON NO MEDICATIONS PRIOR TO ADMISSION, EXCEPT FOR OCCASIONALLY ANT ACIDS. HE CAME TO US, I AM A SURGICAL FELLOW AND UNDERWENT A PROVE LACTIC LACTIC ACIDOSEISICT TOTAL GHAST OFFICER AS SCHEGHOST RONOSEIS. THE PROCEDURE WAS UNSUCCESSFUL HE WAS INTERNATIONAL CLASSIFICATION INTUBATED IN THE OPERATING ROOM. HE WAS TACKY CARDIC AND RISING WHITE BLOOD CELL COUNT. SO FOR US, FIRST THING WE DO IS GET A CHEST X-RAY WHICH SHOWED AS FOLLOW. THIS SHOWED EXTENSIVE FREEAR FOR ALL OF YOU WHICH MEANT HE A BREAK DOWN OF HIS ANAST MOASIS. TAKEN BACK TO THE OPERATING ROOM FOR REPAIR OF WHAT WAS AN OFTMOTTIC LEAK, THIS PROCEDURE WENT SMOOTHLY AND HE LEFT THE OR INTUBATE THD TIME WITH DRAINS IN PLACE. FOLLOWING EXBATION ON POST OPERATIVE DAYS FOUR AND TWO, HE WAS NOTED TO BE TACKY CARDIC TO THE 130S AND 140S. EXTREMELY HYPER TENSIVE, 170S AND 190S SYSTOLIC. ANXIOUS,AGEITATED NOT FOLLOWING COMMANDS SO SHE WAS GIVEN 5-MILLIGRAMS OF PERO DOLL, INTERMITTENT PUSHES OF HALLOW PERO DOLL OVER THE NEXT SIX HOURS. HE WAS START TED IMPERICALLY ON DOCIN AND VANCMYOCIN. THE NEXT MORNING HE REQUIRED REINTERNATIONAL CLASSIFICATION BATION FOR AIR WAY PROTECTION, NOTED TO HAVE A FEVER OF 38.8 WITH A WHITE CELL BLOOD COUNT OF 15. ELEVATED FROM THE MORNING. TRANSAMILLIO NAISS WERE TO BE NOTED TO BE SIGNIFICANTLY ELEVATED. DID A U-WAY WHICH SHOWED GREATER THAN 43,000 RED BLOOD CELLS AND A POSITIVE URINE MY O GLOBIN WITH AN ELEVATED CK OF GREATER THAN 4000. AT THIS TIME HE WAS REQUIRING EXTENSIVE AMOUNTS OF SEDATION ON PROP FALL AND FATINAL VERY HIGH LEVELS AND EVEN WITH THESE LEVELS OF SEDATION, HE HAD THESE EPISODES OF EXTREME TACHYCARDIA AND HYPERTENSION. >> SO A LOT HERE. SO I THINK THE MAJOR THING THAT COMES TO MIND THIS GUY IS POST-OPERATING GLOBALLY AND SICK AS STINK WITH AN OUTPOURING OF SYMPATHETIC DISCHARGE AND IT MAKES YOU WONDER ABOUT THE DRUG INDUCED PROBLEMS POST ANESTHESIA, HE HAS RAB DO MIRROR IMAGAL SIS, HE HAS CPK THAT'S MARKEDLY ELEVATED AND EURO MY O GLOBIN AND HE ALSO DOES HAVE SOME HEMOTURIA, BUT, I WOULD WONDER IF THIS IS ONE OF THE FORMS OF DRUG INDUCED MUSCLE SPASMS THAT SEROTONEIN SYNDROME LIKE PICTURE. OTHER THINGS, WE CLEARLY HAVE TO WORRY ABOUT SEPSIS, BUT THE DEGREE OF SYMPATHETIC DISCHARGE SEEMS TO GO AGAINST IT. NOW, THE GENETIC MUTATION HE HAS, THAT CAN SAY, I DON'T KNOW ANYTHING ABOUT THAT, BUT I DO KNOW THAT SOME OF THE GENETIC MUTATIONS TO PREDISPOSE YOU TO SEROTONIN SYNDROME LIKE AFTER MEDICATIONS AND I'DEB INTERESTED IN KNOWING IF THAT'S THE CASE=2w8 HERE. SO I'M THINKING OF SOME SEROTONIN SYNDROME, ANESTHESIA DRUG INDUCED WITHDRAWAL WAS MY FIRST THOUGHT, BECAUSE WE HAVE SO MANY PEOPLE WHO DO WELL BECAUSE THEY'RE TAKING THEIR DRUGS AT HOME AND WE BRING THEM INTO THE HOSPITAL AND THEY STOP, AND THEN THEY WITHDRAW, BUT ALCOHOL WITHDRAWAL, THIS IS BEYOND WHAT I WOULD EXPECT. IT IS TOO FAST AND ALMOST MORE VIOLENT THAN I WOULD HAVE THOUGHT. SEPSIS HAS TO BE KEPT IN MIND, BUT, RABDO-MILEIS FOLLOWING SERGEANTRY MAKES ME THINK SORT OF THIS SEROTONIN LIKE SYNDROME. >> HE DID GO THROUGH AN INFECT YOWS WORK UP INCLUDING WHEREON COSCOPY, CULTURES ALL WHICH WERE NEGATIVE. >> POST OPEN MEETING DAY SEVEN AND FIVE, COMPLETED THIS WORK UP. HE WAS HAVING THE SIGNIFICANT EPISODES OFAGEITATION, TACHYCARDIA AND HYPERTENSION, HE CONTINUED TO RESEIF BOTH IV-DELOT EDUCATIONAL FOR PAIN AND PERO DOLL FORAGEITATION, AT THIS POINT WE CONSULTED OUR NEUROLOGY AND PSYCHIATRY COLLEAGUES FOR ASSISTANCE. >> ONE OF THE OTHER THINGS THAT COMES TO MIND IS COULD THIS BE ACUTE INTERMITTENT PORT FERIA WHICH CAN ALSO BE PROVOKES, ONE OF THE PROVOCATURES OF AIP IS SURGERY. OR A MEDICAL ILLNESS. AND I THINK--I DON'T KNOW IF IAIP IS MORE COMMON IN THIS AREA OF THE WORLD HE COMES FROM, SAY FAMILIAR MED TERRAINION FEVER IS MORE COMMON IN THE AREA, BUT I WOULD ADD THAT, ADD THAT TO THE LIST. SO OUR COLLEAGUES RECOMMENDED A THERAPEUTIC TRIAL OF A CERTAIN DRUG WHICH WAS ADMINISTERED, AND THE PATIENT WAS NOTED TO IMPROVE DRAMATICALLY AFTER THE THERAPEUTIC TRIAL. >> SO I MUST SAY I DON'T KNOW HOW TO TREAT THE SEROTONIN SYNDROME. >> NORDID WE. >> THE INTRA LEAN WAS THE DRUG THAT WAS RECOMMENDED WHICH LED TO RESOLUTION OF THE FEVERS AND STEP WISE IMPROVEMENT INNAGEITATION. HE WAS DIAGNOSED WITH NEUROLEFT ANT MALIGNANT SYNDROME, WHICH WE HAD NEVER SEEN BEFORE AND PROBABLY WILL NEVER SEE AGAIN IN NIGH CAREER. A LITTLE BIT ABOUT IT THAT I'VE LEARNED, THE DIAGNOSTIC CRITERIA, AND IT REALLY IS A DIAGNOSIS OF EXCLUSION, EXPOSE TOWER A DOPAMINE ANTAGONIST OR AGANIST WITHDRAWAL. IN HIS CASE, HE DID IMPROVE AFTER CESSATION OF HAL DOLL, HYPOTHERMIA, RIGIDITY WHICH WE DID NOT HAVE, MENTAL STATUS ALTERATION, PK ELEVATIONS AND CCANs ROSE TO I LEVEL OF GREATER THAN 300,000 OVER THE COURSE OF HIS HOSPITAL COURSE. SYMPATHETIC NERVOUS SYSTEM ABILITY WHICH HE DEFINITELY HAD, HYPER METABOLISM AND TACHYCARDIA, A NEGATIVE WORK UP FOR OTHER UNDERLYING CAUSES. A REMINDER THE MUSCLE RELAXANT THAT REACTS BY THE EXCITATION ASK COUPLING AND MUSCLE CELLS BY DECREASING INTRACELLULAR CALCIUM CONCENTRATIONS. HE HAD EVERY SINGLE ONE OF THESE CRITERIA WITH THE EXCEPTION OF RIGIDITY AND HE DID IMPROVE. THAT WAS THE DIAGNOSIS WE WERE LEFT WITH. >> AND I APOLOGIZE BUT THOSE WERE THE TERMS I WAS TRYING TO THINK OF OF WITH THIS POST ANESTHESIA WHICH CAN RESEMBLE THE SEROTONEIN SYNDROMES THAT CAN BE PRODUCED BY OTHER DRUGS. >> HE DID MAKE A COMPLETE CLINICAL RECOVERY. >> THANKS VERY MUCH, I WAS WONDERING IF WE COULD TRY TO GET TO THE FOURTH CASE. [ APPLAUSE ] >> I UNDERSTAND OUR FOURTH PRESENTER WAS OFFERED A BOX OF CHOCOLATES IF SHE COULD NOT PRECEPT HER FOURTH CASE. >> I GUESS I WILL HAVE TO GIVE THEM BACK. >> SHARE. >> I'M RACHEL I'M ONE OF THE FELLOWS, SO, WE HAVE A 26 YEAR-OLD MOROCCO AN MALE, PREVIOUSLY HEALTHY UNTIL FOUR YEARS AGO, AND AT THAT TIME, AFTER SWIMMING HE DEVELOPED INCREASED CHEST PRESSURE, WHEEZING AND COUGHING UP A HALF A CUP OF BLOOD. HE WAS TREAT WIDE ANTIBIOTICS AND TOLD HE HAD ASTHMA, HOM NUMBER OF PATIENTSIS OCCUR WIDE SWIMMING OR EXCESSIVE RUNNING OUT DOORS. SO PERT WERE NIGGATIVE. NO DISNIA, NO FEVERS, NIGHT SWEATS OR CHILLS, NO RECURRENT INFECTIONS OR OTHER PRIOR RESPIRATORY INFECTIONS NO JOINT PAIN OR RASHES. SOCIAL HISTORY, BORN IN MOROCCO O, MOVED TO THE UNITED STATES 10 MONTHS BEFORE ONSET OF SYMPTOMS. WORK INDEED A RESTAURANT PART-TIME AND GOES TO SCHOOL FULL-TIME AT MONTGOMERY COLLEGE. HE SMOKED TOBACCO ABOUT ONE CIGARETTE FOR TWO DAYS FOR FOUR YEARS AND QUIT ONE YEAR AGO. HE DRINKS ONE BEER PERDAY AND SHIMS MORE ON OCCASIONS, NO ILISSITY DRUG USE AND NO PETS AT HOME. TWO BROTHERS, ONE SISTER, ONE SISTER HAD TUBERCULOSIS SIX YEARS AGO THAT WAS TREATED. HIS PARENTS ARE ALIVE AND HIS FATHER HAS SCHIZOPHRENIA. UNSURE ABOUT THE HEALTH OF HIS GRAND PARENTS THEY DON'T SEEK MEDICAL HELP IN MOROCCO O. >> SO COUGHING UP A HALF CUP OF BLOOD IN A YOUNG PERSON MAKES ME THINK OF A COUPLELE OF THINGS, THE FIRST IS FIND OUT WHETHER HE'S COULD YOU HAVING IT UP FROM HIS LUNGS. WE HAD A CASE NOT TO MANY YEARS AGO OF SOMEBODY WHO THOUGHT TO HAVE AMOPPETIS BUT HAD A TONGUE LESION FROM A SEIZURE AND THEY WERE BLEED PROGRESS THEIR TONGUE AND PEOPLE MISTOOK THEY WERE BLEEDING THE TOURS OF THE BLOODING. YOUNG PERSON YOU THINK ABOUT INFECTIONS BUT INFECTIONS USUALLY DON'T GIVE YOU SUCH MASSIVE HOM OPTICAL IMAGES SIS. TB CAN, AND SO I THINK THAT THAT'S ON THE LIST. IN A YOUNG PERSON WHO HAS HOM OPEN MEETING THEY--THE SIS, I WOULD THINK OF SOME INFLAMMATORY ANOMALY OF A BLOOD VESSEL IN A LONG TACKY CRASHUE O HOM OPTICAL IMAGES SIS TURNS OUT TO BE ONE OF THE NOT UNCOMMON FAN FESTATIONS OF TACK CRASHY, IT'S ONE OF THE FEW FORMS OF VASC LIGHTIS THAT COULD INVOLVE LARGE VESSELS AND INVOLVE THE LUNG. SARKOID IN WHICH YOU CAN GET A POCKET FROM A FUNGUS CAN ALSO GIVE YOU HOM OPEN MEETING THEY--THE SIS, PULMONARY HYPERTENSION COULD DO THIS, WOULD WONDER ABOUT A CONGENITAL ANOMALY AND THEN THINGS THAT CAUSE YOU TO BLEED INTO THE LUNG ID NO PATHIC HEMOSITTAROSIS OR GOOD PASTURES DISEASE, BUT I WOULD EXPECT HE WOULD BE SICKER IN BETWEEN. >> SO HE'S ON NO MEDICATION AND HE HAS NO DRUG ALLERGIES. >> I'M SORRY, I LEFT OUT SWORD SWALLOWING, THAT'S ALWAYS ONE THAT--ONE SHOULD KEEP IN MIND. [LAUGHTER] >> PHYSICAL EXAM, HE WAS AFIBERAL, NORMAL ATTENTIVE REZONING PIRRATIONS 18, ONE NOTWITHSTANDING ROOM AIR, ALERT, ORIENTED NO ACUTE DISSTRESS, HIS EXAMS WERE EQUAL AROUND REACTIVE LIGHT, ORAL FEIGN EXCITATORY WAS CLEAR, PULMONARY NONLABORRED BREATHING BILATERALLY, ABNORMALITIES DOMIN WAS SOFT, NONTENDER NONDISTEND, NO CLUBBING, NO JOINT SWELLING, NO EDEM AND NO RASHES. >> SO TACK CRASHYS IF YOU CAN DO ONE THING IT'S EXAMINE THE LEFT ARM BECAUSE THE MOST COMLY INVOLVED ARTERY IS THE LEFT SUBCLAVIAN. AND NOTING A DIFFERENCE IN BLOOD PRESSURE PARTICULARLY IN THE LEFT BRACHIAL, SOMETIMES IN TACK CRASHYS CAN YOU HEAR [INDISCERNIBLE] OVER A VARIETY OF PLACES. DOESN'T SOUND LIKE THAT WAS HEARD HERE. AND THERE'S NO LYMPHADENOPATHY TO SUGGEST SARKOID, YOU HAVEN'T DESCRIBED THE HEART EXAM SO I'M INTERESTED IN HEARING ABOUT THAT. >> IT WAS NORMAL. >> OKAY. >> NO MURMURS. >> OKAY. THESE ARE THE LABS WE HAD. SO NORMAL WHITE BLOOD CELL COUNT, HEMOGLOBIN, MILDLY ELEVATED 15.8. 48.5. CHEMISTRY LISTED THERE, NORMAL, HIV WAS NEGATIVE, AND ANK O WERE NEGATIVE, AND ANTIDOUBLE STRANDED DNE AND ENA AND WERE ALL NEGATIVE. ANTITRIP SIN, CTHREE, CFOUR COMPLEMENTS NEGATIVE AND RHEUMATOID FACTOR JUST MILDLY ELEVATED. >> SO A LOT OF DISUSES RULE TED TED--RULED OUT. THE RHEUMATOID FACTOR KEEPS SARKOID IN AND YOU CAN GET HYPER GAM GLOBUE LYNN AND A POSITIVE FACTOR, I THINK WE'RE KEEN TO IMAGE TO CHEST. >> FUNCTION TEST FIRST. BUT THEY WERE NORMAL EXCEPT A MODERATE DIFFUSION DEFECT AND THE CARDIAC ECHO SHOWS NO EVIDENCE OF PULMONARY HYPERTENSION AND NORMAL RV SIZE AND FUNCTION. OKAY. >> CHEST X-RAY. >> HARD TO SEE ON HERE, BUT YOU CAN SEE, THERE, OKAY. >> LOOKS LIKE SOMETHING IN THE RIGHT [INDISCERNIBLE]. SO THERE'S-- >> LARGE PULMONARY ARTERY. >> AND IT'S SORT OF FAINT BUT CAN YOU SEE CYSTIC STRUCTURES IN THE PATHWAY GIVES RENKT MA AND THE LATERAL SHOWS YOU A BIT BETTER FROM THE PA. >> COULD THIS BE BRONCH ECTO SIS AND IN A YOUNG PERSON COULD THIS BE SIS TICK FIBROSIS WITH HEMORRHAGE. >> A LITTLE CLEAR ON THIS. SO TREMENDOUS CYST FORMATION AND A LOT OF IT SEEMS TO BE MORE AT THE BASES WHICH WOULD MAKE ME WONDER ABOUT ALPHA ONE AND TRIP SIN DEFICIENCY BECAUSE AT THE BASIS IS MORE COMMON THERE. >> TRANSCRIPTIN WAS NEGATIVE. OR IN THE NORMAL RANGE. SO THERE WAS DIAGNOSISTIC TEST PERFORMED WHICH I THINK WAS THE MOST HELPFUL FOR EVERYONE INVOLVED IN THIS CASE. AND THE PESMENS WERE TAKEN FROM THE LEFT LOWER LOBE AND THIS IS THE PATHOLOGY FROM THE LEFT LOWER LOBE, SO YOU SEE, ECENOFELLIC AND HYLAND AMORPHIS MATERIAL WITHIN THE PARAVASCULAR AND INTERSTITIAL SPACE HERE; THAT HAVE ABSOLUTELY NO NORMAL LOW ARCHITECT NUR THE SLIDE. >> I DON'T FINISH THIS IS PULMONARY HEMASITROSIS BUT WHY DON'T YOU TELL ME? >> WELL THERE'S ONE MORE STAIN PROBABLY BE VERY HELPFUL. SO THERE WAS A CONGATE KEEPER RED STAIN WITH THESE MORPHIS MATERIAL STAINING, ORANGE SALMON COLOR IN A NODULAR PATTERN. >> SO, PULMONARY AMLLOYD IS THE THING I LEFT OFF THE LIST AND SO, WHY DOES THIS YOUNG GUY HAVE AMLLOYDOSEIS AND AND I THINK OF DISEASE OR SOME CHRONIC INFECTION, AT THIS AGE, I WOULD THINK HE'S GOT ONE OF THE FAMILIARIAL FORMS OF FAMILIES ILLEGALSIAM FORMS OF AMLLOYD, YOU VALID TO HAVE MEDITERRANEAN FEVER OR SOMETHING LIKE THIS BUT I THINK THIS IS ONE OF THE FAMILIES ILLEGALSIAM VALID AND RELIABLE VARIANTS OF AMLLOYD. >> SO HE WAS DIAGNOSED WITH PULMONARY NODULAR AMLLOYDOSEIS. SO JUST BRIEFLY, AMLLOYDOSEIS LIMIT TO THE RESPIRATORY TRACT IS UNCOMMON HOWEVER SHOUGH GRINS AND CYSTIC HAVE BEEN INVOLVE WIDE THIS AND TYPES OF PULMONARY AMLLOYDOSEIS AFFECT IT IS TRACHEAL BRONCHIAL TREE OR CAUSE DIFFUSE ABNORMALITYS OR SINGLE MULTIPLE CYST OR NODULES AND DIAGNOSIS IS, DIAGNOSTIC IMAGES ISN'T ALWAYS SPECIFIC AND RELYS ON THE BIOPSY AND TREATMENT IS LIMITED BUT THIS PATIENT IS STILL DOING PRETTY WELL RIGHT NOW. >> THANK YOU. I THOUGHT THEY WERE FANTASTICALLY INSTRUCTIVE CASES. I ENJOYED BEING WITH YOU. THANK YOU VERY. KD--THANK YOU VERY MUCH. [ APPLAUSE ] .