Maputo Morning Report (Kwan)-1

8/14/2019 Maputo Morning Report (Kwan)-1

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U C S D M O R N I N G R E P O R T

P R E S E N T E D B Y B R I A N K W A N

Yellow Man Syndrome

Sndrome do homem amarelo


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Uma Histria Muito Peculiar

Chief Complaint: People are telling me that I amyellow, and my urine is dark.

History of Present Illness:

84 yo male with past medical history of emphysemaand hypothyroidism presents with painless jaundiceas well as darker urine for one week. He was seen inclinic the day of admission and was told that his skin

was yellow, which he did not personally notice. He

thought that he was coming to the hospital for hisshortness of breath, which was unchanged from

baseline.


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A Histria Continua

This patient was a very poor historian. He noticedthat he has had darker urine for several days prior topresentation. He also noted pale stools for several

days prior to presentation accompanied by diarrhea.He thought that they were strange appearing becausethey floated around the toilet bowl like wispy clouds.He does not keep track of how many times he goes a

day but just that they are large and loose andbothersome. Denies any abdominal pain, fevers orchills. Has not been nauseous and has not vomited.


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Questions? Tm perguntas?


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The plot thickens

He denies ever having this problem before, claiminghe is very healthy and does not take any medications.

He denies any black stools or bright red blood per

rectum. He has not lost any weight as far as heknows. He denies any night sweats.

He has had a problem with itching all over his bodyfor a couple of weeks associated with a rash on his

trunk. He lives at a Veterans home in which thedoctor prescribed an unknown substance which thenurses smeared all over his body, which caused hisskin to burn.


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Past Medical History (Otra Histria Mdica)

Chronic Obstructive Pulmonary Disease

Hypothyroidism

Cataract Surgery (11/2009, 2/2007)


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Allergies (Alergias)

No known drug allergies


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Medications (Medicamentaes)

Prednislone eye drops


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Social History (Histria Social)

Tobacco: Smoked for 56 years; quit 13 years ago.

Alcohol: Drinks rarely now. Drank moderately as ayouth in the Marines.

Illicits: Denies any recreational drug use. Denies anyhistory of intravenous drugs.


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Social History (Histria Social)

Other: Denies having any tattoos or bloodtransfusions. Of note, he frequently goes to Mexicoto help improverished families. He drinks bottled

water usually, but when he eats with the families, hepartakes in their food.

Back in 1950s, he did report living in northern Africafor a few years briefly and also reported spending

some time in Okinawa and Japan.


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Physical Exam (Examinao Fsica)


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Skin: Jaundiced. Multiple erythematous pruriticpapules on anterior chest wall with excoriationmarks, multiple skin tags and moles throughouttrunk. Some ecchymoses on the arms. Noteleangiectasias.


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Head, Ears, Nose, Throat: Pupils equal and reactiveto light and accomodation, icteric sclera, conjunctivapink, oropharynx clear, moist mucous membranes

Neck: Supple, no cervical lymphadenopathy, noaxillary, epitrochlear, or inguinal lymphadenopathy

Cardiovascular: Flat jugular venous pulsations, rateregular. Normal S1 with physiological splitting of the

S2. No murmurs, rubs or gallops. Lung: Diminished breath sounds and bibasilar

wheezes at the bases


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Thoughts? Differential?


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Labs (Laboratrios)

Coagulation Tests:Prothrombin Time (PT) = 13.1 [9.6-13]

International Normalized Ratio (INR) = 1.2

Partial Thromboplastin Time (PTT) = 36.3 [24.2-36] Urinalysis: Cloudy, orange, pH = 6 [4.5-8.5], 3+

bilirubin, 2-5 WBCs, trace LE, negative nitrites,negative blood/ketones/protein

Other labs:Acetaminophen Level < 10

Ethanol < 10


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Abdominal Ultrasound

1. Gallbladder sludge with gallbladder wall thickening,nonspecific, representing nonfasting state versuscholecystitis.

2. Prominent pancreatic duct.3. A 6.3 cm left midpole renal cyst.

4. A 1.7 cm right superior pole renal cyst.

5. A 1.4 cm right hepatic lobe cyst.


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CT Abdomen and Pelvis


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CT Abdomen and Pelvis


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Other labs? Outros Laboratrios?


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Labs (Laboratrios)

Hepatitis Serologies:

Anti-HAV Antibodies: Positive

Hepatitis B Surface Antibody: Positive

Hepatitis B Surface Ag: NegativeHepatitis B Core Antibody: Negative

Hepatitis C Antibody: Negative


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Asleep? Adormecido?


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Labs (Laboratrios)

Iron Studies:

Fe = 135 [36-160], TIBC = 133 [228-428]

Transferrin = 95 [200-400], Iron Saturatation = 102%.

Ferritin = 7814 [30-400] Autoimmune tests:

ANA within normal limits, antismooth muscleantibodies within normal limits, P-ANCA and C-

ANCA not detectable


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Labs (Laboratrios)

Other interesting labs:

Anti-HAV IgM = Positive

HFE genetic mutation = Heterozygous for C282Y

mutation


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Summary

The patient had a conjugated hyperbilirubinemiawith a story concerning for obstructive jaundice.However, after imaging, there was no evidence for anobstructive jaundice. We did receive the positiveIgM Hep A, and his liver function tests began totrend down at the time of discharge.

However, we did investigate other causes of acute

hepatitis, and found that the patient had a very highferritin, high iron saturation, and was heterozygousfor the HFE gene, concerning for his being a carrierfor hemochromatosis.


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Iron Absorption

FERROPORTIN

DMT1 moves iron into cell

until apoferritin saturated

Ferroportin moves iron out of

cell, but controlled by hepcidin

If iron does not leave cell

within 72 hrs, cell sloughedand iron lost


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Hepcidin

Hepcidin20-25 AA member of the defensin familyof antimicrobial peptides mainly expressed byliver, inhibits iron export from enterocytes

(decreasing absorption) and macrophages Uptake from the gut is controlled by Hepcidin

which regulates the activity of Ferroportin

Hepcidin binds to Ferroportin causing it to beinternalized and degraded

R l f H idi (IL 6)


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Role of Hepcidin(IL-6)

Ganz T. Blood 2003;102:783-8

Iron deficiency andhypoxia inhibit hepcidin

synthesis, and ironexcess and inflammatorycytokines stimulate itsproduction.


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Hereditary Hemochromatosis

HFe gene on chr 6; homozygosity for mutation(CY) at position 282(85% of cases), 63(


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Hereditary Hemochromatosis

Prevalence is about 1 case in every 300 people. Mostare of Northern European origin.

10% estimated carrier prevalence in Western

countries Adult onset disease

H h t i S t


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Hemochromatosis:SymptomsPrevalence(%)

Symptom Women Men

Fatigue 65 42

Pigmentation 58 45Arthritis 45 35

Cirrhosis 14 26

Cardiac 14 15Diabetes 7 16

Moirand et al, Ann Intern Med 1997;127:105


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Thank you! Obrigado!

Maputo Morning Report (Kwan)-1

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