Case ConferenceCase Conference大林慈濟醫院心臟內科大林慈濟醫院心臟內科

林志達醫師林志達醫師

Chart No:T123380231Chart No:T123380231Name:Name:孔孔XXXXSex: MaleSex: MaleAge: 21 Age: 21 y/oy/oDate of admission: 94Date of admission: 94--1212--3030Date of discharge: 95Date of discharge: 95--0101--1111

Chief complaintChief complaint

Abdominal pain since 10 days agoAbdominal pain since 10 days ago

Present illness ( I )Present illness ( I )

The The 21 y/o21 y/o male patient was relatively well male patient was relatively well before.He suffered from before.He suffered from abdominal painabdominal painsince 10 days ago. Besides, since 10 days ago. Besides, headacheheadache, , excessive sweatingexcessive sweating were also complained. were also complained. He was admitted to NCKUH at first, where He was admitted to NCKUH at first, where high blood pressurehigh blood pressure and and abdominal massabdominal masswere noted.were noted.

Present illness ( II )Present illness ( II )

Because the symptoms did not improve, Because the symptoms did not improve, he was sent to our ER, where BP up to he was sent to our ER, where BP up to 248/149 mmHg248/149 mmHg was found. Abdominal CT was found. Abdominal CT revealed a revealed a large retroperitoneal masslarge retroperitoneal mass. He . He was admitted for further evaluation and was admitted for further evaluation and treatmenttreatment

Past historyPast history

1.1.DM(DM(--), HTN(), HTN(--), Hyperlipidemia (), Hyperlipidemia (--), ), 2.No operation history 2.No operation history 3.No alcohol consumption3.No alcohol consumption4.No Smoking4.No Smoking5.Drug allergy: 5.Drug allergy: pyrinepyrine

Family historyFamily history

No DM, HTN, heart disease No DM, HTN, heart disease history, endocrine diseasehistory, endocrine disease

Physical examination at ER ( I )Physical examination at ER ( I )

Vital sign: BT:37.8 C, BP: Vital sign: BT:37.8 C, BP: 248/149248/149 mmHg, mmHg, HR: HR: 9191 bpmbpm, RR: 18, RR: 18Cons: clearCons: clearHEENT: conj: not pale; sclera: not HEENT: conj: not pale; sclera: not ictericictericNeck: supple, no LAP,no jugular vein Neck: supple, no LAP,no jugular vein engorgementengorgementChest: symmetric, clear breathing soundChest: symmetric, clear breathing sound

Physical examination ( II )Physical examination ( II )

Heart: RHB, no murmur, S3(Heart: RHB, no murmur, S3(--), S4(), S4(--))abdomen: soft, abdomen: soft, mild diffuse tendernessmild diffuse tenderness, , bowel sound: bowel sound: normoactivenormoactive, bruit(, bruit(--))Extremities: freely movable, no pitting Extremities: freely movable, no pitting edemaedemaSSkin: kin: wetwet

Lab ( I )Lab ( I )

WBC: WBC: 11600/ul 11600/ul N. band 7%,N. band 7%, N. N. segseg 62%62%Lym.12%, Mono 5%, Lym.12%, Mono 5%, EosinoEosino 1%1%Aty.lym.2%, Aty.lym.2%, Meta 1%, Meta 1%, MyeloMyelo 6%, 6%, PromyeloPromyelo 4%4%HbHb: 18.5 g/dl, : 18.5 g/dl, HctHct 50.3 %, PL 423000/ul50.3 %, PL 423000/ul

Lab ( II )Lab ( II )

BUN 13 mg/dl, Cr 0.7 mg/dlBUN 13 mg/dl, Cr 0.7 mg/dlGPT GPT 138138 IU/L, DBI 0.4 mg/dl, TBI 1.3 mg/dlIU/L, DBI 0.4 mg/dl, TBI 1.3 mg/dlGluGlu 211211 mg/dl, TCH 179 mg/dl, TG137 mg/dlmg/dl, TCH 179 mg/dl, TG137 mg/dlK K 3.23.2mmol/L, Na mmol/L, Na 128128 mmolmmol/L/LLipase 29 IU/L, UA 6.4 mg/dlLipase 29 IU/L, UA 6.4 mg/dlTP 7.5 g/dl, ALB 4.1 g/dl, GLO 3.4 g/dl TP 7.5 g/dl, ALB 4.1 g/dl, GLO 3.4 g/dl CortisolCortisol 6.6 6.6 ugug/dl (8AM), 7.3 /dl (8AM), 7.3 ugug/dl (4PM)/dl (4PM)

Lab (III): urine catecholamineLab (III): urine catecholamine

Epinephrine: Epinephrine: 436436 ug/24 hrs ( 0~22.4 )ug/24 hrs ( 0~22.4 )NorepinephrineNorepinephrine: : 1236912369 ugug ( 11~85 )( 11~85 )Dopamine: Dopamine: 86738673 ugug ( 50~450 )( 50~450 )VMA ( VMA ( vanillylmandelicvanillylmandelic acid):acid): 116116 ugug(1~7.5)(1~7.5)

EKGEKG

Other ExamOther Exam

CXR, KUB, abdominal CT (12/30): to be CXR, KUB, abdominal CT (12/30): to be presentedpresented

DiagnosisDiagnosis

1. Hypertensive crisis1. Hypertensive crisis2. Retroperitoneal mass, favor 2. Retroperitoneal mass, favor pheochromocytomapheochromocytoma3. 3. LeukocytosisLeukocytosis with severe left shiftwith severe left shift4. 4. HyponatremiaHyponatremia, , hypokalemiahypokalemia

Hospitalization course and Hospitalization course and treatmenttreatment

After admission, After admission, nipridenipride, , phenoxybenzaminephenoxybenzamine, , inderalinderal, , amlodipineamlodipine, , captoprilcaptopril, , isormolisormol were added for BP were added for BP control. Slow K was given for control. Slow K was given for hypokalemiahypokalemia. . NiprideNipride was stopped 3 days later. After was stopped 3 days later. After BP became stable, patient was transferred BP became stable, patient was transferred to GS department for surgery.to GS department for surgery.

DiscussionDiscussion

1. 1. Hypertension, secondary hypertension, Hypertension, secondary hypertension, hypertensive crisishypertensive crisis2. 2. PheochromocytomaPheochromocytoma

何謂血壓何謂血壓??

血壓血壓是血流衝擊血管壁引起的一種壓力是血流衝擊血管壁引起的一種壓力

心臟收縮時心臟收縮時, , 所測得血管壁所受的壓力稱為所測得血管壁所受的壓力稱為收縮壓收縮壓

心臟舒張時心臟舒張時, , 所測得血管壁所受的壓力稱為所測得血管壁所受的壓力稱為舒張壓舒張壓

何謂高血壓何謂高血壓??

高血壓就是收縮壓高血壓就是收縮壓 超過超過 140 140 mmHgmmHg, , 或舒張壓或舒張壓超過超過 90 90 mmHgmmHg

原因原因

1. 1. 原發性高血壓原發性高血壓 (Primary hypertension): (Primary hypertension): 原因不明原因不明, , 可能與遺傳可能與遺傳, , 環境環境, , 情緒情緒, , 鹽分有鹽分有關關

2. 2. 續發性高血壓續發性高血壓 (Secondary hypertension): (Secondary hypertension): 因其他疾病引起血壓升高因其他疾病引起血壓升高, , 如腎臟病如腎臟病, , 內分內分泌系統毛病泌系統毛病

續發性高血壓續發性高血壓

嗜鉻細胞瘤嗜鉻細胞瘤 ( ( PheochromocytomaPheochromocytoma))庫氏症候群庫氏症候群 ((CushingCushing’’s syndrome)s syndrome)發性高醛固酮血症發性高醛固酮血症( ( Primary Primary hyperaldosteronismhyperaldosteronism))腎動脈狹窄腎動脈狹窄 ( ( Renal artery Renal artery stenosisstenosis))腎實質疾病腎實質疾病 ( ( Renal Renal parenchymalparenchymal disease)disease)主動脈窄縮主動脈窄縮 ( ( CoarctationCoarctation of the aorta)of the aorta)其它其它

何時懷疑病人有續發性高血壓？何時懷疑病人有續發性高血壓？

1. Severe hypertension1. Severe hypertension2. age < 30 2. age < 30 y/oy/o or > 55 or > 55 y/oy/o3. poor response to antihypertensive 3. poor response to antihypertensive therapy therapy 4. S/S of a secondary cause4. S/S of a secondary cause

Hypertensive crisisHypertensive crisis

: Definition: A substantial acute increase in : Definition: A substantial acute increase in BP, usually a BP, usually a DBP > 130 mmHgDBP > 130 mmHg, , associated some associated some symptomssymptoms or or acute or acute or ongoing ongoing target organ damagetarget organ damage..

Hypertensive crisisHypertensive crisisClassification: Classification:

( I ) ( I ) Hypertensive urgency Hypertensive urgency ( ( 高血壓緊急高血壓緊急 ):): elevate BP with elevate BP with associated symptoms but no acute or ongoing target organ damage,associated symptoms but no acute or ongoing target organ damage,可用急速口服降壓藥在一天內將血壓降至安全範圍可用急速口服降壓藥在一天內將血壓降至安全範圍

shortshort--acting acting nifedipinenifedipine, , captoprilcaptopril and and clonidineclonidine. .

( II ) ( II ) Hypertensive emergencyHypertensive emergency ( ( 高血壓急症高血壓急症 )): elevated BP with : elevated BP with acute or ongoing target organ damage,acute or ongoing target organ damage,須在一小時內將血壓降至安全須在一小時內將血壓降至安全範圍範圍. . CommmonCommmon manifestations aremanifestations areRetinal : hemorrhage, Retinal : hemorrhage, exudateexudate, , papilledemapapilledemaCardiac : pulmonary edema, myocardial ischemia, myocardial Cardiac : pulmonary edema, myocardial ischemia, myocardial infarctioninfarctionCNS: mental status changes, seizure, comaCNS: mental status changes, seizure, coma

PheochromocytomaPheochromocytoma

1. Definition1. Definition2. Characteristics2. Characteristics3. Clinical symptoms3. Clinical symptoms4. Diagnosis4. Diagnosis5. Location5. Location6. Treatment6. Treatment7. Prognosis7. Prognosis

DefinitionDefinition

CateolamineCateolamine ––producing tumorsproducing tumors that that originate from originate from chromaffinchromaffin cellscells of the of the adrenergic system. They generally secrete adrenergic system. They generally secrete both both norepinephrinenorepinephrine and epinephrine, but and epinephrine, but norepinephrinenorepinephrine is usually the predominant.is usually the predominant.

CharacteristicsCharacteristics

1. 1. ““Rough rule of 10Rough rule of 10””=> 10% are => 10% are extraadrenalextraadrenal, 10% involve , 10% involve

both adrenals, 10% are malignant, 10% are both adrenals, 10% are malignant, 10% are familial, 10% occur in children, 10% are multiplefamilial, 10% occur in children, 10% are multiple2. Clinical presentation: 2. Clinical presentation: ““ Five HFive H’’ss””

=> Headache, Hypertension, => Headache, Hypertension, HyperhidrosisHyperhidrosis, , Hyperglycemia, Hyperglycemia, HypermetabolismHypermetabolism

SymptomsSymptoms

HypertensionHypertension: sustained : sustained (55%)/paroxysmal(45%)(55%)/paroxysmal(45%)HeadacheHeadache(80%): paroxysmal in nature(80%): paroxysmal in naturePalpitationPalpitation (70%): with or without (70%): with or without tachycardiatachycardiaHyperhidrosisHyperhidrosis (60%)(60%)

DiagnosisDiagnosis

Obtain family historyObtain family historyPhysical exam: normal or high BP, profuse Physical exam: normal or high BP, profuse sweating, visual disturbance ( retinopathy), sweating, visual disturbance ( retinopathy), dilated pupils, dilated pupils, paresthesiaparesthesia in the lower in the lower extremities ( severe vasoconstriction), extremities ( severe vasoconstriction), tremor, tachycardiatremor, tachycardiaLab: Lab: 24 hrs urine VMA24 hrs urine VMA, , norepinephrinenorepinephrine, , epinephrine, dopamineepinephrine, dopamineClonidineClonidine suppression test suppression test

LocationLocation

Chest X ray, abdominal echoChest X ray, abdominal echoAbdominal CT, MRIAbdominal CT, MRI125125II--MIBG: this MIBG: this norepinephrinenorepinephrine analog analog localizes in adrenergic tissue; it is localizes in adrenergic tissue; it is particular useful in localizing particular useful in localizing extraadrenalextraadrenalpheochromocytomapheochromocytomaSelective vena cava sampling for Selective vena cava sampling for norepinephrinenorepinephrine levellevel

Treatment: SurgeryTreatment: Surgery

Preoperative stabilizationPreoperative stabilizationA. volume expansion: to prevent A. volume expansion: to prevent postoperative hypotensionpostoperative hypotensionB. Control BP: B. Control BP: nipridenipride, , ααblockerblocker: : phenoxybenzaminphenoxybenzamin 10 mg bid to 10 mg bid to qidqid, , ββblockerblocker ( used only after ( used only after ααblocker)blocker)

PrognosisPrognosis

After surgery, 5 year survival rate > 95%, After surgery, 5 year survival rate > 95%, recurrence rate < 10%recurrence rate < 10%Malignant Malignant pheochromocytomapheochromocytoma 5 year survival 5 year survival rate < 50%rate < 50%Catecholamine level return to normal 1 week Catecholamine level return to normal 1 week laterlater術後應追蹤數年術後應追蹤數年,,以確保沒有復發的情形發生以確保沒有復發的情形發生

Thanks for your attention !Thanks for your attention !

Case ConferenceChief complaintPresent illness ( I )Present illness ( II )Past historyFamily historyPhysical examination at ER ( I )Physical examination ( II )Lab ( I )Lab ( II )Lab (III): urine catecholamineEKGOther ExamDiagnosisHospitalization course and treatmentDiscussion何謂血壓?何謂高血壓?原因續發性高血壓何時懷疑病人有續發性高血壓？Hypertensive crisisHypertensive crisisPheochromocytomaDefinitionCharacteristicsSymptomsDiagnosisLocationTreatment: SurgeryPrognosisThanks for your attention !