The Curious Case of John Dick Group 3 Clinical Clerk Batch 2012 SY

Objectives  To discuss an intriguing case of an elderly woman with abdominal pain  To elaborate on the approach to jaundice  To discuss the diagnostic approaches to jaundice  To present the management of obstructive jaundice and review therapeutic options

Identifying Data  L.S.  64-year-old  Widow  Vegetable vendor  Tondo, Manila

Chief Complaint Generalized jaundice of 1 month duration

6 mos PTA 4 wks PTA 2 wks PTA 4 days PTA 1 wk PTAAdmission Colicky Abdominal Pain Temporal Profile Weight loss Jaundice Tea-colored urine Loss of appetite

 Past Medical History :  Osteoarthritis, right ankle – took unrecalled medication for 1 month  Exposure to Tuberculosis  G 4 P 4 (4004) via NSD without complications  No history of cancer  No history of heart failure or valvular defects  No history of Hepatitis B or C  No hemolytic disorders  No dyslipidemia  No history of blood transfusion  No history of needle prick injury  No history of prolonged or high-dose intake of drugs (e.g. Quinacrine, Rifampicin, etc)  No previous hospitalization, surgery, dental surgery

 Family History  Tuberculosis – Mother  No history of Cancer  No history of hemolytic disorders  Social History :  Non-smoker, non-alcoholic beverage drinker  No IV illicit drug use

Review of Systems  Weight loss (~50 kg  ~36 kg in 1 month)  No weakness  No persistent cough, night sweats, hemoptysis, fever  No edema, difficulty of breathing, orthopnea  No breast lump, pain or discharge  No abnormal vaginal bleeding  No history of abdominal trauma, changes in bowel movement, nausea and vomiting, fatty food intolerance

Physical Examination GeneralAwake, conscious, coherent, not in pain, appears ill-looking Vital Signs BP 90/50 mmHg HR 64 bpm Ht 154 cm RR 18 cpm T C Wt 36 kg BMI 15.1 kg/m 2 HEENT Icteric sclerae, yellowish palpebral conjunctivae, yellowish oral mucosa, no tonsillopharyngeal congestion, no cervical lymphadenopathies ChestEqual chest expansion, no retractions, clear breath sounds, No spider angioma CVSAdynamic precordium, normal rate, regular rhythm, distinct S1 and S2, no murmurs, concordant apex beat and PMI at 5 th ICS LMCL

Physical Examination Abdomen Globular, No caput medusae, No bulging flanks, Abdominal girth = 29 inches Normoactive bowel sounds, Tympanitic, Soft, Positive direct tenderness over epigastric area, No palpable masses, Liver span = 9cm, Spleen not palpable, No fluid wave, No shifting dullness, Negative Murphy’s sign

Physical Examination ExtremitiesFull and equal pulses, no edema, no cyanosis, Generalized jaundice Mental Status Exam Oriented to person, place and time. Remote, recent past, immediate memory not impaired. Cranial NervesIntact Motor, Sensory, Cerebellar Intact

Pertinent Findings PositiveNegative Weight lossDrug or alcohol use Abdominal enlargementBlood transfusion or donation JaundiceTattoos or IV illicit drugs Tea-colored urineHistory of Hepatitis AnorexiaFamily history of Hemolytic disordes Changes in bowel movement Nausea and vomiting Fever Fatty food intolerance History of abdominal trauma

Pertinent Findings PositiveNegative Icteric scleraeFluid wave, shifting dullness, bulging flanks JaundiceSpider angioma, caput medusae Globular abdomen, softHepatomegaly Splenomegaly Murphy’s sign

Assessment  Primary Impression  Obstructive Jaundice secondary to Pancreatic Head Mass  Differential Diagnoses:  TB Lymphadenitis  Peribiliary cancer  Choledocholithiasis

JAUNDICECAROTENEMIA DRUG INTAKE OF PROBENECID/ RIFAMPICIN HYPERBILIRUBINEMIA EXCESSIVE PRODUCTION (Hemolytic Anemia) IMPAIRED CLEARANCE UPTAKE/CONJUGATIONEXCRETIONHEPATICPOST-HEPATIC

Jaundice Carotenemia DRUG INTAKE PROBENECID/ RIFAMPICIN HYPERBILIRUBINEMIA Excessive intake of carotene containing food such as carrots, leafy vegetables, squash, peaches, and oranges Yellowish discoloration concentrated on palms, soles, forehead & nasolabial folds

Jaundice Carotenemia DRUG INTAKE PROBENECID/ RIFAMPICIN HYPERBILIRUBINEMIA Excessive intake of carotene containing food such as carrots, leafy vegetables, squash, peaches, and oranges Yellowish discoloration concentrated on palms, soles, forehead & nasolabial folds

Jaundice Carotenemia DRUG INTAKE PROBENECID/ RIFAMPICIN HYPERBILIRUBINEMIA

Jaundice Carotenemia DRUG INTAKE PROBENECID/ RIFAMPICIN HYPERBILIRUBINEMIA

Jaundice Carotenemia DRUG INTAKE PROBENECID/ RIFAMPICIN HYPERBILIRUBINEMIA

Jaundice Carotenemia DRUG INTAKE PROBENECID/ RIFAMPICIN HYPERBILIRUBINEMIA (+) Jaundice (+) Tea-colored urine (+) yellow discoloration of the skin (+) Icteric sclerae (-) Murphy’s sign (-) fluid wave, bulging flanks and shifting dullness (-) spider angioma and caput medusae (-) Hepatomegaly (liver span = 9 cm) (-) splenomegaly

HYPERBILIRUBINEMIA EXCESSIVE PRODUCTION (Hemolytic Anemia) IMPAIRED CLEARANCE Ssx of anemia (pallor, fatigue, weakness, dizziness, confusion, shortness of breath, and potential for heart failure) Usually normal colored urine and stool If inherited symptoms should have been present at an earlier age jaundice, splenomegaly, hepatomegaly, tachycardia, murmur

HYPERBILIRUBINEMIA EXCESSIVE PRODUCTION (Hemolytic Anemia) IMPAIRED CLEARANCE

UPTAKE/ CONJUGATION EXCRETIONHEPATICPOST-HEPATIC

IMPAIRED CLEARANCE UPTAKE/ CONJUGATION EXCRETIONHEPATICPOST-HEPATIC Crigler-Najjar syndromes – complete/incomplete absence of UDPGT activity Gilbert’s syndrome – reduced bilirubin UDPGT activity Manifestations of disorders in conjugation should appear earlier

IMPAIRED CLEARANCE UPTAKE/ CONJUGATION EXCRETIONHEPATICPOST-HEPATIC

(-) spider angioma and caput medusae (-) fluid wave, bulging flanks and shifting dullness (-) Hepatomegaly (liver span = 9 cm) (-) splenomegaly

IMPAIRED CLEARANCE UPTAKE/ CONJUGATION EXCRETIONHEPATICPOST-HEPATIC

GallbladderBiliary TreePancreasIntestine

Primary Impression Obstructive jaundice secondary to Pancreatic head mass r/o pancreatic ductal adenocarcinoma

Incidence rate 37,700 cases in the US, leading to 34,300 deaths. No predilection between genders Incidence is more common within the elderly population No established early warning symptoms Overall 5-year survival rate, <5% Pancreatic Adenocarcinoma

Causes are still unknown although it is considered that environmental causes play a role: Cigarette smoking Obesity Chronic pancreatitis History of diabetes mellitus Diet (increased intake of red meat or dairy products) Pancreatic Adenocarcinoma

Said to arise from a series of gene mutations Early on its onset, the mass would originate within the area of the ductal epithelium and would gradually spread to adjacent areas. Pancreatic intraepithelial neoplasia  invasive carcinoma Activation of the KRAS2 oncogene and inactivation of the tumour suppressor genes CDKN2A and TP53 Pancreatic Adenocarcinoma

Presentation of the symptoms would greatly depend on the area where the tumour is located. In 80% of cases, the tumour would be located within the area of the pancreatic head and this would have a great likelihood to cause obstructive cholestasis. Abdominal pain or discomfort as well as nausea are common clinical presentations. Diagnosis and staging

Systemic signs would include weakness, weight loss as well as anorexia. Physical examination: Signs of jaundice Wasting Hepatomegaly Ascites Routine laboratory tests might reveal anemia, abnormal liver function tests and hyperglycemia. Pancreatic Adenocarcinoma

Common complaints would include abdominal pain with the possibility of radiating to the back. Weight loss Splenomegaly, varices in the stomach and esophagus, GI bleeding DM symptoms, glucose intolerance