CPC #7: Wednesday, March 12, 2003
Hurd Hall, The Johns Hopkins Hospital
12:00 Noon

Chief Complaint

A 42 year-old white male presented with abdominal pain and mental status changes.

History of Present Illness

The patient was in his usual state of good health until 05/2000 when he presented to an outside hospital's Emergency Department for abdominal pain and distention. Initial evaluation identified the presence of ascites. In retrospect, the patient had reported gradual weight gain and abdominal distension over months without any pain. No further information from that visit is available.

On 08/16/2000, he returned to the outside hospital with increasing abdominal girth, abdominal pain and mental status changes. He subsequently became comatose and was transferred to the intensive case unit. Initial laboratory evaluation demonstrated serum ammonia of 436 µM/L. He responded to lactulose and was transferred to JHH on 08/25/2000 for further diagnostic and therapeutic evaluation.

He had no prior known history of liver disease and no history of viral hepatitis. Prior to 8/00, he had used alcohol in moderate amounts rarely, and none since the diagnosis of liver disease. There was no history of jaundice or itching. At JHH, the patient was managed with an ultrasound-guided paracentesis which yielded straw colored fluid without any evidence of infection. The patient's lactulose was continued while Lasix and aldactone were added to the regimen. He was evaluated as a candidate for liver transplantation with the usual battery of serologic and serum studies. A liver biopsy was not performed. He was discharged on 09/02/2000 with a normal mental status and mild ascites.

The patient returned as outpatient on 09/07/2000 and was treated with another paracentesis, removing 4.8 L of ascitic fluid. On 09/08/200, he developed mental status changes, and was readmitted to the outside hospital. He developed progressive confusion, became combative, and ultimately became obtunded and unresponsive. The patient was hypotensive and managed with fluid boluses totaling 3500 cc. His hypotension persisted and pressors (dopamine 10 mcg/min) were initiated. He subsequently developed respiratory failure requiring endotracheal intubation and mechanical ventilation. On 09/09/2000, he was transferred to JHH via helicopter.

Past Medical History

Unremarkable. No prior history of lung disease, GI bleeding, peptic ulcer disease, pancreatitis, cholelithiasis, hypercholesterolemia, diarrhea, bloody stools, or arthritis. No history of hematologic, autoimmune, malignant, psychiatric or infectious diseases.

Past Surgical History

Open reduction - internal fixation for leg fracture (1998)

Social History

Father of three children; Prior employment as a truck driver; Discontinued all alcohol intake which was previously rare; No tobacco or illicit drugs. No risk factors for HIV.

Family History

Notable for a strong family history of end stage liver disease. His sister died with cirrhosis at age 15, he has an uncle and a cousin with liver disease. He has no other siblings. His parents have mild hypertension but no gastrointestinal diseases.

Allergies: NKDA

Medications

• Lactulose 20 gm po qac
• Pyridoxine 50 mg po qd
• Spironolactone 200 mg po bid
• Lasix 80 mg po qd

Physical Exam on Admission

• Vital signs: T 37.6, Tm 39.3, P 100, R 32 (agonal), BP 75/45
• General: jaundiced, unresponsive, intermittent tonic-clonic movements
• HEENT: scleral icterus, pupils 8mm, minimally reactive
• Lungs: coarse breath sounds bilaterally
• Heart: RRR with distant heart sounds, no murmurs, rubs, or gallops
• Abdomen: distended with ascites, no palpable liver and spleen
• Extremities: 1+ pulses (radial, brachial, femoral, dorsalis pedis)
• Neurological: unresponsive to noxious stimuli, intermittent tonic-clonic movements

Laboratory values on transfer

Na 135 mEq/L, K 8.2 mEq/L, Cl 97 mEq/L, HCO3 10 m Eq/L, BUN 68 mg/dl, Cr 4.2 mg/dl
Glu 256 mg/dl, iCa 0.89 mg/dl (1.12-1.32), Mg 2.4 mEq/L

Arterial Blood Gas

pH 7.29, PaCO2 18 mm Hg, HCO3 16 mEq/L
Anion gap 34 mEQ/L, lactate 17.6 mg/dl

Liver Function Tests

AST 668 IU/L, ALT 267 IU/L, BR total 10.6 mg/dl, NH4 464 ?M/L
Protein total 4.5, Albumin 1.9
PT 29.8 sec, PTT 92.3 sec, INR 5.8

CBC
WBC: 15.6 cells/mm3, HgB 10.6 g/dl, Hct 31.7%, platelets 149/mm3
MCV 109 fL, ANC 13,388 cells/mm3

Blood cultures: no growth

Radiology: CT imaging showed diffuse nodularity throughout the liver (See Figure 1). The nodules were of high attenuation on non-contrast CT and on arterial phase imaging.

Figure 1A

Figure 1B

Hospital Course

Upon transfer, the patient was hypotensive despite fluid resuscitation and pressor support started at the outside hospital. His hypotension persisted despite maximal doses of dopamine, norepinephrine, phenylephrine, epinephrine, and vasopressin. He had seizures that were refractory to benzodiazepine therapy but controlled with pentobarbital. While his initial blood cultures showed no growth, he was treated with broad-spectrum antibiotics and his blood was re-submitted for culture. He was anuric throughout the hospital course and was managed for acute hyperkalemia, including 30 mg Kayexalate enemas. Despite aggressive supportive therapy, his multisystem organ failure remained unresponsive to maximal therapy and liver transplantation was not an option. Because of his dismal prognosis and after multiple discussions with his family, supportive care was removed. He died on 09/10/2000.

What is the most likely cause of his ascites and liver dysfunction?

What are possible causes of his acute deterioration and death?

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