The Case of the Jaundiced Musician

Nurses General Nursing

Published

I found this fun little case study on a medical journal site. I'll post the answers tomorrow.

The Case of the Jaundiced Musician

from Medscape Surgery

Posted 08/19/2003

Albert B. Lowenfels, MD

Introduction

Chief Complaint: A 57-year-old white male musician presents with a chief complaint of vomiting and swelling of the legs and abdomen. The patient is short (168 cm), stocky, pale, and deeply jaundiced. He is awake, responds to questions, but is drowsy. The skin is jaundiced with petechial hemorrhages over the abdomen, and there is marked scleral icterus. He has a hearing deficit at all frequencies. On auscultation of the chest there are some basal rales. The abdomen is without scars and there is massive ascites. After abdominal paracentesis, a firm irregular liver is palpated upon deep inspiration. His spleen is palpable below the left costal margin.

Past History: His illness dates back 4 months, characterized over the past few weeks by progressive weakness, increased swelling of the abdomen, marked swelling of the legs, recurrent febrile episodes (fever and chills), and gradually increasing jaundice. He had a long history of gastrointestinal complaints beginning at age 21 years, when he had an initial attack of abdominal pain accompanied by diarrhea, followed by similar attacks every few years. Four years prior to the present illness he experienced abdominal pain, vomiting, and jaundice; the jaundice persisted for a few months. Initially, alcohol alleviated the pain, but as the attacks became more frequent, alcohol exacerbated the symptoms.

At age 17, he had an attack of what appeared to be asthma. He had experienced frequent attacks of joint pain and "gout" and occasional bouts of diarrhea. At age 53, he experienced a painful ocular condition leading to diminished visual acuity. His mother died of tuberculosis when the patient was 16 years old; his father died of complications of alcoholism. He reported being a pipe smoker and consuming alcohol regularly, sometimes as much as a liter of wine per day.

Disease Course: During the 4-month interval from the onset of current symptoms until death, the patient had recurrent attacks of abdominal pain, vomiting, and diarrhea. He also had an attack of pneumonia. His jaundice persisted and he required paracentesis on 4 occasions. About a week before his death, he developed massive edema, fever, and diminished urinary output. About 48 hours before his death, he lapsed into a progressively deepening coma. An autopsy was performed on the day following the patient's death.

1. Who was our mystery patient?

A) Enrico Caruso

B) Wolfgang Amadeus Mozart

C) Kurt Cobain

D) Ludwig van Beethoven

2. Which of the following diseases is least likely to be present?

A) Pancreatitis

B) Cirrhosis of the liver

C) Stomach cancer

D) Inflammatory bowel disease

3. Which of the findings is least likely to be explained by heavy drinking?

A) Hearing deficit

B) Jaundice

C) Ascites

D) Coma

Ummmm, Beethoven, Stomach cancer, Hearing deficit.

Can't wait for the answers!

I think everybody had the correct musician.

"Our mystery patient is Ludwig van Beethoven (1770-1827), considered to be one of the world's outstanding composers (Figure 1). Even people with little musical training can hum the opening few bars of Beethoven's fifth symphony, and the voyager space probes, launched in 1977, carry Beethoven melodies on gold-plated phonograph records into outer space.

The end of the 18th century and the earliest part of the 19th century were not the healthiest times to be alive. Tuberculosis was rampant, claiming the lives of Beethoven's mother and one of his brothers. Beethoven survived childhood smallpox, another extremely common lethal infectious disease, and as an adult suffered from many baffling symptoms, the most distressing being progressive deafness."

Stomach Cancer was the LEAST likely of the diseases listed to be present.

"On the basis of the clinical history and the autopsy findings, several other diseases have been suggested, including sarcoidosis,[2] Paget's disease, primary hyperparathyroidism, syphilis, and even lead poisoning. However, liver failure, probably alcohol-induced, was the immediate cause of Beethoven's death. The patient had symptoms including jaundice for several years, compatible with liver disease, but highly unusual for gastric cancer. Furthermore, there was no evidence of bleeding into the digestive tract, which is common with gastric cancer."

And the answer to question 3 was hearing deficit. I think everybody got that one too. The following clinical commentary was also offered:

"Was an autopsy performed?

Yes. Beethoven was curious about his many diseases and concerned about the implications of his health problems for other family members. In a letter written to his brothers 25 years before his death, he requested that upon his death, his physicians should try to determine the cause of his death. The autopsy was performed in Vienna at Beethoven's home by Dr. Johann Wagner, the teacher of the much more famous pathologist Carl von Rokitansky.

What were the major findings at autopsy?

The body was emaciated, skin deeply jaundiced, abdomen was distended with ascitic fluid, auditory nerves were shriveled, and the calvarium was dense and about half an inch in thickness. The liver appeared shrunken to half its normal size; surface was nodular. Spleen was firm, double its normal size. Pancreas was firm and the excretory duct as wide as a goose quill. Kidney calices all contained irregular concretions about the size of a split pea. The diseased organs were examined grossly; histology became available only around the middle of the 19th century.

Is there a single disease that could explain all of Beethoven's symptoms?

Experienced clinicians always attempt to find a single diagnosis to explain the patient's findings. Although it seems impossible to attribute all of Beethoven's symptoms and signs to one cause, many of Beethoven's problems can be attributed to his drinking. Beethoven consumed moderate-to-large amounts of alcohol, often drinking a bottle of wine with meals. Complications relating to alcohol-induced liver failure could explain many of the findings such as the jaundice, ascites, shrunken liver, and splenomegaly.

The findings in the pancreas can also be explained by alcohol, the most common cause of chronic pancreatitis. In addition to alcohol, tobacco is a suspected risk factor for pancreatitis -- and Beethoven was a pipe smoker. Chronic alcoholic pancreatitis could explain the persistent diarrhea, and often leads to diabetes. The findings in the kidney are characteristic of renal papillary necrosis, which is commonly found in patients with diabetes.

Alcohol is still a major cause of liver cirrhosis. We have no information about the frequency of this disorder during Beethoven's lifetime or about levels of alcohol consumption, but at present, death rates from liver cirrhosis are high in Austria, and throughout Europe there is a strong correlation between alcohol consumption and liver cirrhosis.

What about Beethoven's hearing loss?

Beethoven's hearing deficit began when he was about 30 years old and became progressively worse, leading to complete deafness during the last 10 years of his life. Despite this devastating impairment, he continued to compose and was unable to hear his famous ninth symphony. He initially used an ear trumpet, but in later years when he suffered complete hearing loss he relied on a "conversation" book. Otosclerosis is the diagnosis favored by otolaryngologists, but auditory nerve damage could also have been present. He would have benefited from a modern hearing device, at least until his hearing loss became total.

What diagnostic tests would we use today to establish the correct diagnosis?

We would now perform a much more adequate physical examination than was possible during Beethoven's time. For example, it would have been impossible for Beethoven's physicians to measure his blood pressure because the sphygmomanometer had not been invented until the second half of the 19th century. A battery of liver function tests would clarify the type of liver disease present and a liver biopsy would establish the exact diagnosis. Endoscopic retrograde cholangiopancreatography would provide a great deal of information about the extent of pancreatic disease. Ultrasonography or endoscopic ultrasonography would also be useful.

What would an ERCP have shown?

An ERCP in a patient with chronic pancreatitis and a history of chronic alcoholism would likely have shown a markedly dilated pancreatic duct. Excessive alcohol ingestion promotes inspissation of duct secretions, which can produce focal obstructions, leading to inflammation. Over time, the pancreatic duct becomes dilated and may fill up with concretions.

What would be appropriate therapy today?

Advising a patient with alcoholic liver disease to become abstinent has always been the most effective strategy for improving prognosis. Beethoven's physician did recommend abstinence, but Beethoven disregarded the warnings about drinking, continuing to consume alcohol until shortly before his death. Nutritional supplements, and perhaps glucocorticosteroids, might help. In patients with ascites, diuretics are often useful, but after development of refractory ascites requiring paracentesis, survival is only about 1 year.

Would an operation have been helpful?

Abdominal paracentesis is still used for the treatment of ascites, but we now can relieve ascites either by shunting the peritoneal fluid back into the venous circulation or by creating a transjugular intrahepatic portosystemic shunt (aka the TIPS procedure).[5] However, side effects and complications arise with both of these procedures.

In selected patients, liver transplantation can be offered as effective treatment for alcoholic cirrhosis. To be eligible, patients must be abstinent for several months. Beethoven continued to drink even during his final illness, so he would not have been a candidate for liver transplantation."

Yikes! Sorry for such long posts. I would have posted a link to the page but you have to be a member to access the site so although its free, I thought that would be a PITA.

Specializes in ICU.

Thank-you Aimee wonderful excercise I enjoyed it very much despite putting my foot in my mouth and not reading quesiton 2 correctly!:chuckle

Thank you. It was interesting.

Since by second career choice would have been in music, I had a good time with this one! Thanks Amy.

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