So-called Meulengracht’s disease (or Gilbert’s syndrome) is a not uncommon disorder or, more precisely, anomaly, named after the Danish internist Jens Einar Meulengracht (b. April 7, 1887, † 1976). Approximately 5 percent of the population is affected, although symptomatology can vary widely.
Cause is dysfunction of the liver
The cause of Meulengracht’s disease is found in the process of hemoglobin breakdown and consists of a dysfunction of the liver, which at times cannot completely or sufficiently break down the red blood pigment. Blood cells are normally recycled after approximately 120 days. The red blood pigment, hemoglobin, is broken down in several steps in the bone marrow, spleen and liver and converted into a water-soluble form.
In Meulengracht patients, however, the uptake and processing of a degradation product necessary for this, unconjugated bilirubin, is disturbed. As a result, elevated concentrations of the yellow blood pigment (= bilirubin) are found in the blood at times.
Symptoms of Meulengracht disease
Symptoms include yellow-colored skin and eyes. Patients may experience episodes of abdominal cramps and indigestion, especially between the ages of 15 and 40. This mainly affects thin patients after alcohol or nicotine consumption (including passive smoking). Starvation and fasting in particular also trigger symptoms and should be avoided at all costs, which is in contrast to other digestive disorders. Occasionally, flatulence, nonspecific abdominal pain, and skin rash are also reported as consequences of the disease.
There is also often intolerance to alcohol, nicotine, hunger and stress. After alcohol or nicotine consumption, there is an increase in bilirubin levels, which is associated with increased yellowing of the eyes and skin. Fatigue, malaise, nausea, and sometimes vomiting may result. Bile salt levels and other liver values are usually normal, resulting in no skin itching as in other conditions associated with yellowing of the skin.
Disease not curable
Meulengracht’s disease can be clearly diagnosed by nicotinic acid or fasting tests. Molecular genetic testing is another diagnostic option. Meulengracht disease is not curable. Although treatment of some symptoms is possible, it is usually refrained from, because the health impairment is too small and would not justify the side effects.
The name suffix “juvenilis” refers to the fact that people are only affected by the disease when they are young. Approximately after the age of 40, it grows.
