Acholuric jaundice: Description, Causes and Risk Factors:
Jaundice with excessive amounts of unconjugated bilirubin in the plasma and without bile pigments in the urine.
In man, acholuric jaundice was first accurately described by Minkowski at the beginning of this century. Little was understood of the disease, however, until Chauffard made the important observation that the red blood cells (RBCs) showed a markedly diminished resistance to hypotonic salt solution and also observed that reticulocytes were usually present in large numbers. Widal was the first to recognize the fundamental hemolytic nature of acholuric jaundice, i.e., that it was caused by an agency which destroyed large numbers of red blood cells.
The human form of this malady is sometimes divided into a hereditary and an acquired type, of which the former is more common. The first kind belongs to the inheritable diseases, occurring often in several generations, occasionally in several members of one family without the descendants having the condition, and also in a single member of a family who may be affected from birth. As a result of the precedence of the above findings, the hereditary form in man is often alluded to as the Minkowski-Chauffard type, and the acquired form as that of the Hayman-Widal syndrome.
Familial acholuric jaundice in man has usually been associated with metabolic derangements such as albinism, pentosuria, glycosuria, etc., of which Gates says, "In all such cases of inborn deranged metabolism the probable cause is the lack of an enzyme in the absence of which a chemical step is missed and some normal metabolic process fails to take place."
Campbell and Warner' have a record of acholuric jaundice in five generations of a family, in which two latent carriers were included. The latter, although normal in appearance upon examination were found to have an increased erythrocyte fragility and also to show slight indications of increased blood breakdown beyond that of normal individuals.
Infants may appear jaundiced and pale. Fatigue, weakness, and shortness of breath are other symptoms that may be considered in elderly patients. The spleen may be enlarged. Gallstones may present in the majority of cases.
The diagnosis rests on (1) the strong family history,(2) the occurrence of considerable blood destruction within a very short period, (3) the characteristic increase in the fragility of the child's erythrocytes and the spherocytosis, (4) the exclusion of other causes of hemolysis at this age. Acholuric jaundice is recognized to be due to an inherited defect of the erythron, which is demonstrated by the abnormal erythropoiesis seen in bone marrow smears, by the abnormal contour of the erythrocytes in the circulating blood, by the excessive fragility in saline solutions, and by their liability to rapid destruction in the body.
Treatment may include splenectomy (removal of the spleen). Once this is done the red blood cell life returns to normal. It is generally agreed that the administration of liver or iron, and the use of x rays or radium, has little or no effect on acholuric jaundice. The management of the individual case turns, therefore, on whether the spleen should be removed, and if so at what stage.
NOTE: The above information is for educational purpose. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition.
DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.
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