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Favism
606
THE
JOURNAL
OF
PEDIATRICS
FAVISM L T H O U G H the lava bean is grown quite extensively in a number of parts of this c o u n t r y where there is a large Italian population, favism is an uneolmnon disease in the United States and not more than a dozen eases have been reported. These have been of a critical type with m a r k e d hemoglobinuria, as a rule. Favism, however, exists in moderate and mild forms, and it has been suggested that some of the eases of hemolytic anemias in children of Mediterranean birth or descent diagnosed as some other type of disease m a y in reality have been eases of favism. ~ A recent extensive report ~ on favism made to the IV I n t e r n a t i o n a l Mediterranean Congress on Hygiene by Prof. Surinyach of Barcelona, Prof. Mareolongo of C a g l i a r i , and Prof. Aleobe and Dr. hlcbaria of Bareelona contains considerable information in r e g a r d to the disease and the differences of opinion as to the pathogenesis. Like Cooley's anemia, it is distinctly a Mediterranean disease, and has loci of distribution. Sardinia is the chief center with thousands of eases a year. Other loci are found in Greece, Spain, and probably i t a l y and Portugal. Sporadic eases are common in these countries and in Turkey and Malta. According' to the report, no eases have been rep o r t e d f r o m Corsica, E g y p t , or Syria. The few eases r e p o r t e d in the United States and other countries were in immigrants from the Mediterranean area or their descendants. Thus there
A
is seemingly a constitutional or genetic f a c t o r involved in some way. M a n y consider the disease to be aliergic in character. On the other hand, some question this, and Suriny a e h points out that the disease is distinctly one of blood destruction without other allergic manifestations, and stresses that other foods will produce w h a t he terms the " a l i m e n t a r y hemolytic s y n d r o m e . " Marcolongo has f o u n d eertain toxic substances in the l a v a bean which act on the red blood cells. Surinyach holds to a dualistic t h e o r y as regards the etio l o g y - - a direet action of toxic substances in the fava bean on the red blood cells in a susceptible individual. Clinically we think of favism as an acute condition which m a y be fatal in children. It follows within a few hours after the ingestion of the bean, partieular]y the raw bean, with nausea, vomiting, and collapse, and characteristically is associated with he'moglobinuria and jaundice. How~ ever, mild a m b u l a t o r y forms occur, according to Surinyaeh, with h a r d l y visible hemoglobinuria and jaundice. It is in r e g a r d to this last group that confusion m a y exist in the diagnosis of the cause of anemia in children of Mediterranean descent. REFERENCES 1. Diggle, J. H.: Arch. Dis. Childhood 28: 369j 1953. 2. Surinyaeh, R., Mareolongo~ F.~ Aleobe, S., and Lbbaria, C. A.: Fabismo y tIemol isis Alimentaria, Barcelona, Spain~ 1953.
THE
JOURNAL
OF
PEDIATRICS
FAVISM L T H O U G H the lava bean is grown quite extensively in a number of parts of this c o u n t r y where there is a large Italian population, favism is an uneolmnon disease in the United States and not more than a dozen eases have been reported. These have been of a critical type with m a r k e d hemoglobinuria, as a rule. Favism, however, exists in moderate and mild forms, and it has been suggested that some of the eases of hemolytic anemias in children of Mediterranean birth or descent diagnosed as some other type of disease m a y in reality have been eases of favism. ~ A recent extensive report ~ on favism made to the IV I n t e r n a t i o n a l Mediterranean Congress on Hygiene by Prof. Surinyach of Barcelona, Prof. Mareolongo of C a g l i a r i , and Prof. Aleobe and Dr. hlcbaria of Bareelona contains considerable information in r e g a r d to the disease and the differences of opinion as to the pathogenesis. Like Cooley's anemia, it is distinctly a Mediterranean disease, and has loci of distribution. Sardinia is the chief center with thousands of eases a year. Other loci are found in Greece, Spain, and probably i t a l y and Portugal. Sporadic eases are common in these countries and in Turkey and Malta. According' to the report, no eases have been rep o r t e d f r o m Corsica, E g y p t , or Syria. The few eases r e p o r t e d in the United States and other countries were in immigrants from the Mediterranean area or their descendants. Thus there
A
is seemingly a constitutional or genetic f a c t o r involved in some way. M a n y consider the disease to be aliergic in character. On the other hand, some question this, and Suriny a e h points out that the disease is distinctly one of blood destruction without other allergic manifestations, and stresses that other foods will produce w h a t he terms the " a l i m e n t a r y hemolytic s y n d r o m e . " Marcolongo has f o u n d eertain toxic substances in the l a v a bean which act on the red blood cells. Surinyach holds to a dualistic t h e o r y as regards the etio l o g y - - a direet action of toxic substances in the fava bean on the red blood cells in a susceptible individual. Clinically we think of favism as an acute condition which m a y be fatal in children. It follows within a few hours after the ingestion of the bean, partieular]y the raw bean, with nausea, vomiting, and collapse, and characteristically is associated with he'moglobinuria and jaundice. How~ ever, mild a m b u l a t o r y forms occur, according to Surinyaeh, with h a r d l y visible hemoglobinuria and jaundice. It is in r e g a r d to this last group that confusion m a y exist in the diagnosis of the cause of anemia in children of Mediterranean descent. REFERENCES 1. Diggle, J. H.: Arch. Dis. Childhood 28: 369j 1953. 2. Surinyaeh, R., Mareolongo~ F.~ Aleobe, S., and Lbbaria, C. A.: Fabismo y tIemol isis Alimentaria, Barcelona, Spain~ 1953.