1. There are fats which are indispensable for children because they contain specific bodies, essential for normal growth.2. If these lipoid bodies, termed “fat soluble A bodies” by McCollum, are absent from the food for a long time, an inhibition of growth will occur and the conditions which I have termed Dystrophia alipogenetica will finally appear. This condition involves a great susceptibility and lowered resistance to all infections and often leads to xerosis of the conjunctivae and corneae associated with night blindness. The xerosis has a great tendency to result in keratomalacia. In its first stage xerophthalmia resembles a slight conjunctivitis; the children develop photophobia, their eyes are red and there is a slight secretion.3. Xerophthalmia generally occurs in spring, the time when growth is at its maximum.4. The disease is most frequent amongst the children of the poorest country folk and is always due to an unsuitable artificial diet, generally the replacing of whole milk partly or completely by centrifuged milk, butter milk or concoctions of flour. It may appear in children who have received whole milk and cream, but in that case the milk and cream have been boiled for too long a time or have been subjected to other kinds of drastic treatment which have destroyed the specific lipoid bodies.5. Xerophthalmia is easily when recognised in time. The best treatment is cod liver oil, but whole milk and probably cream, butter, eggs and other fats containing the specific lipoid bodies are also curative. It is important to remember that these foods should only be subjected to the ordinary short boiling.6. The disease is fairly common in Denmark, apparently more so than in other countries. It is, for instance, practically unknown in Sweden and Norway. According to Mori's statistics it used to be rare in Germany. The reason for its being so frequent in Denmark is probably partly due to the fact that this country exports most of its dairy produce, especially butter.7. Many of the cases of blindness and leucoma attributed to eye complaints during infancy, are perhaps due to xerophthalmia. Of late it has been said that blindness amongst children in Denmark is increasing while gonorrhoeal ophthalmia in new born infants at the maternity hospital is decreasing. Ophthalmo-blenorrhoea has been the cause of blindness in very few of the recent admissions to the blind institute, all of which supports my contention that xerophthalmia is the actual cause of the large number of blind children in Denmark.
My description of the diseases occurring in children from a deficiency in the fat-soluble A factor was quickly acknowledged by those physiologists who were working at vitamin problems. This was due to the close parallel, which in many respects exists between the disease I have observed in children and the condition that can be produced in young rats, by keeping them for a long time on a diet poor in the f at \ x=req-\ soluble A factor but otherwise adequate. With clinicians it was a different matter. Although nearly a decade has passed since I published my first contributions on this subject, there are still many clinicians, especially in Germany, who have never appreciated the fact that the absence of the fat-soluble A factor gives rise to a well defined disease just as, for example, the absence of the C factor does. There are, however, many German authors who trace a connection between the deficient growth of the children and the great prevalence and serious course of tuberculosis and similar diseases during the war on the one hand, and the lack of the proper food substances (i. e., those containing the fat-soluble A factor) on the other, but that it is an indication of the absence of the fat-soluble A factor is usually not understood or not alluded to. With regard to the characteristic symptom, xerophthalmia, it is stated by many that this condition is not caused by a deficiency in the fat-soluble A factor alone but is merely the result of general marasmus.
This case report describes the simultaneous manifestation of acute necrotizing encephalopathy in 2 consanguineous patients after infection with influenza B based on the autosomal dominant missense mutation of the RANBP2-gene. Differential diagnosis of acute encephalopathy, clinical and radiological clues, and treatment strategies are outlined.
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