Winckel's Disease

WINCKEL'S DISEASE (Winckel) is of chiefly historical interest. It is observed in the first days of life and is characterized by sharply pronounced jaundice, cyanosis, and hemoglobinuria. Winckel described it in 1879 under the name «cyanosis afebrilis perniciosa icterica cum haemoglobinuria»; however, it had been described repeatedly even before him (Pollak—1871, Laroyenne, Parrot—1873, Bigelow—1875). The etiology and pathogenesis of Winckel's disease are unclear, but at the present time investigators are inclined to classify it, much like Buhl's disease, with which it shares many similarities, among the varieties of sepsis. Winckel thought of poisoning or intoxication; for a time, the cause was sought in tap water, in the contamination of amniotic waters, or in wiping the child's throat. Pollak, Lesage, and Demelin placed Winckel's disease in an etiological connection with intestinal catarrh. Among microbes, streptococcus, micrococcus, and Bact. coli have been found; bacteremia has been observed repeatedly. It is likely that this symptom complex may occur in various kinds of infections and is almost never observed in the present aseptic era. Autopsy reveals cyanosis and jaundice of the internal organs, a large black-red spleen, small hemorrhages everywhere, especially in the kidneys, hemoglobin infarcts in the pyramids, and fatty degeneration mainly of the liver and heart. Diseases of the umbilical vessels are rare. The disease usually begins on the 4th day in well-nourished children with restlessness and cyanosis; jaundice increases («maladie bronzée» of Bar), the urine acquires a dark color and contains hemoglobin, red blood cells, epithelial cells, granular casts, and so forth. Bar asserts that it is a matter of hematuria, that is, the presence of red blood cells in the urine. Dyspnea and catarrh of the gastrointestinal tract are observed. The temperature is low; death occurs with symptoms of collapse. The disease often assumes an epidemic character (Winckel—23 cases, Bigelow—10 cases). The diagnosis is based chiefly on the character of the urine, cyanosis, and the sharp bronze coloration of the integument. The prognosis is grave, as recoveries are rare. Prophylaxis consists in strict asepsis not only of labor, but also of the pre- and postnatal periods. Strict isolation of the affected is necessary. Treatment consists in complete rest, support of cardiac activity and bodily strength, the use of hemostatics, and transfusions of maternal blood to the sick child.