We recently defined functional asplenia in children with sickle-cell anemia as impaired splenic reticuloendothelial function despite clinical enlargement of the organ. In three children infusions of fresh plasma did not restore function, indicating that functional asplenia probably is not a consequence of a contracted plasma volume or deficient opsonins or other humoral factors. Transfusions of normal red cells were given, and in five young children, splenic function was restored. Seri al studies suggested that a level of normal red cells of about 50 per cent was necessary for function. The best explanation for functional asplenia is that the high viscosity of sickle-cell blood causes a diversion of splenic blood flow through intrasplenic shunts, thus bypassing the phagocytic reticuloendothelial elements of the organ. When sickled cells are replaced by normal red cells through transfusion, splenic circulation and function are temporarily restored.
The experienced clinician makes a judgment (hereafter called overall assessment [OA]) about the degree of illness of a febrile child prior to physical examination. In order to define the history and observation variables on which OA is based, 262 febrile children less than or equal to 24 months of age were evaluated simultaneously by multiple observers including attending pediatricians, practicing pediatricians, pediatric house officers, and nurses. The observer listed history and observation variables he/she thought most important in making an OA on a blank, lined form and then scored those variables and OA as normal, or mildly, moderately, or severely impaired. Scoring for observation rather than history variables was better correlated with scoring for OA and serious illness. The observation variables most frequently mentioned by all observers were the child's "looking at the observer" and "looking around the room." There were 20 observation variables frequently mentioned, the scoring of which significantly correlated with scoring for OA; four of these 20 variables related to eye function. The child's response to a stimulus was noted in 105/186 different observation variables listed; both the attending pediatrician and the house officer scored these stimulus-response variables significantly different in children with, vs those without, serious illnesses. For attending pediatricians, house officers, and nurses, serious illness was five to seven times as likely if an OA of moderate or severe impairment was made than if it were not made. OA is a key skill in evaluating febrile children; these data identify variables on which OA is based, document the importance of assessing eye function in young, febrile children, and demonstrate that eye function is one key type of stimulus-response behavior on which the pediatrician as clinician and developmentalist relies to make judgments about febrile children.
HUNTER-HURLER'S disease is an born error of metabolism characterized by excessive deposition of acid mucopolysaccharides (chondroitin sulfate B and heparitin sulfate) in the liver, spleen, skin, and other organs whereas the nerve cells accumulate an abnormal amount of ganglioside containing lipids.1,2The main clinical features consist of dwarfism, hepatosplenomegaly, bone and joint deformities, mental and physical deterioration, corneal clouding and abnormal and coarse facial features: bushy eyebrows, sunken and upturned nose, thick lips, and hirsutism. The onset is commonly between a few months and 5 years of age and these patients rarely survive past the age of adolescence. Dorfman and Lorincz3and subsequently many others demonstrated that the affected children excrete an excessive amount of acid mucopolysaccharide (AMPS) in their urine, while Danes and Bearn4and Matalon and Dorfman5showed that skin fibroblasts from these patients grown in tissue culture accumulate a large quantity of
Nephrogenic diabetes insipidus occurred in a 7-year-old child who had received a high dose of demethylchlortetracycline hydrochloride (DMC). The patient had a relatively elevated urinary sodium concentration in addition to isosthenuria. The nephrogenic diabetes insipidus was completely reversible within one month after cessation of DMC administration.
