SummaryA study of 27 patients with cystic fibrosis of the pancreas who lived to be more than 10 years of age presents a wide range of clinicd states. Four of these children on whom observations were made after they had reached the age of 10 years have died at various ages up to nearly 20 years, all with the picture of progressive purulent bronchopneumonia. One of the fatal cases, coming to autopsy after a life span of 13½ years, showed cardiac changes consistent with cor pulmonale.Among the 23 living patients all have cough and clubbing of the fingers as continuing evidence of broncho‐pulmonary infection. The healthiest show no other signs of impaired respiratory function, either by roentgenogram or following a work tolerance test; likewise, some of these healthier patients have attained a body weight and height which do not differ significantly from mean values for the appropriate age and sex. For the group as a whole, however, a trend toward significant retardation of growth in height and of gain in weight is evident, the degree of retardation being roughly correlated with the severity of the pulmonary infection. Pubertal changes may be delayed. A number of the most seriously involved patients are in effect respiratory invalids, having a markedly reduced exercise tolerance. Regardless of the severity of their somatic functional status, however, almost all of them sustain a cheerful outlook for the future and build ambitious plans in the manner of all normal children.Although their nutritional state depends primarily on the severity and activity of their broncho‐pulmonary infection, as a group these children respond fairly uniformly to changes in diet. Almost without exception they prefer to avoid fat foods, having learned that ingestion of appreciable quantities of fat is followed by abdominal pain or by a change for the worse in the number and character of their stools. Many, having taken pancreatin for several years, now appear to do equally well without it.It would appear that adequate compensation for lost pancreatic digestive function in cystic fibrosis is quite readily attainable, but that the bronchopulmonary infection, once entrenched, is difficult to eradicate. Perhaps the best hope for the future lies in early recognition of the pancreatic lesion and in the institution of an effective prophylaxis of the infection.
I. The plan is described of a prospective study of the outcome of 5,964 pregnancies, with special emphasis on the relationship of factors in fetal environment to the incidence of abortions, stillbirths, neonatal mortality and congenital malformations. II. Data are presented on the overall incidence of congenital malformations according to sex, race, weight at delivery, maternal age and order of birth. A. The incidence of congenital malformations among 5,739 products of conception weighing over 500 grams was 7.5%. The rate was 7.0% among infants born alive and surviving the neonatal period, 13.6% among antepartum deaths, 23.3% among intrapartum deaths, 29.6% among neonatal deaths and 70.6% among deaths occurring between the ages of one and 12 months. B. The following relationships in incidence rates among live-born infants surviving the neonatal period were found. 1. The rate among males (8.4%) was half again as high as among females (5.5%). 2. Non-white infants had a higher rate (7.8%) than white infants (6.3%). 3. Infants weighing 2,500 grams or less had a higher proportion with defects (9.7%) than did those weighing over 2,500 grams (6.7%), although this difference occurred entirely among females. 4. Maternal age had no effect on the incidence of congenital malformations. 5. White infants of lower birth order had a lower rate (5.3%) than did those of higher birth orders (8.2%). No difference according to birth order was observed among non-white infants. III. A system of classification of malformations is described and discussed. Incidence rates by organ system groups are presented. A. Less than one-half of the malformations found among live-born infants were suspected or noted at birth. B. Malformations of musculo-skeletal system and skin were more frequent than malformations of other systems. Incidence rates by organ system groups are directly related to problems of diagnosis. C. Of the malformed live-born infants, 14.8% had more than one malformation and in 9.1% more than one system was involved. Of the malformed stillbirths and neonatal deaths, 66.0% had more than one malformation and in 51.1% more than one system was involved.
called attention to the changes occurring from one year to another in the clinical manifestations of the rheumatic state. More striking, even, are the modifications through which the clinical concept of the rheumatic state has passed in recent years ; and it has seemed to us of some interest to review the records of our youngest group of rheumatic patients, in the hope of obtaining a picture of the clinical and anatomic manifestations of the disease at an early age. We had the impression that rheumatic fever expresses itself in a subject under 3 years of age in a manner different from that by which one is accustomed to recognize it in older children. At the same time, its infrequency in early life throws a formidable obstacle in the path of the individual observer who attempts to formulate from his own experience a not too one-sided picture of the disease. We have selected from the records of the Babies Hospital, covering a period of about twentyfive years, a group of 24 cases, in all of which the disease appears to have begun before the age of 3 years and in which the evidence warrants, in our judgment, the diagnosis of rheumatic infection.Rheumatic infection is predominantly a disease of childhood. Analy¬ sis of the records of large groups of cases has brought out the fact that the greatest number of first attacks occurs in children between the ages of 5 and 15 years. Poynton2 found the average age of onset to be 7 years ; Coombs,3 10.2 years ;
The value, both academic and practical, of accurate knowledge regarding the mechanism of mammary secretion requires no emphasis. Our relative ignorance of what is actually going on in the mammary gland during its activity has been justly lamented from time to time by several writers. Attempts have recently been made to remedy this lack of knowledge, particularly in regard to the nature of the precursors, in the blood as it reaches the gland, of the characteristic milk constituents. It is with a major point in the technique of conducting experiments on bovine blood constituents that this paper is mainly concerned. An apparently direct method of obtaining information on the question of milk precursors is to make an accurate comparison between the composition of blood entering and that of blood leaving the udder of an actively lactating animal, the samples being taken approximately simultaneously. With due care, it is now possible to obtain useful information by this method. It does not, however, necessarily follow that a change in the amount of any given organic constituent of the blood during its passage through the mammary gland is due to such a substance being withdrawn to form a milk constituent. Part at least may be used up—in fact, some organic material borne by the blood must be used up—to provide the energy required for maintenance and secretory activity. The metabolic activity of the gland is evidenced by the marked change in the colour of the blood of the mammary vein as compared with the arterial blood, despite the speed ( see following paper) with which blood flows through the gland.
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