CONGENITAL AFIBRINOGENEMIAPRICHARD, ROBERT W.; VANN, ROBERT L.
1954 American journal of diseases of children
doi: 10.1001/archpedi.1954.02050100705001
THE COMPLETE congenital absence of fibrinogen is apparently one of the most unusual anomalies, since only 21 cases have been reported to date.* In this presentation the 22d patient is discussed and a brief review appended. Aside from their rarity, these patients present findings which throw light on other, commoner problems and are examples of conditions which are impossible to produce experimentally at present.
A 19-day-old white boy, the child of unmarried parents, was referred to the North Carolina Baptist Hospital on Feb. 1, 1952, for the diagnosis and treatment of persistent bleeding from the umbilical cord. He was the second child of a 35-year-old mother, the product of a full-term gestation, without complications, with spontaneous delivery after an eight-hour labor in another city. The mother had been given 5 mg. of a vitamin K analogue prior to delivery. On the second day of life, oozing of blood from the
AFIBRINOGENEMIALEWIS, JESSICA H.; FERGUSON, JOHN H.
1954 American journal of diseases of children
doi: 10.1001/archpedi.1954.02050100713002
CONGENITAL hemorrhagic disease due to a lack of fibrinogen has been observed on a number of occasions. In the preceding paper, Prichard 1 has reviewed the published cases, and in this communication we report an additional case.
METHODS
Most of the test methods employed in this paper have been described in detail.2 Prothrombin was assayed by three methods: (1) the standard two-stage method3; (2) a new, specific one-stage method,4 and (3) the BaSO4-eluate method,4 a new test in which prothrombin and proconvertin are quantitatively removed from plasma and the prothrombin titered after activation in the presence of accelerin, proconvertin, thromboplastin (Soluplastin, Schieffelin & Company, courtesy Dr. E. W. Blanchard), and calcium. The proconvertin index is obtained during the same procedure by determining the percentage of thrombin formed during the first minute of activation before addition of proconvertin. Proconvertin was also determined by the method
ACUTE ENDOCARDITIS IN INFANCY AND EARLY CHILDHOODMACAULAY, DUNCAN
1954 American journal of diseases of children
doi: 10.1001/archpedi.1954.02050100717003pmid: 13206386
ACUTE ENDOCARDITIS is uncommon in early childhood and especially so in infancy. The purpose of this paper is to report in detail a case occurring in a newborn infant, to give an account of 13 others in children under age of 2 years, and to review the literature.
REPORT OF CASE IN A NEWBORN
A healthy woman aged 21 years was delivered of her first child on May 30, 1952. Delivery was normal, and the infant, a girl, weighed 8 lb. 6 oz. (3,800 gm.) at birth. She was routinely examined on the second day and presented no evidence of any abnormality. Breast feeding was instituted, and progress was satisfactory until the evening of the seventh day. She then vomited several times and was found to have a rectal temperature of 102.6 F. Examination showed a distended abdomen, with palpable liver and spleen. The terminal phalanx of the left thumb
SALMONELLA MENINGITIS IN INFANCYSMITH, ESMOND S.
1954 American journal of diseases of children
doi: 10.1001/archpedi.1954.02050100734004pmid: 13206387
INTRODUCTION
THE MORTALITY in Salmonella meningitis is high. In a large group reviewed by Henderson,1 in 1948, there was an 87.5% mortality. However, it was pointed out that the sulfonamides had favorably affected the course of the illness in that there were 10 survivals in 19 cases that had received the drug. In the group cited by Beene,2 in 1951, the majority of which had occurred subsequent to the advent of antibiotics, there was an 82.7% mortality. However, the most recent cases seemed to show an increase in the survival rate with the use of broad antibiotic coverage.
A recent case of Salmonella meningitis at Childrens Hospital aroused my interest in the problem. Since 1943, six children have been admitted with proved Salmonella meningitis. A survey of the recent English literature revealed 10 additional cases.* This article is concerned with this group of 16 cases and the effect
CONGENITAL LEUKEMIAO'CONNOR, ROBERT E.; McKEY, ROBERT W.; SMITH, JACK
1954 American journal of diseases of children
doi: 10.1001/archpedi.1954.02050100742005pmid: 13206388
REFERENCES to congenital leukemia are few in number.* Bernhard, Gore, and Kilby,9 in reviewing the literature in 1951, found 14 acceptable cases and added 4 of their own. In 1952, Casilli, Rumsey, and Satulsky10 reported a case of myeloblastic leukemia in the neonatal period. The criteria for acceptance as a case of congenital leukemia are not too clearly defined, but the presence of leukemia cells in the peripheral blood, bone marrow, and visceral organs of an infant in the neonatal period is highly suggestive. It is the purpose of this paper to report a case of congenital leukemia in a 5-day-old boy, with septicemia as a terminal event.
REPORT OF CASE
The infant was born at this hospital after a labor of approximately four hours. The mother was an Rh-positive 19-year-old woman, unipara and secundagravida, whose Kline and Kahn reactions were negative. Total length of pregnancy was estimated
URINARY TRACT CALCULI IN CHILDRENMOORE, C. A.; DODSON, C. C.
1954 American journal of diseases of children
doi: 10.1001/archpedi.1954.02050100745006
A REVIEW of the literature reveals a paucity of reports of urinary tract calculi in infants and children, except in autopsy series. Even in these, the incidence is quite low except in certain areas of the world outside the United States.
INCIDENCE
Thomson,1 in reviewing 2,962 cases of vesical calculus operated on at the Canton Hospital, Canton, China, prior to 1920, found that 25% of the patients were under 10 years of age. Bokay,* in Hungary, collected 1,826 cases of urinary stone in infants and children under 15 years, with 45 being under 1 year of age. In contrast, Hager and Magath,2 of the Mayo Clinic, reported 2,195 cases of vesical calculus, of which only 33 (1.63%) were under 15 years of age, and Campbell,3 in a series of 15,919 autopsies in children under 15 years of age in the United States, found only an incidence of
SULFHEMOGLOBINEMIAFICHTER, ELAINE G.
1954 American journal of diseases of children
doi: 10.1001/archpedi.1954.02050100751007pmid: 13206390
THE SUDDEN appearance of severe cyanosis in a child presents a diagnostic problem to the attending physician. When there are no obvious pulmonary or cardiac abnormalities, and, when in spite of frank cyanosis there is neither dyspnea nor increase in cardiac and respiratory rates, one must consider the presence of abnormal pigment within the red cell.
The following case is noteworthy in that clinically detectable cyanosis occurred in a child to whom a triple sulfonamide preparation was administered in therapeutic dosage.
REPORT OF CASE
The patient, a 5-year-old white boy, was admitted to Charity Hospital of Louisiana complaining of abdominal pain, nausea, and vomiting for five days and cyanosis for one day. There was no previous history of cyanosis, nor were the past and family histories contributory. Ten days prior to admission the child's grandmother thought he needed a tonic and started him on "sulfur and molasses," three times daily.
CONGENITAL TELANGIECTATIC ERYTHEMA RESEMBLING LUPUS ERYTHEMATOSUS IN DWARFSBLOOM, DAVID
1954 American journal of diseases of children
doi: 10.1001/archpedi.1954.02050100756008
SINCE 1941, we have had under observation at the New York Skin and Cancer Unit a dwarf who presented an eruption on the face resembling lupus erythematosus, in addition to a bullous eruption on the lips which was due to hypersensitivity to sunlight. The reason for this report is the fact that in 1953 two almost identical cases were presented at the New York Academy of Medicine, one from the New York Hospital and the other from the Presbyterian Hospital. The occurrence of three almost identical cases speaks strongly for a syndrome entity and justifies this report.
REPORT OF CASE
G. S., a white boy aged 9 years, born in England of Jewish parents, was seen for the first time at the New York Skin and Cancer Hospital on July 13, 1941, because of an eruption on the face which had been present since the age of 2 years.
History.
LARGE CAVERNOUS HEMANGIOMA OF THE LIVERPRYLES, CHARLES V.; HEGGESTAD, GILMAN E.
1954 American journal of diseases of children
doi: 10.1001/archpedi.1954.02050100761009
TUMORS of the liver are exceedingly uncommon in infancy and childhood. When such tumors are found, they are most likely to represent a primary malignant growth. Cavernous hemangiomas of the liver in infancy and childhood, presenting as a primary hepatic tumor, are extremely rare.
Shumacker,1 in 1942, in a thorough review of the literature, was able to find 67 operative cases of hemangiomas of the liver. Of these, only three occurred in the pediatric age group. Two of them occurred in newborn infants; one was a 7-day-old infant reported by Hammer,* and the other, only 3 days of age, was reported by Kissinger and co-workers.2 Both these infants suffered a spontaneous hemorrhage, with death. The other was a 6-year-old girl3 who had a large cavernous hemangioma of the liver resected but died approximately one month postoperatively, with ascites and respiratory failure.
Hendricks, in 1948,4 reported a
CYSTIC CHANGES OF THE LUNGS IN HISTIOCYTOSISKEATS, THEODORE E.; CRANE, JACKSON F.
1954 American journal of diseases of children
doi: 10.1001/archpedi.1954.02050100766010pmid: 13206393
LETTERER-SIWE disease, Hand-Schüller-Christian disease, and eosinophilic granuloma are now generally considered as definite, but not always sharply defined, anatomicoclinical entities.1 They present a common histopathological process characterized, essentially, by the proliferation of histiocytic cellular elements. The usual clinical manifestations of these three varieties of histiocytoses are well recognized. Pulmonary involvement, although not uncommon, is less well appreciated. This is particularly true of primary pulmonary involvement associated with cystic changes.
A review of the literature reveals 12 previously reported cases of histiocytoses with cystic, so-called "honeycomb" lungs.* Of these, the Letterer-Siwe variety comprised the smallest group. At the time lung cysts appeared, most patients did not present a problem of diagnosis, inasmuch as the full-blown anatomicoclinical picture was usually present. The present case, one of Letterer-Siwe disease, is reported because of the unusual primary and apparently isolated pulmonary localization dominated by cystic change.
REPORT OF CASE
A 2½-year-old white girl