Showing papers by "Boston Children's Hospital published in 1969"

Human C′3: Evidence for the Liver as the Primary Site of Synthesis

Abstract: The liver is the primary, if not sole, site of synthesis of the third component of human complement, as shown by a change in the recipient from C'3 FS(0.6) to C'3 SS, the donor type, following homotransplantation of the liver.

Endotoxins and the Immune Response

Abstract: This review deals with immunologic aspects of the effects of endotoxins in susceptible hosts. The subject matter is divided into three sections as follows: (1) biologic effects of endotoxins and immunologic implications, (2) endotoxins as adjuvants, and (3) endotoxins as immuno-suppressants. Not considered is the extensive literature on the antigenic make-up of endotoxins, characterizing the numerous O and R antigens, and endotoxins as immunogens.

Congenital hyperextension with anterior subluxation of the knee. Surgical treatment and long-term observations.

Abstract: 1. Fifteen congenitally hyperextended and anteriorly subluxated knees of eleven patients have been described. All deformities were resistant to conservative treatment and required open reduction. 2. Every patient had associated congenital abnormalities of the hip, seven patients had club-foot deformities, one had calcaneovalgus deformities of the feet at birth, and seven were considered to have arthrogryposis multiplex congenita. 3. Fibrous replacement of the quadriceps was encountered in all of the knees operated on. The lateral portion of the quadriceps was characteristically affected and the medial portion was relatively spared. 4. Lateral placement of the patella and its extensor mechanism was found in over half of the cases. 5. Quadriceps lengthening, anterior capsulotomy, and mobilization of the collateral ligaments were required to effect reduction. The amount of available normal-appearing muscle varied; that which was present was aligned to exert a direct axial pull on the patella in the line of the femur. 6. Twelve knees were followed for from two to twenty-five years (mean, twelve years) after surgery. 7. Seven knees were considered to have good or excellent functional results, three were graded fair, and two poor (one requiring arthrodesis). No patient had any residual hyperextension deformity, and more active extension was obtained than was anticipated from the limited amount of normal muscle observed at operation. 8. The lasting results found in this study suggest that if a congenitally hyperextended knee with subluxation fails to respond to conservative treatment, early open reduction is indicated.

The single ectopic ureter

Abstract: Objectives To correlate renal function with the site of the ectopic orifice in patients with a single ectopic ureter and to evaluate the role of ureteric reimplantation in the preservation of renal function. Patients and methods Forty-four patients (41 female, age 1.5 months to 20 years) with a single ectopic ureter have been managed in our institution in the last 21 years. The classical symptom of continuous wetting with intermittent normal micturition was reported in most of the female patients. The investigative evaluation included intravenous urography (IVU), cysto-urethroscopy, vaginoscopy with retrograde ureteric catheterization, micturating cysto-urethrography (MCU) and ultrasonography. Diuretic renography was carried out in four patients after it became available in 1992. Renal function was assessed in relation to urinary tract anomalies and with outcome after ureteric re-implantation. Results Thirty-eight patients (two males) had a unilateral ectopic ureter; the ectopic orifice was vaginal in 12, vestibular in 11, urethral in nine, at the bladder neck in two, the seminal vesicle in one and undetermined in three. Twenty-one patients had renal and/or ureteric abnormalities, with reflux detected on MCU in three ureters. Associated anomalies included hypospadias (two, one female), skeletal anomalies (two), anorectal malformations (three), cryptorchidism (two), and unilateral cystic ovary (one). Two patients had preoperative hypertension. In 15 patients, renal function was considered sufficient to justify ureteric reimplantation, 14 of whom regained continence. One girl had suprapubic leakage from the bladder and died during secondary nephroureterectomy. Another girl had persistent incontinence; she was found to have contralateral duplex ureters with a vestibular ectopic orifice and was cured after upper polar hemi-nephroureterectomy. IVU and renography carried out in two patients each within 4 weeks of surgery showed a moderate improvement in renal function. Eight patients reported for follow-up after ureteric reimplantation (mean duration 11 months); none had hypertension or urinary infection. Twenty-three patients with rudimentary kidneys underwent nephroureterectomy. Histopathological examination of the excised kidneys showed moderate to severe dysplasia with chronic pyelonephritis. Six patients (one male) had bilateral single ectopic ureters, with normal renal function in the five females. Unilateral reimplantation in the boy resolved the symptoms; one girl died before surgery and the other four underwent bilateral ureteric reimplantation, after which one was dry for up to 3 h while the other three were incontinent, one of whom subsequently underwent urinary diversion. Conclusions There was no clear correlation of renal function with the site of the ectopic ureteric orifice, as most of the patients with a vaginal ectopic ureter had sufficient renal function to justify renal preservation. Ureteric reimplantation preserved renal function, although the improvement after surgery was determined by the degree of renal dysplasia.

Mouse leukemia virus activation by chemical carcinogens.

Abstract: The induction of lymphomas in C57BL mice by methylcholanthrene, urethan, or diethylnitrosamine was accompanied by the development of murine leukemia viral antigen in most of the lymphoid tumors. The cell-free transmission of lymphomas induced by methylcholanthrene and the development of antibody to murine leukemia virus prior to the detection of overt lymphoma in these mice suggest that unmasking of a latent leukemia virus is an indigenous actuating cause of the lymphomas.

Incidence and mechanism of neonatal jaundice related to glucose-6-phosphate dehydrogenase deficiency.

Abstract: Extract: In three population surveys in Greece, the incidence of glucose-6-phosphate dehydrogenase (G-6-PD) deficiency and neonatal jaundice was examined in randomly selected male newborns. The incidence rate of G-6-PD deficiency was 2.92% in the Alexandra survey (population of Athens and southern Greece), 4.93% in the Lesbos survey and 12.5% in the Rhodes survey. In all surveys, severe jaundice without blood group incompatibility or low birth weight appeared much more frequently in G-6-PD-deficient than in normal newborns, but there were striking differences. In Lesbos, severe jaundice was found much more frequently in both G-6-PD-deficient and normal groups than in comparable groups in the other two surveys. Similarly, at 4 days of age, values for serum bilirubin in control groups were 9.43±4.39 mg/100 ml in Lesbos and 7.88±4.8 mg/100 ml in Rhodes (p <0.05). These differences indicate that in Lesbos, an unknown icterogenic factor alone increases the incidence of neonatal hyperbilirubinemia and, in conjunction with G-6-PD deficiency, causes severe neonatal jaundice (serum bilirubin values higher than 16 mg/100 ml) in 43% of G-6-PD deficient infants. In the Rhodes survey, values for hemoglobin and serum bilirubin and reticulocyte count in the G-6-PD-deficient group were compared with values in the cord blood and in samples of blood drawn on the 4th day of life from the control group. The G-6-PD-deficient group had lower hemoglobin values and higher serum bilirubin values than the control group. The mean values (g/100 ml) for hemoglobin in cord blood were 14.42±1.24 and 15.36±1.36 in the G-6-PD-deficient and control groups, respectively. In samples obtained at 4 days of age, the values were 15.6±1.34 and 16.65±1.37, respectively, in the two groups. The mean values (mg/100 ml) for serum bilirubin in cord blood were 1.98±0.64 and 1.68±0.42, respectively, in the two groups. At 4 days of age, the values were 9.86±6.6 and 7.88±4.8, respectively. These results indicate a diminished life span of G-6-PD-deficient erythrocytes in the fetal and neonatal period. Speculation: This study demonstrates the existence in some newborn populations of an icterogenic factor unrelated to blood group incompatibility or G-6-PD deficiency. Further studies are needed to determine the hemolytic or hepatic nature of this factor and whether this factor interacts with ABO incompatibility or prematurity. In this work, the relation of this factor to G-6-PD deficiency has been studied.

Familial neuroblastoma with regression and maturation to ganglioneurofibroma.

Abstract: Two sisters with congenital disseminated neuroblastoma are reviewed clinically and pathologically. Both showed regression of their retroperitoneal tumors to fibrocalcific residues and maturation to ganglioneuroma. In one case, metastatic tumor nodules of the skin, which had matured to ganglioneuroma, through continued loss of ganglion cells came to closely resemble neurofibromas. A similarity to von Recklinghausen9s disease became apparent. The question of a genetic basis for neuroblastoma and its relationship to von Recklinghausen9s disease is discussed.

Neurofibromatosis with paraplegia. Report of eight cases.

Abstract: 1. Previous reports of generalized neurofibromatosis (von Recklinghausen's disease) associated with paraplegia are reviewed and eight additional cases are presented and discussed. 2. Paraplegia in these cases may result from spinal deformity (kyphoscoliosis, subluxation, or dislocation), tumor (neurofibroma, meningioma, or fibrofatty tissue), or a combination of tumor and deformity. 3. Myelography should be carried out in all neurofibromatosis patients with paraplegia to determine the site and cause of spinal cord compression. 4. Early spine stabilization should be considered when the potential for paraplegia exists. 5. Laminectomy may increase instability and contribute to paraplegia. When decompression is performed, stabilization of the spine by fusion is indicated as part of the treatment plan. 6. Anterior cervical-spine fusion may afford the best means of stabilization if the destruction is extensive, if laminectomy has already been performed, or if later decompression may be necessary.

R Factors Improving Survival of Escherichia coli K-12 After Ultraviolet Irradiation

Abstract: Two R factors which have the capacity to improve survival of some strains of Escherichia coli K-12 by approximately 60% after ultraviolet light have been identified and characterized. Both are fi−, but neither produce colicins. The ability to enhance survival can be separated from all other identified R-factor functions. Improved survival does not result from improved excisional capacity, but does require an intact host capacity for genetic recombination. No effects on host cell growth or postirradiation lag were observed. A proposed mechanism of action is described.

Migration and proliferation of diploid human fibroblasts following "wounding" of confluent monolayers.

Abstract: Six diploid human fibroblast strains were grown in confluent monolayers. Holes were scraped in these monolayers and the number of cells proliferating into these “wounds” with time were determined. The migration and mitotic aspects of the proliferation of fibroblasts into these wounds were analyzed separately. Small amounts of undialysed or dialysed serum were essential for cell division but not migration. Saline extracts of skin could not substitute for serum in the medium. Neither zinc nor cupric ion at tolerable concentrations (10−5M) increased the rate of cell proliferation. Normal human fibroblasts did not immediately start to divide from confluency into the “wound” space. Their generation time was about 32–39 hours. Fibroblasts from patients with cystic fibrosis began to divide almost immediately into the “wounded” area. Their generation time was about 48 to 56 hours.

I. The Development of the Hering‐Breuer Inflation Reflex

Abstract: Summary The Hering-Breuer inflation reflex has been studied in babies of varying postmenstrual ages. The strength of the reflex was assessed by relating the relative increase of the length of the breathing cycle to the transpulmonary pressure when the airway was occluded. The Hering-Breuer inflation reflex was found to be very weak at a postmenstrual age of 32 weeks. It was then found to increase to a maximum strength at a postmenstrual age of 36 to 38 weeks. Later on there was a decline of the strength of the reflex. The significance of the findings in relation to the frequent occurrence of periodic and irregular respiratory rhythm in infants of low gestational age has been discussed.

INTRAVENOUS GLUCOSE TOLERANCE, PLASMA INSULIN, FREE FATTY ACIDS AND β‐HYDROXYBUTYRATE IN UNDERWEIGHT NEWBORN INFANTS

Abstract: Summary Blood glucose, plasma insulin, FFA and β-hydroxybutyrate values during intravenous glucose tolerance were reported in 20 small for gestational age (SGA) and 15 appropriate for gestational age (AGA) low birthweight infants. The babies were divided into three groups according to their age when tested; 48 hours. Both the SGA and AGA infants cleared glucose more rapidly with increasing age. The change was more marked in the SGA babies. The clearance rates were similar to those reported in normal full-sized infants. The insulin values before the glucose load were similar in all groups and comparable to those reported in normal newborn infants. The insulin response to glucose was variable. There were no significant differences with increasing age or between the two groups of infants. The insulin curve of the individual infant followed one of three patterns. Most commonly seen was a double-peak curve. The infants who showed a single-peak insulin response had a better but not significantly different glucose tolerance than that of the other babies. Infants with no appreciable insulin response still removed glucose from plasma at a rate similar to those with a double-peak insulin curve. It is concluded that insulin as measured in peripheral plasma could not explain the rate of removal of glucose from the plasma of the newborn low birthweight infant. Infants of low birthweight had higher plasma FFA values as compared to that reported in normal full term infants. The FFA values in SGA infants were higher than those in AGA babies. In both groups of infants, the jS-hydroxybutyrate values were comparable to those reported in normal full-term babies. Thus there was an unexpected discrepancy between the high FFA and relatively low β-hydroxybutyrate levels in plasma. The fall in plasma FFA and β-hydroxybutyrate after glucose was minimal but similar in both groups of infants. The findings are compatible with a decreased sensitivity to insulin in the infants studied.

Hereditary nephritis. Early clinical, functional, and morphological studies.

Abstract: Extract: The accumulation of literature on hereditary nephritis with or without deafness attests to the increased awareness of the existence of this syndrone. It is the purpose of his report to show that there are distinguishing histologic findings in young paitents with minimal disease and normal renal function. Eleven paitents were studies; 4 were females and 7 were males. Releveant family histories and clinical laboratory findings are shown in table I. In all paitents, hematuria, gross or microscopic, was present. Exacerbations of the hematuria occurred as a part of the exaggerated response of these paitents to upper respiratory infection. Renal functional data are shown in table II. These data were generally within normal limits for our laboratory. Table III outlines the morphologic findings observed on renal biopsy. In renal biopsies obtained from 10 patients, there was observed persitence of fetal-like glomeruli characterized by the circumferential crowding of visceral epithelium and an apparent decrease in the number of patent capillaries. The most striking finding in the tubules was the presence of red cell and blood cell casts seen mainly in the distal and collecting tubules. Foam cells were an infrequent finding but, when presnt, were unassociated with interstitial fibrosis. Examination of kidney specimens under the electron microscope reveated the presence of at least ferential crowing of visceral epithelial cells, particularly at the surface, facing Bowman's capsule. These epithelial cells were characterized by large nuclei, small amounts of cytoplasm, apparently poorly developed introacytoplasmic organelles, and fiequent villous-like cytoplasmic projections on the surface. In families from whom past histories are unavailable, recognition of distinct renal biopsy characteristics would help identify this syndrome in the early stages of disease. At this stage, the combination of morphologic features considered to be characteristic would include the presence of a) fetal-like glomeruli, which were seen by electorn microcscopy in 7 paitents and by light microscopy in 10 patients; blood cell casts; and d) foam cells in the absence of interstitial fibrosis. The fetal-like glomeruli present in our paitents did not correspond to any of the normal stages of glomerular development observed under electron microscopy by other investiagators, but rather, appeared to represent dysgenesis. Most patients from whom data are reported were more than 2 years of age, at which time fetal gomeruli are exceptional. Of interest was the observaton that almost identical histology was found to exist among the siblings. Speculation: The fetal-like glomeruli observed on renal biopsy might be the result of either a developmental abnormality or dysgensis. The changes obeserved in all glomeruli studied by electron microscopy suggest that even those glomeruli that appear to be morphologically unchanged under light microscopy were involved in or by the pathologic process. It is suggestd that there is a common rather than a divers etiology for ranal disease in children. Longgitudinal follow-up with repeated renal functionl studies and biopsies will be needed to delineate the natural history.