Showing papers by "Boston Children's Hospital published in 1981"

Perivascular meningeal projections from cat trigeminal ganglia: possible pathway for vascular headaches in man

Abstract: Peroxidase-containing cell bodies were found in the ipsilateral trigeminal ganglia after horseradish peroxidase was applied to the proximal segment of the middle cerebral artery in seven cats. Cell bodies containing the enzyme marker were located among clusters of cells that project via the first division. The existence of sensory pathways surrounding large cerebral arteries provides an important neuroanatomical explanation for the hemicranial distribution of headaches associated with certain strokes and migraine.

Postnatal development of renal function in pre-term and full-term infants

Abstract: This study has been designed to examine the effect of gestational age (GA) on the postnatal development of renal function and has been performed in pre-term (PT) infants (GA=30-34 weeks) and in full-term (FT) infants (GA=39-41 weeks). Postnatal age has ranged from 1-35 days. From 8 hour urine samples collected after spontaneous voiding and a capillary blood sample, determinations have been made of the clearance of creatinine (CCr), the fractional excretion of beta 2-microglobulin (FE beta 2) and the fractional excretion of sodium (FENa). In some infants receiving fluid parenterally, simultaneous determinations were made of the clearance of creatinine and inulin. As judged from this study, CCr is a reliable indicator of the glomerular filtration rate (GFR). GFR was almost the same in newborn PT and FT, but from 0.3--1 week of age GFR increased significantly more rapidly in FT than in PT. From 1--5 weeks of age GFR increased at approximately the same rate in PT and FT infants. The absolute value for GFR in 3--5 weeks old infants was lower in PT than in FT. FE beta 2 was higher in PT than in FT infants during the entire first month of life and FENa was higher in PT than in FT infants during the first week of life, suggesting a glomerular tubular imbalance at least at the level of the proximal tubule in PT infants. It is concluded that different stages of maturation will alter the preconditions for the renal adaptation to extrauterine life during at least the first month of life. Therefore special attention must be paid to the limited renal function in PT during their entire first month of life.

Limited wrist arthrodesis. Part II: Intercarpal and radiocarpal combinations

Abstract: Fusion of selected bones of the carpus occasionally combined with the radius or metacarpal bases, has been used to treat a wide variety of serious pathologic conditons of the wrist. Indications include severe localized degenerative arthritis and posttraumatic changes secondary to subluxation, dislocation, or residual instability resulting in significant pain. The procedure was performed on 28 wrists in 26 patients. There were three failures to obtain union initially, two of which united after secondary grafting procedures. All patients had less pain and most had functional, though limited, ranges of wrist motion. Limited wrist arthrodesis provides a workable alternative to complete wrist fusion and Silastic wrist arthroplasty for many wrists with localized destruction.

Systems for the controlled release of macromolecules

Abstract: Delivery systems manufactured in the form of polymeric compositions for the controlled delivery of macromolecules to environments of use are disclosed. The systems are characterized as two-phase compositions comprising a phase formed of an insoluble polymeric matrix having limited water sorptivity containing in admixture therein an interpenetrating phase formed of a particulate hydrophilic water swellable, biologically active macromolecular material.

The "cat eye syndrome": dicentric small marker chromosome probably derived from a no.22 (tetrasomy 22pter to q11) associated with a characteristic phenotype. Report of 11 patients and delineation of the clinical picture.

Abstract: Eleven patients with the so-called Cat Eye syndrome are reported including a more detailed description of the original cases reported by Schmid and Fraccaro. All cases had, in addition to a normal karyotype, a small extra G-like chromosome which appeared to be an isochromosome for the juxtacentromeric region (pter to q11) of an acrocentric chromosome. None were mosaics. Clinical findings and further cytogenetic studies in a few cases suggest that these markers probably derive from a No. 22 chromosome. Characteristic features of the Cat Eye syndrome in these 11 patients and those reviewed from the literature are: ocular coloboma which may involve the iris, choroid and/or optic nerve, preauricular skin tags and/or pits which are probably the most consistent feature, congenital heart defect, anal atresia with a fistula, renal malformations such as unilateral absence, unilateral or bilateral hypoplasia, and cystic dysplasia, and antimongoloid position of eyes. Intelligence is usually low-normal, although moderate retardation is also seen. There is great variability in the clinical findings ranging from near normal to lethal malformations. Less frequent, but also characteristic findings are: microphthalmia, microtia with atresia of the external auditory canal, intrahepatic or extrahepatic biliary atresia and malrotation of the gut. Direct transmission of the marker from one generation to the other was observed in both sexes. In those families, there was considerable variability in the clinical findings between affected family members. These cases show that there is a bias of ascertainment for patients who have the more striking malformations, especially those with ocular coloboma and anal atresia, a combination which appears to be present in only a minority of cases. Many mildly affected patients probably remain undetected. It is proposed that the term Cat Eye syndrome should be applied only to cases with trisomy or tetrasomy of not more than 22pter to q11 and without additional duplication or deletion of another autosomal segment.

Heritable fragile sites on human chromosomes. VI. Characterization of the fragile site at 12q13.

Abstract: The fragile site at Xq27 which is associated with X-linked mental retardation with macroorchidism has been studied in 21 retarded males. These males were from 12 families, and studies of nine of the families were possible. Detection of carrier females is difficult, especially with increasing age. The fragile site was demonstrated in only five of 13 obligate carrier females. It is concluded that using present methods, cytogenetic detection of carriers is fairly reliable in females aged less than 20–25 years but unreliable in older females.

Focal nodular hyperplasia of the liver: a study of 21 pediatric cases.

Abstract: Twenty-one cases of focal nodular hyperplasia of the liver in pediatric patients are presented and compared with 40 cases previously reported. In this series, the lesion was usually asymptomatic (95%) and presented as a nontender mass noted on routine physical examination or was discovered incidentally at autopsy. There was a distinct female predominance (81%). Radiographic examination demonstrated a vascular space-occupying hepatic mass. The lesions were noted bilaterally or in the left lobe in 62% of cases. They were large, nonencapsulated, firm masses with central stellate areas subdividing the lesions into multiple lobules. Microscopically, septa contained eccentrically thickened vessels, small bile ducts, and an acute and/or chronic inflammatory infiltrate. Hepatocytes resembled those of the normal liver but some contained increased glycogen and fat. The lesions had no malignant potential and, except in women taking oral contraceptives, could be treated conservatively.

Sleep patterns in hyperkinetic and normal children.

Abstract: Sleep patterns in nonmedicated hyperkinetic (n = 11) and normal control (n = 11) male children (8-12 years old) were compared to document possible sleep disturbance in hyperkinetic children. Electroencephalographic, electro-oculographic, electromyographic, and autonomic measures were monitored continuously for five consecutive nights. Analysis of sleep pattern variables revealed a significantly longer rapid eye movement onset latency (p less than 0.05) and marginally significant greater absolute and relative amounts of movement time (p less than 0.07) for the hyperkinetic group relative to controls. No other sleep parameters differentiated the groups. It was concluded that baseline tonic sleep parameters do not indicate marked sleep disturbance in hyperkinesis. The results are discussed within the context of hypothesized arousal dysfunction underlying this disorder. Key Words: Hyperkinetic children--Hyperkinesis--NREM and REM sleep cycles--Spontaneous skin potential responses.

Amphetamine Addiction and Pregnancy: II. Pregnancy, delivery and the neonatal period. Socio-medical aspects

Abstract: The adverse effects of amphetamine addiction during pregnancy and the neonatal period were studied in 69 Swedish women. Almost one-third of the women (Group I) succeeded in overcoming their addiction in early pregnancy. The women in Group I (n = 17), unlike those in Group II (n = 53), received the same amount of prenatal care as the average Swedish woman. An increased rate of preterm deliveries (25%) as well as a higher perinatal mortality (7.5%) was found in Group II. During the neonatal period an increased incidence of mother-infant separation was found since many of the infants (46%) were transferred to pediatric wards for medical and social reasons. All newborns in Group I and 74% of infants born to mothers with continuous amphetamine addiction throughout pregnancy remained in their mother's custody following discharge from the maternity clinic.

Granulosa theca cell tumors in premenarchal girls: a clinical and pathologic study of ten cases.

Abstract: Granulosa theca cell tumor (GTCT) in the premenarche accounted for 7% of all ovarian tumors treated at the Children's Hospital Medical Center from 1928 through 1979. The average age of the ten girls at the time of diagnosis was 5 years (range 11 months-121/2 years) with precocious "pseudopuberty" and an abdominal mass being the most common presentation. GTCTs were solitary (five right, five left) with an average diameter of 12.1 cm. Histologic examination showed a predominantly diffuse or solid pattern with prominent luteinization; Call-Exner bodies and folded ("coffee-bean") nuclei were inconspicuous. Ultrastructural examination of one GTCT confirmed the presence of both granulosa and theca components with the latter showing extensive luteinization; estradiol, testosterone and prolactin were demonstrated in the same tumor using immunologic techniques. The average follow-up time was 21 years with nine of the ten patients being followed for more than ten years. Salpingo-oophorectomy resulted in cures despite the occurrence of tumor spillage in two patients. The prognosis for GTCTs in the premenarche appears more favorable than for those occurring in adulthood, but further study is needed to completely define their full biologic potential; the significance of subsequent breast cancer in two long-term survivors merits further investigation as well.