Brief report: treatment of chronic inflammatory bowel disease in glycogen storage disease type Ib with colony-stimulating factors.
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Abdomen↗
Biomedical subjects
V Gilsanz
Publications and source records attributed to V Gilsanz.
Changes in vertebral bone density in black girls and white girls during childhood and puberty.
BACKGROUND: The prevalence of osteoporosis and the incidence of vertebral fractures are lower in black women than in white women, findings generally attributed to racial differences in adult bone mass. Little is known, however, about the factors that contribute to racial variations in bone mass or the time of life when such differences become manifest. This study was done to characterize the changes in vertebral bone density at various stages of sexual development in black and white females. METHODS: We measured cancellous vertebral bone density by quantitative computed tomography in 75 black female subjects between 2 and 20 years old and 75 whites matched for age and stage of sexual development. RESULTS: The vertebral bone density did not differ between black girls and white girls before puberty. Bone density increased during puberty in each racial group, but the magnitude of the increase from prepubertal values was substantially greater in black than in white subjects (34 percent vs. 11 percent). CONCLUSIONS: The marked difference between black and white females in cancellous vertebral bone density occurs during a relatively brief period late in puberty. Metabolic and hormonal events related to the achievement of sexual maturity during adolescence may be important determinants of racial differences in bone mass in women.
Adolescent↗
Vertebral bone density in insulin-dependent diabetic children.
To determine the effect of insulin-dependent diabetes mellitus (IDDM) on bone mass, we compared the trabecular and cortical bone density in lumbar vertebrae, measured by quantitative computed tomography (CT), in 48 white diabetic patients (23 females, 25 males; 5.2 to 19.6 years of age) with those of a control group of 48 healthy subjects, matched for race, sex, and age. Patients with neuropathy, retinopathy, nephropathy, and those with recent ketoacidosis were excluded from the study. The patient and control groups did not differ in sexual or skeletal maturation, weight, height, surface area, body mass index, abdominal fat, or paraspinal musculature. In diabetic children, cortical bone density was slightly but significantly lower than in controls (3.5% lower, P less than .02); there was no difference between patients and controls regarding trabecular bone density. The decrease in cortical bone density in the diabetic group did not correlate with age, sex, duration of diabetes, or glycosylated hemoglobin levels. These results suggest that in children with uncomplicated IDDM, decreased vertebral bone density is a minor abnormality that only affects cortical bone.
Adolescent↗
Subperiosteal abscess in children: scintigraphic appearance.
A subperiosteal abscess is an uncommon complication of osteomyelitis in childhood. Bone scanning is very sensitive for the detection of early skeletal infection; however, a subperiosteal abscess has a different and distinctive scintigraphic appearance. The usual increased tracer uptake of osteomyelitis is overshadowed by the presence of a "cold" lesion on the delayed views, probably due to interruption of the vascular supply of the bone. Early recognition of the condition is important, as surgery is indicated to prevent extensive bone necrosis and chronic osteomyelitis. In suspected osteomyelitis, a central photopenic area is highly suggestive of subperiosteal abscess; when such an area is seen, a computed tomographic (CT) scan can be helpful in planning the surgical approach for drainage. Plain radiographs may be unremarkable even when the bone scan and CT findings are dramatic. Five such cases are presented, and a recommended diagnostic approach is discussed that views the two procedures as complementary.
Abscess↗
Effect of dietary calcium on bone density in growing rabbits.
Reductions in peak bone mass at skeletal maturity may increase the risk for the subsequent development of osteoporosis. Although changes in calcium intake can modify the rate of decline in bone density in the mature skeleton, longitudinal assessments of the effect of dietary calcium supplementation during skeletal growth on peak bone mass have not been done in humans or experimental animals. Thus quantitative computed tomography (QCT) was used to monitor changes in vertebral bone density at 6-wk intervals during growth from 8 wk of age until skeletal maturity at 35 wk in male New Zealand White rabbits maintained on diets containing 0.15% (low Ca), 0.45% (normal Ca), or 1.35% (high Ca) calcium. Serum parathyroid hormone (PTH) and calcitriol levels increased, and renal calcium excretion decreased in low Ca compared with normal Ca; in contrast, serum calcitriol levels decreased and renal calcium excretion increased from control values in high Ca. Vertebral bone density by QCT did not differ during growth between high Ca and normal Ca, and peak values at epiphyseal closure also did not differ in these two groups. Vertebral bone density was lower, however, throughout the study in low Ca, and peak values at epiphyseal closure remained below those in either normal Ca or high Ca. Quantitative bone histology revealed decreases in cortical thickness in the third lumbar vertebra in low Ca, whereas trabecular bone area did not differ among groups; there was no histological evidence of osteomalacia in low Ca. Thus dietary calcium restriction during growth reduces peak bone mass at skeletal maturity, but raising dietary calcium intake above normal levels does not increase peak bone mass in this experimental model.
Animals↗
Bone mineral density and its association with inherited protein S deficiency.
Two unrelated children with thrombotic disease associated with inherited protein S deficiency and osteopenia have been identified. Measurement of protein S yielded markedly reduced levels of free protein S (less than 12.5%) in both propositi, a normal level of total protein S (79%) in propositus #1, and markedly reduced level of total protein S (34%) in propositus #2. Bone densitometry measurements of the two children revealed trabecular vertebral bone mineral content below two standard deviations. This defect is associated with vertebral body compression fractures in propositus #2. Therefore, it is hypothesized that protein S deficiency is associated with abnormalities of bone mineral density.
Adult↗
Role of chemotherapy in pediatric pulmonary blastoma.
Pulmonary blastoma is a rare malignant tumor of the lung that has been treated primarily with surgery. The effect of combination chemotherapy has not been systematically investigated. Two pediatric cases are reported in whom combination chemotherapy consisting of vincristine, actinomycin-D, cyclophosphamide, cis-platinum, and adriamycin was successfully used. The first case, a 5-year-old boy, underwent incomplete surgical excision of the tumor followed by a 104-week course of combination chemotherapy. The second case is a 3-year-old boy who was initially treated with combination chemotherapy that resulted in an objective response; he subsequently underwent surgical excision. This intensive combination chemotherapy is effective both in inoperable tumors as initial therapy as well as a surgical adjuvant.
Antineoplastic Combined Chemotherapy Protocols↗
Osteoporosis after cranial irradiation for acute lymphoblastic leukemia.
A prospective study was conducted to investigate the possibility of osteoporosis after treatment for childhood acute lymphoblastic leukemia (ALL). Forty-two survivors of ALL had the trabecular bone density of the spine evaluated by quantitative computed tomography, 6 to 98 months (mean 42 months) after completion of chemotherapy. The ALL survivors had significantly lower bone density than age-, gender-, and race-matched nonleukemic control subjects had (10% less, p less than 0.001); this decrease was accounted for solely by the subset of patients who had received cranial irradiation (n = 30; p less than 0.001). The relative reduction in bone density in ALL survivors was unrelated to age at the time of diagnosis or time without therapy. The effects on bone density of 18 Gy and of 22.5 to 25.2 Gy were indistinguishable. We conclude that survivors of ALL commonly have reduced bone density in the lumbar spine and suggest that the diminution is related to nervous system irradiation, not to the disease or to chemotherapy.
Bone Density↗
Infantile hepatic hemangiomas. Clinical features, radiologic investigations, and treatment of 20 patients.
The clinical features, radiologic investigation, and treatment of 20 infants with hepatic hemangiomas are presented. Palpable abdominal mass (n = 18) and cardiac failure (n = 11) were the common presenting features. Nine patients had hyperconsumptive coagulopathy. Seven patients had other hemangiomas. Ultrasound (n = 15) showed the number and distribution of the hemangiomas within the liver. Hypoechoic and hyperechoic elements were present in addition to prominent vascular channels and diminished caliber of the distal aorta. Radionuclide sulfur colloid (n = 12) and labeled red blood cell (n = 7) studies showed the distribution and vascularity of the hemangiomas. Computed tomography (n = 8) revealed central hypointensity with marked peripheral enhancement after contrast. Arteriography now performed only as a prelude to therapeutic embolization demonstrated hypervascularity in each patient, contrast pooling in six and early draining veins in five. Magnetic resonance scanning (n = 3) showed decreased signal intensity on T1 images and high intensity signal on T2. In two patients, there was resolution or improvement of the hemangiomas without therapy. Four patients had surgery (lobectomy [2], trisegmentectomy [1], and surgical evacuation of a central hematoma [1]). Steroids and radiation were given to seven patients, and one patient also required therapeutic embolization. Steroids were the initial therapy in five patients, one of whom later required therapeutic embolization and another cyclophosphamide. Two patients were treated initially with radiation therapy, one of whom also needed emergency hepatic artery ligation. Seventeen of the 20 patients are alive and well from 6 months to 14 years after diagnosis.
Angiography↗
Congenital central hypoventilation syndrome associated with multiple ganglioneuromas.
A five-year-old girl had congenital central hypoventilation syndrome and mediastinal and adrenal tumors. The mediastinal mass was though to be present, retrospectively, for at least four years prior to surgery. Pathology of the excised tumors revealed benign ganglioneuromas. This is the first case reported of an association between CCHS and multiple ganglioneuromas. This suggests that CCHS, like neural crest tumors, may result from maldevelopment of the embryonic neural crest.
Adrenal Gland Neoplasms↗
Vertebral bone density in Scheuermann disease.
The densities of the trabecular bone of two or three lumbar vertebral bodies in twenty adolescents, twelve to eighteen years old, who had Scheuermann disease were measured by quantitative computed tomography. These densities were then compared with those for the same vertebrae of twenty age, sex, and race-matched adolescents who were examined by computed tomography because of trauma. The same scanner and phantom were used in all examinations. Both the adolescents who had Scheuermann disease and the control patients were in good health before the quantitative computed-tomography examination and had not been taking any medications. The density of the trabecular bone density in the patients who had Scheuermann disease was not significantly different (p = 0.28) from that in the controls. There also were no significant differences between the patients and the controls with regard to height, weight, surface area, or body-mass index.
Adolescent↗
Peak trabecular vertebral density: a comparison of adolescent and adult females.
To determine when spinal bone density reaches its peak, the trabecular vertebral density was assessed, via quantitative computed tomography, among females from two age groups: (1) adolescents (aged 14-19 years; N = 24); and (2) young adults (aged 25-35 years; n =24). The adolescent girls had a higher mean trabecular vertebral density (P less than 0.01), suggesting that spinal density reaches its peak around the time of cessation of longitudinal growth and epiphyseal closure.
Adolescent↗
Osteoporosis in cystic fibrosis.
To determine if osteoporosis is prevalent among patients with cystic fibrosis, we compared the vertebral bone density measured by quantitative computed tomography in 57 such patients (29 male, 28 female, aged 3 to 21 years) with those of an age-, race-, and sex-matched control group of 57 healthy subjects. Patients with cystic fibrosis had significantly lower bone density (10% lower, p less than 0.001) than in controls. The decrease in bone density in patients with cystic fibrosis was unrelated to age. Shwachman clinical evaluation scores (based on case history, pulmonary physical findings, growth, and x-ray findings) correlated positively with age-standardized bone density values (p less than 0.01). Male patients had substantially lower bone density than did female patients (p less than 0.02), but bone density differences related to gender were not significant when effects of disease severity were controlled for. Decreased bone density was more common in patients with poor nutritional status as determined by anthropometric measurements (p less than 0.05). We conclude that osteoporosis is a frequent complication in children with cystic fibrosis regardless of their age and is more prevalent in patients with greater disease severity.
Adolescent↗
Hepatic masses in children.
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Carcinoma, Hepatocellular↗
Vertebral bone density in children: effect of puberty.
To determine changes in bone density during growth, trabecular vertebral density and an index of spinal cortical bone were measured with quantitative computed tomography in 101 children. The children were divided by age into three groups: prepubertal, indeterminate, and pubertal. Compared with prepubertal children, pubertal adolescents had significantly higher trabecular bone density and more compact bone in the spine (P less than .001). After controlling for puberty, vertebral bone density failed to correlate significantly with age, sex, weight, height, surface area, and body mass index. The results indicate that bone density increases markedly during puberty.
Adolescent↗
Primary liver tumors in children: comparison of CT and MR imaging.
A prospective study was performed at two institutions to determine the role of magnetic resonance (MR) imaging in the evaluation of primary pediatric liver tumors. The study compared the usefulness of MR imaging and computed tomography (CT) in providing a correct diagnosis, assessing resectability, and determining tumor recurrence in 23 children with liver tumors. The information obtained with both modalities was correlated with surgical data. The results indicate that the accuracy of MR imaging in helping to differentiate benign from malignant lesions is high and identical to that of CT, but not good enough to obviate surgical diagnosis. While all five hepatocellular carcinomas were correctly diagnosed preoperatively with both modalities, neither CT nor MR imaging could help differentiate these carcinomas from aggressive hepatoblastoma or primary hepatic lymphoma. MR imaging helped correctly predict tumor resectability in all 18 children with malignant hepatic tumors; in one, exact tumor extension was misjudged with CT. Postoperative MR studies suggested tumor recurrence in three children who had unremarkable CT examinations. Second-look surgery showed recurrent tumor in two and fibrosis with chronic inflammation at the surgical margin in the third child.
Adolescent↗
Effect of sex steroids on peak bone density of growing rabbits.
To determine the effect of sex hormones on bone density (BD) during growth, longitudinal quantitative computed tomography (QCT) measurements were obtained in growing, castrated New Zealand White rabbits following administration of normal saline, testosterone, or estrogen from 6 wk of age until the time of skeletal maturity. Vertebral QCT densities increased during growth, were highest at the time of epiphyseal closure, and were significantly greater (P less than 0.001) in hormone-treated animals. In vivo QCT measurements in 12 vertebraes correlated strongly (r = 0.92) with percentage of calcium per weight assessed in vitro by neutron activation analysis.
Animals↗
Pulmonary sulcus metastases simulating intraabdominal malignancy in childhood tumors.
Wilms tumor, the most common solid childhood malignancy, is frequently associated with pulmonary metastases, while hepatic metastases occur less frequently. Metastases to the lower lobes of the lungs, when deep in the costophrenic sulcus, may simulate an intraabdominal mass. The differentiation of these lesions is important both diagnostically and therapeutically. Three cases are presented in whom pulmonary sulcus metastases simulated abdominal lesions on computed tomography and could not be clearly localized as thoracic in origin on liver/spleen scintigraphy. Ultrasound evaluation was the most useful; however, integration with other imaging techniques was necessary to correctly identify these lesions as being of pulmonary origin.
Adolescent↗