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F V White

Publications and source records attributed to F V White.

At least 19 recordsLinked to original sources
PubMed2001

Paraneoplastic pemphigus and bronchiolitis obliterans associated with a mediastinal mass: A rare case of Castleman's disease with respiratory failure requiring lung transplantation.

Castleman's disease is an infrequent and usually benign lymphoproliferative disorder. Resection of the tumor usually is curative. The immunostimulatory nature of the tumor can, in rare instances, result in paraneoplastic manifestations. The authors present a case of a 14 year old with mucocutaneous ulcerations and progressive dyspnea that was found to have a large mediastinal mass and circulating autoantibodies that were responsible for his paraneoplastic pemphigus and bronchiolitis obliterans. In spite of aggressive immunotherapy to control the autoimmune mucocutaneous lesions, the pulmonary fibrosis was irreversible and progressed to pulmonary failure necessitating lung transplantation. J Pediatr Surg 36:E22.

Adolescent↗
PubMed2000

Gliomatosis peritonei as a complication of a ventriculoperitoneal shunt: case report and review of the literature.

Gliomatosis peritonei, the implantation of neuroglial tissue upon the peritoneal surfaces, is a rare event most often associated with solid or immature teratomas of the ovary in young girls. The authors report a case of a 10-month-old girl with a ventriculoperitoneal shunt (VPS) who presented with bilateral inguinal hernias. Herniorrhaphy was uneventful. Microscopic examination of the hernia sacs showed exuberant mesothelial hyperplasia containing multiple nests of differentiated glial tissue. Subsequent computed tomography and laparoscopy disclosed normal ovaries with no evidence of intraabdominal or pelvic abnormalities. Gliomatosis peritonei in this case was attributed to transport of glial tissue from the cerebrospinal fluid into the peritoneal cavity via the shunt. With the exclusion of an ovarian germ cell neoplasm and in the presence of a VPS, the clinical course with regard to the glial implants in these children is uneventful. If it is appreciated that gliomatosis peritonei may be a complication of a VPS, an extensive clinical evaluation generally is unnecessary.

Choristoma↗
PubMed2000

Prolonged survival in hereditary surfactant protein B (SP-B) deficiency associated with a novel splicing mutation.

Hereditary surfactant protein B (SP-B) deficiency has been lethal in the first year of life without lung transplantation. We tested the hypothesis that SP-B gene mutations may result in milder phenotypes by investigating the mechanisms for lung disease in two children with less severe symptoms than have been previously observed in SP-B deficiency. Immunostaining patterns for pulmonary surfactant proteins were consistent with SP-B deficiency in both children. DNA sequence analysis indicated that both children were homozygous for a mutation in exon 5 that created an alternative splice site. Reverse transcriptase PCR and sequence analysis confirmed use of this splice site, which resulted in a frameshift and a premature termination codon in exon 7. The predominant reverse transcriptase PCR product, however, lacked exon 7, which restored the reading frame but would not allow translation of the exons that encode mature SP-B. Western blot analysis detected reduced amounts of mature SP-B as well as an aberrant SP-B proprotein that corresponded to the size expected from translation of the abnormal transcript. We conclude that a novel splicing mutation was the cause of lung disease in these children and that hereditary SP-B deficiency can be the cause of lung disease in older children.

Female↗
PubMed1999

Congenital disseminated malignant rhabdoid tumor: a distinct clinicopathologic entity demonstrating abnormalities of chromosome 22q11.

The clinical, pathologic, and immunohistochemical features of a widely disseminated tumor with rhabdoid phenotype are described in nine infants < or = 3 months of age. Five neonates had tumor evident at birth, two of which had placental metastases. The average survival following diagnosis was < 6 weeks. None of the infants had an apparent primary tumor in either the kidney or brain. In four cases, the dominant mass involved the head and neck region, and in two cases, the primary mass was paraspinal. The histologic features were those of a high-grade, round cell neoplasm with abundant cytoplasm and containing cells with cytoplasmic filamentous inclusions. Immunohistochemical studies revealed polyphenotypic antigen expression. Genetic information was available from eight of nine cases. Karyotype analysis revealed abnormalities of chromosome band 22q11-12 in three of six tumors. Fluorescence in situ hybridization studies or molecular studies demonstrated 22q11.2 deletions in all five cases with available frozen tissue, two of which had translocations involving 22q by karyotype analysis. The similar clinical and pathologic findings in these rapidly fatal tumors in infants and the demonstration of abnormalities of chromosome 22q11 in a majority of the cases supports their histogenetic and nosologic relationship to the family of malignant rhabdoid tumors that typically occur in young children in several anatomic sites, including kidney, soft tissues, liver, and brain. Like neuroblastoma and rhabdomyosarcoma, malignant rhabdoid tumor can appear as disseminated disease at birth or shortly thereafter.

Chromosomes, Human, Pair 22↗
PubMed1997

Liver transplantation after jejunoileal bypass for morbid obesity.

BACKGROUND: Jejunoileal (JI) bypass was developed as a therapy for morbid obesity in the late 1960s but has since been abandoned because of a high rate of complications, including cirrhosis. The need for liver transplantation after JI bypass has been infrequent, with only four previous patients reported in the literature; however, because the time to develop symptomatic end-stage liver disease after JI bypass may be quite long (25 years or more), the incidence of patients who will require liver transplantation may only now be increasing. STUDY DESIGN: We reviewed our experience with JI bypass and liver transplantation in 380 consecutive adult patients since 1985. RESULTS: Four patients underwent liver transplantation for cirrhosis after JI bypass, all within the last 48 months. The mean duration of time from JI bypass to transplantation was 22.3 years. All patients had complications, in addition to their liver disease, which were related to the JI bypass, which included nephrolithiasis, cholelithiasis, vitamin deficiencies, renal insufficiency, and d-lactic acidosis. One patient had the JI bypass taken down before transplantation, which precipitated acute liver and renal failure, necessitating urgent transplantation. One patient, who had the JI bypass taken down at the time of transplant, has developed recurrent morbid obesity, while the other three patients have not. The one patient who has not had the JI bypass taken down has not developed evidence of recurrent liver disease and is followed with monthly liver function tests and yearly biopsies. CONCLUSIONS: The incidence of patients who require liver transplantation after JI bypass may be on the increase. Take down of the JI bypass may precipitate acute liver failure in the cirrhotic patient. JI bypass should be accomplished either at the time of transplantation or if signs of liver dysfunction occur after transplantation. Liver transplant recipients can be at risk for recurrent obesity after takedown of the JI bypass. Transplantation for those patients with decompensated cirrhosis after JI bypass has demonstrated excellent early results.

Aged↗
PubMed1997

Pathologic features of rhabdomyosarcoma before and after treatment: a clinicopathologic and immunohistochemical analysis.

Few studies have analyzed the relationship among pathology, therapy-induced changes, proliferative activity, and outcome for rhabdomyosarcoma (RMS), despite the challenges of histopathologic interpretation of this tumor after treatment. Although cytodifferentiation and decreased mitotic activity after treatment were documented previously, the clinical consequences of these changes are uncertain because of the small number of cases analyzed. We analyzed 16 RMSs with pre- and post-treatment specimens for clinicopathologic features, outcome, and immunohistochemical data on formalin-fixed, paraffin-embedded tissue for vimentin, smooth muscle actin, muscle-specific actin, desmin, myoglobin, p53 protein, topoisomerase II-alpha, and MIB-1 proliferative activity. Four of eight alveolar (ARMS), five of five botryoid (BRMS), and two of three nonbotryoid embryonal (ERMS) RMSs displayed varying degrees of post-therapeutic histologic maturation and expressed one or more myoid markers. The remaining five RMSs had no cytodifferentiation. Myoid marker expression did not change significantly. In BRMS, MIB-1 and topoisomerase II-alpha proliferative activity decreased after therapy and correlated with cytodifferentiation and survival. This relationship was less clear for ERMS and ARMS. Five nonbotryoid RMSs without cytodifferentiation had either unchanged or increased proliferative activity, and four of these patients died of RMS. Six nonbotryoid RMSs with both cytodifferentiation and residual foci of undifferentiated cells had variable outcomes, including longer survival. We conclude that BRMS and ERMS exhibit therapy-induced cytodifferentiation more frequently than does ARMS. Cytodifferentiation and decreased proliferative activity are associated with favorable outcome in BRMS; unchanged or increased post-therapeutic proliferative activity suggests aggressive biologic potential in ERMS and ARMS. Combined patterns of cytodifferentiation and residual undifferentiated foci might be associated with increased, decreased, or unchanged proliferative activity and are difficult to interpret, but the presence of cytodifferentiation might presage an improved survival. Immunohistochemical analysis for proliferation markers might be useful for highlighting foci of less differentiated RMS or cytodifferentiated tumor cells in contrast to non-neoplastic, terminally differentiated muscle cells.

Actins↗
PubMed1995

Nonrandom chromosomal gains in pilocytic astrocytomas of childhood.

Low-grade astrocytomas are the most common central nervous system (CNS) tumor occurring in the pediatric age group. Although many of these tumors are karyotypically normal, various studies have reported gains of chromosomes in a significant proportion of cases. We have the opportunity to karyotype two pilocytic astrocytomas occurring in 5- and 15-year-old children. These tumors were characterized by stemlines of 49,XY,+4,+7,+8 and 48,XX,+7,+8. Using these patients as index cases and based on additional karyotypic data that have been published, we performed fluorescence in situ hybridization on 25 additional cases of low-grade astrocytomas in children using pericentromeric probes for chromosomes 4, 6, 7, 8, 9, 10, 11, 12, 15, and 17. Six of 18 (excluding the two index cases), or one third, of the pilocytic astrocytomas were characterized by chromosomal gains, most commonly chromosomes 7 and 8, suggesting that trisomy 7 and 8 are relatively common events in the tumorigenesis of pilocytic astrocytomas. In contrast, chromosomal trisomies were not detected in seven well-differentiated fibrillary astrocytomas with any of the probes chosen.

Adolescent↗
PubMed1995

Pathology of intestinal transplantation in children.

The role of mucosal biopsy in the monitoring of pediatric intestinal allografts is analyzed. We performed a retrospective review of all biopsy, resection, and autopsy material from 22 bowel allografts in 21 patients, followed from 6 months to 3 1/4 years and treated on an immuno-suppressive regimen based on FK 506 (Tacrolimus). There were 579 biopsies, of which 35 were stomal, with two to three fragments taken at each biopsy. There were three explanted bowels and three autopsies. Stomal biopsies proved to be inappropriate for monitoring. Biopsies with three to five pieces of tissue per site, under endoscopic direction, provided the most information. Early cellular infiltrate with lymphoid activation in the absence of epithelial apoptotic figures was not considered sufficient to diagnose rejection although preceded it in most instances. At least two apoptotic figures in a gland or several single apoptotic cells in the presence of a lymphoid infiltrate with activated lymphoid follicles and prominent endothelium correlate best with clinical rejection and response to antirejection measures. Epstein-Barr viral disease is common in this population, and early, late, and noncontiguous bowel involvement can be subtle and difficult to distinguish from rejection, though without the apoptosis. Epstein-Barr virus in situ probes are essential to make the differential diagnosis and the two conditions may co-exist. Mucosal biopsy monitoring appears to be of clinical utility and is part of a program that involves clinical, endoscopic, microbiological, and morphologic assessment.

Adolescent↗
PubMed1995

A syndrome involving immunodeficiency and multiple intestinal atresias.

Multiple intestinal atresias (MIA) is a severe form of intestinal atresias throughout the gastrointestinal tract. In two reports, MIA have been associated with severe immunodeficiency. We report a newborn girl who had profound humoral and B cell immunodeficiency and impaired T cell function. The patient had agammaglobulinemia and decreased blood lymphocytes, with virtually no B cells in the blood or in intestinal lymph nodes. T cells were reduced in number and weakly proliferated to mitogens. These data suggest that a syndrome involving the development of MIA is associated with various forms of severe immunodeficiency, and therefore newborns with MIA should be examined for immunodeficiency.

Agammaglobulinemia↗
PubMed1993

Occult breast calcifications sampled with large-core biopsy: confirmation with radiography of the specimen.

In a series of nine stereotaxic large-core biopsies of calcifications, radiography of the specimen was used to ensure that appropriate tissue was removed. In eight cases, calcifications were evident at radiography as well as at histologic evaluation. Four cases were malignant and four were benign, and in one case in which calcification was not evident at either radiographic or histologic evaluation, a benign process was confirmed at surgical excision. Radiography of the specimen is a key component in diagnosis of breast calcifications sampled with large-core technique.

Biopsy↗
PubMed1989

Lipid metabolism during early stages of Wallerian degeneration in the rat sciatic nerve.

We examined changes in biosynthetic capacity of sciatic nerve during the early stages of Wallerian degeneration, utilizing a model that permits exclusion of nonresident cells from degenerating nerve. Sciatic nerve segments were placed in either 5-microns pore (allowing infiltration of nonresident cells) or 0.22-microns pore (excluding nonresident cells) Millipore diffusion chambers and then implanted in the peritoneal cavity of the same 32-34-day-old rat. At times up to 7 days postsurgery, nerve segments from the chambers, as well as control segments from the contralateral sciatic nerve, were removed and their capacity to incorporate radioactive precursors into lipids and proteins assayed in vitro. In nerve segments from both the 0.22- and 5-microns pore chambers, incorporation of [14C]acetate into total lipids was decreased relative to control by 50% at 12 h postsurgery and by 85% at day 3. This decreased incorporation of [14C]acetate reflects primarily decreased de novo synthesis of cholesterol and of fatty acyl residues incorporated into glycerolipids and sphingolipids. There was a preferentially decreased synthesis of cerebrosides and cholesterol (components enriched in myelin) relative to other lipids, while cholesterol esters and free fatty acids (products of membrane degradation) accounted for a greater proportion of the greatly reduced levels of total lipid label. In contrast to [14C]acetate, incorporation of [3H]glycerol into lipids was increased up to fourfold, relative to control, 1 day after nerve transection.(ABSTRACT TRUNCATED AT 250 WORDS)

Acetates↗
PubMed1988

Metabolism of functional groups modifying the CNS myelin-associated glycoprotein.

We have examined the metabolic turnover of the peptide backbone of the CNS myelin-associated glycoprotein (MAG) and of the fucose and sulfate groups modifying this protein. Rats (20 or 90 days old) were injected intracranially with mixtures of [3H]fucose and [14C]glycine, [3H]glycine and [35S]sulfuric acid, or [3H]fucose and [35S]sulfuric acid. At times ranging from 30 min to 4 weeks later, myelin was isolated, and radioactivity in MAG was determined following electrophoretic separation. Following the peak of incorporation, glycine-derived radioactivity in the MAG peptide backbone declined several-fold during the first week and was then metabolically stable (half-life much greater than 1 month). Declines with time in [3H]fucose- and [35S]sulfate-derived radioactivity in MAG were similar to that of [3H]glycine, an observation indicating that the fucose and sulfate groups modifying MAG are metabolized together with the peptide backbone as a single metabolic entity. These results were confirmed by experiments involving selective immunoprecipitation of MAG. The rates of incorporation of labeled glycine, fucose, and sulfate into MAG all decreased approximately 12-fold between 20 days of age and adulthood, a finding providing further evidence for concerted turnover of the entire molecule. Because of this concerted turnover, we suggest that functional groups modifying MAG serve some permanent structural role in protein configuration.

Animals↗
PubMed1988

Expression of myelin components in mouse Schwann cells in culture.

Mouse Schwann cells cultured in vitro are capable of expressing basal levels of the major myelin components P1, P2, P0, and galactocerebroside. Numerical counts of immunostained cultures indicated that between 22 and 40% of the cells are positive up to 21 days for all of the components indicated. Electrophoretic analysis of Schwann cells labeled with a 14C-amino acid mixture revealed the presence of proteins with relative mobilities identical to those of P0 and P1. Positive identification of the two proteins was indicated by immunoprecipitation of P1 and immunoblotting of P0. These data show that in the absence of neurites, Schwann cells in culture can express low levels of myelin characteristic components even in the absence of myelin assembly.

Amino Acids↗
PubMed1986

Trembler mouse Schwann cells in culture: anomalies in the synthesis of lipids and proteins.

We investigated the biochemical and growth properties of Schwann cells from the sciatic nerve of Trembler and unaffected mice in culture. Both Trembler and control cultures showed similar growth rates. The specific activity of 2',3'-cyclic nucleotide 3'-phosphodiesterase (CNP) and enzymes involved in lipid metabolism of cerebrosides and sulfatides were studied. UDP-galactose: ceramide galactosyltransferase was significantly decreased in Trembler cultures less than 21 days in vitro. No differences were found in the specific activities of cerebroside sulfotransferase, arylsulfatase A or CNP between Trembler and control cultures. Schwann cells from Trembler and control mice were labeled with [35S]methionine and the protein analyzed by two-dimensional gel electrophoresis. Our study revealed few but consistent differences in the protein pattern synthesized by the Trembler Schwann cells.

Animals↗
PubMed1986

Developmental pattern for phosphatidylserine decarboxylase in rat brain.

In adult rats, a significant portion of brain ethanolamine glycerophospholipids are synthesized by a pathway involving phosphatidylserine decarboxylase, a mitochondrial enzyme. We have now examined whether this enzyme plays a particularly prominent role during development. Activities for both phosphatidylserine decarboxylase and succinate dehydrogenase (another mitochondrial enzyme) were determined in brain homogenates from rats 5 days of age to adulthood. Succinate dehydrogenase activity, expressed on a per unit brain protein basis, increased markedly during development. This pattern has been reported previously and is as expected from the postnatal increase in oxidative metabolism. In contrast, phosphatidylserine decarboxylase activity decreased 40% from 5 to 30 days of age. The apparent Km for brain phosphatidylserine decarboxylase was 85 microM in both young (8- and 20-day-old) and adult animals. Parallel studies in vivo were carried out to determine the contribution of the phosphatidylserine decarboxylase pathway, relative to pathways utilizing ethanolamine directly, to the synthesis of brain ethanolamine glycerophospholipids. Animals were injected intracranially with a mixture of L-[G-3H]serine and [2-14C]ethanolamine and incorporation into the base moieties of the phospholipids determined. The 3H/14C ratio of ethanolamine glycerophospholipids decreased about 50% during development. Our studies in vitro and in vivo both suggest that phosphatidylserine decarboxylase plays a significant role in the synthesis of brain ethanolamine glycerophospholipids at all ages, although it is relatively more prominent early in development.

Animals↗
PubMed1983

Growth properties and biochemical characterization of mouse Schwann cells cultured in vitro.

Purified secondary cultures of mouse Schwann cells (less than 5% fibroblast contamination) have been obtained by taking advantage of the differential adhesion of Schwann cells and fibroblasts during trypsinization. The growth properties of the purified subcultures changed with time in culture. Cells passaged after 5 days in vitro (DIV) divided rapidly (doubling time 22 h), whereas cells that had been in vitro for longer periods progressively decreased their growth rate, becoming quiescent after 20 or more days. Schwann cells lacked the Thy 1.2 surface antigen, but were positively stained with antigalactocerebroside antibodies after prefixation. Biochemical analyses showed Schwann cells to be enriched in the activities of enzymes characteristic of the myelin-forming cells: 2'3'-cyclic nucleotide 3'-phosphodiesterase (CNP), cerebroside sulfotransferase (CST) and UDP-galactose: ceramide galactosyltransferase (CGalT).

2',3'-Cyclic Nucleotide 3'-Phosphodiesterase↗
PubMed1979

The use of liposomes as acceptors for the assay of lipid glycosyltransferases from rat brain.

Preparation and characterization of sonicated vesicles of various lipid composition containing hydroxy and normal fatty acid ceramides are reported. Such vesicles have been successfully used for the first time as acceptors for the assays of lipid glycosyltransferases, UDP-galactose:ceramide galactosyltransferase and UDPglucose: ceramide glucosyltransferase. Stability of the vesicles and the optimal enzyme activities were the criteria used to select the final composition of the vesicles. The activities of the glycosyltransferases were dependent not only on the appropriate assay conditions but also on the type and source of the phospholipids used to form the liposomes. Ceramides containing normal fatty acids were incorporated into phosphatidylcholine vesicles in a molar ratio of 1 : 3.4 and used as the acceptor for the assay of UDPglucose:ceramide glucostyltransferase. For the assay UDP-galactose:ceramide galactosyltransferase, vesicles were prepared by sonication of bovine brain ethanolamine phospholipids, phosphatidylcholine and ceramide containing alpha-hydroxy fatty acids, in a molar ratio of 6 : 0.57 : 1. The size of the vesicles as determined by electron microscopic measurement ranged mostly between 200--500 A. The results obtained by selective labelling of the outer surface amino groups with the membrane-impermeable reagent, 2,4,6-trinitrobenzenesulfonic acid, indicated that the ethanolamine phospholipid-containing liposomes consisted of closed vesicles. After incubation with the appropriate cofactors and labelled sugar nucleotides, the radioactive reaction products were shown to cochromatograph with the authentic standards by thin-layer chromatography and autoradiography.

Animals↗