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[In vitro study of the antimutagenic activity of alphahederin].

Authors have studied the antimutagenic power of alphahederin (a saponin extracted from Hedera helix) versus a clastogenic agent, doxorubicin and an aneugenic agent, carbendazim. We have applied a protocol of incorporation of alphahederin (pretreatment, simultaneous treatment and post treatment) to determine a mechanism of action. According to this protocol, alphahederin induces a significant diminution of the rate of micronuclei wathever the phases of the protocol. These results demonstrate the antimutagenic activity of alphahederin with a mechanism of action, both desmutagenic and bioantimutagenic.

Air Pollutants, Occupational↗

Contrary to the literature, vomiting is not a common manifestation associated with plant exposures.

A review of clinical toxicology references and the contemporay literature illustrates that vomiting is reported as a nearly universal symptom associatedwith exposure to cultivated and wild plants. However, the literature fails to put thesymptom of vomiting into a clinical perspectivewith regard to its frequency of occurrence. To clarify this issue a retrospective review of plant exposures was conducted. AAPCC TESS 1997-1999 was queried electronicaly to identify and extract all plant ingestion exposures where vomiting was documented as being related to the exposure. All exposures with documented ipecac syrup use were excluded. Data were stratified through the use through the use of a relational database. Plant ingestion exposures accounted for 229,538 reports and vomiting was reported in 5,917 (2.6%) of exposures. Vomiting was attributed as a related symptom in 753 different plants. It was reported once in 323 plants, 1-3 times in 495 plants (65.7%), and < or = 10 times in 657 (87.4%) of all plants in the database. Ten plants accounted for 32.3% of the reports of vomiting Philodendron, Spathiphyllum, Narcissus, Dieffenbachia, Phytolacca, Epipremnum, Euphorbia, Eucalyptus, Ficus, Hedera) and represented 1.3% of the plants associated with vomiting. Even with the most common plant exposures, vomiting is not a frequent adverse event.

Humans↗

Survey of scale insects of ornamental plants in Alexandria Governorate, Egypt.

This investigation covers a survey of the scale insects associating with some ornamental plants at three chosen public gardens as well as at the experimental farm of the Agricultural Research Station in Alexandria Governorate, Egypt. A total of nineteen scale insect species belonging to sixteen genera related to four families of the super-family Coccoidea were found infesting eighteen ornamental plants during the period from April, 1998 up to March, 1999. These species are: Family: Asterolecaniidae--Represented by one species only The fig scale Russelaspis pustulans; (Cockerell) = (Asterolecanium pustulans Cock). Family: Coccidae--Represented by the seven species Florida wax scale. Ceroplastes floridensis Comstock, Green shield scale. Chloropulvinaria psidii (Maskell), Long brown scale. Caccus elongatus (Douglas), Brown soft scale Coccus hesperidum (Linn.), Tessellated scale. Eucalymnatus tessellatus (Signoret), Hemispherical scale. Saissetia coffeae (Walker), and Olive soft scale. Saissetia oleae (Olivier) Family: Diaspididae--Represented by the ten species: Oleander scale. Aspidiotus hederae (Vallot), Minute cypress scale. Carulaspis minima (Targioni-Tozzetti), Dictyosprmum scale Chrysomphalus dictyospermi (Morgan), Palm fiorinia scale. Fiorinia fioriniae (Targioni), Latania scale Hemiberlisia lataniae (Signoret), Fig scale. Lepidosaphes ficus (Signoret), Snow scale. Lineaspis striata (Newstead), Masked scale. Mycetaspis personata (Comstock), Olive scale. Parlatoria oleae (Colvee), and White peach scale Pseudaulacaspis pentagona (Targioni-Tozzetti), Family: Eriococcidae--Represented by one species only Eriococcus araucariae (Maskell). During the same study, many species of natural enemies (nine parasitoids and eight predators), were also noticed to be associated with the aforementioned scale insects.

Animals↗

Membrane bioreactors for final treatment of wastewater.

The full-scale existing treatment plant in a paper mill in Hedera, Israel, includes equalization, solids separation by either straining or by dissolved air flotation and biological treatment by activated sludge. The operation of the existing biological process is often characterized by disturbances, mainly bad settling, voluminous bioflocs, followed by wash-out of the biosolids. This paper summarizes the results obtained in a study based on a pilot plant including a membrane biological reactor (MBR) compared to the "conventional" activated sludge process in the aerobic treatment of the effluent obtained from an anaerobic reactor. During the pilot operation period (about 90 days after achieving steady state) the MBR system provided steady operation performance, while the activated sludge produced effluent characterized by oscillatory values. The results are based on average values and indicate much lower levels of suspended solids in the MBR effluent, 2.5 mg/L, as compared to 37 mg/L in the activated sludge. As a result, the total organic mater content was also substantially lower in the MBR effluent, 129 vs 204 mg/L as COD, and 7.1 vs 83 mg/L as BOD. The MBR enabled better nitrification. The ability to develop and maintain a concentration of over 11,000 mg/L of mixed liquor volatile suspended solids in the MBR bioreactor enabled an intensive bioprocess at relatively high cell residence time. As a result the biosolids which had to be removed as excess sludge were characterized by relatively low volatile/total suspended solids ratio, around 0.78. This could facilitate and lower the cost of biosolids treatment and handling. The results of this comparative study indicate that in the case of MBR there will be no need for further treatment, while after activated sludge additional filtration will be required. The study leads to the conclusion that MBR will be the best technology for aerobic treatment of the anaerobic effluent of the paper mill.

Bioreactors↗

New and remarkable records of microfungi from Turkey.

During a botanical excursion of the University of Essen in the year 2002 to North-, Central- and South-Anatolia we have collected representatives of about 100 genera of microfungi as parasites or saprophytes on crops and wild plants. Some of them are new for Turkey: Passalora dubia on Atriplex hortensis, Pseudocercospora ligustri on Ligustrum ovalifolium, Passalora smilacis on Smilax aspera, Uromyces limonii on Limonium spec., Puccinia jasmini on Jasminim spec., Sawadea bicornis on Acer negundo, Puccinia sii-falcariae on Falcaria vulgaris, Phomia hedericola on Hedera helix, Camorosporium pistaciae on Pistacia terebinthus, Erysiphe bahrii on Silene spec., Ramularia heraclei on Apium graveolens. All specimens are located in the Herbarium ESS, Mycotheca parva, collection G.B. Feige & N. Ale-Agha.

Fungi↗

Advanced treatment by anaerobic process followed by aerobic membrane bioreactor for effluent reuse in paper mill industry.

The operation of an activated sludge process at a paper mill (AIPM) in Hedera, Israel, was often characterized by disturbances. As part of a research and development project, a study on new biological treatment was initiated. The study included the operation of three pilot units: a. anaerobic treatment by upflow anaerobic sludge blanket (UASB); b. aerobic treatment by two pilot units including activated sludge and membrane bioreactor (MBR), which have been operated in parallel for comparison reasons. The pilot plant working on anaerobic treatment performed COD reduction from 2,365 to 755 mg/L, expressed as average values. Based on the pilot study, a full scale anaerobic treatment system has been erected. During a period of 100 days, after achieving steady state, the MBR system provided steady operation performance, while the activated sludge produced effluent characterized by oscillatory qualities. The following results, based on average values, indicate much lower suspended solids concentrations in the MBR effluent, 2.5 mg/L, as compared to 25 mg/L in the activated sludge. The ability to develop and maintain a concentration of over 11,000 mg/L of mixed liquor volatile suspended solids in the MBR enabled an intensive bioprocess at relatively high cell residence time. This study demonstrates that the anaerobic process, followed by aerobic MBR can provide effluent of high quality which can be considered for economic reuse in the paper mill industry.

Bacteria, Aerobic↗

[Numerical taxonomy of several pathovars of Xanthomonas campestris].

Numerical methods were used in order to study the relationships between 26 bacterial strains of 18 pathovars of Xanthomonas campestris according to 115 morphological, cultural, physiological and pathogenic characteristics. Similarity for each pair of cultures was calculated by simple matching coefficient. Using single linkage method of association, the strains fall into a major group formed by pathovars undulosa, translucens, secalis, hordei, pelargonii, hederae, pruni, cucurbitae, zinniae, citri, holcicola, vesicatoria, juglandis, campestris and begoniae, clustered at a similarity level of 76.5%. Xanthomonas campestris pv. gummisudans, X. campestris pv. papavericola and X. campestris pv. ricini appeared in separate branches at similarity levels of 74, 66, and 62%, respectively. The results demonstrated that Xanthomonas campestris pathovars formed a close group with high similarity of morphological and physiological characteristics, being only distinguished by their host range. In cross-inoculations, the strains showed many relationships but this "pathogenicity" may not of itself constitute a differential character.

Bacterial Typing Techniques↗

Compositae dermatitis in childhood.

Compositae dermatitis occurred in a 9-year-old boy with a strong personal and family history of atopy. Positive patch test reactions were 2+ for dandelion (Taraxacum officinale), false ragweed (Ambrosia acanthicarpa), giant ragweed (Ambrosia trifida), short ragweed (Ambrosia artemisifolia), sagebrush (Artemisia tridentata), wild feverfew (Parthenium hysterophorus), yarrow (Achillea millifolium), and tansy (Tanacetum vulgare) and 1+ for Dahlia species and English ivy (Hedera helix). Patch tests were negative for another 30 plants, including cocklebur (Xanthium strumarium), dog fennel (Anthemis cotula, fleabane (Erigeron strigosus), sneezeweed (Helenium autumnale), and feverfew (Tanacetum parthenium). The eruption resembled atopic dermatitis morphologically but was prominent on the palms and face and dramatically spared the area of the boy's feet covered by his shoes. The condition has always been seasonal, worsening in summer, especially July, and it clears on avoidance of contact. This case is believed to represent a contact dermatitis to oleoresins of Compositae plants; inhalants as a cause of systemic aggravation are not likely to be important in this patient.

Child↗

Treatment of Wilson disease with ammonium tetrathiomolybdate: III. Initial therapy in a total of 55 neurologically affected patients and follow-up with zinc therapy.

BACKGROUND: It is unclear what anticopper drug to use for patients with Wilson disease who present with neurologic manifestations because penicillamine often makes them neurologically worse and zinc is slow acting. OBJECTIVE: To evaluate the frequency of neurologic worsening and drug adverse effects with ammonium tetrathiomolybdate. DESIGN: Open-label study of 55 untreated patients (22 of them new) presenting with neurologic Wilson disease treated with tetrathiomolybdate varying from 120 to 410 mg/d for 8 weeks and then followed up for 3 years. Neurologic function was assessed with scored neurologic and speech tests. SETTING: A university hospital referral setting. PATIENTS: All untreated, newly diagnosed patients with neurologic Wilson disease. INTERVENTION: Treatment with tetrathiomolybdate. MAIN OUTCOME MEASURES: Neurologic function was evaluated by neurologic and speech examinations. Drug adverse effects were evaluated by complete blood cell counts and biochemical measures. RESULTS: Only 2 (4%) of 55 patients treated with tetrathiomolybdate showed neurologic deterioration, compared with an estimated 50% of penicillamine-treated patients. Five of the 22 new patients exhibited bone marrow suppression and 3 had aminotransferase elevations. These numbers are higher than in the original 33 patients and appear to be due primarily to a more rapid dose escalation. CONCLUSIONS: Tetrathiomolybdate shows excellent efficacy in patients with Wilson disease who present with neurologic manifestations. With rapid escalation of dose, adverse effects from bone marrow suppression or aminotransferase elevations can occur.

Adult↗

Myelopolyneuropathy and pancytopenia due to copper deficiency and high zinc levels of unknown origin: further support for existence of a new zinc overload syndrome.

OBJECTIVE: To describe a patient with idiopathic zinc overload without an identifiable source and secondary copper deficiency causing myelopolyneuropathy and pancytopenia. DESIGN: Case report. PATIENT AND RESULTS: A 46-year-old man presented with severe bone marrow suppression and subsequently developed progressive myelopathy with sensory ataxia. No identifiable cause of myelopathy was detected, and his neuroimaging findings were unremarkable. Plasma analysis demonstrated a low copper level and an increased zinc level (<10 micro g/dL [<12.6-18.9 micro mol/L] and 184 micro g/dL [28.2 micro mol/L], respectively; normal range for both, 80-120 micro g/dL [12.6-18.9 micro mol/L and 12.3-18.4 micro mol/L, respectively) and a low level of ceruloplasmin. There was no evidence for an external source of zinc. Daily oral supplementation with 2 mg resulted in the prompt reversal of hematologic abnormalities, improved but still subnormal plasma copper levels, and normalization of ceruloplasmin values. The patient's neurologic condition deteriorated further, with worsening of myelopathy and development of polyneuropathy. Analyses of plasma copper and zinc levels demonstrated persisting hyperzincemia and subnormal copper levels during 4 years of follow-up. Increased copper supplementation to 8 mg/d partially reversed his neurologic signs. A clinical investigation of 6 siblings and 1 surviving parent did not identify family members with similar abnormalities. CONCLUSIONS: Persistent hyperzincemia without an identifiable external source appears to be a primary metabolic defect, while copper deficiency is a secondary phenomenon, causing hematologic and neurologic abnormalities. Two unrelated patients with similar idiopathic hyperzincemia and hypocupremia have been recently described. This suggests the existence of a new metabolic disorder with idiopathic zinc overload.

Copper↗

The second kindred with autosomal dominant distal myopathy linked to chromosome 14q: genetic and clinical analysis.

BACKGROUND: Distal myopathies (MPDs) are genetically heterogeneous. Genetic causes within this subgroup of muscle disorders remain largely unknown. An MPD linked to chromosome 14q11-q13 (MPD1) is rare, and to our knowledge, only one family with definitive linkage has been described. OBJECTIVE: To describe the results of clinical and genetic analysis of the second kindred with MPD1. PATIENTS AND METHODS: We have identified a family with an MPD segregating in an autosomal dominant fashion. We tested linkage to previously identified genetic loci on chromosomes 2p, 2q, and 14q. The coding sequence of PABP2 (the polyadenylate-binding protein 2 gene) was analyzed. RESULTS: Every affected individual had selective weakness of foot extensors, with the average age of symptom onset at 20 years. Some patients also had proximal weakness, but none had signs of finger or hand extensor muscle involvement, even in advanced stages of the disease. Two typically affected individuals had signs of idiopathic dilated cardiomyopathy. Genetic analysis detected a tight linkage to chromosome 14q11-q13. Recombination at the telomeric end of the 14q11-q13 locus was found in an unaffected individual who was not considered to be at risk, potentially reducing the locus interval by 2 centimorgans. No mutations in the PABP2 gene were identified. CONCLUSIONS: To our knowledge, our described family is only the second known kindred with a chromosome 14-linked MPD in whom the linkage has been unequivocally established. We did not detect signs of involvement of hand or finger extensors and neck muscles, seen in the original family with MPD1. The degree and frequency of proximal weakness seem to be more prominent than in other patients with MPD1. Haplotype analysis suggests that the gene is located between polymorphic microsatellite markers D14S283 and D14S1034 on chromosome 14q11-q13. The presence of cardiomyopathy in some affected individuals may help in the identification of candidate genes.

Adult↗

Novel mutation in the SPG3A gene in an African American family with an early onset of hereditary spastic paraplegia.

BACKGROUND: Mutations in a novel GTPase gene SPG3A cause an autosomal dominant hereditary spastic paraplegia linked to chromosome 14q (SPG3), which accounts for approximately 10% to 15% of all autosomal dominant hereditary spastic paraplegia cases. The mutational spectrum of the SPG3A gene and the phenotype/genotype correlations have not yet been established. OBJECTIVE: To describe a kindred with an infantile onset of hereditary spastic paraplegia caused by a novel mutation in the SPG3A gene. PATIENTS: Complete neurological examination and genetic analysis were performed on 6 affected members of a small African American kindred. Linkage analysis to genetic markers near autosomal dominant hereditary spastic paraplegia loci on chromosomes 2p and 14q was performed. The coding sequence of the SPG3A gene was analyzed, and the identified change in the sequence was tested for being a benign polymorphism by sequencing 200 chromosomes from normal controls. RESULTS: Every affected individual had signs of uncomplicated spastic paraparesis without additional neurological abnormalities. None of the affected family members had ever walked normally. The history was consistent with an infantile onset, despite the normal acquisition of motor milestones. Genetic analysis suggested linkage to the SPG3A locus on chromosome 14q. Analysis of the SPG3A gene revealed a missense mutation C635T, predicted to result in a threonine to isoleucine substitution at codon 156. Analysis of 200 normal chromosomes did not identify the same change in healthy subjects. CONCLUSION: We report a novel mutation in the SPG3A gene in an African American family with an infantile onset of autosomal dominant hereditary spastic paraplegia.

Adolescent↗

Treatment of Wilson disease with ammonium tetrathiomolybdate: IV. Comparison of tetrathiomolybdate and trientine in a double-blind study of treatment of the neurologic presentation of Wilson disease.

OBJECTIVE: To compare tetrathiomolybdate and trientine in treating patients with the neurologic presentation of Wilson disease for the frequency of neurologic worsening, adverse effects, and degree of neurologic recovery. DESIGN: A randomized, double-blind, controlled, 2-arm study of 48 patients with the neurologic presentation of Wilson disease. Patients either received 500 mg of trientine hydrochloride 2 times per day or 20 mg of tetrathiomolybdate 3 times per day with meals and 20 mg 3 times per day between meals for 8 weeks. All patients received 50 mg of zinc 2 times per day. Patients were hospitalized for 8 weeks, with neurologic and speech function assessed weekly; discharged taking 50 mg of zinc 3 times per day, and returned annually for follow-up. SETTING: A university hospital referral setting. PATIENTS: Primarily newly diagnosed patients with Wilson disease presenting with neurologic symptoms who had not been treated longer than 4 weeks with an anticopper drug. INTERVENTION: Treatment with either trientine plus zinc or tetrathiomolybdate plus zinc. MAIN OUTCOME MEASURES: Neurologic function was assessed by semiquantitative neurologic and speech examinations. Drug adverse events were evaluated by blood cell counts and biochemical measures. RESULTS: Six of 23 patients in the trientine arm and 1 of 25 patients in the tetrathiomolybdate arm underwent neurologic deterioration (P<.05). Three patients receiving tetrathiomolybdate had adverse effects of anemia and/or leukopenia, and 4 had further transaminase elevations. One patient receiving trientine had an adverse effect of anemia. Four patients receiving trientine died during follow-up, 3 having shown initial neurologic deterioration. Neurologic and speech recovery during a 3-year follow-up period were quite good. CONCLUSION: Tetrathiomolybdate is a better choice than trientine for preserving neurologic function in patients who present with neurologic disease.

Adolescent↗

Prenatal diagnosis of hereditary spastic paraplegia.

Hereditary spastic paraplegia (HSP) is a degenerative neurologic disorder that causes progressive, often severe, spastic weakness in the legs. Autosomal dominant HSP is a highly penetrant, genetically heterogeneous disorder with loci present on chromosomes 2p21-24, 2q24-34, 8q23-24, 10q23.3-24, 12q13, 14q12-23, 15q11-14 and 19q13.1. We identified a large HSP kindred in which the disorder was tightly linked to chromosome 14q12-23. We tested chorionic villus DNA samples of two at-risk fetuses for inheritance of microsatellite polymorphisms flanking and within this locus that segregated with the disease in this family. Whereas samples from the first fetus showed inheritance of a haplotype segregating with the disease allele (indicating high risk of developing HSP), samples from the second fetus showed inheritance of a haplotype segregating with the normal allele (indicating low risk of developing HSP). This is the first report of prenatal testing for HSP. Published in 2001 by John Wiley & Sons, Ltd.

Adult↗

Possible third case of Lin-Gettig syndrome.

We report a patient with craniosynostosis, severe mental retardation, absence of the corpus callosum, camptodactyly, hypogonadism, and ventricular septal defect. We propose that he has Lin-Gettig syndrome and that he is the third reported patient with this entity. Our patient also had additional phenotypic features, including palatal cleft and absent rapid eye movement (REM) sleep that were not present in the two previously described patients with this syndrome. High-resolution karyotype and subtelomeric fluorescence in situ hybridization (FISH) for cryptic telomeric rearrangement were normal. The existence of an unrelated patient with Lin-Gettig syndrome supports that this is a separate and distinct clinical entity.

Abnormalities, Multiple↗

Retinitis pigmentosa, growth hormone deficiency, and acromelic skeletal dysplasia in two brothers: possible familial RHYNS syndrome.

Here we report two brothers with retinitis pigmentosa, growth hormone deficiency, and acromelic skeletal dysplasia. We propose that their clinical picture is consistent with RHYNS syndrome (retinitis pigmentosa, hypopituitarism, nephronophthisis, and skeletal dysplasia) and that they represent the first instance of a familial occurrence of this syndrome. The presence of RHYNS in two siblings supports an autosomal recessive mode of inheritance; however, since all four known cases were male, an X-linked mode of inheritance cannot be excluded. The combination of clinical features found in these affected males is unique and supports the existence of RHYNS syndrome as a separate and distinct entity.

Abnormalities, Multiple↗