Strategies for limb lengthening in achondroplasia using the Ilizarov method--the experience of the hospital of Lecco, Italy.
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Achondroplasia↗
Biomedical subjects
R Cattaneo
Publications and source records attributed to R Cattaneo.
Limb lengthening in achondroplasia by Ilizarov's method.
In achondroplasts limb lengthening can restore the normal proportion of the body. The Ilizarov method of limb lengthening has been used in 37 patients, 23 of whom have had an adequate follow-up after operation. In this technique the cortex of the bone is partially cut, leaving the medulla intact; the two bone segments are then subjected to distraction by means of a special apparatus. The bone tissue between the two segments starts regenerating and this allows a considerable increase in length. The results and complications are reported. It is considered that this method is based on sound principles and is free of major risks.
Achondroplasia↗
Restriction of measles virus gene expression in measles inclusion body encephalitis.
Measles virus (MV) infection in brain tissue of a patient with measles inclusion body encephalitis was characterized by immunologic and biochemical techniques. Of the five major structural proteins of MV, only nucleocapsid (N) protein and phosphoprotein (P protein) were consistently detected in diseased brain areas. In contrast, hemagglutinin protein was seen only occasionally, and no membrane and fusion proteins were found in any of the sections studied. Messenger RNAs (mRNAs) specific for these five viral proteins were detected in all brain extracts examined; however, the mRNAs for the envelope proteins were clearly underrepresented in comparison with lytically infected cells. Only the mRNAs for N and P proteins appeared active in in vitro translations. These findings indicate quantitative differences in the pattern of mRNA expression in brain tissue and a restricted expression of MV envelope proteins in infected cells as observed in subacute sclerosing panencephalitis.
Brain↗
HLA antigens and thrombocytopenia in HIV seropositive subjects.
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HIV Seropositivity↗
Multiple viral mutations rather than host factors cause defective measles virus gene expression in a subacute sclerosing panencephalitis cell line.
A measles virus (MV) genome originally derived from brain cells of a subacute sclerosing panencephalitis patient expressed in IP-3-Ca cells an unstable MV matrix protein and was unable to produce virus particles. Transfection of this MV genome into other cell lines did not relieve these defects, showing that they are ultimately encoded by viral mutations. However, these defects were partially relieved in a weakly infectious virus which emerged from IP-3-Ca cells and which produced a matrix protein of intermediate stability. The sequences of several cDNAs related to the unstable and intermediately stable matrix proteins showed many differences in comparison with a stable matrix protein sequence and even appreciable heterogeneity among themselves. Nevertheless, partial restoration of matrix protein stability could be ascribed to a single additional amino acid change. From an examination of additional genes, we estimated that, on average, each MV genome in IP-3-Ca cells differs from the others in 30 to 40 of its 16,000 bases. The role of extreme variability of RNA virus genomes in persistent viral infections is discussed in the context of the pathogenesis of subacute sclerosing panencephalitis and of other human diseases of suspected viral etiology.
Animals↗
[The Ilizarov method in the treatment of severe axial deviations of the limbs].
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Adolescent↗
[Evaluation of a new multi-RAST method in the screening of allergy to inhalants].
Sera from 1040 adult patients were tested by a new multi-RAST test and PRIST to determine the efficiency of each test in a screening for allergy to inhalants. The patients had been referred to our Institutions for allergological evaluation of respiratory diseases. All the patients had been thoroughly investigated by clinical history, skin prick test and RAST. In 505 a diagnosis of respiratory allergy to one or more inhalants allergens was established, while in 535 a diagnosis of atopy was not confirmed. The new multiRAST test consented to correctly detect 464 out of 505 atopic patients (efficiency 92%) and 517 out of 535 non atopic patients (efficiency 97%). PRIST showed in both cases a minor efficiency (71% in detecting atopy and 77% in detecting non atopy).
Adult↗
[Sensitization against Parietaria pollen in a group of 1143 patients in the Brescian area].
1143 patients were selected among atopic outpatients followed up at our Institution (Clinical Immunology-University of Brescia) on the basis of documented sensitization to one or more inhalant allergens. All patients had been investigated by skin prick tests employing a large panel of allergens. Specific sensitization had been confirmed by clinical history and when necessary by RAST. The patients were investigated retrospectively for the prevalence of sensitization to Paretaria. 880 patients were sensitized to one or more pollens and among these 427 (48.5) to Parietaria. Among pollen monosensitized patients (with no concomitant allergy to other inhalants), 222 were sensitized to Graminacee and 125 to Parietaria. In these groups there was no difference in prevalence of asthma. Our study thereby shows that the prevalence of sensitization to Parietaria in Brescia is higher than described in other cities in North Italy. Our findings are supported by pollen concentration in atmosphere as determined by Burkard pollen trap.
Adult↗
Lymphocytosis of large granular lymphocytes in splenectomized subjects.
Lymphocytosis of large granular lymphocytes (LGL) has been observed in 6 patients splenectomized for various pathological conditions. In all of them the LGL count was higher than 3.5 x 10(9)/l. No patient showed neutropenia nor suffered from rheumatoid arthritis. A surface markers heterogeneity was observed by immunophenotypic studies. A reversal of the CD4/CD8 ratio was observed in all patients, indicating that LGL are in the majority CD8+. Three patients showed the phenotype CD2+ CD3+ CD4- CD8+ indicating the T-lineage derivation of LGL; patient 6 showed a non-T non-B phenotype (CD2- CD3- CD4- CD8+/-). The percentage of lymphocytes presenting LGL-related markers (HNK-1, CD16, CD11b) was higher than that observed in normal subjects in 4 out of 5 examined patients. However, the percentage of cells bearing these markers was inferior to the LGL counts indicating that not all LGL express them. NK cytotoxic activity was similar to that of normal subjects in the three examined patients. Our data suggest that lymphocytosis of LGL in splenectomized subjects is a reactive process favoured by the asplenic state.
Adult↗
Neurofunctional assessment of early phases of Alzheimer's disease: a preliminary note on hemispheric EEG characteristics during cognitive tasks.
EEG characteristics, in terms of power distribution in 1-4, 4-6, 6-8, 8-10, 10-13, 13-18, 18-30 Hz frequency bands were evaluated in six putative non-medicated male Alzheimer patients. The latter were compared with six age and sex-matched normal controls during both resting conditions and tactile identification tasks specific for the left and right hemispheres. The result indicate a significant difference in the power of the 8-10 frequency band between the two groups and a significant hypovariability in the 6-8 and 8-10 bands during tactile tasks: Alzheimer patients showed reduced power and, also, where less reactive, no differences between the left and right hemispheres having appeared. These findings seem to confirm the specificity of EEG modifications in Alzheimer's disease, even in the very early phases of the pathological process. Possible implications are discussed in the light of some biochemical hypotheses.
Alzheimer Disease↗
A procedure for selective full length cDNA cloning of specific RNA species.
A method allowing routine establishment of full length and functionally competent cDNA clones of particular mRNAs from small preparations of polyadenylated RNA is described. Pairs of synthetic primers are used for first and second strand synthesis. They include sequences complementary to the 3' terminal regions of the mRNAs and of the full length first cDNA strands, respectively and bear a few additional nucleotides at their 5' ends. After synthesis of both cDNA strands in one tube, they are precisely trimmed back with T4 DNA polymerase in presence of only two nucleoside triphosphates, to yield sticky ends fitting into a vector plasmid cleaved with two restriction endonucleases. The procedure was first applied to the simultaneous cloning of all five major measles virus (MV) mRNA species from a persistently infected cell line. Two thirds of all clones contained full length MV-specific cDNAs. Screening of less than 200 clones was sufficient to obtain several independent clones corresponding to each mRNA, except for gene F which was represented only once.
Cloning, Molecular↗
Altered transcription of a defective measles virus genome derived from a diseased human brain.
Measles virus (MV) is a negative strand RNA virus which usually causes acute disease, but in rare cases its persistence in the human brain induces the lethal disease subacute sclerosing panencephalitis (SSPE). The transcription of MV and of a defective MV derived from autopsy material of a SSPE case was studied in cultured cells. In the lytic infection the levels of the MV mRNAs decreased progressively with the distance of the six cognate genes from the 3' end of the genome, reflecting transcriptional attenuation at every gene junction. Transcripts covering two or three adjacent genes accounted for up to 20% of single gene transcripts; incidentally the MV intergenic transcription signals were found to be less conserved than the analogous signals of other negative strand RNA viruses. Although the analysed SSPE-derived defective MV showed a localized transcription defect at the phosphoprotein--matrix gene junction (substitution of the mRNAs by readthrough transcripts), the corresponding intergenic 'consensus' sequence and the surrounding nucleotides were not altered. This implies that factor(s) involved in the transcription of this defective SSPE virus fail to recognize this particular signal sequence, a constellation which in this and other cases might be causally related to the development of MV persistence.
Base Sequence↗
Altered ratios of measles virus transcripts in diseased human brains.
In rare cases measles virus (MV) induces subacute sclerosing panencephalitis (SSPE) or measles inclusion body encephalitis (MIBE), two lethal diseases of the human central nervous system. MV transcripts present in the brains of two SSPE patients and one MIBE patient were analyzed by quantitative Northern blots. In all three cases the transcripts from the first MV gene were relatively abundant, amounting to about one-tenth of that in lytically infected cells. However, the quantity of transcripts decreased sharply for each subsequent MV gene, arriving at 200-fold lower levels for the fifth MV gene. In comparison gradients of transcript levels are more shallow in either lytically or persistently infected cultured cells, where the transcripts of the fifth MV gene are only about five times less abundant than those of the first. These altered ratios of mRNAs appear to be typical for persistent MV brain infections and most likely lead to reduced expression of the viral envelope proteins, encoded by distal MV genes, at the surface of brain cells. This could account for the lack of viral budding and allow persistent MV infections to elude immune surveillance.
Brain↗
Replication strategy of human hepatitis B virus.
To study the replication strategy of the human hepatitis B virus, the 5' end of the RNA pregenome and the initiation sites of DNA plus and minus strands have been mapped. The RNA pregenome was found to be terminally redundant by 120 nucleotides; it is initiated within the pre-C region and may also function as mRNA for synthesis of the major core protein and the hepatitis B virus reverse transcriptase. The hepatitis B virus DNA minus strand is initiated within the direct repeat sequence DR1, it contains a terminal redundancy of up to eight nucleotides, and its synthesis does not require any template switch. The DNA plus strand is primed by a short oligoribonucleotide probably derived from the 5' end of the RNA pregenome, and its synthesis is initiated close to the direct repeat sequence DR2. For its elongation to pass the discontinuity in the DNA minus strand an intramolecular template switch occurs using the terminal redundancy of this template. Thus, the route of reverse transcription and DNA replication of hepatitis B viruses is fundamentally different from that of retroviruses.
Animals↗
Kaposi's sarcoma in a young man in absence of AIDS or other known causes of immune suppression.
Kaposi's Sarcoma (KS) in young individuals is unusual and most often associated with cellular immunodeficiency caused by infective or other neoplastic diseases. It has recently been highly associated with the Acquired Immunodeficiency Syndrome (AIDS). We report the case of a heterosexual 29 year aged man with no evidence of underlying malignancy or infectious diseases. Antibodies to the Human Immunodeficiency Virus (HIV) were absent on repeat testing. His immunological profile demonstrated elevated number of CD8+ cells, normal number of CD3+ and CD4+ cells and hypogammaglobulinemia. These data are distinctly different from those described with AIDS associated KS. The development of KS in young individuals of mediterranean origin may reflect mild degree of immune abnormalities in the absence of infection with HIV.
Adult↗
[Immunological follow-up of 95 HIV seropositive drug abusers in the bresciana area].
We evaluated 95 HIV seropositive drug-addicts during a follow-up period of one year. The patients were classified and reclassified at each visit, according to the criteria proposed by the Centers for Disease Control (CDC) in 1986. At the first visit the patients were classified as follows: 70 in IIa-IIIa, 23 in IIb-IIIb and 2 in group IV. a and b indicate the absence or the presence of immunological and/or hematological alterations. 1 out of 70 IIa-IIIa patients versus 8 out of 23 IIb-IIIb patients developed AIDS in one year (p less than 0.001). Only one patient belonging to IIa-IIIa groups at the first visit progressed to AIDS in one year. This patient was reclassified in subgroup b after six months. No differences were noted among patients classified in IIb (5 patients) and IIIb (4 patients) who progressed to AIDS. Taken together these data indicate that the belonging to b subgroup is a risk factor for developing AIDS and the passage through a b subgroup (II or III) is a necessary step before the appearance of clinical manifestations of AIDS.
Acquired Immunodeficiency Syndrome↗
Accumulated measles virus mutations in a case of subacute sclerosing panencephalitis: interrupted matrix protein reading frame and transcription alteration.
Subacute sclerosing panencephalitis (SSPE) is a fatal disease affecting the human central nervous system several years after acute measles infection. Measles virus (MV) genomes replicating in SSPE brains do not give rise to budding particles and present various defects in gene expression, mostly concerning the matrix (M) protein. For one SSPE case (K), shown previously to be devoid of M protein expression, we examined here in detail the features involved in this defect. In the brain of patient K the normal, monocistronic MV M mRNA was completely substituted by a bicistronic RNA containing the coding sequence of the preceding phosphoprotein (P) gene in addition to the M coding sequence. Analysis of the P-M intercistronic region by direct cDNA sequencing showed that the consensus sequence at this RNA processing site was unaltered but revealed several distant point mutations. cDNA cloning and sequencing of the entire M coding region established that one of the point mutations leads to a stop codon at triplet 12 of the M reading frame. It is unknown whether this defect, explaining by itself the lack of M protein, is related also to the block of M mRNA formation. In addition we note that as much as 1% of the nucleotides differed between two overlapping clones from the same brain. This high sequence variability could possibly account for the diversity of defects observed in MV gene expression in SSPE brains and may be a general phenomenon associated with RNA virus persistence.
Amino Acid Sequence↗
Expression of defective measles virus genes in brain tissues of patients with subacute sclerosing panencephalitis.
The persistence of measles virus in selected areas of the brains of four patients with subacute sclerosing panencephalitis (SSPE) was characterized by immunohistological and biochemical techniques. The five measles virus structural proteins were never simultaneously detectable in any of the brain sections. Nucleocapsid proteins and phosphoproteins were found in every diseased brain area, whereas hemagglutinin protein was detected in two cases, fusion protein was detected in three cases, and matrix protein was detected in only one case. Also, it could be shown that the amounts of measles virus RNA in the brains differed from patient to patient and in the different regions investigated. In all patients, plus-strand RNAs specific for these five viral genes could be detected. However, the amounts of fusion and hemagglutinin mRNAs were low compared with the amounts in lytically infected cells. The presence of particular measles virus RNAs in SSPE-infected brains did not always correlate with mRNA activity. In in vitro translations, the matrix protein was produced in only one case, and the hemagglutinin protein was produced in none. These results indicate that measles virus persistence in SSPE is correlated with different defects of several genes which probably prevent assembly of viral particles in SSPE-infected brain tissue.
Adolescent↗