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Biomedical subjects

P Weller

Publications and source records attributed to P Weller.

At least 37 records · Page 2Linked to original sources
PubMed1994

Cloning of PBDX, an MIC2-related gene that spans the pseudoautosomal boundary on chromosome Xp.

The pseudoautosomal boundaries are the interface between pseudoautosomal and sex chromosome-specific DNA sequences. We have isolated a gene, PBDX, from the human pseudoautosomal boundary region of Xp. The three exons at the 5' end of PBDX are situated in the pseudoautosomal region immediately downstream of MIC2, whereas the other seven exons are in the X-specific region. Hence, PBDX is inherited in two modes: its 5' end is pseudoautosomally inherited and its 3' end is X-linked. The predicted amino acid sequence of the 540 bp coding region is 48% homologous to 12E7, the product of MIC2. By virtue of its position, PBDX becomes an excellent candidate for the XG blood group gene.

12E7 Antigen↗
PubMed1994

Changes in plasma norepinephrine concentration and thrombocyte alpha 2-adrenoceptor density during long-term antihypertensive therapy with nitrendipine and captopril.

Antihypertensive drugs influence the sympathetic nervous system in different ways that may cause adverse or beneficial effects. We treated 48 hypertensive patients with either nitrendipine (10-20 mg twice daily, b.i.d.) or captopril (25-50 mg b.i.d.) for 16 weeks to evaluate changes in plasma catecholamines, platelet alpha 2- and lymphocyte beta 2-adrenoceptors. Blood pressure (BP) decreased from 153/95 to 135/87 mm Hg with captopril and from 155/99 to 137/89 mm Hg with nitrendipine. Treatment with nitrendipine significantly stimulated plasma norepinephrine (NE) from 327 +/- 37 to 446 +/- 50 pg/ml, and treatment with captopril resulted in a significant reduction in platelet alpha 2-adrenoceptor density from 265 +/- 39 to 171 +/- 26 fmol/mg protein. Despite having equal BP-lowering properties, captopril and nitrendipine have different effects on the sympathetic nervous system. Stimulation of plasma NE during long-term treatment with nitrendipine may contribute to possible adverse effects, whereas reduction in alpha 2-adrenoceptors induced by captopril may contribute to the vasodilating effect of angiotensin-converting enzyme (ACE) inhibition.

Adult↗
PubMed1994

The chest radiograph in cystic fibrosis: a new scoring system compared with the Chrispin-Norman and Brasfield scores.

BACKGROUND: Scoring systems for the chest radiograph in cystic fibrosis are used to compare patients and different treatment regimens, and to monitor respiratory disease in individual patients. The Northern chest radiograph score was designed to allow one person to assess the radiological features of lung involvement in cystic fibrosis in as simple, rapid and equally reproducible manner as the established Chrispin and Norman, and Brasfield scoring systems. METHODS: Forty five chest radiographs were scored by 10 physicians with a special interest in cystic fibrosis according to the Brasfield and Northern methods, and by five pairs of physicians according to the Chrispin-Norman criteria. Three individuals and two pairs rescored the radiographs after an interval of 3-5 months. The Northern score was performed with and without a lateral view, using the original posteroanterior radiograph. RESULTS: The Northern score showed a better agreement between observers for the ranking of the radiographs. It was equally well related to respiratory function tests, the Shwachman-Kulczycki score of overall clinical status, and in its discrimination between different radiographs as the other two scoring systems. The Northern score performed equally well with or without a lateral film. CONCLUSIONS: The Northern system fulfils the requirements of a chest radiograph score more successfully than the Chrispin-Norman or Brasfield systems, and does not require a lateral film.

Cystic Fibrosis↗
PubMed1994

Plasma catecholamines and adrenoceptors in young hypertensive patients.

An elevated sympathoadrenal tone and an imbalance in postsynaptic alpha- and beta-adrenoceptor function are discussed as factors in the pathogenesis of essential hypertension. This study examined plasma catecholamines, thrombocyte alpha2-adrenoceptors and lymphocyte beta2-adrenoceptors in 16 young patients with newly detected essential hypertension and 26 normotensive age matched controls (27.1 +/- 4.5 vs. 24.8 +/- 2.8 years; NS). Plasma noradrenaline (276 +/- 34 vs. 216 +/- 18 pg/ml, P < 0.05) and plasma adrenaline (96 +/- 15 vs. 31 +/- 4 pg/ml, P < 0.0001) were significantly elevated in hypertensive patients. Thrombocyte alpha2-adrenoceptor density was only nonsignificantly decreased (230 +/- 37 vs. 311 +/- 36 fmol/mg protein, NS), whereas lymphocyte beta2-adrenoceptor density was markedly reduced (15.3 +/- 2.3 vs. 22.6 +/- 1.8 fmol/mg protein, P < 0.01) in hypertensive patients. Elevated plasma catecholamines are consistent with a pathophysiological role for increased sympathetic neural activity in young hypertensive patients. Assuming that results of adrenoceptor studies on blood elements are applicable on vascular receptors, our results are consistent with an imbalance of postsynaptic adrenoceptor functions which promotes the pressor effects of the sympathetic system.

Adult↗
PubMed1993

[Pulmonary valve endocarditis and atrial fibrillation].

A 61-year-old man became ill with a fever of 39.4 degrees C, decreased exercise tolerance and headache as well as chest pain. Physical examination 3 weeks after the onset of symptoms merely revealed irregular heart rate at 100 beats/min. Erythrocyte sedimentation rate was increased (30/61 mm), as were serum bilirubin, lactate dehydrogenase, alkaline phosphatase, gamma-GT and C-reactive protein. The ECG showed atrial fibrillation with a rapid and irregular ventricular rate, as well as ventricular extrasystoles (Lown type IIIA), there were no abnormal findings on either the chest radiography or transthoracic echocardiography. Antiarrhythmic treatment brought about atrial flutter with 4:1 a-v conduction. Transoesophageal echocardiography now revealed vegetation on the pulmonary valve and microthrombi in the left atrial appendage. Ten days after starting intravenous penicillin G (10 mega units four times daily), gentamycin (60 mg three times daily) and heparin (30,000 units over 24 h) sinus rhythm was restored, the vegetation had got smaller and no thrombi were demonstrated. After 27 days antibiotic treatment was changed to oral penicillin V. After 4 weeks the patient was discharged symptom-free.

Atrial Fibrillation↗
PubMed1993

PUVA, UVB, psoriasis, and nonmelanoma skin cancer.

This article reviews the English-language literature on the risk of nonmelanoma skin cancer from phototherapy (UVB) or photochemotherapy (PUVA) in the treatment of psoriasis. The evolution of the debate on the carcinogenic potential of these treatment is discussed, particularly with reference to PUVA. Available data indicate that therapeutic UVB has a low risk of producing cutaneous cancers, with the possible exception of those on male genital skin. However, there is a definite cutaneous carcinogenic risk from oral PUVA. The major associated risk factors are reviewed. We provide recommendations for the assessment and treatment of patients to minimize this risk.

Dose-Response Relationship, Drug↗
PubMed1993

Ocular complications of PUVA therapy.

PUVA, the combination of psoralen and long wave ultraviolet radiation is widely used in the management of psoriasis, vitiligo and several other dermatological disorders. The potential for long term treatment to cause ocular damage remains to be determined and despite the large numbers of patients who have received PUVA treatment, development of cataract is exceedingly rare. This paper discusses cataract formation, reviews the literature concerning the ocular complications of PUVA therapy and proposes guidelines for ocular protection during photochemotherapy.

Cataract↗
PubMed1991

[Pleural fibrosis as a side effect of years-long methysergide therapy].

A 58-year-old man was admitted to hospital because of exertional dyspnoea and a cardiac murmur not previously heard. For one year he was known to have left-sided fibrinous pleuritis. For 5 years he had been taking methysergide for cluster headaches. From 1950-1980 he had worked with asbestos-containing insulating material. Erythrocyte sedimentation rate was greatly increased to 117/136 mm and there was an hypochromic anaemia (Hb 10.4 g/dl). The cholestasis enzymes were elevated (gamma-GT 88 U/l; alkaline phosphatase 511 U/l). Computed tomography of the thorax demonstrated left-sided pleural thickening of up to 3 cm. Endocarditis was excluded (sterile blood culture; normal echocardiogram). There was no evidence of an infectious, immunological or malignant cause for the pleural fibrosis. One year after pleurectomy and having discontinued methysergide all biochemical tests were normal.

Biopsy↗
PubMed1991

Human junctional epithelium: demonstration of a new marker, its growth in vitro and characterization by lectin reactivity and keratin expression.

We have studied lectin reactivity in normal human junctional, sulcular, and attached gingival epithelia with 15 lectins and identified the epithelia by parallel staining with monoclonal anti-keratin antibodies. Dolichos biflorus agglutinin reacted uniquely with junctional epithelium, not staining other gingival cells of non-blood group A1 donors. We have demonstrated that the moiety recognized in junctional epithelium is not blood group A1 antigen or Tn antigen. Using a panning technique with this lectin to isolate the cells, we have grown keratinocytes from human junctional epithelium, and compared their phenotype in vitro to that of cells grown from the sulcular and attached gingival epithelium. Colonies established from each epithelial type were examined in frozen section with the anti-keratin antibodies. All expressed keratin 14 (keratinocyte marker), keratins 4 and 13 (suprabasal non-cornification markers), and keratins 7, 18, and 19 (simple epithelia keratins). Keratins 1, 10, and 8 were not expressed. Vimentin, the intermediate filament of mesenchymal cells, was also expressed by all types of cells in culture. Thus we have shown that when cells from the three areas of the gingiva were grown in culture they revert to one phenotype, at least with respect to their keratin expression. These results support the hypothesis that the epithelial phenotype is influenced by the sub-epithelial mesenchyme, and it is this that is responsible for the unique phenotype of the junctional epithelium.

ABO Blood-Group System↗
PubMed1991

Neonatal screening for cystic fibrosis in Wales and the West Midlands: clinical assessment after five years of screening.

Screening of the newborn for cystic fibrosis by measurement of immunoreactive trypsin has been undertaken on alternate weeks in Wales and the West Midlands for five years since 1985 to evaluate the possible clinical benefits of early diagnosis. Patients detected by screening and those diagnosed by clinical symptoms alone were assessed annually for differences in clinical, anthropometric, and biochemical variables. Fifty eight infants not considered to be at risk of cystic fibrosis (they did not present with meconium ileus and do not have a sibling with cystic fibrosis) have been detected by screening and they have been compared with 44 children who were diagnosed clinically. This latter group includes nine children whose screening was negative but who were recognised subsequently to have cystic fibrosis. The mean age at diagnosis of the screened group was significantly lower than that of the group diagnosed clinically. Excluding admissions for diagnostic tests for cystic fibrosis, the screened group spent a significantly shorter time in hospital during the first year of life. The results of all other comparisons made between the screened group and those diagnosed clinically were similar up to the age of 4 years.

Body Height↗
PubMed1989

Nuclear factor I can functionally replace transcription factor Sp1 in a U2 small nuclear RNA gene enhancer.

Polymerase II transcription of a human gene for the small nuclear RNA U2 is dependent on two different promoter elements: a TATA-equivalent proximal sequence element and a distal enhancer element, which has been shown to contain Sp1- and octamer-binding sites. We have investigated the functional interplay between these transcription factor-binding sites of the enhancer, following transfection of U2 maxigene constructions into HeLa cells. There is a functional non-additive co-operation between the octamer-binding factor and Sp1, which is not dependent on the evolutionally conserved steric arrangement of these binding sites. We demonstrate that the conserved Sp1-binding site of the U2 enhancer can be fully substituted by a nuclear factor I (NFI) binding site, and that the octamer-binding factor functions in stimulating transcription in conjunction with either Sp1 or NFI. Since the octamer-binding factor is most probably the same protein as nuclear factor III (NFIII), the results imply that the NFI/NFIII complex, involved in adenovirus DNA replication, also can function as an efficient activator of transcription.

Base Sequence↗
PubMed1988

Transcription analysis of a human U4C gene: involvement of transcription factors novel to snRNA gene expression.

We have investigated the promoter requirements for in vivo transcription of a human U4C snRNA gene following transfection into HeLa cells. Two elements required for maximal U4C transcription were identified. The first, located upstream of -50, provides a basal level of transcription 2-3% of the full activity, and probably corresponds to the previously identified snRNA gene proximal element. The distal element, centered around -220, acts as a transcriptional enhancer and contains motifs for three previously recognized transcription factors: the octamer-binding protein, NF-A, which binds to motifs in the distal elements of other snRNA genes, and two factors not previously shown to be involved in snRNA gene transcription, cAMP response element binding protein (CREB) and AP-2. The octamer and putative AP-2 motifs are required for maximal transcription of the U4C gene. Specific binding of NF-A and CREB to the motifs in the distal element has been shown in vitro by DNase I and DMS methylation protection footprint competition analyses using HeLa nuclear extracts. The presence of a binding motif for the inducible factor CREB, together with the transcriptional requirement for the putative AP-2 motif, suggests a means by which expression of snRNA genes might be regulated.

DNA-Binding Proteins↗
PubMed1987

The sweat test.

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Cystic Fibrosis↗
PubMed1987

Ultrasound findings in children with cystic fibrosis.

The results of a prospective study using ultrasound to assess abdominal complications in 76 children with proven cystic fibrosis are reported. Fifty-six patients (74%) had normal liver ultrasound scans. The most striking abnormality was an irregular, inferior edge to the liver occurring in 85% of abnormal liver scans and in 70% there was corresponding clinical and biochemical evidence of abnormal liver function. This finding has been only briefly mentioned before. Increased reflectivity in abnormalities of the pancreas and gallbladder is also described.

Adolescent↗
PubMed1986

[Kasabach-Merritt syndrome].

The Kasabach-Merritt syndrome is a rare, congenital disease, characterized by giant haemangiomatosis and disseminated intravasal coagulation. It is reported on an at present 34-year-old patient with such a disease and the long-term course is described. Complications with haemorrhages can be prevented by means of anticoagulants.

Adult↗