Rapid Prototyping of Slot Die Devices for Roll to Roll Production of EL Fibers.

There is a growing interest in fibers supporting optoelectrical properties for textile and wearable display applications. Solution-processed electroluminescent (EL) material systems can be continuously deposited onto fiber or yarn substrates in a roll-to-roll process, making it easy to scale manufacturing. It is important to have precise control over layer deposition to achieve uniform and reliable light emission from these EL fibers.

Passivated aluminum nanohole arrays for label-free biosensing applications.

We report the fabrication and performance of a surface plasmon resonance aluminum nanohole array refractometric biosensor. An aluminum surface passivation treatment based on oxygen plasma is developed in order to circumvent the undesired effects of oxidation and corrosion usually found in aluminum-based biosensors. Immersion tests in deionized water and device simulations are used to evaluate the effectiveness of the passivation process.

Reversal of metabolic block in glycolysis by enzyme replacement in triosephosphate isomerase-deficient cells.

Inherited deficiency of the housekeeping enzyme triosephosphate isomerase (TPI) is the most severe clinical disorder of glycolysis. Homozygotes manifest congenital hemolytic anemia and progressive neuromuscular impairment, which in most cases pursues an inexorable course with fatal outcome in early childhood. No effective therapy is available.

Porphyrins in urine, plasma, erythrocytes, bile and faeces in a case of congenital erythropoietic porphyria (Gunther's disease) treated with blood transfusion and iron chelation: lack of benefit from oral charcoal.

Congenital erythropoietic porphyria is a rare genetic disorder in which deficiency of uroporphyrinogen III synthase results in excessive production of Type I porphyrins. The main clinical features are severe photodestruction of the skin and haemolytic anaemia. Treatment consists of shielding from light, blood transfusions and splenectomy, but is generally unsatisfactory.

Metabolic correction of triose phosphate isomerase deficiency in vitro by complementation.

Inherited deficiency of triose phosphate isomerase (TPI), the enzyme that catalyses the interconversion of dihydroxyacetone phosphate (DHAP) and glyceraldehyde-3-phosphate, is characterised by an accumulation of intracellular DHAP and markedly reduced enzyme activity in cells and tissues, resulting in progressive, usually fatal neuromuscular dysfunction. Since specific enzyme replacement for TPI deficiency is not currently available, the secretion and recapture of the missing enzyme was investigated in a co-culture model comprising K562 human erythroleukaemia cells and lymphoblastoid cells taken from a TPI deficient patient. A sevenfold reduction in intracellular DHAP with concomitant increase in intracellular TPI activity from 7.

Evidence for founder effect of the Glu104Asp substitution and identification of new mutations in triosephosphate isomerase deficiency.

Triosephosphate isomerase (TPI) deficiency is an autosomal recessive disorder of glycolysis characterized by multisystem disease and lethality in early childhood. Among seven unrelated Northern European kindreds with clinical TPI deficiency studied, a single missense mutation at codon 104 (GAG;Glu-->GAC;Asp) predominated, accounting for 11/14 (79%) mutant alleles. In three families molecular analysis revealed compound heterozygosity for Glu104Asp and novel missense mutations.

Two novel mutations in the reduced nicotinamide adenine dinucleotide (NADH)-cytochrome b5 reductase gene of a patient with generalized type, hereditary methemoglobinemia.

Hereditary methemoglobinemia due to reduced nicotinamide adenine dinucleotide (NADH) cytochrome b5 reductase (b5R) deficiency is classified into two types, an erythrocyte (type I) and a generalized (type II). We investigated the b5R gene of a patient with type II from a white United Kingdom (UK) family and found that the patient was a compound heterozygote for two novel mutations. The first mutation was a C-to-A transversion changing codon 42 (TAC: Tyr) to a stop codon in the one allele.

Tucaresol increases oxygen affinity and reduces haemolysis in subjects with sickle cell anaemia.

The primary pathophysiological event in sickling is the intracellular polymerization of deoxygenated haemoglobin S. Tucaresol (589C80;4[2-formyl-3-hydroxyphenoxymethyl] benzoic acid), a substituted benzaldehyde, was designed to interact with haemoglobin to increase oxygen affinity and has been shown to inhibit sickling in vitro. We administered tucaresol to sickle cell patients in the steady state to examine the anti-sickling effect in vivo.

Prenatal diagnosis of triosephosphate isomerase deficiency.

First-trimester prenatal diagnosis was undertaken by chorionic villus DNA analysis in two unrelated families with the inherited glycolytic disorder triosephosphate isomerase (TPI) deficiency. The propositus in each family was shown to be homozygous for a missense mutation (GAG --> GAC) at codon 104 of the TPI gene. In the first case the fetus was heterozygous for the codon 104 mutation and therefore clinically unaffected.

Emergence of ciprofloxacin resistance during treatment of Salmonella osteomyelitis in three patients with sickle cell disease.

The treatment of salmonella osteomyelitis in sickle cell disease (SCD) is difficult and the emergence of antibiotic resistance in Salmonella spp presents further problems for clinicians treating SCD. Three patients presented with salmonella bacteraemia. Treatment with several intravenous antibiotics did not prevent the subsequent development of osteomyelitis.

Hereditary red cell enzymopathies.

The hereditary red cell enzymopathies are an uncommon but important cause of chronic haemolytic anaemia. Their clinical diversity is mirrored by increasingly evident heterogeneity at the molecular level. The structure, function, and expression of the genes encoding red cell enzymes and the nature of the gene defects in the deficient state are examined.

Salmonella bacteraemia in sickle cell disease at King's College Hospital: 1976-1991.

A review of all blood culture isolates for the 16 years from 1976 were collated with prospective laboratory and clinical records of 620 sickle cell patients treated at King's College Hospital. Over half of all salmonella bacteraemias diagnosed in the clinical laboratory occurred in sickle cell disease (SCD) patients. Of 21 bacteraemias in SCD patients, 11 (52.

Pathology.

In summary, the College is a long way forward in the preparation for the implementation of Calman and the unified training grade. It has identified a number of problems that could hinder its implementation and is anxious to develop, along with its sister Colleges, a unified approach to these problems to ensure that the doctors of tomorrow have an effective and satisfying training experience for the benefit of all the patients for whom we care.

Screening for ophthalmic manifestations of sickle cell disease in the United Kingdom.

There are marked variations in the manifestations of sickle disease in different populations. The ocular complications of this condition amongst the Afro-Caribbeans living in the United Kingdom have not previously been reported. We present the preliminary results of an ophthalmic screening programme at King's College Hospital, London.

Current problems in haematology. 2: Hereditary spherocytosis.

Hereditary spherocytosis is a relatively common haematological disorder and will be encountered by all haematologists. The abundance of new information, dealing principally with molecular and genetic aspects of pathophysiology, is beginning to have implications for its investigation and management. While these advances have not yet exerted a large influence at therapeutic level, the promise of such advents as prenatal diagnosis make this an exciting field to watch.

Anticardiolipin antibodies in patients from Malaysia with systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) is highly prevalent in Malaysia, which has a mixed population of Malays, Chinese, and Indians. A quantitative enzyme linked immunosorbent assay (ELISA) was used to determine anticardiolipin antibody (aCL) levels (total immunoglobulin, IgG, and IgM) in 200 patients with SLE (164 Chinese, 26 Malay, and 10 Indian) attending the University Hospital of Kuala Lumpur, Malaysia, and 103 matched controls. Only 33 (16.

The osteodystrophy of congenital erythropoietic porphyria.

Congenital erythropoietic porphyria (CEP) is a rare disorder of heme biosynthesis that results in the production of large quantities of photoactive porphyrins. The clinical syndrome is dominated by extreme photosensitivity with mutilation of light exposed extremities and hemolytic anemia. Bone disease has been occasionally noted, but is not well characterised.

Neonatal screening for haemoglobin variants using filter paper-dried blood specimens.

Neonatal screening for haemoglobinopathies utilizing cord blood samples is well established, although it has a high miss rate and has the inherent problem of possible misdiagnosis from maternal contamination of the sample. The use of dried Guthrie card samples which are taken at six days of age avoids these problems and has the advantage of using an established system of sample collection. Controversy exists as to the method of choice for analysis of dried samples, this study of 2406 samples found that Iso-electric focusing (IEF) analysis of dried specimens gives excellent correlation when compared with cellulose acetate/citrate agar electrophoresis of liquid cord blood samples.

Clinicopathological features of acute undifferentiated leukaemia with a stem cell phenotype.

Over a 4 1/2-year period, 141 patients with acute leukaemia had morphologic, immunophenotypic and cytochemical studies performed at King's College Hospital. Seven cases were noted to have blast cells which did not express myeloid or lymphoid antigens or cytochemical staining indicative of differentiation but were HLA DR and CD 34 positive. Based on these criteria we have used the term stem cell acute leukaemia to denote these patients.

A logical approach to the investigation of red cell enzymopathies.

This relatively rare group of disorders may cause quite marked morbidity and occasionally be life-threatening. As their inheritance is largely known accurate information in one family member has obvious benefits to other family members as well as the patient. The identification of the defect is dependent on an accurate clinical story which can be used to guide both the use and the interpretation of the various laboratory tests available.