Effects of hedgehog proteins on tissue engineering of cartilage in vitro.

The effects of three derivatives of the N-terminal signaling domain of hedgehog proteins on cartilage engineered in vitro were investigated, with specific focus on the ability to increase tissue growth rate and concentrations of major extracellular matrix components, that is, glycosaminoglycans (GAG) and collagen, and on the effects on morphological appearance of the tissue. Bovine articular chondrocytes were cultured on biodegradable polyglycolic acid (PGA) scaffolds with or without the addition of dipalmitoylated sonic hedgehog (dp-shh), dipalmitoylated indian hedgehog (dp-ihh), or sonic hedgehog dimer (shh-dimer) to medium with either 1% or 10% fetal bovine serum (FBS). All three hedgehog proteins dose-dependently increased construct weights (by up to 1.

Nutritional implications of chronic dyspepsia in childhood.

Chronic dyspepsia is common in children. However, the association of individual predominant symptoms with gastric emptying and their impact on nutritional status are poorly defined. We therefore studied 31 children (mean age 11 years, 14 boys) with chronic dyspepsia and classified their predominant symptoms (PS) by their character and severity.

Sexual maturation of Greek boys.

Background: Sexual maturation is dependent on genetic and environmental factors, therefore each ethnic group has to have its own normative data.

Aim: This study describes the sexual maturation of Greek boys.

Subjects And Methods: We examined cross-sectionally 1266 healthy boys, aged 8-16 years.

A new stop codon mutation (Y52X) in the myophosphorylase gene in a Greek patient with McArdle's disease.

We identified a novel stop codon mutation in the myophosphorylase gene in a Greek patient with typical symptoms of McArdle's disease. This is the first genetic study of myophosphorylase deficiency in a Greek family, showing that the proband was a compound heterozygous for the common "caucasian" mutation (R49X) and a new nonsense mutation (Y52X), both within exon 1. The new point mutation, a C-to-G transversion at codon 52, converts an encoded tyrosine to a stop codon.

A novel missense adenine nucleotide translocator-1 gene mutation in a Greek adPEO family.

Autosomal dominant progressive external ophthalmoplegia (adPEO) is caused by mutations in at least three different genes: ANT1 (chromosome 4q34-35), TWINKLE, and POLG. The ANT1 gene encodes the adenine nucleotide translocator-1 (ANT1). We identified a heterozygous T293C mutation of the ANT1 gene in a Greek family with adPEO.

Clinical features of parkinsonian patients with the alpha-synuclein (G209A) mutation.

The motor and neuropsychological abnormalities in eight Greek patients with Parkinson's disease (PD) carrying the alpha-synuclein gene mutation (G209A) were studied. These patients (five men, three women) belonged to six different families. Their symptoms started between 32-50 years of age (mean +/- SD, 39.

The effects of Tween 20 and sucrose on the stability of anti-L-selectin during lyophilization and reconstitution.

We have chosen an anti-L-selectin antibody as a model protein to investigate the effects of sucrose and/or Tween 20 on protein stability during lyophilization and reconstitution. Native anti-L-selectin secondary structure is substantially retained during lyophilization in the presence of sucrose (1 or 0.125%).

Retrospective study of a large population of patients affected with mitochondrial disorders: clinical, morphological and molecular genetic evaluation.

Mitochondrial disorders are human genetic diseases with extremely variable clinical and genetic features. To better define them, we made a genotype-phenotype correlation in a series of 207 affected patients, and we examined most of them with six laboratory examinations (serum CK and basal lactate levels, EMG, cardiac and EEG studies, neuroradiology). We found that, depending on the genetic abnormality, hyperckemia occurs most often with either chronic progressive external ophthalmoplegia (CPEO) and ptosis or with limb weakness.

Sex differences in the secular changes in pubertal maturation.

Objective: It is a common experience among pediatricians in the industrialized countries that many girls-but many fewer boys-present with secondary sex characteristics at a younger age than normal. This study examines whether there are sex differences in the secular changes toward earlier pubertal maturation.

Methods: The author collected the growth data from various studies performed in Greece in the 20th century (from 1928 to 1995) and estimated the age at peak annual height increment in each study by calculating mean increase in height in each successive year.

The anabolic steroid oxandrolone increases muscle mass in prepubertal boys with constitutional delay of growth.

The aim of this study was to investigate the effect of oxandrolone on body composition in boys with constitutional delay of growth and puberty. In 14 prepubertal boys, height, weight, triceps and subscapular skinfolds and upper arm circumference were measured. Body mass index, the ratio of subscapular to triceps skinfolds and the upper muscle area were also determined.

A new homogeneous enzyme immunoassay for thyroxine using glycogen phosphorylase b-thyroxine conjugates.

Background: [corrected] Measurement of serum thyroxine (T(4)) concentration is important for diagnosis of thyroid gland diseases. We developed a practical homogeneous enzyme immunoassay for thyroxine analysis in unextracted sera.

Methods: A thyroxine derivative conjugated to a reactive sulfhydryl group of glycogen phosphorylase b (GPb).

Hypercalciuria in children with febrile convulsions.

Background: The purpose of the present study was to investigate whether idiopathic hypercalciuria may be implicated in the pathogenesis of febrile convulsions.

Methods: We studied 38 children (22 boys) with febrile convulsions (mean (+/- SD) age 3.25 +/- 1.

Clinical phenotype in patients with alpha-synuclein Parkinson's disease living in Greece in comparison with patients with sporadic Parkinson's disease.

Objective: An Ala53Thr mutation of the alpha-synuclein gene has been recently identified as a rare cause of autosomal Parkinson's disease (PD). The clinical characteristics of 15 patients with PD living in Greece with the Ala53Thr alpha-synuclein mutation (alpha-synPD) were compared with patients with sporadic Parkinson's disease (sPD).

Methods: An investigator, blind to the results of the genetic analysis, examined 15 patients with alpha-synPD and 52 consecutive patients with sPD.

Spinal cord compression due to extramedullary haematopoiesis in two patients with thalassaemia: complete regression with blood transfusion therapy.

Extramedullary haematopoiesis, a common finding in thalassaemia, is rarely localized in the spinal cord and even more rarely has neurological manifestations. We present two patients suffering from thalassaemia (intermedia and beta homozygous) and paraparesis due to spinal cord compression from intrathoracic extramedullary haematopoietic masses. Diagnosis was based on magnetic resonance imaging findings, and treatment consisted of blood hypertransfusions.

Platysma muscle turnover flap correction of tracheostomy scarring deformity.

There are numerous techniques for the reconstruction of the tracheostomy scarring deformity that give acceptable results. In this paper, we present a new method for improving this defect, using two rectangular turnover platysma muscle flaps to fill the concavity. The skin is then sutured over them.

Homogeneous enzyme immunoassay for triiodothyronine in serum.

Background: The concentration of triiodothyronine (T3) in human serum is extremely low and can be determined only by very sensitive methods. We developed a homogeneous enzyme immunoassay for T3 analysis in unextracted serum.

Methods: A T(3) derivative was conjugated to the -SH groups of glycogen phosphorylase b (GPb) from rabbit muscle.

Stiff-person like syndrome in a patient with multiple pituitary hormone deficiencies.

A patient with long-standing, occult pituitary insufficiency, who developed painful muscle stiffness and superimposed spasms, closely resembling stiff-person syndrome, was described. Complete resolution of neuromuscular symptoms with hormone replacement in this case, as well as in a previously reported one, led to the suggestion that a syndrome like stiff-person could represent a rare consequence of multiple pituitary hormone deficiencies.

Plasma immunoreactive endothelin levels in children with cystic fibrosis.

Unlabelled: Plasma immunoreactive endothelin levels were determined in 31 children and adolescents with cystic fibrosis and it was examined whether these levels correlated with the severity of the disease. The study comprised 16 cystic fibrosis patients (mean (SD) age 13.0 (4.

Atrial natriuretic peptide in children with idiopathic hypercalciuria.

We measured plasma atrial natriuretic peptide (ANP) levels in 30 children with idiopathic hypercalciuria (IH) and 19 normal controls (NC). A calcium (Ca) loading test was performed in all patients to determine the type of IH. Subsequently plasma ANP, cAMP and renin activity (PRA), serum total and ionized Ca, intact parathyroid hormone, aldosterone, and 1,25-dihydroxyvitamin D as well as urine Ca, cAMP, and electrolytes were determined in all subjects.

"Checkerboard" assessments of periodontal microbiota and serum antibody responses: a case-control study.

Background: We explored the association between subgingival microbial profiles and serum IgG responses to periodontal microbiota in relation to clinical periodontal status.

Methods: One hundred thirty-one (131) periodontitis patients aged 29 to 74 years (mean 51.8) were age- and gender-matched with 74 periodontally intact controls (range 26 to 77, mean 49.

Mitochondrial neurogastrointestinal encephalomyopathy: an autosomal recessive disorder due to thymidine phosphorylase mutations.

Mitochondrial neurogastrointestinal encephalomyopathy (MNGIE) is an autosomal recessive disorder defined clinically by severe gastrointestinal dysmotility; cachexia; ptosis, ophthalmoparesis, or both; peripheral neuropathy; leukoencephalopathy; and mitochondrial abnormalities. The disease is caused by mutations in the thymidine phosphorylase (TP) gene. TP protein catalyzes phosphorolysis of thymidine to thymine and deoxyribose 1-phosphate.

Recombinant antibody toxins specific for ErbB2 and EGF receptor inhibit the in vitro growth of human head and neck cancer cells and cause rapid tumor regression in vivo.

Overexpression of the ErbB2 and epidermal growth factor receptor (EGFR) tyrosine kinases is frequently observed in squamous cell carcinomas of the head and neck, and has been correlated with shorter overall survival. By immunoblot analysis, we have found EGFR and ErbB2 expression in 6 out of 6 established head and neck cancer cell lines. Elevated EGFR protein levels were noted in 3 and elevated ErbB2 levels in 5 of them.

Preserved negative patterning and impaired spatial learning in pigeons (Columba livia) with lesions of the hippocampus.

Pigeons (Columba livia) with bilateral electrolytic lesions of the hippocampus and area parahippocampalis were compared with control pigeons on 2 tasks: negative patterning and delayed spatial alternation. Negative patterning demands configural stimulus representations for its successful solution. The only effect of hippocampal lesions on this task was an increased response rate to the rewarded stimuli.