Dental malocclusion is associated with reduced systemic bone mineral density in adolescents.

There is no published data about associations between the state of dentition and bone mass in adolescents. The objective of this study was to investigate whether the prevalence of caries and dental malocclusion is associated with bone mass during growth. In 123 healthy Caucasian subjects (72 males, 51 females) aged 14-18 yr, DMFT figures (decayed teeth, missing teeth, filled teeth) and presence of malocclusion, according to Angle classification, were determined. Participants completed a questionnaire regarding dental hygiene, physical activity level, and consumption of sweets. Anthropometry and pubertal stages were examined. Bone mineral density (BMD) was examined using dual energy X-ray absorptiometry (DXA) in the total body, head, and lumbar spine. No association was found between DMFT (mean+/-SD: 8.33+/-3.9) and BMD or Z-scores for BMD. Malocclusion was found in 49 subjects (39.8%) and was more prevalent in females than males. Malocclusion was associated with lower total BMD independently of body size (p=0.001; Z-scores: -0.21+/-0.27 vs +0.33+/-0.17; p=0.1) in males (but not females), producing odds ratio 1.6 (95% confidence interval: 1.09-2.34%; p=0.02). Head BMD was also lower in the males with malocclusion than in those without (p=0.004). Neither caries nor the tooth loss appear to be associated with BMD during growth. Boys with malocclusion are at higher risk of reduced BMD. This suggests that inadequate bone mass accrual in males coexists with impaired growth of the masticatory system in childhood and adolescence, however, the causal pathway is unknown. Factors that produce malocclusion may also affect bone mass or size but further prospective studies are needed to evaluate the relationship.     

Impact of activated protein C resistance on general vascular surgical patients

Purpose: The prevalence of activated protein C resistance (APCR) and associated thrombotic morbidity among patients who undergo arterial reconstruction were investigated.Methods: Preoperative assays for functional APCR and factor V (Leiden) mutation were performed on 262 patients who underwent arterial reconstructions that consisted of cerebrovascular surgery (109), aortic or iliofemoral procedures (76), or infrainguinal bypass procedures (77). Patients were monitored for thrombotic complications during the postoperative period.Results: Depending on the stringency of the definition used, functional APCR was detected in 10.6% to 22.0% of patients tested. Factor V (Leiden) was found in 5.3% of patients. Thrombotic morbidity consisting of myocardial infarction, cerebrovascular event, or graft thrombosis occurred in 9.9% of patients, who were followed-up for a mean of 4.8 months. No significant overall correlations were found between APCR and thrombotic morbidity. Subgroup analysis revealed significant associations between functional APCR and total early postoperative thrombotic complications and early graft failure, and between factor V (Leiden) and early cerebrovascular events and late graft thrombosis (p < 0.03).Conclusions: Functional APCR is somewhat more prevalent among general vascular surgical patients than in the general population, but factor V (Leiden) is no more prevalent. APCR is not a prominent cause of thrombotic morbidity in contemporary vascular surgery. Nonetheless, it is a sufficiently important potential contributor to morbidity among some subgroups to warrant selective testing and directed therapy pending further study. (J Vasc Surg 1997;25:1054-60.)     

Raymond's syndrome following petrosal sinus sampling

Summary Inferior petrosal sinus sampling (IPSS) is used to evaluate the pituitary-dependency of Cushing's disease, and to predict the laterality of a microadenoma prior to transsphenoidal operation. A serious complication occured in a 14-year-old boy: he suffered brain stem ischaemia with abducens nerve palsy and hemiparesis (Raymond's syndrome). The case prompted us to reconsider the indication for this investigation.     

Syntactic language processing: ERP lesion data on the role of the basal ganglia.

The role of the basal ganglia in syntactic language processing was investigated with event-related brain potentials in fourteen neurologically impaired patients. Seven of these patients had basal ganglia lesions while 7 other patients primarily had lesions of the left temporo-parietal region excluding the basal ganglia. All patients listened to sentences that were either correct or included a verb argument structure violation. In previous experiments this type of violation elicited a biphasic pattern of an N400-P600 complex in young healthy participants. While the N400 may result from incorrect semantic-thematic role assignment, the P600 reflects the fact that verb information does not license the syntactic structure at present. Results of the patient experiment revealed a double dissociation: patients with left temporo-parietal lesions only show a P600, whereas patients with lesions of the basal ganglia showed no P600, but a negativity with extended duration that resembled an N400. The latter pattern not only confirms previous reports that the basal ganglia modulate the P600 but extends these results by showing that the N400 as a late semantic-thematic integration process appears partially modulated by the basal ganglia.     

Psychological and family functioning and quality of life in adolescents with cystic fibrosis.

BACKGROUND: The life expectancy of individuals with CF has increased to 33 years. Thus, issues such as quality of life and psychological well-being, previously thought to be of lesser importance than physical well-being, are now recognised as significant factors. This study examined the interrelationships between quality of life, family functioning, individual psychopathology and optimism of adolescents with CF. METHODS: Adolescents attending the CF clinic completed a number of questionnaires. Quality of Life was measured using the Cystic Fibrosis Questionnaire, family functioning by the Family Environment Scale (3rd edition), general psychopathology with the Symptom Checklist-90-Revised and optimism for the future by the Hunter Opinions and Personal Expectations Scale. Disease severity was assessed using the Shwachman score and spirometry at the time of questionnaire completion. RESULTS: The level of psychopathology (12.5% of those 13 years and over) in the group was lower than that reported for young people in Australia (15-20%). The results indicated that young people with a delayed diagnosis and those who are alienated from their families may be in need of additional psychosocial support. The group was hopeful and positive about their future and these attributes were independent of clinical measures of disease severity. In general, these young people scored relatively highly on the quality of life scale. For example the mean standardised score for physical functioning was 70 points, for respiratory symptoms was 63 points and for emotional state was 78 points. Increased levels of psychopathology and lack of hope for the future were however associated with lower ratings on a number of quality of life measures. Family cohesiveness, expressiveness and organization were associated with better psychological functioning in the young people. CONCLUSIONS: Adolescents with CF appear to be a psychologically well functioning and well-adjusted group. These findings support the importance of a more sophisticated model of well-being for adolescents with CF, which explores the young person's views on their quality of life and wider support frameworks rather than relying solely on measures of physical health to gauge well-being.     

CADASIL or CADVaSIL?

In the present study, morphological examination of patients from two unrelated Polish families with CADASIL was performed. Using light microscopy, there were evident changes characteristic to the disease. On electron microscopy, deposits of granular osmiophillic material (GOM) were found not only in cerebral arteries and veins but also in cerebral capillaries and vessels of the internal organs. These findings indicate that pathological process in CADASIL is generalized and involves also small vessels devoid of smooth muscle cells. Therefore, we propose to consider a replacement for the name CADASIL that better reflects the morphological picture of the disease like, for example, cerebral autosomal dominant vasculopathy with subcortical infarcts and leukoencephalopathy (CADVaSIL) or, to preserve the commonly known acronym, cerebral autosomal dominant angiopathy with subcortical infarcts and leukoencephalopathy.     

CAG repeat polymorphism within the KCNN3 gene is a significant contributor to susceptibility to anorexia nervosa: a case-control study of female patients and several ethnic groups in the Israeli Jewish population.

The human small-conductance Ca(2+)-activated potassium channel gene KCNN3 has been involved in mechanisms underlying neuronal function and plasticity. A multiallelic CAG repeat polymorphism within the KCNN3 has been associated with schizophrenia and bipolar disorder. We have previously reported in a family-based study that longer CAG repeats are preferentially transmitted to patients with anorexia nervosa (AN). The present study extends the analysis of KCNN3 allele distribution to a larger series of AN female patients and control groups, incorporating information on ethnicity and co-morbidities associated with AN. The data analysis is presented while considering separately the two alleles of each individual, namely a minor (shorter) and a major (longer) allele. This study has found that the KCNN3 allele distribution in the general Israeli population does not differ significantly in at least four Jewish ethnic groups of Ashkenazi, North African, Iraqi, and Yemenite origin. These have been used as control groups in a matched case-control analysis that has demonstrated a significant over-representation of KCNN3 alleles with longer CAG repeats among AN patients (P < 0.001 for the major allele and P = 0.035 for allele sum). Under dichotomization, a significantly higher prevalence of the L allele (>19 repeats) has been observed among AN patients (P < 0.001). While considering AN and co-morbid phenotypes, a tendency towards longer (L) alleles has been observed in the subset of patients with obsessive-compulsive disorder (OCD) co-morbidity. These findings further implicate KCNN3 as a significant contributor to predisposition to AN.     

Microorganisms isolated from clinical specimens of patients from the Department of Thoracic Surgery

The clinical specimens received from patients hospitalized in Department of Thoracic Surgery between 1997 and 2001 were microbiologically examined. The main specimen for microbiological examination was pleural fluid (median 34%). The frequency of specimens from bronchial tree increased significantly (from 4% to 26%) with concurrent decrease of sputum (from 29% to 6%). Among isolated pathogens, Gram negative rods were the most frequent (median 48%) and Pseudomonas sp. was the main pathogen among them. Occurrence of staphylococci was median 22% and Staphylococcus aureus, with a little decrease in analyzed period, was still the main Gram positive pathogen. Simultaneously the occurrence of MRSA in the last three years dropped three times. The number of isolations of yeasts have risen from 5.8% to 10.3%.