Skeletal dysplasia presenting as a neuromuscular disorder - report of three children

Three pediatric patients were investigated because of suspected muscle disorder. They were clumsy with an awkward looking waddling gait and had increasing muscle weakness and pain in the legs. Serum CK-values, electroneuromyography (ENMG) and muscle biopsy were all normal. A post-traumatic X-ray of the ankle of one of them showed epiphyseal changes and his condition was diagnosed as Camurati-Engelmann disease. Because of similarities in the clinical presentation of these boys, bone changes were looked for in the two other patients and a diagnosis of multiple epiphyseal dysplasia was made. Skeletal dysplasia should be considered as a diagnostic alternative when a child presents with an unexplained muscle weakness accompanied with pain in the limbs. Specific treatment for bone dysplasias can alleviate symptoms and prevent fractures.     

Consumption of sugar products and associated life- and school-satisfaction and self-esteem factors among schoolchildren in Kuwait

OBJECTIVE: The objective of this study was to assess how frequently schoolchildren report consuming sweets, soft drinks, and cakes, and whether life- and school-satisfaction and self-esteem factors are associated with the consumption of these sugar products. MATERIAL AND METHODS: A total of 2,312 schoolchildren between the ages of 11 and 13 years from the government schools in Kuwait completed an anonymous structured questionnaire during 2002 and 2003. A representative sample of children from all six governorates of the country was drawn into the study. The questionnaire of the Health Behaviour in School-Aged Children (HBSC) survey was translated from English to Arabic and was used after modification to suit Kuwaitis. The chi-square test and logistic regression model were used in the analysis. RESULTS: A large proportion of children reported consuming sweets (42%), soft drinks (43%), and cakes (31%) several times a day. Almost every fourth child reported consuming all these sugar products more than once a day. All life-satisfaction and self-esteem variables and almost all school-satisfaction variables seemed to associate with more-than-once-a-day consumption of sugar products. When all the associated variables were analyzed together using the logistic regression model, the life- and school-satisfaction and self-esteem factors seemed to have a stronger association with frequent sugar consumption than did gender, grade, or nationality. CONCLUSIONS: Consumption of sugar products was common among schoolchildren in Kuwait, and both positive and negative life-satisfaction and self-esteem factors were associated.     

Comparison of additional versus no additional heparin during therapeutic oral anticoagulation in patients undergoing percutaneous coronary intervention

Uninterrupted oral anticoagulation (OAC) therapy can be the preferred strategy in patients with atrial fibrillation at moderate to high risk of thromboembolism undergoing percutaneous coronary intervention (PCI). To evaluate the need for additional heparins in addition to therapeutic peri-PCI OAC, we assessed bleeding complications and major adverse cardiac and cerebrovascular events in 414 consecutive patients undergoing PCI during therapeutic (international normalized ratio 2 to 3.5) periprocedural OAC. Patients were divided into those with no (n = 196) and with (n = 218) additional use of periprocedural heparins. No differences in major adverse cardiac and cerebrovascular events (4.1% vs 3.2%, p = 0.79) or major bleeding (1.0% vs 3.7%, p = 0.11) were detected, but access site complications (5.1% vs 11.0%, p = 0.032) were less frequent in those without additional heparins. When adjusted for propensity score, patients with additional heparins had a higher risk of access site complications (odds ratio 2.6, 95% confidence interval 1.1 to 6.1, p = 0.022) without any increased risk of any other adverse event. Analysis of 1-to-1 propensity-matched pairs showed a significantly higher risk of access site complication in patients receiving additional AC (13.1% vs 5.7%, p = 0.049). In conclusion, therapeutic warfarin treatment seems to provide sufficient AC for PCI. Additional heparins are not needed and may increase access site complications.     

Caries experience in the primary dentition of Tanzanian and Finnish 3–7-year-old children

Caries experience of primary teeth in relation to age, sex, ethnic group, socioeconomic status and frequency of consuming sweet snacks and drinks was studied among groups of Tanzanian and Finnish 3-7-yr-olds, a total of 1118 children. In all age groups the Tanzanian children had higher mean dmft scores than Finnish children did. In the Finnish group the mean dmft score was higher at age 7 than at age 3, while in the Tanzanian group no statistically significant difference in dmft scores was found between age groups. In both groups maxillary incisors and molars were the main teeth affected by caries. Frequent consumption of sweet snacks and drinks increased the risk for caries in Tanzanian but not in Finnish children. High socioeconomic status decreased the risk for caries in Finnish children but among Tanzanian children high SES was not associated significantly with the risk of developing caries. According to these results, caries experience in Tanzanian urban nursery school children was significantly higher than in Finnish children of the same age, the differences being especially high among the youngest age groups.     

Occlusion among a group of Tanzanian urban schoolchildren

Occlusion and its variations were studied in a group of primary schoolchildren (n = 642, age 11-18 yr) in Dar es Salaam. Most children (96%) had Angle's Class I occlusion. Distal and mesial bite were rare in the sample, representing 3% and 1%, respectively. Crowding was found to be the most common dental disorder (16%) and the frequency of moderate and severe crowding decreased with age. No clear differences in occlusion were found between boys and girls. The results of this study show smaller occlusal variation compared with Caucasian children, and also some differences compared with previous African studies.     

Smoking and peripheral type of cancer are related to high levels of pulmonary cytochrome P450IA in lung cancer patients.

A specific member of the cytochrome P450 superfamily of enzymes, designated P450IA (including 2 isozymes, P450IA1 and P450IA2), which is involved in the metabolic activation of polycyclic aromatic hydrocarbons and aromatic amines, was studied in lung tissue from 25 lung cancer patients by immunohistochemistry. The pulmonary activity of a P450IA1-dependent enzyme, aryl hydrocarbon hydroxylase (AHH), from the same patients was also measured. Cytochrome P450IA was localized principally in the peripheral airways in alveolar epithelium of types I and II and in ciliated columnar and cuboidal bronchiolar epithelium. The amount of P450IA in the bronchial wall was minimal and was localized mainly in the capillary endothelium and the epithelium of the bronchial glands. Smoking was the most important factor related to the presence of P450IA and the AHH activity in lung tissue. None of the 10 ex-smokers, but all except I of the current smokers had detectable level of P450IA. The localization of the cancer was also correlated with the presence of cytochrome P450IA. Peripheral lung tissue stained positively in all patients with a peripheral adenocarcinoma who currently smoked (8/8) but in less than half of those with a bronchial cancer who were smokers (3/7). Our data suggest that the smokers who have an inducible cytochrome P450IA are especially at increased risk of developing lung cancer of the peripheral adenocarcinomatous type.     

Factors behind oral health behaviour

In this report the background factors of four oral health habitsof young adults are analysed. Toothbrushing, consumption ofsweets, participation in fluoride rinsings and choosing xylitolchewing gum have been examined in relation to knowledge, values,beliefs, attitudes, life situation, socioeconomic status andpersonality factors. All 18 year olds (n = 1190) living in Pori,Finland, were sent a questionnaire in 1985. The response ratewas 85%. Almost all of the background factors were associatedwith the habits of toothbrushing and choosing xylitol chewinggum. Only the belief in the possibility of retaining one's ownteeth throughout life was associated with consumption of sweets.Participation in fluoride rinsings was associated with positiveattitudes towards oral health education lessons, life situation,current school performance and knowledge. In the multiple regressionmodel (P < 0.001) the background variables explained only18% of the variation in frequencies of toothbrushing and stillless of the variation in the other health habits. All four healthhabits were adopted by 16% of the young adults and in only 4%of cases were none of these habits at the desired level. Whenall four health habits were included in a summary variable offavourable behaviours, this variable was associated with mostof the background factors. However, only 12% of this summary variable was explained bythe background factors studied.     

The Copper Induced Modification of Duodenal Biotransformation Reactions in Rats During Fat Deficiency

Abstract The effect of ingested copper on the composition of duodenal postmitochondrial supernatnat fraction (PMS), the aryl hydrocarbon hydroxylase (AHH) and UDPglucuronosyltransferase (GT) activities was analyzed in rats given normal or fat free diets. There were only minor changes in the composition of PMS or in the activities of the measured enzymes when rats received a normal diet. During fat deficiency the phospholipid-protein ratio and the GT activity were markedly increased following 10 mM copper concentration in the drinking water. It is concluded that during fat deficiency copper modifies the structure of the microsomes, as analyzed in the PMS, and that this is reflected in the membrane bound GT activity.