Oral health status in a group of children and adolescents with myotonic dystrophy type 1 over a 4-year period

Aim.  The aim of this longitudinal study was to evaluate changes in oral health, orofacial function, and dental care in children with myotonic dystrophy type 1 (DM1) in comparison with a control group.
Methods.  Thirty-six DM1 patients and 33 control patients out of originally 37 in each group were examined on two occasions about 4 years apart. Caries, plaque, and gingivitis were registered, mouth opening capacity assessed and the ability to cooperate in dental treatment estimated. Questionnaires concerning different aspects of oral health and care, symptoms of temporomandibular dysfunction (TMD), and dental trauma were also used.
Results.  The DM1-patients, in particular the boys, had significantly more caries, plaque, and gingivitis than the control patients on both occasions and the increase in decayed missing or filled permanent teeth (DMFT) and surfaces (DMFS) was significantly larger. They received more dental care and had lower cooperation ability. Mouth opening capacity and increase of it was significantly lower and symptoms of TMD were significantly more frequent.
Conclusions.  DM1 patients, as they grow older, have increasing amounts of plaque and risk of caries and gingivitis. They have more TMD problems. Behaviour management problems do not seem to decrease with age. Increased prophylactic care is essential for DM1 patients.     

Abrupt withdrawal of isosorbide-5-mononitrate in Durules(R) (Imdur(R)) after long term treatment in patients with stable angina pectoris

In a single-blind study of 6 weeks' duration, 32 patients withstable angina pectoris, who hadbeen receiving controlled-release,Durules®, isosorbide-5-monoitrate (Imdur®) 60 to 180mg daily for at least 1 year, were assessed after abrupt withdrawalof the nitrate. After 2 weeks of placebo treatment nitrate therapywas re-instituted, and the patients followed for antoher 2 weeks.The possibility of developemnt of tolerance and rebound phenomenawas also investigated. Three patietns experienced severe anginal symptoms necessitatinghospitalization when controlled-release isosorbide-5-monoitratewas withdrawn abruptly. Patients complained of more severe anginalsymptoms during the placebo period, experienced more frequentanginal attacks and used more glyceryl trinitrate tablets thanduring active treatment. ST segment changes during exercisewere more pronounced with placebo. After controlled-releaseisosorbide-5-mononitrate was re-introduced, these variablesindicated significant improvement. On the other hand, no deteriorationoccurred in exercise performance during the placebo phase. Responsivenessto glyceryl trinitrate was maintained, as shown by comoparisonsof exercise tests performed after the long term treatment andduring the placebo phase. Controlled-release isosorbide-5-mononitrate retains a beneficialeffect in patients with angina pectoris during prolonged use,although some attenuation of the effect is seen. Abrupt withdrawarlof the drug is not recommended because of the possibility ofsevere exacerbation of anginal symptoms, although no clearcutrebound phenomena were seen.     

Biplane transoesophageal echocardiography, transthoracic Doppler, and magnetic resonance imaging in the assesment of coarctation of the aorta

This study compared flow-sensitive magnetic resonance imagingwith biplane transoesophageal echocardiography in combinationwith continuous wave Doppler from the suprasternal notch inpatients with native coarctation or after surgical repair. Twenty patients (mean age 33 years, range 17–60) wereinvestigated, of whom 15 had undergone surgery at mean age 13years, range 5.43. Peak and mean flow in the ascending and descendingaorta as well as coarctation peak velocity were determined withthe magnetic resonance imaging phase contrast technique. Coarctationpeak velocity was also measured by Doppler from the jugulum.Magnetic resonance imaging axial sections as well as biplanetransoesophageal echocardiography were used to measure the smallestdiameter of the constricted segment. Sixteen healthy volunteers,mean age 36 years, range 22.63, provided reference values formagnetic resonance imaging determined volume of flow in theaorta. Peak flow in the descending aorta was 9.2 ±3.71.min ^{– 1} (reference 130 ± 2.5, P<0.01) and meanflow 3.1 ±0.9 I. min^{– 1} (reference 3.4 ±0.8,P>0.05). The ratio of descending-to-ascending peak flow was0.54 ±0.17 (reference 0.69 ± 0.10, P<0.01)and mean flow 0.68 ± 0.15 (reference 0.69 ± 0.08,P>0.05). The coarctation velocity was slightly higher withDoppler than with magnetic resonance imaging (+ 0.24 ±0.44 m. s^{– 1}, 95% confidence interval +0.45 to + 0.02m.s^{– 1}, P= 0.05). The coarctation diameter was slightlylarger with magnetic resonance imaging than with transoesophagealechocardiography (1.4 ±3.5 mm, 95% confidence interval+ 3.1 to – 0.3 mm, P= 0.11). Both methods are suitable for the assessment and follow-up ofcoarctation of the aorta Flow assessment with magnetic resonanceimaging provides a hitherto unavailable measure with which toassess the severity of obstruction.     

A Solid Phase Enzyme-linked Immunosorbent Assay (ELISA) for the Demonstration of Antibodies against Denatured, Single-stranded DNA in Patient Sera

A solid phase enzyme-linked-immunosorbent assay (ELISA) for the determination of antibodies against denatured, single-stranded (ss-) DNA is described. Polystyrene cuvettes coated with ss-DNA were incubated with serum samples and the anti-ss-DNA antibodies bound were detected by means of an anti-IgG-alkaline phosphatase conjugate. The binding of anti-ss-DNA antibodies in individual sera was expressed as units calculated as % of the absorbance in relation to the absorbance value obtained with a reference pool. Absorption experiments showed that the assay is specific for antibodies against denatured DNA. By using immunologically purified anti-ss-DNA antibodies the assay was shown to detect specific antibodies in concentrations down to 1 ng/ml. Antibodies against DNA could be detected in 94% of sera with antinuclear antibodies.     

Ultrasound imaging of the masseter muscle in myotonic dystrophy patients

summary Ultrasound technique was applied to measure the thickness and examine the internal structure of the masseter muscle in a group of 16 adult patients (nine women and seven men) with myotonic dystrophy (MyD) and 16 healthy individuals matched in age, sex and number of occluding teeth. The masseter thickness was measured bilaterally under both relaxed conditions and during maximal clenching. The error of measurement was found to be small, not exceeding 0.45 mm. The imaging characteristics of the masseter in most of the MyD patients was an obvious atrophy of the muscle with increased echointensity of the intramuscular tissue and loss of the internal structure concerning tendons and fasciae. The mean masseter thickness (±SD) in the MyD group was 10.4 (±2.2) mm under relaxed conditions and 11.1 (±2.4) mm during maximal clenching, compared with 13.3 (±2.2) mm and 14.1 (±2.4) mm, respectively, in the healthy group (P < 0.001). In conclusion, our results indicate that, in most of the myotonic dystrophy patients, the masseter muscle is atrophic with obvious signs of degeneration. Ultrasound is a useful method for both qualitative and quantitative evaluation of the condition of the masseter muscle.     

Anti-anginal Efficacy of a Controlled-Release Formulation of lsosorbide-5-mononitrate Once Daily in Angina Patients on Chronic β-Blockade

ABSTRACT The anti-anginal effect of a controlled-release (Durules®) formulation of isosorbide-5-mononitrate (5-ISMN) 60 mg, Imdur®, once daily was evaluated in a randomised double-blind, placebo-controlled, crossover study with a placebo run-in period. Each period lasted for 2 weeks. A total of 70 patients (58 men and 12 women) with stable exertional angina pectoris on β-blockade, mean age 59 years (range 39–71), were included. Exercise testing was performed on a bicycle ergometer 3 hours after the dose at the end of each period. Anginal attacks and intake of sublingual nitroglycerin tablets were noted. Imdurr? in combination with a β-blocker significantly increased the total exercise capacity, the time and total work until the onset of chest pain and at 1 mm ST-depression compared with β-blockade alone. The attack rate and the nitroglycerin consumption were significantly decreased. Headache was the only significant side-effect. In conclusion, the addition of Imdur® once daily to β-blockade significantly increased the anti-anginal effect.