Echocardiographic diagnosis of supravalvular aortic stenosis

The echocardiographic features of supravalvular aortic stenosis seen in a patient with valvular aortic and multiple pulmonary branch stenosis are described. Since patterns of valvular, discrete subvalvular, and supravalvular aortic stenosis can be recognized by echocardiography, it is now possible to screen close relatives of patients having supravalvular aortic stenosis and pulmonary branch stenosis by this non-invasive technic.     

Familial supravalvular aortic stenosis. Report of a large family and review of the literature

Familial supravalvular aortic stenosis has been recognized as a distinct syndrome. A large family with five proven cases and a review of the literature on familial supravalvular aortic stenosis are presented. The diagnosis was substantiated in all 63 cases by cardiac catheterization, surgery, or postmortem examination.     

Mycotic aneurysm of the innominate artery with supravalvular aortic stenosis

A case of mycotic innominate artery aneurysm in association with supravalvular aortic stenosis has been presented with successful resection as the first such case documented. Blood flow was restored with low cervical autogenous internal jugular vein, as an end-to-side vein graft from the left common carotid and end-to-end to the right subclavian and right common carotid arteries. The future repair of the supravalvular aortic stenosis could then be more easily accomplished.     

Surgical repair for supravalvular aortic stenosis: intermediate to long-term follow-up

The prevalence of different malocclusion features was investigated in 396 6-year-old Icelandic children, using the epidemiologic registration method described by Bj?rk et al. in 1964. Girls were ahead of boys with regard to dental stage (P < 0.01). One or more permanent teeth were congenitally missing in 5% of the children. Postnormal occlusion was found in 27% of the boys and in 31% of the girls, and prenormal occlusion was found in 6% and 5%, respectively. Straight terminal plane at the second deciduous molars was found in individuals with either normal or postnormal occlusion. Thus, it can be misleading to use the relation of the terminal planes as a measurement of the sagittal relation between the jaws. The prevalence of hypodontia was much lower than has been reported previously for Icelandic children.     

Concurrence of supravalvular aortic stenosis and peripheral pulmonary stenosis in three generations of a family: a form of arterial dysplasia

Isolated supravalvular aortic stenosis (SVAS) commonly is an autosomal dominant trait; it may also occur in the Williams syndrome (WS). While peripheral pulmonary stenosis (PPS) can occur in the same individual with familial isolated SVAS, concurrence of these lesions in different relatives of a family is uncommon. We describe five affected individuals in one family; three had isolated SVAS, one had isolated PPS, and one had SVAS and PPS. Based on this family and review of literature, we suggest that SVAS is a form of arterial dysplasia encompassing PPS in its spectrum. It is developmentally distinct from other left heart obstructive lesions that are hypothesized to be related to blood flow abnormalities in the developing embryo. We also conclude that the clinical disorder in this family represents one that is distinct from WS.     

Laryngotracheal stenosis in children after intubation. Report of five cases

In this study we present a modified diagnostic routine PCR (polymerase chain reaction) technique for the detection of specific enteroviral nucleic acid sequences in human body fluids, the use of which would reduce the number of false-positive results, the costs and the concentration of reagents required in PCR. Cerebrospinal samples, pharyngeal swabs and faeces were tested. To this end, general primers, selected in the highly conserved 5' non-coding region were used, with a closed-tube RT semi-nested PCR protocol, and the PCR product was analysed using a hybridization in solid phase. A total of 32 patients with suspected enteroviral infection, 17 with suspected viral meningitis and 15 with a possible acute enteroviral infection different from meningitis were analysed, and 80% of the patients with possible acute enteroviral infection were PCR-positive. A broad range of enteroviruses can be detected and false-positive results can be reduced. The availability of results in less than 12 h and the lack of need for radiolabelled probes also increase the convenience of this protocol.     

Magnetic resonance features of the enigmatic oligodendroglioma

RATIONALE AND OBJECTIVES: The authors characterize magnetic resonance (MR) features of oligodendroglioma that permit diagnosis. These features may be used to guide MR stereotactic biopsies. METHODS: Fifteen patients with mixed oligodendroglioma and four with pure oligodendroglioma were studied on a 1.5-tesla MR unit with contrast and also by computed tomography (CT). All patients had their diagnosis confirmed by light and electron microscopy, and by immunohistochemical staining. RESULTS: The most characteristic finding was a honeycomb tumor matrix (4 of 19) in oligodendroglioma. The most common finding was an amorphous pattern (12 of 19) and the least common was a large cystic pattern (3 of 19). Contrast enhancement most commonly was minimal and dot-like or lacy in appearance (11 of 19), or absent (5 of 19). Calcification was present in the majority (11 of 19). CONCLUSIONS: The honeycomb and stippled amorphous patterns reflect the cytoarchitecture of oligodendroglioma and were the most characteristic MR features. The amorphous pattern was the most commonly encountered pattern. There was a wider range of imaging findings on MR compared with CT; the flocculent calcification on CT was typical, but it was not always detected on MR. Including these characteristic areas that appear along the path of the stereotactic biopsy may ensure a correct diagnosis of oligodendroglioma because it most often is seen with another tumor, usually of the astrocytic family. Identifying the oligodendrogliomatous portion of the tumor will influence the therapy. No differences could be found in the MR appearance or biologic behavior of pure versus mixed oligodendrogliomas.     

Clinical features and treatment of joint dislocations in Larsen's syndrome. Report of three cases in one family

Of 3 cases of Larsen's syndrome in one family, one had bilateral dislocation of the hip and the knee joints. The knee joint was not reduced by manipulation and corrective cast, while both hip and knee joints were simultaneously reduced by skeletal traction of the tibia at the age of 4 months, and the course was satisfactory. Reduction of joint dislocations should be attempted by a conservative method such as skeletal traction as early as possible. Various radiographic skeletal abnormalities occurred in all 3 cases. Larsen's syndrome very likely has a genetic origin.     

Subglottic tracheal stenosis in Wegener''s granulomatosis. Report of 3 cases]

OBJECTIVE: This study was performed to assess whether coadministration with grapefruit juice significantly affects the pharmacokinetics of amlodipine, a dihydropyridine class calcium antagonist with slow absorption, distribution and low plasma clearance. The primary objective was to evaluate whether short exposure to grapefruit juice could affect the metabolism of amlodipine to an extent similar to that previously demonstrated for other dihydropyridines (e.g. felodipine, nisoldipine, nitrendipine). METHODS: Twelve healthy male volunteers followed a randomised, open crossover study design, comparing the effect of a single oral dose of amlodipine (5 mg) taken together with a glass of grapefruit juice (250 ml) vs water. Blood samples to determine plasma concentration were taken and blood pressure (BP) and heart rate (HR) were measured throughout the study. RESULTS: When amlodipine was coadministered with grapefruit juice, Cmax was 115% and AUC(0-72 h) was 116% compared with water, but tmax was not significantly changed. There were no significant differences in BP and HR between the two treatments. A small decrease in diastolic BP, however, was observed in both treatments 4-8 h after drug administration, coinciding with Cmax, but this was normalised after 12 h. The BP reduction seen was compensated by a slight increase in HR, which remained throughout the study. CONCLUSION: An interaction between grapefruit juice and amlodipine was demonstrated. The haemodynamic data showed that a dose of 5 mg was sufficient to achieve a BP reduction in healthy subjects, but the increase in amlodipine plasma concentration seen after intake of grapefruit juice was too small to significantly affect BP or HR. The clinical significance of this food/drug interaction, however, cannot be ignored since there is considerable variation between individuals and a more extensive intake of grapefruit juice might give more pronounced effects.     

Magnetic resonance imaging in 80 cases of orbital tumors

Magnetic resonance imaging (MRI) is a new medical imaging technique which has been used in evaluating ocular and orbital diseases in recent years. Owing to the high resolution of soft tissues and lesions, it is superior to ultrasound and computed tomography in the localization and characterization of an orbital tumor in diagnosis. In this paper, we analysed 80 cases of MR images of orbital tumors by which all the lesions were detected. Benign or malignant disorders were recognized in 59 cases and complete tumors with accurate localization were shown in 79 cases. The principles of MRI, signal intensity of various tumors and the indications of MRI were discussed.     

Magnetic resonance imaging features of submitral left ventricular aneurysm

Rotational atherectomy can generate heat from the friction of the burr as it ablates atherosclerotic plaque. The objective of this study is to correlate Rotablator technique and heat generation using two experimental models. First, 2.0 mm burrs were advanced through a lesion model derived from bovine bone implanted with thermal probes. Intermittent ablation with minimal decelerations resulted in a smaller temperature increase than continuous ablation with maximal decelerations (2.6 +/- 1.3 vs. 13.9 +/- 1.0 degrees C, respectively, P < 0.01). The second model used porcine femoral arteries cradled in constricting polyethylene grafts with thermal probes in contact with the adventitia. As the burr advanced through the segment, RPM decreases of 5-7 k resulted in a temperature rise of 4.1 +/- 1.2 degrees C, whereas decelerations of 10-20 k resulted in a 11.3 +/- 6.2 degrees C temperature increase. We conclude that excessive drops in speed and aggressive advancement of the burr are related to significant increases in temperature and potential thermal injury.     

Computerised axial tomography of abdominal aortic aneurysm. Report of 5 cases

Purple sulfur bacterium Thiocapsa roseopersicina strain BBS requiring vitamin B12 may grow in the dark in media containing no other organic compounds. Under such conditions the cells oxidize sulfide and thiosulfate with the use of O2 and assimilate carbon dioxide. After 10--30s assimilation of NaH14CO3 about 60% of radioactivity is found in phosphorylated compounds characteristic for the reductive pentose phosphate cycle. The possibility of the function of this cycle in the dark in the presence of O2 is confirmed by the capacity of cells grown under such conditions to synthesize ribulose-1,5-diphosphate carboxylase. All this evidence suggests the ability of T. roseopersicina to change from phototrophy to aerobic chemolithoautotrophy.