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Angelman syndrome: difficulties in EEG pattern recognition and possible misinterpretations.

PURPOSE: This study aimed to evaluate the sensitivity of the EEG in Angelman syndrome (AS), to verify the age at onset of suggestive EEGs and to study EEG patterns, analyzing variations and comparing our findings with nomenclature previously used. METHODS: Seventy EEG and 15 V-EEGs of 26 patients were analyzed. Suggestive EEG patterns of AS were classified in delta pattern (DP), theta pattern (TP), and posterior discharges (PDs). Generic terms were used to simplify the analysis. RESULTS: Suggestive EEGs were observed in 25 (96.2%) patients. DP occurred in 22 patients with four variants-hypsarrhythmic-like: irregular, high-amplitude, generalized delta activity (DA) with multifocal epileptiform discharges (EDs); slow variant: regular, high-amplitude, generalized DA with rare EDs; ill-defined slow spike-and-wave: regular, high-amplitude, generalized DA with superimposed EDs characterizing a slow wave, with notched appearance; triphasic-like: rhythmic, moderate-amplitude DA over anterior regions with superimposed EDs. TP was observed in eight patients, as generalized or over the posterior regions. PDs were seen in 19 patients as runs of sharp waves or runs of high-amplitude slow waves with superimposed EDs. TP was the only age-related pattern (younger than 8 years) and observed only in patients with deletion. In 15 patients who had an EEG before the clinical diagnosis, 60% had a suggestive tracing. CONCLUSIONS: Although some EEG descriptions are not very detailed, and every author describes findings in a slightly different manner, obviously a common denominator must exist. In this context, EEG seems to be a very sensitive method for the diagnosis of AS, offering an opportunity to corroborate this etiologic diagnosis. Conversely, we do not believe that these patterns may be accounted as specific, except for the delta pattern, which seems to be extremely unusual in other syndromes. Other EEG patterns observed in AS, such as theta activity and PDs, occur in a wide variety of disorders. Nonetheless, their importance for the EEG diagnosis of AS is supported by the fact that they are associated with other features and may be helpful in a proper clinical setting.

Adolescent↗

Childhood verbal abuse and risk for personality disorders during adolescence and early adulthood.

Data from a community-based longitudinal study were used to investigate whether childhood verbal abuse increases risk for personality disorders (PDs) during adolescence and early adulthood. Psychiatric and psychosocial interviews were administered to a representative community sample of 793 mothers and their offspring from two New York State counties in 1975, 1983, 1985 to 1986, and 1991 to 1993, when the mean ages of the offspring were 5, 14, 16, and 22 years, respectively. Data regarding childhood abuse and neglect were obtained from the psychosocial interviews and from official New York State records. Offspring who experienced maternal verbal abuse during childhood were more than three times as likely as those who did not experience verbal abuse to have borderline, narcissistic, obsessive-compulsive, and paranoid PDs during adolescence or early adulthood. These associations remained significant after offspring temperament, childhood physical abuse, sexual abuse, neglect, physical punishment during childhood, parental education, parental psychopathology, and co-occurring psychiatric disorders were controlled statistically. In addition, youths who experienced childhood verbal abuse had elevated borderline, narcissistic, paranoid, schizoid, and schizotypal PD symptom levels during adolescence and early adulthood after the covariates were accounted for. These findings suggest that childhood verbal abuse may contribute to the development of some types of PDs, independent of offspring temperament, childhood physical abuse, sexual abuse, neglect, physical punishment during childhood, parental education, parental psychopathology, and co-occurring psychiatric disorders.

Adolescent↗

Arthroscopic suture tying: A comparison of knot types and suture materials.

PURPOSE: The purpose of this study was to determine the structural properties of 3 arthroscopically tied knots using 2 different suture materials: the French knot, the Duncan loop knot, and the original Revo knot. TYPE OF STUDY: Cohort analytic study. METHODS: The sutures used were No. 1 PDS II, an absorbent monofilament, and No. 1 Ethibond (Ethicon, Somerville, NJ), a braided nonabsorbent material. The resulting 6 suture-knot combinations were individually tested to failure in both open- and closed-loop configurations. RESULTS: The French knot showed the greatest strength compared with the Duncan loop and the Revo knot with both No. 1 Ethibond and No. 1 PDS II sutures (P <.05). The No. 1 Ethibond exhibited higher initial stiffness than the No. 1 PDS II for all 3 knot types (P <.05). Results were similar for both open and closed-loop configurations. Also, the French knot failed predominantly by suture breakage instead of knot slippage for both suture materials. CONCLUSIONS: The results of this study suggest that, among the suture and knot combinations investigated in this study, the arthroscopic repair of musculoskeletal injuries should be performed using the French knot and No. 1 Ethibond suture.

Arthroscopy↗

Pulsed Doppler sonography for the diagnosis of strangulation in small bowel obstruction.

BACKGROUND/PURPOSE: This study was undertaken to assess the usefulness of pulsed Doppler sonography (PDS) for the detection of strangulation in small bowel obstruction by evaluating the hemodynamics in the superior mesenteric artery (SMA). METHODS: The authors performed PDS in 117 normal children: 22 children aged 0 to 1 months (group I), 27 children aged 1 to 12 months (group II), 36 children aged 1 to 6 years (group III), and 32 children aged 7 to 15 years (group IV). Patients included 25 with simple obstruction: 1 in group II, 10 in group III, and 14 in group IV; and 9 with strangulating obstruction: 2 in group I, 2 in group II, 3 in group III, and 2 in group IV. The authors measured the peak-systolic velocity, end-diastolic velocity (EDV), and mean average velocity and calculated the resistive index (RI). RESULTS: The authors observed both a significant decrease in the EDV and increase in the RI for the SMA in strangulating obstruction compared with simple obstruction. CONCLUSION: Analysis of the hemodynamics in the SMA using PDS is useful to differentiate strangulating obstruction from simple obstruction.

Adolescent↗

Pulsed Doppler sonography of the hilar renal artery: differentiation of obstructive from nonobstructive hydronephrosis in children.

BACKGROUND/PURPOSE: It is difficult to detect the arcuate or cortical renal arteries when performing pulsed Doppler sonography (PDS) for congenital hydronephrosis. This study was undergone to assess the usefulness of PDS of the hilar renal artery to differentiate obstructive from nonobstructive hydronephrosis. METHODS: The authors performed PDS of the hilar renal artery in 80 normal children: 20 aged 0 to 1 months (group I), 20 aged 1 to 12 months (group II), 20 aged 1 to 6 years (group III), and 20 aged 7 to 15 years (group IV). Based on diuretic renography findings, 22 kidneys from 19 children with a ureteropelvic junction (UPJ) stricture were divided into 7 dilated obstructed and 15 dilated nonobstructed kidneys. The peak-systolic velocity (PSV), end-diastolic velocity (EDV), mean average velocity (Vm) and the resistive index (RI = [PSV - EDV]/PSV) were measured at the hilar renal artery. RESULTS: There was a significant difference in the RI of the hilar renal artery between obstructive and nonobstructive hydronephrosis. CONCLUSION: A pulsed Doppler evaluation of the hilar renal artery is useful for detecting an obstructive UPJ stricture compared with assessing arcuate or cortical renal arteries.

Adolescent↗

Comparison of different diluents and chromophores for spectrophotometric determination of livestock blood cholinesterase activity.

Effects of seven different blood diluents (distilled water, Triton X-100, saponin, isotonic saline solution, pH 7.5 and 8 phosphate buffers and bovine serum albumin) and two chromophores: 5, 5'-dithiobis 2-nitrobenzoic acid (DTNB) and 2,2'-dithiodipyridine (2- PDS) on blood cholinesterase determination in four domestic species (cow, sheep, goat and horse) are described and compared. Haemolytic diluents (distilled water, Triton X-100 and saponin) gave the best precision results when fresh blood was assayed. However, Triton X-100 induced lower ChE activity values in horses, and saponin yielded very high backgrounds in all species tested; so distilled water was recommended as diluent for fresh blood cholinesterase determination. In frozen samples all diluents (except Triton X-100) gave homogeneous final ChE results and showed good between-run precision. Use of 2- PDS as chromophore allowed to do kinetic measurements with approximately 1/3 less haemoglobin interference than when DTNB was employed. This fact allows the use of more concentrated whole blood samples, improving measurements accuracy and decreasing the possible reactivation of inhibited ChE. On the basis of these results, distilled water as diluent and 2- PDS as chromophore are recommended for ChE determination in whole blood.

2,2'-Dipyridyl↗

Effectiveness of operative treatment of internal orbital wall fracture with polydioxanone implant.

Many implants, some made from teflon or silicone, have been used for internal orbital wall reconstruction. Late complications relating to use of such implants have been reported. In this prospective study a polydioxanone (PDS) implant absorbable in vivo was used for internal orbital wall reconstruction. Follow-up involved clinical examination, magnetic resonance imaging (MRI) and computerized tomography (CT). Clinical examinations were undertaken before operation and up to 36 weeks postoperatively. Sixteen consecutive patients (10 pure blow-out fractures, six with associated zygomatic fracture) took part in the study. Prevalences of diplopia, proptosis and enophthalmus were recorded during each follow-up examination. This study revealed no muscle entrapment within the fracture line. Although CT results confirmed bone growth in the internal orbital wall, shape was unsatisfactory, and orbital volume was not reduced. MRI revealed thick scar formations in six cases (37.5%), fibrotic sinuses filled with air or gas in three cases (19%) and a fibrotic sinus with fluid around the PDS in one case (6%). Our results suggest that use of PDS in reconstructing the internal orbital wall is inadvisable.

Absorbable Implants↗

The regulation of adipose tissue pyruvate dehydrogenase activity of dietary fiber.

In vitro studies have established that insulin enhances the oxidation of pyruvate to acetyl CoA by the stimulation of mitochondrial pyruvate dehydrogenase (PDH) activity through plasma membrane binding response (Jarett and Seals 1979; Kiechle, Jarett, Dennis and Kotagal 1980). In the present study adipose tissue PDH activity was utilized as a marker for insulin responsiveness. The metabolic response of this enzyme to exogenous insulin was employed to test the hypothesis that dietary fiber enhances tissue responsiveness to insulin using adipose tissue from male weanling Sprague Dawley rats. Eight groups of rats (n = 5 per group) were fed ad libitum various diets containing different levels of cellulose and protein as already reported elsewhere (Ogunwole, Knight, Adkins, Thomaskutty and Pointer 1985). Percent insulin stimulation of PDH from basal activity (PDS) was utilized as an index of insulin responsiveness. Compared to all fiber treated groups, both basal (PDB) and insulin stimulated (PDI) activities were significantly lower (P less than 0.05) in the fiber free groups at both low (10%) and high (20%) protein levels. At all fiber levels tested (0, 5, 15 and 30%) protein intake resulted in a significant increase in both PDB and PDI. Gradual increase in cellulose intake resulted in a biphasic increase in PDS in both protein groups at the 5% and 30% fiber levels. PDS was higher (P less than 0.05) in the 10% protein groups than the 20% protein group at all fiber levels tested. A significant interaction effect of protein and fiber was observed on PDB (P less than 0.001) and PDI (P less than 0.04) when caloric intake was held constant as a covariate.(ABSTRACT TRUNCATED AT 250 WORDS)

Adipose Tissue↗

Sternal closure with resorbable synthetic suture material in children.

The use of resorbable sutures for sternal closure after median sternotomy in children has developed to a clinically routine procedure. Since there is no follow-up study so far about the influence of these synthetic sutures on sternal stability, wound healing, and compatibility in children, we evaluated the use of polydioxanon (PDS) cord in children particularly regarding those properties. In 59 children (weighing up to 30 kg) we could show that the use of synthetic resorbable materials (Vicryl 4/0, PDS) allows a complication-free stability of the sternum, good wound healing, and very good compatibility. It is concluded that the use of PDS cord is a suitable and reliable method for sternal closure with good clinical results.

Biocompatible Materials↗

[Suitability of resorbable sutures in the treatment of corneal wounds].

In a series of cataract operations with a corneal incision PDS 10-0 absorbable sutures were tested. In contrast to deeper PDS sutures, such as are used in corneoscleral incisions, the corneal sutures opened as early as between 13 and 30 days postoperatively in 5 out of 7 cases. Postoperative irritation was more severe and lasted longer than is usual when non absorbable sutures are used. This was probably caused by ends of the thread which had not yet been absorbed, resulting in a foreign body irritation of long duration. Altogether, absorbable PDS 10-0 sutures are only suitable for corneal wound closure to a limited extent.

Absorption↗

Power Doppler Sonography: Applications in Musculoskeletal Imaging.

Power Doppler sonography (PDS) is a recently developed technique that depicts moving blood volume within tissues. The enhanced sensitivity of PDS makes it possible to depict soft tissue vascularity, facilitating its role in the evaluation of musculoskeletal inflammatory diseases. A broad range of new and potential applications of PDS in the musculoskeletal system will be described including its use in the setting of bursal, tendon, articular, and muscle diseases, and in the evaluation of fluid collections and soft tissue masses.

Journal Article↗

Orbital floor reconstruction with flexible Ethisorb patches: a retrospective long-term follow-up study.

OBJECTIVE: The purpose of the study was to investigate whether a flexible, biodegradable material (Ethisorb) shows better long-term results with regard to diplopia, bulbus motility, and exophthalmos/enophthalmos compared to the use of lyophilized dura-patches and polydioxanone (PDS) foils. METHODS: During a period of 6 years 435 patients with an orbital fracture were investigated retrospectively. Inclusion criteria were patients with fractures of the orbital floor with a maximum size of 2 x 2 cm. Bulbus motility, exophthalmos, enophthalmos, and diplopia were investigated during a period of 2 years. RESULTS: One hundred twenty orbital floors were reconstructed by lyophilized dura-patches, 81 by PDS, and 136 by Ethisorb. An exploration without an implantation was performed in 91 patients. The long-term investigation 12 to 15 months after surgery showed an exophthalmos and enophthalmos incidence of 1%, whereas a reduced bulbus motility and diplopia were found in 5% and 4%, respectively. Fifteen to 24 months after surgery 2% of the patients had an exophthalmos and 1% had an enophthalmos. A reduction of bulbus motility was found in 4% of the patients, and diplopia was found in 3%. The use of Ethisorb resulted in a significantly lower incidence of exophthalmos 3 months after surgery compared to PDS. CONCLUSION: The low rate of acquired bulbus motility demonstrates acceptable results in using Ethisorb in the floor of the orbit.

Absorbable Implants↗

Polyglactin 910/polydioxanone bicomponent totally resorbable vascular prostheses.

Previous studies from our laboratory have shown that bioresorbable vascular prostheses woven from lactide-glycolide copolymers and implanted into arteries of several animal models become replaced by cellular tissues; the rate of replacement parallels the kinetics of prosthetic resorption. This study evaluates the efficacy of bicomponent resorbable prostheses as a method of augmenting resistance to dilatation during the resorption period of the more rapidly resorbed component. Bicomponent prostheses (n = 37) were woven from compound yarns containing 74% polyglactin 910 (PG910) and 26% polydioxanone (PDS) and were interposed into adult white New Zealand rabbit infrarenal aortas. Resultant prosthesis-tissue complexes were harvested after 2 weeks to 12 months. Specimens were photographed and sectioned for light, scanning, and transmission electron microscopy. Randomly selected fresh explants at 1 and 3 months and control aortic segments from the same rabbits were simultaneously perfused with culture media (37 degrees C, 100/80 mm Hg, 60 ml/min) and perfusates assayed by means of tritiated radioimmunoassay techniques for the stable prostacyclin metabolite 6-keto-PGF1 alpha before and after the addition of sodium arachidonate (10 micrograms/ml) to the media. Results showed 100% patency, no aneurysms, and stenosis in 1 of 37 prostheses (3%). PG910 was totally resorbed by 2 months and PDS by 6 months. By 1 month inner capsule thickness was 303 +/- 30 microns. In contrast to previous reports this was significantly thicker than that within 100% PDS (230 +/- 40 microns) and significantly less thick than in 100% PG910 (530 +/- 62 microns). Inner capsules in all three groups stabilized at similar thicknesses (417 to 502 microns).(ABSTRACT TRUNCATED AT 250 WORDS)

6-Ketoprostaglandin F1 alpha↗

Pendrin, encoded by the Pendred syndrome gene, resides in the apical region of renal intercalated cells and mediates bicarbonate secretion.

Pendrin is an anion transporter encoded by the PDS/Pds gene. In humans, mutations in PDS cause the genetic disorder Pendred syndrome, which is associated with deafness and goiter. Previous studies have shown that this gene has a relatively restricted pattern of expression, with PDS/Pds mRNA detected only in the thyroid, inner ear, and kidney. The present study examined the distribution and function of pendrin in the mammalian kidney. Immunolocalization studies were performed using anti-pendrin polyclonal and monoclonal antibodies. Labeling was detected on the apical surface of a subpopulation of cells within the cortical collecting ducts (CCDs) that also express the H(+)-ATPase but not aquaporin-2, indicating that pendrin is present in intercalated cells of the CCD. Furthermore, pendrin was detected exclusively within the subpopulation of intercalated cells that express the H(+)-ATPase but not the anion exchanger 1 (AE1) and that are thought to mediate bicarbonate secretion. The same distribution of pendrin was observed in mouse, rat, and human kidney. However, pendrin was not detected in kidneys from a Pds-knockout mouse. Perfused CCD tubules isolated from alkali-loaded wild-type mice secreted bicarbonate, whereas tubules from alkali-loaded Pds-knockout mice failed to secrete bicarbonate. Together, these studies indicate that pendrin is an apical anion transporter in intercalated cells of CCDs and has an essential role in renal bicarbonate secretion.

Animals↗

Adaptive evolution in the Arabidopsis MADS-box gene family inferred from its complete resolved phylogeny.

Gene duplication is a substrate of evolution. However, the relative importance of positive selection versus relaxation of constraints in the functional divergence of gene copies is still under debate. Plant MADS-box genes encode transcriptional regulators key in various aspects of development and have undergone extensive duplications to form a large family. We recovered 104 MADS sequences from the Arabidopsis genome. Bayesian phylogenetic trees recover type II lineage as a monophyletic group and resolve a branching sequence of monophyletic groups within this lineage. The type I lineage is comprised of several divergent groups. However, contrasting gene structure and patterns of chromosomal distribution between type I and II sequences suggest that they had different evolutionary histories and support the placement of the root of the gene family between these two groups. Site-specific and site-branch analyses of positive Darwinian selection (PDS) suggest that different selection regimes could have affected the evolution of these lineages. We found evidence for PDS along the branch leading to flowering time genes that have a direct impact on plant fitness. Sites with high probabilities of having been under PDS were found in the MADS and K domains, suggesting that these played important roles in the acquisition of novel functions during MADS-box diversification. Detected sites are targets for further experimental analyses. We argue that adaptive changes in MADS-domain protein sequences have been important for their functional divergence, suggesting that changes within coding regions of transcriptional regulators have influenced phenotypic evolution of plants.

Arabidopsis↗

Intracavernous injection during diagnostic screening for erectile dysfunction; five-year experience with over 600 patients.

Psychophysiological diagnostic screening (PDS) was carried out on just over 1000 consecutive patients with erectile dysfunction (ED) during 1995 through 1999. Roughly one-third did not require an intracavernous injection (ICI) because of optimal penile response during visual and tactile penile sexual stimulation. The present article deals with those other patients (n = 609) who required an ICI and completed a 24-hour-postinvestigation questionnaire (PIQ). The two ICI preparations used, prostaglandin and papaverine + fentolamine, were virtually equally effective in the doses applied, with very low and similar percentages of unwanted side effects, for example, prolonged erection. Somatic ED patients displayed the lowest penile responses to ICI, whereas psychogenic ED patients had the highest penile responses. ED patients who also suffered from premature ejaculation (PE) ejaculated significantly more readily during PDS that those without PE. An ICI following an ejaculation/orgasm could lead to an erection presumably sufficient for intromission, which indicates ICI as a therapeutic option for rapid ejaculators. Our elaborate and lengthy PDS procedure, particularly the use of penile vibration as an addition to the visual sexual stimulation, obviates the diagnostic use of the PIQ.

Adjuvants, Immunologic↗

Parenting stress among mothers of Malaysian children with cerebral palsy: predictors of child- and parent-related stress.

A hospital study was carried out to compare parenting stress among 87 Malaysian mothers of children with cerebral palsy and a control group (comprising 87 mothers of children without disability who attended the walk-in paediatric clinic), using the Parenting Stress Index (PSI) questionnaire. Multiple regression analysis was used to determine socio-demographic and medical factors associated with child-domain stress (CDS) and parent-domain stress (PDS). Mothers of children with cerebral palsy scored significantly higher than control subjects on all sub-scales of CDS and PDS (p < 0.01), except for the sub-scale of 'role restriction'. The presence of cerebral palsy (p < 0.001) and activities of daily living (ADL) scores (p < 0.001) were significantly associated with CDS. Factors predictive of PDS were ADL scores (p < 0.001), number of hospitalizations over the past year (p = 0.024), level of maternal education (p = 0.018) and Chinese mothers (p < 0.001). Although this study demonstrated that Malaysian mothers of children with cerebral palsy experienced higher levels of stress than controls, the impact of cerebral palsy per se on parenting stress was modified by other factors such as increased care-giving demands, low maternal education and ethnic background. Habilitation should be directed at easing the burden of daily care, minimizing hospital re-admissions and targeting appropriate psychosocial support at specific subgroups to change parental perception and expectations.

Adult↗

Comparison of parenting stress between Malaysian mothers of four-year-old very low birthweight and normal birthweight children.

UNLABELLED: A study was carried out to compare parenting stress between 116 mothers of very low birthweight (VLBW) children and 96 mothers of normal birthweight (NBW) children at 4 y of age, using the Parenting Stress Index (PSI). Multiple regression analysis was used to determine factors associated with child-domain stress (CDS) and parent-domain stress (PDS). There was a significantly higher proportion (39.7%) of mothers of VLBW children with high CDS scores >90th percentile than mothers of NBW children (20.8%). No significant differences were observed for PDS scores. Lower intelligence quotient (IQ) scores and adverse child behaviour, as evidenced by higher Child Behavior Checklist (CBCL) scores, were significantly associated with higher CDS scores (p < 0.001). Factors associated with higher PDS scores were higher CBCL scores (p < 0.001), mothers who were the primary caregivers (p < 0.001), male sex (p = 0.018) and lower level of maternal education (p = 0.048). These factors remained statistically significant even when physically and cognitively impaired children were excluded from the analysis. CONCLUSION: Specific child characteristics and the social environment appear to have a greater impact on parenting stress than the biological risk of VLBW birth per se.

Adult↗