Hemichorea-Hemiballism Secondary to Non-Ketotic Hyperglycemia.

Non-ketotic hyperglycemia is an unusual and rare cause of hemichorea-hemiballismus. Correction of the hyperglycemia usually results in total resolution of the signs and symptoms. We present the case and medical imaging findings of a 66-year-old female who presented with steadily worsening choreiform and ballistic movements of the right upper and lower extremities over a 2-week period. Her serum glucose was greater than 600 mg/dL, and no ketones were present. CT scan and MR demonstrated left basal ganglia abnormalities suggesting hyperglycemia-related hemichorea-hemiballismus syndrome. Restoration of euglycemia led to eventual resolution of all symptoms. Knowledge of this disorder is paramount so as to rule out other causes of intracranial pathology.

Management of bladder dysfunction in Wolfram syndrome with Mitrofanoff appendicovesicostomy: long-term follow-up.

PURPOSE: To present the long-term outcomes of appendicovesicostomy using the Mitrofanoff principle for end-stage Wolfram bladder dysfunction as an alternative to clean intermittent self-catheterization (CIC) per urethra mainly following blindness. METHODS: Twelve Wolfram patients presenting with bilateral hydroureteronephrosis and advanced bladder dysfunction were included in this study. All patients were managed initially by CIC per urethra. All of these patients became blind during follow-up and were unable to perform urethral CIC independently. Out of these patients, six patients agreed to proceed to appendicovesicostomy. Appendicovesicostomy urinary diversion using the Mitrofanoff principle was performed in these six blind patients. The rest of the patients stopped CIC or performed CIC irregularly. RESULTS: Severe hydroureteronephrosis and large bladders were found in all patients prior to intervention. All patients were able to conduct CIC independently through the stoma and maintained overnight bladder free drainage. In all patients with urinary diversion and CIC, the hydroureteronephrosis was reduced and renal function returned to normal. However, the non-intervention group ended with different degrees of progressive renal failure with three mortalities during the follow-up. CONCLUSIONS: We suggest appendicovesicostomy as a safe and lifesaving procedure for long-term management of bladder dysfunction in Wolfram syndrome particularly after progression to blindness.

Repeated transsphenoidal surgery or gamma knife radiosurgery in recurrent cushing disease after transsphenoidal surgery.

BACKGROUND: This study compared Gamma knife radiosurgery (GKRS) and repeated transsphenoidal adenomectomy (TSA) to find the best approach for recurrence of Cushing disease (CD) after unsuccessful first TSA. MATERIAL AND METHODS: Fifty-two patients with relapse of CD after TSA were enrolled and randomly underwent a second surgery or GKRS as the next therapeutic approach. They were followed for a mean period of 3.05 ± 0.8 years by physical examination and hormone measurement as well as magnetic resonance imaging. RESULTS: No significant difference was observed in sex ratio, mean age, adenoma type, follow-up duration, and initial hormone level between the two groups. No significant relationship was found between preoperative 24-hour free urine cortisol and disease-free months or tumor volume among both groups. Our statistical analysis showed higher recurrence-free interval in the GKRS group compared with TSA group. CONCLUSION: With longer recurrence-free interval, GKRS could be considered a good treatment alternative to repeated TSA in recurrent CD.

Assessing whether parents and children perceive the meaning of the items in the PedsQLTM 4.0 quality of life instrument consistently: a differential item functioning analysis.

Limited studies have examined the effect of differential item functioning (DIF) on comparing health related quality of life (HRQoL) scores across child self-reports and parent proxy-reports. This study aims to determine whether parents and children respond differently to the items in the Persian version of the PedsQoLTM 4.0 measure. The PedsQLTM 4.0 Generic Core Scales was completed by 938 child-parent dyads. The graded response model (GRM) was used to detect DIF between parents and children. The IRT analyses were conducted using IRTPRO 2.1.On the whole, our findings showed that 50% (4 out of 8) of the items in the physical subscale and 40% (2 out of 5) in both emotional and school subscales were flagged with DIF. Among the DIF items, 62.5% (5 out of 8) were uniform and the remaining 37.5% (3 out of 8) were non-uniform. Parents and children interpret certain items of the PedsQLTM 4.0 in a different ways, except for the social subscale. Hence, we should be cautious about using parent proxy-report as a substitute for a child's ratings.

Identifying causes of laboratory turnaround time delay in the emergency department.

BACKGROUND: Laboratory turnaround time (TAT) is an important determinant of patient stay and quality of care. Our objective is to evaluate laboratory TAT in our emergency department (ED) and to generate a simple model for identifying the primary causes for delay. METHODS: We measured TATs of hemoglobin, potassium, and prothrombin time tests requested in the ED of a tertiary-care, metropolitan hospital during a consecutive one-week period. The time of different steps (physician order, nurse registration, blood-draw, specimen dispatch from the ED, specimen arrival at the laboratory, and result availability) in the test turnaround process were recorded and the intervals between these steps (order processing, specimen collection, ED waiting, transit, and within-laboratory time) and total TAT were calculated. Median TATs for hemoglobin and potassium were compared with those of the 1990 Q-Probes Study (25 min for hemoglobin and 36 min for potassium) and its recommended goals (45 min for 90% of tests). Intervals were compared according to the proportion of TAT they comprised. RESULTS: Median TATs (170 min for 132 hemoglobin tests, 225 min for 172 potassium tests, and 195.5 min for 128 prothrombin tests) were drastically longer than Q-Probes reported and recommended TATs. The longest intervals were ED waiting time and order processing.  CONCLUSIONS: Laboratory TAT varies among institutions, and data are sparse in developing countries. In our ED, actions to reduce ED waiting time and order processing are top priorities. We recommend utilization of this model by other institutions in settings with limited resources to identify their own priorities for reducing laboratory TAT.

Notch inhibition during corneal epithelial wound healing promotes migration.

PURPOSE: To determine the role of Notch signaling in corneal epithelial migration and wound healing. METHODS: Immunolocalization of Notch1 was performed during epithelial wound healing in vivo in mouse corneal epithelial debridement wounds and in vitro in primary human corneal epithelial cells following a linear scratch wound. The effects of Notch inhibition, using the γ-secretase inhibitor N-(N-[3,5-difluorophenacetyl]-l-alanyl)-S-phenylglycine t-butyl ester (DAPT) or following stable transfection with Notch1-short hairpin RNA (shRNA), was evaluated in a scratch assay and transwell migration assay. Likewise, in vitro adhesion, proliferation and the actin cytoskeleton was examined. The DAPT effect was also evaluated in vivo in a mouse model of corneal epithelial wound healing. RESULTS: The expression of Notch1 was reduced at the leading edge of a healing corneal epithelium both in vivo and in vitro. Notch inhibition using DAPT and using Notch1-shRNA both enhanced in vitro migration in scratch and transwell migration assays. Consistent with this increased migratory behavior, Notch inhibited cells demonstrated decreased cell-matrix adhesion and enhanced lamellipodia formation. Notch inhibition by DAPT was also found to accelerate corneal epithelial wound closure in an in vivo murine model without affecting proliferation. CONCLUSIONS: The results highlight the role of Notch in regulating corneal epithelial migration and wound healing. In particular, Notch signaling appears to decrease in the early stages of wound healing which contributes to cytoskeletal changes with subsequent augmentation of migratory behavior.

Mental health in patients with pemphigus: an issue to worth consideration.

BACKGROUND: There exists a high prevalence of psychiatric disorders in dermatological patients. Although, investigators have evaluated psychiatric aspects of the patients suffering from skin diseases; there are rare studies concerning mental health in pemphigus patients. The objective of this study was to evaluate mental health status and quality of life of newly diagnosed pemphigus patients. MATERIALS AND METHODS: Between April 2007 and June 2008, all newly diagnosed pemphigus patients attending the outpatient clinic of a dermatological hospital were given a questionnaire comprising the GHQ-28 and DLQI to fill out. RESULTS: Of 283 patients, 212 complete forms were returned. The bimodal score of GHQ ranged from 0 to 26 (Mean = 9.4) and the Likert score of GHQ ranged from 6 to 68 (Mean = 31.9). The DLQI score ranged between 0 and 30 (Mean of 13.8). A total of 157 patients (73.7%) were yielded to be possible cases of mental disorder considering GHQ-28 bimodal scores. Significant correlation was detected between the DLQI score and bimodal and Likert scoring of GHQ-28. CONCLUSION: Our study has depicted high prevalence of psychiatric comorbidity in pemphigus patients. It underlines the fact that physicians, who are in-charge of care for these patients, are in an exceptional position to distinguish the psychiatric comorbidity and to take appropriate measures.

Health related quality of life of Iranian children with type 1 diabetes: reliability and validity of the Persian version of the PedsQL™ Generic Core Scales and Diabetes Module.

BACKGROUND: The aim of this study was to measure health related quality of life (HRQOL) in Iranian children with type 1 diabetes and to test the psychometric properties of the Persian version of the PedsQL™ 4.0 Generic Core Scales and the PedsQL™ 3.0 Diabetes Module. METHODS: Participants were 94 children and adolescents diagnosed with type 1 diabetes for at least 3 months in Shiraz, southern Iran. Convergent, discriminant, and construct validity of the PedsQL™ 4.0 Generic Core Scales and the PedsQL™ 3.0 Diabetes Module were assessed. Moreover, internal consistency was checked by Cronbach's alpha coefficient. RESULTS: Cronbach's α for the PedsQL™ 4.0 Generic Core Scales and the PedsQL™ 3.0 Diabetes Module was greater than 0.80 both in the child self-report and parent proxy-report. Both generic and disease-specific versions of the PedsQL showed excellent convergent and acceptable discriminant validity except for 'diabetes symptoms' subscale in the child self-report of the disease-specific module. Moreover, Iranian children with diabetes, as compared with other countries, had lower HRQOL scores. CONCLUSIONS: While this study showed that the Persian version of the PedsQL™ 4.0 Generic Core Scales has good psychometric properties in children with type 1 diabetes, the PedsQL™ 3.0 Diabetes Module needs some modifications to be used as a disease-specific quality of life (QOL) measure. Also, more support should be provided for the care of Iranian children with diabetes.

Perivascular nitric oxide delivery to saphenous vein grafts prevents graft stenosis after coronary artery bypass grafting: a novel sheep model.

OBJECTIVES: Graft stenosis is a major complication of coronary artery bypass grafting with autologous saphenous vein grafts. Nitric oxide (NO) is believed to prevent this phenomenon. We studied the effect of perivascular application of an NO donor on the degree of stenosis of such grafts in an ovine model. METHODS: Twenty white Iranian ewes were randomized to coronary artery bypass grafting using autologous saphenous vein grafts with application of an elastomer gel containing diethylenetriamine NO adduct in 0.9% sodium chloride solution around the grafted vessel (intervention group) or with the gel containing the saline solution alone (controls). Graft vessels were studied after 1 year using spot angiography and histological examination. RESULTS: The mean degree of stenosis was significantly lower in the intervention group than in the controls as found by histology (92.3 ± 5.5 vs. 80.9 ± 8.3%; p = 0.004). Although the difference in the angiographic score was not significant, the mean score was still lower in the intervention group (9.5 ± 11.3 vs. 12.0 ± 11.8). CONCLUSIONS: Perivascular application of an NO donor was, at least histologically, effective in reducing graft stenosis in our ovine model. This can be a step toward the development of drug-eluting coronary artery bypass grafts.

Atherosclerosis and vascular injury: the effect of a perivascular nitric oxide donor in a cholesterol-fed rabbit model.

Nitric oxide (NO) has been shown to prevent neointimal hyperplasia and decrease atherosclerosis in several animal models. It is a major modulator of vascular homeostasis and has vasoprotective effects against atherosclerosis. However, NO-based therapies with such purposes have not been used in the clinical arena. Our objective was to combine a medical grade elastomer and an NO donor, diethylenetriamine NO adduct (DETA/NO), to determine whether its perivascular administration can attenuate atherosclerosis and vascular injury. Aortic intimal injury was produced using paediatric pulmonary valvoplasty catheter in 22 healthy male New Zealand White rabbits, which were fed a high-cholesterol diet for 4 weeks beforehand. A mixture of the elastomer Silastic and DETA/NO was applied locally to cover the aortas in the experiment group. After 6 additional weeks on the high-cholesterol diet, the aortas and blood samples were harvested for pathologic analysis and comparison with the control group. Mean atherosclerosis and vascular injury surface area was 6.68 x 10(5) microm2 in the experiment group, compared with 3.44 x 10(5) microm2 in the controls. However, there was no statistically significant difference in atherosclerotic surface area between the two groups. Perivascular application of the NO donor DETA/NO, in the concentration we used, did not prevent atherosclerosis in high cholesterol-fed rabbits. This finding prompts more careful assessment before possible clinical uses.