Juvenile dermatomyositis presenting with periorbital edema

Juvenile dermatomyositis is a rare disease that affects the skin and muscles. It often presents with a classic heliotrope eyelid rash. We present a case of juvenile dermatomyositis presenting with significant bilateral periorbital edema, with its complete resolution after systemic antiinflammatory therapy.     

Optic disc coloboma and localized chorioretinal defects in constitutional partial trisomy 8 mosaicism

Constitutional partial trisomy 8 mosaicism is a rare chromosomal multisystem disorder with systemic and ophthalmologic manifestations. We report the case of a 15-year-old female mosaic for trisomy 8 who has unilateral colobomatous microphthalmia and multiple chorioretinal defects. These congenital anomalies have not been previously reported in association with constitutional partial trisomy 8 mosaicism.     

Ultrasonographic visualization of lower eyelid structures and dynamic motion analysis

AIM

To define the ultrasonographic structure of normal lower eyelid anatomic compartments and their spacial relationship in dynamic motion.

METHODS

High resolution ultrasound (15MHz) was performed on the lower eyelids of 7 normal subjects. Movements of the lower eyelid and its compartments were visualized with ultrasound. In addition, the maximal excursion area of the lower eyelid fat compartments and retractor motions was measured before and after motion.

RESULTS

The orbicularis muscle could be seen as an echolucent structure between the dermis and the echodence fat pads. Lower eyelid fat pad seems to be divided into 2 compartments as range of motion and direction of movement of each of them varies. It seems that these compartments have also different behavior. The measured profile area of the visible normal lower eyelid fat pads during movement of globe from up-gaze to down-gaze decreased by 50%. Order of movement of lower eyelid structures seems to be as follows: after globe movement fist we see retractor movement, anterior orbital fat pad, then skin and septum, and finally movement of inferior fat pad.

CONCLUSION

Ultrasound represents a noninvasive tool for the visualization of lower eyelid morphology. Expanding its application could help us understand the compartmental changes in physiological eyelid movement, in aging and diseased study populations, as well as assess operative outcomes.     

Association of persistent hyperglycemia with outcome of severe traumatic brain injury in pediatric population

Purpose

Hyperglycemia is a common secondary insult associated with an increased risk of mortality and poor outcome in traumatic brain injury (TBI), but the effect of hyperglycemia on outcomes of severe TBI in children and adolescents is less apparent. The aim of this study was to evaluate the association of hyperglycemia with mortality in pediatric patients with severe TBI.

Methods

In this cross-sectional study, data of all children and adolescents with severe TBI admitted to Poursina Hospital in Rasht, including age, gender, Glasgow Coma Scale (GCS) upon admission, mortality rate, hospital length of stay, and serial blood glucose during the first three consecutive ICU days following admission, were reviewed from April 2007 to May 2011. After univariate analysis and adjustment for related covariates, logistic regression model was established to determine the association between persistent hyperglycemia and outcome.

Results

One-hundred and twenty-two children were included with a median admission GCS of 6 (interquartile range (IQR) 5–7) and a median age of 13?years (IQR 7.75–17). Among them, 91 were boys (74.6?%) and 31 were girls (26.6?%); the overall mortality was 40.2?% (n?=?49). Patients who died had a significantly greater blood glucose levels than survivors for the first 3?days of admission (P?=?0.003, P?P?=?0.001, respectively). Moreover, persistent hyperglycemia during the first 3?days of admission had an adjusted odds ratio of 11.11 for mortality (P?Conclusion Early hyperglycemia is associated with poor outcome, and persistent hyperglycemia is a powerful and independent predictor of mortality in children and adolescents with severe TBI.     

Importance of Mean Platelet Volume in Predicting Cardiac Mechanics Parameters and Carotid‐Intima Media Thickness in Children With End‐Stage Renal Disease and Comparison With Healthy Children

Cardiovascular disease (CVD) is the major cause of death in children with ESRD. Echocardiography and Doppler ultrasound are useful devices for diagnosing cardiovascular abnormalities in such patients. However, they are expensive, difficult to perform as a routine, and not available in many centers. Therefore, finding a more accessible and inexpensive method for CVD evaluation biomarkers is needed. The aim of this study was to evaluate the relationship between mean platelet volume (MPV) as a routine hematological parameter with cardiac mechanics characteristics in children with ESRD. Forty‐two children under dialysis and 60 age‐ and sex‐matched healthy subjects as control group were enrolled in the study. Carotid‐intima media thickness (CIMT) and echocardiographic parameters were measured in both groups. In addition, hematological and biochemical variables were evaluated in blood samples of participants. MPV was significantly higher in patients than in controls. CIMT, left ventricular mass index (LVMI), end diastolic diameter, strain rate, and global longitudinal strain were significantly different between the two groups. MPV was positively correlated with LVMI and inversely with ejection fraction. In receiver operating characteristic (ROC) curve analysis, the area under the ROC curve (AUC) values for MPV in predicting left ventricular hypertrophy (LVH) and abnormal CIMT were 0.65 (P = 0.07) and 0.53 (P = 0.74), respectively. MPV was correlated with some cardiac abnormalities in children with ESRD. However, it could not show appropriate predictive values in diagnosing LVH and subclinical atherosclerosis. Further studies with prospective design could shed more light in this topic.     

Prevalence of human papillomavirus in esophageal carcinoma in Tangshan,China

AIM:to study the prevalence of human papillomavirus(HPV) in esophageal carcinoma in tangshan,China,a high-incidence area.METHODS:Formalin-fixed,paraffin-embedded tissue specimens from 198 patients who were pathologically diagnosed with esophageal squamous cell carcinoma from 2011 to 2013 were obtained from a pathology department in Tangshan.DNA was extracted from all198 specimens to detect HPV by polymerase chain reaction(PCR).β-globin PCR was performed to check the quality of the DNA extraction procedure.PCR was performed to detect a wide range of HPV types,and type-specific PCR was performed to detect HPV types16 and 18.Negative and positive controls were used for HPV 16 and 18 detection.RESULTS:the DNA extraction method in this study appeared to be more effective than other previously reported methods.After DNA extraction,more than98%of the tissue specimens had an acceptable result in the DNA qualification test(β-globin PCR).the overall prevalence of HPV in tumor tissues by GP6+/GP5+PCR was 79.79%,and the prevalence of HPV types16 and 18 was 40.40%and 47.47%,respectively.PCR demonstrated the presence of HPV,and direct sequencing confirmed the HPV genotypes.All HPVpositive PCR products were checked by DNA sequence analysis using DNAman and compared with the known HPV sequences listed in the basic Local Alignment Search tool database to evaluate the HPV types.this analysis confirmed the presence of HPV types 16 and18.CONCLUSION:DNA of high-risk HPV types 16 and 18is present in esophageal tumors,implicating HPV as a possible etiologic factor for esophageal squamous cell carcinoma.     

Uptake of the yeast Malassezia furfur and its allergenic components by human immature CD1a+ dendritic cells

Atopic dermatitis (AD) is a chronic inflammatory skin disease with increasing prevalence, though still little is known of the pathomechanisms and the causes of the disease. Patients with AD often have specific IgE reactivity to the yeast Malassezia furfur (M. furfur), present in the normal microflora on human skin. To investigate the possible interaction of immature and mature antigen-presenting dendritic cells with the yeast M. furfur and its allergenic components. Monocyte-derived dendritic cells (MDDCs) generated from human peripheral blood were allowed to interact with FITC-labelled whole M. furfur yeast cells, M. furfur extract, a recombinant allergen from M. furfur designated rMal f 5 and M. furfur mannan, in the absence of IgE antibodies. Interaction and uptake were detected using flow cytometry and confocal laser scanning microscopy. Internalization of M. furfur yeast cells and yeast components by immature MDDCs was found using confocal laser scanning microscopy. Results from flow cytometric studies showed that a median of 94% (range, 65-98%) of the immature CD1a+ MDDCs were M. furfur extract positive, 81% (75-97%) rMal f 5 positive and 93% (62-98%) mannan positive. Mature CD1a+ MDDCs were significantly less efficient in this respect, with the corresponding figures only 26% (6-37%, P < 0.01), 6% (2-15%, P < 0.05) and 32% (9-50%, P < 0.01), respectively. Uptake of the non-glycosylated rMal f 5 by immature CD1a+ MDDCs was decreased to 27% (15-38%) by inhibition of pinocytosis. The binding of M. furfur extract and mannan was inhibited in a dose-dependent manner by methyl-alpha-D-mannopyranoside, suggesting uptake via the mannose receptor. Human immature CD1a+ MDDCs can efficiently take up M. furfur and allergenic components from the yeast in the absence of IgE antibodies, implying that sensitization of AD patients to M. furfur can be mediated by immature dendritic cells in the skin.     

Can serum level of N‐terminal pro B‐type natriuretic peptide be used in patients with psoriasis as a predictor of cardiovascular disease?

Past studies have reported associations between psoriasis, metabolic syndrome, and atherosclerotic cardiovascular disease. According to studies, N‐terminal pro B‐type natriuretic peptide (NT‐proBNP) is a useful screening test for cardiac disease. We examined the serum NT‐proBNP level in patients with psoriasis and compared them with nonpsoriatic healthy control subjects. Sixty‐one patients with psoriasis were enrolled, along with 61 age, sex, and body mass index (BMI) matched control subjects. In both groups, NT‐proBNP serum levels and lipid profile parameters were investigated. Means and 95% confidence intervals (CIs) were reported. The median serum concentration of NT‐proBNP was higher in psoriatic patients than the control group (26.67 [interquartile range (IQR): 15.15–43.03 and range: 5–250] vs. 17.45 [IQR: 12.35–20.80 and range: 5–45.09, p < 0.0001). NT‐proBNP serum level in psoriatic arthritis patients (11%; 55.6 ± 25.7 pg/mL, 95% CI: 31.9–79.4 pg/mL) was higher than psoriasis patients without arthritis (35.8 ± 40.6 pg/mL, CI: 24.7–46.9, p < 0.001). NT‐proBNP levels were also positively correlated with BMI, lipid profile, and disease duration. NT‐proBNP is elevated in patients with psoriasis, consistent with the high risk of cardiovascular disease associated with psoriasis.