Plexiform hypoglossal schwannoma of the tongue and the submandibular region

ABSTRACT: Hypoglossal schwannomas usually develop in the intracranial portion of the brain. Incidence of hypoglossal schwannomas of the submandibular region is extremely rare. A 27-year-old patient presented to us with hypoglossal schwannoma of the tongue and the submandibular region. The tumor was excised intraorally combined with submandibular approach. Histopathologic examination revealed a plexiform schwannoma (a rare variant).     

Use of recombinant human parathyroid hormone in hypocalcemic cardiomyopathy

Hypocalcemia secondary to hypoparathyroidism is a rare cause of congestive heart failure. However, its early recognition and treatment lead to significant improvement in cardiac function. We report a middle-aged woman presenting with symptoms of heart failure with a serum calcium level of 3.7 mg/dl and a serum inorganic phosphate level of 17.6 mg/dl 22 years after subtotal thyroidectomy. Besides calcium and calcitriol supplementation, she was the first patient with severe hypocalcemic cardiomyopathy to be given off-label recombinant human parathyroid hormone (PTH) because of an elevated serum calcium-phosphate product. We discuss the management and outcome of the patient and then present a brief review of similar previously reported cases. We also describe the pivotal role of calcium ion and the potential role of PTH in maintaining myocardial contractility, effective natriuresis, and possible pathogenic mechanisms contributing to heart failure secondary to hypocalcemia and hypoparathyroidism.     

High-sensitivity C-reactive protein (hs-CRP) in systemic lupus erythematosus patients without cardiac involvement; relation to disease activity,damage and intima-media thickness

Aim of the workTo assess the high sensitivity C-reactive protein (hs-CRP level) in systemic lupus erythematosus (SLE) patients without cardiac involvement and find its relation with clinical and laboratory findings, disease activity, damage index and intima-media thickness (IMT).Patients and methodsForty-five female SLE patients were recruited in the present study without any cardiac involvement. History taking, examination and laboratory investigations were performed for patients. Disease activity was evaluated by the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) and damage by the Systemic Lupus International Collaborating Clinics (SLICC) index. Thirty age matched female healthy subjects were considered as a control group. hs-CRP was measured quantitatively by microplate immunoenzymometric assay and the IMT measured by ultrasonography.ResultsThe hs-CRP in the patients was significantly higher (4.84 ± 3.91 mg/l) compared to the control (1.74 ± 0.61 mg/l) (p < 0.001). The IMT in the patients was significantly increased (0.72 ± 0.37 mm) compared to the control (0.54 ± 0.15 mm) (p 0.004). There was no difference in the level of hs-CRP according to the presence or absence of clinical manifestations. However, it was significantly higher in those with positive DNA (5.71 ± 4.36 mg/L) compared to those with negative results (3.12 ± 1.97 mg/L) (p 0.009). There was a significant correlation of the hs-CRP level with the IMT (r 0.49, p 0.001) and SLEDAI (r 0.67, p < 0.001).ConclusionsThese findings suggest that SLE patients without traditional major cardiovascular risk factors may have increased risk of future cardiac events. Measuring hs-CRP may be useful as a marker of disease activity, increased IMT and subclinical atherosclerosis in SLE especially those with positive ds-DNA.     

The Association of Metabolic Syndrome, Insulin Resistance and Non-alcoholic Fatty Liver Disease in Overweight/Obese Children

Background/Aim:

To study the prevalence of metabolic syndrome (MS), insulin resistance (IR) and non-alcoholic fatty liver disease (NAFLD) in overweight/obese children with clinical hepatomegaly and/or raised alanine aminotransferase (ALT).

Patients and Methods:

Thirty-three overweight and obese children, aged 2-13 years, presenting with hepatomegaly and/or raised ALT, were studied for the prevalence of MS, IR and NAFLD. Laboratory analysis included fasting blood glucose, serum insulin, serum triglycerides (TG), total cholesterol, high-density lipoprotein cholesterol (HDL-c), low-density lipoprotein cholesterol (LDL-c) and liver biochemical profile, in addition to liver ultrasound and liver biopsy.

Results:

Twenty patients (60.6%) were labeled with MS. IR was present in 16 (48.4%). Fifteen (44%) patients had biopsy-proven NAFLD. Patients with MS were more likely to have NAFLD by biopsy (P=0.001). Children with NAFLD had significantly higher body mass index, waist circumference, ALT, total cholesterol, LDL-c, TG, fasting insulin, and lower HDL-c compared to patients with normal liver histology (P< 0.05) and fitted more with the criteria of MS (80% vs. 44%). IR was significantly more common among NAFLD patients (73% vs. 28%).

Conclusion:

There is a close association between obesity, MS, IR and NAFLD. Obese children with clinical or biochemical hepatic abnormalities are prone to suffer from MS, IR and NAFLD.     

Gender Differences in the Heritability of Musculoskeletal and Body Composition Parameters in Mother-Daughter and Mother-Son Pairs

Bone mass and body composition traits are genetically programmed, but the timing and gender and site specificities of their heritability are unclear. Mother-child correlations of bone mineral density (BMD) and bone mineral content, lean mass, and fat mass were studied in 169 premenopausal mothers and their 239 children. Heritability estimates of lean mass, fat mass, BMD, and area were derived for each gender and pubertal stage. There were significant correlations for most densitometry-derived variables at the spine, hip, femoral neck (FN), and total body (r = 0.192–0.388) in mother-postmenarcheal daughter pairs, for bone areas at all sites in early puberty (r = 0.229–0.508) and for volumetric-derived density at FN and spine (r = 0.238–0.368) in mother-son pairs. Fat mass correlations were significant in both genders after puberty (r = 0.299–0.324) and lean mass in postmenarcheal girls only (r = 0.299). Heritability estimates varied between 21% and 37% for mother-daughter and 18% and 35% for mother-son pairs for density-derived variables and between 26% and 40% for body composition variables. Maternal inheritance of bone traits is expressed in early-pubertal boys for several skeletal site traits but consistently involves most site traits in girls and boys by late puberty. Body composition inheritance is more variable.     

Diagnostic Accuracy of Multiparametric Magnetic Resonance Imaging for Differentiation Between Parotid Neoplasms

PurposeThis study was designed to evaluate the role of multiparametric magnetic resonance imaging (MRI) for differentiation of parotid gland neoplasms.MethodsProspective study was conducted upon 52 consecutive patients (30 men, 22 women; aged 24–78 years; mean, 51 years) with parotid tumours that underwent multiparametric MRI using combined static MRI, dynamic contrast enhanced (DCE) MRI, and diffusion-weighted imaging (DWI). The static MRI parameter, time signal intensity curves (TIC) derived from DCE-MRI, and apparent diffusion coefficient (ADC) values of parotid tumours were correlated with histopathological findings.ResultsStatic MRI revealed a significant difference between both benign and malignant lesions in regards to margin definition (P < .001) and T2 hypointensity (P < .013), with a diagnostic accuracy 95% and 78.33% respectively. Study of the TIC type on DCE-MRI revealed statistically significant difference between benign and malignant lesions (P < .001) and diagnostic accuracy 96.55%. There was no statistically significant difference (P = .181) between the ADC values of benign and malignant lesions. ROC curve analysis revealed that by using ADC cut-off value of 1 × 10^?3 mm²/s had accuracy of 84.62% respectively for differentiating Warthin from malignant tumours that could be modified to higher value (94.28%) by excluding lymphoma from malignant lesions. By using cutoff value of 1.3 × 10^?3 mm²/s to differentiate pleomorphic adenoma from malignancy, ROC curve analysis had high accuracy of 97.06%.ConclusionMultiparametric MRI can be used for differentiation of malignant from benign parotid tumours and characterization of some benign parotid tumours.     

Favorable outcome to glucocorticoid therapy for engraftment syndrome in pediatric autologous hematopoietic cell transplant

ES remains an important cause of morbidity and mortality in children undergoing auto‐HCT. Glucocorticoid use in ES is an area of debate. We retrospectively analyzed single‐institution experience from September 2000 through December 2012 to evaluate the use of glucocorticoids in auto‐HCT patients. ES was defined by the occurrence of new onset of non‐infectious fever plus diarrhea, rash, or pulmonary infiltrates 24‐h before or within five days after neutrophil engraftment. Sixty‐five pediatric patients (<21 yr) with different solid tumors underwent auto‐HCTs in the study period. Fifteen patients (23%) fulfilled criteria for ES, of which 13 received methylprednisolone (2 mg/kg IV for 3–5 days). Clinical improvement occurred in all patients within 48 h without significant complications. In the non‐ES group, 11 patients received glucocorticoid without significant complications as well. MEL‐based regimens were found to be significant factor for ES (p < 0.05). Fever, edema, non‐infectious diarrhea, and serum albumin concentration were statistically different between the two groups. Median hospital length of stay was higher in the ES group. Conclusion: ES is a common complication in children after auto‐HCT and short‐course glucocorticoid therapy is an effective and well‐tolerated treatment, even in those who did not completely fulfill diagnostic criteria.     

Secular Trends in Hip Fractures Worldwide: Opposing Trends East Versus West

Despite wide variations in hip rates fractures worldwide, reasons for such differences are not clear. Furthermore, secular trends in the age‐specific hip fracture rates are changing the world map of this devastating disease, with the highest rise projected to occur in developing countries. The aim of our investigation is to systematically characterize secular trends in hip fractures worldwide, examine new data for various ethnic groups in the United States, evidence for divergent temporal patterns, and investigate potential contributing factors for the observed change in their epidemiology. All studies retrieved through a complex Medline Ovid search between 1966 and 2013 were examined. For each selected study, we calculated the percent annual change in age‐standardized hip fracture rates de‐novo. Although occurring at different time points, trend breaks in hip fracture incidence occurred in most Western countries and Oceania. After a steep rise in age‐adjusted rates in these regions, a decrease became evident sometimes between the mid‐seventies and nineties, depending on the country. Conversely, the data is scarce in Asia and South America, with evidence for a continuous rise in hip fracture rates, with the exception of Hong‐Kong and Taiwan that seem to follow Western trends. The etiologies of these secular patterns in both the developed and the developing countries have not been fully elucidated, but the impact of urbanization is at least one plausible explanation. Data presented here show close parallels between rising rates of urbanization and hip fractures across disparate geographic locations and cultures. Once the proportion of the urban population stabilized, hip fracture rates also stabilize or begin to decrease perhaps due to the influence of other factors such as birth cohort effects, changes in bone mineral density and BMI, osteoporosis medication use and/or lifestyle interventions such as smoking cessation, improvement in nutritional status and fall prevention. © 2014 American Society for Bone and Mineral Research.