Three-dimensional radiographic assessment of the mandibular interforaminal donor site in different vertical facial growth types.

PURPOSE: To assess volumetrically, the impact of vertical facial growth types (VFGT) on the mandibular interforaminal region as a potential bone donor site. MATERIAL AND METHODS: 60 cone beam computed tomography (CBCT) scans of adult individuals were classified in three groups according to their SN-GoGn angle: hypodivergent group (hG) (N=20), normodivergent group (NG) (N=19) and hyperdivergent group (HG) (N=21). Total harvestable bone volume (TBV), cortico-cancellous bone volume (CBV-cBV), and cortical bone surface (CBS) were evaluated. ANOVA test followed by Tukey post hoc tests were used to compare the mean continuous outcomes according to their VFGT. RESULTS: The whole sample showed a mean TBV of 1376.32±541.01mm3, CBV of 468.52±121.54mm3 and cBV of 908.73±474.71mm3. The mean CBS amounted to 782.58±146.80mm2. The comparison between the groups stated a significantly different mean TBV and cBV (-p-value<0.001). The mean CBS was significantly different (-p-value=0.015): the smallest for the NG, but not significantly different (-p-value<0.001): the highest for the HG, intermediate for the NG and the smallest for the hG. CONCLUSION: Hypodivergent individuals have the thickest cancellous bone suitable for an onlay bone graft, while hyperdivergent individuals have the thinnest bone ideal for a 3D grafting approach.

Periodontal Inflammation Primes the Systemic Innate Immune Response.

The presence of periodontal diseases (PDs) often strongly correlates with other severe chronic inflammatory conditions, including cardiovascular disease, diabetes, and arthritis. However, the mechanisms through which these diseases interact are unclear. In PD, tissue and bone destruction in the mouth is driven by elevated recruitment of polymorphonuclear neutrophils (PMNs), which are primed and recruited from the circulation to sites of inflammation. We predicted that systemic effects on PMN mobilization or priming could account for the interaction between PD and other inflammatory conditions. We tested this using a mouse model of ligature-induced PD and found elevated PMN counts specifically in bone marrow, supporting a systemic effect of periodontal tissue inflammation on PMN production. In contrast, mice with induced peritonitis had elevated PMN counts in the blood, peritoneum, and colon. These elevated counts were further significantly increased when acute peritonitis was induced after ligature-induced PD in mice, revealing a synergistic effect of multiple inflammatory events on PMN levels. Flow cytometric analysis of CD marker expression revealed enhanced priming of PMNs from mice with both PD and peritonitis compared to mice with peritonitis alone. Thus, systemic factors associated with PD produce hyperinflammatory PMN responses during a secondary infection. To analyze this systemic effect in humans, we induced gingival inflammation in volunteers and also found significantly increased activation of blood PMNs in response to ex vivo stimulation, which reverted to normal following resolution of gingivitis. Together, these results demonstrate that periodontal tissue inflammation has systemic effects that predispose toward an exacerbated innate immune response. This indicates that peripheral PMNs can respond synergistically to simultaneous and remote inflammatory triggers and therefore contribute to the interaction between PD and other inflammatory conditions. This suggests larger implications of PD beyond oral health and reveals potential new approaches for treating systemic inflammatory diseases that interact with PD.

Crohn's disease: A retrospective analysis between computed tomography enterography, colonoscopy, and histopathology.

INTRODUCTION: To investigate the spectrum of computed tomography enterography (CTE) findings of active Crohn's disease (CD) in comparison to endoscopic, histopathologic and inflammatory markers. METHODS: Hospital records of 197 patients with known or suspected CD who underwent CTE over a period of 5 years were reviewed. Eighty-nine patients fulfilled the inclusion criteria. Three-point severity scores for endoscopy, pathology, and haematologic inflammatory markers were recorded. The findings on CTE were identified by three readers and correlated with endoscopic, pathologic, and haematologic severity scores. Statistical analysis was carried out employing a Pearson Chi square test and Fisher exact test. Receiver operating characteristic (ROC), visual grading characteristic (VGC) and Cohens' kappa analyses were performed. RESULTS: The CTE findings which were significantly correlated with the severity of active disease on endoscopy include bowel wall thickening, mucosal hyperenhancement, bilaminar stratified wall enhancement, transmural wall enhancement, and mesenteric fluid adjacent to diseased bowel (p < 0.05). Only bowel wall thickening and bilaminar stratified wall enhancement correlated with the pathological severity of active CD. ROC and VGC analysis demonstrated significantly higher areas under the curve (p < 0.0001) together with excellent inter-reader agreement (k = 0.86). CONCLUSION: CTE is a reliable tool for evaluating the severity of active disease and helps in the clinical decision pathway.

Intravenous thrombolysis and thrombectomy decisions in acute ischemic stroke: An interrater and intrarater agreement study.

PURPOSE: We aimed to assess agreement on intravenous tissue-plasminogen activator (IV tPA) and mechanical thrombectomy (MT) management decisions in acute ischemic stroke (AIS) patients. Secondary objectives were to assess agreement on Diffusion-Weighted-Imaging-Alberta-Stroke-Program-EArly-CT-Score (DWI-ASPECTS), and clinicians' willingness to recruit patients in a randomized controlled trial (RCT) comparing medical management with or without MT. MATERIALS AND METHODS: Studies assessing agreement of IV tPA and MT were systematically reviewed. An electronic portfolio of 41 AIS patients was sent to randomly selected providers at French stroke centers. Raters were asked 4 questions for each case: (1) What is the DWI-ASPECTS? (2) Would you perform IV tPA? (3) Would you perform MT? (4) Would you include the patient in a RCT comparing standard medical therapy with or without MT? Twenty responders were randomly selected to study intrarater agreement. Agreement was assessed using Fleiss' Kappa statistics. RESULTS: The review yielded two single center studies involving 2-5 raters, with various results. The electronic survey was answered by 86 physicians (60 vascular neurologists and 26 interventional neuroradiologists). The interrater agreement was moderate for IV tPA treatment decisions (κ=0.565 [0.420-0.680]), but only fair for MT (κ=0.383 [0.289-0.491]) and for combined treatment decisions (κ=0.399 [0.320-0.486]). The intrarater agreement was at least substantial for the majority of raters. The interrater agreement for DWI-ASPECTS was fair (κ=0.325 [0.276-0.387]). Physicians were willing to include a mean of 14±9 patients (33.1%±21.7%) in a RCT. CONCLUSION: Disagreements regarding the use of IVtPA or MT in the management of AIS patients remain frequent. Further trials are needed to resolve the numerous areas of uncertainty.

Hemorrhagic transformation after stroke: inter- and intrarater agreement.

BACKGROUND AND PURPOSE: Hemorrhagic transformation (HT) is a complication of stroke that can occur spontaneously or after treatment. We aimed to assess the inter- and intrarater reliability of HT diagnosis. METHODS: Studies assessing the reliability of the European Cooperative Acute Stroke Study (ECASS) classification of HT or of the presence (yes/no) of HT were systematically reviewed. A total of 18 raters independently examined 30 post-thrombectomy computed tomography scans selected from the Aspiration versus STEnt-Retriever (ASTER) trial. They were asked whether there was HT (yes/no), what the ECASS classification of the particular scan (0/HI1/HI2/PH1/PH2) (HI indicates hemorrhagic infarctions and PH indicates parenchymal hematomas) was and whether they would prescribe an antiplatelet agent if it was otherwise indicated. Agreement was measured with Fleiss' and Cohen's κ statistics. RESULTS: The systematic review yielded four studies involving few (≤3) raters with heterogeneous results. In our 18-rater study, agreement for the presence of HT was moderate [κ = 0.55; 95% confidence interval (CI), 0.41-0.68]. Agreement for ECASS classification was only fair for all five categories, but agreement improved to substantial (κ = 0.72; 95% CI, 0.69-0.75) after dichotomizing the ECASS classification into 0/HI1/HI2/PH1 versus PH2. The inter-rater agreement for the decision to reintroduce antiplatelet therapy was moderate for all raters, but substantial among vascular neurologists (κ = 0.70; 95% CI, 0.57-0.84). CONCLUSION: The ECASS classification may involve too many categories and the diagnosis of HT may not be easily replicable, except in the presence of a large parenchymal hematoma.

Hyperphosphatemic familial tumoral calcinosis secondary to fibroblast growth factor 23 (FGF23) mutation: a report of two affected families and review of the literature.

Hyperphosphatemic familial tumoral calcinosis (HFTC), secondary to fibroblast growth factor 23 (FGF23) gene mutation, is a rare genetic disorder characterized by recurrent calcified masses. We describe young Lebanese cousins presenting with HFTC, based on a retrospective chart review and a prospective case study. In addition, we present a comprehensive review on the topic, based on a literature search conducted in PubMed and Google Scholar, in 2014 and updated in December 2017. While the patients had the same previously reported FGF23 gene mutation (homozygous c.G367T variant in exon 3 leading to a missense mutation), they presented with variable severity and age of disease onset (at 4 years in patient 1 and at 23 years in patient 2). A review of the literature revealed several potential patho-physiologic pathways of HFTC clinical manifestations, some of which may be independent of hyperphosphatemia. Most available treatment options aim at reducing serum phosphate level, by stimulating renal excretion or by inhibiting intestinal absorption. HFTC is a challenging disease. While the available medical treatment has a limited and inconsistent effect on disease symptomatology, surgical resection of calcified masses remains the last resort. Research is needed to determine the safety and efficacy of FGF23 replacement or molecular therapy, targeting the specific genetic aberration. Hyperphosphatemic familial tumoral calcinosis is a rare genetic disorder characterized by recurrent calcified masses, in addition to other visceral, skeletal, and vascular manifestations. It remains a very challenging disease.

Application of the DAWN clinical imaging mismatch and DEFUSE 3 selection criteria: benefit seems similar but restrictive volume cut-offs might omit potential responders.

BACKGROUND AND PURPOSE: An external validation of the selection criteria of diffusion-weighted imaging or computerized tomography perfusion assessment with clinical mismatch in the triage of wake-up and late-presenting strokes undergoing the Neurointervention with Trevo (DAWN) and the Endovascular Therapy Following Imaging Evaluation for Ischemic Stroke (DEFUSE3) trials was conducted in a cohort of unknown onset stroke (UOS) patients treated with thrombectomy. METHODS: A validation cohort of UOS patients was selected from a prospectively collected thrombectomy database to match the inclusion criteria of DAWN and DEFUSE 3. Patients with an initial National Institutes of Health Stroke Scale (NIHSS) ≥10 were stratified according to the DAWN selection criteria. Patients ≤90 years old with an initial NIHSS ≥6 were stratified according to the DEFUSE 3 selection criteria. The proportions of patients with a modified Rankin Scale (mRS) ≤2 at 3 months follow-up were compared between DAWN-eligible patients and the DAWN trial thrombectomy group, and between DEFUSE 3-eligible patients and the DEFUSE 3 trial thrombectomy group. RESULTS: Of the 60/102 (59%) DAWN-eligible patients, 26 patients (43%) reached a mRS ≤2 at 3 months follow-up [versus 52/107 patients (49%) in the DAWN trial thrombectomy group; P = 0.52]. Of the 100/117 (85%) DEFUSE 3-eligible patients, 48 patients (48%) reached a mRS ≤2 at 3 months follow-up [versus 41/92 patients (45%) in the DEFUSE 3 trial thrombectomy group; P = 0.67]. Of the DAWN-ineligible and DEFUSE 3-ineligible patients who underwent thrombectomy, 38% (16/42) and 41% (7/17) of patients reached a mRS ≤2, respectively. CONCLUSION: The results of the DAWN and DEFUSE 3 trials were externally validated in a UOS cohort where the trials' selection criteria identified a similar proportion of responders to thrombectomy.

Coeliac disease presenting as severe hypoglycaemia in youth with type 1 diabetes.

BACKGROUND: Coeliac disease is an autoimmune disorder classically characterized by gastrointestinal symptoms and poor growth. The disease can be difficult to recognize in patients with Type 1 diabetes mellitus. Some clinicians find treatment of the disease in asymptomatic individuals controversial. CASE REPORTS: Two adolescent female patients with Type 1 diabetes experienced recurrent hypoglycaemic seizures. Neither patient reported gastrointestinal symptoms or poor growth. After diagnosis and treatment of coeliac disease, hypoglycaemia resolved. CONCLUSION: These cases illustrate how frequent unexplained severe hypoglycaemia can be an atypical presentation of coeliac disease in youth with Type 1 diabetes. Furthermore, they emphasize the importance of screening and treatment of coeliac disease in asymptomatic patients with Type 1 diabetes. Although controversial, management of coeliac disease in these asymptomatic patients can prevent the vicious cycle of recurrent hypoglycaemia and decrease risk for morbidity and death.

Structural bases for central nervous system malfunction in the quaking mouse: dysmyelination in a potential model of schizophrenia.

The dysmyelinating mouse mutant quaking (qk) is thought to be a model of schizophrenia based on diminution of CNS myelin (Andreone et al., 2007) and downregulation of the Qk gene (Haroutunian et al., 2006) in the brains of schizophrenic patients. The purpose of this study was to identify specific structural defects in the qk mouse CNS that could compromise physiologic function and that in humans might account for some of the cognitive defects characteristic of schizophrenia. Ultrastructural analysis of qk mouse CNS myelinated fibers shows abnormalities in nodal, internodal, and paranodal regions, including marked variation in myelin thickness among neighboring fibers, spotty disruption of paranodal junctions, abnormal distribution of nodal and paranodal ion channel complexes, generalized thinning and incompactness of myelin, and on many axonal profiles complete absence of myelin. These structural defects are likely to cause abnormalities in conduction velocity, synchrony of activation, temporal ordering of signals, and other physiological parameters. We conclude that the structural abnormalities described are likely to be responsible for significant functional impairment both in the qk mouse CNS and in the human CNS with comparable myelin pathology.

A preliminary investigation of Beta-hCG expression in patients with osteosarcoma.

There are eight reported cases in the literature of osteosarcomas secreting ß-hCG. Our primary aim was to investigate the rate of ß-hCG expression in osteosarcoma and attempt to understand the characteristics of osteosarcomas that secrete ß-hCG. We reviewed 37 histopathology slides (14 biopsies and 23 surgical specimens) from 32 patients with osteosarcoma. The slides were retrospectively stained for ß-hCG expression. Patient and tumour characteristics, including age, gender, tumour location, subtype, proportion of necrosis, presence of metastases and recurrence were recorded. A total of five of the 32 tumours were found to be positive for ß-hCG expression (one strongly and four weakly). This incidence of this expression was found in tumours with poor histological response to neoadjuvant chemotherapy. The use of ß-hCG expression as a diagnostic, prognostic or follow-up marker is questionable and needs further investigation with a larger sample size.

Intraoperative translabial ultrasound for urethral diverticula: a road map for surgeons.

PURPOSE: To highlight the importance of intraoperative translabial ultrasound, for identification of diverticular neck allowing complete resection of periurethral diverticula and decrease in the recurrence rate. MATERIAL AND METHODS: This study included 4 women of age range between 38 and 68 years presenting for recurrent urinary tract infections and urethral pain. All had translabial urethral ultrasound and cystoscopy with and without U/C guidance. RESULTS: Prior cystoscopy in all these patients failed to demonstrate the diverticulum. Translabial ultrasound showed the diverticula some of which were infected. Ultrasound was used intraoperatively to guide the surgeon. With this approach the abnormality was confirmed and the neck of the diverticulum was identified through percutaneous needle insertion. This allowed complete resection of the diverticula. CONCLUSION: Translabial ultrasound is a non-invasive technique that plays a major role in examining the urethra and identifying the periuthral diverticula. In our experience, it was very useful as an adjunct to guide the surgeon intraoperatively allowing complete excision of the diverticulum.

Postnatal betamethasone vs dexamethasone in premature infants with bronchopulmonary dysplasia: a pilot study.

OBJECTIVE: As effects of glucocorticoids differ with respect to preparation, dose and duration, we hypothesized that a postnatal regimen of a low-dose, short-course betamethasone treatment had comparable efficacy and a better safety profile compared to the conventional high-dose, dexamethasone. STUDY DESIGN: To test our hypothesis, we selected premature neonates with a birth weight 10 postnatal days with an FiO(2)>0.4 and no ability to wean mechanical support for >or=3 consecutive days. These neonates either received twice daily dexamethasone 0.25 mg kg(-1) per dose intravenously for 3 days tapered to 0.125 mg kg(-1) per dose for 4 days (June 1999 to December 2000) or betamethasone 0.125 mg kg(-1) per day intramuscularly once per day for 3 days (January 2001 to December 2002). RESULT: We found a significant reduction in FiO(2) after 3 days in both glucocorticoid treatment groups. There were no significant differences between the two treatment groups in the clinical parameters including decrease in FiO(2), oxygenation index, mean airway pressure and percent extubation. Duration of ventilation, number of oxygen days and length of hospital stay were comparable in the two groups. Of particular interest, the betamethasone group showed fewer adverse effects, such as poor weight gain and high blood glucose, than the dexamethasone group. CONCLUSION: A short course of low-dose betamethasone has comparable efficacy and seemingly a better short-term safety profile compared to conventional dexamethasone treatment.

Oral bisphosphonates in treatment of transient osteoporosis.

Transient osteoporosis is a rare clinical syndrome of unknown etiology. It is believed that this syndrome is self-limiting; however, the data in the literature support the use of anti-resorptive agents that may reduce pain and decrease the duration of the illness. Herein, we describe two cases of transient osteoporosis of the hip and one case of transient osteoporosis of the knee where the use of oral bisphosphonates provided successful objective and subjective outcome.

Reversible encephalopathy associated with cholesterol embolism syndrome: magnetic resonance imaging and pathological findings.

We describe a patient found to have acute diffuse and reversible encephalopathy on magnetic resonance imaging (MRI) associated with cholesterol emboli syndrome (CES). The initial MRI showed extensive white matter, basal ganglia and cortical damage without evidence of brain infarction. Dramatic clinical and MRI improvement was observed with corticosteroids. Pathologically, cholesterol crystal emboli were found in the lumen of skin and brain arteries and were associated with varying degrees of inflammation of the arteriole wall. This case suggests that CES may be responsible for extensive, acute and reversible encephalopathy underlined by an inflammation of brain arteries.

[A case of alcohol-induced ketoacidosis?]. / Une acidocétose alcoolique?

We report a case of severe ketoacidosis. Initially the patient showed metabolic acidosis, the anion gap was positive and there was neither hyperlactatemia nor intoxication with acid substances. As the rate of glycemia was high (17.8 mmol/L), the diagnosis of diabetic ketoacidosis was proposed. Under treatment with continuous IV injection of insulin, hypoglycemia (1.8 mmol/L) appeared rapidly, while urine bioreactive test was positive for ketonuria, but negative for glycosuria. We finally concluded that it was an alcoholic ketoacidosis. The history of the patient confirmed the diagnosis : chronic alcoholism with recent increased of alcohol intake which provoked vomiting and fasting. This case report shows the difficulty in distinguishing between alcoholic ketoacidosis and diabetic ketoacidosis. We discuss the diagnostic strategy and particularly biologic data in the light of pathophysiologic mechanism of alcoholic ketoacidosis.

Frequency of the CCR5-delta 32 chemokine receptor gene mutation in the Lebanese population.

A direct correlation between HIV infection and mutation in the chemokine receptor (CCR5) gene has been established. However, such correlation has never been investigated in Lebanon. We report the frequency of the CCR5-delta 32 mutation in a random sample of 209 healthy, HIV-1 seronegative Lebanese aged 19-68. Overall, 4.8% were heterozygous for the mutation. Homozygosity was absent from our sample. The frequency for the CCR5-delta 32 allele was 2.5%. Distribution of the mutation was unaffected by sex, age, religion or educational level. The frequency in the Lebanese population is consistent with that in the origin of the mutation in northern Europe. This could be attributed to a gene flow into the Middle East from northern Europe.

[Hemophagocytosis associated with an Escherichia coli sepsis: a case report]. / Syndrome d'activation macrophagique associé à une septicémie à Escherichia coli: à propos d'un cas.

INTRODUCTION: Hemophagocytic lymphohistiocytosis syndrome (HLS) is defined by activated macrophage proliferation. These cells phagocyte the blood elements. This syndrome can be primary as an autosomal recessive disease or secondary to neoplasia, immune diseases or infections-viral, parasitary or bacterian. CASE: Our case concerns an association of HLS and Escherichia coli (E. coli) sepsis in a metastatic prostatic cancer. The evolution was rapidly improved by antibiotics alone. The clinical and biological aspects as well as the differential diagnosis are discussed. CONCLUSION: The HLS is fatal. It can be caused by a severe infection, even an E. coli sepsis. The treatment focused on etiology can be sufficient.