Granulocytic sarcoma: An atypical presentation in the oral cavity

Acute myelogenous leukemia (AMU is a hematologic disorder that is characterized by an abnormal proliferation of immature myeloid cells. Granulocytic sarcomas are clusters of leukemic myeloid cells that may develop as a result of AML. Oral manifestations of AML are common and often involve enlargements of the gingiva and/or mucosal tissue from direct leukemia cell infiltration. We describe the case history of a 50-year-old man who had an ulcera-tive lesion of the oral mucosa that was determined to be a granulocytic sarcoma of AML-M0 subtype. The combination of both the subtype and clinical presentation of the leukemia makes this presentation unusual, and to the best of our knowledge, of a type that has not been previously reported in the literature.     

A reduced segment II/III graft for neonatal liver failure with absence of detectable hepatocytes. A case report and literature review

Alawi K, Mitros FA, Bishop WP, Rayhill S, Wu Y. A reduced segment II/III graft for neonatal liver failure with absence of detectable hepatocytes. A case report and literature review.
Pediatr Transplantation 2011: 15:e60–e63. © 2010 John Wiley & Sons A/S. Abstract: When hepatic failure occurs in newborns, most cases are because of viral hepatitis, metabolic diseases, and neonatal hemochromatosis. It is rare to have liver failure presenting in the first day after birth. We report a unique case of a newborn baby with liver failure in the first day of life who received a reduced segment II and III graft when she was 19 days old and became the youngest survivor of LDLT. Common and rare causes of liver failure in this age group were excluded by appropriate testing. She underwent a liver biopsy that showed a liver devoid of hepatocytes. Similar pathological findings were found in the explanted liver. She was discharged from the hospital with normal graft function three months after the transplant.     

The influence of covalent and non-covalent functionalization of GNP based nanofluids on its thermophysical,rheological and suspension stability properties

Covalent functionalization (CF-GNPs) and non-covalent functionalization (NCF-GNPs) approaches were applied to prepare graphene nanoplatelets (GNPs). The impact of using four surfactants (SDS, CTAB, Tween-80, and Triton X-100) was studied with four test times (15, 30, 60, and 90 min) and four weight concentrations. The stable thermal conductivity and viscosity were measured as a function of temperature. Fourier transform infrared spectroscopy (FTIR), thermo-gravimetric analysis (TGA), X-ray diffraction (XRD) and Raman spectroscopy verified the fundamental efficient and stable CF. Several techniques, such as dispersion of particle size, FESEM, FETEM, EDX, zeta potential, and UV-vis spectrophotometry, were employed to characterize both the dispersion stability and morphology of functionalized materials. At ultrasonic test time, the highest stability of nanofluids was achieved at 60 min. As a result, the thermal conductivity displayed by CF-GNPs was higher than NCF-GNPs and distilled water. In conclusion, the improvement in thermal conductivity and stability displayed by CF-GNPs was higher than those of NCF-GNPs, while the lowest viscosity was 8% higher than distilled water, and the best thermal conductivity improvement was recorded at 29.2%.

Covalent functionalization (CF-GNPs) and non-covalent functionalization (NCF-GNPs) approaches were applied to prepare graphene nanoplatelets (GNPs).     

Prevalence, predictors, and outcomes of conservative medical management in non-ST-segment elevation acute coronary syndromes in Gulf RACE-2

We assessed the prevalence, predictors, and in-hospital and long-term outcomes of conservative medical management for patients with non-ST-segment elevation acute coronary syndrome (NSTEACS) compared with percutaneous coronary intervention (PCI) and coronary artery bypass graft surgery (CABG). This prospective study conducted from October 2008 to June 2009 in 65 hospitals from 6 Arabian Gulf countries included 30-day and 1-year mortality follow-up for 3661 patients. Compared with conservative management group (2859 patients; 78.1%), the PCI group (638; 17.4%) had significantly better unadjusted and adjusted in-hospital (odds ratio [OR]: 0.40, 95% confidence interval [CI]: 0.17-0.97), 30-day (OR: 0.44, 95% CI: 0.24-0.76) and 1-year (OR: 0.58, 95% CI: 0.40-0.87) mortality rates. Comparison with the CABG group (164; 4.5%) yielded similar results with inclusion of patients scheduled for CABG after hospital discharge. Independent predictors of conservative medical management were mainly country of residence and history of prior CABG.     

Relationship between white blood cell count and in-hospital outcomes in acute coronary syndrome patients from the Middle East

We evaluated the relationship between admission white blood cell (WBC) count and in-hospital outcomes in acute coronary syndrome (ACS) patients from the Middle East. Data were analyzed from 7806 consecutive patients with ACS who were divided into 4 groups (G) according to their WBC count (× 10(9)/L; G1: < 6.00; G2: 6.00-9.99; G3: 10.00-11.99; G4: ≥ 12.00). After significant covariate adjustment, those in G4 were 68% more likely to have cardiogenic shock than those in G1 (95% confidence interval [CI]: 1.05-2.68; P = .030) and G2 (odds ratio [OR], 2.02; 95% CI: 1.51-2.71; P < .001). Those in G4 were 2.02 times (95% CI: 1.11-3.67; P = .021) and 65% (95% CI: 1.17-2.32; P = .004) more likely to die in hospital than those in G1 and G2, respectively. Admission WBC count is an independent risk factor for in-hospital cardiogenic shock and mortality, in Middle Eastern patients with ACS. Novel therapeutic agents targeting WBCs in patients with ACS may improve outcomes.     

Molecular and Genetic Aspects of Odontogenic Lesions

In this article we outline the molecular findings of select odontogenic tumors. In each section, we briefly review selected the clinicoradiographic, histologic, immunologic features, focusing on the molecular findings and their applications in practice. The understanding of molecular pathobiology at various other organ sites has developed quite rapidly in recent years, however much remains unknown about the genetic profile of odontogenic tumors. Improved understanding of mutations in odontogenic tumors may clarify classification schema and elucidate targets for novel therapies. Molecular testing will no doubt improve our understanding of odontogenic tumor pathogenesis and will likely be, someday, an important component of routine clinical practice and its role will only increase in the coming years.     

An AP4B1 frameshift mutation in siblings with intellectual disability and spastic tetraplegia further delineates the AP-4 deficiency syndrome

The recently proposed adaptor protein 4 (AP-4) deficiency syndrome comprises a group of congenital neurological disorders characterized by severe intellectual disability (ID), delayed or absent speech, hereditary spastic paraplegia, and growth retardation. AP-4 is a heterotetrameric protein complex with important functions in vesicle trafficking. Mutations in genes affecting different subunits of AP-4, including AP4B1, AP4E1, AP4S1, and AP4M1, have been reported in patients with the AP-4 deficiency phenotype. We describe two siblings from a non-consanguineous couple who presented with severe ID, absent speech, microcephaly, growth retardation, and progressive spastic tetraplegia. Whole-exome sequencing in the two patients identified the novel homozygous 2-bp deletion c.1160_1161delCA (p.(Thr387Argfs*30)) in AP4B1. Sanger sequencing confirmed the mutation in the siblings and revealed it in the heterozygous state in both parents. The AP4B1-associated phenotype has previously been assigned to spastic paraplegia-47. Identification of a novel AP4B1 alteration in two patients with clinical manifestations highly similar to other individuals with mutations affecting one of the four AP-4 subunits further supports the observation that loss of AP-4 assembly or functionality underlies the common clinical features in these patients and underscores the existence of the clinically recognizable AP-4 deficiency syndrome.