Bartsocas-Papas syndrome with fusion of the lips and posterior fusion defects of the thoracic vertebrae.

Bartsocas-Papas syndrome is a rare popliteal pterygial syndrome with multiple anomalies including microcephaly, facial clefts, filiform bands, ankyloblepharon, syndactyly, and other ectodermal anomalies. Affected infants usually die perinatally. The authors present an 8-month-old female infant with manifestations of this syndrome and some additional traits including fusion of the lips, intraoral filiform bands, alopecia totalis, and posterior fusion failure of the vertebrae. The fused lips were opened by incising the fibrotic bands closing her mouth. Details of this patient and a brief review of the literature is presented.     

Unusual case of difficult double-lumen endotracheal tube removal

A reusable Robertshaw red rubber double-lumen endotracheal tube (DLT) was placed to facilitate lung isolation for thoracoscopy in a 49-year-old atopic patient. In spite of its smooth insertion, it was then not possible to remove the DLT. Direct laryngoscopy showed severe laryngeal edema. After 48 hours of medical treatment with steroids, the trachea was extubated. The laryngeal edema could have been the result of physical and chemical irritation by the reusable rubber DLT itself, or from the substances formed during repeated cleaning and sterilization of the DLT. Atopic patients who are prone to developing latex allergy are also more liable to develop severe reactions to chemical, mechanical, and physical irritation from reusable red rubber DLTs or from the chemical solution used for its cleaning and sterilization.     

Oxidative stress, glucose metabolism, and the prevention of type 2 diabetes: pathophysiological insights

With the rising epidemic of type 2 diabetes worldwide, including the United States, the death and disability due to the suboptimal control of cardiovascular disease associated with this epidemic has made prevention of type 2 diabetes emerge as a primary strategic intervention. Several modalities have been assessed in large randomized controlled trials for diabetes prevention such as lifestyle interventions and various pharmacologic agents. Included in these agents are metformin, thiazolidinediones, acarbose, angiotensin converting enzyme inhibitors, as well as angiotensin receptor blockers. Abrogation of oxidative stress appears to be a common soil hypothesis that explains the favorable effects of these agents on glucose metabolism, including the prevention of diabetes and its complications. This comprehensive review highlights the role of oxidative stress in the pathogenesis of diabetes, with emphasis on the major clinical trials conducted on prevention of type 2 diabetes.     

The other 'neglected' eradication programme: achieving the final mile for Guinea worm disease eradication?

Guinea worm disease is one of two diseases targeted for eradication, the other being polio. Since the late 1980s, the number of new cases per year has been reduced from approximately one million to some 25 000 in 2006. However, there was an increase from 2005 owing to improved surveillance in Sudan and problems in Ghana. The International Commission argues that more resources are required to ensure that the goal of eradication is completed. Elimination of transmission throughout Asia has now been confirmed and the disease is now confined to a small number of African countries requiring increased efforts to achieve the global goal.     

G gamma and A gamma globin-chain biosynthesis by adult and umbilical cord blood erythropoietic bursts and reticulocytes

We examined gamma-globin-chain biosynthesis by adult and umbilical cord blood or erythropoietic bursts in methylcellulose clonal culture and gamma-chain synthesis by cord blood reticulocytes. Globin chains were labeled with 14C-amino acids and guantitated by using autoradiography or fluorography. Alpha, beta, and G gamma and A gamma chains were separated by isoelectric focusing in polyacrylamide gels containing 8 M urea and 3% Nonidet P-40 (a nonionic detergent). Time course examinations of the gamma-chains synthesized by the bursts revealed no changes in the G gamma:A gamma ratio between days 10 and 18 of culture. The ratio of G gamma/(G gamma + A gamma) in cultures of adult circulating erythroid precursors was 0.38 +/- 0.09, which corresponds to the known ratio in adult peripheral blood erythrocytes. The relative G gamma-chain biosyntheses in the cord blood bursts and reticulocytes were 0.56 +/- 0.02 and 0.66 +/- .008, respectively. Both are intermediate between the accepted newborn and adult ratios. Natal erythropoietic precursors appear to be in the transitional stage with respect to the switching of G gamma-A gamma ratios.     

Ischemic Conditioning Is Safe and Effective for Octo- and Nonagenarians in Stroke Prevention and Treatment

Symptomatic intracranial arterial stenosis (SIAS) is very common in octo- and nonagenarians, especially in the Chinese population, and is likely the most common cause of stroke recurrence worldwide. Clinical trials demonstrate that endovascular treatment, such as stenting, may not be suitable for octogenarians with systemic diseases. Hence, less invasive methods for the octogenarian patients are urgently needed. Our previous study (unique identifier: {"type":"clinical-trial","attrs":{"text":"NCT01321749","term_id":"NCT01321749"}}NCT01321749) showed that repetitive bilateral arm ischemic preconditioning (BAIPC) reduced the incidence of stroke recurrence by improving cerebral perfusion (confirmed by single photon emission computed tomography and transcranial Doppler sonography) in patients younger than 80 years of age; however, the safety and effectiveness of BAIPC on stroke prevention in octo- and nonagenarians with SIAS are still unclear. The objective of this study was to evaluate the safety and effectiveness of BAIPC in reducing stroke recurrence in octo- and nonagenarian patients with SIAS. Fifty-eight patients with SIAS were enrolled in this randomized controlled prospective study for 180 consecutive days. All patients enrolled in the study received standard medical management. Patients in the BAIPC group (n = 30) underwent 5 cycles consisting of bilateral arm ischemia followed by reperfusion for 5 min each twice daily. Those in the control group (n = 28) underwent sham-BAIPC twice daily. Blood pressure, heart rate, local skin status, plasma myoglobin, and plasma levels of thrombotic and inflammatory markers were documented in both groups before beginning the study and for the first 30 days. Finally, the incidences of stroke recurrence and magnetic resonance imaging during the 180 days of treatment were compared. Compared with the control, BAIPC had no adverse effects on blood pressure, heart rate, local skin integrity, or plasma myoglobin, and did not induce cerebral hemorrhage in the studied cohort. BAIPC reduced plasma high sensitive C-reactive protein, interleukin-6, plasminogen activator inhibitor-1, leukocyte count, and platelet aggregation rate and elevated plasma tissue plasminogen activator (all p < 0.01). In 180 days, 2 infarctions and 7 transient ischemic attacks were observed in the BAIPC group compared with 8 infarctions and 11 transient ischemic attacks in the sham BAIPC group (p < 0.05). BAIPC may safely inhibit stroke recurrence, protect against brain ischemia, and ameliorate plasma biomarkers of inflammation and coagulation in octo- and nonagenarians with SIAS. A multicenter trial is ongoing.Clinical Trial Registration: www.clinicaltrials.gov, unique identifier: {"type":"clinical-trial","attrs":{"text":"NCT01570231","term_id":"NCT01570231"}}NCT01570231.

Electronic supplementary material

The online version of this article (doi:10.1007/s13311-015-0358-6) contains supplementary material, which is available to authorized users.Key Words: Remote ischemic preconditioning, octogenarian, nonagenarians, intracranial arterial stenosis, stroke, recurrence