Inherited pulmonary cylindromas: extending the phenotype of CYLD mutation carriers.

BACKGROUND: Germline mutations in the tumour suppressor gene CYLD are recognized to be associated with the development of multiple cutaneous cylindromas. We encountered such a patient who presented with breathlessness because of multiple pulmonary cylindromas. OBJECTIVES: To search for clinical and radiological features of multiple pulmonary cylindromas in a cohort of 16 patients with CYLD mutations. METHODS: A retrospective case-note review was carried out in a tertiary dermatogenetics clinic where CYLD mutation carriers are reviewed on an annual basis. In-depth investigation was carried out for patients with pulmonary tumours. RESULTS: Four patients had radiological imaging of their lungs, of which two had multiple pulmonary cylindromas that were confirmed histologically. Serial computed tomography monitoring allowed for pre-emptive endobronchial laser ablation, preventing major airway obstruction and pulmonary collapse. CONCLUSIONS: Pulmonary cylindromas are an unrecognized, but infrequently symptomatic, aspect of the phenotype in these patients that can have implications for patient care. They should be considered in patients with a high tumour burden that present with respiratory symptoms, and where appropriate, monitored with serial imaging.

Evaluation of the role of angiogenic factors in the pathogenesis of schistosomiasis.

Schistosomiasis is one disease produced by helminths, which affect many people in tropical areas. Granuloma formation is the main mechanism involved in the pathogenesis of this disease. Experimental studies have demonstrated angiogenesis (blood vessels formation from pre-existing vessels) in the initial phase of granuloma formation. In the present work, VEGF (vascular endothelial growth factor) levels were analyzed in sera from people diagnosed with different helminthic infections. Patients with schistosomiasis and filariasis had significantly high VEGF levels in compared with healthy people and patients diagnosed with hookworms. In addition, the effects of angiogenesis inhibition using anti-angiogenic factors (endostatin) were evaluated in a schistosomiasis murine model. A lesion decrease was observed in mice infected with Schistosoma mansoni and treated with endostatin. Finally, mechanisms of angiogenesis induction were studied and observed that cercariae antigens stimulated the angiogenic factors by host alveolar macrophages.

Biochemical parameters and pathogenesis of SARS-CoV-2 infection in vital organs: COVID-19 outbreak in Iran.

Since its emergence, severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) has spread worldwide, and led to ever-increasing mortality. SARS-CoV-2 infection perturbs the function of the body's vital organs, making patients of all ages susceptible to the disease. Nevertheless, individuals developing critical illness with poor outcomes were mostly the elderly and people with co-morbid conditions, who constituted the vast majority of coronavirus disease 2019 (COVID-19) fatalities. Complications of COVID-19 mostly involve the respiratory, renal and cardiovascular systems, and in severe cases secondary infections leading to pneumonia and acute respiratory distress syndrome, which may precede the death of the patient. Multi-organ failure in individuals with COVID-19 could be a consequence of their co-morbidities. A patient's pre-existing conditions may affect the disease prognosis, requiring immediate attention to accurately detect and evaluate them in SARS-CoV-2-infected individuals. This review addresses several issues in relation to manifestations of the body's vital organs along with potential diagnostic blood factors in SARS-CoV-2 infection. It is hoped that the review will lead to more comprehensive understanding of this complex disease.

Trichinella: differential expression of angiogenic factors in macrophages stimulated with antigens from encapsulated and non-encapsulated species.

The newborn larval stage of Trichinella spiralis enters the host striated skeletal muscle cell resulting in the formation of the nurse cell. Vascular Endothelial Growth Factor (VEGF) was detected in cells in the area immediately surrounding the nurse cells. However, no data are available on the antigens involved, the role of other angiogenic factors or the relationship of angiogenesis with Nitric Oxide (NO) production. Using macrophage cell culture we study the effect of different Trichinella L1 antigens from one encapsulated (T. spiralis) and one non-encapsulated (Trichinellapseudospiralis) on the expression of VEGF and basic Fibroblast Growth Factor (FGF2). Also, we investigate the relationship between the production of NO and angiogenic mediators. The results show that encapsulated and non-encapsulated Trichinella species are different in their capacity to stimulate the expression of VEGF and FGF2 from host macrophages. Finally, there is no relationship between angiogenic factors and NO production by T. spiralis antigen.

A solution for enhancement of transient performance in nonlinear adaptive control: Optimal adaptive reset based on barrier Lyapunov function.

In this paper, an adaptive controller based on barrier Lyapunov function combined with an optimal reset rule is devised in order to improve the transient performance of nonlinear adaptive control. A novel reset rule is designed such that the estimated parameters of the adaptive controller jump to the optimal values in a way that optimizes a cost function representing the transient performance index. It is proved that asymptotic tracking is achieved and the output remains in a desired bound by ensuring boundedness of the barrier Lyapunov function. Besides, the convergence rate is increased by resetting the estimated parameters to optimal values. A regularly referred example is simulated to demonstrate the effectiveness of the proposed method and the results are compared with the existing investigations.

Therapeutic effects of oral liothyronine on aluminum phosphide poisoning as an adjuvant therapy: A clinical trial.

BACKGROUND: In aluminum phosphide (AlP) poisoning, death is mainly due to cardiovascular failure and refractory acute heart failure. There is a lot of evidence showing thyroid hormones have cardioprotective effects. OBJECTIVE: The purpose of this study was to evaluate the effect of oral liothyronine in the treatment of AlP poisoning. METHODS: Twenty-four patients from intensive care unit of Baharloo Hospital, Tehran, Iran, were included based on the inclusion and exclusion criteria. They were randomly divided into two parallel groups of 12 cases and 12 controls. Intervention in the case group was administration of 50 µg liothyronine via nasogastric tube after gastric lavage, in the first 6 h of poisoning. In both groups, the routine treatment of AlP poisoning was performed. Blood samples were prepared at the beginning of the study and after 12 h. Patients were followed up till discharge from the hospital or death. RESULTS: The findings demonstrated that oral liothyronine was able to significantly improve systolic blood pressure, arterial blood pH, and total thiol molecules and also could decrease lipid peroxidation, increase catalase activity, and prevent further decline in total antioxidant capacity. CONCLUSION: Liothyronine administration is effective in controlling AlP poisoning and can improve patients' outcome.

Three-year outcome of endovascular treatment of superficial femoral artery occlusion.

HYPOTHESIS: Patency after primary percutaneous transluminal angioplasty (PTA) and stenting of superficial femoral artery (SFA) occlusions is better than historical experience with PTA alone. DESIGN: Consecutive case series of primary PTA with stenting, and follow-up with duplex imaging every 6 months (mean +/- SD follow-up, 32 +/- 15 months). SETTING: Veterans Affairs medical center. PATIENTS AND METHODS: Patients were 57 previously untreated men with 71 limbs having chronic atherosclerotic SFA occlusion with suprageniculate reconstitution and patent tibial runoff. Critical ischemia (Society for Vascular Surgery [SVS] category, 4-6) was present in 7 (10%), the remainder had intermittent claudication only (SVS, 1-3). INTERVENTIONS: Guidewire recanalization followed by PTA, Wallstent deployment, and adjunctive thrombolysis as necessary; 19 limbs (27%) required thrombolysis to manage periprocedural thrombosis. MAIN OUTCOME MEASURES: Cumulative patency, limb salvage, and complications. RESULTS: Length (mean +/- SD) of occlusion was 14.4 +/- 9.9 cm. Length of stented artery was 24.3 +/- 11.1 cm. Ankle brachial index increased from 0.59 +/- 0.14 to 0.86 +/- 0.16 (P<.001) after stenting. One- and 3-year patencies were as follows: primary, 54.6% +/- 6.3% and 29.9% +/- 6.6%; assisted primary, 72.3% +/- 5.6% and 59.0% +/- 6.8%; and secondary, 81.6% +/- 4.8% and 68.3% +/- 6.5%. Three-year secondary patency when periprocedural thrombolysis was required was 35.7% +/- 12.5% compared with 70.6% +/- 7.4% for limbs not requiring periprocedural thrombolysis (P=.02); the differences in occlusion length and severity of ischemia were not significant between these 2 groups. Limbs undergoing adjunctive PTA during angiography 6 to 12 months after initial stenting had 63.0% +/- 13.3% patency at 3 years compared with 100% patency in limbs not requiring PTA at 6 to 12 months angiography (P=.046). Periprocedural mortality and morbidity were 2.8% and 15.5%, respectively. Three of the 7 limbs with critical ischemia underwent amputation during follow-up compared with 2 (3%) of 64 limbs with functional ischemia (chi(2) test, P<.006). A mean of 1.8 endovascular interventions per limb were performed. CONCLUSIONS: Percutaneous transluminal angioplasty and stenting yielded higher patency rates than historical controls undergoing PTA alone. When periprocedural thrombolysis is required, subsequent patency appears to be significantly worse. Poor results after PTA and stenting of limbs with critical ischemia and the need for additional endovascular therapy limit the technique's utility.

Spinal cord injury increases the risk of abdominal aortic aneurysm.

We have observed apparently disproportionate numbers of abdominal aortic aneurysms (AAAs) in chronic spinal cord injury (SCI) patients. To test whether aortic enlargement is more frequent in SCI, we measured maximum infrarenal aortic diameters (AoDmax) by B mode ultrasound in 89 SCI subjects and 223 age and sex matched controls. The average AoDmax in SCI subjects (mean age 60.3 years) was 2.27+/-0.80 cm compared to 2.07+/-0.69cm in the controls. This difference was significant (P = 0.023), as were the proportions of subjects with AoDmax >/- 3cm. A total of 20.2 per cent of the SCI group had AoDmax >/- 3cm compared to 8.9 per cent for the controls (P < 0.0001, chi-square). Race, height, and weight distributions were similar. SCI patients had lower levels of hypertension and cigarette smoking than controls. Within the SCI and control groups, subjects with AoDmax >/- 3cm had increased cigarette consumption compared to /- 3cm group than in the >/- 3cm group, T6 versus T8, but not significantly (P = 0.23)> Based on these results, SCI patients have over a two-fold risk of aortic enlargement as a consequence of spinal cord injury.