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Chronic thromboembolic pulmonary hypertension is a potentially curable form of pre-capillary pulmonary hypertension (PH) resulting from incomplete resolution of pulmonary thromboemboli. We describe an 11-year-old boy with homozygous sickle cell disease with an indwelling catheter found to have severe PH on routine screening echocardiography. The diagnosis was confirmed by CT, ventilation-perfusion scintigraphy, and right heart catheterization. The patient was medically managed until undergoing pulmonary thromboendarterectomy with resolution of his PH. This case highlights the need for pediatric providers to be aware of this underdiagnosed form of PH, particularly for patients at high risk.Background Developing countries are profoundly affected by the burden of congenital heart disease (CHD) because of limited resources, poverty, cost, and inefficient governance. The outcome of pediatric cardiac surgery in developing countries is suboptimal, and the availability of sustainable programs is minimal. Aim This study describes the establishment of a high quality in-situ pediatric cardiac surgery program in Lebanon, a limited resource country. Methods We enrolled all patients operated for CHD at the Children's Heart Center at the American University of Beirut between January 2014 and December 2018. Financial information was obtained. We established a partnership between the state, private University hospital, and philanthropic organizations to support the program. Results In 5 years, 856 consecutive patients underwent 993 surgical procedures. Neonates and infants constituted 22.5 and 22.6% of our cohort, respectively. Most patients (82.6%) underwent one cardiac procedure. Our results were similar to those of the Society of Thoracic Surgeons (STS) harvest and to the expected mortalities in RACHS-1 scores with an overall mortality of 2.8%. The government (Public) covered 43% of the hospital bill, the Philanthropic organizations covered 30%, and the Private hospital provided a 25% discount. The parents' out-of-pocket contribution included another 2%. The average cost per patient, including neonates, was $19,800. Conclusion High standard pediatric cardiac surgery programs can be achieved in limited-resource countries, with outcome measures comparable to developed countries. We established a viable financial model through a tripartite partnership between Public, Private, and Philanthropy (3P system) to provide high caliber care to children with CHD.Persistent pulmonary hypertension of the newborn (PPHN) is one of the main causes of neonatal morbidity and mortality. It is characterized by sustained elevation of pulmonary vascular resistance (PVR), preventing an increase in pulmonary blood flow after birth. The affected neonates fail to establish blood oxygenation, precipitating severe respiratory distress, hypoxemia, and eventually death. Inhaled nitric oxide (iNO), the only approved pulmonary vasodilator for PPHN, constitutes, alongside supportive therapy, the basis of its treatment. However, nearly 40% of infants are iNO resistant. The cornerstones of increased PVR in PPHN are pulmonary vasoconstriction and vascular remodeling. A better understanding of PPHN pathophysiology may enlighten targeted and more effective therapies. Sildenafil, prostaglandins, milrinone, and bosentan, acting as vasodilators, besides glucocorticoids, playing a role on reducing inflammation, have all shown potential beneficial effects on newborns with PPHN. Furthermore, experimental evidence in PPHN animal models supports prospective use of emergent therapies, such as soluble guanylyl cyclase (sGC) activators/stimulators, l-citrulline, Rho-kinase inhibitors, peroxisome proliferator-activated receptor-γ (PPAR-γ) agonists, recombinant superoxide dismutase (rhSOD), tetrahydrobiopterin (BH4) analogs, ω-3 long-chain polyunsaturated fatty acids (LC-PUFAs), 5-HT2A receptor antagonists, and recombinant human vascular endothelial growth factor (rhVEGF). selleck inhibitor This review focuses on current knowledge on alternative and novel pathways involved in PPHN pathogenesis, as well as recent progress regarding experimental and clinical evidence on potential therapeutic approaches for PPHN.Inflammatory bowel disease (IBD), most commonly known as Crohn's disease (CD) and ulcerative disease (UC), is a chronic and relapsing intestinal disease which cannot be cured completely. The prevalence of IBD in Europe and in North America has increased over the past 20 years. As most IBD patients are young at onset, their quality of life (QOL) can be influenced to varying degrees. Thus, current treatment goals are typically focused on preventing complications, including maintaining clinical remission and improving the QOL. Adjuvant therapies have been widely concerned as an effective treatment in alleviating IBD symptoms, including dietary intervention, traditional Chinese medicine, smoking, alcohol, and physical activities. This review focuses on different ancillary therapies for IBD treatments, in particular the mechanism of reducing inflammation based on the actual data from research studies. Moreover, comparing the latest data, this review also presented potential future prospect for adjuvant therapies.
Choristoma is a non-neoplastic growth of normal tissues in non-indigenous site of origin. Osseous Choristoma is an ectopic bone formation which is a rare entity with 72 cases has been reported in the tongue, 15 cases in the buccal mucosa, 1 case in the lower lip.
A 43 year old male patient experienced a mild, intermittent, dull aching type in the upper lip. An excisional biopsy was done by placing a single incision under local anesthesia. To our knowledge case is first to be reported in the upper lip as a result of reactive pathogenesis.
As per literature, surgical excision is the treatment of choice for osseous Choristoma. Malignant transformation has not been reported yet.
As per literature, surgical excision is the treatment of choice for osseous Choristoma. Malignant transformation has not been reported yet.
Extra-pulmonary tuberculosis (EPTB) arising in extra-oral region in head and neck are rare, and when swellings arise from other sites such as infraorbital region, cheek, etc, tuberculosis is not usually considered for the differential diagnosis (DD) and often the diagnosis is missed and appropriate treatment is delayed.
We report a rare entity of primary tuberculosis, which presented as infraorbital swelling and our technique of performing sublabial approach to the swelling with endoscopic guided excision of the swelling and also we have review of literature of similar cases of primary tuberculosis presenting as swelling over the face over the past 5 year.
Primary EPTB should be considered as DD in cases of chronic facial swelling.
Primary EPTB should be considered as DD in cases of chronic facial swelling.
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