Assessing Post-Marketing Requirements for Orphan Drugs: A Cross-Sectional Analysis of FDA and EMA Oversight.

The U.S. Food and Drug Administration (FDA) and the European Medicines Agency (EMA) oversee pharmaceutical regulations, including orphan drugs targeting rare diseases with limited patient populations.

Surgical Management of Total Sternal Cleft in a Pentalogy of Cantrell Neonate.

Sternal cleft is a rare malformation with significant morbidity and mortality. It has been associated with other midline fusion defects, most significantly Cantrell's pentalogy, involving the sternum, pericardium, heart, diaphragm, and abdominal wall. This study reported a successfully managed case of a newborn with a total sternal cleft and Cantrell's pentalogy.

Surgical treatment for mediastinitis after endobronchial ultrasound-guided transbronchial needle aspiration: 2 case reports.

We report two cases of severe mediastinitis accompanied by abscess due to endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA), that were successfully treated by effective surgical drainage. A 68-year-old woman was referred to our hospital due to chest discomfort and high fever after EBUS-TBNA, and a 54-year-old man was referred due to general weakness, chills, and high fever after the same procedure. Both were diagnosed with EBUS-related mediastinitis and discharged after surgical treatment.

Type A aortic dissection after 'zone 0' thoracic endovascular aortic repair for type 1 hybrid aortic arch replacement of arch aneurysm.

The recent rise in minimally invasive cardiovascular procedures is being accompanied by an increase in related complications. We report on an acute type A aortic dissection performed in an 82-year-old man 1 week after staged 'zone 0' hybrid thoracic endovascular aortic repair (TEVAR). Previously, the patient had undergone type I hybrid arch debranching and staged 'zone 0' TEVAR for an aortic arch aneurysm.

Single-center experience of extracorporeal membrane oxygenation mainly anticoagulated with nafamostat mesilate.

Background: Bleeding remains the chief concern during extracorporeal membrane oxygenation (ECMO). Recently, several studies proposed nafamostat mesilate (NM) as an alternative anticoagulant to heparin due to reduced bleeding complications and comparable thromboembolic episodes. The aim of this study was to evaluate the clinical outcomes of ECMO anticoagulated mainly with NM.

A Life-Threatening Bronchogenic Cyst.

A bronchogenic cyst causing cardiac tamponade is a rare condition. We report an unusual case of a bronchogenic cyst that caused cardiac tamponade. A 49-year-old female patient presented at our emergency room with complaints of palpitations and shortness of breath that had lasted for 5 days preceding the visit.

Carney Complex: Eleven Open Heart Operations in a Single Family.

Herein, we report on a family with Carney complex. Four members of the family underwent a total of 11 open heart operations as well as 9 other operations to treat extrathoracic masses. All the family members met at least 2 major clinical criteria and 1 supplemental criterion.

Anomalous right coronary artery from pulmonary artery discovered incidentally in an asymptomatic young infant.

Isolated anomalous right coronary artery originating from the pulmonary artery (ARCAPA) is a rare congenital coronary anomaly that is asymptomatic and discovered incidentally in most cases. ARCAPA is generally not considered a fatal defect in infancy or childhood, although cases of sudden death have been reported. Here, we report a 2-month-old female infant who presented with a prolonged fever that was determined to be caused by rhinovirus infection.

Classic Peripheral Signs of Subacute Bacterial Endocarditis.

A 50-year-old female patient with visual disturbances was referred for further evaluation of a heart murmur. Fundoscopy revealed a Roth spot in both eyes. A physical examination showed peripheral signs of infective endocarditis, including Osler nodes, Janeway lesions, and splinter hemorrhages.

Extracorporeal Membrane Oxygenation in a 1,360-g Premature Neonate after Repairing Total Anomalous Pulmonary Venous Return.

With advancements in complex repairs in neonates with complicated congenital heart diseases, extracorporeal membrane oxygenation (ECMO) has been increasingly used as cardiac support. ECMO has also been increasingly used for low birth weight (LBW) or very low birth weight (VLBW) neonates. However, since prematurity and LBW are risk factors for ECMO, the appropriate indications for neonates with LBW, especially VLBW, are under dispute.

In Vivo Neuroprotective Effect of Histidine-Tryptophan-Ketoglutarate Solution in an Ischemia/Reperfusion Spinal Cord Injury Animal Model.

Background: Paraplegia is a devastating complication following operations on the thoracoabdominal aorta. We investigated whether histidine-tryptophan-ketoglutarate (HTK) solution could reduce the extent of ischemia/reperfusion (IR) spinal cord injuries in a rat model using a direct delivery method.

Methods: Twenty-four Sprague-Dawley male rats were randomly divided into four groups.

Outcomes of the Tower Crane Technique with a 15-mm Trocar in Primary Spontaneous Pneumothorax.

Background: Video-assisted thoracoscopic surgery (VATS) pulmonary wedge resection has emerged as the standard treatment for primary spontaneous pneumothorax. Recently, single-port VATS has been introduced and is now widely performed. This study aimed to evaluate the outcomes of the Tower crane technique as novel technique using a 15-mm trocar and anchoring suture in primary spontaneous pneumothorax.

Aortic periannular abscess invading into the central fibrous body, mitral valve, and tricuspid valve.

A 61-year-old man was diagnosed with aortic stenoinsufficiency with periannular abscess, which involved the aortic root of noncoronary sinus (NCS) that invaded down to the central fibrous body, whole membranous septum, mitral valve (MV), and tricuspid valve (TV). The open complete debridement was executed from the aortic annulus at NCS down to the central fibrous body and annulus of the MV and the TV, followed by the left ventricular outflow tract reconstruction with implantation of a mechanical aortic valve by using a leaflet of the half-folded elliptical bovine pericardial patch. Another leaflet of this patch was used for the repair of the right atrial wall with a defect and the TV.

Growth of diminutive central pulmonary arteries after right ventricle to pulmonary artery homograft implantation.

Background: The management of tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collateral arteries is controversial because of the wide variability of pulmonary artery (PA) and major aortopulmonary collateral arteries morphology. Several different staged strategies have been used to promote growth of diminutive PA branches. We have preferred a right ventricular (RV)-PA homograft for symmetrical growth of the central PA branches.

Clinical features of deep neck infections and predisposing factors for mediastinal extension.

Background: Deep neck infections (DNI) can originate from infection in the potential spaces and fascial planes of the neck. DNI can be managed without surgery, but there are cases that need surgical treatment, especially in the case of mediastinal involvement. The aim of this study is to identify clinical features of DNI and analyze the predisposing factors for mediastinal extension.

An Isolated True Aneurysm of the Superficial Femoral Artery in a Young Woman - A case report -.

A 39-year-old woman was admitted to the hospital due to a pulsatile mass on her right inner thigh that was evident for two months. She did not exhibit any risk factors of atherosclerosis, no evidence of vasculitis, or any signs of previous trauma history. Ultrasound and computed tomography revealed an adult fist-sized aneurysm on the distal superficial femoral artery.

Stress-induced Cardiomyopathy during Pulmonary Resection (Takotsubo Syndrome) - A case report -.

Stress-induced cardiomyopathy is caused by emotional or physical stressors and mimics acute myocardial infarction, though Stress-induced cardiomyopathy is characterized by reversible left ventricular (LV) apical ballooning in the absence of significant coronary artery disease. We describe a 51-year-old male who underwent left upper lobectomy for non-small cell lung cancer, and during which cardiogenic arrest occurred due to stress-induced cardiomyopathy, successfully managed by intra-aortic balloon pumping and extracorporeal membrane oxygenation.

Transient use of oral bosentan can be an additional option to reduce pulmonary arterial hypertension in a patient with severe pulmonary arterial hypertension associated with atrial septal defect.

Atrial septal defect (ASD) with severe pulmonary arterial hypertension (PAH) is thought to preclude shunt closure. However, there are several reports that vasodilator treatment is associated with good clinical outcome in these patients, recently. We report a case of good clinical outcome in a patient with ASD and severe PAH successfully treated with operative closure of ASD and subsequent use of oral bosentan medication.

Benign metastasizing leiomyoma: metastasis to rib and vertebra.

Benign metastasizing leiomyoma is very rare and characterized by the presence of pelvic, peritoneal, nodal, or pulmonary nodules in women with a history of uterine leiomyomas. We report a case of benign metastasizing leiomyoma in a 30-year-old woman who had undergone a prior myomectomy due to uterine cellular leiomyoma 3 years earlier. The patient had a mass on the right sixth rib and 2 masses in the sixth thoracic vertebra.