• Journal of Chest Surgery
• /
• v.20 no.2
• /
• pp.404-410
• /
• 1987

An 18-day-old male neonate with hypoplastic left heart syndrome underwent surgical intervention by modification of the Norwood procedure on September 23, 1986. Hypoplastic left heart syndrome is a serious congenital cardiac anomaly that has a fatal outlook if left untreated. Included in this anomaly are [1] aortic valve atresia, and hypoplasia of the ascending aorta and aortic arch, [1] mitral valve atresia or hypoplasia, and [3] diminutive or absent left ventricle. Patent ductus arteriosus is essential for any survival, and there is usually a patent foramen ovale. Coarctation of the aorta is frequently associated with the lesion.z With a limited period of cardiopulmonary bypass, deep hypothermia, and circulatory arrest, the ductus arteriosus was excised. The main pulmonary artery was divided immediately below its branches, and the distal stump of the divided pulmonary artery was closed with a pericardial patch. The aortic arch was incised, and a 1 5mm tubular Dacron prosthesis was inserted between the main pulmonary artery and the aortic arch. A 4mm shunt of polytetrafluoroethylene graft was established between the new ascending aorta and the right pulmonary artery to provide controlled pulmonary blood flow. Following rewarming, the heart started to beat regularly, but the patient could not be weaned from cardiopulmonary bypass. At autopsy, the patient was found to have hypoplasia of the aortic tract complex with mitral atresia and aortic atresia. A secundum atrial septal defect was noted. Right atrial and ventricular hypertrophy was present, and the left ventricle was entirely absent. Although unsuccessful in this case report, continuing experience with hypoplastic left heart syndrome will lead to an improvement in result.

• Clinical and Experimental Pediatrics
• /
• v.50 no.5
• /
• pp.493-496
• /
• 2007

Diamino-diphenyl-sulfone (Dapsone) is widely used in the treatment of leprosy and a variety of blistering skin diseases. It sometimes has adverse side effects with common usual doses, such as skin, nervous system, gastrointestinal tract, liver, kidney and hematologic toxicity. One of these side effects is a rare but serious hypersensitivity reaction called dapsone syndrome, which occurs several weeks after the initial administration of the drug and results in unpredictable, sometimes fatal outcomes. This report deals with a 13-year-old girl's case with typical features of dapsone syndrome that included fever, exfoliative dermatitis, jaundice, hemolytic anemia and pleural effusion after being treated with dapsone for four weeks.

• Tuberculosis and Respiratory Diseases
• /
• v.58 no.1
• /
• pp.73-77
• /
• 2005

Lemierre syndrome is characterized by an acute oropharyngeal infection with secondary septic thrombophlebitis of the internal jugular vein and frequent metastatic infections such as septic pulmonary emboli and suppurative arthritis. In the preantibiotic era, this condition generally had a fatal outcome. The presentation is so distinctive that a clinical diagnosis is possible in most cases, and a cure is expected with the appropriate therapy in the majority of patients. We present a case report of Lemierre syndrome with a review of the relevant literature.

• Journal of Dental Anesthesia and Pain Medicine
• /
• v.23 no.1
• /
• pp.45-51
• /
• 2023

Andersen-Tawil syndrome (ATS) is a rare genetic disease characterized by a triad of episodic flaccid muscle weakness, ventricular arrhythmias, and physical anomalies. ATS patients have various cardiac arrhythmias that can cause sudden death. Implantation of an implantable cardioverter-defibrillator (ICD) is required when life-threatening cardiac arrhythmias do not respond to medical treatment. An 11-year-old girl underwent surgery for an ICD implantation. For general anesthesia in ATS patients, anesthesiologists should focus on the potentially difficult airway, serious cardiac arrhythmias, such as ventricular tachycardia (VT), and delayed recovery from neuromuscular blockade. We followed the difficult airway algorithm, avoided drugs that can precipitate QT prolongation and fatal cardiac arrhythmias, and tried to maintain normoxia, normocarbia, normothermia, normoglycemia, and pain control for prevention of sympathetic stimulation. We report the successful application of general anesthesia for ICD implantation in a pediatric patient with ATS and recurrent VT.

• Tuberculosis and Respiratory Diseases
• /
• v.74 no.2
• /
• pp.51-55
• /
• 2013

Acute eosinophilic pneumonia is a severe and rapidly progressive lung disease that can cause fatal respiratory failure. Since this disease exhibits totally different clinical features to other eosinophilic lung diseases (ELD), it is not difficult to distinguish it among other ELDs. However, this can be similar to other diseases causing acute respiratory distress syndrome or severe community-acquired pneumonia, so the diagnosis can be delayed. The cause of this disease in the majority of patients is unknown, even though some cases may be caused by smoke, other patients inhaled dust or drugs. The diagnosis is established by bronchoalveolar lavage. Treatment with corticosteroids shows a rapid and dramatic positive response without recurrence.

• Journal of Chest Surgery
• /
• v.45 no.6
• /
• pp.404-407
• /
• 2012

Aortic dilatation and dissection are severe complications during pregnancy that can be fatal to both the mother and the fetus. The risks of these complications are especially high in pregnant patients with Marfan syndrome; however, incidents of descending aortic dissection are very rare. This case report involves a successful Bentall procedure for and recovery from a rare aortic dissection in a pregnant Marfan patient who developed acute type II aortic dissection with severe aortic regurgitation and chronic descending aortic dissection immediately after Cesarean section. Regular follow-up will be needed to monitor the descending aortic dissection.

• Pediatric Infection and Vaccine
• /
• v.8 no.1
• /
• pp.114-117
• /
• 2001

We experienced a case of streptococcal toxic shock-like sundrome in a 12 year old boy. Symptoms such as fever, sore throat, diffuse erythematous rashes on whole body developed 3 days before admmision. His symptoms rapidly aggravated to develop hypotension, hepatic and renal dysfunction and thrombocytopenia. After admission, intravenous fluid and antibiotic therapy were done and he was succesfully treated. Attention should be paid to recognize and diagnose this fatal disese. We report this case with review of related literatures.

• Journal of Yeungnam Medical Science
• /
• v.39 no.2
• /
• pp.150-152
• /
• 2022

Complications related to the vascular anastomosis of the placental vessels in monochorionic twins are fatal. The clinical syndromes of feto-fetal transfusion include twin anemia polycythemia sequence (TAPS), twin-twin transfusion syndrome, and twin reversed arterial perfusion sequence. We present an extremely rare case of TAPS in a dichorionic diamniotic pregnancy. A 36-year-old woman, gravida 0, para 0, was referred to our hospital with suspected preterm premature membrane rupture. Although her pelvic examination did not reveal specific findings, the non-stress test result showed minimal variability in the first fetus and late deceleration in the second one. An emergency cesarean section was performed. The placenta was fused, and one portion of the placenta was pale, while the other portion was dark red. The hemoglobin level of the first fetus was 7.8 g/dL and that of the second one was 22.2 g/dL.

• Journal of The Korean Society of Clinical Toxicology
• /
• v.5 no.1
• /
• pp.74-78
• /
• 2007

The carbamates are a group of insecticides derived from carbamic acid, with a broad spectrum of uses as agricultural and household garden insecticides. Carbamate insecticides are reversible cholinesterase inhibitors. Their inhibitory action is mediated by reversible carbamylation of acetylcholine, as with the organophosphate insecticides. Carbamates are absorbed by the body through multiple routes, including inhalation, ingestion, and dermal absorption. Although poisoning can result from occupational exposure or accidental ingestion, in most cases there is suicidal intent. This is particularly true in developing countries, where the highest incidence of morbidity and mortality from this cause occurs. Cardiac complications often accompany poisoning by carbamate compounds, which may be serious and often fatal. The extent, frequency, and pathogenesis of cardiac toxicity from carbamate compounds has not been clearly defined. Possible mechanismsinclude sympathetic and parasymphatetic overactivity, hypoxemia, acidosis, electrolyte derangements, and a direct toxic effect of the compounds on the myocardium. Patients with carbamate poisoning should immediately be transferred to an intensive or coronary care unit where appropriate monitoring and resuscitative facilities are available. We here report a case of acute coronary syndrome resulting from acute carbamate ingestionthat resulted in a healthy discharge.

• Childhood Kidney Diseases
• /
• v.20 no.2
• /
• pp.97-100
• /
• 2016

Toxic epidermal necrolysis (TEN) is a rare, acute, serious, and potentially fatal skin disease, in which cell death causes the epidermis to separate from the dermis. It is thought to be a hypersensitivity complex that affects the skin and mucous membranes, and is caused by certain medications, infections, genetic factors, underlying immunologic disease, or more rarely, cancers. We report two cases of TEN associated with deflazacort (DFZ), a derivative of prednisolone, used in the first episode of nephrotic syndrome (NS). The skin eruption appeared on the $4^{th}$ and $5^{th}$ weeks after DFZ administration, while NS was in remission. The widespread lesions were managed by intensive supportive treatment, discontinuation of DFZ, and oral administration of cyclosporine. Both patients showed a rapid improvement in symptoms of TEN without any complications or relapse of NS.