BACKGROUND Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening disease. Despite of proper treatment and improving treatment regimens, HLH patients still show a fatal prognosis. Therefore the evaluation of prognostic factor is important and there are many studies about hyperbilirubinemia as a prognostic factor in HLH. So we studied the prognostic value of hyperbilirubinemia in HLH children. METHODS: A retrospective analysis was performed about 33 patients who were diagnosed with HLH at Pusan National University Hospital and Yangsan Pusan University Hospital between January 2000 to December 2012. We reviewed the clinical characteristics, laboratory findings, and results of treatment to identify hyperbilirubinemia as a prognostic factor in HLH patients. RESULTS: The median age of patients at diagnosis was 32 months. Most of patients presented with fever, pale appearance, abdominal pain and jaundice. Forty-eight point five percentage of patients showed normal serum bilirubiln level (<2.0 mg/dL) and 51.5% showed hyperbilirubinemia (> or =2.0 mg/dL). In normal serum bilirubin group, 1 patient (6.3%) was relapsed and 1 patient (5.9%) was relapsed in hyperbilirubinemia group. In the hyperbilirubinemia group, the mortality was higher than the normal bilirubin group but, there was no statistical significance. CONCLUSION: As a prognostic factor serum bilirubin at diagnosis in HLH patients, there was no significant correlation between hyperbilirubinemia and poor outcome. But, our study has a limitation that the number of patients is too small and almost showed good prognosis.
BACKGROUND Lung cancer is the most common cause of cancer-related death worldwide and in Korea, and small cell lung cancer (SCLC) is the most deadly tumor type in the different lung cancer histology. Chemotherapy is the main strategy of the treatment for SCLC, and etoposide and platinum regimen has been the only standard chemotherapy for about 30 years. To test feasibility of weekly divided dose irinotecan and carboplatin for Korean patients is the aim of this study. METHODS: Patients with histologically or cytologically confirmed extensive stage SCLC were included. Patients with limited stage (LD), who could not tolerate concurrent chemoradiotherapy were also included. All the patients received irinotecan 60 mg/m2, carboplatin 2 area under the curve at day 1, 8, and 15 every 4 weeks. Study regimen was discontinued when the disease progressed or intolerable side effects occurred. No more than 6 cycles of chemotherapy were given. RESULTS: Total 47 patients were enrolled, among them 9 patients were LD. Overall response rate was 74.5% (complete response, 14.9%; partial response, 59.6%). Side effects greater than grade 3 were neutropenia (25.5%), fatigue (12.8%), thrombocytopenia (8.5%), sepsis (4.3%), and pancytopenia (2.1%). There was no treatment related death. CONCLUSION: Weekly divided irinotecan and carboplatin regimen is effective, and safe as a first line therapy for both stage of SCLC. Large scaled, controlled study is feasible.
BACKGROUND This study was conducted to investigate the epidemiological features, clinical courses, and diagnoses of neonates who are transferred to neonatal intensive care unit of Yeungnam University Hospital due to tachypnea soon after birth. METHODS: Based on medical records, we performed a retrospective study of neonatal intensive care unit admissions due to tachypnea from January 2010 to December 2013. RESULTS: A total of 311 neonates were included in this study. The patient characteristics showed male predominance at 2.65:1. Among the 311 neonates with tachypnea, 127 (40.8%) neonates needed oxygen supply, and 54 (17.4%) neonates needed assisted mechanical ventilation. Transient tachypnea of the newborns (TTN) (158, 50.8%) showed the highest incidence, followed by pneumonia (63, 20.3%), extrapulmonary infection (37, 11.9%), respiratory distress syndrome (21, 6.8%), air leak (16, 5.1%), meconium aspiration syndrome (12, 3.9%), congenital heart disease (5, 1.6%), metabolic acidosis (3, 1%), primary pulmonary hypertension of newborns (2, 0.6%) and anemia (2, 0.6%). CONCLUSION: Although the neonates with tachypnea showed no other respiratory distress symptom, clinicians should be aware of the possibility of other pulmonary diseases as well as TTN and their extra-pulmonary causes. If tachypnea does not improve within a few hours, the clinician should consider further evaluation and management as soon as possible.
BACKGROUND We analyzed the changes in QT dispersion (QTd) in children with Kawasaki disease (KD), and determined the presence of repolarization abnormality in these children even in the absence of coronary artery abnormalities. METHODS: Ninety-one children with KD and 20 healthy controls were enrolled in this retrospective study. Serial echocardiographic and electrocardiographic (ECG) measurements in the beginning of treatment, 2nd month and 6th month after the diagnosis were compared. Fifty-one of 91 children had at least 2 serial ECG data. The number of patients who had 3 consecutive ECG data was 23. RESULTS: Among the 67 KD patients with no coronary artery changes, the consecutive mean QTd values were 41.86 ms, 37.84 ms, and 25.47 ms, respectively (26 ms for controls). In the analysis of changes among KD patients without coronary artery abnormalities, QTd showed a significant decrease with time (p=0.01). Especially, the 1st month and the 6th month QTd values were significantly different (p=0.028). The mean QTd values in KD patients with coronary artery changes were significantly higher than those in KD patients with no coronary artery changes at each time (1st, 2nd, and 6th month exam). CONCLUSION: QTd is significantly increased in children during the early stage of KD. Repolarization abnormality may exist during the acute stage of KD, regardless of the echocardiographic changes.
Diabetic ketoacidosis (DKA), a fatal acute diabetic complication, is characterized by severe metabolic decompensation and intravascular volume depletion. These conditions may result in hypercoagulability and prothrombic state. Pulmonary thromboembolism (PTE) could be presented as an uncommon and life-threatening complication of DKA. Reported herein is a case involving a 54-year-old male patient who was admitted with DKA due to chronic alcohol consumption and stopping the intake of oral antidiabetic drugs. After low-molecular-weight heparin and warfarin treatment because of PTE during the DKA treatment, the patient's condition improved over the week that he was discharged on insulin and warfarin.
Thyroglossal duct cyst (TGDC) carcinoma generally shows a favorable prognosis. If metastasis is present latently, it may not threaten the patient's life immediately. It has been shown, however, that larger than 1 cm papillary carcinoma (PC), level VI metastasis to the lymph node (LN), which is the nearest to the thyroid, independently predicts a worse prognosis. In the case presented herein, a 61-year-old female patient was diagnosed with an about 3 cm PC in the TGDC, particularly the columnar variant subtype, one of the aggressive variants. She had occult papillary thyroid microcarcinoma, but no LN metastasis. Even though she underwent the Sistrunk procedure and total thyroidectomy with central compartment neck dissection followed by high-dose radioactive iodine remnant ablation, however, the cancer cells spread to level IV neck LN, and finally to the lung. Therefore, when a patient is diagnosed with an aggressive histologic variant of PC in the TGDC, even without LN metastasis, the invasive surgical approach and close postoperative surveillance are necessary, with consideration of the risk of disease progression. Therefore, if it is possible to stratify the risk for patients, higher-risk patients can be offered a more invasive therapeutic approach.
A pulmonary artery sling is a very rare congenital abnormality in which the left pulmonary artery rises from the posterior surface of the right pulmonary artery and then passes between the trachea and the esophagus, causing tracheal compression. It is associated with tracheo-bronchial abnormalities (50%) and cardiovascular abnormalities (30%). It may produce respiratory symptoms through the airway compression of the abnormal left pulmonary artery and congenital abnormalities associated with it. Because most (90%) pulmonary artery sling patients present symptoms during infancy, their condition is often diagnosed in the first year of life. However, a pulmonary artery sling is occasionally found in adults. It is usually asymptomatic and found incidentally. This is a very rare case of an asymptomatic pulmonary artery sling in an adult. A 38-year-old man presented symptoms of mild exertional dyspnea. His spiral computed tomography showed a pulmonary artery sling. He was discharged without specific treatment because his symptoms improved without specific treatment and might not have been associated with a pulmonary artery sling. We report an adult case of an asymptomatic pulmonary artery sling diagnosed via spiral computed tomography, accompanied by a literature review.
A 52-year-old man was referred to our hospital due to fever and myalgia that occurred 2 weeks earlier. He showed a complete atrioventricular block on his electrocardiogram, and his vital signs were unstable. On his transthoracic echocardiograph, the 1.5 cm vegetation in the aortic valve with severe aortic regurgitation suggested infective endocarditis. His transesophageal enchocardiograph showed abscess in his mitral-aortic intervalvular fibrosa and vegetation was suspected on his anterior mitral valve leaflet. The patient underwent an emergent operation for valve replacement with temporary epicardial pacing. Intraoperatively, the septal leaflet of his tricuspid valve was injured during the debridement of the abscess pocket that was extended to the membranous septum. The aortic, mitral, and tricuspid mechanical valves were replaced with annular reconstruction without complications. After 14 days of intravenous antibiotics, we successfully changed the epicardial pacemaker into a transvenous DDD-type permanent pacemaker by placing a left ventricular lead via the coronary sinus and an atrial lead in the right atrium appendage. The patient was discharged in a tolerable state and was examined uneventfully in our hospital's outpatient clinic for 8 months.
Transarterial chemoembolization (TACE) is a widely accepted nonsurgical modality used for the treatment of multinodular hepatocellular carcinoma (HCC). The careful selection of the candidate is important due to the risk of developing various side effects. Fever, nausea, abdominal pain, and liver enzyme elevation are commonly known side effects of TACE. Hepatic failure, ischemic cholecystitis, and cerebral embolism are also reported, although their incidence might be low. Pulmonary complication after TACE is rare, and the reported cases of lipiodol pneumonitis are even rarer. A 53-year-old man was treated with TACE for ruptured HCC associated with hepatitis B virus infection. On day 19 after the procedure, the patient complained of dyspnea and dry cough. Chest computed tomography showed diffuse ground glass opacities in the wholelung fields, suggesting lipiodol-induced pneumonitis. After 2 weeks of conservative management, the clinical symptoms and radiologic abnormalities improved. Reported herein is the aforementioned case of lipiodol-induced pnemonitis after TACE, with literature review.
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Lipiodol Pneumonitis Following Transcatheter Arterial Chemoembolization for Hepatocellular Carcinoma Sungkeun Kim, Hee Yeon Kim, Su Lim Lee, Young Mi Ku, Yoo Dong Won, Chang Wook Kim Journal of Liver Cancer.2020; 20(1): 60. CrossRef
Secondary adrenal insufficiency caused by sorafenib administration in a patient with hepatocellular carcinoma Soo Yeon Jo, Soo Hyung Ryu, Mi Young Kim, Jeong Seop Moon, Won Jae Yoon, Jin Nam Kim Yeungnam University Journal of Medicine.2016; 33(2): 155. CrossRef
Coronary vasospasm is one of the fatal complications that may occur in patients undergoing open heart surgery. To date, however, there are not many cases in this series and no definite pathophysiology has been documented. We experienced a case of coronary artery vasospasm after atrial septal defect (ASD) surgery and then successfully treated it with both transbrachial intra-aortic balloon pump and percutaneous cardiopulmonary support. Only several hours after ASD surgery, the patient exhibited the cardiovascular collapse, the ST-segment elevation, followed by ventricular fibrillation and normal coronary angiography findings. It is important to make a differential diagnosis of coronary artery vasospasm in patients presenting with ST-segment elevation who had no notable coronary artery diseases. This case indicates that clinicians should be aware of the possibility that the coronary artery vasospasm may also occur in patients undergoing ASD surgery.
Autoimmune thyroiditis is the most common cause of hypothyroidism in the world. It is characterized clinically by gradual thyroid failure, goiter formation, or both, because of the autoimmune-mediated destruction of the thyroid gland. Renal involvement presenting proteinuria in autoimmune thyroiditis is not uncommon, occurring in 10% to 30% of the cases. Glomerulonephropathy associated with autoimmune thyroiditis, however, is a rare disease. Most reports of autoimmune thyroiditis with glomerulonephropathy have demonstrated a mixed pathological morphology and have been predominantly associated with membranous glomerulopathy. The case of minimal-change disease associated with thyroiditis presenting acute kidney injury is a rare disease that has not been reported in South Korea. Reported herein is the case of a 16-year-old man diagnosed with Hashimoto's thyroiditis, with minimal-change disease presenting acute kidney injury. He revealed hypothyroidism, proteinuria, and impaired renal function. Renal biopsy showed minimal-change disease and minimal tubular atrophy. The patient was treated with thyroid hormone, and his renal function and proteinuria improved. Therefore, for patients with autoimmune thyroiditis presenting unexplained proteinuria, glomerulonephropathy should be ruled out. Conversely, for patients with glomerulonephropathy and persistent proteinuria despite proper treatment, thyroid function and antibody tests should be performed.
Familial isolated primary hyperparathyroidism(FIPH) is associated with multiple endocrine neoplasia type 1 (MEN1) syndrome, primary hyperparathyroidism accompanied by jaw-tumor syndrome, and familial hypocalciuric hypercalcemia. FIPH may be an early stage of MEN1 or an allelic variant of MEN1. Thymic carcinoid tumor is a rare tumor in MEN1 syndrome. Here, the authors report the case of a 40-year-old man diagnosed with recurrent thymic carcinoid tumor and FIPH. Both the patient and his elder sister had been previously diagnosed to have FIPH with a novel frameshift mutation in the MEN1 gene. Initially, the patient underwent thymectomy because of an incidental finding of a mediastinal mass in his chest X-ray, and had remained asymptomatic over the following 4 years. Pancreas computed tomography conducted to evaluate MEN1 syndrome revealed anterior and middle mediastinal masses, and resultantly, massive mass excision was performed. Histological findings disclosed atypical carcinoids with infiltrative margins. In view of the thymic carcinoid tumor relapse that occurred in this patient, the authors recommend that regular pancreas and pituitary imaging studies be conducted for FIPH associated with a MEN1 gene mutation.
A 34-year-old female was suffered from pain and numbness of right arm for 2 months after undergoing a thoracoscopic procedure for a posterior mediastinal mass that was diagnosed as neurilemmoma. The patient was diagnosed as a complex regional pain syndrome type 2 with brachial plexopathy developed during thoracoscopic excision of posterior mediastinal mass, and stellate ganglion block (SGB) with 0.2% ropivacaine 10 mL was performed every 3-4 days. The patient revealed slightly prolonged blepharoptosis as Horner syndrome accompanied after every SGB and recovered. However, following the 23rd SGB, the blepharoptosis persisted and patient was recovered spontaneously from blepharoptosis after about 12 months. The possibility that the persistent blepharoptosis might be caused by brachial plexopathy related to patient's pathology or surgical manipulation and/or repeated SGB. If Horner syndrome occurs, its etiology should be assessed, and it would be necessary to explain and to assure the patient the possibility of recovery spontaneously from the complication within a year, without any sequelae.
Spontaneous intramuscular hematoma of the abdominal wall is a rare condition characterized by acute abdominal pain. It is often misdiagnosed as a surgical condition. It used to be associated with risk factors such as coughing, pregnancy, and anticoagulant therapy. Most cases of abdominal wall hematomas were rectus sheath hematomas caused by the rupture of either the superior or inferior epigastric artery, but spontaneous internal oblique hematoma was extremely rare. In this report, we present a case of spontaneous internal oblique hematoma in a 69-year-old man with non-dialysis chronic kidney disease who was taking cilostazol. The patient complained of abrupt abdominal pain with a painful palpable lateral abdominal mass while sleeping. The abdominal computed tomography showed an 8 cm-sized mass in the patient's left internal oblique muscle. The administration of cilostazol was immediately stopped, and the intramuscular hematoma of the lateral oblique muscle disappeared with conservative management.
Despite the necessity of surgical aortic valve replacement, many patients with symptomatic severe aortic stenosis (AS) cannot undergo surgery because of their severe comorbidities. In these high-risk patients, percutaneous transcatheter aortic valve implantation (TAVI) can be safely accomplished. However, no study has shown that TAVI can be performed for patients with severe AS accompanied by acute decompensated heart failure. In this case report, 1 patient presented a case of severe pulmonary hypertension with decompensated heart failure after diagnosis with severe AS, and was successfully treated via emergency TAVI. Without any invasive treatment, acute decompensated heart failure with severe pulmonary hypertension is common in patients with severe AS, and it can increase mortality rates. In conclusion, TAVI can be considered one of the treatment options for severe as presented as acute decompensated heart failure patients with pulmonary hypertension.