BACKGROUND: Although impaired endothelial function is well known in patients with diabetes mellitus, the precise mechanism and the factors that contribute to this dysfunction remain to be clarified. We examined the effect of acute hyperglycemia on patients with impaired glucose metabolism in vivo by plethysmography. METHODS: Seven patients with diabetes mellitus or impaired glucose metabolism were studied. In each patient, endothelial function was examined in the fasting state and at two levels of hyperglycemia, which were achieved by the infusion of glucose, insulin, and somatostatin. Forearm blood flow was measured while acetylcholine was infused in increasing concentrations(7.5, 15, and 30 microgram/min) through the brachial artery. RESULTS: Glucose concentrations increased accordingly at each stage, from 135.3+/-18.4 mg/dl at stage 1(the fasting state), to 239.0+/-15.2 mg/dl at stage 2(the first level of hyperglycemia), and to 378.3+/-25.3 at stage 3 (the second level of hyperglycemia) [p<0.01]. Maximal acetylcholine-dependent vasodilation achieved by infusion of acetylcholine at 30 microgram/min was significantly aftenuated during stages 2 and 3 compared with stage 1(p<0.05 by AVOVA; forearm blood flow ratio was 2.87+/-0.18 and 2.56+/-0.14 versus 3.58+/-0.21, respectively). This was also evident during the infusion of 15 microgram/min and 7.5 microgram/min of acetylcholine. CONCLUSIONS: Endothelium-dependent vasodilation is significantly aftenuated by acute hyperglycemia in patients with diabetes mellitus or impaired glucose metabolism. Our findings suggest that elevated glucose may contribute to the endothelial dysfunction observed in patients with diabetes mellitus or impaired glucose metabolism.
Acetylcholine ; Brachial Artery ; Diabetes Mellitus* ; Endothelium ; Fasting ; Forearm ; Glucose* ; Humans ; Hyperglycemia* ; Insulin ; Metabolism* ; Plethysmography ; Somatostatin ; Vasodilation*

Rubinstein-Taybi syndrome (RTS) is a congenital disorder characterized by typical facial features, broad thumbs and toes, with mental retardation. Additionally, tumors, keloids and various congenital anomalies including congenital heart defects have been reported in RTS patients. In about 50% of the patients, mutations in the CREB binding protein (CREBBP) have been found, which are understood to be associated with cell growth and proliferation. Here, we describe a typical RTS patient with Arnold-Chiari malformation. A mutation in the CREBBP gene, c.4944_4945insC, was identified by mutational analysis.
Arnold-Chiari Malformation ; Congenital, Hereditary, and Neonatal Diseases and Abnormalities ; CREB-Binding Protein ; Heart Defects, Congenital ; Humans ; Intellectual Disability ; Keloid ; Rubinstein-Taybi Syndrome ; Thumb ; Toes

BACKGROUND AND OBJECTIVES: Scintigraphic swallowing test is a noninvasive, quantitative and safe measurement of swallowing. The purposes of this study were to establish a normative data of scintigraphic swallowing test and to determine that this procedure could be a simple screening test of swallowing disorders. MATERIALS AND METHODS: Scintigraphic tests of the oropharyngeal transit with thick liquid bolus were performed in 51 normal subjects, who were 15 to 61 years of age. Each subject was instructed to swallow the single viscous liquid bolus mixed with 1mCi Tc-99m tin-colloid. Dynamic imaging data were obtained with the patients in a supine position and recorded at a frame rate of 25 per second over 10 seconds. Inspection of summed images permitted the section of regions of interest (ROI) to represent the mouth, pharynx, and esophagus. Transit times between each ROI were calculated and compared. Time activity data were used to compute oral discharge and pharyngeal transit times, oropharyngeal transit time, percentage residues in the mouth and pharynx, oral and pharyngeal swallowing efficiencies, and oropharyngeal clearance in 2.5 seconds. Twenty subjects repeated the test in two weeks. RESULTS: Mean+/-standard deviation of oral discharge time, pharyngeal transit time, oropharyngeal transit time, oral residue, pharyngeal residue, oral swallowing efficiency, pharyngeal swallowing efficiency, and oropharyngeal clearance in 2.5sec were 0.37+/-0.11sec, 0.64+/-0.19 sec, 1.01+/-0.21 sec, 7.05+/-3.60%, 6.12+/-3.80%, 273.58+/-78.82%/sec, 160.74+/-51.11%/sec, and 90.63+/-6.65% respectively. Repeated studies in 20 individuals indicated that these parameters were statistically similar and pharyngeal transit time was highly reproducible. Other parameters were intermediately reproducible except oral and pharyngeal residues. CONCLUSION: Oropharyngeal scintigraphy provides a rapid, noninvasive and cost effective screening test as well as a quantitative study of swallowing disorders.
Deglutition Disorders ; Deglutition* ; Esophagus ; Humans ; Mass Screening ; Mouth ; Pharynx ; Radionuclide Imaging* ; Supine Position

BACKGROUND: While procarbazine, CCNU (lomustine), and vincristine (PCV) has been an alternative chemotherapy option for malignant gliomas, it is worth investigating whether the combination of only procarbazine and CCNU is comparable because vincristine adds toxicity with uncertain benefit. The purpose of this study was to evaluate the feasibility of procarbazine and CCNU chemotherapy for recurrent glioblastoma multiforme (GBM) with O6-methylguanine-DNA-methyltransferase (MGMT) promoter methylation. METHODS: Eight patients with recurrent GBM following concurrent chemoradiotherapy and temozolomide (TMZ) adjuvant therapy were enrolled in this trial; they received no other chemotherapeutic agents or target therapy. They received CCNU (75 mg/m²) on day 1 and procarbazine (60 mg/m²) through days 11 and 24 every 4 weeks. The median cycle of CCNU and procarbazine was 3.5 (range: 2–6). RESULTS: One patient achieved stable disease. The median progression-free survival (PFS) with procarbazine and CCNU chemotherapy was eight weeks (range: 5–73), and the PFS rates were 25% and 12.5% at 16 and 30 weeks, respectively. The median overall survival (OS) from the initial diagnosis to death was 40 months, and the median OS from the administration of procarbazine and CCNU chemotherapy to death was 9.7 months (95% confidence interval: 6.7–12.7). Serious adverse events were found at six visits, and two cases were considered to be grade 3 toxicities. CONCLUSION: The efficacy of procarbazine and CCNU chemotherapy is not satisfactory. This study suggests the need to develop other treatment strategies for recurrent and TMZ-refractory GBM. Trial registry at ClinicalTrials.gov, NCT017337346.
Chemoradiotherapy ; Diagnosis ; Disease-Free Survival ; Drug Therapy* ; Glioblastoma* ; Glioma ; Humans ; Lomustine* ; Methylation* ; Procarbazine* ; Vincristine

A 60-year-old male patient with coronavirus disease-2019 showed new onset ST-segment elevation in V1–V2 leads on electrocardiogram and cardiac enzyme elevation in intensive care unit. He had a history of type 2 diabetes mellitus, hypertension, and dyslipidemia. He was receiving mechanical ventilation and veno-venous extracorporeal membrane oxygenation treatment for severe hypoxia. Two-D echocardiogram showed regional wall motion abnormalities. We performed primary percutaneous coronary intervention for acute myocardial infarction complicating cardiogenic shock under hemodynamic support. He expired on the 16th day of admission because of cardiogenic shock and multi-organ failure. Active surveillance and intensive treatment strategy are important for saving lives of COVID-19 patients with acute myocardial infarction.

Thyrotoxic periodic paralysis (TPP) is a rare complication of hyperthyroidism, with recurrent muscle paralysis and hypokalemia that are caused by an intracellular shift of potassium. TPP is relatively common in Asian males, but is extremely rare in children and adolescents, even for those of Asian descent. We describe a 16-year-old Korean adolescent presenting with a two-week history of episodic leg weakness in the morning. He showed sinus tachycardia, lower leg weakness, and hypokalemia. Thyroid function test showed hyperthyroidism, and thyroid ultrasonography revealed a diffuse enlarged thyroid with increased vascularity, consistent with Graves' disease. He was treated with beta-adrenergic blocker and antithyroid drugs. He has been symptom free for one year, as his hyperthyroidism has been controlled well with antithyroid drugs. TPP should be considered in children and adolescents with acute paralysis of the lower extremities and hypokalemia.
Adolescent* ; Antithyroid Agents ; Asian Continental Ancestry Group ; Child ; Graves Disease* ; Humans ; Hyperthyroidism ; Hypokalemia ; Leg ; Lower Extremity ; Male ; Paralysis* ; Potassium ; Tachycardia, Sinus ; Thyroid Function Tests ; Thyroid Gland ; Ultrasonography

Most pancreatic pseudocysts are located in or around the pancreas, but they can be found in all the potential spaces around viscera in and outside of the abdominal cavity. The complications of pancreatic pseudocysts are infection, rupture, fistula, obstruction and hemorrhage. However, an upper gastrointestinal bleeding caused by pancreatic pseudocyst is rare. Pseudocysts with complication like hemorrhage require percutaneous, endoscopic or surgical treatment. We report a case of the pancreatic pseudocyst involving duodenal wall with upper gastrointestinal hemorrhage, which was improved by conservative treatment.
Abdominal Cavity ; Duodenum* ; Fistula ; Gastrointestinal Hemorrhage* ; Hemorrhage ; Pancreas ; Pancreatic Pseudocyst* ; Rupture ; Viscera

PURPOSE: The aim of this study was to evaluate the efficacy and safety of levetiracetam in children younger than 4 years with intractable epilepsy. METHODS: A retrospective analysis of pediatric epilepsy patients was performed. Data were obtained from the medical records of 30 patients (male 19, female 11) with intractable epilepsy, who were treated with levetiracetam. RESULTS: Seizure types were partial in 18, and generalized in 12. Fifteen patients had symptomatic etiologies. The median age of the patients at the time of levetiracetam administration was 26 months old (range: 4-47 months). The median starting dose was 13 mg/kg/ day, and the median maintenance dose was 52 mg/kg/day (range: 10-123 mg/kg/day). Ten (10/30, 33%) patients experienced more than 50% reduction in seizure frequency, and 4 (4/30, 13%) partial epilepsy patients became seizure-free. Eight partial epilepsy patients (44%) had more than 50% seizure reduction, while 2 patients (17%) with generalized epilepsy did. All of patients with infantile spasms and Lennox-Gastaut syndrome except one, had less than 50% reduction in seizure frequency. Adverse events reported in 8 patients (27%), included lethargy, behavioral problems, sleep disturbance, and seizure aggravation. CONCLUSIONS:Levetiracetam is effective in children aged 4 years or less with intractable epilepsy, and also seems to be safe to use in this age group.
Aged ; Anticonvulsants ; Child ; Child, Preschool ; Epilepsies, Partial ; Epilepsy ; Epilepsy, Generalized ; Female ; Humans ; Infant ; Infant, Newborn ; Intellectual Disability ; Lethargy ; Medical Records ; Piracetam ; Retrospective Studies ; Seizures ; Spasms, Infantile

OBJECTIVE: We evaluated pseudoprogression (PsPD) following radiation therapy combined with concurrent temozolomide (TMZ), and we assessed pseudoresponse following anti-angiogenic therapy for patients with recurrent disease using the Response Assessment of the Neuro-Oncology Working Group. METHODS: Patients who were pathologically confirmed as having high-grade glioma received radiotherapy with concurrent TMZ followed by adjuvant TMZ. Bevacizumab (Avastin) with CPT-11 were used as a salvage option for cases of radiologic progression. Magnetic resonance imaging (MRI) was routinely performed 1 month after concurrent radiochemotherapy (CRT) and every 3 months thereafter. For cases treated with the bevacizumab-containing regimen for progressive disease, MRI was performed every 2 months. RESULTS: Of 55 patients, 21 (38%) showed radiologic progression within 4 weeks after CRT. Of these patients, 16 (29%) showed progression at second post-CRT MRI (etPD) and five (9%) showed improvement (PsPD). Seven of thirty-four initially non-progressed patients showed progression at the second post-CRT MRI (ltPD). No difference in survival was observed between the etPD and ltPD groups (p=0.595). Five (50%) of ten patients showed a radiological response after salvage bevacizumab therapy. Four of those patients exhibited rapid progression immediately after discontinuation of the drug (drug holiday). CONCLUSION: Twelve weeks following treatment could be the optimal timing to determine PsPD or true progression. MRI with gadolinium enhancement alone is not sufficient to characterize tumor response or growth. Clinical correlation with adequate follow-up duration and histopathologic validation may be helpful in discriminating PsPD from true progression.
Chemoradiotherapy ; Follow-Up Studies ; Gadolinium ; Glioma* ; Humans ; Magnetic Resonance Imaging ; Radiotherapy ; Bevacizumab

Osteochondroma, also known as osteocartilaginous exostosis or simply exostosis, comprises about 8.5% of all primary bone tumors and about 36% of those that are benign. Osteochondromas affect mostly the long bones; only 3% of solitary osteochondromas originate in the spine, while a solitary lesion in the cervical spine is distinctly rare. We present a case of osteochondroma of the upper cervical spine, presenting as a posterior neck mass. Although rare, spinal osteochondromas should be considered in the differential diagnosis of neck masses, and surgical treatment should be undertaken to correct secondary pain, disability, neurologic deficits, or cosmetic deformities.
Congenital Abnormalities ; Diagnosis, Differential ; Exostoses ; Neck* ; Neurologic Manifestations ; Osteochondroma* ; Spine