Pseudohypoparathyroidism (PHP) is a rare clinical syndrome characterized by hypocalcemia, hyperphosphatemia and increase of serum parathyroid hormone in association with unique clinical features. We recently experienced a typical PHP type Ia patient who presented with recurrent seizure and muscle spasms and electroencephalogram (EEG) showed generalized spike-and-wave discharges. With the correction of hypocalcemia, seizures did not recur and epileptiform discharges disappeared. We suggest that the possibility of PHP should be considered in patients with seizures showing hypocalcemia and hyperphosphatemia.
Electroencephalography ; Humans ; Hyperphosphatemia ; Hypocalcemia ; Parathyroid Hormone ; Pseudohypoparathyroidism ; Seizures ; Spasm

OBJECTIVES: We aimed to develop a common health information exchange (HIE) platform that can provide integrated services for implementing the HIE infrastructure in addition to guidelines for participating in an HIE network in South Korea. METHODS: By exploiting the Health Level 7 (HL7) Clinical Document Architecture (CDA) and Integrating the Healthcare Enterprise (IHE) Cross-enterprise Document Sharing-b (XDS.b) profile, we defined the architectural model, exchanging data items and their standardization, messaging standards, and privacy and security guidelines, for a secure, nationwide, interoperable HIE. We then developed a service-oriented common HIE platform to minimize the effort and difficulty of fulfilling the standard requirements for participating in the HIE network. The common platform supports open application program interfaces (APIs) for implementing a document registry, a document repository, a document consumer, and a master patient index. It could also be used for testing environments for the implementation of standard requirements. RESULTS: As the initial phase of implementing a nationwide HIE network in South Korea, we built a regional network for workers' compensation (WC) hospitals and their collaborating clinics to share referral and care record summaries to ensure the continuity of care for industrially injured workers, using the common HIE platform and verifying the feasibility of our technologies. CONCLUSIONS: We expect to expand the HIE network on a national scale with rapid support for implementing HL7 and IHE standards in South Korea.
Computer Security ; Computer Systems ; Continuity of Patient Care ; Delivery of Health Care ; Electronic Health Records ; Health Level Seven ; Humans ; Information Services* ; Korea ; Privacy ; Referral and Consultation ; Workers' Compensation

BACKGROUND: Nausea and vomiting are predominant accompanying symptoms of migraine attacks. Although the underlying mechanism is not yet clear, gastric stasis is assumed to be the main factor. However, few studies have used direct methods to establish delayed gastric emptying of migraine patients. We compared interictal gastric motility between migraine patients and normal controls with the aid of gastric scintigraphy. METHODS: The study population comprised patients who had been diagnosed with episodic migraine, according to the International Classification of Headache Disorders, edition II. The entire study population was completely free of gastrointestinal symptoms during the headache-free period. Gastric scintigraphy was performed to determine the time to half emptying (T 1/2) and the percentage of radioactive material remaining in the stomach (%RMR) at 30, 60, 90, and 120 min. RESULTS: Twenty-six migraine patients and 12 normal controls were recruited. The mean T 1/2 did not differ between the two groups (101.8 vs 95.2 min; p=0.432). The %RMR values in the stomach at 30, 60, 90, and 120 min also did not differ significantly between the two groups [87.5% vs 88% (p=0.900), 70.8% vs 71.2% (p=0.950), 54.2% vs 53.3% (p=0.753), and 39.0% vs 37.3% (p=0.583), respectively]. CONCLUSIONS: There is no gastric stasis in patients with episodic migraine during headache-free periods. Our results suggest that the main mechanism underlying the nausea and vomiting in migraine patients is not a gastric stasis in interictal periods, but rather a central process, as a result of changes occurring in the brainstem during acute migraine attacks.
Brain Stem ; Gastric Emptying ; Gastroparesis ; Headache Disorders ; Humans ; Migraine Disorders ; Nausea ; Stomach ; Vomiting

Multiple endocrine neoplasia type 1 (MEN1) is an autosomal dominant tumor syndrome. Thymic carcinoid tumors in MEN1 are not common and their natural history is little known. But development of thymic carcinoid tumors is important because in 1993, they were identified as a frequent case of death. There has not been a report of case in Korea so far. We encountered a case of thymic carcinoid in MEN1. A 42 year old man was referred presenting with diabetes of 12 years duration. Abnormal findings in his blood chemistry were hypercalcemia and hyperprolactinemia. 99mTc- sestamibi scintigraphy showed parathyroid adenoma and hyperplasia. Sella MRI showed pituitary macroadenoma. Abnormal CT scan demonstrated multiple pancreas islet cell tumors, bilateral adrenal tumor and thymoma. Subtotal parathyroidectomy with thymectomy was perfomed and thymic carcinoid was confirmed. This is the first report of thymic carcinoid with MEN1 in Korea.
Adenoma, Islet Cell ; Adult ; Carcinoid Tumor* ; Chemistry ; Humans ; Hypercalcemia ; Hyperplasia ; Hyperprolactinemia ; Korea ; Magnetic Resonance Imaging ; Multiple Endocrine Neoplasia Type 1* ; Multiple Endocrine Neoplasia* ; Natural History ; Pancreas ; Parathyroid Neoplasms ; Parathyroidectomy ; Radionuclide Imaging ; Thymectomy ; Thymoma ; Tomography, X-Ray Computed

BACKGROUND: Electromyography and acceleromyography are common neuromuscular monitoring devices. However, questions still remain regarding the use of acceleromyography in children. This study compared the calibration success rates and intubation conditions in children after obtaining the maximal blockade depending on each of the devices METHODS: Children, 3 to 6 years old, were randomly allocated to the TOF-Watch SX acceleromyography group or the NMT electromyography group. The induction was performed with propofol, fentanyl, and rocuronium. The bispectral index and 1 Hz single twitch were monitored during observation. The calibration of the each device was begun when the BIS dropped to 60. After successful calibration, rocuronium 0.6 mg/kg was injected. A tracheal intubation was performed when the twitch height suppressed to 0. The rocuronium onset time (time from administration to the maximal depression of twitch height) and intubating conditions were rated in a blinded manner. RESULTS: There was no difference in the calibration success rates between the two groups; and the calibration time in the electromyography group (16.7 +/- 11.0 seconds) was shorter than the acceleromyography group (28.1 +/- 13.4 seconds, P = 0.012). The rocuronium onset time of the electromyography group (73.6 +/- 18.9 seconds) was longer than the acceleromyography group (63.9 +/- 18.8 seconds, P = 0.042) and the intubation condition of the electromyography group (2.27 +/- 0.65) was better than the acceleromyography group (1.86 +/- 0.50, P = 0.007). CONCLUSIONS: Electromyography offers a better compromise than acceleromyography with respect to the duration of calibration process and surrogate for the optimal time of tracheal intubation in children.
Anesthesia, General* ; Calibration ; Child* ; Child, Preschool ; Depression ; Electromyography* ; Fentanyl ; Humans ; Intubation ; Neuromuscular Monitoring ; Propofol ; Prospective Studies*

Background and Objectives: The association between multifocal papillary thyroid carcinoma (PTC) and tumor aggressiveness remains controversial. The aim of study is to evaluate molecular subtypes of multifocal PTCs using multiplatform genomic analysis. Materials and Methods: Statistical analysis and genomic analysis were performed for gene expression data and clinical data of multifocal PTCs in The Cancer Genome Atlas data. Clinicopathologic findings, recurrence-free survival (RFS), copy number alteration and somatic mutation status in patients in relation to molecular subtypes were analyzed. Results: Multiplatform genomic analysis revealed that multifocal PTCs (n=226) were divided into two distinct molecular subgroups. Participants in cluster 2 showed significantly increased risk of extrathyroidal extension, lymph node metastasis, and BRAFV600E mutation compared to patients in cluster 1. To exclude the effect of BRAF mutation and RAS mutation on tumor aggressiveness, we compared clinical parameters between two clusters in patients without BRAF or RAS mutation. Cluster 2 showed significantly higher risk of lymph node metastasis compared to cluster 1. Conclusion Multifocal PTC has two distinct molecular subtypes with distinctive clinical behaviors. Our data suggested the clinical implications of the transcriptomic signature to predict clinical outcomes of multifocal PTC.

Members of the inhibitors of differentiation (Id) family of helix-loop-helix (HLH) proteins are known to play important roles in the proliferation and differentiation of many cell types. Thyroid-stimulating hormone (TSH) regulates proliferation and differentiation by activating TSH receptor (TSHR) in thyrocytes. In this study, we found that Id2, one of the Id family proteins, is a major target for regulation by TSH in FRTL-5 thyroid cells. TSH rapidly increases the Id2 mRNA level in FRTL-5 thyroid cells but the Id2 protein showed biphasic regulatory patterns, being transiently reduced and subsequently induced by TSH treatment. Transient reduction of Id2 protein was noted within 2 hr of TSH treatment and was mediated by proteasomal degradation. Moreover, reduced Id2 expression correlated with the activity of the phosphatidylinositol 3 kinase pathway, which is activated by TSH. Although TSH increases the activity of the Id2 promoter, TSH-induced activation of this promoter was independent of c-Myc. Id2 did not alter TTF-1- and Pax-8-mediated effects on the regulation of the Tg promoter. Thus, in summary, we found that TSH regulates Id2 expression, but that Id2 does not alter the expression of thyroid-specific genes, such as Tg, in FRTL-5 thyroid cells.
1-Phosphatidylinositol 3-Kinase/metabolism ; Animals ; Cattle ; Cell Differentiation ; Cell Proliferation ; *Gene Expression Regulation ; Inhibitor of Differentiation Protein 2/metabolism ; Insulin/metabolism ; Paired Box Transcription Factors/metabolism ; Promoter Regions, Genetic ; Proto-Oncogene Proteins c-myc/metabolism ; Rats ; Thyroglobulin/metabolism ; Thyroid Gland/*cytology ; Thyrotropin/*metabolism