Exercise, Mitohormesis, and Mitochondrial ORF of the 12S rRNA Type-C (MOTS-c)

Low levels of mitochondrial stress are beneficial for organismal health and survival through a process known as mitohormesis. Mitohormetic responses occur during or after exercise and may mediate some salutary effects of exercise on metabolism. Exercise-related mitohormesis involves reactive oxygen species production, mitochondrial unfolded protein response (UPRmt), and release of mitochondria-derived peptides (MDPs). MDPs are a group of small peptides encoded by mitochondrial DNA with beneficial metabolic effects. Among MDPs, mitochondrial ORF of the 12S rRNA type-c (MOTS-c) is the most associated with exercise. MOTS-c expression levels increase in skeletal muscles, systemic circulation, and the hypothalamus upon exercise. Systemic MOTS-c administration increases exercise performance by boosting skeletal muscle stress responses and by enhancing metabolic adaptation to exercise. Exogenous MOTS-c also stimulates thermogenesis in subcutaneous white adipose tissues, thereby enhancing energy expenditure and contributing to the anti-obesity effects of exercise training. This review briefly summarizes the mitohormetic mechanisms of exercise with an emphasis on MOTS-c.

Leprous Neuropathy Associated with Predominant Proprioceptive Sensory Loss / 대한임상신경생리학회지

Some patients with leprosy may present with atypical features, such as isolated peripheral neuropathy without skin lesions, or marked proprioceptive dysfunction. We report a 56-year-old female who presented with predominant proprioceptive loss without skin lesion, but was finally confirmed as leprous neuropathy by sural nerve biopsy. It is postulated that large myelinated fibers were affected by chronic immunological reactions triggered by inactive bacterial particles, producing a peripheral neuropathy presenting as predominant proprioceptive sensory loss without typical skin lesions.

Attenuation of Spinal Curvature and Pelvic Obliquity by Body Shape Molded Inner System in Cerebral Palsy with Non-Fixed Scoliosis

OBJECTIVE: To investigate the effect of the body shape molded inner system on attenuation of spinal curvature and pelvic obliquity in cerebral palsy (CP) with scoliosis. METHOD: Fifteen patients with CP who had fixed or non-fixed scoliosis were recruited. By radiographic studies, Cobb's angle and pelvic obliquity were measured with or without sitting in the body shape molded inner system. RESULTS: Spinal curvature assessed by Cobb's angle was significantly reduced when CP patients were seated in the body shape molded inner system rather than in conventional seats. Although pelvic obliquity was not improved in patients with fixed scoliosis, it was significantly ameliorated in patients with non-fixed scoliosis when seated in the body shape molded inner system. CONCLUSION: The body shape molded inner system attenuated spinal curvature and pelvic obliquity in CP patients with non-fixed scoliosis which had a flexible spinal curve.

Dopa-responsive Dystonia Misdiagnosed as Cerebral Palsy and Hereditary Spastic Paraplegia 2 Cases: Two cases report

Dystonia is a movement disorder caused by involuntary, sustained muscle contractions, frequently resulting in twitching and repetitive movements or abnormal postures. Dopa-responsive dystonia (DRD) is characterized by early childhood onset, marked diurnal fluctuation of symptoms and dramatic response to levodopa. The aim of this report is to present the two cases of DRD misdiagnosed respectively as cerebral palsy and hereditary spastic paraplegia. Proper understanding of this disease entity and its treatment options are necessary for comprehensive rehabilitative management of DRD.

Clinical Characteristics of Biliary Tract Cancer Associated with Choledochal Cyst

PURPOSE: Choledochal cysts are rare congenital or acquired cystic dilatations of the intra- or extra-hepatic bile ducts. The mechanism of carcinogenesis in choledochal cyst has not been clearly elucidated, although stasis of bile and reflux of pancreatic juice appear to be important factors. The aim of this study was to identify the clinical risk factors predicting development of biliary tract cancers in patients with choledochal cyst. METHODS: The study subjects included 170 consecutive patients who underwent surgery for choledochal cysts at Seoul National University Hospital between December 1980 and May 2008. We analyzed the demographic characteristics, clinical symptoms, laboratory findings, type of choledochal cysts, pathologic characteristics, and long-term outcomes of the patients with associated biliary tract cancers. RESULTS: Out Of 170 patients with choledochal cysts, combined biliary tract cancers ware identified in 29 patients, which included extrahepatic bile duct (n=15; 51.7%), gallbladder (n=12; 41.4%), and ampulla of Vater cancers (n=2; 6.9%). There were no significant differences in gender, clinical symptoms (abdominal pain, jaundice, and abdominal masses), laboratory findings (leukocytosis, hyperbilirubinemia, and increased alkaline phosphatase), and Todani classification of choledochal cysts between the two groups with or without combined biliary tract cancer. Multivariate analysis revealed that age > or =41 years and pancreatico-choledochal type APBDU were associated with the development of biliary tract cancers in patients with choledochal cysts. CONCLUSION: Age > or =41 years and pancreatico-choledochal type APBDU were associated with the development of biliary tract cancers in patients with choledochal cysts. Therefore, the possibility of associated biliary tract cancers should be considered when planning surgical management for patients with these risk factors.

Clinical Characteristics of Biliary Tract Cancer Associated with Choledochal Cyst

PURPOSE: Choledochal cysts are rare congenital or acquired cystic dilatations of the intra- or extra-hepatic bile ducts. The mechanism of carcinogenesis in choledochal cyst has not been clearly elucidated, although stasis of bile and reflux of pancreatic juice appear to be important factors. The aim of this study was to identify the clinical risk factors predicting development of biliary tract cancers in patients with choledochal cyst. METHODS: The study subjects included 170 consecutive patients who underwent surgery for choledochal cysts at Seoul National University Hospital between December 1980 and May 2008. We analyzed the demographic characteristics, clinical symptoms, laboratory findings, type of choledochal cysts, pathologic characteristics, and long-term outcomes of the patients with associated biliary tract cancers. RESULTS: Out Of 170 patients with choledochal cysts, combined biliary tract cancers ware identified in 29 patients, which included extrahepatic bile duct (n=15; 51.7%), gallbladder (n=12; 41.4%), and ampulla of Vater cancers (n=2; 6.9%). There were no significant differences in gender, clinical symptoms (abdominal pain, jaundice, and abdominal masses), laboratory findings (leukocytosis, hyperbilirubinemia, and increased alkaline phosphatase), and Todani classification of choledochal cysts between the two groups with or without combined biliary tract cancer. Multivariate analysis revealed that age > or =41 years and pancreatico-choledochal type APBDU were associated with the development of biliary tract cancers in patients with choledochal cysts. CONCLUSION: Age > or =41 years and pancreatico-choledochal type APBDU were associated with the development of biliary tract cancers in patients with choledochal cysts. Therefore, the possibility of associated biliary tract cancers should be considered when planning surgical management for patients with these risk factors.

The Usefulness of the 3-D Overlapped Reconstruction MR Angiographic Technique in Patients with Hemifacial Spasm - A Preliminary Study

PURPOSE: To investigate the applicability of the new three-dimensional overlapped reconstruction MR angiography (3-D ORMRA) technique in patients with hemifacial spasm and to compare the new 3-D reconstruction images with conventional MRA source images. MATERIALS AND METHODS: The study group comprised 27 patients with surgically proven hemifacial spasm. In all patients, conventional MRA source images and 3-D fast imaging employing steady-state acquisition (FIESTA) images were obtained prospectively. After 3-D MR angiographic images were obtained, the 3-D MRA and FIESTA images were overlapped at the workstation by using GE A/W 4.2 add/sub software. We analyzed the relationship between the offending vessels and root exit zone of the facial nerve using both 3-D ORMRA images and conventional MRA source images. RESULTS: In 25 of 27 patients, the offending vessel at the REZ of the facial nerve could be correctly identified on conventional MRA source images. In all patients, the presumed offending vessels depicted by the overlapped 3-D reconstruction MRA image corresponded well with the intraoperative findings. The 3-D reconstruction image showed more clear visualization of the spatial relationship between the offending vessels and the root exit zone of the facial nerve. CONCLUSION: The overlapped 3-D reconstruction MR angiography technique is very useful and informative in patients with hemifacial spasm, as compared with conventional MRA angiography technique.

Usefulness of a Alvarado Scoring System for the Diagnosis of Acute Appendicitis in Children / 대한소아소화기영양학회지

PURPOSE: Alvarado scoring system was evaluated regarding its usefulness for the early diagnosis of acute appendicitis in adult and in reduction of the incidence of negative appendicectomies. To evaluate the accuracy of diagnosing appendicitis using the Alvarado score in children. METHODS: Prospectively, we surveyed 122 patients (male 67, female 55) suffering from abdominal pain, who had visited to the emergency department of Chosun University Hospital from June 2002 to May 2003. The Alvarado score has been computed from the white blood cell count, neutrophil count, body temperature, resistance in the right lower quadrant, length of symptoms, nausea and vomiting. Each patient was evaluated by a pediatric resident and then by a general surgeon independently. RESULTS: Out of 170 total children who visited to the emergency department due to abdominal pain, 122 patients were associated with appendicitis. A total of 122 patients (67 male and 55 female) were visited to the emergency room with suspected appendicitis. From 105 operated patients, 92 (87.6%) were diagnosed acute appendicitis and erronous diagnostic rate was 12.4%, pathologically. Mean alvarado score of appendicitis group was 5.40+/-1.24 whereas those of non-appendicitis group was 3.73+/-1.82 (p<0.05). From 6 Alvarado score high sensitivity (86.4%) and high specificity (80.0%) were observed. Sensitivity of ultrasonography or computed tomography was 92.5%. CONCLUSION: We found that Alvarado score system is a noninvasive, safe diagnostic method, which is simple, reliable and repeatable. Alvarado score is useful system for a first, rapid and economic evaluation for the appendicitis in children.

Pulmonary Hemorrhage with Hemoptysis in Systemic Lupus Erythematosis / 소아알레르기및호흡기학회지

Pulmonary hemorrhage is a rare, but serious manifestation of systemic lupus erythematosus (SLE). In this report we describe a 13-year-old girl with pulmonary hemorrhage as the sole presenting clinical manifestation of SLE. Asymptomatic pulmonary lupus needs no treatment; however, pulmonary involvement in lupus may be life threatening, in which case prompt and aggressive treatment is mandatory. The different aspects of pulmonary lupus are demonstrated through the clinical histories of patients who suffered from pleuro-pulmonary lupus. Early diagnosis of acute pulmonary complications in systemic lupus erythematosus patients is essential to improve the chances of recovery.

A Case of Congenital Chloride Diarrhea in Premature Infant

Congenital chloride diarrhea is a serious autosomal recessive disease, and defect of intestinal electrolyte absorption that involves, specifically, Cl-/HCO3- exchange in the distal part of the ileum and colon. The clinical feature is dominated by profuse, watery diarrhea containing high concentrations of chloride(> 90 mmol/L) and sodium. The chloride loss results in severe dehydration with a hypochloremic alkalosis. The molecular pathology involves an epithelial Cl - /HCO3 - exchanger protein. Mucosal ion transport is affected to differing degrees and the severity of the disease may thus vary. Recently, a gene defect on chromosome 7 has been identified. However, there was a deficit in replacement of fluid and electrolyte, abdominal distension remained and the character of stools was watery. We report a case of congenital chloride diarrhea in a premature female who presented with watery diarrhea containing high concentrations of chloride and abdominal distension.

Relationship between Admission and Clinical Features of Children who visited the Emergency Department with Seizures

PURPOSE: To review the seizure-related complaints and analyze the relationship between admission rates and clinical features in children who had visited the emergency department with seizures. METHODS: Retrospectively, we reviewed 180 patients(male 100, female 80) suffering from seizures, who had visited to the emergency department of Chosun University Hospital from January 2000 to June 2002. We have analyzed the correlation between admission rate and clinical features such as age, seizure type, seizure duration and individual laboratory findings(CT or MRI, and CSF). RESULTS: Out of 4,865 total children who visited the emergency department, 180 patients(3.7%) were seizure related. The most common seizure type was simple febrile seizure(52.2%). The admission rate of children with seizures was 48.9%. The admission rate according to age, sex and abnormal laboratory findings revealed no significant correlations(P>0.05). There was a significant correlation between admission and both status epilepticus 82.4%(14/17) and complex febrile seizure 63.6%(14/22) (P<0.05). According to the duration of convulsions, admission rates were 41.2% when within five minutes, 60% when six-15 minutes, 58.8% when 16-30 minutes, 85.7% when 30 minutes, to one hour and 66.7% when above one hour of duration. According to the seizure frequency, admission rates of recurrent seizure patients(61.4%=43/70) was higher compared to the first time seizure patients(40.9%=45/110). CONCLUSION: We found that the admission rate of children visiting the emergency department for seizure treatment was 48.9% and significantly correlated with duration, type and frequency of seizure.

A Case of Cerebral Tuberculoma who has been Suffered from Recurrent Idiopathic Pleural Effusion

Central nervous system(CNS) involvement by tuberculosis is uncommon in comparison to the involvement of other systems. Untreated tuberculous meningitis is characterized by progressive stupor and a fatal outcome within 4 to 8 weeks of the onset of symptoms. So rapid detection of Mycobacterium tuberculosis is of vital importance for these patients. In almost all patients with tuberculous meningitis, acid-fast bacteria are undetectable in the CSF. Culture techniques that permit the identification of the isolated mycobacterial species require several weeks. We report a case of tuberculous meningitis with intracranial tuberculoma in 8-year old male who experienced pleural effusion, repeatedly. CSF findings did not reveal diagnosis tuberculous meningitis. In MRI study, multiple intracranial tuberculomas were located at the cerebellum. Prolonged treatment with anti- tuberculous chemotherapy and high-dose corticosteroids led to complete recovery.