Beneficial effect of pyruvate therapy on Leigh syndrome due to a novel mutation in PDH E1α gene

Leigh syndrome (LS) is a progressive untreatable degenerating mitochondrial disorder caused by either mitochondrial or nuclear DNA mutations. A patient was a second child of unconsanguineous parents. On the third day of birth, he was transferred to neonatal intensive care units because of severe lactic acidosis. Since he was showing continuous lactic acidosis, the oral supplementation of dichloroacetate (DCA) was introduced on 31st day of birth at initial dose of 50 mg/kg, followed by maintenance dose of 25 mg/kg/every 12 h. The patient was diagnosed with LS due to a point mutation of an A-C at nucleotide 599 in exon 6 in the pyruvate dehydrogenase E1α gene, resulting in the substitution of aspartate for threonine at position 200 (N200T). Although the concentrations of lactate and pyruvate in blood were slightly decreased, his clinical conditions were deteriorating progressively. In order to overcome the mitochondrial or cytosolic energy crisis indicated by lactic acidosis as well as clinical symptoms, we terminated the DCA and administered 0.5 g/kg/day TID of sodium pyruvate orally. We analyzed the therapeutic effects of DCA or sodium pyruvate in the patient, and found that pyruvate therapy significantly decreased lactate, pyruvate and alanine levels, showed no adverse effects such as severe neuropathy seen in DCA, and had better clinical response on development and epilepsy. Though the efficacy of pyruvate on LS will be evaluated by randomized double-blind placebo-controlled study design in future, pyruvate therapy is a possible candidate for therapeutic choice for currently incurable mitochondrial disorders such as LS.     

Shoulder and elbow pain in junior high school baseball players: Results of a nationwide survey

BackgroundDespite proposals and guidelines to prevent baseball injuries in young players by societies and organizations, many shoulder and elbow injuries continue to occur among junior high school baseball players. In order to investigate the training conditions of junior high school baseball players and the risk factors for shoulder and elbow pain in the players, we conducted a questionnaire survey among junior high school baseball players throughout the country.MethodsThe questionnaire survey was conducted among junior high school baseball players in September 2016.ResultsA total of 11,134 junior high school baseball players belonging to 495 teams responded to the survey. Among these, 4004 players trained every day of the week and 1151 players played baseball games every month with no off-season. Among 9752 players who did not have shoulder and/or elbow pain in the spring and summer of 2015, 19.2% of players experienced elbow pain over the course of one year, 13.6% of players experienced shoulder pain, and 28.0% complained of shoulder and/or elbow pain. The frequency of elbow pain was more than that of shoulder pain. At risk for shoulder pain were pitchers and catchers and second-year students, while risk factors for elbow pain were playing pitcher and catcher positions, pitching or throwing ≥300 balls per week, playing ≥10 games on average per month and being left-handed.ConclusionRisk factors for shoulder pain were different from those for elbow pain. To prevent elbow pain, coaches should pay attention to pitchers and catchers and left-handed players and not allow players to pitch or throw ≥300 full-power balls per week or participate in ≥10 games per month. They should also pay attention to pitchers and catchers and second-year students to prevent shoulder pain. It is important for coaches to train multiple pitchers and catchers.     

Shoulder and elbow pain in elementary school baseball players: The results from a nation-wide survey in Japan

Background

Despite recommendations on how to prevent baseball injuries in youths by the Japanese Society of Clinical Sports Medicine, shoulder and elbow pain still frequently occurs in young baseball players. We conducted a questionnaire survey among baseball players at elementary schools across the country to understand the practice conditions of players, examining the risk factors of shoulder and elbow pain in baseball players.

Improvement of regional ischemia after successful percutaneous intervention of bypassed native coronary chronic total occlusion: an application of the CART technique

Myocardial ischemia has been associated with patent saphenous vein grafts (SVG) for reasons that may be attributable to graft-related endothelial dysfunction. Recanalization of the bypassed native coronary arteries may relieve the regional ischemia. In this clinical scenario, percutaneous coronary intervention (PCI) of the challenging chronic total occlusions (CTO) can be facilitated by a retrograde approach utilizing the SVGs as access conduits. Controlled antegrade and retrograde subintimal tracking (CART) is a technique developed to facilitate the recanalization of CTOs by aggressive wire manipulation from both ends of the lesions. Success rates of CART are high in experienced hands with few complications. We discuss 2 patients who presented with anginal symptoms, ischemia on stress nuclear scintigraphy and patent SVGs on angiography. After the corresponding native CTOs in the bypassed coronary arteries were successfully recanalized using the CART technique, both patients' symptoms and reversible scintigraphic defects resolved. The CART technique is highlighted utilizing SVGs as access conduits for the retrograde approach.     

A case of gallbladder adenomyomatosis with pancreaticobiliary maljunction and an anomaly of the cystic duct joined the common channel

A 46-yr-old woman was admitted to our hospital with mild epigastric pain. Ultrasonography and computed tomography revealed an extremely thickened gallbladder wall. Endoscopic retrograde cholangiopancreatography demonstrated that the main pancreatic duct joined the nondilated common bile duct at the outer point of the duodenal wall (P-C type of pancreaticobiliary maljunction), and the cystic duct joined the common channel directly. The intraoperative amylase levels of the bile juices both in the common bile duct and the cystic duct were high. A cholecystectomy was performed. The wall of the gallbladder was markedly thick, yellowish, elastic, and soft. Histologically, Rokitansky-Aschoff sinus proliferation, hypertrophy of smooth muscles, and fibrosis were seen. The diagnosis was a generalized type of adenomyomatosis. The pathogenesis of the adenomyomatosis was believed to result from chronic stimulation as a result of pancreatic juice reflux. The etiology of this unusual type of junction was considered to be the result of the combination of pancreaticobiliary maljunction and an anomaly of lower junction of the cystic duct.     

Increased activity in the right prefrontal cortex measured using near-infrared spectroscopy during a flower arrangement task

Objective: Flower arrangement program (FAP) horticultural therapy promotes psychological, social and physiological wellness and recovery. Moreover, FAPs have been used to evaluate the outcomes related to visuospatial working memory; yet, most of these studies used subjective outcome measures such as behavioural observations and questionnaires. Few studies report objective evaluations of FAP effects in humans. In the present study, we measured the effects of an FAP task on frontal lobe activity in healthy participants using near-infrared spectroscopy. We quantified salivary amylase levels as an indicator of stress level during the FAP.

Methods: The FAP task involved a predetermined arrangement pattern of natural materials (flowers and leaves) that required the participants to identify where a given material should be placed and temporarily memorise the designated position to complete the flower arrangement. The FAP task was compared to the block-tapping task (BTT), which is routinely used to evaluate visuospatial working memory.

Results: Both the FAP task and BTT positively stimulated the right prefrontal cortex; however, stress was more effectively limited during the performance of the FAP task.

Conclusions: Our data suggest that FAP therapy may be useful for the rehabilitation of patients who are sensitive to stress.