Execution of anticipatory guidance and the knowledge and practice gap among caregivers in Southern Taiwan: A retrospective study.

BACKGROUND/PURPOSE: This study examined the practice rate of Anticipatory Guidance (AG) and the gap between knowledge and practice among caregivers. METHODS: We retrospectively collected data from caregivers who brought their children for seven age-based well-child visits (birth to 7 years old) and seven corresponding AG checklists for practice (each ranged from 16 to 19 guidance items, 118 items in total) between 2015 and 2017. Practice rates of guidance items and their association with children's sex, age, residence, and body mass index were collected and analyzed. RESULTS: We enrolled 2310 caregivers (330 per well-child visit). Average practice rates of guidance items in the seven AG checklists were 77.6%-95.1%, generally without significant differences between urban/rural or male/female children. However, lower (<80%) rates were observed for 32 items, including dental check-ups (38.9%), use of fluoride toothpaste (44.6%), screen time (69.4%), and drinking less sugar-sweetened beverages (SSBs) (75.5%), with corresponding knowledge-to-practice gap rates of 55.5%, 47.9%, 30.3%, and 23.8%, respectively. "Drinking less SSBs" was the only item with a higher obesity rate in the non-achieved group versus the achieved group (16.7% vs. 7.4%, p = 0.036; odds ratio: 3.509, 95% CI: 1.153-10.677, p = 0.027). CONCLUSION: Caregivers in Taiwan practiced most AG recommendations. However, dental check-ups, fluoride toothpaste use, drinking less SSBs, and limiting screen time were less executed items. A higher obesity rate was found among 3-7-year-old children whose caregivers failed to practice the "Drink less SSBs" guidance. Strategies to overcome the gap between knowledge and practice are needed to improve these less-achieved guidance items.

Unnecessary radiation exposure during diagnostic radiography in infants in a neonatal intensive care unit: a retrospective cohort study.

Unnecessary radiation exposure (URE) during radiographic examination is an issue among infants in neonatal intensive care units (NICUs). The causes of URE have not been fully explored. This study investigated the incidence and identified the causes of URE in infants during diagnostic radiography in a NICU. This was a retrospective cohort study. We retrieved and analysed requests and radiographs taken at a tertiary NICU between September and November 2018. URE was defined as the rate of discordance between requests and images taken (DisBRI) and unnecessary radiation exposure in irrelevant regions (UREIR) during radiography. We compared the rates of URE between very low-birth-weight (VLBW, birth weight < 1500 g) infants and non-VLBW infants. A total of 306 radiographs from 88 infants were taken. The means ± standard deviations (SDs) of gestational age and birth weight were 35.7 ± 3.6 weeks and 2471 ± 816 g, respectively. Each infant underwent an average of 3.5 radiographs. The DisBRI rate was 1.3% and was mostly related to poor adherence to requests. The UREIR rates in thoraco-abdominal babygrams were 89.6% for the head, 14.8% for the elbows and 18.4% for the knee and were mainly related to improper positioning of and collimation in infants while performing radiography. The UREIR rates for the head, knee and ankle were higher in VLBW infants than in non-VLBW infants (94.6% vs. 85.6%, 27.0% vs. 11.5% and 5.4% vs. 0.7%, respectively, p < 0.05). CONCLUSIONS: URE during diagnostic radiography is common in sick infants and is mainly related to improper positioning and collimation during examinations. Adherence to protocols when performing radiographic examination or using ultrasonography may be a solution to reduce URE in infants in NICUs. WHAT IS KNOWN: • The risk of unnecessary radiation exposure (URE) during radiography has been a common and important issue in sick infants in neonatal intensive care units (NICUs). • The new point-of-care ultrasound (POCUS) technique decreases the need for chest films and prevents radiation exposure in neonates. WHAT IS NEW: • In the NICU, URE is still a common issue in critically ill infants during radiographic examinations. The causes of URE during diagnostic radiography are mainly due to improper positioning and collimation during examinations. • The incidence of URE in irrelevant regions is higher in very low-birth-weight (VLBW) infants than in non-VLBW infants.

Arecae pericarpium extract induces porcine lower-esophageal-sphincter contraction via muscarinic receptors.

BACKGROUND: Gastroesophageal reflux disease (GERD) is associated with lower esophageal sphincter (LES) incompetence. In some patients, GERD is refractory to acid reduction therapy which is the main treatment for GERD. So far, medications that can increase LES tone are few. Arecae pericarpium (A. pericarpium) is a medication in Traditional Chinese Medicine known to promote intestinal motility. METHODS: We investigated the effect of A. pericarpium extracts on porcine LES motility. In addition, we used tetrodotoxin (TTX) and atropine to study the underlying mechanism of A. pericarpium extracts-induced contractions of LES. RESULTS: The results of this study showed that A. pericarpium extracts and their main active ingredient, arecoline, can induce the contractions of porcine LES sling and clasp muscles in a dose-response manner. TTX did not have an inhibitory effect on the contractions induced by A. pericarpium extracts and arecoline in LES. However, atropine significantly inhibited A. pericarpium extracts- and arecoline-induced contractions of LES. CONCLUSION: A. pericarpium extracts can induce the contractions of porcine LES in a dose dependent manner, possibly through muscarinic receptors, and hence, may be worth developing as an alternative therapy for GERD.

Maternal Age, the Disparity across Regions and Their Correlation to Sudden Infant Death Syndrome in Taiwan: A Nationwide Cohort Study.

Sudden infant death syndrome (SIDS) has always been a regrettable issue for families. After sleeping in the supine position was proposed, the incidence of SIDS declined dramatically worldwide. However, SIDS still accounts for the top 10 causes of infant deaths in Taiwan. Recognizing the risk factors and attempting to minimize these cases are imperative. We obtained information on cases with SIDS from the National Health Insurance Research Database in Taiwan and interconnected it with the Taiwan Maternal and Child Health Database to acquire infant-maternal basal characteristics between 2004 and 2017. The SIDS subjects were matched 1:10 considering gestational age to normal infants. After case selection, a total of 953 SIDS cases were included. Compared with healthy infants, SIDS infants had younger parents, lower birth weight, and lower Apgar scores. After adjusting for potential confounders, infants with mothers aged <20 years had 2.81 times higher risk of SIDS. Moreover, infants in the non-eastern region had a significantly lower risk of SIDS than those in the eastern region. We concluded that infants of young mothers (especially maternal age <20 years) and infants in the eastern region of Taiwan had a higher risk of SIDS than their counterparts.

Weight Is More Accurate than Gestational Age When Estimating the Optimal Endotracheal Tube Depth in Neonates.

Determining the optimal endotracheal tube (ETT) depth in neonates remains challenging for neonatologists. The guideline for optimal ETT depth is based on the patients' weight or gestational age. However, there is a discrepancy in the suggested ETT depth between these two parameters. The aim of this retrospective study was to compare the recommended weight-based and age-based formulas for optimal ETT depth and obtain the optimal reference before intubation. Participants were assigned to group 1 if the recommended ETT insertion depth based on weight was concordant with the recommended depth based on gestational age, and to group 2 if the weight and age-based depth recommendations were discordant. After exclusion, 180 patients were included in the analysis. Results indicated that the predicted ETT depth suggested by age required more adjustment than by weight (p < 0.05). Furthermore, the required adjustment in the weight-based formula was smaller than the age-based formula (p < 0.05). Multivariate linear regression analysis revealed that weight was the key factor affecting the optimal depth (p < 0.001). These results imply that when there is a discrepancy in ETT depth between the weight-based and age-based recommendation, the weight-based one will be more accurate than the age-based one.

Initial Experience With Patent Ductus Arteriosus Ligation in Pre-term Infants With Bidirectional Shunt Pattern.

Background: Patent ductus arteriosus (PDA) with a bidirectional shunt reflects critical clinical conditions. The operability of PDA with a bidirectional shunt in pre-term infants is still not clearly clarified. This study aimed to investigate the feasibility and the outcomes of PDA ligation in pre-term infants with a bidirectional shunt PDA. Methods: All pre-term infants receiving PDA ligation between 2013 and 2019 were enrolled in this prospective study. Patients were allocated into two groups based on the shunting direction of PDA, which were the left-to-right group (group A) and the bidirectional group (group B). Clinical characteristics and pre-op comorbidities were analyzed. Intraoperative complications, post-op neurological sequelae, necrotizing enterocolitis, survival, and mortality were compared between these two groups. Results: Thirty-seven pre-term infants were enrolled (18 in group A, 19 in group B). The mean post-menstrual age at PDA surgery was 32.0 ± 1.3 and 32.8 ± 1.5 weeks, respectively. Before surgery, 44.4 and 89.5% (group A vs. B) of the patients were using invasive mechanical ventilator (p < 0.01). The requirement of high-frequency oscillatory ventilatory support was significantly higher in group B. PDA rupture-related bleeding during exposing PDA or ligating PDA occurred in four infants, and all were all in group B, including one with delayed hemothorax. Early surgical mortality within 30 days of surgery was higher in group B (0 vs. 21.1%, p < 0.05), but only one death could be attributed to the surgery, which was caused by a pain-induced pulmonary hypertension crisis. The 5-year survival was 100% in group A, and 73.7% in group B (p < 0.05). Conclusion: We did not recommend routine PDA ligation in pre-term infants with a bidirectional shunt. However, a bidirectional shunt should not be an absolute contraindication if they fulfill indications of PDA closure. Unexpected intraoperative PDA rupture and delayed hemothorax in a bidirectional shunt PDA should be carefully monitored. Aggressive post-op pain control is also warranted to avoid pulmonary hypertension crisis. The post-op early mortality rate was higher in the bidirectional group, which could be inherent to their poor pre-operative lung condition. Only one death was directly related to the surgery.

Long-Chain Fatty Acid Receptors Mediate Relaxation of the Porcine Lower Esophageal Sphincter.

Long-chain fatty acids activate the free fatty acid receptor 1 (FFA1) and FFA4. In the gastrointestinal system, FFA1 and FFA4 have been found in the pancreas and intestine. Fatty food and decreased lower esophageal sphincter (LES) motility are associated with gastroesophageal reflux disease. The effect of long-chain fatty acids on the esophageal motility is unknown. The purpose of this study is to investigate the effects of long-chain fatty acids on the porcine LES motility ex vivo using isometric transducers. In endothelin 1-precontracted porcine LES strips, the FFA1 selective agonists, fasiglifam, TUG424, and GW9508, caused marked relaxations in a concentration-dependent manner. The relative efficacies to elicit relaxation were GW9508 > TUG424 > fasiglifam in both clasp and sling strips. In contrast, the FFA4 specific agonists, TUG891 and GSK137647, produced mild relaxations. In addition, the endogenous FFA1 agonist DHA caused a mild relaxation whereas GW1100, an FFA1 antagonist, inhibited GW9508 induced relaxation of the porcine LES clasp and sling muscle. Both real-time PCR and immunohistochemistry revealed that FFA1 and FFA4 were expressed in the porcine LES. Real-time PCR analysis showed that the FFA4 expression was much lower than FFA1. Taken together, long-chain fatty acid receptor agonists elicit relaxation of the porcine LES. FFA1 might influence LES motility.

Association between maternal pre-delivery body mass index and offspring overweight/obesity at 1 and 2 years of age among residents of a suburb in Taiwan.

BACKGROUND: Overweight and obesity among children can cause metabolic syndrome in adulthood and are a significant public health issue. Some studies suggest that maternal pre-pregnancy body mass index (BMI) and excessive gestational weight gain during pregnancy are associated with overweight and obesity in offspring. However, it is difficult to collect information on accurate pre-pregnancy BMI and pregnancy weight gain for women living in areas where medical resources are scarce. Maternal pre-delivery BMI might be predictive of the risk of overweight and obesity among offspring of pregnant mothers living in suburban areas. METHODS: We retrospectively collected data on term neonates with appropriate weights for their gestational age born between April 2013 and October 2015. We excluded neonates with major congenital anomalies or diseases and incomplete data. Mothers with systemic diseases or drug abuse were also excluded. Offspring body weights and heights at 1- and 2-years-old were recorded. Maternal pre-delivery BMI was divided into following groups: <25, 25-29.9, and ≧30 kg/m2. RESULTS: We included 261 mother-child pairs in this study. The BMIs of the offspring differed significantly among the three maternal pre-delivery BMI groups at the age of 2 years (15.18 ± 1.04, 15.83 ± 1.28, and 16.29 ± 1.61 kg/m2, p < 0.001, respectively). After adjusting for potential cofounders possibly affecting weight using multivariate linear regression, the children's BMIs (adjusted 95% CI: 0.71 [0.31-1.11]; p = 0.001) and BMI percentiles (adjusted 95% CI 15.80 [7.32-24.28]; p < 0.001) at the age of 2 years were significantly higher in those born to mothers with pre-delivery BMIs of 25-29.9 kg/m2 compared to mothers with pre-delivery BMIs <25 kg/m2. Maternal pre-delivery BMI ≧30 kg/m2 was significantly associated with increased BMIs (adjusted 95% CI: 1.17 [0.72-1.63]; p < 0.001) and BMI percentiles (adjusted 95% CI: 23.48 [13.87-33.09]; p < 0.001) in their children. A maternal pre-delivery BMI of 27.16 kg/m2 was the optimal cut-off for predicting offspring overweight/obesity at the age of 2 years. DISCUSSION: Our results indicate that the maternal pre-delivery BMI was significantly associated with offspring BMI and weight gain at the age of 2 years. A maternal pre-delivery BMI of 27.16 kg/m2 might be a useful predictor for estimating the risk of overweight or obesity in offspring at the age of 2 years.

Heteronemin Induces Anti-Proliferation in Cholangiocarcinoma Cells via Inhibiting TGF-ß Pathway.

A marine sesterterpenoid-type natural product, heteronemin, retains anticancer effects. In the current study, we investigate the antitumor mechanism of heteronemin in cholangiocarcinoma cells and further explore its molecular targets. Initially, heteronemin exhibited potent cytotoxic effects against cholangiocarcinoma HuccT1 and SSP-25 cells. In vitro, heteronemin altered the abilities of cell adhesion and cell migration in HuccT1 and SSP-25 cell lines. It repressed messenger ribonucleic acid (mRNA) expression levels of transforming growth factor (TGF)-ß, mothers against decapentaplegic homolog (SMAD) and Myc, whose protein products play important roles in regulating cell growth, angiogenesis, and metastasis. In addition, heteronemin altered several signaling pathways. The results indicate that heteronemin was able to modulate cell adhesion, the expression of extracellular matrix (ECM) receptors, the TGF-ß pathway, cell motility, the membrane integration, metastasis response, matrix metalloproteinase (MMP) remodeling, the regulation of metabolism, sprouting angiogenesis, transcription factors, and vasculogenesis in cholangiocarcinoma cell lines. The results also suggest that it activated multiple signal transduction pathways to induce an anti-proliferation effect and anti-metastasis in cholangiocarcinoma. In conclusion, heteronemin may be used as a potential medicine for anticancer therapy.

The beneficial effect of air cleanliness with ISO 14644-1 class 7 for surgical intervention in a neonatal intensive care unit: A 10-year experience.

Whether critically ill neonates needing a surgical intervention should be transferred to an operating room (OR) or receive the intervention in a neonatal intensive care unit (NICU) is controversial. In this study, we report our experience in performing surgical procedures in a NICU including air cleanliness.This was a retrospective study performed at a metropolitan hospital. The charts of all neonates undergoing surgical procedures in the NICU and OR were retrospectively reviewed from January 2007 to June 2017. Data on baseline characteristics, procedure and duration of surgery, ventilator use, hypothermia, instrument dislocations, surgery-related infections and complications, and outcomes were analyzed.Ninety-two neonates were enrolled in this study, including 44 in the NICU group and 48 in the OR group. The air cleanliness was International Organization for Standardization (ISO) 14644-1 class 7 in the NICU and class 5-6 in the OR. The NICU group had a younger gestational age and lower birth body weight than the OR group. The OR group had a higher incidence of hypothermia than in the NICU group (56.3% vs 9.1%, P < .001). However, there were no significant differences in surgical site related infections or mortality between the 2 groups.This study suggests that performing surgical procedures in a NICU with air cleanliness class 7 is as safe as in an OR, as least in part, when performing patent ductus arteriosus ligation and exploratory laparotomy.

Mechanism of resveratrol-induced relaxation of the guinea pig fundus.

BACKGROUND: Resveratrol is a polyphenolic compound that can be isolated from plants and also is a constituent of red wine. Resveratrol induces relaxation of vascular smooth muscle and may prevent cardiovascular diseases. PURPOSE: Impaired gastric accommodation plays an important role in functional dyspepsia and fundic relaxation and is a therapeutic target of functional dyspepsia. Although drugs for fundic relaxation have been developed, these types of drugs are still rare. The purpose of this study was to investigate the relaxant effects of resveratrol in the guinea pig fundus. STUDY DESIGN: We studied the relaxant effects of resveratrol in the guinea pig fundus. In addition, we investigated the mechanism of resveratrol-induced relaxation on the guinea pig fundus by using tetraethylammonium (a non-selective potassium channel blocker), apamine (a selective inhibitor of the small conductance calcium-activated potassium channel), iberiotoxin (an inhibitor of large conductance calcium-activated potassium channels), glibenclamide (an ATP-sensitive potassium channel blocker), KT 5720 (a cAMP-dependent protein kinase A inhibitor), KT 5823 (a cGMP-dependent protein kinase G inhibitor), NG-nitro-L-arginine (a competitive inhibitor of nitric oxide synthase), tetrodotoxin (a selective neuronal Na+ channel blocker), ω-conotoxin GVIA (a selective neuronal Ca2+ channel blocker) and G-15 (a G-protein coupled estrogen receptor antagonist). RESULTS: The results of this study showed that resveratrol has potent and dose-dependent relaxant effects on the guinea pig fundic muscle. In addition, the results showed that resveratrol-induced relaxation of the guinea pig fundus occurs through nitric oxide and ATP-sensitive potassium channels. CONCLUSION: This study provides the first evidence concerning the relaxant effects of resveratrol in the guinea pig fundic muscle strips. Furthermore, resveratrol may be a potential drug to relieve gastrointestinal dyspepsia.

Estradiol mediates relaxation of porcine lower esophageal sphincter.

Most pregnant women have symptoms of gastroesophageal reflux disease (GERD) during pregnancy. Postmenopausal hormone replacement therapy is associated with GERD. The effects of estradiol on lower esophageal sphincter (LES) motility and GERD are not clearly known. The purpose of this study is to investigate the effects of estradiol on the motility of the porcine LES. Relaxations of clasp and sling strips of porcine LES caused by estradiol were measured using isometric transducers. We investigated the mechanism of estradiol-induced relaxation of the porcine LES using tetraethylammonium, apamine, iberiotoxin, glibenclamide, KT5720, KT5823, NG-nitro-l-arginine, tetrodotoxin, and ω-conotoxin GVIA. Reverse transcription polymerase chain reaction (PCR) analysis and immunohistochemistry (IHC) were performed to determine the existence of the G protein-coupled estrogen receptor (GPER) in the porcine LES. In endothelin-1-precontracted porcine LES strips, estradiol caused marked relaxations in a concentration-dependent manner. The mechanism of estradiol-induced relaxation on the porcine LES was associated with the potassium channel. Reverse transcription PCR analysis and IHC revealed that GPER was expressed in the sling and clasp fibers of the porcine LES. This finding suggests that GPER mediates the relaxation of the porcine LES. Estradiol may play a role in LES motility.

Mechanism of resveratrol-induced relaxation in the human gallbladder.

BACKGROUND: Resveratrol is a polyphenolic compound extracted from plants and is also a constituent of red wine. Resveratrol produces relaxation of vascular smooth muscle and may prevent cardiovascular diseases. Although resveratrol has been reported to cause relaxation of the guinea pig gallbladder, limited data are available about the effect of resveratrol on the gallbladder smooth muscle in humans. The purpose of this study was to investigate the relaxation effects of resveratrol in human gallbladder muscle strips. METHODS: We studied the relaxant effects of resveratrol in human gallbladder. In addition, we also investigated mechanism of resveratrol-induced relaxation in human gallbladder by tetraethylammonium (a non-selective potassium channels blocker), iberiotoxin (an inhibitor of large conductance calcium-activated potassium channel), glibenclamide (an ATP-sensitive potassium channel blocker), charybdotoxin (an inhibitor of large conductance calcium-activated potassium channels and slowly inactivating voltage-gated potassium channels), apamine (a selective inhibitor of the small conductance calcium-activated potassium channel), KT 5720 (a cAMP-dependent protein kinase A inhibitor), KT 5823 (a cGMP-dependent protein kinase G inhibitor), NG-Nitro-L-arginine (a competitive inhibitor of nitric oxide synthase), tetrodotoxin (a selective neuronal Na+ channel blocker), and ω-conotoxin GVIA (a selective neuronal Ca2+ channel blocker). RESULTS: The present study showed that resveratrol has relaxant effects in human gallbladder muscle strips. In addition, we found that resveratrol-induced relaxation in human gallbladder is associated with nitric oxide, ATP-sensitive potassium channel, and large conductance calcium-activated potassium channel pathways. CONCLUSIONS: This study provides the first evidence concerning the relaxant effects of resveratrol in human gallbladder muscle strips. Furthermore, these results demonstrate that resveratrol is a potential new drug or health supplement in the treatment of biliary colic.

Neurodevelopmental Outcomes in Very Low Birth Weight Infants Using Aminophylline for the Treatment of Apnea.

BACKGROUND: Aminophylline has been widely used in the treatment of apneic episodes in premature infants. Animal models suggest caution in the use of aminophylline as it may increase the cerebral metabolic rate and decrease the rate of anoxic survival in neonates. This study aimed to evaluate the neurological outcomes in very low birth weight (VLBW) infants treated with aminophylline for apnea in our neonatal intensive care unit. METHODS: All VLBW infants (body birth weight < 1500 g) admitted to our neonatal intensive care unit between January 2000 and December 2011 were enrolled in this retrospective study. Clinical characteristics and outcomes of these infants were reviewed and recorded. Scores on the Bayley Scales of Infant Development at 6 months, 12 months, and 18 months of corrected age were also recorded. The controls (who did not receive aminophylline) were matched for gestational age with the aminophylline group. RESULTS: The baseline characteristics of the aminophylline group and the control group were similar. The neurodevelopmental outcomes as well as rates of patent ductus arteriosus, brain injury, severe retinopathy of prematurity, and necrotizing enterocolitis were not significantly different between the two groups. Only bronchopulmonary dysplasia remained significantly higher in the aminophylline group after adjusting for risk factors (48.08% vs. 21.15%; adjusted odds ratio: 12.50; p < 0.001). CONCLUSION: Aminophylline therapy for apnea of prematurity had no apparent and additional risk on the neurodevelopmental outcomes of VLBW infants at a corrected age of 18 months. Further studies with a larger sample size are needed to confirm the adverse neurological effects of aminophylline treatment.

Mechanism of bombesin-induced tonic contraction of the porcine lower esophageal sphincter.

Gastroesophageal reflux disease (GERD) is a disorder that is related to an incompetent lower esophageal sphincter (LES). Previous studies showed that bombesin could increase LES pressure in humans and opossums. The aim of the present study was to characterize the effects of bombesin on porcine LES contraction. We used the selective agonists, neuromedin B (NMB), gastrin-releasing peptide (GRP), and [D-Tyr(6),Apa-4Cl(11),Phe(13),Nle(14)]bombesin-(6-14) (DTACPN-BN), as well as receptor antagonists of bombesin receptor subtype 2 (BB2), and 3 (BB3) for ex vivo contraction studies. Atropine, nifedipine, tetrodotoxin, and ω-conotoxin GVIA were used to explore the agonist-induced LES contraction mechanism. Reverse transcription polymerase chain reaction and immunohistochemistry were applied to detect bombesin receptor expression. Our results indicate that GRP and DTACPN-BN, but not NMB, induced tonic contractions of the porcine LES in a dose-dependent manner, and the contractions were inhibited with selective BB2 and BB3 antagonists. The GRP-induced contraction is mainly caused by L-type Ca(2+) channel-mediated Ca(2+) influx. However, DTACPN-BN-induced contractions are associated with neuronal conduction. RT-PCR and immunohistochemistry revealed that BB2 and BB3 were expressed in the porcine LES. Bombesin-induced tonic contraction of the LES is mediated through BB2 and BB3. Bombesin, BB2, and BB3 agonists might have the potential to treat GERD.

Salvia miltiorrhiza Induces Tonic Contraction of the Lower Esophageal Sphincter in Rats via Activation of Extracellular Ca2+ Influx.

Up to 40% of patients with gastroesophageal reflux disease (GERD) suffer from proton pump inhibitor refractory GERD but clinically the medications to strengthen the lower esophageal sphincter (LES) to avoid irritating reflux are few in number. This study aimed to examine whether Salvia miltiorrhiza (SM) extracts induce tonic contraction of rat LES ex vivo and elucidate the underlying mechanisms. To investigate the mechanism underlying the SM extract-induced contractile effects, rats were pretreated with atropine (a muscarinic receptor antagonist), tetrodotoxin (a sodium channel blocker), nifedipine (a calcium channel blocker), and Ca(2+)-free Krebs-Henseleit solution with ethylene glycol tetraacetic acid (EGTA), followed by administration of cumulative dosages of SM extracts. SM extracts induced dose-related tonic contraction of the LES, which was unaffected by tetrodotoxin, atropine, or nifedipine. However, the SM extract-induced LES contraction was significantly inhibited by Ca(2+)-free Krebs-Henseleit solution with EGTA. Next, SM extracts significantly induce extracellular Ca(2+) entry into primary LES cells in addition to intracellular Ca(2+) release and in a dose-response manner. Confocal fluorescence microscopy showed that the SM extracts consistently induced significant extracellular Ca(2+) influx into primary LES cells in a time-dependent manner. In conclusion, SM extracts could induce tonic contraction of LES mainly through the extracellular Ca(2+) influx pathway.

Infantile form of muscle phosphofructokinase deficiency in a premature neonate.

Muscle phosphofructokinase (PFK) deficiency is a rare autosomal recessive disease. We report the case of a preterm female infant who was diagnosed with the infantile form of phosphofructokinase deficiency due to a lack of PFK activity in her muscles, manifesting at a corrected age of 1 month as floppy infant syndrome, congenital joint contracture, cleft palate and duplication of the pelvicalyceal system. She died at a corrected age of 6 months due to respiratory failure. We further reviewed other infantile cases in the literature. Congenital hypotonia (78.6%), arthrogryposis (64.3%) and other systemic involvement including encephalopathy (35.7%) and cardiomyopathy (21.4%) are common presentations of the infantile form of PFK deficiency. The overall survival rate of the infantile form is low. The early recognition of multiple system involvement is essential to provide better clinical care for infants with the infantile form of PFK deficiency.