OBJECTIVEPostural tachycardia syndrome (POTS) is one of the major causes of orthostatic intolerance in children. We systematically reviewed the pathogenesis and the progress of individualized treatment for POTS in children.

DATA SOURCESThe data analyzed in this review are mainly from articles included in PubMed and EMBASE.

STUDY SELECTIONThe original articles and critical reviews about POTS were selected for this review.

RESULTSStudies have shown that POTS might be related to several factors including hypovolemia, high catecholamine status, abnormal local vascular tension, and decreased skeletal muscle pump activity. In addition to exercise training, the first-line treatments mainly include oral rehydration salts, beta-adrenoreceptor blockers, and alpha-adrenoreceptor agonists. However, reports about the effectiveness of various treatments are diverse. By analyzing the patient's physiological indexes and biomarkers before the treatment, the efficacy of medication could be well predicted.

CONCLUSIONSThe pathogenesis of POTS is multifactorial, including hypovolemia, abnormal catecholamine state, and vascular dysfunction. Biomarker-directed individualized treatment is an important strategy for the management of POTS children.

Adrenergic alpha-Agonists ; therapeutic use ; Adrenergic beta-Antagonists ; therapeutic use ; Catecholamines ; metabolism ; Humans ; Postural Orthostatic Tachycardia Syndrome ; drug therapy ; metabolism ; pathology ; therapy

Takotsubo cardiomyopathy (TTC) is an infrequent cardiac syndrome characterized by acute onset chest pain with apical ballooning on echocardiography. It is often triggered by severe emotional or physical stress, and in contrast to acute myocardial infarction (AMI), the regional wall motion abnormality returns to normal within days. Here, we describe a 62-year-old female who presented with acute onset chest pain during treatment for a liver abscess. We presumed a diagnosis of AMI because of ST segment elevation on electrocardiography and elevated cardiac enzyme levels. However, the patient's coronary arteries were normal on angiography, and apical ballooning was seen on echocardiography. A diagnosis of TTC was made, and the patient was managed with intensive cardiopulmonary support using vasopressors in our hospital's medical intensive care unit. The patient's symptoms improved, but persistent severe left ventricular dysfunction was detected on follow-up echocardiography. After 5 weeks, a new apical mural thrombus appeared, and anticoagulation therapy was started. The apical ballooning persisted 3 months later, although the patient's overall ejection fraction was slightly improved. The apical thrombus was completely resolved without any embolic event. Non-adrenergic inotropics can be recommended in TTC with shock, and clinicians should keep in mind the potential risk of thrombus formation and cardioembolism.
Adrenergic beta-Antagonists/therapeutic use ; Angiotensin-Converting Enzyme Inhibitors/therapeutic use ; Catecholamines/blood ; Chest Pain ; Diuretics/therapeutic use ; Female ; Humans ; Middle Aged ; Takotsubo Cardiomyopathy/*diagnosis/drug therapy/pathology ; Thrombosis ; Ventricular Dysfunction, Left/diagnosis/drug therapy/pathology

OBJECTIVETo investigate the effects of neoadjuvant chemotherapy on the response of surgical stress during perioperative period.

METHODSFrom July 2005 to June 2006, 30 SD rats with the same age were distributed into 3 experimental groups and 1 control group randomly. Each experimental group included 8 rats, and the control group included 6 rats. The rats in experimental groups received intravenous infusion of cyclophosphamide, while the rats in control group received IV infusion of normal sodium. The blood of rats from experimental groups was taken 30 min before and during the stimulation of abdominal surgery which was given at the end of the first, the second and the third week after receiving IV infusion cyclophosphamide. Then the plasma concentration of noradrenaline was measured respectively.

RESULTSThe IV infusion of cyclophosphamide before operation could greatly improve the plasma level of norepinephrine, and it reached peak at the end of the second week. When surgeries were given at that time, the stress response became much more seriously. The response induced by neoadjuvant chemotherapy disappeared 3 weeks later.

CONCLUSIONSNeoadjuvant chemotherapy with cyclophosphamide can induce significant stress response which is characterized by the increase of plasma level of norepinephrine. It indicates that the best time of surgery for patients who have received neoadjuvant chemotherapy with cyclophosphamide is 2 weeks later.

Animals ; Catecholamines ; blood ; Chemotherapy, Adjuvant ; Cyclophosphamide ; administration & dosage ; adverse effects ; therapeutic use ; Female ; Immunosuppressive Agents ; administration & dosage ; adverse effects ; therapeutic use ; Infusions, Intravenous ; Male ; Perioperative Care ; adverse effects ; methods ; Random Allocation ; Rats ; Rats, Sprague-Dawley ; Stress, Physiological ; etiology

OBJECTIVETo compare the effects of Shenfu Injection (SFI) and epinephrine (EPI) on catecholamine levels in a porcine model of prolonged cardiac arrest (CA).

METHODSAfter 8 min of untreated ventricular fibrillation, 24 Wuzhishan miniature pigs were randomly assigned to one of the three groups (n=8 per group) and received central venous injection, respectively: SFI group (1 mL/kg), EPI group (20 μg/kg EPI), and normal saline (NS) group. Cardiac output (CO), maximum rate of increase/decrease in left ventricular pressure (±dp/dt), serum levels of EPI, norepinephrine (NE), and dopamine (DA) were determined at baseline and at 0.5, 1, 2, and 4 h after restoration of spontaneous circulation.

RESULTSThe duration of cardiopulmonary resuscitation was shorter in the EPI and SFI groups than in the NS group (P<0.05). The EPI level increased significantly after restoration of spontaneous circulation (ROSC) in all three groups, and was significantly different between the EPI group and the other two groups immediately after ROSC (both P<0.01), but these differences gradually disappeared over time. There were no significant differences in NE or DA levels among the three groups, and there were no correlations between catecholamine levels and CO or dp/dt (P>0.05).

CONCLUSIONSSFI did not significantly affect endogenous catecholamine levels during cardiopulmonary resuscitation after prolonged ventricular fibrillation. However, SFI improved oxygen metabolism, and produced a better hemodynamic status compared with EPI. SFI might be a potentially vasopressor drug for the treatment of CA.

Animals ; Cardiac Output ; drug effects ; Cardiopulmonary Resuscitation ; Catecholamines ; blood ; Disease Models, Animal ; Drugs, Chinese Herbal ; administration & dosage ; pharmacology ; therapeutic use ; Epinephrine ; pharmacology ; therapeutic use ; Heart Arrest ; blood ; drug therapy ; Heart Ventricles ; physiopathology ; Injections ; Lactic Acid ; blood ; Sus scrofa

Percutaneous cardiopulmonary support (PCPS) is a widely accepted treatment for severe cardiopulmonary failure. This system, which uses a percutaneous approach and autopriming devices, can be rapidly applied in emergency situations. We sought to identify the risk factors that could help predict in-hospital mortality, and to assess its outcomes in survivors. During a 2-yr period, 50 patients underwent PCPS for the treatment of severe cardiopulmonary failure, and of those, 22 (44%) were classified as survivors and 28 (56%) as non-survivors. We compared the 2 groups for risk factors of in-hospital mortality and to establish proper PCPS timing. Twenty patients underwent PCPS for acute myocardial infarction, 20 for severe cardiopulmonary failure after cardiac surgery, 7 for acute respiratory distress syndrome, and 3 for acute myocarditis. Multivariate analysis showed that an acute physiology, age, and chronic health evaluation (APACHE) III score > or =50 prior to PCPS was the only significant predictor of in-hospital mortality (P=0.001). Overall 18-month survival was 42.2%. Cox analysis showed patients with APACHE III scores > or =50 had a poor prognosis (P=0.001). Earlier application of PCPS, and other preemptive strategies designed to optimize high-risk patients, may improve patient outcomes. Identifying patients with high APACHE scores at the beginning of PCPS may predict in-hospital mortality. Survivors, particularly those with higher APACHE scores, may require more frequent follow-up to improve overall survival.
*APACHE ; Adult ; Aged ; Aged, 80 and over ; *Cardiopulmonary Resuscitation/methods/utilization ; Catecholamines/therapeutic use ; Female ; Heart Failure/mortality/*therapy ; Hospital Mortality ; Humans ; Male ; Middle Aged ; Regression Analysis ; Retrospective Studies ; Risk Factors ; Survival Rate ; Treatment Outcome

BACKGROUNDShen-Fu injection (SFI) can attenuate ischemia-reperfusion injury, protect cardiac function, and improve microcirculation during cardiopulmonary resuscitation. We hypothesized that SFI may also have an influence on myocardial metabolism during ventricular fibrillation (VF). In this study, we used SFI pretreatment prior to VF to discuss the changes of myocardial metabolism and catecholamine (CA) levels during untreated VF, trying to provide new evidence to the protection of SFI to myocardium.

METHODSTwenty-four pigs were divided into three groups: Saline group (SA group), SFI group, and SHAM operation group (SHAM group). Thirty minutes prior to the induction of VF, the SFI group received 0.24 mg/ml SFI through an intravenous injection; the SA group received an equal amount of sodium chloride solution. The interstitial fluid from the left ventricle (LV) wall was collected through the microdialysis tubes during VF. Adenosine diphosphate (ADP), adenosine triphosphate (ATP), and Na + -K + -ATPase and Ca2 + -ATPase enzyme activities were measured after untreated VF. Peak-to-trough VF amplitude and median frequency were analyzed for each of these 5-s intervals.

RESULTSThe levels of glucose and glutamate were lower after VF in both the SA and SFI groups, compared with baseline, and the levels in the SFI group were higher than those in the SA group. Compared with baseline, the levels of lactate and the lactate/pyruvate ratio increased after VF in both SA and SFI groups, and the levels in the SFI group were lower than those in the SA group. In both the SA and SFI groups, the levels of dopamine, norepinephrine, and epinephrine increased significantly. There were no statistical differences between the two groups. The content of ATP, ADP, and phosphocreatine in the SFI group was higher than those in the SA group. The activity of LV Na + -K + -ATPase was significantly higher in the SFI group than in the SA group. Amplitude mean spectrum area (AMSA) was significantly lower in the SA and SFI groups at 8- and 12-min compared with 4-min. The AMSA in the SFI group was higher than that in the SA group at each time point during untreated VF.

CONCLUSIONSSFI pretreatment can improve myocardial metabolism and reduce energy exhaustion during VF, and it does not aggravate the excessive secretion of endogenous CAs.

Animals ; Catecholamines ; metabolism ; Disease Models, Animal ; Drugs, Chinese Herbal ; administration & dosage ; therapeutic use ; Energy Metabolism ; Extracellular Fluid ; metabolism ; Female ; Injections, Intravenous ; Male ; Myocardium ; metabolism ; Swine ; Ventricular Fibrillation ; drug therapy ; metabolism

OBJECTIVETo analyze the clinical characteristics of the patient with tyrosine hydroxylase deficiency, and investigate it's molecular mechanism.

METHODThe clinical characteristics of a patient with tyrosine hydroxylase deficiency were summarized and analyzed, his and his family's peripheral blood specimens were collected after informed consent was signed. All exons and the intron-exon boundaries of guanosine triphosphate hydroxylase I gene, tyrosine hydroxylase gene and sepiapterin reductase gene were examined by DNA-PCR, bi-directional sequencing.

RESULTThe patient was a 3-year-old boy, presented with unexplained dystonia for 3 years, without significant impairment of intelligence. Physical examination showed limb muscle strength grade V, rigidity of extremities, hypertonicity, brisk deep tendon reflexes in limbs, without obvious abnormalities in auxiliary examination, such as brain MRI, hepatic biochemical panel, creatine kinase, and ceruloplasmin. He dramatically responded to small doses of levodopa in the follow-up for half a year. A homozygous missense change in exon 5 of TH gene, c.605G > A (p.R202H), which was a known pathogenic mutation, was found in the patient. His parents were heterozygous for the R202H mutation.

CONCLUSIONThe age of onset in tyrosine hydroxylase deficiency patients is usually within the first year of life. Unexplained dystonia and hypokinesia were the main clinical features of tyrosine hydroxylase deficiency. The dopa-responsive effects for some patients are so obvious that we should strengthen awareness of the disease. TH gene c.605G > A (p.R202H) may be a common type of causative mutations for the mild form at home and abroad.

Brain ; metabolism ; pathology ; Catecholamines ; biosynthesis ; Child, Preschool ; DNA ; genetics ; DNA Mutational Analysis ; Dopamine Agents ; administration & dosage ; therapeutic use ; Dystonic Disorders ; drug therapy ; genetics ; metabolism ; Homozygote ; Humans ; Hypokinesia ; drug therapy ; genetics ; metabolism ; Levodopa ; administration & dosage ; therapeutic use ; Male ; Muscle Rigidity ; drug therapy ; genetics ; metabolism ; Mutation, Missense ; Polymerase Chain Reaction ; Tyrosine 3-Monooxygenase ; deficiency ; genetics ; metabolism