Health related quality of life after corrective surgery for congenital heart disease.

UNLABELLED: After corrective surgery for congenital heart defects (CHD) many patients suffer from residual defects, some with reduced cardiorespiratory capacity and possible impairment of their health related quality of life (HRQoL). The aim of our study is to evaluate, how children after surgery for CHD rate their HRQoL. METHOD: A standardised questionnaire deve-loped for self-rating in children between 8 and 18 years and dealing with 7 different domains concerning the HRQoL (friends, family, physical functioning, cognition, body image, emotional function and autonomy) was independently answered from patients and their parents during an outpatient visit at their pediatric cardiologist throughout Germany. RESULTS: 173 patients (40% female, 60% male, mean age 11.6 years) were interviewed, 167 questionnaires could be evaluated. The mean time interval after surgery was 9.8±3.4 years. Patient had had surgery for complete different types of CHD (ventricular septal defects n=50, Tetralogy of Fallot n=51, univentricular heart n=26, transposition of great arteries n=40). The results were compared with those of an age-matched control group (n=169). Patients with CHD reported a better HRQoL than the controls for all items (p<0.01). There was no significant gender specific difference. After puberty, the rating for most items, except of "body image", had become very similar compared to controls. Pa-rents assessed their children significantly worse in 3 domains (friends, body image and emotion; p<0.01). CONCLUSION: Children with congenital heart defects are able to develop coping structures, that enable them to live a normal life from their individual point of view. Integration in psychosocial structures seems to be rather normal when compared to healthy controls. Many patients considered their HRQoL as even better.

Relating road salt to exceedances of the water quality standard for chloride in New Hampshire streams.

Six watersheds in New Hampshire were studied to determine the effects of road salt on stream water quality. Specific conductance in streams was monitored every 15 min for one year using dataloggers. Chloride concentrations were calculated from specific conductance using empirical relationships. Stream chloride concentrations were directly correlated with development in the watersheds and were inversely related to streamflow. Exceedances of the EPA water quality standard for chloride were detected in the four watersheds with the most development. The number of exceedances during a year was linearly related to the annual average concentration of chloride. Exceedances of the water quality standard were not predicted for streams with annual average concentrations less than 102 mg L(-1). Chloride was imported into three of the watersheds at rates ranging from 45 to 98 Mg Cl km(-2) yr(-1). Ninety-one percent of the chloride imported was road salt for deicing roadways and parking lots. A simple, mass balance equation was shown to predict annual average chloride concentrations from streamflow and chloride import rates to the watershed. This equation, combined with the apparent threshold for exceedances of the water quality standard, can be used for screening-level TMDLs for road salt in impaired watersheds.

The evolution of the science of Bear Brook Watershed in Maine, USA.

The Bear Brook Watershed in Maine (BBWM), USA is a paired watershed study with chemical manipulation of one watershed (West Bear = WB) while the other watershed (East Bear = EB) serves as a reference. Characterization of hydrology and chemical fluxes occurred in 1987-1989 and demonstrated the similarity of the ca. 10 ha adjacent forested watersheds. From 1989-2010, we have added 1,800 eq (NH(4))(2)SO(4) ha(-1) y(-1) to WB. EB runoff has slowly acidified even as atmospheric deposition of SO4(-2) has declined. EB acidification included decreasing pH, base cation concentrations, and alkalinity, and increasing inorganic Al concentration, as SO4(-2) declined. Organic Al increased. WB has acidified more rapidly, including a 6-year period of increasing leaching of base cations, followed by a long-term decline of base cations, although still elevated over pretreatment values, as base saturation declined in the soils. Sulfate in WB has not increased to a new steady state because of increased anion adsorption accompanying soil acidification. Dissolved Al has increased dramatically in WB; increased export of particulate Al and P has accompanied the acidification in both watersheds, WB more than EB. Nitrogen retention in EB increased after 3 years of study, as did many watersheds in the northeastern USA. Nitrogen retention in WB still remains at over 80%, in spite of 20+ years of N addition. The 20-year chemical treatment with continuous measurements of critical variables in both watersheds has enabled the identification of decadal-scale processes, including ecosystem response to declining SO4(-2) in ambient precipitation in EB and evolving mechanisms of treatment response in WB. The study has demonstrated soil mechanisms buffering pH, declines in soil base saturation, altered P biogeochemistry, unexpected mechanisms of storage of S, and continuous high retention of treatment N.

Landscape controls on mercury in streamwater at Acadia National Park, USA.

Fall and spring streamwater samples were analyzed for total mercury (Hg) and major ions from 47 locations on Mount Desert Island in Maine. Samples were collected in zones that were burned in a major wildfire in 1947 and in zones that were not burned. We hypothesized that Hg concentrations in streamwater would be higher from unburned sites than burned watersheds, because fire would volatilize stored Hg. The Hg concentrations, based on burn history, were not statistically distinct. However, significant statistical associations were noted between Hg and the amount of wetlands in the drainage systems and with streamwater dissolved organic carbon (DOC). An unexpected result was that wetlands mobilized more Hg by generating more DOC in total, but upland DOC was more efficient at transporting Hg because it transports more Hg per unit DOC. Mercury concentrations were higher in samples collected at lower elevations. Mercury was positively correlated with relative discharge, although this effect was not distinguished from the DOC association. In this research, sample site elevation and the presence of upstream wetlands and their associated DOC affected Hg concentrations more strongly than burn history.

Mass balances of mercury and nitrogen in burned and unburned forested watersheds at Acadia National Park, Maine, USA.

Precipitation and streamwater samples were collected from 16 November 1999 to 17 November 2000 in two watersheds at Acadia National Park, Maine, and analyzed for mercury (Hg) and dissolved inorganic nitrogen (DIN, nitrate plus ammonium). Cadillac Brook watershed burned in a 1947 fire that destroyed vegetation and soil organic matter. We hypothesized that Hg deposition would be higher at Hadlock Brook (the reference watershed, 10.2 microg/m(2)/year) than Cadillac (9.4 microg/m(2)/year) because of the greater scavenging efficiency of the softwood vegetation in Hadlock. We also hypothesized the Hg and DIN export from Cadillac Brook would be lower than Hadlock Brook because of elemental volatilization during the fire, along with subsequently lower rates of atmospheric deposition in a watershed with abundant bare soil and bedrock, and regenerating vegetation. Consistent with these hypotheses, Hg export was lower from Cadillac Brook watershed (0.4 microg/m(2)/year) than from Hadlock Brook watershed (1.3 microg/m(2)/year). DIN export from Cadillac Brook (11.5 eq/ha/year) was lower than Hadlock Brook (92.5 eq/ha/year). These data show that approximately 50 years following a wildfire there was lower atmospheric deposition due to changes in forest species composition, lower soil pools, and greater ecosystem retention for both Hg and DIN.

Controls on mercury and methylmercury deposition for two watersheds in Acadia National Park, Maine.

Throughfall and bulk precipitation samples were collected for two watersheds at Acadia National Park, Maine, from 3 May to 16 November 2000, to determine which landscape factors affected mercury (Hg) deposition. One of these watersheds, Cadillac Brook, burned in 1947, providing a natural experimental design to study the effects of forest type on deposition to forested watersheds. Sites that face southwest received the highest Hg deposition, which may be due to the interception of cross-continental movement of contaminated air masses. Sites covered with softwood vegetation also received higher Hg deposition than other vegetation types because of the higher scavenging efficiency of the canopy structure. Methyl mercury (MeHg) deposition was not affected by these factors. Hg deposition, as bulk precipitation and throughfall was lower in Cadillac Brook watershed (burned) than in Hadlock Brook watershed (unburned) because of vegetation type and watershed aspect. Hg and MeHg inputs were weighted by season and vegetation type because these two factors had the most influence on deposition. Hg volatilization was not determined. The total Hg deposition via throughfall and bulk precipitation was 9.4 microg/m(2)/year in Cadillac Brook watershed and 10.2 microg/m(2)/year in Hadlock Brook watershed. The total MeHg deposition via throughfall and bulk precipitation was 0.05 microg/m(2)/year in Cadillac Brook watershed and 0.10 microg/m(2)/year in Hadlock Brook watershed.

Paleoecological assessment of watershed history in PRIMENet watersheds at Acadia National Park, USA.

Paleoecological reconstructions of forest stand histories for two upland watersheds at Acadia National Park in Maine were completed to support related watershed chemistry studies. The project hypothesis was that forest type and fire history influence long-term cycling and storage of atmospheric mercury and nitrogen within watersheds. The reconstructions document differences in major vegetation composition and disturbance between the burned and unburned watersheds during the past several centuries. Pollen and charcoal stratigraphies from organic sediment accumulations in forested wet depressions indicate that the present experimental design of contrasting disturbance and forest histories has persisted during recent centuries. The unburned watershed has been dominated by spruce (Picea rubens) and fir (Abies balsamea) for 500 years or more and has not recently burned or been substantially cleared. The burned watershed is dominated by a heterogeneous forest of patchy hardwood, mixed wood, and softwood stands. A large portion of this watershed burned severely in 1947 and probably more than once in the 1800s, and has supported heterogeneous successional forests for 200 years or longer. Overall, these results support the underlying premise that the experimental design of this watershed research can be used to infer landscape controls on biogeochemical processes.

Watershed nitrogen and mercury geochemical fluxes integrate landscape factors in long-term research watersheds at Acadia National Park, Maine, USA.

This paper is an overview of this special issue devoted to watershed research in Acadia National Park (Acadia NP). The papers address components of an integrated research program on two upland watersheds at Acadia NP, USA (44 degrees 20' N latitude; 68 degrees 15' E longitude). These watersheds were instrumented in 1998 to provide a long-term foundation for regional ecological and watershed research. The research was initiated as part of EPA/NPS PRIMENet (Park Research and Intensive Monitoring of Ecosystems Network), a system of UV-monitoring stations and long-term watershed research sites located in US national parks. The initial goals at Acadia NP were to address research questions about mercury, acid rain, and nitrogen saturation developed from prior research. The project design was based on natural differences in forests and soils induced by an intense wildfire in one watershed in 1947. There is no evidence of fire in the reference watershed for several hundred years. We are testing hypotheses about controls on surface water chemistry, and bioavailability of contaminants in the contrasting watersheds. The unburned 47-ha Hadlock Brook watershed is 70% spruce-fir mature conifer forest. In contrast, burned 32-ha Cadillac Brook watershed, 4 km northeast of the Hadlock watershed, is 20% regenerating mixed northern hardwoods and 60% shrub/rocky balds. Differences in atmospheric deposition are controlled primarily by forest stand composition and age. The watersheds are gauged and have water chemistry stations at 122 m (Cadillac) and 137 m (Hadlock); watershed maximum elevations are 468 and 380 m, respectively. The stream water chemistry patterns reflect, in part, the legacy of the intense fire, which, in turn, controls differences in forest vegetation and soil characteristics. These factors result in higher nitrogen and mercury flux from the unburned watershed, reflecting differences in atmospheric deposition, contrasting ecosystem pools of nitrogen and mercury, and inferred differences in internal cycling and bioavailabilty.

Effect of acid rain on building material of the El Tajín archaeological zone in Veracruz, Mexico.

Three limestone slabs (approx. 20 cm2 each) were extracted from the El Tajin archaeological zone in Veracruz, Mexico. X-ray diffraction analysis revealed three components: calcite (81.2%), quartz (17.9%) and feldspar (0.9%). Calcite content by x-ray diffraction analysis was slightly higher than that determined by chemical reaction between the limestone sample and nitric acid. The latter analysis, carried out in triplicate, yielded a calcite content of 77.1%. Mean water absorption, density and porosity of the limestone samples were also determined. Dissolution of limestone samples was investigated using an experimental rainfall simulation chamber, in which the stone samples were irrigated with artificial rain matching the pH and the ionic composition with 40 rainfall samples collected at El Tajin from August 18, 2002, to April 9, 2003. According to calcium and bicarbonate net concentrations found in the effluent of the chamber, a chemical mechanism by which limestone at El Tajin is dissolved by acid rain is proposed. A model used to investigate the air transport pathways corresponding to precipitation events at El Tajin shows that air parcels come mainly from the Gulf of Mexico, although no directional preference is evident for acidic vs. non-acidic events.

[The vaccination coverage among children aged 24 - 30 months in pediatric offices November 1999 - May 2001. The influence of socio-demographic characteristics]. / Die Durchimpfung von 24 - 30 Monate alten Kindern in pädiatrischen Praxen im Zeitraum von November 1999 bis Mai 2001 - Der Einfluss soziodemografischer Faktoren.

BACKGROUND: The goal of this study was to investigate the influence of socio-demographic factors on vaccination coverage of 24-30 month old children in paediatric practices. METHODS: Vaccination coverage (VC) was documented for 15,682 children in 196 paediatric practices mainly in the region of Nordrhein. Data on socio-demographic factors were collected for 8,457 children and their influence on the vaccination status (VS) was investigated by means of logistic regression analysis. RESULTS: Complete age-appropriate VC for all vaccines recommended by the Standing Committee for Vaccination (STIKO) was 49.9 %. Complete VC for Diphtheria/Tetanus (DT) was 74.6 % and for first dose measles/mumps/rubella (MMR), 82.1 %. The following factors were associated with complete VS for DT and MMR: completion of last recommended well-child visit, parents living together, low number of siblings, short time to reach practice, parents stating they felt adequately informed about vaccinations. Complete vaccination for DT was inversely associated with being health insured through social security and for MMR with the mother having graduated from high school. CONCLUSIONS: A high proportion of 24-30 month old children did not have an age appropriate VS. Targeted parental education and timely vaccination strategies that take into account socio-demographic risk factors for low vaccination uptake are required.

Estimation of critical loads of acidity for lakes in northeastern United States and eastern Canada.

The New England Governors and Eastern Canadian Premiers (NEG/ECP) adopted the Acid Rain Action Plan in June 1998, and issued a series of action items to support its work toward a reduction of sulfur dioxide (SO(2)) and nitrogen oxide (NO(x)) emissions in northeastern North America. One of these action items was the preparation of an updated critical load map using data from lakes in the NEG/ECP area. Critical load maps provide a more complete index of the surface water sensitivity to acidification. Combined sulfur and nitrogen critical loads and deposition exceedances were computed using Henriksen's Steady-State Water Chemistry (SSWC) model. Results show that 28% of all 2053 lakes studied have a critical load of 20 kg/ha/year or less, making them vulnerable to acid deposition. Emission reductions, and more specifically SO(2) emission reductions have proven beneficial because critical loads were exceeded in 2002 for 12.3% of all studied lakes. Those lakes are located in the more sensitive areas where geology is carbonate-poor. Of these lakes, 2.9% will never recover even with a complete removal of SO(4) deposition. Recovery from acidification for the remaining 9.4% of the lakes will require additional emission SO(2) reductions.

Background mercury concentrations in river water in Maine, U.S.A.

Mercury concentrations in 58 rivers in Maine was measured to range from below detection up to 7.01 ng L(-1) and averaged 1.80 +/- 1.29 ng L(-1). The concentration gradient for mercury in rivers across the state was not uniform. Mercury strongly correlated with dissolved organic carbon (DOC) and aluminum, and less strongly with copper, lead, and zinc. Mercury exhibited significant differences in correlations with chemical variables and local geology when partitioned by flow state (high or low). Mercury concentrations were greatest in rivers flowing across either wacke-type bedrock at low metamorphic grade, or glacial-till deposits. Elevated concentrations of mercury formed a locus in northern Maine under both high and low-flow states while in southwestern Maine a locus formed only during high-flow states. These regional differences were statistically significant when compared by geographical location. We suggest that there is a bedrock source of mercury in northeastern Maine that is diluted during periods of high runoff. The elevated concentrations detected under high-flow states, as noted in southwestern Maine, may reflect mercury released from storage in association with DOC during periods of high runoff. The association of mercury with flow state indicates that watershed processes and local geology can modulate the concentration of mercury in rivers.

Association of methylmercury with dissolved humic acids.

Sorption of methylmercury (MeHg) to three different humic acids was investigated as a function of pH and humic concentration. The extent of sorption did not show a strong pH dependence within the pH range of 5-9. Below pH 5, a decrease in adsorption for all humic samples was observed. The experimental data for equilibrium sorption of MeHg were modeled using a discrete log K spectrum approach with three weakly acidic functional groups. The modeling parameters, which were the equilibrium binding constants and the total binding capacities, represented the data well at all MeHg and humic concentrations and pH values for a given humic sample. The estimated binding constants for complexes of MeHg with humic acids were similar in magnitude to those of MeHg with thiol-containing compounds, suggesting that binding of MeHg involves the thiol groups of humic acids. The results show that only a small fraction of the reduced sulfur species in humic substances may take part in binding MeHg, but in most natural systems, this subfraction is considerably higher in concentration than ambient MeHg. The model developed here can be incorporated into speciation models to assess the bioavailability of MeHg in the presence of dissolved organic matter and competing ligands such as chloride and sulfide.

Template-free segmental synthesis of oligonucleotides containing nonnative linkages.

Protected oligonucleotides containing 3'-alkyl carboxylic acids or 3'-alkylamines were obtained from photolabile solid-phase synthesis supports (1 and 4). Protected oligonucleotides containing 5'-alkylamines and 3'-hydroxyl groups were obtained using a photolabile solid-phase synthesis support (2) and a commercially available phosphoramidite reagent (3). Depending upon the source of alkylamine-containing oligonucleotide, the segments were coupled under mild conditions to form products containing either 5'-3' or 3'-3' linkages in good yield and high purity. Oligonucleotides as long as 40 nucleotides were prepared, and coupling yields of protected biopolymer segments were independent of length over the range examined. This method is particularly well suited for the convergent synthesis of oligonucleotides containing nonnative linkages and should be useful for the rapid assembly of modified biopolymers that are useful in biochemical studies.

The use of dreams in psychotherapy: a survey of psychotherapists in private practice.

Since the publication of Sigmund Freud's The Interpretation of Dreams, dream interpretation has been a standard technique often used in psychotherapy. However, empirical studies about the frequency of working on dreams in therapy are lacking. The present study elicited, via a self-developed questionnaire, various aspects of work on dreams applied by psychotherapists in private practice. The findings indicate that dreams were often used in therapy, especially in psychoanalysis. In addition, a significant relationship was found between the frequency of the therapists' working on their own dreams and frequency of work on dreams in therapy. Because work on dreams was rated as beneficial for the clients, further studies investigating the effectiveness and the process of working on dreams will be of interest.

Herbivore-induced ethylene suppresses a direct defense but not a putative indirect defense against an adapted herbivore.

Herbivory induces both direct and indirect defenses in plants; however, some combinations of these defenses may not be compatible. The jasmonate signal cascade activated both direct (nicotine accumulations) and indirect (mono- and sesquiterpene emissions) whole-plant defense responses in the native tobacco Nicotiana attenuata Torr. Ex Wats. Nicotine accumulations were proportional to the amount of leaf wounding and the resulting increases in jasmonic acid (JA) concentrations. However, when larvae of the nicotine-tolerant herbivore, Manduca sexta, fed on plants or their oral secretions were applied to leaf punctures, the normal wound response was dramatically altered, as evidenced by large (4- to 10-fold) increases in the release of (i) volatile terpenoids and (ii) ethylene, (iii) increased (4- to 30-fold) accumulations of endogenous JA pools, but (iv) decreased or unchanged nicotine accumulations. The ethylene release, which was insensitive to inhibitors of induced JA accumulation, was sufficient to account for the attenuated nicotine response. Applications of ethylene and ethephon suppressed the induced nicotine response and pre-treatment of plants with a competitive inhibitor of ethylene receptors, 1-methylcyclopropene, restored the full nicotine response. This ethylene burst, however, did not inhibit the release of volatile terpenoids. Because parasitoids of Manduca larvae are sensitive to the dietary intake of nicotine by their hosts, this ethylene-mediated switching from direct to a putative indirect defense may represent an adaptive tailoring of a plant's defense response.

Ecophysiological comparison of direct and indirect defenses in Nicotiana attenuata.

After herbivore attack, plants launch a suite of direct and indirect defense responses that must be coordinated if plants are to realize a fitness benefit from these responses. Here we characterize the volatile emissions in the native tobacco plant, Nicotiana attenuata Torr. ex Wats., that are elicited by tobacco hornworm (Manduca sexta L.) attack and are known to function as attractants for parasitoids. To provide the first ecophysiological comparison of examples of both types of defense in the same species, we characterize the elicitation and signaling mechanisms, the resources required, and the potential costs and benefits of the volatile release and compare these traits with those of the well-described induced direct defense in this species, nicotine production. The release of (E)-ß-ocimene, cis-α-bergamotene and linalool is dramatically induced within 24 h by application of methyl jasmonate (MeJA), caterpillar feeding, and the treatment of mechanical wounds with larval oral secretions (OS), but not by mechanical damage alone. Plants from different geographic locations produce volatile blends that differ in composition. The most consistently released component from all genotypes, cis-α-berga-motene, is positively related to the amount of MeJA and the level of wounding if OS are applied to the wounds. The volatile release is strongly light dependent, dropping to undetectable quantities during dark periods, even when temperatures are elevated to match those of the light period. Inhibitors of wound-induced jasmonate accumulation (salicylates and auxins), which are known to inhibit wound-induced nicotine production, do not inhibit the release of volatiles. By individually inducing different leaf positions with OS and, on other plants, excising them after induction, we demonstrate that the emission is largely a systemic, whole-plant response, which is maximally triggered when the second fully expanded leaf is induced. We conclude that while both are whole-plant, systemic responses that utilize recently acquired resources for their production and are activated by the jasmonate cascade, the elicitation of the volatile release exhibits greater tissue sensitivity and utilizes additional signaling components than does nicotine production. In contrast to the large investment of fitness-limiting resources required for induced nicotine production or the resources used in benzyl acetone release from flowers for pollinator attraction, the resource requirements for the volatile release are minor. Hence the argument that the volatile release incurs comparatively large physiological costs cannot be supported in this system.

Development of a decision support system for the introduction of alternative methods into local irritancy/corrosivity testing strategies. Development of a relational database.

For new chemical substances that are notified within the European Union, data sets have to be submitted to the National Competent Authorities. The data submitted have to demonstrate the physicochemical and toxic properties of the new chemical, such as solubility, partition coefficients and spectra, as well as acute toxic properties and the potential to cause local irritant or corrosive effects. In order to minimise testing for notification purposes (for example, animal testing), it is necessary to develop stepwise assessment procedures, including structure-activity considerations, alternative methods (for example, in vitro tests), and computerised structure-activity relationship (SAR) models. An electronic database was developed which contains physicochemical and toxicological data on approximately 1300 chemical substances. It is used for regulatory structure-property relationship (SPR) and SAR considerations, and for the development of rules for a decision support system (DSS) for the introduction of alternative methods into local irritancy/corrosivity testing strategies. The information stored in the database is derived from proprietary data, so it is not possible to publish the data directly. Therefore, the database is evaluated by regulators, and the information derived from the data is used for the development of scientific information about SARs. This information can be published, for example, by means of tables correlating measured physicochemical values and specific toxic effects caused by the measured chemical. This information is introduced to the public by means of a DSS that predicts local irritant/corrosive potential of a chemical by listing so called exception rules of the kind IF (physicochemical property) A THEN not (toxic) Effect B and so-called structural rules of the kind IF Substructure A THEN Effect B. These DSS rules "translate" proprietary data into scientific knowledge that can be published.

Acetaminophen glucuronidation accurately reflects gluconeogenesis in fasted dogs.

To assess whether acetaminophen glucuronide accurately reflects uridyl diphosphate-glucose (UDP-glucose) derived from gluconeogenesis during fasting, three mongrel dogs received infusions of [U-14C]lactate, [1-13C]galactose, and [6-3H]glucose (after fasting overnight or for 2.5 days). After initiation of the isotopes (3 h), acetaminophen was given, and the urinary acetaminophen glucuronide was isolated. The mean plasma [14C]glucose specific activity (SA) was similar to the mean urinary acetaminophen glucuronide SA both after fasting overnight [299 +/- 19 vs. 296 +/- 14 disintegrations.min-1 (dpm).mumol-1, respectively] and after 2.5 days of fasting (511 +/- 8 vs. 562 +/- 32 dpm/mumol, respectively). Mean plasma glucose flux calculated using [6-3H]glucose decreased (P < 0.05) with two additional days of fasting (18.7 +/- 1.2 vs. 13.6 +/- 0.6 mumol.kg-1.min-1), as did intrahepatic (P < 0.05) UDP-glucose flux measured using [1-13C]galactose (8.6 +/- 0.7 vs. 5.5 +/- 0.3 mumol.kg-1.min-1). We conclude that, in fasted dogs, plasma glucose and UDP-glucose, as sampled by acetaminophen, equally reflect gluconeogenesis and appear to come from the same pool of glucose 6-phosphate. In addition, cycling of glucose moieties through UDP-glucose and glycogen decreases with an increased period of fasting.

Accelerated contractures after administration of ryanodine to skeletal muscle of malignant hyperthermia susceptible patients.

A genetic disorder of the calcium releasing ryanodine receptor has recently been postulated in malignant hyperthermia (MH) and ryanodine-induced contractures differ between subjects who are malignant hyperthermia susceptible (MHS) and non-susceptible (MHN). We tested 39 patients from 26 families for MH, using the procedure of the European Malignant Hyperthermia Group. A ryanodine contracture test was performed by both cumulative (0.4-10.0 mumol litre-1 every 3 min) and bolus (10.0 mumol litre-1) application. Contracture with cumulative ryanodine application started significantly earlier in MHS (9.6 (SEM 0.5) min) than in MHN patients (24.6 (1.3) min). A significant difference in start of contracture between MHS (4.8 (0.6) min) and MHN (14.5 (0.6) min) patients occurred also after bolus application of ryanodine. The ryanodine contracture test seems to be a potentially specific in vitro diagnostic test for MH.