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Frozen Summer Treat Recipe: Pineapple Sorbet
Pineapple Sorbet Ingredients: 1 pineapple 1/4 cup sugar 1/4 cup lemon juice Directions: You can scoop out the pineapple meat from the pineapple so you can use it later as a container or you can just peel,core and dice the pineapple. Combine the ingredients in a blender and process until smooth. You can leave some pineapple meat and just roughly chop it, to add later if you don't like a smooth sorbet. If you have an ice cream maker, you can use that to freeze the sorbet. If you don't have an ice cream maker, put the mixture in a container and freeze. You have to stir it with a fork from time to time to achieve desired texture.
Thank you for sharing, Peachy! This looks like such an easy and refreshing summer dessert.
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"Anywhere I Lay My Head" (CD single)
What parents need to know
Parents Need to Know
Parents need to know that the title single from the upcoming cover album of Tom Waits' songs is a bit sober sounding, but it doesn't contain any objectionable content. The updated techno-ish beat will probably appeal more to kids than the original version of the song.
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What's the story?
Box-office bombshell covers a raspy blues icon? The pairing is a curious one. Scarlett Johansson is set to release an album of Tom Waits covers in May, with this being the title track and first single. Waits, an iconic singer/songwriter, is probably most famous for his trademark raspy voice, and Johansson follows other musicians -- from Rod Stewart to Bruce Springsteen -- in revamping some of his classics. With some help from production effects (predominantly drum machine beats), she covers a song about desolation and alienation.
Is it any good?
At first listen, the single sounds sadly over-produced. Johansson's voice is initially lost to the thumping beats and techno tricks. It's not the raw emotion of the Tom Waits' original; instead this interpretation is emotionally and musically buffered. Johansson's voice even sounds like she's singing from a great distance away. Even though her vocals may sound more like speaking at times, this is a solid attempt at a difficult song and Johansson should be credited with creating an original, moody single that stands apart from its predecessor.
Families can talk about...
Families can talk about why Johansson chose to break into singing while enjoying such a successful acting career. And, why do you think she chose to cover Waits' songs for her debut album? Families can also talk about why performers make cover albums. Listening to the original versions of this song can offer a good way to compare and contrast vocal and production styles.
Music details
|Scarlett Johansson
|Release date:
|April 17, 2008
|Atco, Rhino Records
|Indie Rock
|Parental advisory:
|Edited version available:
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Nathan VeldhoenShared publicly -
Triathlon training camp in Las Vegas this past week.
After Christmas is general a bit tough for me with the lack of vitamin d , so naturally the Every Man Jack training camp is something to look forward to. This year it was in Las Vegas, moved from San Diego the previous year. A few of the members live in the area, so we had local knowledge of the sweet spots to ride and run. Coming into camp I did a massive number of rides outside, so naturally I would feel comfortable riding with 55 others.
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Folk Arts of Japanby Hugo Munsterberg Soetsu Yanagi
This edition was originally published in print form by Tuttle Publishing in 1958.The Folk Arts of Japan by Dr. Hugo Munsterberg deals with a rediscovered branch of Japanese art which, in straightfoward beauty of its products, is currently delighting the Western world. Although these folkcraft creations have their roots in the country's ancient and colorful art tradition, their unassuming grace makes them unmistakably in harmony with modern functional design.Here Dr. Munsterberg bringing to his work the fruits of four years of study in Japan and a deep knowledge of Oriental culture, makes available for the first time in English a comprehensive guide and commentary on this significant branch of Japan's varied arts.
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In the Courts of the Conqueror: The 10 Worst Indian Law Cases Ever DecidedThe fate of Native Americans has been dependent in large part upon the recognition and enforcement of their legal, political, property, and cultural rights as indigenous peoples by American courts. Most people think that the goal of the judiciary, and especially the US Supreme Court, is to achieve universal notions of truth and justice. In this in-depth examination, however, Walter Echo-Hawk
Pawnee Scouts in the U.S. ArmyBetween 1864 and 1877, during the height of the Plains Indian wars, Pawnee Indian scouts rendered invaluable service to the United States Army. They led missions deep into contested territory, tracked resisting bands, spearheaded attacks against enemy camps, and on more than one occasion saved American troops from disaster on the field of battle. In War Party in Blue, Mark van de Logt tells the
Racial Identity at the End of the Age of RaceOne day at the end of the twentieth century, Roger Echo-Hawk decided to give up being an Indian. After becoming an American Indian historian, he started to question our widespread reliance on a concept of race that the academy had long-since discredited, and embarked on a personal and professional journey to giving up race himself. This passionate book offers a powerful meditation on racialism and
The Rise of Human Rights in Native America and the UN Declaration on the Rights of Indigenous PeoplesIn 2007 the United Nations approved the United Nations Declaration on the Rights of Indigenous Peoples. United States endorsement in 2010 ushered in a new era of Indian law and policy. This book highlights steps that the United States, as well as other nations, must take to provide a more just society and heal past injustices committed against indigenous peoples.
Canada's Indigenous ConstitutionCanada's Indigenous Constitution reflects on the nature and sources of law in Canada, beginning with the conviction that the Canadian legal system has helped to engender the high level of wealth and security enjoyed by people across the country. However, longstanding disputes about the origins, legitimacy, and applicability of certain aspects of the legal system have led John Borrows to argue that
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Stories of Nishnaabeg Re-Creation, Resurgence, and a New EmergenceMany promote Reconciliation as a "new" way for Canada to relate to Indigenous Peoples. In Dancing on Our Turtle's Back: Stories of Nishnaabeg Re-Creation, Resurgence, and a New Emergence activist, editor, and educator Leanne Simpson asserts reconciliation must be grounded in political resurgence and must support the regeneration of Indigenous languages, oral cultures, and traditions of governance.
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Prevalence, mutation spectrum, and cardiac phenotype of the Jervell and Lange-Nielsen syndrome in Sweden
Aims To explore the national prevalence, mutation spectrum, cardiac phenotype, and outcome of the uncommon Jervell and Lange-Nielsen syndrome (JLNS), associated with a high risk of sudden cardiac death.
Methods and results A national inventory of clinical JLNS cases was performed. Genotype and area of origin were ascertained in index families. Retrospective clinical data were collected from medical records and interviews. We identified 19 cases in 13 Swedish families. A JLNS prevalence >1:200 000 was revealed (five living cases <10 years of age). The mutation spectrum consisted of eight KCNQ1 mutations, whereof p.R518X in 12/24 alleles. Geographic clustering of four mutations (20/24 alleles) and similarities to Norway's mutation spectrum were seen. A high prevalence of heterozygotes was suggested. Three paediatric cases on β-blockers since birth were as yet asymptomatic. Seven symptomatic cases had suffered an aborted cardiac arrest and four had died suddenly. QTc prolongation was significantly longer in symptomatic cases (mean 605 ± 62 vs. 518 ± 50 ms, P = 0.016). β-Blockers reduced, but did not abolish, cardiac events in any previously symptomatic case. β-Blocker type, dosage, and compliance probably affect outcome significantly. Implantable cardioverter-defibrillator therapy (ICD, n = 6) was associated with certain complications; however, no case of sudden death.
Conclusion Founder effects could explain 83% of the Swedish JLNS mutation spectrum and probably contribute to the high JLNS prevalence found in preadolescent Swedish children. Due to the severe cardiac phenotype in JLNS, the importance of stringent β-blocker therapy and compliance, and consideration of ICD implantation in the case of therapy failure is stressed.
- Jervell and Lange-Nielsen syndrome
- Founder effects
- KCNQ1 gene
- Cardiac phenotype
- Therapy efficacy
The Jervell and Lange-Nielsen syndrome (JLNS), first reported in Norway 1957, is characterized by congenital hearing loss, marked prolongation of the QT interval, a propensity for life-threatening ventricular arrhythmias, and a high risk of sudden cardiac death.1,2 Jervell and Lange-Nielsen syndrome prevalence remains largely unknown due to the high mortality in infancy and lack of comprehensive neonatal screening methods. Norway is, to date, the country where this uncommon hereditary disorder is reported most often, with a JLNS prevalence estimated to at least 1 : 200 000.3 Jervell and Lange-Nielsen syndrome is caused by homozygous or compound heterozygous mutations that affect the KCNQ1 and/ or KCNE1 genes, encoding subunits that co-assemble into a voltage-gated potassium ion (K+) channel.4–7 The function of the K+ channel is essential for the endolymph production of the inner-ear and the slow rectifier current of the myocyte action potential. The loss of function caused by the JLNS mutations corresponds to the associated surdocardiac phenotype.2,8,9 Heterozygous carrier-ship of JLNS mutations is associated with the long QT syndrome (LQTS), where QT prolongation, but not hearing loss, is inherited via an autosomal-dominant pattern with incomplete penetrance.10
In Sweden, where strong founder effects have caused a high regional prevalence of KCNQ1 mutations, a high probability for JLNS occurrence would intuitively be inferred.11 The aim of this study was to explore the prevalence, mutation spectrum, cardiac phenotype, and outcome of JLNS in Sweden.
In Sweden, no diagnostic code for JLNS has been available, and the prevalence of JLNS is previously unknown. A national inventory of clinical JLNS cases was performed between 2007 and 2010, and all Swedish clinics or departments of paediatric cardiology, cardiology, medicine, clinical genetics, otorhinolaryngology, audiology, and cochlear implantation were approached and asked for knowledge of cases (number, gender, and birth year). All Swedish schools teaching deaf children were similarly approached, and information regarding the inventory was spread at national meetings in paediatric cardiology, cardiology, and molecular genetics.
All identified cases and families were first approached by the tending clinicians and asked for approval of first contact. A letter was provided to each case or family with information regarding the study and an invitation to participate. A signed informed consent to participate in the study was obtained from all living cases or their legal guardian and the study was approved by the Regional Ethical Review Board, Umeå University.
Inclusion criteria were congenital bilateral hearing loss (incomplete or complete) and a clinical JLNS diagnosis (a personal history of syncopal attacks or electrocardiographic findings of a prolonged QT interval corrected for heart rate (QTc), often in combination with a family history of LQTS).
The causative mutations in participating JLNS families and cases were identified either through medical records or sequencing of the KCNQ1 and KCNE1 genes, using DNA extracted from whole-blood by standard methods, previously described in detail by the authors.11 The geographic origin of cases and recent ancestors was noted on a regional map.
Clinical data were collected from full medical records and a personal interview. The semi-structured interview was conducted with cases and/or family members, lasted on average between 2–4 h, and focused on symptoms and experiences throughout life living with JLNS. During the interview, an amnestic information regarding any deceased relative with JLNS diagnosis, and knowledge of cardiac symptoms occurrence in first-degree relatives without hearing-loss (parents, siblings, and children), were asked for specifically. Standard 12-lead electrocardiograms (ECGs), preferably recorded at the time of JLNS diagnosis, were acquired from medical records and manually measured by one observer. The measurement method has been previously described in detail.12
Statistical analysis was performed using the Mann–Whitney U test for comparison between numerical and nominal variables and the χ2 test for comparison between nominal variables. A two-tailed value for P < 0.05 was considered statistically significant.
A rough estimation of the prevalence of heterozygous mutation-carriers in the Swedish population, derived from the number of genotype-ascertained JLNS probands, was calculated using the Hardy–Weinberg equilibrium, with the reservation that the principle is based on assumptions that are not fulfilled outside a laboratory setting.13
Regarding the aim of estimating JLNS prevalence, we took the high mortality before adulthood in JLNS into consideration, and deemed a preadolescent paediatric population most appropriate for prevalence estimation. During the last decade, routine ECG screening has been implemented in the national cochlear implant clinic and the Swedish schools teaching deaf children, rendering the risk of non-detection of JLNS in children <10 years of age low. Population demographics are available through Statistics Sweden (www.scb.se) and the annual birth count is ∼100 000, rendering the number of Swedish children <10 years of age approximately one million.
Thirteen JLNS index families of Swedish descent were identified in the national inventory, including a total of 19 JLNS cases (11 females, Table 1). Seven families (12 cases) were previously known from clinical practice and/or the authors' LQTS research in the northern Swedish region, including five deceased cases. Among the deceased JLNS cases, four cases (two females) had died from suspected JLNS-related death (drowning or sudden cardiac death) and one adult male had died due to malignancy.
|KCNQ1 mutations (amino acid change)
|Region of origin (bold allele)
|Gender (F/M)
|Case (no.)a
|1, 3b
|7, 8b
|14, 16–19b,c
|DNA not available
Mutations present in ≥2 alleles are depicted in bold.
F, female; M, male;.
aCase number (no.) in accordance with Figure 2 (living cases 1–14 according to age at study, deceased cases 15–19 according to birth year).
bThe cases in families SWE1, SWE6, and SWE12 are siblings. SWE12 has been previously described.29 We present data derived from direct contact with the proband in 2009.
cJervell and Lange-Nielsen syndrome cases in family SWE12 and SWE13 are, as of 2011, all deceased.
The prevalent JLNS cases (n = 14, whereof 9 females), from 12 genotyped families, were invited to a personal interview. Mean age was 31 ± 24 years (median 31 years, range 5–88 years), and median age was the same (31 years) in both males and females.
Five now living cases <10 years of age (mean age 6 years, range 5–8 years) were identified, corresponding to a rough estimate of at least one JLNS case being born every other year, and an absolute minimum JLNS prevalence of 1 : 200 000 in Swedish children <10 years of age.
Genotype and mutation spectrum for Jervell and Lange-Nielsen Syndrome in Sweden
Genotype was investigated and ascertained in all but one of the 13 families, 6 of homozygous genotype and 6 of compound heterozygous genotype (Table 1). All identified mutations (n = 8) were located in the KCNQ1 gene, and were previously reported, either as associated with JLNS or LQTS.3,4,6,14–19 The identified KCNQ1 mutations included three missense mutations (4/24 alleles), two frame-shift mutations (5/24 alleles), one splice site mutation (1/24 alleles), and two nonsense mutations (14/24 alleles), whereof one specific nonsense mutation (p.R518X) in 12/24 alleles (Table 2). The p.Y111C missense mutation, a Swedish founder mutation with dominant negative properties, never before reported as a causative of JLNS, was identified in homozygous form in one proband.11,12 Three additional KCNQ1 mutations identified in the Swedish probands, p.R190W, p.S349W, and p.S277del, have not to our knowledge been reported previously in association with JLNS (Table 2). No KCNE1 mutations were identified.
|Nucleotide change
|Amino acid change
|Mutation type
|Alleles identified (n)a
|c.1522C > T16,19
|12 (3)
|4 (2)
|c.332A > G18
|2 (1)
|c.1588C > T3
|c.477 + 1G > A4,14
|Splice error
|c.568C > T17
|c.1046C > G18
aNumber of homozygous probands is given in parentheses.
bMutation not previously reported in association with JLNS, to our knowledge.
Ten out of 13 identified JLNS families had ancestral roots in upper northern Sweden (Table 1). Among the four mutations present in at least two alleles there were evidences of geographical clustering of probands and recent ancestors (Figure 1). Eleven of the p.R518X alleles and all p.Y111C alleles (n = 2) originated from two northern regions, and 1 p.Q530X allele and all c.572_576del alleles (n = 4) originated from two mid-western regions on the Norwegian border (Figure 1). Similarities between the JLNS mutation spectra in Sweden and Norway regarding three mutations (p.R518X, c.572_576del and p.Q530X) were seen and a high frequency of heterozygous mutation-carriers was suggested by the relatively high prevalence of JLNS probands (Table 3).
|Mutation (KCNQ1)
|12 Swedish probands (alleles = 24)
|12 Norwegian probands23 (alleles = 24)
|p.R518X, n (%)
|12 (50)
|5 (21)
|c.572_576del, n (%)
|4 (17)
|12 (50)
|p.Q530X, n (%)
|2 (8)
|6 (25)
|Sum, n (%)
|18 (75)
|23 (96)
|Estimation of heterozygotesa
|1 : 31723
aCalculated using the Hardy–Weinberg equilibrium of allele frequencies.13
Cardiac phenotype
An overview of the cardiac phenotype seen over time in 19 JLNS cases (11 females), whereof 5 deceased cases (2 females), is depicted in Figure 2. Experience of a first cardiac event was present in 16/19 cases. The three cases without experience of a first cardiac event (all females) were between 4 and 6 years at the time of the study, and had received prophylactic β-blocker therapy since birth. Age at first cardiac event was available in 11/16 symptomatic cases; mean age 2.4 ± 1.2 years, median 2.5 years, and range 0.5–4.5 years (Table 4). In five cases (all siblings), symptoms debut according to anamnesis occurred in 'early childhood', the parents of the siblings were deceased, and there were no medical records available (Figure 2).
|All cases
|Cases, n (%)
|19 (100)
|11 (58)
|8 (42)
|Experience of a first CE, n (%)
|16 (84)
|8 (73)
|8 (100)
|Age at first CE, years (median)
|2.4 ± 1.2 (2.5)
|2.7 ± 1.2 (3)
|2.1 ± 1.4 (2)
|ACA/SCD, n (%)
|12 (63)
|6 (55)
|6 (75)
|ECG, n (%)
|14 (74)
|9 (82)
|5 (63)
|QTc, ms, (median)
|587 ± 69 (563)
|587 ± 84 (559)
|586 ± 36 (565)
|≥550 ms, n (%)
|11 (79)
|6 (67)
|5 (100)
|β-Blocker therapy, n (%)
|15 (79)
|9 (82)
|6 (75)
|Age at therapy start, years (median)
|6.8 ± 13 (2)
|3.4 ± 4 (2)
|12 ± 20 (3)
|Total follow-up, years (median)
|30 ± 22 (30)
|27 ± 18 (30)
|34 ± 28 (25)
CE, cardiac event; ACA, aborted cardiac arrest or verified ventricular fibrillation/fast ventricular tachycardia; SCD, sudden cardiac death.
Cardiac symptoms in the JLNS cases were often interpreted as seizures by witnesses or medical personnel and six cases (four females) had undergone electroencephalography. In three cases (two females) there were reports of 'fever spells' and 'seizures' in infancy, possibly representing manifestations of cardiac events, indicating that symptoms debut could have occurred earlier than reported.
All symptomatic cases experienced recurrent syncopal episodes, ranging between a minimum of two reported cardiac events (Case 5) and over 30 cardiac events (Case 9). Ten out of 12 symptomatic cases (83%) with available data had experienced 10 or more cardiac events.
Cardiac events typically occurred more frequently in childhood in all cases (∼2–12 years of age, Figure 2). Adult females commonly continued to experience cardiac events in adulthood, including later periods of more frequent cardiac events, while in males the frequency of attacks abated in adulthood (Figure 2).
Experience of life-threatening cardiac events was common among the JLNS cases (10/19). Seven cases (three females) suffered an aborted cardiac arrest that required resuscitation (mean age 22 ± 27 years, median 6 years) and four cases (two females) suffered a probable fatal cardiac event (at age 5, 20, 27, and 37 years, respectively). Three of the sudden deaths occurred in relation to entering into, or swimming, in cold water.
If regarding verified fast ventricular tachycardia (260–500 beats per minute in Case 4) or ventricular fibrillation (Case 10) as a proxy for life-threatening cardiac events, 12/19 Swedish JLNS cases had experienced a life-threatening cardiac event (Table 4).
Triggers for cardiac events were typically physical exercise or emotional excitement (such as stress, fear, or anger), and in ∼10% of described events two or more concurrent triggers were present. A cold temperature, both regarding water when swimming and air-temperature during physical exercise, appeared to be a common trigger potentiator. Out of all described cardiac events (>100), the attributable fraction of each reported trigger was 47% for physical exercise, 35% for emotional excitement, 14% for swimming/cold water, 9% for fever/infections, 8% for unspecific, and 2% for a forgotten β-blocker dose.
Electrocardiograms from 14/19 cases (nine females) were available, whereof three asymptomatic cases (Table 3). Mean QTc was markedly prolonged in the JLNS cases at 587 ± 69 ms, median 563 ms, and range 461–735 ms, with no significant difference between the sexes. One case presented with a QTc < 500 ms and 11/14 cases had a QTc > 550 ms. QTc prolongation was significantly longer in symptomatic cases (mean 605 ± 62 ms, median 584 ms) vs. asymptomatic cases (mean 518 ± 50 ms, median 540 ms, P = 0.016).
Medical therapy and interventions
All now living and one of the deceased cases received prophylactic β-blocker therapy. Mean age at therapy start was 6.8 ± 13 years, median 2 years (Table 3). Type of β-blocker prescribed at therapy start was typically propranolol (80%) and to a lesser extent pindolol (13%) or alprenolol (7%). β-Blocker dosage was noted to increase over time, with lower doses being prescribed during the 70s and early 80s (i.e. propranolol <1 mg/kg per day). One case (Case 15) received the lower, then recommended, dose up until his fatal cardiac event at 5 years of age (0.6 mg/kg per day). Among the 11 cases on a propranolol dose ≥1 mg/kg per day 3 were as yet asymptomatic (27%) and 8 (73%) had experienced at least one cardiac event while on therapy. A total of six cases (55%) had suffered a maximum of one cardiac event on propranolol therapy at the time of the study (mean time on therapy 15 ± 14 years, median 7 years, sum 91 years). In adulthood four cases (Cases 7, 9, 10, and 13) were switched to metoprolol and all experienced recurrence of frequent cardiac events, including two aborted cardiac arrests (mean time on therapy 17 ± 6 years, median 17 years, sum 67 years).
Three cases (Cases 6, 9, and 10) were treated with left cardiac sympathetic denervation (LCSD) due to recurrent syncopal episodes in spite of β-blocker treatment. Mean time to symptom recurrence after the procedure (all cases) was ∼1 year, median 1 year. Recurrences occurred while on β-blocker therapy (propranolol ≥1 mg/kg per day) in two cases and after a gradual planned discontinuation of β-blocker therapy in one case (Case 10).
Six cases (all previously symptomatic) have received an implantable cardioverter-defibrillator (ICD). Indication for ICD implantation was, in the three previously mentioned cases with LCSD continuous recurrent syncopal episodes in spite of surgical intervention and β-blocker therapy, and in the other three cases either recurrent cardiac events in spite of β-blocker therapy (Case 4) or aborted cardiac arrests (Cases 7 and 13). Mean age at implantation was 22 ± 13 years, median 21 years, and range 6–39 years. Two cases suffered peri-operative or post-operative complications (pericarditis and heart tamponade). All cases with ICDs have received appropriate shocks after implantation, according to medical records (cardiologists' appraisal and/or after device interrogation). Two cases (Cases 4 and 7) received >25 shocks each during the first years after implantation. In Case 4, cardiac events were often noted to occur in the afternoon before administration of the evening dose, and frequency of shocks and syncopal events were significantly reduced after changing from a three per day to four doses per day β-blocker administration and an overall tightened compliance.
Phenotype in hearing first-degree relatives
Cardiac phenotype in first-degree relatives with normal hearing was largely asymptomatic, according to the interview data. Syncope was reported in 5/36 (14%) of heterozygous first-degree relatives (4/26 parents and 1/10 hearing siblings/children with ascertained genotype), and in none of the first-degree relatives with normal or uncertain genotype (n = 6). The symptomatic heterozygous cases were of p.M159sp (1/1), p.S349W (1/2), p.R518X (1/18), and c.572_576del (2/6) genotype (where the denominator is the total number of heterozygous first-degree relatives with available anamnestic information in the JLNS index families, for the specific allele).
Mean QTc in confirmed heterozygous first-degree relatives with available ECGs (n = 12, all of heterozygous p.R518X genotype) was 463 ± 19 ms, range 429–486 ms (P < 0.001 as compared with JLNS cases). A prolonged QT interval and other electrocardiographic aberrations were additionally reported in three cases (whereof two symptomatic) where an ECG was not available.
We have performed a national inventory and identified 13 JLNS families, including a total of 19 cases. Among children <10 years of age, five now living cases were identified, corresponding to a rough JLNS prevalence of at least 1 : 200 000 infants (annual birth rate ∼100 000), comparable with that reported in Norway.3 Importantly, as the study was not designed to identify deceased cases, JLNS prevalence might be underestimated.
Mutation spectrum and founder effects
The JLNS mutation spectrum in Sweden consisted solely of KCNQ1 mutations (n = 8). The majority (20/24) of the identified alleles were frame-shift, splice, or nonsense mutations, in accordance with the concept that JLNS is more commonly caused by mutations that in the heterozygous form cause haploinsufficiency.20 The mutation spectrum was dominated by the p.R518X nonsense mutation, a C to T transition (c.1552C > T) leading to an amino acid exchange of the residue Arginine-518 (R) for a premature stop codon (X), resulting in a C-terminal truncation and a non-functioning protein. Worldwide, p.R518X is a commonly occurring hot spot mutation, associated with both LQTS and JLNS.3,20–23 In Sweden and Norway, defined by geographical proximity and the historical union between the two kingdoms in 1814–1905, the geographic clustering of JLNS probands according to genotype (p.R518X, c.572_576del and p.Q530X)3 and the here revealed similarities between the mutation spectra is suggestive of a Scandinavian founder effect.
While the presence of strong founder effects and the mild cardiac phenotype seen specifically in first-degree p.R518X relatives probably have contributed to the relatively high prevalence of JLNS in Sweden, it surprised us that the Y111C founder mutation, identified in over 170 heterozygous mutation-carriers and with a documented high regional prevalence, was found in only one homozygous proband and in none of the compound heterozygotes.11 It has been reported that a mere 10% residual KCNQ1 function rescues hearing in homozygous mutation-carriers,24 and it could be seen as a limitation that the national inventory was not designed to detect such cases. Furthermore, a Swedish family in which compound heterozygosity for p.R518X and p.A525T resulted in a severe cardiac phenotype without hearing loss has been described,16 suggesting that the national prevalence of cases with a genotype predisposing for a severe cardiac phenotype might be well over the presented JLNS prevalence estimate.
However, during our research we have not identified any additional homozygous or compound heterozygous Y111C mutation-carriers, irrespective of hearing loss. An alternative hypothesis that we are considering is that homozygous Y111C pregnancies are somehow selected against.
Cardiac phenotype and therapy efficacy
The severity of the JLNS phenotype is well documented; however, the differences in cardiac phenotype between the sexes seen over time need to be further elucidated.2,25
Knowledge regarding clinical outcome with respect to different medical interventions is limited and methodological differences over time (i.e. β-blocker type and dosage, techniques regarding LCSD procedures, etc.) complicate comparisons.
In this study, β-blocker therapy was associated with reduction of event frequency and in three cases asymptomatic phenotype, but was not successful in completely abolishing events in any previously symptomatic individual. However, inadequate dosage and possibly type of β-blocker used may at least partly explain the limited efficacy seen in this study. A more favorable outcome for propranolol as compared with metoprolol was suggested. Concerns regarding differences in efficacy between types of β-blockers in heterozygous LQTS patients have been reported.26,27
There is furthermore reason to suspect that therapy non-compliance played a more prominent role in triggering cardiac events than what was reported in this study, as has been shown for β-blocker treatment 'failures' in heterozygous LQT1 mutation-carriers.28 Not surprisingly, a positive effect on symptoms frequency via stringency regarding the timing of medication and avoidance of triggers was reported by many cases during the interviews.
Regarding LCSD, performed on only three of the Swedish cases, no convincing preventive effect was seen in this retrospective study, although this could be related to the surgical techniques used.
Among the cases treated with an ICD there was no case of sudden death, emphasizing the previously seen efficacy of the ICD in prevention of sudden cardiac death in JLNS.25 On the other hand, two out of six cases with an ICD suffered from frequent multiple discharges during the first years after implantation, two cases suffered from peri-/post-operative complications and, as is well known, device deployment in small children is associated with significant complications.
Based on our compiled data, we would, as initial therapy from birth and onwards, recommend β-blockers (propranolol 2–4 mg/kg per day, administrated 3–4 times per day) with utmost stress on patient compliance. Due to the rapid growth of children, these patients need close monitoring and dose adjustments. We recommend that the effect of the given therapy (and compliance) should be regularly evaluated by ambulatory 24 h electrocardiography, and dosage increased if appropriate, ensuring an adequate β-blockade over day and night. Furthermore, the level of β-blockade during physical exertion should be evaluated by exercise test when the child is old/tall enough to comply. In cases of true therapy failure, LCSD and/or ICD implantation must be considered. Further studies regarding efficacy of medical and surgical interventions in this high-risk group is needed.
In conclusion, a JLNS prevalence of at least 1 : 200 000 in Swedish children <10 years of age was revealed. Scandinavian founder effects regarding four mutations (p.R518X, p.Q530X, c.572_575del, and p.Y111C) could explain as much as 83% of JLNS in Sweden and a high prevalence of heterozygous mutation-carriers was suggested.
This study was supported by grants from the Swedish Heart-Lung Foundation, the Northern County Councils Cooperation Committee and the faculty of Medicine, Umeå University.
Firstly, we would like to thank all the Swedish JLNS families and all clinicians who contributed to the national inventory, for making this study possible. Secondly, we acknowledge the substantial contribution of our colleague, former paediatric cardiologist Hans Wendel (Pediatric Cardiology Clinic, Umeå University Hospital, 1967–1991), who diagnosed the first JLNS case in the northern region in the early 70s, and whose legacy of clinical expertise and research spirit continues to inspire. Original artwork and figures were provided by illustrator Erik Winbo (erikwinbo.artworkfolio.com).
Conflict of interest: none declared.
↵† Present address. Cardiovascular Genetics, Leon H. Charney Division of Cardiology, New York University School of Medicine, New York, NY, USA.
- Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2012. For permissions please email: email@example.com.


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Grain Milling Table
I am helping a neighbor build a milling table from 8/4 hickory. This table is for milling grain to brew beer. I will keep posting images as we progress.
Grain Milling Table
07-28-2013 02:24 PM by Brian Lackey | 1 comment »
I am helping a neighbor build a milling table from 8/4 hickory. This table is for milling grain to brew beer. I will keep posting images as we progress.
Tool thoughts #42: Rediscovered a Lumber Source in CNY (central NY)
02-17-2013 12:25 AM by NedB | 3 comments »
I just made a happy re-discovery of a 'local' sawyer whom I've dealt with in the past. For those in the central NY area, chime in here if you're looking for some decent hardwoods, Zach is the sawyer, and he has maple, ash, elm and hickory in 4/4 up through 10/4 at $1/bf. It may need some seasoning, but hey, that's what lumber racks are for, right? I'm planning on making a run up his way in about a month, month and a half from now, just about the time that m...
My Journey As A Creative Designer - Woodworking and Beyond #941: Moving Right Along . . .
01-23-2013 12:36 PM by Sheila Landry (scrollgirl) | 8 comments »
I am happy to say that yesterday turned out to be a very productive day. While Kieth and I both needed scroll saw time, I had plenty to do while he was cutting to keep me busy. I spent the morning working on the final touches for the article that I wrote on different methods to apply the pattern to the wood for scroll sawing (which will be featured in the May issue of Creative Woodworks and Crafts magazine) and picked my wood piece for the set of masks that I was scrolling out. I chose a ...
Krokskaft....or, how Mjolnir died.
11-16-2012 10:02 PM by RaggedKerf | 0 comments »
For the pictures, please click here. Ah, it's good to be back in the shop. Took a break there for a while to go on a trip to the Bahamas with the family. We had a blast but the only woodworking I did was reading about it and sawing some "Z"s by the pool. To get back in the swing of things (projects and ideas are starting to pile up) I decided to knock out something that's been on the list since the beginning: the mallet. Poor, sad, Mjolnir, assembled crooked...
Mjolnir (part 3), or, A lesson in sticking with the plan
10-26-2012 12:19 PM by RaggedKerf | 2 comments »
For all the evidence—-I mean pictures——please click here. As the title says, things didn't go exactly as planned with the hammer the past couple days. I tried my best but it just didn't work out as I imagined. Here's what happened. Best pull up a chair and get some popcorn. It started after the handle was finally cut free from the excess wood. I decided to put all the pieces together and see if I like the general shape and weight of the mallet. H...
Mjolnir (part 2)
10-24-2012 01:33 AM by RaggedKerf | 0 comments »
To see the pictures, please click here. After finishing up the leg vise yesterday, I had a little bit of time leftover and decided to get cracking on Mjolnir (yeah, it thinks a lot of itself!). I finalized the design and drew it on the handle. It has the typical laminated mallet shape, tapered inside the head to lock the handle in place. I decided ti add a little detail to the handle and a pommel. Totally not necessary but I'm having fun now. I started to cut the handle free...
Mjolnir (part 1)
10-21-2012 12:39 PM by RaggedKerf | 2 comments »
To see the pictures, please click here. At long last I have begun construction of my first mallet! I've been seeing all these great pounders, thumpers, whackers, and smackers that everyone has been displaying on the internet and on TV shows and finally could take no more of the bounce-back prone rubber mallet I've been using. As it happens, when I bought the supplies to build the bench back in August, I also acquired a nice hickory plank (4"x3/4" x 4') to ...
Exercises in Artisanship #25: Practices in Spoonology: Part four
09-10-2012 10:32 PM by jjw5858 | 7 comments »
So my business of country spoon sloyd craft carries on. Forgive me if my tales on this exploration have become boring, I realize there is not a lot of joinery going on. I am closing in as I enter the words here for session 4 of 5 of this spoon blog and have really made some nice strides. My greatest findings have been using the soil I live on to adventure more deeply into the woods and come out with new species I have never worked with before such as Hickory, Black Walnut, and Black Locust...
Practices in Spoonology: Part three
09-04-2012 11:36 PM by jjw5858 | 6 comments »
Well I thought it might be best this evening to get to the shop and keep up with the spoon practicing and see where are am at with things. I felt good and energized after a fine meal of venison burger, potatoes and greens. I have to say that deer meat is a terrific source of rocket fuel and lucky for me I have no better source than my uncle Topper who lives in the cold hills of the upstate NY. When it comes to some well hunted eats there's no better of a choice than Toppers freezer for some d...
Bottle Holder progress
03-23-2012 05:49 PM by Chuck | 1 comment »
Last weekend I got 2 coats of linseed oil applied to the bottle holder and last night I got 3 coats of shellac applied. Tomorrow I will give it a light sanding and then 2 more coats of shellac. I like this picture because it shows how much color and warmth the oil added to the hickory.
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Definition of skied
Example sentences containing 'skied'At one point he shot across the slope so fast he skied off the piste and part-way up a tree. AND GOD CREATED THE AU PAIRThey climbed up mountains and skied down them, sometimes stopping on the way to make love. IN REAL LIFESince I last skied 20 years ago and Michael even longer, we will no doubt both prove to be a great embarrassment to them all. AND GOD CREATED THE AU PAIR
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Former Alabama offensive tackle D.J. Fluker will likely go in the first round of the NFL draft on Thursday. And since this is draft week, one would think that Fluker would be lying low to be sure that he doesn't do anything stupid that could affect his draft stock at the last minute.
Former Alabama lineman D.J. Fluker tweets he took money in college, then promptly deletes tweet
Well paid Bob Stoops thinks unpaid college athletes are 'paid quite handsomely'
Bob Stoops or "Big game Bob" if you're from the state of Texas, was asked his opinion on whether or not college athletes should be paid. It must be stated that Stoops takes home an annual salary of nearly $5 million for coaching players that take home an annual salary of $0. But Stoops is […]
Tim Pernetti's parting gift? A year's salary, a $679,500 one-time payment, a car allowance, health insurance, an iPad, and a laptop
As Rutgers cleans house, they're also giving out fabulous parting gifts to the departed. For instance, former Rutgers coach Mike Rice got a fat bonus in the amount of $100,000 that they wouldn't have had to give him if they had fired him the moment they saw that crazy video.
Brutus Buckeye took a snap in Ohio State's spring practice, gets leveled for his trouble
During Ohio State's spring practice on Saturday, Brutus Buckeye made an appearance not on the sideline or in the stands, but on the field. At some point in the scrimmage, Ohio State's mascot lined up in the shotgun, took a snap, and began to scrambling. What happened next was unexpected to say the least.
Apparently, Mississippi State held a football formal
It's March, so that means spring football is in full effect. But in Starkeville, Mississippi, there's more than just spring football. There's also apparently a football formal. And what you'll see below is video of Mississippi State players dressed all nice accompanied by dates for a night of dining and dancing.
Maryland doesn't have a copy of their Big Ten contract
And they also don't have a copy of the ACC's current television contract. But there's no reason to be alarmed according to Maryland's assistant vice president for marketing and communications Brian Ullmann because it's "typical" for schools not to have a copy of a contract they've signed with a conference. [Washington Post]
South Carolina is using Jadeveon Clowney almost killing that Michigan running back as the cover of their spring guide
South Carolina's best player is Jadeveon Clowney. The best moment from South Carolina's season was Clowney almost killing Michigan running back Vincent Smith in the Outback Bowl. So it only makes sense that South Carolina is using its best player and the best moment as the cover for their spring guide.
Spring practice has begun at South Carolina and Steve Spurrier is shirtless
It's a yearly ritual. Steve Spurrier, shirtless at South Carolina spring practice. So it shouldn't surprise you that Spurrier was once again photographed at spring practice shirtless. [Reddit]
Former NC State coach Tom O'Brien used the UVA equipment truck to move his belongings from Raleigh condo
Back in November, NC State head coach Tom O'Brien was fired seemingly out of the blue. Then a couple of months later, O'Brien landed a job on Mike London's staff at Virginia. We never really got the opportunity to hear publicly how O'Brien took his firing, but one could guess that it wasn't well. Mainly […]
ESPN went behind the scenes with Johnny Manziel and his Mercedes-Benz
ESPN did an All-Access segment on Heisman Trophy winner Johnny Manziel. The first 2:20 of the 2:33 profile follows Manziel during the first day of spring practice. And just in case the fact that Manziel is taking all online classes isn't enough to prove that Manziel is living the life in College Station, ESPN uses […]


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Lake Oswego School District may shift bond measure to May
School board worries that crowded November ballot and a time crunch could put proposal at risk
Faced with a crowded November ballot, the Lake Oswego School Board now appears likely to wait until the May 2017 election before placing a proposed bond measure before voters.
Board members discussed the option during a two-day retreat last week, where no formal decisions were made. But given the number of issues facing voters in November including presidential and gubernatorial elections, a local mayoral race, three City Council contests and a major corporate tax measure there was general consensus that delaying the bond makes the most sense.
Our schools shouldnt be caught up in the craziness of national elections, board member Bob Barman told his colleagues, and I think it would be very difficult to get our message out when were lost in all the commercials.
Board members also raised concerns about whether they would have enough time to make sure the community understands the impact of years of deferred maintenance on the districts elementary schools and junior highs. A final list of projects is not expected to be chosen until mid-August for a bond that would have appeared before voters on Nov. 8.
School board member Liz Hartman said its possible that local volunteers could raise awareness to some extent before the fall election, but she said she was not sure if a November date provided enough time for an outreach campaign to fully educate the public about the facilities poor condition.
Hartman said the chances for success if the bond is presented to voters in November are also lower than in May, according to survey data.
I think every statistic weve looked at and every piece of advice weve gotten is, Dont take that risk, she said.
A final decision by the board is expected in August.
The voters
During the July 12-13 retreat, board members listened to an analysis of voting patterns and the details of a phone survey conducted in March and April that gauged voter tolerance for a bond measure.
Melissa Martin, survey research director for The Nelson Report, said the percentage of school bonds that passed in Oregon from 1997 to 2015 differed greatly not only between November and May, but also between odd and even years.
In all years, an average of 45 percent of bonds were approved by voters in November elections, Martin said. In even years, it was 47 percent; in odd years, it was 38 percent.
For elections held in May, an average of 52 percent of bonds were approved. In even years, it was 46 percent; in odd years as it will be next spring the turnout was the highest at 61 percent, Martin said.
The data show we have better odds of winning a May election, board chairwoman Sarah Howell said after hearing those statistics, and its important to set (the bond) up for success.
Martin said the phone survey, which polled 380 people and had a margin of error of plus or minus 5 percent, revealed that local voters are mostly older and that many of them are unaware of the condition of district buildings.
Seventy percent of registered voters in the district are age 45 or older, Martin said. Of those who voted in four elections analyzed by The Nelson Report (May 2012, November 2012, May 2014 and November 2014), 94 percent were 45 or older, and 75 percent of that group was 60 or older.
The survey asked whether respondents would support a $250 million bond measure that would protect the communitys large investment in school buildings by making repairs and improvements districtwide, such as replacing roofs, flooring, siding and masonry. Of those who responded to that question, 80 percent said they would support the measure, 17 percent said they would not and 3 percent werent sure.
When the districts pool was added to the list of potential projects, support for a bond measure dropped: 54 percent said they were in favor, 34 percent were opposed and 12 percent werent sure. When covered seating and a press box for the Lakeridge High stadium was added, support dropped even further: 29 percent in favor, 61 percent opposed and 10 percent who werent sure.
Martin said thats no surprise, because athletic facilities have a small core group of strong advocates. The quiet majority generally isnt as supportive, she said.
Theyre not supportive of a stadium for Lakeridge, and the pool is a pretty big wild card right now, Martin said. I cant tell you what to put or not put into your bond, but I will tell you that its a calculated risk. If you put the pool in, you may be risking the rest of the project.
Martin said the survey also revealed that there is a widespread lack of understanding about the condition of the districts schools. About 50 percent of those surveyed rated the physical condition of the buildings as either excellent (12 percent) or pretty good (38 percent). That's in stark contrast to a series of studies conducted as part of the bond process.
In a Facilities Condition Assessment prepared for the district in 2015, Lake Oswego High was rated in good condition, the best rating possible, and Lakeridge High was ranked as fair, the next best rating. (LOHS was rebuilt in 2005 and Lakeridge was fully remodeled in 2004, using funds from an $85 million bond voters approved in November 2000.)
But the district's five elementary schools were ranked as poor, and the sixth is in critical condition as a result of design flaws, according to the report. And both junior highs are in poor condition but considered close to critical, which is the worst possible ranking.
Because there are so many improvements to be made, the board long has discussed proposing an evergreen bond that could be renewed within a few years. Board member John Wendland has also suggested a phased approach, with one set of bond projects done in two parts over an extended period of time.
The list of projects is too large to accomplish in one bond, Wendland said in a written presentation he prepared for fellow board members. The ultimate bond amount still needs to be determined, as well as timing. We cant fix everything all at once, but we can plan for phasing projects over time.
The board looked at the impact on voters of three possible timelines, all assuming a $250 million bond package: 20 years at $1.95 per $1,000 of assessed property value, 25 years at $1.50 per $1,000 APV and 30 years at $1.40 per $1,000 APV. (Taxes are levied against assessed value, not the generally 30-percent higher real market value.)
No scenario was selected, but Martin told the board that $1.50 per $1,000 is generally a well-received amount although Lake Oswego residents may be more willing to support a higher amount than a typical city, Howell said.
The projects
Regardless of the financing timeline or eventual election date, the board still needs to pick the projects it will include in a bond measure. The districts Bond Development Committee offered two possible plans:
Plan A, which would cost $297 million, would involve projects such as replacing each junior high with a two-story, 150,000-square-foot school with the capacity for 1,100 students.
Plan B, with a price tag of $245 million, would replace only Lakeridge Junior High and upgrade Lake Oswego Junior Highs gym to make it structurally strong enough to serve as a post-disaster evacuation shelter. Plan B would also include adding maker spaces, which are areas that offer opportunities for creative enterprises, and improving facilities for science, technology, engineering, art and math (STEAM).
My hope for this bond is that all of our buildings will be safe, warm and dry, and that we will have two new junior high schools, Howell said. I like Plan A, but I think we may be asking too much.
During a hands-on exercise designed to narrow the list of projects, board members generally agreed that technology, security and safety upgrades were good for the bond. Improving the gym at Lake Oswego Junior High for $3.81 million rather than constructing a replacement school was the least popular idea. An $85.25 million project to rebuild Lakeridge Junior High received more support under Plan A than under Plan B an indication that the board would prefer to replace both schools if its financially possible.
Im not as interested in building a new gymnasium, Howell said, as I am in building a new junior high school.
Barman suggested that creating cookie cutter junior highs could reduce costs. But Randy Miller, the districts executive director of project management, said that idea may not reduce the price as much as one might hope. The two sites differ, he said, and that would affect how each structure is designed.
In any case, Superintendent Heather Beck said, the current cost estimates may be the best anyone will see.
These numbers are not something thats going to get less expensive in the future, she said. Overall, the bigger buildings do not become less expensive as time passes.
Safety, security and technology upgrades ($16.18 million all together) were very popular, regardless of whether they were in Plan A or Plan B. Howell said that its important for there to be something for everyone in this bond, and Miller agreed.
In terms of something for everyone, he said, I think the one thing we can look at is the tech improvement and the safety and security improvements. We can get on those as soon as the bond is passed.
STEAM facilities, listed under Plan B at $7 million, were widely embraced by the board, while support was mixed for creative areas or maker spaces. And there was a lot of debate about capital improvement maintenance, which fell under three categories:
Level 1 ($81.32 million), which includes critical seismic upgrades, emergency notification/evacuation, hazardous materials and critical electrical loads;
Level 2 ($5.32 million), which includes integrity/adequacy of systems and non-critical seismic upgrades; and
Level 3 ($400,700), which includes access issues, aesthetics and end-of-life-cycle replacement for items such as outdated boilers.
"There are some items in the capital improvement (category) that could come out because of some things that could possibly be delayed," said John Wallin, a board member.
Board members debated whether all seismic upgrades were crucial, and said they wanted to see if each level could be further itemized.
We asked for the maintenance budgets to be broken down a little bit, Wendland said, for what could be delayed for a while and what could be put toward projects we need to do now.
Plans A and B both include about $18.94 million to cover a host of miscellaneous costs, including funding personnel, moving students between schools during construction, issuing bonds and adding furniture and fixtures. Those are all packaged as owner bond management costs, although Hartman suggested that it might be wise to use words that make it easier for voters to understand how important the services are.
Well work on the language, Beck said.
As part of the bond process, district officials and board members have attended public meetings of groups such as School Advisory Committees and neighborhood associations, gathered public input at other meetings, conducted The Nelson Report phone survey and commissioned an online forum.
For the past two years, the school district has been assessing the condition of its buildings. A study done last year found at least $98 million in seismic upgrades and deferred maintenance at all school buildings, not counting soft costs such as personnel and design. Subsequent studies have revealed further maintenance needs and higher costs.
The volunteer Bond Development Committee met from February to May to determine which district buildings to focus on. They first narrowed the list to the 10 operating schools and then recommended that most of the work be done at the junior highs and elementary schools.
The next step is for the board to finalize a project list for the bond; that decision is likely by mid-August. And then the task will be to develop a campaign to raise awareness in the community.
Stick with the messaging and the wording, Martin told the board. If you are all consistent, the chances of success grow.
By Jillian Daley
503-636-1281, ext. 109
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This is a football cake done in all buttercream. I was asked to do something for UNC Tarheels , and this is what I was able to come up with on short notice. It's done all in buttercream except for the football picks.
Very cool design...Looks great!!!
Love it! GO HEELS!!!!!
CakeCentral.com is the world's largest cake community for cake decorating professionals and enthusiasts.
(if this is a private computer)
Yes please, register now!


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Bristol Motor Speedway officials drummed up a brilliant concept this past fall, one used often in other sports to reveal a bit of a team's personality and the personalities of its players: Let each athlete choose a song, and blare it on the loudspeaker when he's introduced.
AP Photo/Mark J. TerrillIf country music star Blake Shelton could pick Kasey Kahne's driver intro song at Bristol, it'd be Aerosmith's "Dude (Looks Like A Lady)."
It hadn't been done in NASCAR before. It should have been. It was genius. Just before the race, all 43 drivers in the field walked across the track in Turn 3 and emerged from a makeshift tunnel to the song of their choice to the glee of 160,000 half-cocked and happy, screaming race fans.
Throughout the past week, many drivers have solicited help from fans via Twitter to pick a song for the March 21 event at BMS. I thought I'd assist.
To do so, I hit up my man Blake Shelton, he of the "Hillbilly Bone" ilk and general hilarity. Dude's funny. He also follows NASCAR pretty dang close. We were discussing the diminutive stature many drivers have, and he said, "Hmmm. That explains it. No wonder Elliott [Sadler] sucks."
He was kidding, of course. Sadler is one of his best buddies.
Here's his list. I'm still laughing.
Kurt Busch -- "Now Kurt's the oldest brother, right? I don't know why, but the theme song to the 'Brady Bunch' comes to mind. Is that weird?"
Mark Martin -- "Easy one. 'Grandpa,' by the Judds."
Robby Gordon -- "'Some Beach,' by me because he wrecked me at Atlanta last weekend. I at least thought I could miss the first few laps and still see the car. Nope. At least he got me on TV."
Kasey Kahne -- "Oh, man. He's pretty. He's the prettiest man I've ever seen. 'Dude (Looks Like A Lady),' by Aerosmith. Great driver. And he's really pretty. Really pretty."
Denny Hamlin -- "Didn't he say something about his car recently, like his motor was bad or something? 'Piece Of S--- Car.' Adam Sandler."
Brad Keselowski -- "Is that guy OK? I thought Carl Edwards was the one that did the flipping. 'Ain't That A Kick In The Head,' by Dean Martin."
Tony Stewart -- "'My Way,' by Frank Sinatra. He bought his own team, and he's doin' it."
Matt Kenseth -- "From what I hear, he's funny, but people think he's boring as hell. 'Personality,' by Lloyd Price."
Kyle Busch -- "'We're Not Gonna Take It,' by Twisted Sister. Because he just gets so pissed and doesn't do anything wrong ever. Ever."
Elliott Sadler -- "'Ol' Red.' He's in the video with me, and played the part of my inbred cousin. He was a natural."
Joey Logano -- "We'll go with another one of mine here: 'The Baby.' Does he shave yet?"
Jeff Gordon -- "'I Kissed A Girl,' by Katy Perry. I don't really know what made me think of that song. But I did. So there you go. Enough said."
Kevin Harvick -- "'Loser,' by Beck. Wait. He's leading the points, isn't he? We'll keep it just for the irony."
Jeff Burton -- "'Love Will Keep Us Together,' by Captain and Tennille. Y'all call him the Mayor, don't ya?"
Clint Bowyer -- "'Hillbilly Bone.' He does a race at his house every year called the Hillbilly 250. It works."
Jimmie Johnson -- "An old Mac Davis song works for him, called '[Oh] Lord It's Hard To Be Humble' when you're perfect in every way."
Scott Speed -- "'Karma Chameleon,' by Culture Club. We'll leave it at that."
Brian Vickers -- "'Wanted Dead Or Alive,' by Bon Jovi. That's true, right?"
Dale Earnhardt Jr. -- "'The More I Drink, The More I Drink.' This is one of mine. He's having that kind of luck."
Carl Edwards -- "'I'm Too Sexy' for my shirt, by Right Said Fred. Enough said."