Morning View From the 24th Floor at 261 Madison

 

Target Health has been on the 23rd and 24 Floors at 261 Madison since 2001. There are a lot of breath-taking views at sunset which we have shared for many years, but this morning view of shadows, the spectacular sky and eclectic architecture, has left us breathless.

View From the 24th Floor.  ©Target Health Inc.

 

For more information about Target Health contact Warren Pearlson (212-681-2100 ext. 165). For additional information about software tools for paperless clinical trials, please also feel free to contact Dr. Jules T. Mitchel or Ms. Joyce Hays. The Target Health software tools are designed to partner with both CROs and Sponsors. Please visit the Target Health Website.

 

Joyce Hays, Founder and Editor in Chief of On Target

Jules Mitchel, Editor

QUIZ

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Cholangiocarcinoma

Digestive system diagram showing bile duct location. Credit: Public Domain, Wikimedia Commons

 

Life cycle of Clonorchis sinensis, a liver fluke associated with cholangiocarcinoma

Credit: cdc.gov; Public Domain, Wikimedia Commons

 

 

Cholangiocarcinoma or bile duct cancer is a form of cancer that is composed of mutated epithelial cells (or cells showing characteristics of epithelial differentiation) that originate in the 1) ___ ducts which drain bile from the liver into the small intestine. Other biliary tract cancers include gallbladder cancer and cancer of the ampulla of Vater. Cancerous tumors of the bile ducts are usually slow-growing and do not spread (metastasize) quickly. However, many of these tumors are already advanced by the time they are found. A cholangiocarcinoma may start anywhere along the bile ducts. These 2) ___ block off the bile ducts. Prominent signs and symptoms of cholangiocarcinoma include abnormal liver function tests, abdominal pain, jaundice, and weight loss. Other symptoms such as generalized itching, fever, and changes in color of stool or urine may also occur.

 

The disease is diagnosed through a combination of 3) ___ tests, imaging, endoscopy, and sometimes surgical exploration, with confirmation obtained after a pathologist examines cells from the tumor under a microscope. Known risk factors for cholangiocarcinoma include primary sclerosing cholangitis (an inflammatory disease of the bile ducts), infection with the parasitic liver flukes Opisthorchis viverrini or Clonorchis sinensis, some congenital liver malformations, and exposure to Thorotrast (thorium dioxide), a chemical formerly used in medical imaging. However, most people with cholangiocarcinoma have no identifiable 4) ___ factors. Cholangiocarcinoma is considered to be an incurable and rapidly lethal cancer unless both the primary tumor and any metastases can be fully removed by surgery. No potentially curative treatment exists except 5) ___, but most people have advanced stage disease at presentation and are inoperable at the time of diagnosis. People with cholangiocarcinoma are generally managed – though not cured – with chemotherapy, radiation therapy, and other palliative care measures. These are also used as additional therapies after surgery in cases where resection has apparently been successful (or nearly so). The most common physical indications of cholangiocarcinoma are abnormal liver function tests, jaundice (yellowing of the eyes and skin occurring when bile ducts are blocked by tumor), abdominal pain (30%-50%), generalized itching (66%), weight loss (30%-50%), fever (up to 20%), and changes in stool or urine color. To some extent, the symptoms depend upon the location of the tumor: patients with cholangiocarcinoma in the extrahepatic bile ducts (outside the liver) are more likely to have jaundice, while those with tumors of the bile ducts within the liver more often have pain without jaundice.

 

Although most patients present without any known risk factors evident, a number of risk factors for the development of cholangiocarcinoma have been described. In the Western world, the most common of these is primary sclerosing cholangitis (PSC), an inflammatory disease of the bile ducts which is closely associated with ulcerative 6) ___ (UC). Certain parasitic liver diseases may be risk factors as well. Colonization with the liver flukes Opisthorchis viverrini (found in Thailand, Laos PDR, and Vietnam) or Clonorchis sinensis (found in China, Taiwan, eastern Russia, Korea, and Vietnam) has been associated with the development of cholangiocarcinoma. Patients with chronic liver disease, whether in the form of viral hepatitis (e.g. hepatitis B or hepatitis C), alcoholic liver disease, or cirrhosis of the liver due to other causes, are at significantly increased risk of cholangiocarcinoma. HIV infection was also identified in one study as a potential risk factor for cholangiocarcinoma, although it was unclear whether HIV itself or other correlated and confounding factors (e.g. hepatitis C infection) were responsible for the association. Infection with the bacteria Helicobacter bilis and Helicobacter hepaticus species can cause biliary cancer.

 

While the presence of gallstones (cholelithiasis) is not clearly associated with cholangiocarcinoma, intrahepatic stones (called hepatolithiasis), which are rare in the West but common in parts of Asia, have been strongly associated with cholangiocarcinoma. Exposure to Thorotrast, a form of thorium dioxide which was used as a radiologic contrast medium, has been linked to the development of cholangiocarcinoma as late as 30-40 years after exposure; Thorotrast was banned in the United States in the 7) ___ due to its carcinogenicity. Although cholangiocarcinoma is known to have the histological and molecular features of an adenocarcinoma of epithelial cells lining the biliary tract, the actual cell of origin is unknown.

 

Recent evidence has suggested that the initial transformed cell that generates the primary tumor may arise from a pluripotent hepatic stem cell. Cholangiocarcinoma is thought to develop through a series of stages, in a process similar to that seen in the development of colon cancer. Chronic inflammation and obstruction of the bile ducts, and the resulting impaired bile flow, are thought to play a role in this progression. Cholangiocarcinoma is definitively diagnosed from tissue, i.e. it is proven by biopsy or examination of the tissue excised at surgery. It may be suspected in a patient with obstructive jaundice. There are no specific blood tests that can diagnose cholangiocarcinoma by themselves. However, they may be useful in conjunction with imaging methods in supporting a suspected diagnosis of cholangiocarcinoma. Ultrasound of the 8) ___ and biliary tree is often used as the initial imaging modality in patients with suspected obstructive jaundice. Ultrasound can identify obstruction and ductal dilatation and, in some cases, may be sufficient to diagnose cholangiocarcinoma. Computed tomography (CT) scanning may also play an important role in the diagnosis of cholangiocarcinoma. Surgical exploration may be necessary to obtain a suitable biopsy and to accurately stage a patient with cholangiocarcinoma. Laparoscopy can be used for staging purposes and may avoid the need for a more invasive surgical procedure, such as laparotomy, in some patients. Surgery is also the only curative option for cholangiocarcinoma, although it is limited to patients with early-stage disease. Cytological scrapings are often non-diagnostic, as these tumors typically have a desmoplastic stroma and, therefore, do not release diagnostic tumor cells with scrapings.

 

Although there are at least three staging systems for cholangiocarcinoma none have been shown to be useful in predicting survival. The most important staging issue is whether the tumor can be surgically removed, or whether it is too advanced for surgical treatment to be successful. Often, this determination can only be made at the time of surgery. Cholangiocarcinoma is considered to be an incurable and rapidly lethal disease unless all the tumors can be fully resected (that is, cut out surgically). Since the operability of the tumor can only be assessed during surgery in most cases, a majority of patients undergo exploratory surgery unless there is already a clear indication that the tumor is inoperable. However, the Mayo Clinic has reported significant success treating early bile duct cancer with liver transplantation using a protocolized approach and strict selection criteria. If the tumor can be removed surgically, patients may receive adjuvant chemotherapy or 9) ___ therapy after the operation to improve the chances of cure. If the tissue margins are negative (i.e. the tumor has been totally excised), adjuvant therapy is of uncertain benefit. However, if the tumor tissue margins are positive, indicating that the tumor was not completely removed via surgery, then adjuvant therapy with radiation and possibly chemotherapy is generally recommended based on the available data.

 

Multiple studies have documented a steady increase in the incidence of intrahepatic cholangiocarcinoma over the past several decades; increases have been seen in North America, Europe, Asia, and Australia. The reasons for the increasing occurrence of cholangiocarcinoma are unclear; improved diagnostic methods may be partially responsible, but the prevalence of potential risk factors for cholangiocarcinoma, such as HIV infection, has also been increasing during this time frame. One can ease the stress of illness by joining a support group with members who share common experiences and problems. 10) ___ is often a good resource for patients with cholangiocarcinoma that cannot be cured.

 

Click on the following link – Patient’s Cells Deployed to Attack Aggressive Cancerto read an interesting story in the NYTimes, published this past week.

 

ANSWERS: 1) bile; 2) tumors; 3) blood; 4) risk; 5) surgery; 6) colitis; 7) 1950s; 8) liver; 9) radiation; 10) Hospice

William Bradley Coley MD – Pioneer of Immunotherapy

William Bradley Coley (center)

 

William Bradley Coley (1862 – 1936) was an American bone surgeon and cancer researcher and the pioneer of cancer immunotherapy. He developed a treatment based on provoking an immune response to bacteria. In 1968 a protein related to his work was identified and called tumor necrosis factor-alpha.

 

William Coley was born to a very old Connecticut family, on January 12, 1862 in Westfield, to Horace Bradley Coley and Clarina B. Wakeman. He went to college at Yale and graduated from Harvard Medical School in 1888. He then joined the staff of the New York Hospital as an intern on the surgical service, following which he began his career as a bone surgeon at New York Cancer Hospital (which later became part of the Memorial Sloan-Kettering Cancer Center). Coley became more interested in cancer treatment when one of his early patients, Elizabeth Dashiell, died from bone cancer. While going through hospital records, Coley found a sarcoma case study of one patient named Fred Stein, whose tumor disappeared following a high fever from erysipelas infection, now known as Streptococcus pyogenes. This sparked Coley’s interest and drove him to find what few examples of similar cancer treatment had been previously recorded. He discovered that other medical pioneers including Robert Koch, Louis Pasteur, and Emil von Behring, had recorded observations of erysipelas infection coinciding with cancer regression.

 

From 1925 to 1933, Coley served as Surgeon-in-Chief of the Hospital for Special Surgery in New York City, where he developed the theory that post-surgical infections had helped patients to recover better from their cancer by provoking an immune response. In 1891 he began to experiment by deliberately causing this phenomenon, injecting streptococcus bacteria directly into people being treated; later because this had the adverse effect of causing infection, he switched to using dead bacteria. Coley published the results of his work as a case series, making it difficult to interpret them with confidence. According to the American Cancer Society, “More research would be needed to determine what benefit, if any, this therapy might have for people with cancer“. Cancer Research UK say that “available scientific evidence does not currently support claims that Coley’s toxins can treat or prevent cancer“. People with cancer who take Coley’s toxins alongside conventional cancer treatments, or who use it as a substitute for those treatments, risk seriously harming their health. By 1901, the development of x-rays as a cancer treatment showed great promise. In particular, the therapy resulted in immediate tumor destruction and pain relief. Although Coley claimed successful treatment of hundreds of patients, the absence of proven benefit or reproducibility led to broader emphasis on surgery and on the newly developing field of radiation therapy. This decision was borne out by the eventual successful treatment of millions of people worldwide with radiation therapy. Coley arranged for a wealthy friend to provide funds to purchase two x-ray machines for his use. However, after several years of experience, Coley came to the conclusion that the effect of that primitive x-ray therapy in the untrained hands of experimenters was localized, temporary and not curative. The scientific majority disagreed, most notably his contemporary James Ewing. His contemporary critics cited the dangerous and unpredictable effects, predominantly the fever caused by the bacteria, that the vaccine had upon individuals weakened by cancer. Furthermore, the vaccine had to be made to a patient’s exact needs, making it more labor-intensive, time-consuming and expensive.

 

In 2009, Coley’s theory that immune systems in humans functioned in a cycle was demonstrated by a research team led by Associate Professor Brendon Coventry, which could have significant ramifications for cancer treatment. In 2005, drug makers including Pfizer and Sanofi-Aventis had a renewed interest in modern versions of Coley’s Toxins. Pfizer has acquired the Coley Pharmaceutical Group, set up in 1997. The historical results of Coley vaccine therapy are difficult to compare with modern results. Coley’s studies were not well controlled and factors such as length of treatment and fever level were not adequately documented. Many of his patients had also received radiation and sometimes surgery. According to the analyses of Coley Nauts and Starnes, treatment success correlated with length of therapy and the fevers induced by the toxins. Coley’s daughter, Helen Coley Nauts, established the nonprofit Cancer Research Institute in 1953 to study her father’s work. The organization has since become a leader in funding research in immunology and tumor immunology at universities and hospitals worldwide.

 

Today immunotherapy (plus personalized medicine) is cutting-edge cancer treatment. But more than 100 years ago, it was extremely difficult to get other physicians to agree with William Coley MD. Here is a more detailed account of the cases contributing to Dr. Coley’s ideas:

 

In the late summer of 1890 young Coley was getting ready to examine a new patient at his practice in New York City. What he didn’t know was that the young woman waiting to see him would change his life and the future of cancer research. Her name was Elizabeth Dashiell, also known as Bessie. Bessie was 17 and showed up complaining of a problem with her hand. It seemed like a minor injury, just a small bump where she’d hurt it, but it wasn’t getting better, and she was in a lot of pain. She’d seen other doctors but nobody could diagnose the problem. At first Coley thought Bessie must have an infection. But when he took a biopsy, it turned out to be a malignant, very advanced cancer called a sarcoma. In those days there wasn’t very much anyone could do for Bessie. This was before radiation and chemotherapy, so Coley did the only thing he could – he amputated Bessie’s right arm just below the elbow in an attempt to stop the disease from spreading. Sadly, it didn’t work, and within a month, according to David Levine, the cancer had spread “to her lungs, to her liver and all over her body.“ Bessie’s final days were wrenching and painful. Coley was with her when she died on Jan. 23, 1891. Bessie’s death made a huge impression on the young surgeon. “It really shocked him,“ says Stephen Hall, who wrote about Coley in his book A Commotion in the Blood: Life, Death and the Immune System. Bessie’s death also spurred Coley into action. There wasn’t a lot known about cancer at the time, so Coley started digging through dozens upon dozens of old records at New York Hospital. He was looking for something that would help him understand this cruel and aggressive disease. As a student, Coley had read Charles Darwin, and one of the lessons he took away from Darwin, Hall says, was to always pay attention when there’s a biological exception to the rule. “To ask yourself: Why this has happened?“ Coley discovered one of these biological exceptions. It was the case of a German immigrant named Fred Stein. Stein had been a patient in New York Hospital eight years earlier. He had a tumor on his neck that doctors tried to remove several times. Unfortunately for Stein, the tumor kept coming back and doctors expected him to die from the disease. Then Stein contracted a serious infection of the skin caused by the strep bacteria. “It looked like Stein’s days were numbered,“ Levine says. But Stein didn’t die. In fact, his tumor disappeared, and he was discharged. Coley wondered if all these years later, Stein could still be alive. So in the winter of 1891, William Coley the surgeon became William Coley the detective. He headed for the tenements of the Lower East Side of Manhattan where the German immigrant community lived. He knocked on door after door asking for a man named Fred Stein who had a distinctive scar across his neck. After several weeks of searching, Coley found him alive and cancer-free. So why did Stein’s cancer go away and stay away after he got a bacterial infection? Coley speculated that the strep infection had reversed the cancer. and wondered what would happen if he tried to reproduce the effect by deliberately injecting cancer patients with bacteria. He decided to test his idea on people who were the most seriously ill. His first subject was an Italian immigrant named Zola who, just like Bessie Dashiell, was suffering from sarcoma. Zola had tumors riddling his throat. He was so sick he could barely eat or speak or even breathe. For months Coley would try to make Zola sick from infection by creating little cuts and rubbing the strep bacteria into them, Hall says. There would be “a slight response but not too much.“ Then Coley got his hands on a much stronger strain of the bacteria. This time, Zola became violently ill with an infection that could easily have killed him. But within 24 hours, Zola’s orange-sized tumor began to liquefy and disintegrate. “This was a phenomenon that occurred rarely, but when you saw it you were utterly astonished,“ Hall says. Zola completely recovered. Coley knew he was on to something. He kept experimenting and refining his use of bacteria. Eventually, he named the treatment Coley’s toxins.

 

It was an exciting time. Coley was having tremendous success and his efforts were celebrated in America and abroad. But Bradley Coley Jr., William Coley’s grandson, says the American medical establishment at the time was skeptical. Nobody knew how Coley’s toxins worked, or why they worked sometimes and not others. Not even Coley could explain it. That’s largely because the immune system was still a mystery and would remain so for decades to come. When radiation therapy came along in the early 1900s, interest in Coley’s toxins was completely overshadowed by this new therapy. When his grandfather died, Bradley Coley says, “All interest in [Coley’s toxins] stopped.“ And quite possibly, that’s where Coley’s legacy would have ended except for this: After Coley’s death in 1936, his daughter, Helen Coley Nauts, started looking through her father’s papers while doing research for his biography. She found about 1,000 files of patients her father had treated with Coley’s toxins. She spent years carefully analyzing these cases and could see that he had extraordinary rates of success in regressing some cancerous tumors. She couldn’t get anyone interested in studying her father’s work, so she decided to do it herself. With a small grant, in 1953 Helen Coley Nauts started the Cancer Research Institute, dedicated to understanding the immune system and its relationship to cancer. In the more than 60 years since, researchers have expanded their understanding of the immune system dramatically and today, that understanding is paying off. Treatments that harness the power of the immune system are now available for a range of cancers such as stomach, lung, leukemia, melanoma and kidney. Jedd Wolchok, chief of the melanoma and immunotherapeutics service at Memorial Sloan Kettering Cancer Center, says any treatment currently in use that exploits the power of the immune system to fight cancer has to “tip its hat“ to the work William Coley began more than 100 years ago. William Coley’s intuitions were correct: Stimulating the immune system may be effective in treating cancer. He was a model of the clinician-scientist, treating patients and using his practice to initiate research and build theories. But he was a man before his time, and he met with severe criticism. Despite this criticism, however, Coley stuck with his ideas, and today we are recognizing their potential value.

Sources: NIH.gov; The University of Iowa; npr.org; Wikipedia

Stem Cell Transplants May Induce Long-Term Remission of Multiple Sclerosis

 

Multiple sclerosis (MS), is an autoimmune disease in which the immune system attacks the central nervous system. MS symptoms vary widely and may include motor and speech difficulties, weakness, fatigue and chronic pain. The most common form of MS is relapsing-remitting MS, which is characterized by periods of mild or no symptoms interspersed with symptom flare-ups or relapses. Over years, the disease can worsen and shift to a progressive form.

 

According to an article, published online (1 February 2017) in Neurology, the medical journal of the American Academy of Neurology, new clinical trial results provide evidence that high-dose immunosuppressive therapy followed by transplantation of a person’s own blood-forming stem cells can induce sustained remission of relapsing-remitting MS. Study results showed that 5 years after receiving the treatment, called high-dose immunosuppressive therapy and autologous hematopoietic cell transplant (HDIT/HCT), 69% of trial participants had survived without experiencing progression of disability, relapse of MS symptoms or new brain lesions. Notably, participants did not take any MS medications after receiving HDIT/HCT. Other studies have indicated that currently available MS drugs have lower success rates.

 

The trial, called HALT-MS, was sponsored by the National Institute of Allergy and Infectious Diseases (NIAID), part of the National Institutes of Health, and conducted by the NIAID-funded Immune Tolerance Network (ITN). The researchers published three-year results from the study in December 2014, and the final five-year results were published this month.

 

The HALT-MS study tested the safety, efficacy and durability of HDIT/HCT in 24 volunteers aged 26 to 52 years with relapsing-remitting MS who, despite taking clinically available medications, experienced active inflammation, evidenced by frequent severe relapses, and worsened neurological disability. The experimental treatment aimed to suppress active disease and prevent further disability by removing disease-causing cells and resetting the immune system. During the procedure, doctors collected a participant’s blood-forming stem cells, give the participant high-dose chemotherapy to deplete the immune system, and return the participant’s own stem cells to rebuild the immune system. The treatment carries some risks, and many participants experienced the expected side effects of HDIT/HCT, such as infections. Three participants died during the study; none of the deaths were related to the study treatment. Five years after HDIT/HCT, most trial participants remained in remission, and their MS had stabilized. In addition, some participants showed improvements, such as recovery of mobility or other physical capabilities.

Couples with Obesity May Take Longer to Achieve Pregnancy

 

According to an article published online in Human Reproduction (3 February 2017), couples in which both partners are obese may take from 55 to 59% longer to achieve pregnancy, compared to their normal weight counterparts. According to the authors, a lot of studies on fertility and body composition have focused on the female partner, but these new findings underscore the importance of including both partners.

 

The couples in the study were part of the Longitudinal Investigation of Fertility and the Environment (LIFE) Study, which examined the relationship between fertility and exposure to environmental chemicals. The study enrolled 501 couples from Michigan and Texas from 2005 to 2009. The women ranged from 18 to 44 years of age, and the men were over 18 years old. Women kept journals to record their monthly menstrual cycles, intercourse and the results of home pregnancy tests. The couples were followed until pregnancy or for up to one year of trying to conceive. The study also calculated body mass index (BMI) for each participant, categorizing couples with obesity into two subgroups: obese class I (with a BMI from 30 to 34.9) and the most obese group, obese class II (a BMI of 35 or greater). The authors compared the average time to achieve a pregnancy among couples in the non- obese group (84 men and 228 women) to that of the couples in the obese class II group (75 men and 69 women). The authors then calculated the probability that a couple would achieve pregnancy by using a statistical measure called the fecundability odds ratio (FOR). The measure estimates couples’ probability of pregnancy each menstrual cycle while trying for pregnancy, relative to their BMIs. Results showed that the class II couples took much longer to achieve pregnancy than couples not struggling with obesity. Couples in the non-obese group had a FOR of 1. Obese class II couples had a FOR of .45 — indicating that they took 55% longer to achieve pregnancy than their normal weight counterparts. When the study took into account other factors known to influence fertility — such as age, smoking status, physical activity level and cholesterol level — the ratio for obese class II couples dropped to .41, or a 59% longer time to achieve pregnancy.

 

The authors concluded that couples’ obesity may reduce fertility chances and that fertility specialists may want to take couples’ weight status into account when counseling them about achieving pregnancy. In addition to the health benefits of a healthy weight for reducing risk of other diseases such as Type 2 diabetes, heart disease and cancer, taking steps to lose weight may help reduce the time needed to conceive.

Newborn Screening System for 4 Rare Metabolic Disorders

 

Target Health is very pleased that it worked closely with Protalix Biotherapeutics for the approval of Taliglucerase alpha for the treatment of Gauche disease, we and continue to work with Protalix in Fabry disease.

 

Lysosomal Storage Disorders (LSDs) are a group of rare, inherited metabolic disorders in which enzymes (proteins) that normally eliminate unwanted substances in the body’s cells are not at normal levels or functioning properly. According to the U.S. Department of Health and Human Services’ Advisory Committee on Heritable Disorders in Newborns and Children, MPS I, Pompe, Gaucher and Fabry occur in approximately 1 in 1,500 to no more than 1 in 185,000 newborns and children, depending on the disorder. If not detected and treated in a timely manner, these disorders may cause organ damage, neurological disability or death.

 

The FDA has permitted marketing of the Seeker System for the screening of four, rare Lysosomal Storage Disorders (LSDs) in newborns. The Seeker system is designed to detect Mucopolysaccharidosis Type I (MPS I), Pompe, Gaucher and Fabry. It is the first newborn screening test permitted to be marketed by the FDA for these disorders.

 

Alberto Gutierrez, Ph.D., director of the Office of In Vitro Diagnostics and Radiological Health in CDRH, said that the Secretary of HHS s recently added Pompe and MPS I to the list of routine recommended newborn screening programs, and it is anticipated that additional states will begin requiring use of screening tests to detect these disorders. Several states currently mandate LSD screening in all newborns, including Arizona, Illinois, Kentucky, Michigan, Missouri, New Jersey, New Mexico, New York, Ohio, Pennsylvania and Tennessee. However, there were there were no FDA-authorized devices for screening of these disorders. Availability of the Seeker System provides laboratories with a screening tool that has been reviewed by the FDA for clinical and analytical validity.

 

The Seeker System, consisting of the Seeker LSD Reagent Kit- IDUA|GAA|GBA|GLA and Seeker Instrument, works by measuring the activity level of proteins required for healthy lysosomal storage found in dried blood samples collected from the prick of a newborn’s heel 24 to 48 hours after birth. The Seeker Instrument is a device that automates the analysis of dried blood spots. Reduced enzyme activity of proteins associated with any of the four LSDs detected by the kit may indicate presence of a disorder. Results showing reduced enzyme activity must be confirmed using other testing methods, such as biopsies, genetic and other laboratory tests.

 

The FDA reviewed the data for the Seeker System through the de novo premarket review pathway, a regulatory pathway for devices of a new type with low-to-moderate-risk that are not substantially equivalent to an already legally marketed device and for which special controls can be developed, in addition to general controls, to provide a reasonable assurance of safety and effectiveness of the devices. During this process, the FDA evaluated data from a clinical study of 154,412 newborns in Missouri whose dried blood samples were tested for protein activity associated with MPS I, Pompe, Gaucher and Fabry. Efficacy was determined because the system was able to accurately identify at least one of each of these four LSDs in 73 of the screened newborns. Risks associated with use of the screening system include false negative findings.

 

As part of this study, the Missouri State Public Health Laboratory conducted active surveillance of four of the state’s metabolic clinical centers for new diagnoses of these disorders. The state laboratory’s surveillance activities extended 15 months following the study’s completion to determine cases of false negatives that had not been identified during the study. No false negative results were identified either through the study or the state’s 15-month surveillance program.

 

The Seeker System was created with funding from the Small Business Innovation Research program in National Institutes of Health’s Eunice Kennedy Shriver National Institute of Child Health and Human Development. It is manufactured by Baebies Inc., located in Durham, North Carolina.

Broccoli Baked with Turkey Bacon, Lemon/Garlic & Cheddar

Trial and error — We ate this recipe three times this past week, until it was just right, as you see above.  ©Joyce Hays, Target Health Inc.

 

Ingredients

 

2 bunches broccoli

2 Tablespoons extra virgin olive oil, for cooking, or more

1 slab of turkey bacon, chopped, put into mixing bowl

20 garlic cloves, sliced, put into bowl with turkey bacon

Pinch black pepper

Pinch chili flakes

1 teaspoon curry powder

Zest of 1 lemon

Juice of 1 lemon

20 garlic cloves, sliced

2 eggs beaten in small bowl.

2 cups Panko, on flat plate

1.5 cups grated sharp cheddar cheese

 

Directions

  1. Slice the garlic
  2. Chop the turkey bacon
  3. Make your lemon zest

 

Chop the turkey bacon into little pieces. ©Joyce Hays, Target Health Inc. 

 

4. Beat the eggs

5. Grate the cheese

6. Pour out the Panko into a flat dish

7. Wash broccoli two or three times and dry well.  You want all sand and grit removed.  Break broccoli into pieces about bite-size, on paper towel.  Use stems for soup or another recipe.

8. Oil a large baking dish that you can bring to table to serve from.

9. Preheat oven to 375 degrees

10. Dip each piece of broccoli into the beaten eggs, and then roll in the plate with Panko.  Set aside

 

11.  In large skillet, put about 2 Tablespoons extra virgin olive oil and heat over medium flame.  Cook the broccoli pieces, moving them around with tongs, so that each piece is cooked to a golden brown color.  Put each cooked piece of broccoli into the baking dish.

 

Put the cooked broccoli into the oiled baking dish. ©Joyce Hays, Target Health Inc.

 

From the bowl, pour the turkey bacon garlic mixture over the cooked broccoli. ©Joyce Hays, Target Health Inc.

 

Finally, sprinkle the grated cheese over the top and put the casserole into the oven, uncovered. ©Joyce Hays, Target Health Inc.

 

Just out of the oven, smelling wonderful. ©Joyce Hays, Target Health Inc.

 

We started with chilled sauvignon blanc and the little sourdough flat crackers we recently discovered & hummus.  Then, a simple salad of tomatoes and avocados in a lemon/oil/garlic dressing.  ©Joyce Hays, Target Health Inc.

 

Then, I brought out the final version, and we loved it.  Crunchy, but still soft where you wanted it.  If I do say so myself, this was fun to experiment with, the colors are beautiful and the end result is delicious.  This is quick and easy to make, now that the trial and error part is behind us.  ©Joyce Hays, Target Health Inc.

 

For dessert, we had some chocolate covered juicy fresh strawberries, that I’m experimenting with for Valentines Day

 

Your favorite chilled white, goes well with this recipe.  ©Joyce Hays, Target Health Inc.

 

This weekend we saw Rigoletto at the MetOpera. As you know, this is a gorgeous opera and one of our favorites. The new MetO production is set in Las Vegas, the perfect setting for the dark story. The voices were fine but could have been stronger. The Russian soprano who sang Gilda has a lovely voice, but at the end of the famous quartet in the third act, (which I wait for eagerly each time we see this opera) she was not able to hit the high notes.  I felt let down. Oh well, we’ll go again next year.

 

Don’t get me wrong, the MetOpera is a glorious place and magical each time we go.  I can’t think of a better way to spend several hours, floating on beautiful waves of sound, while the stresses of life fade, momentarily.

 

https://www.youtube.com/watch?v=l6S_kx0gDzc

Here is the Rigoletto quartet, (Act 3) the way it should be heard.  It doesn’t get any better than this.  The brilliant soprano here is Dame Joan Sutherland, the glorious tenor is the greatest, Luciano Pavarotti

 

 

From Our Table to Yours !

 

Bon Appetit!