Zevra Therapeutics, Inc. (NasdaqGS: ZVRA) (Zevra, or the Company), a rare disease therapeutics company, today announced that the U.S. Food and Drug Administration (FDA) Genetic Metabolic Diseases Advisory Committee (GeMDAC) voted favorably (11 yes, 5 no) that the data support that arimoclomol is effective in the treatment of patients with Niemann-Pick disease type C (NPC).
“We are extremely pleased with the committee’s recognition of the benefits of arimoclomol for people living with NPC,” said Neil F. McFarlane, President and Chief Executive Officer of Zevra. “Based on the totality of the clinical data, including data from the pivotal trial, the long-term data from the arimoclomol open label extension study, and data from our expanded access programs (EAP: NCT04316637), we remain confident in the clinical benefit offered by arimoclomol as a treatment for NPC, and are optimistic about its continued path to approval.”
The GeMDAC, which consists of experts in the fields of medical genetics, inborn errors of metabolism, epidemiology and other related specialties, discussed the benefits and risks of arimoclomol, including the data recently presented at the 45th Annual Meeting of the Society for Inherited Metabolic Disorders (SIMD), and reviewed comments received from independent experts, NPC patients, and patient advocacy group representatives. The committee’s recommendation will be considered by the FDA as it completes its independent review of the arimoclomol NDA; however, the feedback from the GeMDAC is not binding upon the Agency. The arimoclomol NDA has been assigned a Prescription Drug User Fee Act (PDUFA) action date of September 21, 2024.
A staffer for Philadelphia Mayor Cherelle Parker 'accidentally posted' a video meant to be released next week which reveals Pennsylvania Governor Josh Shapiro as Kamala Harris' running mate.
"Philly political sources have told me that a staffer connected with Mayor Cherelle Parker's team accidentally posted the video today," posted journalist Ernest Owens on X.
"The video was scheduled for Monday...after VP Kamala Harris was expected to announce her pick."
"It's Josh Shapiro, y'all," Owens continued.
Or, as Owens also suggested - this could be an 'accident' which serves as a trial balloon, and Harris could go in a different direction.
Shapiro notably canceled fundraising events in New York this weekend and "is likely to meet with Vice President Kamala Harris as she works to pick a running mate," reported WHTM earlier in the day.
Meanwhile, Shapiro has come under fire from women's groups after his administration settled a sexual harassment claim against one of his longtime aides for $300,000.
In a statement headlined “Gov. Shapiro’s Failures Enabled Sexual Harassment,” the National Women’s Defense League said that the Harris vetting team should “consider the handling of past complaints of sexual harassment inside the Pennsylvania Governor’s office.” The group claims to be a nonpartisan organization dedicated to preventing sexual harassment. -Daily Beast
We're guessing said women's groups will promptly fade into the bushes.
An inspector general’s report has accused the Biden-Harris Administration’s Department of Homeland Security (DHS) of covering up a significant report regarding the Secret Service’s response to the protest at the U.S. Capitol on January 6th.
As reported by the Daily Caller, Inspector General Joseph Cuffari, who was originally appointed by former President Donald Trump, has launched two investigations into the Secret Service’s role during the peaceful protests on January 6th, 2021. As the Secret Service is under the jurisdiction of DHS, it is that agency which makes the final determination on which reports to release or withhold.
Jonathan Meyer, the current head lawyer for DHS, issued a statement denying Inspector General Cuffari’s claims, saying that DHS will only redact “security sensitive” information, but otherwise will not prevent Congress from seeing any report they want to see.
Speaking to Politico, an anonymous source from within DHS said claimed that it is the inspector general who “has exclusive authority to determine when to release a report to Congress.”
The report in question is titled “USSS Preparation for and Response to the Events of January 6, 2021.” Sources from within Congress told Real Clear Investigations that the report has been in Secretary Alejandro Mayorkas’ possession “since at least April.”
Similarly to the security failures that led to the assassination attempt against President Trump on July 13th, it has been reported that people with weapons were spotted outside the White House on January 6th. In another security failure, the car in which then-Senator Kamala Harris (D-Calif.) was traveling drove right by the alleged pipe bomb that was found outside the Democratic National Committee (DNC) headquarters.
In July of 2022, the Office of Inspector General (OIG) sent a letter to the House and Senate Homeland Security Committees revealing that “many [Secret Service] text messages, from January 5 and 6, 2021, were erased as part of a device-replacement program.” Cuffari’s office had previously requested that those exact messages be handed over, only for them to be deleted.
These revelations add to a growing sense of strong dissatisfaction with the Secret Service in the wake of the July 13th assassination attempt, with numerous security failures, lack of preparedness, and overall incompetence being blamed for the incident which cost one rallygoer his life and injured two others, with President Trump narrowly dodging a bullet himself. Director Kimberly Cheatle ultimately resigned after receiving bipartisan backlash for her role in the decline in the quality of the Secret Service.
We may well be on the front end of arecession, as the economic storm clouds darken even more and that's the subject of the riff. With today's terrible jobs report and another big smackdown of stocks, it certainly looks like the U.S. economy is standing on the front-end of a recession.
The economic storm clouds continue to darken. Recession indicators are proliferating. Here's one: the unemployment rate has moved up to 4.3% from its low of 3.4% last year. Some economists believe that when the 3-month average unemployment rate rises above the 3-month average rate in the prior year, that's a recession signal.
So, what you get is: last year, the lowest 3-month average rate was 3.5%. Today, it's jumped up to 4.1%. Again, those are 3-month smoothing techniques, but it tells a very disappointing story. Besides, the rising unemployment rate in July, 352,000 were reported as unemployed. That's the biggest number in a long time.
The small business household employment level increased a scant 57,000. On the corporate side, private payrolls rose only 97,000 -- way below expectations and another big recession threat indicator is a drop in overall hours worked, and an even bigger drop in manufacturing hours worked. This comes directly after yesterday's recession-prone manufacturing report from the Institute of Supply Managers. There's more evidence that a housing recession is deepening, and consumer confidence has been in the dumps for quite some time.
We can say with certainty that a recession has arrived when the levels of income, jobs, industrial production, and total retail sales begin falling. That's the classic recession definition from the NBER, National Bureau of Economic Research, but that has not happened yet.
The Biden-Harris administration has already suffered at least a mini-recession in the first half of 2022, when skyrocketing 9% inflation drove GDP down for two consecutive quarters and, of course, the soft underbelly of Bidenomics (now Kamalanomics?) is the ongoing affordability crisis where the level of consumer prices has surged 20% during their term.
The year-to-year change in the CPI has gone from 9% to 3%, but tell that to middle-class working folks sitting around their kitchen table trying to figure out how to buy groceries, gasoline, pay insurance costs, and afford day-to-day essential consumer goods and these consumer price numbers no longer include high borrowing costs for mortgages, car insurance, and today's 20% to 25% credit cards.
Meanwhile, all the liberals want the Fed to slash their interest rate targets to avoid a recession, but Fed policy changes in August or September wouldn't impact the economy for at least a year and, meanwhile, the plunge in market bond yields looks like a coincident indicator of recession, even while lower interest costs are welcome.
If you take a look at what Kamala Harris seems to be promoting, she wants the Fed to cut their interest rates, but meanwhile she wants to put into place huge tax hikes and keep up the over-regulation of business and energy. You know what you get out of that? Inflationary recession. An easy-money Fed increases demand while tax and regulatory penalties reduce production supply, but you're never going to believe this folks, Kamala’s people are actually blaming Trump for this downturn -- 4 years after he’s left office.
That’s even better than she doesn't believe in a ban on fracking. Trump on the other hand is much smarter. He wants major tax cuts and regulatory rollbacks to make it easier and more productive for businesses to produce and bring down energy costs. That'll increase the supply of goods, curb the inflation rate and generate a tremendous economic boom. So, folks, tighten your belt for the moment, but help is on the way. That's the riff.
A Texas rancher has filed a lawsuit against President Joe Biden and the Department of Homeland Security (DHS) accusing them of adopting policies that have resulted in “the biggest influx of illegal aliens into America in our history.”
The lawsuit was filed in the U.S. District Court for the Southern District of Texas on July 31 by the Immigration Reform Law Institute on behalf of the rancher, identified in the filing as Michael Vickers, along with Kinney County Sheriff Brad Coe and Atascosa County, Texas
It also lists DHS Secretary Alejandro Mayorkas, U.S. Customs and Border Protection Acting Commissioner Troy Miller, U.S. Immigration and Customs Enforcement Acting Director Patrick J. Lechleitner, and U.S. Citizenship and Immigration Services Director Ur M. Jaddou as defendants.
“With full awareness of the likely consequences, Defendants have adopted unlawful policies that, working in concert, have frustrated Congress’s purposes in the immigration laws,” the lawsuit states.
According to the legal filing, Vickers—who is also a veterinarian—lives with his wife on a 1000-acre ranch in Brooks County, Texas.
The ranch is located about 70 miles north of the international border with Mexico, according to the lawsuit.
“Because of Defendants’ policies, tens of thousands of illegal aliens have been released into the interior who thereafter travel cross country across the grasslands of Plaintiff’s ranch,” the filing reads.
Ranch Suffered ‘Thousands of Dollars in Damage’
In so doing, the illegal immigrants “routinely cause thousands of dollars in damage to fences or gates as they pass through the ranch,” and leave “tons of trash and litter,” the lawsuit states.
In some cases, Vickers has found plastic bags and trash inside the stomachs of his cows, according to the lawsuit.
At other times, his cattle have escaped through cut fences and gates torn down by illegal immigrants, the filing alleges.
As a result, since early 2021, Vickers has incurred “more than $50,000 in fence and gate damages alone” and has been forced to spend thousands of dollars to mitigate environmental damage, according to the lawsuit.
The legal filing also notes that Vickers and his wife have to keep dogs on their ranch for security.
The dogs have caught “hundreds of criminal trespasses,” many of whom are members of various gangs including the notoriously violent MS13, Tangoblast, Pistoleros, and the Mexican Mafia, among others, the lawsuit states.
Because of the presence of criminal groups facilitating illegal immigration, Vickers must always carry a pistol to feel safe, according to the lawsuit.
The lawsuit argues that the defendants’ actions amount to a violation of the take care clause of the U.S. Constitution that is “conceptually clear, historically unique, and actionable by those it especially harms,” the lawsuit states.
The take care clause requires the president to “take Care that the Laws be faithfully executed.”
The U.S. flag flies at half-staff at a port of entry at the U.S.–Mexico border in Brownsville, Texas, on Feb. 24, 2021. (John Moore/Getty Images)
Illegal Border Crossings Decline
“Congress has passed numerous laws aimed at achieving operational control of the border, defined as zero illegal entries. But Defendants’ policies, issued under the authority of these laws, are calculated to result in, and have resulted in, the current, massive flood of illegal entries by foreign nationals from around the world,” the lawsuit states.
The lawsuit asks the court to declare the federal government’s various border policies unconstitutional, including parole programs for Cubans, Haitians, Nicaraguans, and Venezuelans; family reunification parole processes; and the memorandum terminating Migrant Protection Protocols, also referred to as the “Remain in Mexico” program.
The plaintiffs are also seeking unspecified costs and attorney’s fees.
Since taking office, Biden has sought to expand lawful pathways into the United States as part of efforts to slash illegal crossings while trying to address the root cause of what he says is a “broken” immigration system.
In June, his administration enacted restrictions on asylum claims at the southern border, which led to a drop in illegal border crossings.
In a July 31 statement, the Immigration Reform Law Institute (IRLI) said the government’s policies have resulted in the “biggest influx of illegal aliens into America in our history.”
“This is no mere policy failure, or just a violation of statutes, but flagrant disobedience to the Constitution,” Dale L. Wilcox, IRLI’s executive director and general counsel, said. “We hope the court sees Biden’s war on the laws he is supposed to be enforcing as the constitutional offense it is, ends these policies, and grants an injunction.”
The Epoch Times contacted the White House for comment but didn’t receive a reply by publication time.
It is well after midnight in the Iranian capital of Tehran and there are two significant developments that suggest an Iranian and Hezbollah major strike against Israel could come at any moment.
First, Iranian state TV is strongly signaling that an attack could come in the overnight and early morning hours of Friday/Saturday - which we should note also marks Jewish shabbat in Israel.
"In coming hours, the world will witness extraordinary scenes and very important developments," an Iranian state news anchor reported said. Iran Internationaland other regional publications have highlighted the alarming clip as well.
While it is unlikely that a news anchor would be directly privy to such state secrets concerning the timing of a major strike on Israel from Iran, it does point to growing anticipation and belief among the Iranian population that retaliation is near.
It is also the case that following memorials and funerals in Tehran and Doha for slain Hamas leader Ismail Haniyeh, he has finally been laid to rest, and Tehran might now be readying its response given that foreign dignitaries have now safely exited the country.
At the same time Israeli media has reported on another big development related to Iran-backed Hezbollah in Lebanon. "Hezbollah is evacuating its senior personnel from the terror group’s strongholds in the southern suburbs of Beirut, in anticipation of potential Israeli strikes, Arabic media sources reported Friday," writes Times of Israel.
"The Iran-backed group has also reportedly moved the military equipment it stored to areas further away from the Lebanese capital," the report adds.
An anonymous US official has also been cited as saying, "Tehran will take time to decide on the type of response it launches, and it will take time to prepare the response."
Via AFP
The prior April 13 ballistic missile and drone attack from Iran on Israel took several days to happen after Iran leaders first said they would hit back. But that was highly telegraphed to be limited in nature, to ensure a broader regional war would be avoided. Any new attack in revenge for killing Haniyeh on Iranian soil is likely to come quicker, and be less predictable - given Iran feels pressure to up the escalation in order to establish greater deterrence.
Iran has continued putting Israel on notice, even crafting messages concerning Lebanon, warning against "full-scale military aggression" against Hezbollah, saying it will lead to "obliterating war" - according to a fresh statement the Islamic republic’s United Nations mission. "All options, [including] the full involvement of all Resistance Fronts, are on the table," the mission wrote in a post on X late in the day Friday.
