Disease: Von-willebrand Disorder Type 2M

Patient1: 13 YO male

Patient2: 16 YO female

Note they are siblings

Variant: VWF NM_000552.5: c.5192C>T p.(Ser1731Leu), heterozygous variant, in A3 domain in exon 30

Phenotype patient 1: several bleedings after tosillectomy, recurrent epistaxis, decreased VWF:CB ratio, decreased VWF:CB/VWF:Ag ratio

Phenotype patient 2: menorrhagia, intermittent gum bleeding, easy bruising, decreased VWF:CB ratio, decreased VWF:CB/VWF:Ag ratio

For both patients: VWF:CB ratio corresponds to collagen type 1, multimer analysis was normal, VWF:Ag, VWF:Ac, and factor VIII activity was normal.

Family: Not listed

Present in dbSNP (rs764077750)

Present in gnomAD, rare MAF (ALL: 0.0012%)

Predictions:

SIFT- predicted deleterious (Score 0.01, median 3.34)

MutationTaster- Predicted disease causing (probability score 1)

PolyPhen2- Predicted probably damaging (Score 0.983)

CADD score- 26.5

Authors mention similar AA substitutions at the p.1731 position which leads to reduced binding of VWF to collagen in other patients.

Authors also cite a functional human cell experiment in COS7 cell line which resulted in functional VWF defect.

Authors conclude this novel variant as likely pathogenic.

Disease: Von Willebrand Disease (VWD)

Patient: 18 yo, Male, heterozygote

Variant: VWF NM_000552.5: c.5456_5842del p.(R1819_C1948delinsS)

Was not present in gnomAD when searched

Dominant negative effect

Phenotypes:

lower collagen-binding capacity

History of bleeding (epistaxis)

gum bleeding

cutaneous bruises

ADAMTS13 resistant

Family: Mother, father, sister are asymptomatic

Suggested as de novo, no picture found in patient's relative of the deletion, loss of A3 loop

They can no longer evaluate a school based how students perform, but based on the physical environment of the school and how that effects the students.

At least half of these schools are accredited with conditions and within those 22 all of the middle schools in the county have been accredited with conditions. This leaves students no other choice for their middle school education. For those that are in poverty as well cannot flock to private schools because of how expensive.

Thankfully, one of the major problems within RPS has been corrected, yet there is still a long way to go with improving these schools.

I agree with this statement because students have not been receiving proper education to learn a second language. A computer program shouldn't be held accountable.

There is already a food shortage in Richmond, along with areas that are in great poverty, so a food tax may not be the best solution.

making or constituting a disturbingly harsh and loud noise

Another student left discouraged when her school doesn't even have up to date basic technology and materials that other schools had.

This is a possible solution as well, but those that do have a higher income often times don't want to have to pay higher taxes to help schools in other zones

How are public schools supporting their students that are in poverty earn they are already in such poor conditions?

They are putting children at risk of health problems and is not allowing them to be fully enthralled with their education.

The plan that would be most ideal to this issue is Congress providing the $100 billion over a decade.

Students are forced to work in extremely cold environments which could harm their academic performance since they do have to be concerned with how cold their school actually is.

Because of the intense spending done in the past, schools are still suffering today and will be for years to come.

Minorities are stuck in their current living situation since they are at an economic disadvantage, while white students can often times afford to receive a better education.

As previously stated, students are performing poorly due to the schools conditions, possibly making their self esteem lower and a possible increase in depression.

The conditions of the schools can miscommunicate about how much they truly care about their students, making them feel discouraged.

Not only are students, physically suffering, but as well as their mental health.

At least it goes to show that this problem is at one of the highest priorities.

Even the superintendent, Jason Kamras, feels that students are sitting through inhumane conditions.

Although this is a good sum of money only 3 schools would be fixed, leaving 19 schools unaccredited.

Not only would this help out Richmond schools considerably, but also help other schools around the U.S. that are also in need of repair.

Since schools are funded by property taxes, urban and rural areas are struggling the most.

This is another possible solution for Richmond, but would require them to seek out a public-private partnership to help bring down the cost of how much it would take to repair the schools.

This would be the bare minimum needed to get schools to full accreditation.

Because of how poor the public schools are kept, this affects the students performance in school. Which can then inhibit them from obtaining their high school diploma or a college education.

Why should a child have to be concerned with the possibility of inhaling mold, a possible ceiling tile striking them as they walk through the hall, or their privacy in the restroom? These are all factors that can be critical to the students and teachers health and mentality.

Not only are students envious of other better performing public schools, but the teachers are as well. Teachers are just as disappointed with schools that have been accredited with conditions.

Small children have to be exposed to unsanitary conditions, on top of teacher having to clean up after rodents which could lead to harmful diseases.

The plan that would be most ideal to this issue is Congress providing the $100 billion over a decade.

In operant conditioning, Keller and Schoenfeld (1950) pointed out that behavior can either increase with reinforcement or sometimes decrease if satiation has occurred. There are two types of conditioning: Type S which is the Pavlovian conditioning and which is measured using latency and response magnitude and Type R which is operant conditioning associated closely with Skinner and is measured using frequency of occurrence. In the Type S conditioning, responses occur by the stimulus presented whereas in the Type R conditioning, responses occur independently. it is interesting to note that both types of conditioning can be present at the same time when a behavior is being studied. Just as Skinner used rats and pressing the bar in operant conditioning, the article by Colon, Ahearn, Clark, and Masalsky (2012) uses verbal operant training in children with autism spectrum disorder (ASD) to decrease the amount of various forms of echolalia used among these children. Using frequency of occurrence, the researchers measured correct and incorrect vocalizations. Children were reinforced with edibles and social praise when they vocally responded appropriately. Just as Skinner used vertical pen marks to tally pellet eating by the rat, Colon et al. (2012) used momentary time-sampling to measure vocal stereotypy. The intervention (vocal operant training) did result in less occurrences of inappropriate vocalizations which in turn, will benefit students socially as they will not become so stigmatized and will be able to participate in conversations as they improve their functional language. Both readings allow the reader to understand the significance of operant conditioning and how it can improve daily living for people.

Question: Think about a time you used operant conditioning to reduce a target behavior. What did you do? What were the results?

Reference Colon, C. L., Ahearn, W. H., Clark, K. M., & Masalsky J. (2012). The effects of verbal operant training and response interruption and redirection on appropriate and inappropriate vocalizations. Journal of Applied Behavior Analysis, 45, 107-120.