OpenLLM-France/wikimedia huggingface dataset:
# Handbook of Genetic Counseling/Zellweger Syndrome

Zellweger Syndrome

## Genetic Etiology

* Mode of inheritance: Autosomal recessive with non-allelic heterogeneity.
* Chromosome location: 2p15, 1q22, 12p13.3, 7q21-q22, 6q23-q24
* Molecular genetics: Phenotype caused by mutation in any of several genes involved: in peroxisome biogenesis (peroxi-1, -2, -3, -5, -6, or -12).
* Peroxisomes are subcellular organelles that play a role in lipid metabolism--these are absent

## Incidence and Carrier Frequency

* Incidence is 1 in 40,000. Males and females are affected equally.

## Clinical Features

* Craniofacial Features
** Flat occiput and face
** Anteverted nares
** Epicanthal folds
** Brushfield spots
** High forehead
** Large fontanels
** Shallow orbits
* Ocular Features
** Cataracts
** Hypoplastic optic disk
* Auditory Features
** Abnormal helices
** Deafness
* Neurological Features
** Early developmental delay
** Seizures
** Brain migrational abnormalities
** MR in survivors
** Brain malformations including microgyria, heteropias, subependymal cysts, astrocytosis and gliosis, hypoplastic corpus callosum, hypoplastic olfactory lobes
* Extremities
** Single palmar creases
** Joint contractures
** Stippled epiphyses
** Camptodactyly
* Cardiovascular Features
** Cardiac septal defects
** PDA
* Other Features
** Hepatomegaly
** Postnatal growth deficiency and low birth weight
** Poor suck
** Albuminuria and cysts of glomeruli in kidneys
** Elevated serum iron level and evidence of excess iron storage
** Absent liver peroxisomes

## Age of Onset, Natural History, Life Span

* Most babies are born breech and exhibit failure to thrive. Some developed icterus and bloody stool.
* Vast majority of children die within the first year of life. Those who survive have profound mental retardation. Average life span is 12-13 weeks.

## Testing

* Diagnosis is made through DNA testing.
* Increase in plasma and fibroblasts of very-long-chain fatty acids and lack of dihydroxyacetone phosphate acyltransferase (DHAP-AT) are biochemical markers for this syndrome

## Surveillance, Management, and Treatment Options

* Multidisciplinary early intervention
* Oral administration of plasmalogens
* Restricted phytanic acid intake
* OT, PT, hearing aids, nutrition
* Skeletal surveys (X-rays, CT and MRI)
* Renal and abdominal ultrasounds

## Differential Diagnoses

* Down syndrome is the most common

## Notes

The information in this outline was last updated in 2002.
# Handbook of Management Scales/Ability
## Ability (alpha = 0.96/0.96)
### Description

Three facets of trustworthiness were measured based on scales designed by Mayer/Davis (1999) to assess the ability, benevolence, and integrity of a supervisor. The Likert-type scale ranged from 1 (strongly disagree) to 5 (strongly agree).

### Definition

Ability reflects concepts such as competence, skills, efficiency, and dedication.

### Items

* My supervisor is very capable of performing his/her job.
* My supervisor is known to be successful at the things s/he tries to do.
* My supervisor has a lot of knowledge about the work to be done.
* I feel very confident about my supervisor’s skills.
* My supervisor has specific capabilities that can increase our performance.
* My supervisor is well qualified.

### Source

* Colquitt/Rodell (2011): Justice, trust, and trustworthiness: A longitudinal analysis integrating three theoretical perspectives. Academy of Management Journal, Vol. 54, No. 6, pp. 1183–1206

### Comments

The mean value was above four. Given that a 5-point scale was used, future researchers could slightly adapt the items in order to shift the mean value to the center (e.g., “very successful” rather than “successful”).

### Related Scales

* Benevolence
* Integrity
* Trust
# Handbook of Genetic Counseling/Von Gierkes Disease (Glycogen Storage Disease Type I)

Von Gierkes Disease (Glycogen Storage Disease Type I)

## Description

* Pathway= The body needs sugar in the form of glucose to function. Glucose is what gives all of our cells the energy they need to function properly. In order to get it, it has to be broken down from larger molecules that store it (glycogen). It is primarily stored in the liver and muscles. This disorder has achnage in the genes that make the pieces that break down the larger molecules into sugar = the levels of sugar are too low and the large molecules start to build up.
* Deficiency in the breakdown of glucose. G6Pase activity requires 2 components of the microsomal membrane: (1) a glucose-6-phosphate specific transport system that shuttles G6P from the cytoplasm to the lumen of the endoplasmic reticulum (a G6P translocase), and (2) an enzyme, glucose-6-phosphate phosphohydrolase, bound to the luminal surface of the membrane.
* Three types
** GSD Ia = the basic defect in glucose-6-phosphatase activity ion the liver, kidney and intestinal lining.
** GSD Ib = defect is in the glucose-6-phosphate transfer gene so you can see activity of glucose-6-phosphatase when testing.
** GSD Ic = defect is in the pyrophosphate transfer - RARE
** GSD Id = defect in glucose transport - RARE

## Characteristics

* Type Ia (most commonly associated with vGD.
** Short stature & growth delay
** Abdomen protrusion
** Build up of glycogen and fat in the liver
** Enlarged liver and malignant/benign growths in the liver
** Yellow plaques on skin (xanthomas)
** Spider-like arteries, gout in the ear or kidney
** Bleeding disorders
** Hypoglycemia
** Hypertension
** Doll-like faces with large cheeks
* Type Ib
** All of the above
** Small numbers of neutrophils in the blood (neutropenia)
** Bacterial infections
** Oral and intestinal sores.

## Genetics

* Type Ia maps to chromosome 17q21.
* Type Ib maps to chromosome 11q23
* All are autosomal recessive

## Diagnosis

* Based on variety of lab results
** G6PD
** Increase in lipids in blood (lipidemia)
** High levels of uric acid in blood (hyperurcemia)
** High levels of ketones in blood (ketonemia)
** Metabolic acidosis (increase of acids or decrease in water fluid)

## Treatment

* Glucose drinks during the day
* Infusion at night through a nasogastric tube
* Cornstarch throughout the day for older children
* Dietary restrictions on fructose and galactose = need to supplement with calcium and multivitamins.
* Add Allopurinol to reduce uric acid levels which occurs because of the decrease in renal clearance and the increased production of uric acid as glycogen is not broken down.

## Notes

The information in this outline was last updated in 2000.
# Handbook of Management Scales/Accountability
## Accountability (alpha > 0.70)
### Description

A multidimensional measure of feedback orientation is developed and validated. This feedback orientation scale (FOS) was developed and refined using two pilot studies and two focal studies. Across these studies, substantial support was found for a four-dimension FOS scale. The four dimensions are utility, accountability, social awareness, and feedback self-efficacy. Study 1 found substantial support for the reliability and validity of the scale. Study 2 provided support for the generalizability of results to a field sample, with five of the six hypotheses retested being supported.

### Definition

Feedback orientation is defined as an individual’s overall receptivity to feedback.
Accountability is defined as an individual’s tendency to feel a sense of obligation to act on feedback.

### Items

Administer these items using a 5-point scale from strongly disagree to strongly agree.
* It is my responsibility to apply feedback to improve my performance.
* I hold myself accountable to respond to feedback appropriately.
* I don’t feel a sense of closure until I respond to feedback.
* If my supervisor gives me feedback, it is my responsibility to respond to it.
* I feel obligated to make changes based on feedback.

### Source

* Linderbaum/Levy (2010): The Development and Validation of the Feedback Orientation Scale (FOS). Journal of Management, Vol. 36, No. 6, pp. 1372-1405.

### Related Scales

* Feedback self-efficacy
* Social awareness
* Utility
# Guide to Social Activity/Masculinity

The current version of this module lists desirable traits in person, and has not much connection to the former title, "masculinity".

## Chivalry

Starts with politeness and common courtesy
Reverence and respect for womanhood. (They are half of the human race, and mothers to the other half!)
"Milady, your chariot awaits" as opposed to *HONK! HOOOOOONK!* on the car horn
Dwelling on a girl's good characteristics, rather than bad characteristics. (Think "Man of La Mancha")
Masculinity- The degree or property of being masculine or manly; manliness
E.g. Girls Were Dressing, to the boys wearing sporty clothes or baggy clothing

## Protectiveness

Some protection that one person can provide to their partner:
* financial stability and security
* honor? safety? (FIXME how?)

## Competitiveness

Also related to ambition.
Some persons adore those who become the best they can be.

## Physical Strength

Some persons adore those with physical strength who do help instead of bully other people. One can use physical strength to help move furniture and other heavy objects.

## Moral Strength

A person that maintains morals has more respect. Furthermore, a person who chooses to be dishonest are often discovered and dumped.

## Humility

It is advantageous to be able to admit fault to one who one loves. It is advantageous to be able to say, "I am sorry".
# Guide to The Lord of the Rings

Welcome to the book for the Lord of the Rings trilogy by J.R.R. Tolkien, and Middle Earth in general. This book strives to provide a detailed source of information about Middle Earth.

## Where to Start

This book has been designed to handle several different types of readers of The Lord of the Rings. Therefore, this book has several pages suggesting reading guides for the specific levels.
* Beginner − Readers who are new to The Lord of the Rings. This should be used by readers who have read only a small amount of the books or who are interested in a general overview of the storyline.
* Intermediate − Readers who have read much of The Lord of the Rings and know many of the main characters and places. Includes characters, places, events and a more detailed view of the storyline.
* Advanced − Readers who have read all of The Lord of the Rings and The Hobbit who wish to acquire detailed knowledge of the books. Almost every topic is covered.

## Index

The index is the best way to navigate.

## Major Sections

* Books − Chapter by chapter guides to the individual books
* Events − Detailed descriptions and analyses on major and minor events in the books
* Characters − Descriptions and analyses of the major and minor characters
* Places − Descriptions and analyses of the locations
* Races − Information on the races of Middle Earth
* Animals and Plants − Information on various species of and individual animals and plants
* Items − Information on the various items in Middle Earth
* Languages − Information on the individual languages and writing scripts
* Timeline − A detailed timeline of events throughout the ages of Middle Earth

## Minor Sections

* Concepts and Ideas
* Emblems and Symbols
* Constellations, Stars and Planets
* Councils and Groups
* Food and Drink

## Book Goals

* To provide a large source of information for The Lord of the Rings trilogy, its accompanying books and Middle Earth in general
* To provide a source of information which is easy to find
* To minimize unnecessary spoilers through structured pages that allows readers to explore content as deep as (s)he wishes to
# Guide to Social Activity/Driving

| Authors | | Wikibooks Guide to Social Activity | Edit template |
Alcohol | Body Language | Courtship | Driving | Observing Sports
Contribute to this book! Start or edit a chapter, then add yourself to the list of Authors.
Driving is a social activity because the driver must share the road with other drivers and pedestrians. Also there are telephones, drive-through shops, cyclists, etc.

### Topics to be added

* Traffic (In English, traffic refers to the mass movement of cars.)
* Road rage
* Accidental deaths
* Races (on the public streets)
** Drivers that want to pass every other car
* Persons who like to buy cars; car culture
* Persons who hate high petrol prices
This page or section of Guide to Social Activity is a stub. You can help Wikibooks by expanding it.
# Guide to Social Activity/Stubborn Difficulties
## Stubborn Difficulties
### "I think I'm ugly"

In as much as there is an objective element to attractiveness, this may well be the case. Remember, however, these five important principles:
* There is a very large subjective element to attractiveness as well.
* Physical attractiveness is not the only aspect of you that a guy cares about.
* Goodness beautifies a person. A joyful countenance, an upbeat and outgoing personality, and a mind or heart that are genuinely engaged or concerned with or about other things and people, will make you more attractive.
* Take care of your appearance. Make the best of what you've got. Perhaps you aren't Sophie Marceau, but by being disciplined in 'doing the basics' (washing, brushing and deodourising), you can make an improvement.
* Bide your time. Strange though it may seem, and contrary to the popular contemporary media, women often get more attractive with age.

### "No one would approve"

If your society, friends or family won't approve then the following advice might help:
* Decide on the morality of your move. If you are offending some higher law, then suffer in silence and wait another spring.
* Grasp the thorn. Let those that disapprove know first. Don't go behind folks backs. Don't ask for advice if you're not prepared for it.
* Listen to them. Maybe he is a waste of time, abusive or capricious. Does their point of view ring true?
* Cool off. Sometimes family approval just takes time. Are you prepared to outwait those who object? Are you prepared to take the time to see what truth there is in their opinions.
* Make your position clear. Stand your ground. Your beloved, if he is true, will hurt as much as you do; but be prepared for the consequences. See en:w:Romeo and Julliet!
* And if he really means all that to you: elope.

### "He just doesn't notice me"

* This isn't necessarily true. He might be as nervous as you are.
* Also, he might have a reason not to notice you (is he in a relationship?)
# Guide to Social Activity/The Catch

Men are infinitely complex. Yes, they may be partly governed by certain primitive urges, but they are also capable of the height of feeling that produced Byron and Pushkin, the immensity of genius that characterised Einstein and Bohr, and the generosity of spirit that became Gandhi and Booth.
Been dumped recently? Only know grief from men in Your life? Don't despair for while such transgression is true for many men most of the time, and all men some of the time, there is another side to the other half.

## Know your Target

Men have a number of general tendencies that it is important to be aware of. Following these pieces of advice will help:
* Be yourself. On pragmatic and moral grounds there is no basis for insincerity. You are who you are because you govern your life.
* Contain yourself. This however does not mean that you need to reveal or lay out on a plate everything that you sense or think or feel or believe. Keep some things back. A certain tentative restraint in a woman is frighteningly attractive.
* Relax. Remember that if it doesn't work it doesn't work. Have fun, and remember, the process takes time for a reason. Look beyond the tree and wait for the forest to come into view. You have as much time as you make it, to decide for sure if anything is permant.
* But now DO SOMETHING. If there are no guys where you are, go or wait patiently. If there is a guy, spend time around him. Let him know somehow. If there is more than one guy in your life, decide on one.
* Let him know. Use your own judgement. Think to yourself, "What if..." Well?
* Offer ideas and input when suggested If you want to do something, don't expect him to have all the answers. Don't assume too much in this area. If you really need to size up the situation, it'll be clear the more you interact and get to know each other. If you have something you like to do, offer it. (See Relax)
* Don't miss the blindingly obvious.
* Say "yes". Maybe it will come out of the clear blue sky, or maybe you've been waiting for years, but when he asks you out, then remember to say "yes".
* The massive secret: Don't be put off. Sometimes men just don't see it. Sometimes there are other things happening in their life. Sometimes there might be a relationship that he had not intended to get entangled up, or maybe he simply hadn't met you yet. Often, in the present author's experience, the girl walked away just as she had began to be properly appreciated. Patience is a necessary virtue and against any setback it is important not to be put off.

## Don't
### Burn the bridge

    Things may not be right/work out until later, much later. You can't see far enough down the road to know for sure. At any rate, the bad blood you instill ends up hurting you just as much in the long run.
# Guide to Social Activity/General Tips
## General Tips

Here are a couple of things to keep in mind when dealing with the fairer sex (or pretty much anyone, it's just a matter of application).
Don't lie. It's simple, but its really important. Always be honest, and you'll avoid many a headache. This doesn't mean you can't exaggerate, but don't over do it. If you say you're going to do something you had better do it. If you can't do it, come up with a really good reason why (i.e. the truth), or make sure your intention to accomplish whatever it is was clear from the start. Lying rarely yields a sustainable benefit.
Don't gloat. No one likes a bragger. Sure, you can reminisce of something awesome, but it needs to be an engaging story.
Always be respectful. Not just to your date, but to everyone you encounter (with or without her). This will build a solid reputation for yourself and leave you in good standing with many. If you are in a position to help, help; even if it is a minor inconvienance. Be curteous and patient with all. Maintain a clean sense of humour, jokes at the cost of others just aren't worth it. Actions always speak louder than words, and by the end of the day if your the woman you're interested can see or has heard of your kindness and generosity you will get you in her scope even if you aren't the best looking or an excellent conversationalist.
While being direct has its benefits, keep it in context. Women tend to enjoy a mystery. Don't skimp out when building a mystique. Keep it fun and interesting. So when a lady asks you who you like don't be a moron and just flat out say "I like you." Not like that, build it up first. "Well I'm sure you know her, blah blah blah." (but only if you're really corny)
# Forth/Structuring vs. words that actually do something

We have described a process of creating definitions of words by listing sequences of other words previously in the dictionary which are to be executed in turn in the order given. One can imagine calling a "high-level" word which in turn calls other "high-level" words lower in the dictionary, which call other words lower still. Note that all this structuring is for the benefit of the programmer, maintenance, and many other good reasons to be there.
There obviously needs to be something else than a means to organize sequences of previously defined words, and so on ad infinitum. Eventually we must come to a different type of word that actually does something: adds two numbers, moves a value, or other operation normally thought of in connection with "machine instructions". These are called "machine code words". At the bottom of the dictionary, classic Forth systems include a set of words of this type that implement a more or less standard "Forth Virtual Machine". Often each machine code word is a short sequence of assembly language instructions that implement a particular standard function, for example a parameter stack manipulation named SWAP. Alternate implementations implement machine code words directly in hardware, as in the gates of an FPGA (Field Programmable Logic Array ~= Desktop hardware development environment).
The process of defining machine code words and "high level" words is formally analagous to "axioms" and "theorems" of such formally built up mathematical systems as Euclidean geometry or Bertrand Russell and Albert North Whitehead's Principia Mathematica . Analagous questions of which axioms are essential, and whether it is necessary to add an axiom to allow a previously undefined operation to take place at all are considered in the design of the Forth dictionary. In this sense, adding a new machine code word that enables, for example, TCP/IP communications that could not be made to occur using a structure based on the previous set of machine code words is seen as adding a "TCP/IP communications axiom" to the dictionary.
Execution of a Forth program typically takes place when the user or another process requests the "execution" of a particular word. If this is a "high level" word, a mixture of tracing through the sequence of calls and sometimes reaching a machine code word and doing instructions on the "Forth Virtual Machine". Clearly, this technique can be used to great benefit when specialized hardware presents a unique set of machine code words necessary to send signals to exotic specialized processors or I/O devices. Adding the standard machine code and higher level words then provides an environment to develop additional "high level" words on top to bring out the functionality of the unique instrument.
# Forth/Introduction

Forth was first developed in the early 1970s for scientific applications by Charles Moore and Elizabeth Rather.
For computers to be useful, they must be programmed. For beginners this usually means programming in BASIC, but for professional programmers this could include:
* BASIC (considered too slow for many applications)
* Pascal
* Fortran
* C
* C++
* Ada
* Assembler (machine code – Not user friendly and limited to one CPU)
Forth is easy to start to learn, is compact and has fast execution.
This book is a complete guide to Forth programming. In this book you will be introduced to the language through examples and comparisons with BASIC. Later we will explore some of the more unusual aspects of Forth, some that have no equivalent in other languages.

## How is Forth Different

There are many statements that at first glance may seem contradictory or confusing when you are talking about Forth. For example, Forth:
* was traditionally a threaded-interpretive language, but now exists as native code systems as well
* is commonly written in Forth, but also has been written in Assembler, C and recently Python
* is extensible,
* is a high-level language with direct access to primitive operators and hardware
* is both an interpreter and a compiler (and most systems have an Assembler)
* is an easy language for absolute beginners to program in,
* is difficult to master for programmers experienced in other languages,
* is a two stack machine (data stack, return stack)
* programmer must use the data stack directly
* uses Reverse Polish Notation (RPN),
* commonly uses integer arithmetic,
* uses spaces to separate program tokens
* does not start to execute any of your typed input until after you press the enter key,
* is an interactive language, like BASIC,
* but can incrementally compile code one line at a time at the console,
* and allows (even promotes) interactive Assembly language,

## Cheat Sheet

You can quickly get a taste of Forth syntax in a LearnXinYminutes tutorial which is written in Forth!
Learn Forth in Five Minutes
This brief tutorial does not teach Forth programming "style" or the mind-set that is used to get the most out of Forth's collection of low level routines.
That is best learned by studying the code of experience Forth programmers and applying the methods to your own programming problem.