Re: Dosage of PhosChol / Biotin - ANDY / anyone?

[Guest guest]

> What is the appropriate dosage of phosphatidylcholine and Biotin for

> a 5 year old 41 lb child?

biotin 2-5 mg 3 or 4 times a day.

phosphatidylcholine 300-500 mg several times a day.

This can be obtained from 1 tsp of lecitin powder (which is soy

derived) a few times a day.

> Thanks a lot

> Sara

[Guest guest]

Andy,

Do you remember where you got that biotin dosage?

In conditions of biotinidase deficiency, where biotin cannot be recycled

(the standard situation in genetic biotin deficiency), the standard

recommendation (as repeated often below) is 10 mg a day, and the same dose

is used for valproic acid induced problems, and there doesn't seem to be

any adjustment for weight or size.

I wouldn't think an " acquired " case would require nearly as much....My

daughter's organic acid test showed problems with biotin, but not anywhere

near as bad as in biotinidase deficiency. Even so, she had the classic

signs: developmental delay, ataxia, hair loss, hypotonia, organic aciduria,

ketosis, metabolic acidosis, etc., and we have been giving less that 5 mg a

day for probably seven years. She's doing fine and doesn't have those

signs any more.

That said, I don't think anyone has ever found a toxicity with biotin.

Epilepsia. 2001 Oct;42(10):1359-62. Related Articles, Links

[Click here to read]

Low serum biotinidase activity in children with valproic acid monotherapy.

Schulpis KH, Karikas GA, Tjamouranis J, Regoutas S, Tsakiris S.

Institute of Child Health, Aghia Sophia Children's Hospital, Athens,

Greece.

PURPOSE: Valproic acid (VPA) is an effective antiepileptic drug (AED),

which is associated with dose-related adverse reactions such as skin rash,

hair loss (alopecia), etc. Profound as well as partial biotinidase

deficiency causes dermatologic manifestations similar these. Therefore, it

was of interest to evaluate serum biotinidase activity in patients

receiving VPA monotherapy. METHODS: Seventy-five patients with seizures,

mean age, 8.6 years (+/-1.9 years) were divided into three groups. Group A

(n = 25) was treated with VPA 28.7 +/- 8.5 mg/kg/24 h, group B (n = 25)

with 41.6 +/- 4.9 mg/kg/24 h, and group C with 54.5 +/- 5.8 mg/kg/24 h.

Their " trough " VPA serum levels were 40.9 +/- 13.2, 86.25 +/- 11.5, and 137

+/- 14.5 microg/ml, respectively. Fifty healthy children were the controls.

Patients and controls underwent clinical and laboratory evaluations

including liver function data, complete blood counts, NH3, and so on, after

45 days of VPA treatment. Biotinidase serum levels were evaluated

fluorometrically. RESULTS: Liver function data were found elevated in the

groups B and C. On the contrary, biotinidase activity was significantly

statistically lowered (p < 0.001) in groups B and C (1.22 +/- 1.11, 0.97

+/- 0.07 mmol/min/L respectively), as compared with controls (5.20 +/- 0.90

mmol/min/L). Strong inverse correlations were observed between liver

enzymes and VPA blood levels with the activity of the enzyme. Additionally,

no inhibitory effect on biotinidase activity was found, when the enzyme was

incubated in vitro with high (1.2 mM) concentrations of the drug. Skin

lesions (seborrheic rash, alopecia) were improved in our patients after

biotin (10 mg/day) supplementation. CONCLUSIONS: It is suggested that VPA

impairs the liver mitochondrial function, resulting in a low biotinidase

activity and or biotin deficiency. Biotin supplementation could restore

some of the side effects of the drug.

Publication Types:

* Clinical Trial

PMID: 11737173 [PubMed - indexed for MEDLINE]

Int J Vitam Nutr Res. 1997;67(5):377-84. Related Articles, Links

Multiple carboxylase deficiency: inherited and acquired disorders of

biotin metabolism.

Baumgartner ER, Suormala T.

University Children's Hospital, Metabolic Unit, Basel, Switzerland.

Acquired biotin deficiency and the two known congenital disorders of

biotin metabolism, biotinidase and holocarboxylase synthetase (HCS)

deficiency, all lead to deficiency of the 4 biotin-dependent carboxylases,

i.e. to multiple carboxylase deficiency (MCD). The underlying mechanism in

HCS-deficiency, discovered in 1981, is decreased affinity of HCS for biotin

impairing the formation of holocarboxylases at physiological biotin levels.

In biotinidase deficiency, discovered in 1983, MCD results from progressive

development of biotin-deficiency due to inability to liberate and recycle

biotin which is lost in urine as biocytin. MCD leads to typical organic

aciduria and severe life-threatening illness. Main symptoms and signs are

feeding difficulties, neurologic abnormalities (hypotonia, impaired

consciousness, seizures, ataxia) and cutaneous changes (rash, alopecia).

However, the clinical presentation and age of onset are extremely variable,

and organic aciduria may initially be absent in biotinidase deficiency.

Therefore, the definitive diagnosis requires enzyme studies. MCD can be

detected in lymphocytes obtained before treatment and biotinidase

deficiency is confirmed or excluded by a colorimetric enzyme assay in

plasma. Newborn screening for biotinidase deficiency has resulted in the

detection of patients with partial deficiency (10-30% of mean normal

activity) in addition to patients with profound deficiency (0-10%). Severe

illness has been observed mainly in patients with O-activity or a

Km-mutation, detection of which requires detailed investigation.

HCS-deficiency has to be confirmed by enzyme assay in cultured cells. Both

congenital disorders respond clinically and biochemically to oral biotin

therapy. Whereas 10 mg/day or less is sufficient to treat profound

biotinidase deficiency, the optimal biotin dose for patients with

HCS-deficiency must be assessed individually. The prognosis of both

disorders is good if biotin therapy is introduced early and continued

throughout life. However, delayed commencement of therapy in biotinidase

deficiency can result in irreversible neurological damage, and in

HCS-deficiency a few patients have responded only partially even to massive

biotin doses of up to 100 mg/day.

Publication Types:

* Review

* Review, Tutorial

PMID: 9350481 [PubMed - indexed for MEDLINE]

Monatsschr Kinderheilkd. 1989 Nov;137(11):737-40. Related Articles, Links

[biotinidase deficiency: a congenital metabolic disease which can be

successfully treatment with vitamin H]

[Article in German]

Nothjunge J, Krageloh-Mann I, Suormala TM, Baumgartner ER.

Abteilung Allgemeine Padiatrie, Universitats-Kinderklinik Tubingen.

At the age of 13 months a patient developed muscular hypotonia,

deafness of the inner ear and cutaneous symptoms (alopecia; skin rash,

complicated by superinfection with monilia). Biochemical assays revealed

compensated metabolic acidosis, pathologically high lactate and pyruvate

concentrations in the blood and cerebro-spinal fluid, as well as increased

urinary excretion of 3-OH-isovaleric acid, 3-methylcrotonylglycine and

lactate. The patient was diagnosed as suffering from autosomal recessive

biotinidase deficiency on the basis of severely reduced biotinidase

activity in plasma (0.05 nmol/min/ml). In both his parents and brother

heterozygosity was found. Institution of therapy with a daily dose of 10 mg

biotin rapidly removed most of the symptoms; after six months of treatment

the deafness had improved significantly.

PMID: 2608075 [PubMed - indexed for MEDLINE]

Neuropediatrics. 1993 Apr;24(2):98-102. Related Articles, Links

Recovery from neurological deficits following biotin treatment in a

biotinidase Km variant.

Ramaekers VT, Brab M, Rau G, Heimann G.

Department of Pediatrics, Ophthalmology and Psychological

Medicine-Technical University of Aachen, Germany.

A 15-year-old boy suffered from progressive bilateral optic neuropathy

of acute onset at the age of 10 years. Subsequently he developed spastic

paraparesis and a predominantly motor type neuro-axonal neuropathy in all

limbs. The basic error has been elucidated to be due to an unusual

biotinidase Km variant with biphasic enzyme kinetics causing systemic

biotin depletion and consequent multiple biotin-dependent carboxylase

deficiency. After daily oral substitution with 10 mg biotin metabolic

derangements subsided rapidly. Follow-up studies over one year after

substitution with biotin demonstrated remarkable recovery from part of the

previously present neuro-ophthalmological, motor and cognitive deficits.

The previously extinguished flash-evoked visual potentials now showed clear

responses after six months of substitution with biotin. In contrast with

reports in literature, these findings indicated that neurological damage

associated with biotinidase deficiency, rather than being permanent, is to

some extent reversible.

PMID: 8352834 [PubMed - indexed for MEDLINE]

At 06:41 AM 7/1/2003 +0000, you wrote:

> > What is the appropriate dosage of phosphatidylcholine and Biotin for

> > a 5 year old 41 lb child?

>

>biotin 2-5 mg 3 or 4 times a day.

>

>phosphatidylcholine 300-500 mg several times a day.

>

>This can be obtained from 1 tsp of lecitin powder (which is soy

>derived) a few times a day.

>

> > Thanks a lot

> > Sara

>

>

>

>=======================================================

>

[Guest guest]

> Andy,

>

> Do you remember where you got that biotin dosage?

It is commonly known among supplement sellers and prescribers that

5-10 mg qid is what actually works for people with, e. g. elevated

pyruvate. This is certainly in line with experience.

> In conditions of biotinidase deficiency, where biotin cannot be

recycled

> (the standard situation in genetic biotin deficiency), the standard

> recommendation (as repeated often below) is 10 mg a day, and the

same dose

> is used for valproic acid induced problems, and there doesn't seem

to be

> any adjustment for weight or size.

I think it is foolish to go to essentiallly allopathic sources for

proper dosing and administration on a nutritional supplement.

> I wouldn't think an " acquired " case would require nearly as

much....My

> daughter's organic acid test showed problems with biotin, but not

anywhere

> near as bad as in biotinidase deficiency. Even so, she had the

classic

> signs: developmental delay, ataxia, hair loss, hypotonia, organic

aciduria,

> ketosis, metabolic acidosis, etc., and we have been giving less that

5 mg a

> day for probably seven years. She's doing fine and doesn't have

those

> signs any more.

>

> That said, I don't think anyone has ever found a toxicity with

biotin.

Yup. So the only mistake one can make is to not use enough. So stick

with what is well known to work - 5-10 mg 4 times a day.

>

, you really don't have to troll up a bunch of medline abstracts

and append them to the ends of your posts. All that makes me think is

that you aren't busy enough and need more to do - they are NOT

informative or useful. If you were actually reading the papers and

making cogent comments on the few that are relevant it would be

another thing.

Andy . . . . . . . . . . .

[Guest guest]

>

>

>

>, you really don't have to troll up a bunch of medline abstracts

>and append them to the ends of your posts. All that makes me think is

>that you aren't busy enough and need more to do - they are NOT

>informative or useful. If you were actually reading the papers and

>making cogent comments on the few that are relevant it would be

>another thing.

>

>Andy . . . . . . . . . . .

>

>

>

It may not help Andy but it helps me. You are probably better at finding

things of relevance than I would be on my own.

That said, I honestly dont always read all of every one posted but

appreciate your efforts.

Ive seen FAR worse examples of bandwidth suck here in the past.

[Guest guest]

Andy,

>, you really don't have to troll up a bunch of medline abstracts

>and append them to the ends of your posts. All that makes me think is

>that you aren't busy enough and need more to do - they are NOT

>informative or useful. If you were actually reading the papers and

>making cogent comments on the few that are relevant it would be

>another thing.

You're absolutely right that I don't have to. But, I think it is important

for people to know where the information came from, and I have a lot of

respect for the work of the people who wrote the articles.

Well, yes, I am plenty busy, Andy. I have in the last week copied more

than 400 pages worth of new articles (not abstracts, but original articles)

which I'm already busy reading...They are all about areas that have fallen

through the cracks in models of the sulfur chemistry, and their relevancy

to interactions with other biochemistry...even chelation Getting into

these areas was suggested by the experiences of children with autism under

various therapy and because of knowledge of the amino acid patterns in

some. However, most of it was inspired by issues raised at the last DAN!

conference, which really pointed out how many black holes there are in our

understanding of how certain vitamin pathways intersect with sulfur

trafficking issues. I'm finding an awful lot of new things have been

characterized, but haven't been noticed, so on a practical level, they have

been useless. That's what I go after in hopes of making it useful.

It is my usual habit to get the original articles I reference, and that

often happens before I say anything about them on the net, but it

definitely happens before I include them as references in academic papers.

I've gotten to be more of a stickler about that as I've gone along, except

for papers written in languages I can't read or locate...where I still

depend on the abstract if I cannot find the information in another

article. When I get the paper, I check out the procedures and the

viability of the argument that leads to whatever conclusion. Many times

the conclusion stated in the abstract is said with more certainty than what

was defended in the paper, and other times, it was obvious that the author

didn't know of another logical explanation for his data because he was

ignorant of the same work completed in another field. I generally find

hidden within papers genuine nuggets of information that were too obscure

to mention in the abstracts. These generally end up in the " this didn't

fit " comments that the authors stated when they didn't understand it

because of other literature they hadn't read. Those rare nuggets often put

all the other pieces together, and that's why I keep looking so hard at the

nitty gritty details.

In graduate school, I spent a lot of time learning how to study papers

critically. Over the four years I was there, I audited three different

discussion courses for senior biology majors (not available in the graduate

school curriculum) but they were taught by different professors covering

different subject matter and were called " Seminal papers in

biology " . During these courses, all we did was read and critically

evaluate papers that had changed scientific views on various subjects. We

reviewed their criticisms as well from other workers, partiularly focussing

on reading papers that had made their data not accepted at first. The

focus was on understanding the scientific process. I also took another

course that was in my graduate school curriculum whose purpose was to train

us how to recognize junk science. I had great fun with that course,

especially dissecting mainstream autism papers! But, in the regular sort

of courses I took in biology and neuroscience, I found that some of my

curriculum was decades outdated. The professors did not realize these

omissions until I brought to them the papers which (compared with each

other and to what we were taught) made it obvious what should be changing

in the curriculum.

In yet another course, I did a term paper aimed at tracing the money flow

into research from various avenues and trying to find out about the time

and effort and basic science that goes into product development for

pharmaceuticals. In the process, I Iearned how and why materials make it

into graduate school and medical school curricula, and that had a lot to do

with where the money went that directed attention one way or another. This

ended up a study of the " politics " of science. I learned that it is the

" star scientists " that write the textbooks, and they just include what they

personally have found to be interesting and relevant, and assume it is OK

to ignore the rest. These choices maintain their control of the body of

infromation in the field, a priviledge they enjoy. Unfortunately, an awful

lot of valuable work gets overlooked and abandonned because it wasn't one

of their own " hot topics " . After all, they were the ones making decisions

behind the scenes (in peer review) that decided what research received

money for further research efforts. Because of this process, a lot of good

research that could have helped people has died on the vine without funding

or notice...ideas based on excellent research...but which when they were

written, were not in the areas of interest of the current star scientists.

Since you mentioned the health food industry, you may not be aware that

about twenty years ago, I spent quite a few years working in that

business. I've always been a sceptic at heart, so even back then, I

wondered at the scientific basis of the information I was learning in that

industry. I didn't " buy " what I was taught without first checking out the

basis and their sources first, so I spent six years reading the literature

back then. That was pre-parenthood and pre-graduate school. But, even

then, I wanted to know how much was real and how much was sales hype and

not scientific at all. During those years, I saw a lot of hot items come

in and go out, and I still go back and visit so I can listen to hear what

is being said today to the same sort of customer. Anyway, Andy, the study

I did back then was a great background for my later academics and gave me a

patient-centered and unique perspective in graduate school.

But, for the discussion of biotin, reading those papers wasn't necessary,

because all I was trying to do is verify if there had been any changes in

what doctors recommend since I had done that research years ago to help my

daughter. The recommendations are still pretty much the same....and not

particularly " scientific " either since no attempt was made to adjust the

dose dependent on the size of the patient.

We certainly didn/t see a sudden positive response to biotin as we did to

g/f c/f diet and to epsom salts therapy, but gradually, the things that

were associated with biotin deficiency improved and went away. If that is

true of other biotin deficiency recoveries, it would be hard to guage what

the dose should be without upping the dose until the carboxylase deficiency

went away. That is the objective way to do it which has been used in

academic settings, sort of akin to monitoring heavy metal excretions during

chelation or fungal metabolites on antifungal medication. But, when they

did this, they found a lot individuality to that magic quantity of biotin.

[Guest guest]

> Andy,

>

> >, you really don't have to troll up a bunch of medline

abstracts

> >and append them to the ends of your posts. All that makes me think

is

> >that you aren't busy enough and need more to do - they are NOT

> >informative or useful. If you were actually reading the papers and

> >making cogent comments on the few that are relevant it would be

> >another thing.

>

> You're absolutely right that I don't have to. But, I think it is

important

> for people to know where the information came from, and I have a lot

of

> respect for the work of the people who wrote the articles.

Simply citing them in the academic style is adequate for that purpose.

Does this imply you have read every article you post an abstract for?

Or simply that you have a lot of respect for people who manage to get

anything indexed in medline and wish to honor them by reproducing it?

Being busy reading the articles hopefully you will soon be discussing

what some of them actually say when that is different than what the

abstract suggests, as I often find to be the case with the most

relevant and informative articles.

> Well, yes, I am plenty busy, Andy. I have in the last week copied

more

> than 400 pages worth of new articles (not abstracts, but original

articles)

> which I'm already busy reading...They are all about areas that have

fallen

> through the cracks in models of the sulfur chemistry, and their

relevancy

> to interactions with other biochemistry...even chelation Getting

into

> these areas was suggested by the experiences of children with autism

under

> various therapy and because of knowledge of the amino acid patterns

in

> some. However, most of it was inspired by issues raised at the last

DAN!

> conference, which really pointed out how many black holes there are

in our

> understanding of how certain vitamin pathways intersect with sulfur

> trafficking issues. I'm finding an awful lot of new things have

been

> characterized, but haven't been noticed, so on a practical level,

they have

> been useless. That's what I go after in hopes of making it useful.

>

> It is my usual habit to get the original articles I reference, and

that

> often happens before I say anything about them on the net, but it

> definitely happens before I include them as references in academic

papers.

> I've gotten to be more of a stickler about that as I've gone along,

except

> for papers written in languages I can't read or locate...where I

still

> depend on the abstract if I cannot find the information in another

> article. When I get the paper, I check out the procedures and the

> viability of the argument that leads to whatever conclusion. Many

times

> the conclusion stated in the abstract is said with more certainty

than what

> was defended in the paper, and other times, it was obvious that the

author

> didn't know of another logical explanation for his data because he

was

> ignorant of the same work completed in another field. I generally

find

> hidden within papers genuine nuggets of information that were too

obscure

> to mention in the abstracts. These generally end up in the " this

didn't

> fit " comments that the authors stated when they didn't understand it

> because of other literature they hadn't read. Those rare nuggets

often put

> all the other pieces together, and that's why I keep looking so hard

at the

> nitty gritty details.

>

> In graduate school, I spent a lot of time learning how to study

papers

> critically. Over the four years I was there, I audited three

different

> discussion courses for senior biology majors (not available in the

graduate

> school curriculum) but they were taught by different professors

covering

> different subject matter and were called " Seminal papers in

> biology " . During these courses, all we did was read and critically

> evaluate papers that had changed scientific views on various

subjects. We

> reviewed their criticisms as well from other workers, partiularly

focussing

> on reading papers that had made their data not accepted at first.

The

> focus was on understanding the scientific process. I also took

another

> course that was in my graduate school curriculum whose purpose was

to train

> us how to recognize junk science. I had great fun with that course,

> especially dissecting mainstream autism papers! But, in the regular

sort

> of courses I took in biology and neuroscience, I found that some of

my

> curriculum was decades outdated. The professors did not realize

these

> omissions until I brought to them the papers which (compared with

each

> other and to what we were taught) made it obvious what should be

changing

> in the curriculum.

>

> In yet another course, I did a term paper aimed at tracing the money

flow

> into research from various avenues and trying to find out about the

time

> and effort and basic science that goes into product development for

> pharmaceuticals. In the process, I Iearned how and why materials

make it

> into graduate school and medical school curricula, and that had a

lot to do

> with where the money went that directed attention one way or

another. This

> ended up a study of the " politics " of science. I learned that it

is the

> " star scientists " that write the textbooks, and they just include

what they

> personally have found to be interesting and relevant, and assume it

is OK

> to ignore the rest. These choices maintain their control of the

body of

> infromation in the field, a priviledge they enjoy. Unfortunately,

an awful

> lot of valuable work gets overlooked and abandonned because it

wasn't one

> of their own " hot topics " . After all, they were the ones making

decisions

> behind the scenes (in peer review) that decided what research

received

> money for further research efforts. Because of this process, a lot

of good

> research that could have helped people has died on the vine without

funding

> or notice...ideas based on excellent research...but which when they

were

> written, were not in the areas of interest of the current star

scientists.

>

> Since you mentioned the health food industry, you may not be aware

that

> about twenty years ago, I spent quite a few years working in that

> business. I've always been a sceptic at heart, so even back then, I

> wondered at the scientific basis of the information I was learning

in that

> industry. I didn't " buy " what I was taught without first checking

out the

> basis and their sources first, so I spent six years reading the

literature

> back then. That was pre-parenthood and pre-graduate school. But,

even

> then, I wanted to know how much was real and how much was sales hype

and

> not scientific at all. During those years, I saw a lot of hot items

come

> in and go out, and I still go back and visit so I can listen to hear

what

> is being said today to the same sort of customer. Anyway, Andy, the

study

> I did back then was a great background for my later academics and

gave me a

> patient-centered and unique perspective in graduate school.

>

> But, for the discussion of biotin, reading those papers wasn't

necessary,

> because all I was trying to do is verify if there had been any

changes in

> what doctors recommend since I had done that research years ago to

help my

> daughter. The recommendations are still pretty much the same....and

not

> particularly " scientific " either since no attempt was made to adjust

the

> dose dependent on the size of the patient.

>

> We certainly didn/t see a sudden positive response to biotin as we

did to

> g/f c/f diet and to epsom salts therapy, but gradually, the things

that

> were associated with biotin deficiency improved and went away. If

that is

> true of other biotin deficiency recoveries, it would be hard to

guage what

> the dose should be without upping the dose until the carboxylase

deficiency

> went away. That is the objective way to do it which has been used

in

> academic settings, sort of akin to monitoring heavy metal excretions

during

> chelation or fungal metabolites on antifungal medication. But, when

they

[Guest guest]

Andy,

At 09:24 PM 7/4/2003 +0000, you asked:

>Does this imply you have read every article you post an abstract for?

Oh, no. I wish I could do that, and I suppose if I lived at the library I

could manage it, but it is quite a long drive, and I don't get to the

library with that sort of frequency. In this area, it hurts me that I am no

longer a student and not employed by a university so that I could log on

remotely to get articles. But I do go back and try to print all the

abstracts I have referred to in list posts and work on getting a copy, if

possible. This year, however, I'm pretty far behind in that effort due to

some major changes in office and home.

>Or simply that you have a lot of respect for people who manage to get

>anything indexed in medline and wish to honor them by reproducing it?

I'm really happy to find people who have done relevant work in basic

science. When I find something very helpful, if I can, I try to contact

them and introduce them to the issues in autism and see if they can make

any comments about my reading of their work and about the issues that

struck me in their papers. Some people don't write back after the initial

contact, but others have been very helpful and interested. Often enough, I

have something useful to them to share because of other readings that they

may have missed. So it really works to do this. I read a lot of articles

that aren't indexed, too, because I find them in reference sections of

papers, so I pay a lot of attention to the bibliography. In fact, because

it is in the references where I find a lot of gems, I end up reading a lot

of papers that are OLD and no longer indexed. I'm also registered to

receive abstracts from journals that aren't in medline, but I usually don't

have much time to look them over, because they aren't indexed. It is an

odd sort of education, for sure.

>Being busy reading the articles hopefully you will soon be discussing

>what some of them actually say when that is different than what the

>abstract suggests, as I often find to be the case with the most

>relevant and informative articles.

Yes, I share with you the experience of finding better things in the

articles, so I hope I will find some goodies worth sharing. But be patient

with me, because especially when I'm in new turf for myself, I do a lot of

reading in a topic first to make sure I understand what the issues are

understood to be, and where people don't agree. I don't always post the

general autism/sulfur stuff here since this is a list about chelating

mercury, but I do try to copy the ones to this list when they seem relevant

to the topic.

.