IL CASO DI DANTE

In Italia e in Europa, a differenza degli USA e di altri stati industrializzati (tra cui il Canada),
la legislazione in materia di Malattie Rare è veramente di notevole pregio;
l'intenzione del legislatore, in Europa e in Italia è di porre L'UOMO davanti a TUTTO
(questa è la percezione) !

Mentre negli USA e in moltissimi altri stati evoluti (tra cui il Canada), pare che prima dell'UOMO....
ci siano interessi più forti che poco hanno a che fare con i diritti dell'UOMO.

Ci chiediamo : è lecito, in uno Stato Democratico,
considerare una Malattia Rara come un problema limitato a una Minoranza di popolo ?
Rispondiamo : NO.....è FOLLIA !

Su queste cose ....a nessuno deve essere dato il DIRITTO di decidere sulla vita degli uomini !
Queste cose sono parte della Scienza....e laddove la scienza non arriva:
c'è l'unico spazio possibile per discuterne: la FEDE in Dio !

LA SCIENZA convive male con la politica e con la democrazia;
la SCIENZA ha regole in continua trasformazione.....[ogni giorno] !!!
......................

Teorie considerate sino ad oggi "Dogmi": diventano all'improvviso INEZIE ...nel giro di alcuni mesi,
Teorie considerate sino ad oggi "FALSE" diventano all'improvviso DOGMI ...nel giro di alcuni mesi.
Questo non è solo un problema di Maggioranza o Minoranza.

Ed è per questo che in POLITICA, quando si decide su argomenti come: MEDICINA, SCIENZA ecc..
non è LOGICO ragionare da politici....
ma è indispensabile ragionare da Uomini: Padri, Madri, Figli, Fratelli, Amici ecc..!

Una Malattia Rara....è UNA MALATTIA .....e BASTA !
Vogliamo DEMOLIRE il termine MALATTIA RARA,
e ridurla al termine corretto di MALATTIA !

Si consideri anche, che i genitori di Enea sono perfettamente consapevoli che ESISTE....
una terapia che porta alla guarigione della L.C.
che si basa sull'utilizzo di un emoderivato che si chiama PLASMINOGENO,
ma che non può essere effettuata facilmente per "problemi etico/normativi".....
e anche per questione di soldi, o forse solo per questo.

By Enea's Father 27 settembre 2008
__________________________________________________________________________________________________________________

Questa pagina è dedicata alla madre di Dante [Margie Di Danieli].
Tra i documenti che seguono c'è la sua lettera;
una lettera ......che è una richiesta di aiuto per il proprio figlio affetto da una malattia rara [Ligneous Conjunctivitis],
e per cui non esiste in commercio IL GIUSTO FARMACO [PLASMINOGENO].

Dante, il figlio di Margie [che ha 7 anni] soffre della stessa malattia rara.... di Enea: [Ligneous Conjunctivitis] !

Margie ha spedito qualche anno fà, questa sua bellissima lettera di denuncia a molte istituzioni internazionali in tutto il pianeta ......
che POSSONO.... E DEVONO risolvere questo GRAVISSIMO problema ! Con scienza, conoscenza e ...umanità !
Grandi Stati come il CANADA e l'Italia, DEVONO dare l'esempio agli altri Stati.....e sono certo che lo faranno !
LO FARANNO ! Il Plasmonogeno deve diventare un FARMACO .....dispopnibile a chiunque ne abbia bisogno !
Il PLASMINOGENO è un emoderivato indispensabile per la vita di moltissime persone...!

Margie ha voluto consegnare anche a noi questa Sua Lettera: a noi, genitori di Enea;
e noi siamo onorati per questa scelta.
Il caso di Dante è identico al caso di Enea;
una grande madre del Canada combatte [da sola] la nostra stessa battaglia per ottenere il plasminogeno !
Suo figlio Dante ha bisogno del PLASMINOGENO...per guarire !
QUESTA E' L'UNICA CERTEZZA.
E' per questo che abbiamo deciso di aggiungere alla nostra causa, la causa di Dante.... e farne un' UNICA CAUSA.

La ns. Organizzazione spontanea nata su Internet.....per il Caso di Enea: è una goccia nell'oceano, ma stiamo lavorando sodo !
Abbiamo già provveduto a indicizzare le nostre pagine e il ns. Data-Base On-Line:
(http://www.budoniambiente.org/meyer)
presso centinaia di motori di ricerca nel mondo: i risultati sono già visibili (nonostante siamo solo all'inizio);
il ns. sito (http://www.budoniambiente.org/meyer) si colloca tra i primi 10 / 20 nei maggiori motori di ricerca internazionali,
con moltissime key-word riferite alle informazioni sulla Congiuntivite Lignea (Ligneous Conjunctivitis) e argomenti correlati.
Stiamo lavorando per ampliare i contenuti del Data-Base e per "limare" una indicizzazione più efficace)
non escludiamo la programmazione di un FORUM specifico e altre iniziative di grande interesse.
Siamo certi che il ns. contributo non andrà perso !

THE PETITION, by Dante's mother

PETITION TO THE HOUSE OF COMMONS IN PARLIAMENT ASSEMBLED
WE THE UNDERSIGNED ALL CITIZENS AND RESIDENTS OF CANADA
RESPECTFULLY WISH TO BRING TO YOUR ATTENTION THE FOLLOWING FACTS:
- http://www.ipetitions.com/petition/orphandrugact-dantedinodidanieli/

LA LETTERA DI MARGIE DI DANIELI

The reasons he has been an out-patient since May, 2003 are as follows: (as per the abstract from The New England Journal of Medicine the little girl pictured at this website has the same condition as Dante does, but hers was an entirely different mutation with much more severe complications” http://content.nejm.org/cgi/content/full/339/23/1679 ):

“Ligneous conjunctivitis is a rare disease characterized by acute or chronic recurrent conjunctivitis in which the conjunctival membranes acquire a wood-like consistency, due primarily to deposits of fibrin. 1 , 2 Corneal involvement and chronic obstruction of the eye may lead to blindness. The disease is frequently associated with nasopharyngitis, tracheobronchial obstruction, otitis media, vulvovaginitis, and defective wound healing. 2 , 3 , 4 , 5 , 6 , 7 , 8 , 9 Pseudomembranous conjunctivitis was first described in 1847 by Bouisson, 10 and the term "conjunctivitis lignosa" was introduced by Borel in 1933. 11 More than 100 cases have been reported in the literature, but no satisfactory treatment has yet been found. The results of therapy with hyaluronidase eye drops, corticosteroids, cyclosporine, and antiviral agents are generally disappointing. 6 , 7 , 12 , 13 , 14 Surgical treatment often causes accelerated recurrence of pseudomembranes. 2 , 4 , 5 , 6 , 14

Family studies have suggested that the disorder is caused by a genetic defect with an autosomal recessive pattern of inheritance. 3 A case of ligneous conjunctivitis with gingival and peritoneal lesions was reported after treatment with the antifibrinolytic agent tranexamic acid, 15 suggesting a relation between ligneous conjunctivitis and impaired fibrinolysis. Although the syndrome may have more than one cause, it has recently been linked to severe plasminogen deficiency . 16 , 17 , 18 Plasminogen - deficient mice generated by targeted gene disruption show signs of ligneous conjunctivitis, defective wound healing, and internal hydrocephalus. 19 , 20

In this report, we describe a patient with homozygous plasminogen deficiency in whom ligneous conjunctivitis developed soon after birth. Other symptoms included hyperviscosity of tracheobronchial and nasopharyngeal secretions, impaired wound healing, and internal hydrocephalus. Replacement therapy with lys - plasminogen (lysine-conjugated plasminogen ) led to rapid regression of the pseudomembranes and normalization of respiratory tract secretions and wound healing.”

I have cited the “CBER Transcript – Biological Therapeutics for Rare Plasma Protein Disorders” Minutes at http://www.fda.gov/cber/minutes/biother061305t.htm and http://www.fda.gov/cber/minutes/biother061405t.htm which delve deeply into the need for more intensive research into, and access to Biological Therapeutics for Rare Plasma Protein Disorders amongst other pertinent information relating to this deficiency. I have also cited a website documenting almost 50 cases in direct relation to this deficiency at http://www.budoniambiente.org/meyer/default.asp .

I have taken the liberty of contacting Health Canada and have had the pleasure of conversing with members of the medical and scientific professions in the Special Access Programme. Due to the fact that Human Plasminogen is classified as an Orphan Drug, they stated that Health Canada would certainly grant special access in this matter, as long as the source for Human Plasminogen was identified and verified as viable, licensed, and approved for human use . As an aside, Human Plasminogen is not available in Canada so I have been attempting to source Human Plasminogen in the United States of America, Europe, and internationally.

Just yesterday morning (August 22, 2006), I spoke with a another doctor at Health Canada and he said that Canada does not have an Orphan Drug Policy/Act in place, that any sourced Plasminogen must be licensed and from an industrialized country, and that they do not (Health Canada) manufacture Plasminogen nor do they have the facilities to do so. As well, the fact that the only provision I found on the internet was that the Special Access Programme is supported by sections C.08.010 and C.08.011 of the Food and Drug Regulations and is intended to provide compassionate access to drugs on a patient-by-patient basis at http://www.hc-sc.gc.ca/dhp-mps/acces/drugs-drogues/sapfs_pasfd_2002_e.html .

In addition, I recently had the pleasure of speaking with a nurse at the Canadian Blood Services (1-888-236-6283) and she was kind enough to gather and transmit a request electronically to the doctors at Canadian Blood Services, on our behalf. She then suggested I follow-up by sending my own request electronically to Canadian Blood Services directly which I have done. As per my conversation of August 22, 2006 with a Doctor at Canadian Blood Services, this is something that must be done collectively by Health Canada 's Special Access Programme and The Hospital for Sick Children in Toronto .

Please allow me to recap the last 3-1/2 + years in a few paragraphs which will provide greater insight into what has transpired to date, as follows:

Dante – Brief Medical History

In May of 2003, lesions/eruptions with red and yellow pus-like discharge began appearing and emanating from both of Dante's eyes, which were visible and obvious to the naked eye. Upon our visit with his paediatrician, and there not being a conclusive and/or definitive determination as to the cause nor the name of his ailment, Dante was referred to Sick Children's Hospital in downtown Toronto . I brought him to the emergency room at Sick Kids and several nurses attempted to swab the discharge from his eyes and begin an IV. We stayed overnight and Dante was discharged the following day by the resident eye-doctor on call that, in turn, referred us to the Eye Clinic at Sick Kids in Toronto .

We attended our appointment at the Sick Kids Eye Clinic with the Ophthalmology specialist who diagnosed Dante as having acquired Chelazion and suggested surgical removal, due to concerns with respect the Chelazion growth affecting his vision. The Chelazion were successfully removed, but 2-3 weeks later a thick growth started to appear above and below his eyelids and grew rapidly, eventually making his irises and surrounding white area almost non-existent. I personally had day-in and day-out visual of his exhibiting mannerisms and head movements, which were akin to those of a blind person.

Dante was referred to another Ophthalmology specialist at the Eye Clinic. Upon another visit to the emergency department and subsequently to the Eye Clinic at Sick Kids, it was determined that another operation would be necessary to remove the growth on top of and beneath his eyelids. If my recollection serves me correctly, and immediately after the second surgery, the growth was analyzed and determined to be bilateral conjuctival psuedomembranes, in essence, Ligneous Conjunctivitis. Immediately following the second surgery by the Ophthalmology specialist, topical Tobradex and topical Heparin were prescribed and administered daily to keep the fibrin from growing further.

Dante was subsequently referred to the Oncology/Haematology Clinic at Sick Kids for complete blood work assessment and the results were conclusive, ligneous conjunctivitis - a direct result of Human Plasminogen deficiency. The protocol of administering topical Heparin and Tobradex continued for quite some time and eventually their use was discontinued due to the growth not exhibiting further fibrin deposit accumulation. At this point in time Dante has slight fibrin accumulation under the right eyelid, which I believe is growing slowly thereby proving that Human Plasminogen is the only biological answer to resolving his deficiency.

At my request, the Ophthalmology Specialist was kind enough to refer us to the Genetic and Metabolic Clinic at Sick Kids and we recently attended same for Dante's full assessment by a Genetic Specialist. We discussed the genetic make-up of Human Plasminogen deficiency and due to his concerns as well as my own, he recommended an MRI of the brain, and hopefully the spine and organs can be imaged during that visit. The reasons I feel his spine and organs should be imaged are due to the clinical manifestations of this disease, which may not be visible to the naked eye. I feel that in-depth genetic testing is a viable option to correctly analyze the genetic sequence and or specific genetic mutation of this deficiency. Moreover, to conclusively and definitively establish whether Dante has Type I or Type II Plasminogen deficiency, his pre-disposition to other medical complications from this rare disease, as well as any mutations that may indicate future manifestations of this deficiency.

The MRI Clinic has since contacted me and questioned me on whether Dante should be awake or asleep during the MRI. I chose the latter due to the fact that Dante is not one to sit still for more than a minute, as is the case with most children, and the fact that I cannot predict his behaviour in reaction to his surroundings on that particular day. As well, it is my understanding that a team of specialists need to be there if the MRI is done while a patient is asleep. Also, the Ear-Nose-Throat Clinic at Sick Kids has since notified me of his upcoming appointment in October, 2006.

Dante and I attended an appointment at the Eye Clinic which was scheduled for Wednesday, August 23, 2006 at 9:30 a.m. , to meet and discuss these issues in greater depth with his Ophthalmologic Specialist. I will advise of any further developments with respect to same.

Research – Plasminogen Deficiency
My research has led me to believe that fibrin growths or deposits could migrate to many areas of Dante's body and as such I have listed medically documented clinical manifestations of this deficiency:

•  membrane formation in the pharynx, renal collecting system (nodular calcified masses), gingival (hyperplasia) , laryngotracheobronchial tree, and vocal chords

•  duodenal ulceration

•  eosinophilic gastric infiltration

•  asthma-like symptoms

•  pneumomediastinum necessitating a bronchoscopy

•  abscess of the lungs necessitating bronchoscopic drainage

•  tracheobronchial hyperviscosity

•  nasopharyngeal secretions

•  impaired wound healing

•  hearing deficiency at the middle-ear level (verified ligneous changes of the middle ears)

•  hypoplasia of the cerebellum

•  hypoplastic corpus callosom

•  congenital hydrocephalus

To name but a few of the clinical manifestations of this disease, which have instilled a fear of the unknown or what is to come with respect to Dante's future and existence, that may be unfounded, but do exist nonetheless. He deserves, at the very least, a collective endeavour ensuring that his health, well-being, and best interests be taken into consideration.

As an aside, this could have happened due to the mechanism by which a certain etiological factor causes disease -- pathos = disease, genesis = development-- via invasiveness and/or toxigenesis. Pathogens usually colonize host tissues that are in contact with the external environment. Sites of entry in human hosts include the urogenital tract, the digestive tract, the respiratory tract and the conjunctiva. Streptokinase and Staphylokinase are produced by streptococci and staphylococci, respectively. Kinase enzymes convert inactive Plasminogen to plasmin which digests fibrin and prevents clotting of the blood. The relative absence of fibrin in spreading bacterial lesions allows more rapid diffusion of the infectious bacteria (mechanisms of bacterial pathogens). I believe the answer to this would be in conducting further genetic/metabolic testing which would verifiably pinpoint the etiological factor of this deficiency.

1. Who is affected? – Dante Dino Di Danieli - dob December 30, 2000, age 5-1/2, Height: 45-1/2 inches, Weight: 82 lbs.

2. What is the definitive cause? – Human Plasminogen Deficiency (Coagulation Cascade) (heterozygous v. homozygous) (type I v. type II) (gram-positive v. gram-negative)

3. Why did this happen? – Genetic inheritance or Pathogenesis - the mechanism by which a certain etiological factor causes disease -- pathos = disease, genesis = development-- via invasiveness and/or toxigenesis. Pathogens usually colonize host tissues that are in contact with the external environment. Sites of entry in human hosts include the urogenital tract, the digestive tract, the respiratory tract and the conjunctiva. Streptokinase and Staphylokinase are produced by streptococci and staphylococci, respectively. Kinase enzymes convert inactive Plasminogen to plasmin which digests fibrin and prevents clotting of the blood. The relative absence of fibrin in spreading bacterial lesions allows more rapid diffusion of the infectious bacteria (mechanisms of bacterial pathogens).

4. Where does the problem stem from? – Chromosome 6 or Chromosome 6q26 and 6q27 respectively - Family studies have suggested that the disorder is caused by a genetic defect with an autosomal recessive pattern of inheritance.

5. When did this happen? – at conception or on or about May, 2003 wherein something triggered this medical anomaly (further genetic testing needed to verify genetic sequence of mutation)

6. How to solve? – The source for Human Plasminogen via topical application or via therapeutic intravenous administration

CONCLUSION

I am profoundly moved and humbled by all this medical attention bestowed upon my son, but it is attention that a parent can never really envision, predict, or imagine. Nevertheless, I am eternally grateful for all the help that Dante has received thus far and hold all the doctors and specialists whom we have met at Sick Kids in the highest conceivable regard. They all have a unique and special gift used to help people affected by rare diseases. I know for a fact that the majority of doctors take their Hippocratic Oath very seriously and I for one have great faith and belief in them.

I truly believe that all of this research has left me with but one conclusion…to fully understand, appreciate, and investigate an anomaly such as this; one must be of an inquisitive mind and explore all possible avenues available to them. My research of this topic will be never-ending until such time that Dante is free from this disease. From all that I have read, researched, and compiled from the internet, the only medically viable solution to eradicating or resolving this deficiency is Plasminogen. This deficiency has the potential of seriously affecting Dante both systemically and histologically as his immune system matures. Due to the potentially life-threatening affects of Plasminogen deficiency, Dante is in great need of Human Plasminogen so that he can live a long, healthy and productive life.

With respect to donating blood, I would generously donate my BLOOD/PLASMA to rid Dante of this deficiency, as would all the members of my immediate family, which include my father (75), mother (72) brother (51), and Dante's two half-brothers, Andrew (19) and Justin (18). Due to the fact that I do not know of the whereabouts of Dante's biological father, his donation, though much needed, is likely not possible due to our not having had any kind of contact since Dante's birth. My research has raised many questions, some of which are being answered by very knowledgeable and ethical members of Health Canada , the members of the Pharmaceutical Industry, and other professionals. Yet no one seems to be able to answer “why” Dante cannot have access to Plasminogen? Other very pertinent questions as to why the plasma has to be pooled? Why is that I cannot donate my Plasminogen to my own son? I verily believe that for every question, there is an answer, but I have yet to find an answer to these perplexing questions. I acknowledge and respect the fact that there is a great concern for HIV and HEPATITIS transmission via plasma and plasma derivatives, as per The Commission of Inquiry on the Blood System in Canada , but these same concerns presently apply, as far as I know, to every blood donation made in Canada .

I am very sympathetic to, and have great empathy for the plight of all children suffering from other medical conditions here and abroad. Whenever I watch programs about children who are going through medical difficulties, my heart goes out to them. I know that I cannot save the world but the least I can do is try and help my son Dante to the best of my ability, which is what any mother in my position would do. It is my duty and obligation as Dante's mother to do whatever is humanly possible, using whatever means attainable, to help him. I have an ethical and moral responsibility to my son to source and acquire Human Plasminogen. I will not stop until I have succeeded in doing everything within my power to acquire Human Plasminogen for him…it would be negligent of me to do otherwise. I wish to reiterate that Dante's need for the sourcing and subsequent acquisition of topical Human Plasminogen or therapeutic intravenous Human Plasminogen is great. Please provide any information that you may have with respect to sourcing Human Plasminogen with the subsequent approval of Health Canada ( Ottawa ), on Dante's behalf.

I have now been researching, e-mailing, etc. for a month now with no success. In addition to my continual research of this rare disease, my attempts to source Human Plasminogen for Dante have been to no avail. I have called and sent e-mails to practically every pharmaceutical corporation and blood organization in the world and not one of them manufactures Human Plasminogen/Human Purified Plasminogen Concentrate (topical or intravenous) to resolve/eradicate Ligneous Conjunctivitis and Plasminogen Deficiency in humans . It seems that because this deficiency is so rare, and the demand is not there, pharmaceutical corporations cannot justify manufacturing/marketing of such a product with little or no return on their investment; there is no incentive for them to do otherwise. Another key fact is that it is manufactured in biological laboratories and/or research facilities, but for research within the confines of those establishments only (ie. animal experiments), and not for human use.

The people of Canada lead such wonderful lives, yet I honestly feel that some individuals take this privilege for granted, I personally do not. I truly believe that people, in general, have become desensitized to world issues such as war, famine, poverty, disease, etc. because it does not affect them personally; they do not live with it day after day, and I for one do not take anything for granted in life, especially when it comes to my children. If anything, I am totally sympathetic and empathetic to the plight of anyone that has had to endure hardship of any kind. Our society and beliefs are based on fundamental freedom and justice, and as per the “Canadian Charter of Rights and Freedoms, e veryone has the right to life, liberty and security of the person and the right not to be deprived thereof except in accordance with the principles of fundamental justice” . I am reaching out to those of you who have much more knowledge, insight and experience since I have nowhere else to turn as a mother whose son is afflicted, and affected by this rare disorder/disease.

We have been waiting 3-1/2 + years for Plasminogen and I have had to resort to sourcing Plasminogen on my own. The wait time for Plasminogen has been exceeded exponentially, and if I may quote directly from the Prime Minister's website at http://pm.gc.ca/eng/feature.asp?pageId=40&featureId=5 :

“Delivering the health care Canadians need, when they need it, by addressing the fiscal imbalance and establishing a patient wait times guarantee with the provinces”; and

“The Government will engage the provinces and territories on a patient wait times guarantee for medically necessary services. This guarantee will make sure that all Canadians receive essential medical treatment within clinically acceptable waiting times”.

It is up to the individuals within the families of those affected with rare diseases and/or deficiencies to join forces with other families in the same situation, and to bring this matter to the attention of all the members of Parliament, up to and including The Prime Minister of Canada. Collectively, not individually, we as a group need to make an effect directly on our politicians and that is the only way we will see progress in the implementation of an Orphan Drug Policy/Act . We need our Canadian Government to provide funding for research & development, clinical studies, and immediate access to drugs/medicine/blood products for rare disorders/diseases. I am personally going to attend upon the offices of the C.O.R.D. - Canadian Organization for Rare Disorders and become a member; their Mission Statement is as follows: “Through an education and information support network, CORD is committed to the enhancement of the lives of all persons affected by rare disorders.” It's the least I can do for Dante.

In conclusion, and to summarize and provide a synopsis of all that I have read on the internet, in direct relation to the links herein, Ligneous Conjunctivitis is a serious complication of Human Plasminogen deficiency and Dante needs your intervention. Any information, no matter how insignificant you feel it may seem, is urgently needed and respectfully requested. Thank you kindly and reverently for allowing me to eloquently convey my thoughts with respect to what has transpired to date. I verily believe that with your assistance, guidance, and direction, working towards obtaining Human Plasminogen is a goal that is achievable and within our grasp. Please allow me to thank you for your time and I look forward to hearing from you, via return e-mail, at your earliest convenience.

Kind Regards,
Margie Di Danieli
Toronto , Ontario , Canada
E-mail addresses: dante4@rogers.com - love4dante@rogers.com - dante12302000@hotmail.com

References and Case Citations from research that I have conducted in direct relation to Dante's Plasminogen deficiency.
The sites that are in blue font and have an diamond: next to them are the ones I found to be most informative and which led me on this search for Human Plasminogen:

http://www.thestar.com/NASApp/cs/ContentServer?pagename=thestar/Render&inifile=futuretense.ini&c=Page&cid=968332188492&pubid=968163964505

http://www.emea.eu.int

http://www.raredisorders.ca/index.php (C.O.R.D. - Canadian Organization for Rare Disorders CANADA)

http://www.rarediseases.org/ (NORD – National Organization for Rare Disorders USA)

http://pm.gc.ca

http://www.baxter.com/about_baxter/center_for_one_baxter/products_and_services_directory.pdf

http://biolex.com/pdfs/BioCentury%20Article%20May%209,%202005.pdf

http://www.zlbbehringcustservices.com/b2b_zlb/general/jsp/newsarticle.jsp?selectedListArticle=true&articleID=4

http://www.hhmi.org/research/investigators/ginsburg.html (Molecular Genetics of Blood Clotting)

http://www.hhmi.org/news/ginsburg2.html (Streptococcus Infects Humans by Thwarting Blood Clotting)

http://www.nature.com/nature/journal/v425/n6960/full/nature02055.html (The DNA Sequence and Analysis of Chromosome 6)

http://www.fda.gov/cber/minutes/biother061305t.htm (Biological Therapeutics for Rare Plasma Protein Disorders Public Workshop June 13, 2005 )

http://www.fda.gov/cber/minutes/biother061405t.htm (Biological Therapeutics for Rare Plasma Protein Disorders Public Workshop June 14, 2005 )

http://www.budoniambiente.org/meyer/default.asp (website citing almost 50 cases of Ligneous Conjunctivitis – Cases and Documentation)

http://srs.embl-heidelberg.de:8000/srs5bin/cgi-bin/wgetz?-e+[omim-id:173350 ]

http://zeta.embl-heidelberg.de:8000/srs5bin/cgi-bin/wgetz?-e+[omim-id:217090 ] (#217090 CONJUNCTIVITIS, LIGNEOUS)

http://www.orpha.net/consor/cgi-bin/OC_Exp.php?Lng=GB&Expert=465 ( D I S E A S E : Plasminogen activator inhibitor type 1 deficiency, congenital)

http://www.merck.com/mrkshared/mmanual/section11/chapter131/131b.jsp ( Haemostasis)

http://www.merck.com/mrkshared/mmanual/section11/chapter131/131c.jsp (Hereditary Coagulation Disorders)

http://www.merck.com/mrkshared/mmanual/section11/chapter131/131d.jsp (Acquired Coagulation Disorders)

http://www.merck.com/mrkshared/mmanual/section11/chapter132/132a.jsp (General - Haematology & Oncology)

- (Thrombotic Disorders: Diseases characterized by formation of a thrombus that obstructs vascular blood flow locally or detaches and embolizes to occlude blood flow downstream (thromboembolism)

http://content.nejm.org/cgi/content/full/339/23/1679 (you must subscribe to The New England Journal of Medicine – easy and free to subscribe)

- Therapy with a Purified Plasminogen Concentrate in an Infant with Ligneous Conjunctivitis and Homozygous Plasminogen Deficiency

http://www.ifcc.org/ejifcc/vol12no3/plasminogen.htm ( Criteria for the specific measurements of plasminogen enzymatic; procedure in human plasma

http://www.ri.ccf.org/moleccard/hoover-plow ( Mechanisms of plasminogen dependent cell migration in inflammation; genetic determinants of atherothrombosis)

http://www.obesityresearch.org/cgi/content/abstract/13/3/381

http://www.blood.ca/CentreApps/Internet/UW_V502_MainEngine.nsf/page/Plasma

http://www.blood.ca/CentreApps/Internet/UW_V502_MainEngine.nsf/page/Collecting%20Plasma?OpenDocument

http://www.blood.ca/CentreApps/Internet/UW_V502_MainEngine.nsf/page/Fractionation?OpenDocument

http://www.blood.ca/CentreApps/Internet/UW_V502_MainEngine.nsf/page/Becoming%20a%20Donor?OpenDocument

http://www.blood.ca/CentreApps/Internet/UW_V502_MainEngine.nsf/page/The%20Donation%20Process?OpenDocument

http://www.blood.ca/centreapps/internet/uw_v502_mainengine.nsf/page/E_Plasma%20Donor%20Clinic%20Hours?OpenDocument

http://www.blood.ca/CentreApps/Internet/UW_V502_MainEngine.nsf/page/E_PlasmaProducts

http://www.americandiagnostica.com

http://www.paion.de

http://www.wfh.org/index.asp?lang=EN

http://www.kingbiomed.com

I have followed these principles in conducting all research to date:

“The definition of research at www.wikipedia.org is as follows:

Research is often described as an active, diligent, and systematic process of inquiry aimed at discovering, interpreting and revising facts . This intellectual investigation produces a greater understanding of events, behaviors , or theories , and makes practical applications through laws and theories. The term research is also used to describe a collection of information about a particular subject, and is usually associated with science and the scientific method .

I verily believe that I have conducted thorough research into this rare disease utilizing a basic concept, as follows:

In journalism , the Five Ws , also known as the Five Ws (and one H) or simply the Six Ws , is a concept in news style , research , and in police investigations that most people consider to be fundamental. It is a formula for getting the "full" story on something. The maxim of the Five Ws (and one H) is that in order for a report to be considered complete it must answer a checklist of six questions, each of which comprises an interrogative word :

·                   who ? what ? why ? where ? when ? how ?

The principle underlying the maxim is that each question should elicit a factual answer — facts that it is necessary to include for a report to be considered complete. Importantly, none of these questions can be answered with a simple "yes" or "no". In the context of the "news style" for newspaper reporting, the Five W's are types of facts that should be contained in the "lede", or first two or three paragraphs of the story, after which more expository writing is allowed.”

© Achille Mauro Porcheddu 2004 ~ 2008 - Enea's Case - achille@budoniambiente.org