Multi morbid diseases of Palestine childern - nabildeeb-Syndrome - Dr.Nabil DEEB

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Multi morbid diseases of Palestine childern - nabildeeb-Syndrome - Dr.Nabil DEEB
Multi morbid diseases of Palestine childern - nabildeeb-Syndrome - Dr.Nabil DEEB

Dr. Nabil DEEB

Arzt – Physician – Doctor

PMI-Registered Doctors'Association

53140 Bonn  /  GERMANY


Multi morbid diseases of Palestine childern - nabildeeb-Syndrome .

The stateless Palestinian refugee children are discriminated in the medical treatment.

                            1.  Anorectal Malformations :-

The little girl, Zaina Mohammed Hussein Al - Gazar was born at 02. November 2009 in Baghdad - Iraq. She is a child stateless Palestinian refugee, and living at the time of political unrest and expelled from Iraq in the UNHCR - the refugee camp in the desert between Iraq and Syria on Syrian territory under temporary in  care and contactor of the UNHCR and waits with her parents and two healthy sisters to live in  a country anywhere in the world through the agency, UNHCR – Geneva .

When the intake of these above mentioned refugees is uncertain and often lasts two to five years waiting in UNHCR - refugee camp in the desert near the Iraqi-Syrian border .

The two-year-old girl suffering from birth under "" Anorectal malformation ""
(= congenital malformations of the rectum and the anus) as a result of the developmental disorder of embryo in the early stages of pregnancy of her mother because of the radiation depleting effect of the environment in the home of their mother in Baghdad .

It was a temporary colostomy (colostomy =) at university hospitals - Baghdad in March 2010 created the child, give the child a temporary chair to give way, which is for her vital moment .

Another treatment for the child in the Palestinian desert in Flüchttlingslager was and still is not possible for many reasons .

The child needs urgent and more vital operational treatment as a pulling operation (posterior sagittal anorectal PSARP = plastic) with a minimally invasive surgery is used .

Such further treatment in a special children's surgery - clinic in Europe, for example in Finland, Norway, Sweden, France or  in the Federal Republic of Germany or in USA , for example possible in the department of pediatric surgery .

The three-year-old girl Zina waiting since her birth to 02 November 2009 with her two little healthy sisters and their parents in the desert between the Iraqi and Syrian deserts in - the refugee UNHCR camp to a reasonable medical surgical treatment in a country .

Without appropriate treatment for the child's life little girl Zina would be destroyed forever because permanent serious infections and cancer degeneration .

In this context, I refer-so called International Humanitarian Law .

In comparison to the treatment of children in the rest of the world as in Europe or the U. S. A. I refer to the latest published medical contribution of colleague Risto J. Rintala, Professor of Paediatric Surgery, Hospital for Children and Adolescents, FIN-00029 HUS, Finland  in Finland and I also add sections of this paper and give the source of this scientific publication for information: -

Journal of Pediatric Gastroenterology & Nutrition:

April 2009 - Volume 48 - Issue - p S79-S82

doi: 10.1097/MPG.0b013e3181a15b5e

Original Articles


Congenital Anorectal Malformations: Anything New?

Rintala, Risto J

Abstract

Management of anorectal malformations, especially that of proximal and more severe forms have developed significantly in the last 25 years. Echography and magnetic resonance imaging have improved the diagnostic accuracy, especially in terms of associated anomalies that are the main cause of fatalities among patients with anorectal malformations. Animal models, novel molecular biological techniques and knock-out gene models have widened our knowledge on aetiology and pathogenesis of these anomalies .

From early 1980s, the posterior sagittal anorectoplasty originally designed by deVries and Pena has systematized the surgical management of anorectal anomalies leading to improved functional outcomes. Surgical mini-invasive techniques are increasingly applied to the management of these anomalies, although their role is as yet not clearly established. Long-term functional follow data are also available today indicating a reasonable favourable faecal and urinary control in the majority of patients with anorectal malformations. For the patients with less favourable functional outcome there are bowel and bladder management options that provide sufficient social continence .

Malformations of  the anorectum include a series of congenital lesions ranging from a slight malposition of the anus to complex anomalies of the hindgut and urogenital organs. The reported incidence of anorectal anomalies range between 1:3300 and 1:5000 live births. In Western communities there is a male preponderance, 55% to 70% of the patients in larger series have been males. The more severe malformations tend to be more common in male patients.
In most cases, anorectal malformations present as an absence of an anus in its normal position. In mild forms of anal anomalies the bowel outlet opens in the perineal region outside the usually well-developed voluntary sphincter complex. In more severe anomalies bowel outlet opens in an ectopic position in the urogenital tract in males or genital tract in females. Neonatal recognition of the type of the malformation is essential for the planning of the surgical management of anorectal anomalies .

There is no generally accepted method to classify anorectal malformations. The most commonly used has been the Wingspread International Classification for Anorectal Malformations. Recently, a new simplified classification has emerged, Krickenbeck classification (Table 1), which is based on consensus recommendations of world authorities (1) .


Table 1

The outcomes of patients with anorectal malformations have greatly improved by modern surgical techniques and neonatal care facilities during the last decades. Early survival is today a rule except in some rare cases with cardiac, urogenital, or chromosomal anomalies that are not compatible with life. The overall long-term functional outcome expectancy in terms of faecal and urinary continence is today relatively optimistic. The majority of patients reaching adolescence and adulthood are able to maintain themselves socially continent .

RECENT BASIC SCIENCE

The aetiology of anorectal anomalies is not known. Genetically determined syndromes with anorectal malformations are relatively uncommon. On the contrary, anorectal anomalies occur commonly in multianomaly sequences such as VACTERL or CHARGE associations. The overall incidence of associated anomalies in patients with anorectal malformations is more than 60%. This suggests that the stimuli that induce abnormal development of anorectum operate universally in the developing foetus and may cause maldevelopment of several organ systems. Anorectal malformations may develop in some syndromes that are caused by a mutation of a single gene. These include Currarrino syndrome, which is caused by a mutation in HLXB9 gene in chromosome locus 7q39 and Townes-Brock syndrome, which has a mutated SALL1 gene in chromosome locus 16q12.1 .

The pathogenesis of anorectal malformations has recently been much clarified by modern embryological studies concerning experimental models of anorectal anomalies. Anorectal anomalies occur spontaneously in some animal models (Danforth's short tail mouse) and can be induced by several substances, such as ethinylthiourea, retinoic acid derivatives, and adriamycin. The spectrum of malformations following administration of these substances is variable, but they are valuable tools for embryological studies. The most significant findings in these embryological models are abnormalities or absence of the cloacal membrane. The abnormal cloacal membrane does not allow normal breakdown of the bowel to the perineum. In the animal models a clinically important discovery has been the character of the rectourogenital or perineal communication; the fistulous communication is actually an ectopic anus. This ectopic anus has the characteristics of a normal anal canal including a distal zone of transitional epithelium, anal glands, and the internal anal sphincter, which is a thickening of the circular muscle layer around the bowel outlet. The voluntary external sphincter complex including the levator ani muscle develops separately in a normal position. The voluntary sphincters are always hypoplastic; the degree of the hypoplasia is dependent on the distance of the rectal pouch from the perineum and on the severity of commonly associated sacral deformity .

DIAGNOSTIC MODALITIES

With the exception of those anorectal malformations that are associated with other major structural anomalies (eg, VACTERL syndrome or caudal regression syndromes), prenatal ultrasound diagnosis of it is uncommon. The sonographic features in the foetus are nonspecific and include dilated or echogenic fetal bowel and hydrometrocolpos. Associated cardiac, renal or vertebral anomalies may be detected. Cloacal malformations can rarely be diagnosed by the presence of a septated cystic mass in the pelvis with hydronephrosis and oligohydramnios .

Despite abundance of sophisticated diagnostic imaging modalities, the diagnosis and classification of anorectal malformations remain on the basis of clinical picture and examination. The clinical examination performed by an experienced examiner reveals the type of the anorectal malformation in >90% of the cases.
Classical imaging with inversion or cross-table radiography is useful in selected cases where clinical examination is equivocal; however, there are many possibilities for erroneous interpretation. Ultrasound has not established itself as a reliable method to determine the position of the terminal colon. It provides similar information as the prone, cross-table radiography and has similar limitations. However, ultrasound is extremely useful in detecting abnormalities in the urinary and genital tracts, especially obstructive uropathy and hydrometrocolpos .

Modern computed tomography technology allows multiplanar imaging, however, this comes at a cost of radiation to a newborn child. Although it provides increased detail, especially of the bony structures as well as pelvic musculature, it is not reliable in revealing the anatomy of bowel termination and the rectourogenital communication .

Magnetic resonance imaging provides the same multiplanar capability as computed tomography scan but without the radiation burden. Although magnetic resonance imaging can accurately delineate the level of the bowel termination, it will detect only a small proportion of the rectourogenital fistulas. In newborn, small body size also impedes spatial resolution .

The most reliable and useful imaging modality is still the colostogram, especially if it is augmented by pressure. All patients who have undergone a primary diverting colostomy for a severe anorectal anomaly should have this imaging study to accurately delineate the anatomy of the bowel termination and the fistula. Micturating cystourethrography has still its place in the imaging of a neonate with an anorectal malformation because of high incidence of renal tract anomalies .

Of absolute importance is imaging to detect associated malformations as some of these defects may be life threatening or have a greater impact on survival of the patients than the anorectal malformation. Typical anomalies of this kind are cardiac defects and tracheo-oesophageal anomalies. Chest radiography with a nasogastric tube in place excludes the presence of an oesophageal atresia .

Cardiac echogram is used to screen cardiac anomalies. Ultrasound of urinary tract is performed in each patient with an anorectal malformation to rule out obstructive uropathies and other urinary tract anomalies .

Vertebral and sacral anomalies are common in patients with anorectal malformations. These are commonly accompanied by spinal cord abnormalities. The spinal cord can also be visualized by ultrasound in a neonate. This is usually performed in combination with radiographs that provide information on the bony structure .

MANAGEMENT
A provisional diagnosis of the severity of an anorectal malformation is usually easily made in a newborn. The most important issue is to ascertain the level of the anomaly, because it determines the operative treatment in the neonatal period. There is rarely any urgency, and most patients tolerate the low bowel obstruction without symptoms for longer than 24 hours if the upper gastrointestinal tract is decompressed by a nasogastric tube. Before any treatment of the anorectal malformation is carried out, life-threatening associated malformations should be ruled out .

The aims of surgical treatment are to provide a normally sited anus of adequate calibre and appearance while preserving as much of the normal intrinsic anorectal anatomy and function as possible. In recent years, the posterior sagittal anorectoplasty (PSARP), developed and popularized by Alberto Peٌa, has become the most frequently performed surgical procedure in the reconstruction of anorectal malformations (2). The PSARP affords a relatively standardized approach to all common varieties of anorectal anomalies, enables direct visualization of the structures without the necessity for blind, potentially hazardous, surgical manoeuvres, and offers the opportunity for anatomically precise reconstruction. Functional results are definitely no worse than with traditional surgical techniques, and there is growing evidence that long-term bowel function is superior. The timing of surgery is variable; today many surgeons try to undertake repair as soon as the patient has stabilised from neonatal procedures. There is some evidence that the functional results are better if the repair is performed as early as possible .

In patients with a perineal bowel outlet or fistula (traditional low anomaly) the anorectal repair can be performed safely in a newborn. In males the options are a simple cutback anoplasty or a limited PSARP procedure that requires significantly more extensive dissection than the cutback procedure. Functional outcomes are similar, but there appears to be fewer complications after the cutback operation (3). In females cutback procedure yields unsightly cosmetic result and probably worse outcome than after a limited PSARP procedure.
Patients with more severe anomalies usually receive a diverting colostomy as a newborn. These anomalies are usually repaired with the PSARP procedure followed by closure of the colostomy. A typical male anomaly is an imperforate anus with a rectourethral fistula and a typical female anomaly an imperforate anus with a rectovestibular fistula. The latter may be repaired in a newborn without previous colostomy, but a safer approach would be a staged operation. Recently, some surgeons have advocated neonatal repair without preceding colostomy for severe anomalies as well. This approach has not gained general acceptance and the functional outcomes remain unclear. Theoretically, repair as early as possible could augment development of neurocircuitry between anal canal and brain, resulting in better functional outcome .

Recently, some surgeons have advocated laparoscopy-assisted repair for high anorectal anomalies. The procedure entails laparoscopic mobilisation of the rectal blind pouch, closure of the rectourethral fistula, and pull-through of the bowel termination through the centre of sphincter funnel that has been identified by muscle stimulation. At the moment laparoscopic surgery for anorectal anomalies is at an experimental stage, but the approach is promising, especially because of its mini-invasiveness. Long-term follow-up results are lacking but warranted .

OUTCOMES
Long-term functional outcome following repair of anorectal malformations with a perineal opening (low malformations) is good in the majority of patients. The bowel function does not equal that of healthy people, but most patients have a good quality of life without social restrictions. The most common complaint especially during childhood is constipation, which is usually manageable with oral laxatives .

Patients with more severe anomalies have somewhat more guarded functional prognosis (4). Although it is evident that modern methods of repair yield superior functional results compared with older techniques such as abdominoperineal or sacroperineal pull-through, a significant percentage of patients still experience abnormal bowel control. These patients can be helped significantly by bowel management programmes that often include formation of a continent catheterisable conduit from the appendix that is used to empty the bowel regularly. With the help of bowel management regimens a majority of patients with severe malformations gain a good quality of life. Novel technical innovations such as artificial anal sphincters, electrically stimulated sphincters, or sacral nerve stimulation have been used in some selected patients with promising outcomes. However, similar cleanliness as with antegrade bowel washouts is rarely achieved .

The patients with anorectal malformations need careful follow-up throughout their childhood. Functional complications such as constipation should be detected and treated early to achieve optimal outcome. The treatment of defective continence should be started well before the child reaches school age to overcome the devastating social consequences of faecal soiling and to integrate the child to the social context of his peers. Because the management of anorectal malformations requires years of commitment and special knowledge concerning the anatomical and physiological characteristics of this complex group of congenital malformations, these children should be treated by specialised medical personnel in specialised referral centres .

I .

REFERENCES  of colleague Risto J. Rintala, Professor of Paediatric Surgery, Hospital for Children and Adolescents, FIN-00029 HUS, Finland  :-


1. Holschneider A, Hutson J, Peٌa A, et al. Preliminary report on the International Conference for the Development of Standards for the Treatment of Anorectal Malformations. J Pediatr Surg 2005; 40:1521-1526.
Cited Here... | PubMed | CrossRef


2. Pena A. Anorectal malformations. Semin Pediatr Surg 1995; 4:35-47.
Cited Here... | PubMed


3. Pakarinen MP, Goyal A, Koivusalo A, et al. Functional outcome in correction of perineal fistula in boys with anoplasty versus posterior sagittal anorectoplasty. Pediatr Surg Int 2006; 22:961-965 .

Cited Here... | PubMed | CrossRef


4. Rintala R, Lindahl H. Is normal bowel function possible after repair of intermediate and high anorectal malformations. J Pediatr Surg 1995; 30:491-494 .

Cited Here... | PubMed | CrossRef


II.
My REFERENCES : -


http://www.aerztezeitung.de/politik_gesellschaft/gp_specials/pid/article/626203/fdp-will-zulassung-pid-werben.html

Nabil DEEB , nabildeeb, pmi , In the pre-implantation diagnosis, the medical indications and the decisions of the European Court of Human Rights (ECHR) in Strasbourg are considered .


Nabil DEEB , nabildeeb, pmi , Dans le diagnostic pré-implantatoire, les indications médicales et les décisions de la Cour européenne des droits de l'homme (CEDH) à Strasbourg sont considérées .


http://www.springermedizin.at/artikel/19708-denken-ohne-affekt


Nabil DEEB , nabildeeb, pmi , Human right to health also applies to the people of Gaza Strip .


Nabil DEEB , nabildeeb, pmi , Droit à la santé de l'homme s'applique également à la population de la bande de Gaza .


http://www.aerztezeitung.de/politik_gesellschaft/gp_specials/pid/article/626883/wachsende-zahl-pid-befuerworter-cdu.html


Nabil DEEB , nabildeeb, pmi , Human right to health also applies to Berlin, despite the various party lines .


Nabil DEEB , nabildeeb, pmi , In deciding on the approval of preimplantation genetic diagnosis (PGD) and on health care reform, human rights, international law and the fundamental law of the land without limits .


Nabil DEEB , nabildeeb, pmi , droit à la santé de l'homme s'applique également à Berlin, malgré les lignes des divers partis .


Nabil DEEB , nabildeeb, pmi , n se prononçant sur l'approbation du diagnostic génétique préimplantatoire (DPI) et sur la réforme des soins de santé, les droits de l'homme, droit international et la loi fondamentale du pays sans limites .


http://www.aerztezeitung.de/medizin/krankheiten/hormonstoerungen/schilddruesen-erkrankungen/article/608829/erhoehtes-risiko-fehlgeburten-leichter-hypothyreose.html


Nabil DEEB , nabildeeb, pmi , The interaction of beta-hCG with the TSH-receptor. & Angiogenic factors in pathogenesis and prediction of preeclampsia .


Nabil DEEB , nabildeeb, pmi , interaction de la beta-hCG avec le récepteur de la TSH . &

Facteurs angiogéniques dans la pathogenèse et la prédiction de la preeclampsia .


http://www.aerztezeitung.de/politik_gesellschaft/gp_specials/pid/article/624740/embryonenschutz-kann-spaltpilz-koalition.html


Nabil DEEB , nabildeeb, pmi , Preimplantation genetic diagnosis up2date in user knowledge society .


Nabil DEEB , nabildeeb, pmi , up2date diagnostic génétique préimplantatoire dans la société les connaissances des utilisateurs .

 

http://www.aerztezeitung.de/medizin/krankheiten/neuro-psychiatrische_krankheiten/depressionen/article/632090/neues-portal-depressiven-stoerungen-netz.html


Nabil DEEB , nabildeeb, pmi , The psychiatric patient in the pregnancy. Unipolar depression, antenatal and postnatal .


Nabil DEEB , nabildeeb, pmi , Le patient psychiatrique de la grossesse. La dépression unipolaire, prénatals et postnatals .


http://www.aerztezeitung.de/politik_gesellschaft/gp_specials/pid/article/635081/meisner-vergleicht-pid-kindermord-bethlehem.html


Nabil DEEB , nabildeeb, pmi , Preimplantation genetic diagnosis up2date in our knowledge society must be based on the German constitution and set up European law .


Up2date diagnostic génétique préimplantatoire dans notre société de la connaissance doit être fondée sur la constitution allemande et mettre en place la législation européenne .


http://www.aerztezeitung.de/medizin/fachbereiche/innere_medizin/article/626363/expertenchat-thema-schilddruese.html


Nabil DEEB , nabildeeb, pmi , The interaction of beta-hCG with the TSH-receptor. & Angiogenic factors in pathogenesis and prediction of preeclampsia .


Interaction de la beta-hCG avec le récepteur de la TSH. & Facteurs angiogéniques dans la pathogenèse et la prédiction de la preeclampsia .


http://www.paliraq.com/index.php?option=com_content&task=view&id=3759&Itemid=49


Nabil DEEB , nabildeeb, pmi , The leukemia and traumatic injury in the toxic war with depleted uranium , Dr. Nabil  Abdul Kadir DEEB , Arzt – Physician – Doctor .


http://www.springermedizin.at/artikel/16612-die-schizoaffektive-stoerung-validitaet-und-reliabilitaet


Nabil DEEB , nabildeeb, pmi , Schizophrenia, race theories, natural law and the diaspora population genetics in the world financial crisis .


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http://www.springermedizin.at/artikel/9522-neues-genetisches-verfahren-fuer-erfolgreiche-kuenstliche-befruchtung


Nabil DEEB , nabildeeb, pmi , PID–Trias & Konnexit
نt zwischen نrztlicher Tنtigkeit und der ـberzeugung von Würde und Schutzwürdigkeit menschlichen Lebens von der Zeugung .


http://www.aerztezeitung.de/medizin/krankheiten/krebs/article/610766/lange-telomere-geringes-krebsrisiko.html


Nabil DEEB , nabildeeb, pmi , The telomerase may play a central role in the carcinogenesis of human cells .


Nabil DEEB , nabildeeb, pmi , La télomérase mai jouer un rôle central dans la carcinogenèse des cellules humaines .


http://www.springermedizin.at/artikel/360-schwierigkeiten-bei-der-evakuierung-aus-krisengebieten-bedarf-an-einheitlichen-und-einfach-anzuwend


Nabil DEEB , nabildeeb, pmi , Human right to health also applies to the people of Gaza Strip .


Nabil DEEB , nabildeeb, pmi , Droit à la santé de l'homme s'applique également à la population de la bande de Gaza .


http://www.aerztezeitung.de/praxis_wirtschaft/klinikmanagement/article/629322/patient-hakenkreuz-arm-juedischer-arzt-verweigert-op.html


Nabil DEEB , nabildeeb, pmi , Human right to health and personal development of our science in society .

 

Nabil DEEB , nabildeeb, pmi , droit à la santé et le développement personnel de notre science dans la société .


http://www.springermedizin.at/artikel/13103-krankheit-mit-migrationshintergrund


Nabil DEEB , nabildeeb, pmi , Schizophrenia, race theories, natural law and the diaspora population genetics in the world financial crisis .


Nabil DEEB , nabildeeb, pmi , Schizophrénie, théories de la race, la loi naturelle et la génétique des populations diaspora dans la crise financière mondiale .


http://www.aerztezeitung.de/medizin/krankheiten/hormonstoerungen/article/622283/forscher-brauchten-jahrzehnte-bis-erste-erfolgreiche-kuenstliche-befruchtung-gelang.html


Nabil DEEB , nabildeeb, pmi , The serious progress in reproductive medicine worldwide in a knowledge society .


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http://www.springermedizin.at/artikel/4764-15-jahre-tschernobyl-kinder-sterben-an-leukaemie-gehirntumoren-und-schilddruesenkrebs


Nabil DEEB , nabildeeb, pmi , Human right to health also applies to the people of Gaza Strip .


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http://www.aerztezeitung.de/medizin/krankheiten/krebs/article/589959/massgeschneiderter-bluttest-krebskranke.html


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http://www.paliraq.com/index.php?option=com_content&task=view&id=6591&Itemid=72


The right to compensation for Palestinian refugees for damages under general international law against any aggressor state or against    members of the aggressor State - Dr. Nabil DEEB .


http://www.paliraq.com/index.php?option=com_content&task=view&id=6581&Itemid=72

Personalized diagnosis and therapy of cancer in our knowledge society
and the UN, WHO and UNHCR - Dr. Nabil DEEB .


http://www.paliraq.com/index.php?option=com_content&task=view&id=6556&Itemid=72


Dr. Nabil AbdulKadir DEEB

International Humanitarian Law And The Palestinian People Throughout The World

Importance of international humanitarian law ?

 

Dr. Nabil DEEB

Arzt – Physician – Doctor

PMI-ؤrzteverein e.V.

Palنstinamedico International ؤrzteverein – ( P M I ) e.V.

Palestine Medico International Doctors Association ( P.M.I.) registered association .

Department of Medical Research

Département de la recherche médicale

P.O. Box 20 10 53

53140 Bonn – Bad Godesberg / GERMANY

doctor.nabil.deeb.pmi.germany@googlemail.com

doctor.nabilabdulkadirdeeb@googlemail.com

22/5/2012

 

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