Pak Jusuf, ini artikelnya. Semoga cepet sembuh ya dedek Nayyara...hiks.....
Penyebabnya tidak diketahui, pak.

Biliary Atresia
Biliary atresia is the congenital absence or
closure of the ducts  that drain bile from the liver. See: Kasai
Procedure<http://www.classkids.org/library/biliaryatresia.htm#Kasai>

Cells within the liver secrete a liquid called bile, which is made up of
cholesterol, bile salts and waste products, including bilirubin. A network
of tubular structures and tiny ducts form the biliary system to drain bile
from the liver to the small intestine where it aids in the digestive
process. Biliary atresia is the closure or disappearance of the biliary
system.

Biliary atresia is a progressive inflammatory process that begins very soon
after birth. In the most common form, called extrahepatic biliary atresia,
the delicate ducts outside the liver are affected first. White blood cells
invade the ducts, which become damaged and may whither or completely
disappear. Bile is trapped inside the liver and rapidly causes damage and
scarring to the liver cells. Further scarring of the liver tissue may result
in cirrhosis.

On average, there is one case of biliary atresia out of every 15,000 live
births. Females are affected slightly more often than males. In the United
States, approximately 300 new cases are diagnosed each year.
What causes biliary atresia?

The cause of biliary atresia is unknown. Auto-immune mechanisms may be
partly responsible for the progressive process that takes place. Recent
research suggests that a viral infection in susceptible infants could result
in biliary atresia. About 10% of cases have other associated congenital
defects in the heart, blood vessels, intestine, or spleen.

Although the cause is uncertain, it is known that biliary atresia affects
only newborns; it is not hereditary; it is not contagious; and it is not
preventable. Parents should be assured that biliary atresia is not caused by
anything the mother did during pregnancy.
Diagnosis

The usual history is a full term infant who appears normal at birth but
develops jaundice after the age of 2 to 3 weeks. The infant has yellow eyes
and skin, light-colored stools and dark urine caused by the build up of
bilirubin in the blood. The abdomen may be swollen with a firm, enlarged
liver. Weight loss and irritability develop as the level of jaundice
increases.

Many tests are needed to rule out other possible causes of jaundice and
diagnosis biliary atresia:
  [image: bullet] Blood tests may show abnormal results which indicate liver
dysfunction with increased bilirubin (a chemical marker in the blood for
jaundice). [image: bullet] An ultrasound test may detect an absent or tiny
gall bladder. [image: bullet] In another test, called a HIDA scan, a special
radioactive dye that acts like bilirubin is injected into the infant's vein.
In biliary atresia, the dye is taken up by the liver but cannot flow through
the damaged biliary system to the small intestine. [image: bullet] A special
needle may be used to take a tiny piece of liver, which under a microscope
may indicate features typical of an obstruction to the biliary system. [image:
bullet] Surgical exploration of the baby's abdomen is necessary in most
cases of suspected biliary atresia to definitively make the diagnosis. [image:
kasai2.jpg (17826 bytes)]TreatmentOnce the diagnosis is confirmed, the
preferred treatment is to remove the atretic biliary ducts outside the liver
and attach the small intestine directly to the liver at the spot where bile
is found or expected to drain. The segment of intestine that connects to the
liver also connects to the rest of the intestine and forms a Y connection
called a "Roux-en-Y hepatoportojejunostomy," or Kasai procedure.

Bile flow is re-established in approximately 80% of infants who are operated
on when younger than 3 months of age. Of these, about 50% will have some
bile drainage and as many as 30% will have complete bile drainage with a
return to normal bilirubin. About 20% of infants will not be helped by the
Kasai procedure. In these cases, the only other treatment option is a liver
transplant.

Early diagnosis of this disease is very important. If surgery is performed
before the baby is 2 months old, success is much more likely. After 3
months, success of the operation is poor. For this reason, all infants who
are jaundiced after the age of 4 weeks should be evaluated for biliary
atresia.
Complications

The most common complication associated with the Kasai procedure is
ascending cholangitis, a bacterial infection of the biliary tree. Bacteria
normally found in the small intestine moves up the Roux-en-Y causing
infection. Signs include unexplained fever, increased jaundice, or lighter
stools. Intravenous and long-term oral antibiotics are used to prevent and
treat ascending cholangitis.

Adequate bile flow is needed for the digestion and absorption of dietary
fats and fat-soluble vitamins, including vitamins A, D, E, and K. When bile
flow is reduced, poor growth and malnutrition may result. Special formulas
containing medium-chain triglycerides (an easily digested form of dietary
fat) and water-soluble vitamin supplements are often prescribed to maximize
the child's growth and development.

Formation of scar tissue eventually leads to portal hypertension in many
children. Portal hypertension refers to increased pressure in the veins
between the intestines and spleen to the liver. Complications include
problems with bleeding and clotting; enlarged weak veins in the esophagus
and stomach; and accumulation of fluid in the abdominal cavity called
ascites. When these complications can no longer be treated effectively, the
child is referred for liver transplantation.
What is the outcome for children with biliary atresia?

If left untreated, the result of blocked bile flow is damage to the liver
such that few children survive beyond the age of two. When bile flow is only
partly restored by surgery, the complications of cirrhosis will gradually
develop. If the Kasai procedure is successful in draining the liver and
returning the bilirubin back to normal, children may live many years with
normal growth and activities. Some will grow into adulthood and some may
never need a transplant.

More often, despite successful surgery, slow, progressive damage to the
liver continues. When progressive cirrhosis occurs, it will eventually
require liver transplantation.

Liver transplantation plays an important role in the long-term treatment of
biliary atresia. Pediatric liver transplantation has evolved into a highly
successful therapy and now offers significant hope for all children born
with biliary atresia


On 8/3/07, Jusuf <[EMAIL PROTECTED]> wrote:
>
>
> Ibue Farhan..
>
> Semoga cepat sehat lagi ded "Nayyara" keponankannya Mba..
>
> bay the way, " Atresia Biliner " itu apa ya ?
> boleh dishare penyebab dan gejalanya...
>
> Regards
> Jusuf, [EMAIL PROTECTED]
>
>
> ----- Original Message -----
> From: "Kartika Nusantari (PS - ENVIRONMENT)" <[EMAIL PROTECTED]>
> To: <balita-anda@balita-anda.com>
> Sent: Friday, August 03, 2007 9:33 AM
> Subject: Re: [balita-anda] Kabar terakhir keponakanku....
>
>
> Mbak, ikut prihatin ya untuk dede' nayya-nya...
> Mudah2an cepet sembuh dan ceria lagi..
> Dan.. mudah2an arti mimpi itu dalam arti yg sebenernya ya.. Dede' nayya
> bisa pulang berkumpul lagi dengan keluarganya.. Amin..
>
> Regards,
>
> Kartika Nusantari
> Environment Section
> PT Tri Polyta Indonesia, tbk.
> Jl. Raya Anyer Km. 123 Ciwandan, Anyer
> Banten
>
> ----- Original Message -----
> From: Bea Cukai <mailto:[EMAIL PROTECTED]>
> To: balita-anda@balita-anda.com <mailto:balita-anda@balita-anda.com>
> Sent: Friday, August 03, 2007 9:26 AM
> Subject: [balita-anda] Kabar terakhir keponakanku....
>
> Dear All,
> Tempo hari aku pernah posting tentang keponakanku yang masih baby umur 1
> bulan 3 minggu yang sempet pula konsultasi dengan Dr Purnawati yang
> ternyata
> divonis " Atresia Biliner "
> Thanks juga buat rekan BA yang dah kasih saran serta perhatian juga
> support
> especially Mbak Sylvia, Mbak Lif Rahayu, Mbak Listi, Jeng Fit-fit dan
> Mbak
> Riva Rayani.
>
> Kemarin hari Minggu, aku dan keluarga mengantar dede kecil yang bernama
> "
> Nayyara Putri Raida Ramdani " ke RSAB Harapan Kita ditangani oleh Dr
> Eva.
> Sampai di RSAB sebelum masuk ruang isolasi aku gendong dan sempet
> menitikan
> air mata  karena sedih sekali anak sekecil ini harus menjalankan operasi
> besar dan akhirnya aku harus berpisah dengan keponakanku tersebut untuk
> diisolasi.
> Hari Selasa jam 09.00 mulai dioperasi sampai jam 15.00 alhamdulilah
> operasinya berjalan lancar.
> Duh ga tega dech rasanya liat selang dimana-mana aku aza ngeliat anak
> kecil
> diinfus rasanya sedih sekali apalagi liat keponakan sendiri hiks...
> Kasihan juga ga boleh minum susu cuma boleh minum air putih dan air teh
> aza.
> Sampai sekarang kondisinya berangsur-angsur pulih BABnya sudah berwarna
> kuning dan BAKnya sudah putih jernih dan kalo diajak ngomong dah bisa
> ketawa.
>
> Cuma yang jadi pikiran aku semalem mimpi dede Nayya itu dah boleh pulang
> kerumah dan kondisinya seperti tidak terjadi apa-apa duh semoga ga
> terjadi
> apa-apa yach.
> Sorry kepanjangan, maaf buat yang tidak berkenan cuma lagi mikirin mimpi
> aza..
>
> Regards,
> Bunda Farhan
>
>
>
>
>
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