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We have term baby mother blood group was O positive , antibody negative.

Baby was A positive, antibody test positive . Serum bilirubin at 6 hours was 130 mmol/L .started on intensive phototherapy , but serum bilirubin still high was 160 at 10 hours 

At 24 hours ,serum bilirubin was 220 but direct was 190 mmol/L.

Retic count was only 7%.

Liver enzymes 3 fold more 

Gamma GT was 80 then 110

HB was 15 grams .not much changed over first 3 days.

US abdomen showed mud in gall bladder.

Is this case cholestatic jaundice from start or may be was indirect then becomes direct ?? Inspissated bile syndrome

Especially normal colour if stool.

TORCH screen are negative

Now baby is 1 month old , still direct hyperbilirubinemia, high liver enzymes, US abdomen is normal gall bladder and bile duct 

 

 

 

Hi, Nashwa.  Any dysmorphic features or involvement of other organs (echocardiogram, brain US, ophtalmologic evaluation, vertebral X-ray...).  I remember few years ago a case in our unit that was an Alagille syndrome and direct bilirrubin was raised from the start. I assume that metabolic work-up is normal (hypothiroidism, alfa-1 antitrypsin deficiency). I'm not sure of the role of hemolytic disease as the leading cause of such an early cholestasis in your case.

Please, keep us updated on his clinical course. 

Greetings from Spain.  

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No dysmorphic features, no other organ involved also, neoborn blood screening is normal. 

My question is , Is it possible that inspissated bile syndrome happened in first 24 hours ???

Unfortunately no direct bilirubin done in first day , only done at 24 hours and it was high .

Any suggestions ???

@nashwa I suggest you check iron status (s-ferritin and s-transferin), we had a case some many years back with neonatal hemochromatosis. If I remember correctly, this patient presented with early conjugated hyperbili, a crazily high s-ferritin helped us to set the diagnosis.

 

On 4/29/2022 at 7:13 AM, nashwa said:

is this case inspissated bile syndrome???

Given that this has lasted a month, if infectious causes and basic metabolic screens are unrevealing, monogenic causes of cholestasis are likely, more so if there is consanguinity in this family.

Usually inpissated bile syndrome resolves with in few weeks, so it is unlikely..

If liver enzymes are still high, I suggest to rule out bilirubin transport  disorder like PFIC..Thanks 

  • 2 weeks later...

THIS TOPIC I JUST READ ABOUT NEWBORN WITH  LIVER FAILURE  WITH OR WITHOUT HEMOCHROMATOSIS  

 

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