طفلة 10 سنوات لديها ارتفاع بسيط في خمائر الكبد، حالة شائعة في سورية

|

الوصف الكامل Background: 

الشكوى الرئيسية CC: 

ارتفاع طفيف في خمائر الكبد كشف صدفة أثناء اختبارات لمعاناتها من شرى urticaria.

القصة المرضية HPI: 

تعاني المريضة من شرى urticaria مزمن يتحرض بعوامل بيئية مختلفة، يترافق الشرى مع حكة ويستجيب جيداً على مضادات الهيستامين.

الأجهزة الأخرى ROS: 

السوابق المرضية الشخصية PMH: 

التهاب أنف تحسسي.

السوابق المرضية العائلية FMH: 

الوضع الصحي والاجتماعي SH: 

الفحص السريري Clinical Exam: 

لا يوجد تبدلات مهمة.

التشخيص التفريقي DD: 

مضمن بالأسفل included below.

الاستقصاءات Investigations: 

الفحوصات الدموية:

CBC طبيعي. ارتفاع طفيف في الحمضات. الكريات البيض: 6.7 cells/mcL3، اللمفاويات 37%، الحمضات: 5%، هيموغلوبين: 12.5، الصفيحات: 308 آلاف.

الـ AST من 71 إلى 100 وحدة خلال قياسات 7 أشهر ماضية متتالية. بدأ الارتفاع مع أخد دواء الـ cetirizine. كذلك الـ ALT بحدود الـ 35 وحدة دولية.

اختبارات لاحقة، تحديث 26 شباط 2008:

أجري اختبار الـ ANA وكان إيجابياً (1:160)، من النمط المتجانس homogeneous. أجريت اختبارات أخرى كانت طبيعية أو سلبية هي: أضداد العضلات الملساء، العامل الرثياني، الاختبارات الحيوية لالتهابات الكبد A, B and C، الاختبارات الحيوية لـ: CMV, EBV. اختبارات الدرق وإيكو الكبد.

بعد النتائج السابقة، تم التوجه نحو الداء الزلاقي، ويتم طلب: anti tissue transglutaminase,anti-gliadin abs,anti-endomysial abs كما ذكر الدكتور هاشم، وكانت النتائج:

The patient's celiac panel revealed a normal total immunoglobulin A (IgA) level (141 mg/dL) but was positive for IgA anti-tissue transglutaminase antibody (tTG; > 597.6 units [normal, < 20]) and IgA anti-endomysial antibody (EMA; 1:640 [normal, < 1:5]).

التدبير Managment: 

كتابة حرة وطرح موضوع النقاش!: 

تكمل المعلومات بعد الإجابة عن السؤال التالي:

في مثل هذه القصة، ضع فقط قائمة التشخيص التفريقي المناسب لو سمحت.

موضوع مناقشة جديد، 26 شباط 2008:

ما التشخيص المؤكد للداء الزلاقي بعد النتائج المخبرية السابقة؟



Transaminases + urticaria
my ddx: viral hepatitis
Celiac disease (dermatitis herptiform)
drug-induced hepatitis
wilson's

صورة Ghufran


virl hepatitis will make liver enzyms over one tousend at least. so it's not the answer i guess.
interesting to think of celiac but he didnt mention any malabsorption complaints or symptoms.
drug induced is n# one here, then comes wilson's

صورة dr.tabban


and no neurological signs,
wilson's is out.

صورة dr.tabban


it could be gilbert disease



Cetirizine may cause abnormal hepatic function ( less than 2% ) ; cholestasis , hepatitis as adverse effects .



[

اقتباس:
it could be gilbert disease

glibert causes elevated bilirubin
اقتباس:
virl hepatitis will make liver enzyms over one tousend at least

it's not necessarily acute hepatitis, it may be chronic

the hx of urticaria and high ast may be irrelevant

صورة Ghufran


اقتباس:
he didnt mention any malabsorption complaints or symptoms

many cases of celiac don't present with diarrhea
اقتباس:
and no neurological signs,
wilson's is out.

u should think about wilson in such an age 2 prevent development of wilson esp. neurological manifestations. u can't say it's out without labs

صورة Ghufran


you forgut one another important possibility: A specific acquired liver disease happening in females more.It could happen at any age and there is very effective and life saving treatment for it?
Can anyone think of it.
Also the hereditary liver diseases is not complete? can someone complete it?

صورة ABIM


اقتباس:
you forgut one another important possibility: A specific acquired liver disease happening in females more.It could happen at any age and there is very effective and life saving treatment for it?

autoimmune hepatitis
steroids

صورة Dr_Ayyad


اقتباس:
and no neurological signs,
wilson's is out

I like your confident tone ,especially when you are wrong

اقتباس:
glibert causes elevated bilirubin

and no transaminitis

اقتباس:
Also the hereditary liver diseases is not complete? can someone complete it

?
hemochromatosis

صورة Dr_Ayyad


Still the a very important hereditary disease is not thought of.
Remember hemochromatosis menifest later in life > 40-50( and in males before females so it does not fit here .
Let me give a hint about this other hereditary disease. In its sever form can lead to emphysema!!!

In regard to Wilson: I was taught that ANY liver enzyme elevation in patients younger that 35 you can include Wilson in your differential : no matter whether neurologic manifestation are present or not. The other clue in Wilson is actually the Alk Phos will be low-low normal.

As for Autoimmune hepatitis : it is a good thought and should be inculded in differential diagnosis.

صورة ABIM


اقتباس:
Remember hemochromatosis menifest later in life > 40-50( and in males before females so it does not fit here .
Let me give a hint about this other hereditary disease. In its sever form can lead to emphysema

oops alpha1-antitrypsin def

hemochromatosis is a late presenting disease for sure and is much less common in females than in males,I was talking in general not about this particular patient!!!

صورة Dr_Ayyad


OK
THere is a very good list of differential diagnosis.
How do we prove of disprove each of the above mentioned dieases(Medication side effects, Autoimmune hepatitis, alph-1 antitrypsine def,wilson disease, viral hepatitis, celiac disease..)
What tests do you want to order?

صورة ABIM


اقتباس:
Medication side effects

stop all medications and observe LFTs periodically

اقتباس:
Viral hepatitis

serology

اقتباس:
Autoimmune hepatitis

ANA,anti-smooth muscle and anti LKM abs

اقتباس:
celiac disease

anti tissue transglutaminase,anti-gliadin abs,anti-endomysial abs

اقتباس:
wilson disease

serum and urine copper,serum ceruplasmin,slit lamp examination

اقتباس:
alph-1 antitrypsine def

serum level

ultimately a biopsy may be needed to reach a dx

صورة Dr_Ayyad


that is a very good start
Dear KMG . Do you have the results for us ?
Or can we take them from medscape?? Just kidding
Waiting for your reply about the test results oredered by Dr_Ayyad

صورة ABIM


آسف على التأخير بوضع القيم Embarrased

صورة KMG

صورة KMG


I prefer to see the results together and see how we get the diagnosis together. You gave the final Diagnosis quickly.
I don't know if you remember Dr_Ayyad back in the discussion of one case I mentioned that and you responded that this was news to you.
Anyhow we have 3 steps to go through
1- The labs test to excule other possibilities (esp Autoimmine hepatitis which is rather a difficult diagnosis to make or exclude)
2- The proces of confirming the diagnosis with both insavie and non invasive testing
3- Finally telling the progosis and the recommendation to the patient and her family

صورة ABIM


himmm..

so, do we have to make all lab tests together when the patient comes to us? Rolling Eyes

I don't think so!

صورة KMG


The gold standard to confirm Celiac disease is : endoscopic small bowel biopsy (duodenal biopsy) . Hallmarks of the biopsy are : subtotal villous atrophy + crypt hyperplasia reversing on gluten-free diet .



Can you elaborate more on your question dear KMG.
I am not sure I got you.
Eventually the diagnosis method relys on 2 things
Exclusion:and
Inclusion (or confirmation)
If you go back to the initial case discription (from medscape) you will find the this is exactly what they did.
Remember also one improtant rule
The presence of one problem does not prevent the presence
of other problems: diseases could co-exist

Typically I personally do the tests on 2 steps:
The first step I test for all liver enzymes and function + the most common causes of their elevation (viral hepatitis) + US for fatty liver.

If that does not answer my question then the second step is to send for everything else(autoimmune hepatitis, hereditary liver diseases, celiac) + follow up after stopping the possibly offending drug.

There are many limitations sometimes (financially speaking) and that could push you to a slower pace and more steps in test ordering.(spreading the testing over longer period of time) but with this you also risk the patient may not follow up or get false reassurance.
Remember of instance for autoimmune hepatitis patient used to die in 2 years or so but if treatment is started early they live for almost a normal life span.

I don't know if that answer your question? or not or if I understood what you are aiming at.

صورة ABIM