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![]() طفلة 10 سنوات لديها ارتفاع بسيط في خمائر الكبد، حالة شائعة في سورية
KMG - الأحد, 2008-02-24 02:15 |
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؟ |
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Transaminases + urticaria
my ddx: viral hepatitis
Celiac disease (dermatitis herptiform)
drug-induced hepatitis
wilson's
interesting to think of celiac but he didnt mention any malabsorption complaints or symptoms.
drug induced is n# one here, then comes wilson's
and no neurological signs,
wilson's is out.
it could be gilbert disease
[
glibert causes elevated bilirubin
it's not necessarily acute hepatitis, it may be chronic
the hx of urticaria and high ast may be irrelevant
many cases of celiac don't present with diarrhea
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
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?
autoimmune hepatitis
steroids
wilson's is out
I like your confident tone ,especially when you are wrong
and no transaminitis
?
hemochromatosis
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.
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!!!
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?
stop all medications and observe LFTs periodically
serology
ANA,anti-smooth muscle and anti LKM abs
anti tissue transglutaminase,anti-gliadin abs,anti-endomysial abs
serum and urine copper,serum ceruplasmin,slit lamp examination
serum level
ultimately a biopsy may be needed to reach a dx
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
آسف على التأخير بوضع القيم
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
so, do we have to make all lab tests together when the patient comes to us?
I don't think so!
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.