Epstein's pearls and Bohn's nodules

Epstein's pearls are tiny cystic lesions of the palate found in approximately 2/3rds of newborns. They are visible over the region of fusion of the posterior palatal segments and are a result of the inclusion of epithelial cells during palatal fusion.  Bohn's nodules are also benign and much less frequent than Epstein's pearls. They occur along the junction of the hard and soft palate or adjacent to the midpalatal raphe and are derived from epithelial remnants of developing palatal salivary glands. Distinction between Epstein's pearls and Bohn's nodule is difficult and clinically irrelevant because both of these lesions regress and require no treatment.

Do not confuse these with the following conditions:

Congenital epulis is a very rare tumor seen in the newborn period (especially in females). It is usually firm and pedunculated and visible on the anterior alveolar ridge of the maxilla. It is benign in nature but may lead to difficulty with feeding and/or respiration requiring excision.

Mucoceles are clear fluid-filled, well-circumscribed, small lesions usually visible on the labial mucosa of the lower lip. These occur as a result of trauma to a submucosal salivary duct, usually from blunt trauma or lip biting. They usually also require no treatment. Large lesions may be de-roofed.

Rett syndrome key points

Rett syndrome
-X-linked dominant (Affects girls).
-Onset is in the second year of life.
-Typically there is regression in motor and language development
-Autistic behaviors are common
-There is acquired microcephaly
-Characteristic hand-wringing behavior

Poor prognosis. No specific treatment.

Vitiligo

Vitiligo is characterized by hypopigmentation of the skin. It is an autoimmune condition that results from melanocyte dysfunction.
Image from Wikimedia Commons.
Available here:http://en.wikipedia.org/wiki/File:Vitiligo2.JPG
Signs and symptoms

-Hypo or depigmented patches that often enlarge and change shape.
-Most prominent on the face, hands and wrists.

Treatment

-UVB phototherapy with or without Psoralen, a material that increases the effect of the UV light. It can be done at home or a few times a week in the clinic.
-Studies have shown that immunomodulators such as topical tacrolimus (Protopic) and pimecrolimus (Elidel) may also cause improvement in some cases, when used with UVB narrowband treatments
-Psoralen and Ultraviolet A light (PUVA) therapy is generally performed in clinic setting.
-Skin camouflage/make up
-Depigmenting with topical drugs like monobenzone, mequinol or hydroquinone may be considered to make the not vitiligous skin appear fairer and uniform. It increases risk of melanoma and consistent sun protection should be advised.

Differential diagnosis

-Pityriasis alba
-Tuberceloid Leprosy
-Post inflammatory hypopigmentation
-Tinea versicolor
-Albinism
-Piebaldism

USMLE pearl: Vitiligo is often associated with other autoimmune diseases including Hashimoto thyroiditis, type I Diabetes Mellitus, Addison's disease, pernicious anemia etc.

Strategies, Tips and Tricks to succeed in the USMLE Step 1 and 2 CK

Five keys to success

1-Know the content
2-Learn how to apply the knowledge at solving multiple choice questions
3-Keep your calm. There will always be questions you will not know the answers to. In fact there will be many!
4-Practice questions and develop expertise at solving multiple choice questions.
5-Know the exam software really well. Practice again and again. You should know where every button is and what they do. You should know exactly how many questions are going to be there in how many blocks. Things keep changing so refer to the latest material from the official website www.usmle.org)

How to approach a question:

1. Read the question stem well

This sounds obvious but believe me (and I am sure others have told you) when I say this is the most important thing. In the stress of the real exam it is easy to overlook key facts. Spend time reading every word. When reading try to pick key facts and if it is a clinical scenario try to think of the diagnosis even if that is not what the question might ultimately ask. It is better to read well in the first go but re-reading is a good idea (although takes more time) if you think you didn't do a good job at the first time around.

2. Guess the answer before reading the options

Once you had read the question stem try to think of the right answer BEFORE reading the options. It is understandably difficult to do this under stress of a real exam but it is well worth trying. This is because most USMLE questions have traps or distractors. Traps are options that sound right but are not. If you read options before giving a good thought to what the answer might be, it might be difficult to think without a bias.

3. Is your answer present in the options provided?

If the answer you thought as above is actually present as one of the options, it is probably right answer but you have to read the other options to make sure that it fits the best. Remember, if two answers seem to be almost the same, then neither one is probably right.

4. If not start excluding the wrong options

If you do not know the right answer try to exclude the wrong ones and narrow down to 2 or 3 options. Depending on how much time you have at hand you might consider re-reading the question stem at this point to find a hint in favor or against any of the options.

5. Guess!

In the end, even after exclusions if you can't reach to a final answer, Guess. There is no negative marking in the USMLE. No answer is as good or bad as wrong answer so simply guess an answer and do not leave any question unanswered.

And on your big  day

1) Be confident but at the same time prepared to feel stupid. You might feel like you did not know more than half the questions. I have multiple friends who cried after the exam thinking they will fail but ended up getting scores >240 (99)

2) Arrive on time. In fact, I recommend you arrive 30-45 minutes before time. Take the scheduling permit and a government issued photo ID. In countries outside the US they sometimes insist on a passport and may not recognize other IDs (for e.g. like driving license).  Don't let any of these be additional anxiety proving issues. The exam itself is enough!

3) Drink strong coffee. Take some along. Also take some snacks with lots of sugar. Remember, glucose is the only fuel our brain can use. It can not use amino-acids, fatty acids etc. unlike other body organs. (You can store everything in the locker which they will provide).

4) Taking breaks: I recommend that at the end of each block when it gives you option to take a break or go to next block, hit 'Take a break'. Then ask yourself: 'Do I need to pee', 'Do I need to eat', 'Do I need to drink'. If the answer to those questions is 'No'. Take a few seconds (or minutes) while sitting on your seat to refresh your brain and tell your self, 'I am ready for more' and then start another block. If the answer is 'Yes', you can leave your seat and do the needful! I recommend taking a slightly longer break of about 10-15 minutes after 3-4 blocks to refresh your brain.

5) Start with question 1 and go till the end. Time yourself so that you do not miss any questions because of lack of time. Every 10 questions or so check to ensure you have enough time for the remaining questions and if not try to speed up.

6) Do not leave a question unanswered to come back to it. You can mark it with a check mark to come back if you have time left at the end of your block and change the answer but do not leave a question without choosing an answer because you may never get back to it.

7) Try not to mark too many questions to come back. If you think that you might get the correct answer if you give it a thought mark it. I would say mark no more than 5-6 questions in a block. One good example is questions on biostatistics that require some math. I always marked a random answer and came back to do the math at the end unless it was very simple. Because it is easy to do the math wrong in a hurry.

8) Some people say when you change an answer, you are more likely to choose a wrong answer. I disagree. In my opinion, if you think a different answer is right, go ahead an change it. In all the practice tests that I had done in preparation for the boards, I found that I changed from a wrong answer to right more often than I did from right to wrong.

Good luck for the test!

Key points to remember about sideroblastic anemia for the USMLE


-Sideroblastic anemia results from hereditary (X-linked) or acquired disorder of heme synthesis.

-It is a microcytic hypochromic anemia with a high RDW (red cell distribution width) Same as iron deficiency anemia.

-Iron studies will distinguish the two. In sideroblastic anemia serum iron, serum ferritin and transferrin saturation are increased.

-The impaired synthesis of heme leads to retention of iron in the mitochondria. The nucleated RBC's show aggregation of iron in the mitochondria and has a perinuclear distribution. These cells are called ringed sideroblasts, which are characteristically seen in sideroblastic anemia.

-Severity can be mild or severe enough to need frequent transfusions.

-Treatment: Pyridoxine helps in some rare inherited cases but manu need periodic transfusions. Stem cell transplantation has been used to treat children who are dependent on blood transfusion.