MCQs R Us!

New Avatar for this blog!

2012-01-19T14:22:00.001-08:00

The MCQ blog has undergone a bit of a facelift. A new-look template, and a spanky new address to go with the new name as well. From today, MCQ Notes is getting a facelift and is being reborn as MCQs “R” us, much like the Toys “R” Us! A new domain address to link to the blog and also a swanky new email address to get in touch with me!

Hopefully more posts and fun times ahead on this blog!

Stay tuned for more MCQs and MCQey stuff!

VSR: CHILD Syndrome: An Almost Exclusively Female Disease

2012-01-15T01:01:00.001-08:00

What is it?

Congenital hemidysplasia with ichthyosiform erythroderma and limb defects (also known as "CHILD syndrome") is a genetic disorder with onset at birth seen almost exclusively in females.[1]:485 The disorder is related to CPDX2, and also has skin and skeletal abnormalities, distinguished by a sharp midline demarcation of the ichthyosis with minimal linear or segmental contralateral involvement.[1]:501

Image Credits: Journal of Medical Genetics, BMJ

Symptoms:

The acronym CHILD stands for the symptoms of the syndrome:

CH = Congenital Hemidysplasia—One side of the body, most of the time the right side, is poorly developed. The right ribs, neck, vertebrae, etc. may be underdeveloped and the internal organs may be affected.
I - Ichthyosiform Erythroderma—At birth or shortly after birth, there are red patches on the side of the body that is affected. Hair loss on the same side may also be possible.

Image Credits: Scielo

LD - limb defects—Fingers on the hand or toes on the foot of the affected side may be missing. An arm or leg may also be shortened or even missing.

References:

1. Freedberg, et al. (2003). Fitzpatrick's Dermatology in General Medicine. (6th ed.). McGraw-Hill.ISBN 0071380760.

2. Wikipedia: CHILD Syndrome

VSR: Stafne Bone Cyst

2012-01-14T01:54:00.001-08:00

VSR stands for VERY Short Review, a 5 minute run through a topic in order to revise the topic for the purpose of garnering MCQ-solving knowledge.

Stafne bone cavity, also known as static bone cyst, is a developmental defect of the mandible in the form of a lingual depression into which lies an aberrant lobe of the submandibular salivary gland. The developmental defect is entirely asymptomatic and does not change in size, hence the term "static" bone cavity.

Usually, this defect is unilateral, although on rare occasions bilateral defects have been reported.

Stafne bone cavity cannot be palpated manually; it is discovered incidentally during radiographic examination.

On a radiograph, the defect is seen in its characteristic location near the angle of the mandible below the mandibular canal. A similar depression related to the sublingual salivary gland is sometimes found in the anterior region. The Stafne bone cavity appears as a well-defined ovoid or round radiolucency with a wide radiopaque border.

Image Credits

To differentiate a Stafne bone cavity from other lesions, sialography of the submandibular gland is performed by injecting a radiopaque dye into the Wharton's duct. If the dye gets carried through the radiolucency, the diagnosis of Stafne bone cavity is confirmed.

Image Credits

Reference:

LSUHSC School of Dentistry Document on the cysts of jaw. PDF. Must read for a good review of the cysts of the jaw

First X-ray: Imaging Consult

Causes: Large, Tender Liver

2012-01-13T23:59:00.001-08:00

Image Credits

Some of the possible common medical causes of Large tender liver may include:

Hepatitis A

Budd-Chiari Syndrome

Liver flukes

Liver abscesses and cysts

Typhoid and paratyphoid Fever

Viral hepatitis

Intra-abdominal sepsis and abscesses

Hemangioma

Hydatid cyst (especially if infected)

Some of the less common causes of Large tender liver may include:

Bearn-Kunkel syndrome
Dubin-Johnson Syndrome
Katayama fever
Leukemia
Infectious mononucleosis
Pylephlebitis
VLCAD deficiency
Weil syndrome

The Small Pox Timeline

2012-01-12T23:32:00.001-08:00

Between 1967 and 1979: WHO launches massive global vaccination program to eradicate small pox

Must read report from SEARO, WHO. Free PDF

17th May 1975: Last indigenous case in India, occurred in Bihar

24th May 1975: India’s last known case of small pox reported, an importation from Bangladesh.

16th October 1975: Last person known to have been infected with naturally occurring Variola major small pox, Rahima Banu, of Bangladesh.

Rahima Banu, aged 2 years, 1975

23rd April 1977: India declared free from Small Pox by an International Commission for Assessment of Small pox Eradication.

From same SEARO Report quoted above

26th October, 1977: The world’s last naturally case of natural infection by Variola minor small pox, Ali Maow Maalin of Somalia. He became the face of the eradication program for polio in Somalia in 2008.

Ali Maow Maalin, from Britannica

11th August 1978: Janet Parker was the last person to die from Small Pox. Parker died after being accidentally exposed to a strain of smallpox virus that was grown in a research laboratory, on the floor below the Anatomy Department. The event led to the suicide of Professor Henry Bedson, the then Head of the Microbiology Department.

Janet Parker, via Birmingham Mail

8th May 1980: WHO declared that Small Pox has been eradicated from the world!

Image credits

This 1980 photograph taken at the Centers for Disease Control, depicted three former directors of the Global Smallpox Eradication Program as they read the good news that smallpox had been eradicated on a global scale. From left to right, Dr. J. Donald Millar, who was Director from 1966 to 1970; Dr. William H. Foege, who was Director from 1970 to 1973, and Dr. J. Michael Lane, who was Director from 1973 to 1981. The worldwide eradication of this dreaded virus was due to the Smallpox Eradication Campaign of the late 1960’s, early 1970s, whereupon, a mass-vaccination program was instituted across the globe that lead to the declaration in 1978 by a global commission that smallpox had been eradicated, which was officially accepted by the 33rd World Health Assembly in 1980.

SEARO Report, WHO, 2009

From End The Neglect

Ether Day

2012-01-11T11:09:00.001-08:00

On October 16, 1846, William T. G. Morton successfully applied inhaled Ether as an anesthetic to one Mr. Edward Gilbert Abbott who had a neck tumor removed painlessly by Dr. John Collins Warren. This demonstration was held at the operating theater of the Massachusetts General Hospital at the behest of Boston surgeon Dr. Henry Jacob Bigelow.

Ever since, October 16 has been celebrated as the World Ether Day or World Anesthesia Day.

It also marks the beginning of Morton’s obsession with etching his name as the discoverer of Anesthesia, a claim that is quite debatable, to put it mildly.

World Ether Day has been asked in some State PG exams in the early 2000s.

Monument commemorating the alleged first discovery of ether's anesthetic use in Boston.

Mesenteric Cysts: VERY Short Review

2012-01-10T23:52:00.001-08:00

Benevieni, the Italian anatomist was the first to report on Mesenteric cysts following his autopsy on an 8 year old girl in 1907. (1) Mesenteric cyst is one of the rarest abdominal tumors, with approximately 820 cases reported since 1507. The incidence varies from 1 per 100,000 to 250,000 admissions. (2) And that qualifies it as an important topic for the Post Graduation entrance exams.

Pathophysiologically speaking, these are benign proliferations of ectopic lymphatics that have no draining connections and are sequestered in the mesentery or the omentum. Hence, pathophysiologically they are similar to cystic hygromas of the neck.

Other etiologic theories include (Emedicine) (1) failure of the embryonic lymph channels to join the venous system, (2) failure of the leaves of the mesentery to fuse, (3) trauma, (4) neoplasia, and (5) degeneration of lymph nodes. (3)

An oft asked question from this disease is the commonest form: Chylolymphatic Cysts.

The treatment of this condition is surgical enucleation.

References:

1. Mohanty SK, Bal RK, Maudar KK. Mesenteric cyst--an unusual presentation. J Pediatr Surg. May 1998;33(5):792-3.

2. Liew SC, Glenn DC, Storey DW. Mesenteric cyst. Aust N Z J Surg. 1994 Nov;64(11):741-4. Review. PubMed PMID: 7945079.
3. Egozi EI, Ricketts RR. Mesenteric and omental cysts in children. Am Surg. Mar 1997;63(3):287-90.

4. Image Credits: Brown Medical School

2,3-BPG in Hemodialysis

2012-01-10T12:47:00.001-08:00

In a 1998 study, erythrocyte 2,3-BPG concentration was analysed during the haemodialysis process. The 2,3-BPG concentration was expressed relative to the haemoglobin tetramer (Hb4) concentration as the 2,3-BPG/Hb4 ratio. Physiologically, an increase in 2.3-BPG levels would be expected to counteract the hypoxia that is frequently observed in this process. Nevertheless, the results show a 2,3-BPG/Hb4 ratio decreased. This is due to the procedure itself: mechanical stress on the erythrocytes is believed to cause the 2,3-BPG escape, which is then removed by haemodialysis.

Image and Information source: Wikipedia. I know this is not the best of sources, but honestly when I need quick info on some topic I usually start my search from here. It is a good way to start off a search and the go into the more traditionally peer reviewed niches to find more specific information!

CCB and Multidrug Resistance in Tumor Cells

2012-01-10T07:12:00.001-08:00

One of the proposed mechanisms for multidrug resistance in cancer cells is the activation of efflux pumps using p-glycoprotein which pump the drugs out of the tumor cells. Naturally, the desire to use p-gp blockers to reverse this resistance arose.

This gave rise to the concept of the usage of the calcium channel blockers, specifically Verapamil and Desverapamil, in the reversal of multidrug resistant tumor cells.

First-generation agents (eg, cyclosporin, verapamil) were limited by unacceptable toxicity, whereas second-generation agents (eg, valspodar, biricodar) had better tolerability but were confounded by unpredictable pharmacokinetic interactions and interactions with other transporter proteins. Third-generation inhibitors (tariquidar XR9576, zosuquidar LY335979, laniquidar R101933, and ONT-093) have high potency and specificity for P-gp. Furthermore, pharmacokinetic studies to date have shown no appreciable impact on cytochrome P450 3A4 drug metabolism and no clinically significant drug interactions with common chemotherapy agents.

However, only a few phase II and phase II trials are running with the third generation agents and they are yet to show major impact.

Reference:
Thomas H, Coley HM. Overcoming multidrug resistance in cancer: an update on the clinical strategy of inhibiting p-glycoprotein. Cancer Control. 2003 Mar-Apr;10(2):159-65. Review. PubMed PMID: 12712010.

Eichwald Silmer Effect

2012-01-10T00:19:00.001-08:00

This is a rather curious effect in which the Y-chromosome linked major histocompatibility antigens cause female mice of certain inbred strains to reject skin grafts from an otherwise identical male.

So the Eichwald Silmer effect is basically Y-chromosome MHC intolerance!

Homophone Syndromes

2012-01-09T07:34:00.001-08:00

No, I did not mean anything like this:
I just wanted to quote three syndromes I get very easily confused with thanks to their similar sounding names:

Fabry’s Disease: Alpha-galactosidase deficiency

Farber’s Disease: Ceramidase deficiency

Forbe’s Disease: Debranching enzyme deficiency.

Forbe’s disease is also known as Limit Dextrinosis or Cori’s Disease.

Do you know more of these? Then drop me a comment here or shoot me a line at my email: