Paul-Bunnell test - The other name for MONOSPOT test

The mononucleosis spot test looks for two antibodies in the blood that indicate infection with the Epstein-Barr virus (EBV).
. It is used to diagnosis infectious mononucleosis, a disease caused by the Epstein-Barr virus (EBV). About 1 week after the onset of the disease, many patients develop heterophile antibodies. Antibodies reach peak levels in 2 - 5 weeks and may persist for up to 1 year. However, a small number of persons with mononucleosis may never develop such antibodies.
Monospot tests are usually positive in approximately 85% of patients with infectious mononucleosis. Positive test results will not occur until 1 - 2 weeks into the illness.

False-positive results may be occur in persons with:

• Hepatitis
• Leukemia or lymphoma
• Rubella
• Systemic lupus erythematosus (SLE)

COURTESY: http://www.nlm.nih.gov

Algorithm for the management of suspected infectious mononucleosis. (IM = infectious mononucleosis; GABHS = group A β-hemolytic streptococcus; VCA = viral capsid antigen; EBV = Epstein-Barr virus)

Courtesy: http://www.aafp.org/afp/2004/1001/p1279.html

Silly Broncodilators - can they cause hypoxia?

Fall in Arterial Oxygen Pressure

A fall in arterial oxygen pressure (PaO2) has been noted with isoproterenol administration during asthmatic bronchospasm, as ventilation improves and the exacerbation is relieved. 

The same effect has subsequently been noted with newer beta agonists such as albuterol and salmeterol. The mechanism for this seems to be an increase in perfusion of poorly ventilated portions of the lung. 

It is known that regional alveolar hypoxia produces regional pulmonary vasoconstriction in an effort to shunt perfusion to lung areas of higher oxygen tension. 

Administration of inhaled beta agonists may reverse hypoxic pulmonary vasoconstriction by beta 2 stimulation, increasing perfusion to underventilated lung regions. 

Preferential delivery of the inhaled aerosol to better ventilated lung regions increases the ventilation-perfusion mismatch. Oxygen tension falls most in subjects with the highest initial PaO2. Decreases in PaO2 rarely exceed 10 mmHg, and the PaO2 values tend to be on the flat portion of the oxyhemoglobin curve, so that drops in arterial oxygen saturation (SaO2) are minimized. Oxygen tensions usually return to baseline within 30 minutes.

Source: http://www.allbusiness.com/pharmaceuticals-biotechnology/pharmaceutical/14173501-1.html#ixzz1njbKDcNh

 Courtesy Source: http://www.ed4nurses.com/breathsnds.aspx?print=Y : 

Necrotising enterocolitis - Radiological findings

Supine abdominal x-rays are the classical picture needed for diagnosis. 
If NEC is suspected clinically, or there is concern on supine films the following can be done:
-cross-table lateral  or 
-left-lateral decubitus film


What do you see in the pictures?
1. Asymmetrically distributed dilated bowel loops
2.bowel wall edema with thumb printing
3.pneumatosis intestinalis (intramural gas)
4.portal venous gas
5.pneumoperitoneum
6. Free gas if there is peritoneal perforation

Source: Wiki Commons: RadsWiki

GOT SALT? Sodium Claims on Food Labels Claim - What they really mean?

Sodium Claims on Food Labels Claim
Explains what sodium claims on food labels such as “sodium-free” or “low-sodium” mean, as defined in federal guidelines.


“Sodium-free”:                                                  Less than 5 mg sodium per serving
“Salt-free”:                                                          Meets requirements for sodium-free
“Low-sodium”:                                                  140 mg sodium or less per serving
“Very low sodium”:                                          35 mg sodium or less per serving
“Reduced sodium”:                                          At least 25 percent less sodium when compared with the   regular version
“Light in sodium”:                                            50 percent less sodium per serving; restricted to foods with more than 40 calories per serving
“Unsalted, no added salt: not a 
sodium-free food”:                                           No salt is added during processing; the labeled product resembles or substitutes for another product that is normally processed with salt


Source: United States Food and Drug Administration (FDA) Food Labeling Guide. IX. Appendix A: Definitions of Nutrient Content Claims. FDA website.www.fda.gov/Food/GuidanceComplianceRegulatoryInformation/GuidanceDocuments/FoodLabelingNutrition/FoodLabelingGuide/ucm064911.htm. Revised April 2008. Accessed June 21, 2010.

As a rough guide, the maximum amount of salt for babies and children is: (source: national health service, UK

• less than 1g (0.4g sodium) a day from 0-12 months,
• 2g (0.8g sodium) a day from 1-3 years,
• 3g (1.2g sodium) a day from 4-6 years,
• 5g (2g sodium) a day from 7-10 years, and
• 6g (2.4g sodium) a day from 11 years.

Source: CDC

Current dietary guidelines recommend that adults in general should consume no more than 2,300 mg of sodium per day. However, if you are in the following population groups, you should consume no more than 1,500 mg per day.

• You are 40 years of age or older.
• You are African American.
• You have high blood pressure.

Tips for Reducing Sodium in Your Diet

	Buy fresh, plain frozen, or canned "with no salt added" vegetables.
	Use fresh poultry, fish, and lean meat, rather than canned or processed types.
	Use herbs, spices, and salt-free seasoning blends in cooking and at the table.
	Cook rice, pasta, and hot cereals without salt. Cut back on instant or flavored rice, pasta, and cereal mixes, which usually have added salt.
	Choose "convenience" foods that are lower in sodium. Cut back on frozen dinners, pizza, packaged mixes, canned soups or broths, and salad dressings — these often have a lot of sodium.
	Rinse canned foods, such as tuna, to remove some sodium.
	When available, buy low- or reduced-sodium, or no-salt-added versions of foods.
	Choose ready-to-eat breakfast cereals that are lower in sodium.

Source:  http://www.nhlbi.nih.gov/hbp/prevent/sodium/tips.htm

Cri du chat syndrome

Information Courtesy: www.ncbi.nlm.nih.gov
Photo: Wikipedia


Chromosome 5p deletion syndrome; 5p minus syndrome; Cat cry syndrome
The syndrome’s name is based on the infant’s cry, which is high-pitched and sounds like a cat.

• Cry that is high-pitched and sounds like a cat
• Downward slant to the eyes
• Low birth weight and slow growth
• Low-set or abnormally shaped ears
• Mental retardation (intellectual disability)
• Partial webbing or fusing of fingers or toes
• Single line in the palm of the hand
• Skin tags just in front of the ear
• Slow or incomplete development of motor skills
• Small head (microcephaly)
• Small jaw (micrognathia)
• Wide-set eyes
  Intellectual disability (mental retardation) is common. Half of children with this syndrome learn enough verbal skills to communicate. The cat-like cry becomes less noticeable over time.

  Support Groups

  5p- Society -- www.fivepminus.org
  
  Facial features of a patient with Cri du Chat syndrome at age of 8 months (A), 2 years (B), 4 years (C) and 9 years (D)

Brudzinski’s sign

Even though Brudzinski  (a very famous Polish Physician)  described several signs in patients with meningitis, Brudzinski’s neck sign is most the most used test and is  referred to as Brudzinski’s sign.
How to elicit this sign?
With the patient supine, the examiner places one hand behind the patient’s head and places the other hand on the patient’s chest. The examiner then raises the patient’s head (with the hand behind the head)
while the hand on the chest restrains the patient and prevents the patient from rising. Flexion of the
patient’s lower extremities (hips and knees) constitutes a positive sign. Brudzinski’s neck sign is more sensitive than Kernig’s sign. 
How is it caused?

This sign is caused by the  motor nerve roots irritation which pass through inflamed meninges because the roots are brought under tension.

Image from Wikipedia: Meninges of the central nervous system: dura mater, arachnoid, and pia mater.

Cystic Hygroma

A cystic hygroma is a congenital multiloculated lymphatic lesion that can arise anywhere, but is classically found in the left posterior triangle of the neck.his is the most common form of lymphangioma
Cystic hygromas occur in approximately 1% of fetuses between weeks 9 and 16 of pregnancy
Cystic hygromas can occur as an isolated finding or in association with other birth defect. Because the risk for a chromosome abnormality approaches 50% when a cystic hygroma is found prenatally, amniocentesis or CVS (chorionic villus sampling) can be performed to diagnose these conditions.Cystic hygromas that develop in the third trimester (after thirty weeks gestation) or in the postnatal period are usually not associated with chromosome abnormalities. There is a chance of recurrence after surgical removal of the cystic hygroma.
A common association is Turner Syndrome. Severe form of this is HYDROPS.

Image Courtesy: DermAtlas

Video: Youtube

What the HECK is FRECKLE and how to differentiate fromLentigines

These are small pigmented spots on the skin with a clearly-defined edge, surrounded by normal-appearing skin. It is a harmless (benign) hyperplasia of melanocytes which is linear in its spread. is restricted to the cell layer directly above the basement membrane of the epidermis where melanocytes normally reside. 
This is in contrast to the "nests" of multi-layer melanocytes found in moles (melanocytic nevi). Because of this characteristic feature, the adjective "lentiginous" is used to describe other skin lesions that similarly proliferate linearly within the basal cell layer
The picture of lentigines below is from DERM ATLAS

Lentigines VS. Freckles:
Freckles have a relatively normal number of melanocytes but an increased amount of melanin. A lentigo has an increased number of melanocytes. Freckles will intensify and fade with sunlight exposure, whereas lentigines will stay stable in their color regardless of sunlight exposure

The pictures of Freckle below are from Wikipedia

Acanthosis nigricans

Darker, thick, velvety skin in body folds and creases. Rarely can cause itching


Commonest cause: Insulin Resistance (Insulin causes hyperplasia of the skin)
Internal Malignancies can cause this as well

Courtesy:

Avril Nimblett, MS, PA-C

 Acanthosis nigricans in children can be a precursor of type 2 diabetes

Image from Wikipedia

LINGUAL THYROID

The tongue is the most frequent ectopic location of the thyroid gland. Incidence of lingual thyroid varies between 1:3000 and 1:10,000.

Picture Courtesy: http://www.ghorayeb.com/LingualThyroid.html

In the first month of life the anlage of the thyroid gland descends from the posterior dorsal midline of the tongue to its final position in the lower neck. The initial site of descent eventually becomes the foramen caecum, located in the midline at the junction of the anterior (oral) tongue and the tongue base. 

If the embryonic gland does not descent normally, ectopic or residual thyroid tissue may be found between the foramen caecum and the epiglottis.

Rarely, parathyroid glands can be associated with the ectopic thyroid.  Many patients with lingual thyroid lack thyroid tissue in the neck.

Surgical excision or radioiodine therapy can be effective treatments for lingual thyroid, but no treatment should be attempted before radioisotope scan has confirmed that there is adequate thyroid tissue in the neck.  In those patients lacking thyroid tissue in the neck, the lingual thyroid could be excised and auto-transplanted in the neck region. 
Most cases require no treatment.
Keeping TSH and Free T4 in a good range will avoid undue enlargement which can sometime result in respiratory distress

Hereditary angioedema (HAE)

Also known as Quincke's disease - An Autosomal Dominant disease

Caused by a deficiency or dysfunction of the C1 esterase inhibitor, a protein of the complement system

People with hereditary angioedema can develop rapid swelling of the hands, feet, limbs, face, intestinal tract, larynx or trachea

Attacks of swelling can become more severe in late childhood and adolescence.

There is usually a family history of the condition. However, relatives may be unaware of previous cases, which may have been reported as an unexpected, sudden, and premature death

Investigations:

• C1 inhibitor function
• C1 inhibitor level
• Complement components C4 and C2
  Treatment:
  Antihistamines and other treatments used for angioedema are of limited benefit in hereditary angioedema. Epinephrine should be used in life-threatening reactions.

  The most effective treatment is a C1 inhibitor concentrate (not available in the US), but this may not be available. Fresh frozen plasma contains C1 inhibitor and will help an episode, but rarely, the swelling will become worse.

  Berinert P (CSL Behring), which is pasteurized C1 inhibitor, was approved by the F.D.A. in 2009 for acute attacks. Cinryze (ViroPharma), which is nanofiltered, was approved by the F.D.A. in 2008 for prophylaxis. Rhucin (Pharming) is a recombinant C1 inhibitor under development that does not carry the risk of infectious disease transmission due to human blood-borne pathogens.

  Newer treatments attack the contact cascade. Ecallantide (Kalbitor, Dyax) inhibits plasma kallikrein, and was approved by the F.D.A. (but not in Europe) for acute attacks in 2009. Icatibant (Firazyr, Jerini) inhibits the bradykinin B2 receptor, and was approved in Europe and approved by the FDA on August 25, 2011.

  
  

  Prophylaxis:

  Attenuated androgens, such as danazol, can reduce the frequency and severity of attacks by increasing the production of C1 inhibitor.

  Helicobacter pylori, a type of bacteria found in the stomach, can trigger abdominal attacks. Getting rid of the bacteria with antibiotic therapy decreases these abdominal attacks.
  Hereditary angioedema can be life threatening and treatment options are limited.

  Courtesy: www.ncbi.nlm.nih.gov, youtube - HAE.org website and Wikipedia

Gila Monster and GLP!

Gila monster is the only venomous lizard native to the United States and one of only two known species of venomous lizards in North America

Venom is produced in modified salivary glands in the Gila monster's lower jaw, unlike snakes, whose venom is produced in the upper jaw. 
In 2005,  drug exenatide was approved (marketed as Byetta) for the management of type 2 diabetes. It is a synthetic version of a protein, exendin-4, derived from the Gila monster's saliva.
Exenatide led to healthy sustained glucose levels and progressive weight loss. The effectiveness is due to the fact that exenatide is about 50 percent identical to glucagon-like peptide-1 analog (GLP-1), a hormone released from the human digestive tract that helps to regulate insulin and glucagon. The lizard protein remains effective much longer than the human hormone, helping diabetics keep their blood sugar levels under control. 
Exenatide slows the emptying of the stomach and causes a decrease in appetite, contributing to weight loss.
The saliva of the Gila monster contains many chemicals which can be deadly. One of these has been shown to affect memory. Several companies have been researching the abilities of this chemical to help memory loss due to various diseases such as Alzheimer’s, schizophrenia, and ADHD. Gilatide, derived from exendin-4, has been shown to dramatically heighten memory in a study with mice. Gilatide is likely to be researched further to provide help to Alzheimer’s patients.

Source: Wikipedia

Marfan Syndrome

Marfan syndrome  an autosomal dominant genetic disorder of the connective tissue caused by mutations in the FBN1 gene on chromosome 15. People with Marfan's tend to be unusually tall, with long limbs and long, thin finger with dislocated lenses and aortic root dilation 

FBN1, which encodes a connective protein called fibrillin-1

The Fibrillin 1 protein is essential for the proper formation of the extracellular matrix including the biogenesis and maintenance of elastic fibers. The extracellular matrix is critical for both the structural integrity of connective tissue but also serves as a reservoir for growth factors

In addition to being a connective protein that forms the structural support for tissues outside the cell, the normal fibrillin-1 protein binds to another protein, transforming growth factor beta (TGF-β)..TGF-β has deleterious effects on vascular smooth muscle development and the integrity of the extracellular matrix.Secondary to mutated fibrillin there is excessive TGF-β at the lungs, heart valves, and aorta, and this weakens the tissues and causes the features of Marfan syndrome.

Lens Dislocation:

In Marfan's the dislocation is typically superotemporal whereas in  homocystinuria, the dislocation is inferonasal.

The Steinberg sign & Walker-Murdoch sign.

THE STEINBERG SIGN (a):

This test is used for the clinical evaluation of Marfan patients.

Procedure:
Instruct the patient to fold his thumb into the closed fist. This test is positive if the thumb tip extends from palm of hand (see figure a).

THE WALKER-MURDOCH SIGN (b):

This test is used for the evaluation of patients with Marfan syndrome.

Procedure:
Instruct the patient to grip his wrist with his opposite hand. If thumb and fifth finger of the hand overlap with each other, this represents a positive Walker-Murdoch sign (see figure b).

Courtesy: http://palmreadingperspectives.wordpress.com/2011/05/20/hands-signs-in-marfan-syndrome-thin-fingers-hand-shape-hypermobility/ and wikipedia

Café au lait spots

Café au lait (French pronunciation: [kafe o lɛ] "coffee with milk") is a French coffee drink. 
Are so called because the spots are light brown in color ( like coffee with milk)
They are pigmented birthmarks.  The spots of neurofibromatosis,  have a smooth border resembling the "coast of California"

Having six or more café au lait spots greater than 5 mm in diameter before puberty, or greater than 15 mm in diameter after puberty, is a diagnostic feature of neurofibromatosis type I, but other features are required to diagnose NF-1. The incidence of single lesions is high and of no significance - a patient is allowed up to four patches of less than 0.5cm diameter, but no patches greater than 1.5cm diameter.


Café au lait spots: In McCune-Albright's syndrome WILL NOT CROSS THE MIDLINE has a irregular border compared to "Coast of Maine"The patches are usually large, and have irregular borders resembling the "coast of Maine". .

Typical lesions that are often found on the nape of the neck and crease of the buttocks are shown (arrows).

Picture Courtesy: Wikkipedia

Interesting Appendix!

One of the most common causes of severe acute abdominal pain worldwide.
A correctly diagnosed non-acute form of appendicitis is known as "rumbling appendicitis".
The term "pseudoappendicitis" is used to describe a condition mimicking appendicitis. It can be associated with Yersinia enterocolitica.

Picture shows an acutely inflamed and enlarged appendix, sliced lengthwise.

Rovsing's sign
Continuous deep palpation starting from the left iliac fossa upwards (counterclockwise along the colon) may cause pain in the right iliac fossa, by pushing bowel contents towards the ileocaecal valve and thus increasing pressure around the appendix.

Psoas sign or "Obraztsova's sign"
is right lower-quadrant pain that is produced with either the passive extension of the patient's right hip (patient lying on left side, with knee in flexion) or by the patient's active flexion of the right hip while supine. The pain elicited is due to inflammation of the peritoneum overlying the iliopsoas muscles and inflammation of the psoas muscles themselves. Straightening out the leg causes pain because it stretches these muscles, while flexing the hip activates the iliopsoas and therefore also causes pain.

Obturator sign
If an inflamed appendix is in contact with the obturator internus, spasm of the muscle can be demonstrated by flexing and internal rotation of the hip. This maneuver will cause pain in the hypogastrium.

Dunphy's sign
Increased pain in the right lower quadrant with coughing

Kocher's (Kosher's) sign
From the history given, the appearance of pain in the epigastric region or around the stomach at the beginning of disease with a subsequent shift to the right iliac region.

Differential diagnosis
In children
Gastroenteritis, mesenteric adenitis, Meckel's diverticulitis, intussusception, Henoch-Schönlein purpura, lobar pneumonia, urinary tract infection, new-onset Crohn's disease or ulcerative colitis, pancreatitis, abdominal trauma from child abuse, distal intestinal obstruction syndrome in children with cystic fibrosis, typhlitis in children with leukemia;

Courtesy: Wikipedia: http://en.wikipedia.org/wiki/Appendicitis