Fig.1: Examples of primary and secondary causes of male hypogonadism. Klinefelter’s syndrome results from the 46,XXY karyotypic abnormality present in approximately 1 in 500 male births. The example of Kallmann’s syndrome is rare isolated deficiency of GnRH, resulting in hypogonadotropic hypogonadism associated with olfactory lobe dysgenesis and anosmia. FSH, follicle-stimulating hormone; GnRH, gonadotropin-releasing hormone; LH, luteinizing hormone; T, testosterone.

Fig. 2: Regulation of testicular function. Note that the single decapeptide gonadotropin-releasing hormone (GnRH) stimulates the release of follicle-stimulating hormone (FSH) and luteinizing hormone (LH). Pulsatile GnRH is required. Chronic exposure downregulates the GnRH receptor and causes impaired FSH and LH release, the mechanism whereby pharmacologic GnRH agonists result in chemical castration. Prolactin excess also results in impaired GnRH pulse generator function and hypogonadotropic hypogonadism. Feedback inhibition of LH secretion is a sex steroid–mediated event, whereas FSH secretion has dual feedback regulation involving inhibition by sex steroids and the Sertoli cell product inhibin. Accordingly, a monotropic elevation of the FSH level (normal LH and testosterone levels) may result from deficient Sertoli cell–spermatogenic function. CNS, central nervous system.

Author: Stephen Kemp, MD, PhD, Professor, Department of Pediatrics, Section of Pediatric Endocrinology, University of Arkansas and Arkansas Children’s Hospital

Introduction

Hypogonadism manifests differently in males and in females before and after the onset of puberty. If onset is in prepubertal males and testosterone replacement is not instituted, the individual has features of eunuchoidism, which include sparse body hair, poor development of skeletal muscles, and delay in epiphyseal closure, resulting in long arms and legs. When hypogonadism occurs in postpubertal males, lack of energy and decreased sexual function are the usual concerns. In females with hypogonadism before puberty, failure to progress through puberty or primary amenorrhea is the most common presenting feature. When hypogonadism occurs in postpubertal females, secondary amenorrhea is the usual concern.

Read More» (eMedicine)

Images Source (1 and 2): Cleveland Clinic

Source: New England Journal of Medicine

What endocrine diagnosis is affecting the identical twin on the right side of the image?

A- Acromegaly

B- Addison’s disease

C- Cushing’s syndrome

D- Hypogonadism

E- Hypothyroidism

Chose your answer?

Sick sinus syndrome (SSS) is a type of bradycardia in which the sinoatrial (SA), or sinus node is not working as it should. The sinus node is a small cluster of cells in the upper right chamber, or atrium, of the heart. It contains special “pacemaker” cells that generate the electrical signals that regulate the pace and rhythm of the heartbeat. These signals travel from the sinus node to the AV node. From the AV node, the signal is conducted along pathways that spread into the ventricles, causing them to contract and pump blood into the lungs and throughout the body.

Sick sinus syndrome (SSS) is not a specific disease, but a group of signs or symptoms that indicate the SA node is not functioning properly.

Some patients with sick sinus syndrome also have rapid heartbeats (tachycardia). Or the heartbeat alternates between too fast and too slow. This is called bradycardia-tachycardia syndrome. Often there is a long pause (asystole) between heartbeats, especially after an episode of tachycardia.

SSS affects about 3 out of every 10,000 people. It becomes more common as we age.

Types of Sick Sinus Syndrome

Sick sinus syndrome may be due to defects in the heart itself, or it can be related to factors outside the heart.

Some medications prescribed to treat high blood pressure, coronary artery disease and other conditions, for example, are known to cause bradycardia. This generally does not cause problems for most people, and drugs that slow the heart rate are useful treatments for some types of arrhythmias or other heart disorders. But they may cause low heart rates and bring out other symptoms of SSS in some individuals, such as elderly people with age-related degeneration of the SA node.

• Sinoatrial block is a type of SSS in which the electrical signals move too slowly through the sinus node.
• Sinus arrest is when the signal from the SA node has a pause in it’s normal pacemaker activity.

Bradycardia also can result from abnormalities of the atrioventricular (AV) node, the part of the heart’s conduction system that conducts electrical signals from the atria to the ventricles. These problems are commonly called heart block.

Symptoms

Many people with sick sinus syndrome have no symptoms, or the symptoms may not seem serious enough for concern. The condition may not be diagnosed until it is in an advanced stage. Typically, the first sign an individual with SSS notices is a consistently slow heart rate or bradycardia. Often, the rate falls to 40 to 50 beats per minute or less before it is noticed.

For more information about the electrical conduction system of the heart and what can go wrong, go to The Normal Heart.

If you experience any sudden, unexplained episodes of fainting, consult your doctor.

Other symptoms may include:

• Fainting (syncope) can occur when the abnormal heart rhythm affects the heart’s ability to pump a sufficient volume of blood to the brain.
• Fatigue and weakness may be signs that the heart is not pumping as well as it should. When the blood supply is inadequate, the body diverts blood to the most vital organs. This reduces blood flow to the muscles of the arms, legs or other parts of the body and the individual may lack energy or be unable to do normal tasks.
• Shortness of breath, or dyspnea. People with this symptom often describe the sensation of not being able to take in enough air, or say they are “out of breath.”
• Chest pains, or angina, that indicate the heart is not getting enough blood.
• Disturbed sleep. The abnormal rhythm of the heart may interfere with sleep and wake people up repeatedly during the night. Some people have a condition called sleep apnea in which they stop breathing off and on during the night. This condition may contribute to SSS and other heart disorders by reducing oxygen supply to the heart.
• Confusion. When the blood supply to the brain is diminished, people may have trouble understanding what is happening around them or make errors in judgment. They also may seem drowsy, have problems with balance or movement, or appear anxious or agitated.
• Palpitations describe an unusual awareness of the beating of the heart. The heartbeat may seem too fast or slow, or to beat in a chaotic, irregular pattern. People may describe a “pounding” in the chest, as if the heart is beating with unusual force. People with SSS who have episodes of tachycardia (an abnormally rapid heartbeat) often describe them as “palpitations.” 

People with unexplained symptoms that may be early signs of sick sinus syndrome should consult their doctor as soon as possible.

Seeing the Doctor

There are some signs that a doctor may discover during a physical examination that suggest a diagnosis of SSS, such as:

• A pulse that is slower than normal.
• An irregular heart rhythm that can be heard with a stethoscope.
• Sinus “pauses,” or gaps between signals from the SA node, that indicate the heart is not being paced at the normal, steady rate.
• “Escape” rhythms are electrical signals that originate from a part of the heart muscle other than the sinus node. If the SA node stops working or doesn’t work properly, another pacemaker at the center of the heart called the AV node may begin signaling the heart to beat. Less often, the lower chambers of the heart also can generate escape rhythms that take over for the function of the SA node.
• A heart rhythm that alternates between slow and fast.
• Low or low-normal blood pressure.

Article Source: Heart Rhythm Society

Image by Mayo Foundation for Medical Education and Research.

The correct answer is letter C- Caput Medusae

Why?

These enlarged veins on his abdomen are consistent with caput medusae.

Biomedical engineers designed a machine that removes the excess sodium and water from blood. If not removed, those items can compromise breathing and heart function. An intravenous catheter withdraws blood and removes fluid before returning it to the body. The process is intended to reduce swelling and ease the load on the heart.

Five million Americans suffer from congestive heart failure, a debilitating illness that can seriously compromise a patient’s quality of life. And as the population ages, the number of people diagnosed with the disease is growing by eight percent each year. Now, as Ivanhoe reports, a new procedure is helping patients feel better and get their condition under control.

Recently, Ken Richard thought he was running out of time. Fluid overload was literally suffocating him. “I’d sit in an easy-chair and I’d press on my back and I couldn’t breathe,” Richard recalls. “I’d lay on the bed and I couldn’t breathe. I’d walk 15 feet — couldn’t breathe.”

The diagnosis — congestive heart failure. Fluid builds up in the body, causing swelling and interfering with the heart’s ability to function. “It was quite serious,” Yvens Laborde, M.D., an internal medicine specialist at Ochsner Medical Center in New Orleans, told Ivanhoe. “The main thing was he actually has a significant compromised cardiac function.”

The diagnosis — congestive heart failure. Fluid builds up in the body, causing swelling and interfering with the heart’s ability to function. “It was quite serious,” Yvens Laborde, M.D., an internal medicine specialist at Ochsner Medical Center in New Orleans, told Ivanhoe. “The main thing was he actually has a significant compromised cardiac function.”

“Oh, it makes an incredible amount of difference for a number of reasons,” Dr. Laborde explains. “Number one, it’s safe, it’s very effective and it’s predictable, and the amount of fluid that you remove, you can remove it very controllably.”

Though this process used to be used only for patients with kidney disease, new studies have found it has a dramatic effect on patients with congestive heart failure and can be more effective than traditional treatment with diuretics.

In 38 hours, Aquadex removed 20 pounds of fluid from ken’s body. “It reduces the amount of congestion and fluid retention and so what happens is it actually enables his heart function — his cardiac function — to improve,” Dr. Laborde says. Now, ken is feeling like himself again ý grateful to have his life back.

Congestive heart failure leads to more than three million hospitalizations every year in the United States, at a cost of more than 23 billion dollars. Doctors say this new procedure actually reduces the amount of time patients spend in the hospital and the chances they’ll have to come back for further treatment.

HAVE A HEART: The heart pumps 5.6 liters of blood through the entire body in roughly 20 seconds; each day your blood travels some 12,000 miles, and your heart beats about 100,000 times. This delivers oxygen and other essential nutrients to the body’s cells and organs.

A heart attack occurs when the blood supply to the heart muscle is cut off, either because part of the heart is damaged (such as the valves to the chambers), or because plaque has built up inside the arteries, narrowing them and severely restricting blood flow. Symptoms of a heart attack include a squeezing discomfort in the center of the chest, pain or tingling in the left arm, shortness of breath, and sometimes a cold sweat, nausea, or dizziness.

ABOUT HEART DISEASE: Most heart diseases arise from hardening of the arteries, especially the buildup of fatty material along the inner lining of the arteries. Coronary arteries supply blood to the heart. When a blockage occurs, this flow is decreased. Heart medications target these blockages in several different ways. Nitrates dilate the veins, decreasing the oxygen requirements of the heart. They also dilate the coronary arteries to increase blood flow to the heart. Beta-blockers decrease the heart rate and the force of the heart’s contractions. Aspirin prevents platelets in the blood from clotting and clumping on blood vessel walls.

Source: ScienceDaily

What is the diagnosis?

A- Subcutaneous Metastases

B- Filariasis

C- Caput Medusae

D- Neurofibromatosis

E- Hepatocellular Carcinoma

Chose your answer?

Source: New England Journal of Medicine

?

The correct answer is letter C - Pituitary tumor.

Why?

The sinus radiograph reveals an enlarged sella turcica. Magnetic resonance imaging confirmed a pituitary macroadenoma that had caused cerebrospinal fluid rhinorrhea.

Source: New England Journal of Medicine

This patient presented with unilateral rhinorrhea. What is the diagnosis?

A- Nasal foreign body

B- Osteoma

C- Pituitary tumor

D- Skull fracture

E- Sinusitis

Chose your answer?

Source: New England Journal of Medicine

?

The correct answer is letter E - Pseudomonas aeruginosa infection.

Why?

Green nails, a form of chromonychia, may be caused by bacterial infection with P. aeruginosa. This syndrome is typically seen in patients with nail disease such as onycholysis, onychotillomania, or paronychia, particularly in those whose abnormal nails have been exposed to moist environments. The green color is caused by the fluorescent siderophore pyoverdin, produced by P. aeruginosa.

Source: New England Journal of Medicine

Every day I’m going to choose a image challenge for you guys… You will have one day to answer, so then I’ll put the correct answer and an article about the topic of the challenge…
And all the credits go to NEJM - New England journal of medicine.
So here comes the first…

Which one of the following conditions is the most likely to be responsible for this clinical picture?

A- Excessive fluoride supplementation

B- Hyperbilirubinemia

C- Treatment with tetracycline

D- Trichophyton rubrum infection

E- Pseudomonas aeruginosa infection

Chose your answer?

-Until now, attention-deficit/hyperactivity disorder (ADHD) was related to alterations in the brain affecting attention and cognitive processes. Researchers at Universitat Autònoma de Barcelona and the Vall d’Hebron University Hospital have now discovered anomalies in the brain’s reward system related to the neural circuits of motivation and gratification. In children with ADHD, the degree of motivation when carrying out an activity is related to the immediacy with which the objectives of the activity are met. This would explain why their attention and hyperactivity levels differ depending on the tasks being carried out.

Susanna Carmona, researcher at the Cognitive Neuroscience Unit of the UAB Department of Psychiatry and Legal Medicine (URNC-IAPS-Hospital del Mar), has worked in collaboration with clinical researchers of the Vall d’Hebron University Hospital on the first research which relates the structure of the brain’s reward system, the ventral striatum, with clinical symptoms in children suffering from ADHD.

Models describing the origin of ADHD tend to emphasise the relevance of attention processes and of the cognitive functions which guide our mental processes in achieving proposed objectives. Nevertheless, recent research has focused on neural gratification/pleasure circuits, which can be found in what is known as the brain’s reward system, with the nucleus accumbens as the central part of this system.

The nucleus accumbens is in charge of maintaining levels of motivation when commencing a task and continues to do so until reaching what experts name the “reinforcement,” the proposed objective. This motivation can be maintained throughout time, even when the gratification obtained is not immediate. However, in children with ADHD motivational levels seem to drop rapidly and there is a need for immediate reinforcements to continue persisting in their efforts.

In this study, researchers selected a sample of 84 participants aged 6 to 18 years and divided them according to presence of ADHD symptoms, with one experimental group of 42 children with ADHD and one control group of 42 children with no signs of mental or behavioural anomalies, paired by sex and age. Magnetic resonance images were taken of all participants to view the structure of their brains. Of these images, the cerebral region corresponding to the ventral striatum, which includes the nucleus accumbens, was demarcated.

Differences in the structure of the ventral striatum — particularly on the right-hand side — could be seen between those with ADHD and those without the disorder. Children with ADHD exhibited reduced volumes in this region. These differences were associated with symptoms of hyperactivity and impulsiveness.

The obtained data corroborate results from previous studies carried out with animals: the importance of the reward system, as well as the relation between nucleus accumbens, impulsive behaviour and the development of motor hyperactivity. This leads researchers to consider that ADHD is not only caused by brain alterations affecting cognitive processes, but also by anomalies which cause motivational deficiencies. This would explain the imbalance in levels of attention and hyperactivity in a child with ADHD depending on his or her motivation when engaged in a specific task and the immediacy of the gratification/pleasure while carrying it out.

Source: Science Daily