Keir Starmer mocks Boris Johnson after resignation.

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UK: Boris Johnson has on Thursday officially resigned from his office.

British Prime Minister Boris Johnson has on Thursday officially resigned from his office.

Johnson had earleir rebuffed calls by his Cabinet to step down in the wake of ethics scandals. He gave in after more than 40 ministers quit his government and told him to go.

It was not immediately clear whether Johnson would stay in office while the Conservative Party chooses a new leader, who will replace him as prime minister.

Minutes before the news broke, Treasury chief Nadhim Zahawi called on Prime Minister Boris Johnson to resign just 36 hours after Johnson put him in the job, while another newly appointed Cabinet minister quit her post.

Zahawi said Johnson knew the right thing to do” was to “go now.

Zahawi was appointed late Tuesday to replace Rishi Sunak, who resigned saying he could no longer support Johnson after a series of ethics scandals.

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The vaginal venous plexus is comprised of small veins on the sides of the vagina.

A venous plexus is complex network of interconnected blood vessels. The vaginal venous plexus is comprised of small veins on the sides of the vagina, located near the uterine veins.

Branches of this plexus communicate with the uterine and rectal venous plexuses. The vaginal plexus acts like a tributary, or a vein that drains into another vein.

The vaginal plexus surrounds the bladder and contains some arteries, including the middle vesical artery and the iniferior vesical artery.

The vaginal venous plexus communicates with the hemorrhoidal plexus, a network of veins around the rectum, and the uterine plexus, which arises from the sides of the uterus. The hemorrhoidal network of veins is also called the rectal venous plexus

The vaginal venous plexus pushes blood into a system of internal iliac veins. These veins, located in the pelvis, unite with other veins, including the external iliac vein and the common iliac vein.

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Vaginitis is an inflammation of the vagina resulting from an infection.

Vaginal conditions

Many conditions can affect the vagina. Here’s a look at some of the main ones.

Vaginitis

Vaginitis is an inflammation of the vagina resulting from an infection. It can cause uncomfortable symptoms, such as:

discharge

itching

burning sensation

There are different types of vaginitis, depending on the cause. The most common types include:

Bacterial vaginosis (BV). BV is a bacterial infection that stems from an overgrowth of healthy vaginal bacteria. This can happen when something changes the vagina’s pH level, such as douching. BV isn’t a sexually transmitted infection (STI), but sex with a new partner or multiple partners can increase someone’s risk for developing it. BV may cause white or gray discharge, but it doesn’t always cause symptoms.

Yeast infection. A vaginal yeast infection happens when there’s an overgrowth of a type of yeast called Candida albicans in the vagina. Vaginal yeast infections are very common. Symptoms may include itching, inflammation, and a thick, white discharge that has the appearance of cottage cheese. Yeast infections can usually be treated using over-the-counter (OTC) antifungal medication.

Trichomoniasis. Often referred to as “trich,” trichomoniasis is an STI caused by a parasite called Trichomonas vaginalis. It often causes a green or yellow discharge with a fishy odor, as well as burning and redness. It’s treated with antibiotics. To avoid reinfection, both partners should be treated.

Vaginismus

Vaginismus causes involuntary contractions of the vaginal muscles. The muscle contractions make penetration painful, if not impossible. It often begins when someone first attempts to have intercourse.

There’s no single cause, but it’s often linked to past sexual trauma or emotional factors. For some, the fear of painful sex due to vaginismus can make the muscles contract even more, leading to more pain.

STIs

STIs are transmitted through sexual contact and can affect the vagina and cause symptoms ranging from discharge to genital warts or sores. Some STIs don’t cause any symptoms and are only found during routine screening. Common STIs include:

chlamydia

genital herpes

gonorrhea

genital warts

Vaginal atrophy

Vaginal atrophy causes the tissues of the vagina to shrink and thin, which can narrow the canal and reduce its elasticity. It’s more common during menopause. During menopause, estrogen production drops, reducing the amount of vaginal fluids and interfering with the vagina’s pH.

Atrophy can also happen earlier in life due to other causes of decreased estrogen, such as breastfeeding, ovary removal, and certain medications. It can cause vaginal dryness, painful intercourse, and irritation.

Vaginal prolapse

Vaginal prolapse happens when the vagina stretches or expands, protruding onto other organs. It’s rare that it involves the vagina alone. The tissue that supports the uterus usually stretches as well, causing it to weaken during straining.

Vaginal childbirth, frequent pressure on the abdomen from obesity or strained bowel movements, and menopause can increase risk for prolapse.

There are different types of prolapse that can involve the vagina, including:

cystocele, which involves the front of the vagina and the bladder

rectocele, which involves the back of the vagina and rectum

enterocele, which involves the front of the vaginal wall and small bowel

Vaginal prolapse doesn’t always cause symptoms. But in some cases, it can cause a sense of fullness or heaviness in the pelvis. Others may feel a pulling sensation in the area.

Symptoms usually go away when someone lies down and might get worse when standing, having a bowel movement, or lifting something. Passing urine when sneezing, laughing, or coughing is also possible.

Vaginal cancer

Vaginal cancer is very rareTrusted Source. There are different types of vaginal cancer, but the most common is squamous cell carcinoma that starts in the lining of the vagina. In its early stages, it may not cause any symptoms. But if it spreads, it can cause unusual vaginal bleeding, discharge, or a lump in the vagina.

Two-thirdsTrusted Source of vaginal cancers are caused by the human papillomavirus (HPV). When found early, vaginal cancer can often be treated.

Symptoms of a vaginal condition

Symptoms of a vaginal condition can range from mild to severe and depend on the underlying cause.

The following are some common symptoms:

a change in the amount, color, or odor of vaginal discharge

irritation in or around the vagina

redness

itching

bleeding during or after sex

bleeding between periods

bleeding after menopause

painful intercourse

Vaginal conditions usually respond best to treatment when caught early, so make sure to follow up with a doctor if you notice any of these symptoms.

Tips for vaginal health

While several conditions can affect the vagina, you can reduce your risk for developing many of them.

Follow these tips to lower your risk:

Avoid douching. The vagina naturally cleanses itself. Douching can upset the natural balance of bacteria and fungi, leading to an infection.

Avoid scented soaps and feminine hygiene products. Perfumes in scented hygiene products, such as soaps, pads, and wipes, can irritate the skin and disrupt the pH balance of the vagina. Opt for unscented products instead.

Be sexually responsible. Always use protection with new partners and make sure to follow up with regular STI testing.

Do Kegel exercises. These help to strengthen the pelvic floor muscles, which can help reduce your risk for vaginal prolapse and pelvic floor weakness. Learn how to do them.

Get vaccinated. Speak to your doctor about vaccinations to protect against HPV and hepatitis B, which can be transmitted through sex.

Get regular checkups. See your doctor for regular Pap smears and screenings for cervical cancer and HPV. The U.S. Preventive Services Task Force recommends that women ages 21 to 65 have cervical cancer screening with a Pap smear every three years. Women ages 30 to 65 can lengthen the screening interval to five years if they have HPV testing in combination with a Pap smear.

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The vaginal opening, also called the vaginal vestibule or introitus.

Vagina Overview

When people talk about the vagina, they’re usually referring to the vulva, which is the outer part of the female genitalia. The vulva includes the:

labia

vaginal opening

clitoris

urethra

The vagina is a muscular canal lined with nerves and mucus membranes. It connects the uterus and cervix to the outside of the body, allowing for menstruation, intercourse, and childbirth.

Anatomy and function

Vaginal opening

The vaginal opening, also called the vaginal vestibule or introitus, is the opening into the vagina. It’s located between the urethra and the anus. The opening is where menstrual blood leaves the body. It’s also used to birth a baby and for sexual intercourse.

Vaginal wall

The vaginal wall is made of muscle covered in a mucus membrane, similar to the tissue in your mouth. The wall contains layers of tissue with many elastic fibers. The surface of the wall also contains rugae, which are pleats of extra tissue that allow the vagina to expand during sex or childbirth.

The tissues of the vaginal wall undergo hormone-related changes during the menstrual cycle. The cells in the outer layer of the tissue stores glycogen. During ovulation, this layer is shed. The glycogen is broken down by bacteria and helps maintain a pH level to protect the vagina against potentially harmful bacteria and fungi.

Hymen

The hymen is a thin membrane that surrounds the opening to the vagina. Though hymens can range in shape and size, most are shaped like a half-moon. This shape allows menstrual blood to leave the vagina.

When someone first has intercourse or inserts something into the vagina, the hymen may tear. This can also happen during vigorous exercise.

Certain hymen shapes and types can interfere with menstrual flow, wearing tampons, or having intercourse. These include:

Imperforate hymen. An imperforate hymen completely covers the opening to the vagina, blocking menstrual flow. It needs to be repaired with minor surgery.

Microperforate hymen. A microperforate hymen is a very thin membrane that almost completely covers the vaginal opening. Minor surgery is used to create a larger opening.

Septate hymen. The membrane of a septate hymen includes an extra band of tissue that creates two openings. It’s treated with minor surgery.

Explore the interactive 3-D diagram below to learn more about the vagina.

Pleural effusion is a build-up of excess fluid between the lining of the lungs and chest cavity.

Definition

Pleural effusion is a build-up of excess fluid between the lining of the lungs and chest cavity. This lining is called the pleura. The area between the pleura is the pleural space. The body normally produces a small amount of fluid between the pleura to facilitate breathing.

A pleural effusion occurs when there is more fluid than normal. The excess of fluid may prevent the lungs from fully expanding. As a result, patients may have hard time breathing. Pleural effusion may be caused by irritation, inflammation or infection of the pleura. Pleural effusion may be a symptom of mesothelioma, lung cancer or other diseases.

Types of Pleural Effusion

There are two types of pleural effusion:

Inflammatory: This is caused by a lung disease like pneumonia or lung cancer, both of which irritates the pleura.

Noninflammatory: This is caused by abnormal fluid pressures inside the lungs. The pressure can be caused by congestive heart failure (CHF). In CHF, extra fluid collects inside the lung tissues because of a weak heart muscle. This extra fluid then leaks into the pleural space. Other causes of noninflammatory pleural effusions include kidney disease, liver disease, and malnutrition.

Pathophysiology

The pleural space normally contains 0.1–0.2 ml/kg body weight of fluid, filtered from systemic capillaries down a small pressure gradient. Fluid drains into the systemic circulation via a delicate network of lymphatics and eventually enters the mediastinal lymph nodes.

Fluid may accumulate in the pleural space by a number of mechanisms: increased pulmonary capillary pressure, decreased (more negative) intrapleural pressure (e.g. atelectasis), decreased plasma oncotic pressure (e.g. hypoalbuminaemia), increased pleural membrane permeability and obstructed lymphatic flow (e.g. pleural malignancy or infection).

Risk factors

The following diseases may cause pleural effusion:

Heart failure

Bacterial pneumonia

lung cancer and other tumours with lung metastases

Pulmonary embolism

Radiation therapy to the chest

Nephrotic syndrome

Hypothyroidism

Ovarian tumours

Tuberculosis

Connective tissue disease (for example, rheumatoid arthritis, lupus)

Rarely: heart attack, acute pancreatitis, mesothelioma, sarcoidosis, yellow-nail syndrome, familial Mediterranean fever.

Causes of Pleural Effusion

There are many causes of a pleural effusion. Depending on the cause, your doctor will determine the treatment option. Common causes include:

Autoimmune disorders – Rheumatoid arthritis or lupus can cause pleural effusions

Cancer – Lung cancer as well as other cancers that have spread to the lung can also result in a pleural effusion

Cirrhosis of the liver – When severe scarring has occurs in the liver due to long term exposure to certain substances such as alcohol, the liver’s function is severely impaired, resulting in a build-up of fluid in the pleural space

Congestive heart failure (CHF) – The most common cause of a pleural effusion, CHF occurs as a result of poor heart function and a decreased ability for the heart to pump blood effectively

Infections – Some diseases caused by viruses or bacteria such as pneumonia or tuberculosis can also lead to a pleural effusion

Previous surgery – A pleural effusion is reasonably common after either heart surgery (often Coronary Artery Bypass Grafting or LVAD implantation) or any lung surgery in that side of the chest

Pulmonary embolism – A blood clot causes a blockage in one of the lungs, which can result in a pleural effusion as a result of poor lung function.

Pleural Effusion Symptoms

The most common pleural effusion symptoms in patients with nonexpandable lung were breathlessness and cough, while chest pain was more common among mesothelioma patients without effusions.

Symptoms of pleural effusion can vary from mild to severe. Some patients experience no symptoms at all. The type and severity of symptoms often correlates with the amount of fluid and any underlying breathing conditions or infections.

Common Pleural Effusion Symptoms

Dry cough

Shortness of breath

Sharp chest pain that worsens with cough or deep breathing

Malaise (general feeling of discomfort)

Fatigue

Hiccups

Rapid, shallow breathing

Fever if fluid becomes infected

Although pleural effusions can resolve on their own, it is best to seek treatment if you have breathing changes or new onset of shortness of breath. The pressure from excess pleural fluid can cause a lung to collapse.

If you don’t seek immediate treatment, pleural fluid may become infected. An infection of the pleural fluid is called empyema.

Complications

The potential complications associated with pleural effusion are:

Lung scarring,

Pneumothorax (collapse of the lung) as a complication of thoracentesis,

Empyema (a collection of pus within the pleural space), and

Sepsis (blood infection) sometimes leading to death.

Diagnosis and test

The Lung Center may conduct the following diagnostic tests and procedures:

Chest CT-scan uses a combination of X-rays and computer technology to produce horizontal, or axial, images of any part of the body, including the bones, muscles, fat, and organs.

Chest X-ray uses invisible electromagnetic energy beams to produce images of internal tissues, bones, and organs on film or digital media.

Magnetic resonance imaging (MRI) uses a magnetic field and pulses of radio-wave energy to detect changes in the normal structure and characteristics of organs or tissues.

Positron emission tomography (PET) computed tomography and nuclear scanning.

Kidney and liver function blood tests

Thoracentesis, a needle is inserted through the back of the ribcage into the pleural space to remove fluid or air.

Ultrasound of the chest and heart

Pleural biopsy, a procedure in which a sample of the inner lining of the ribcage is removed with a special biopsy needle or during surgery to determine if infection, cancer, or another condition is present. There are three types:

Needle biopsy or thoracentesis removes a fluid sample with a needle inserted between the ribs. Ultrasound or CT-scan may be used to guide the biopsy needle insertion.

Pleural fluid analysis examines the fluid under a microscope to look for bacteria, protein and cancer cells.

Thoracoscopic biopsy- A special type of telescope (thin, flexible, lighted tube) is inserted into the pleural space under general or local anesthesia. With a direct view inside the chest, the doctor is able to visualize the pleural tissue and take a biopsy to determine the cause of the effusion.

Open biopsy- After a general anesthetic is given, your surgeon makes an incision in the skin and surgically removes a piece of the pleura. Depending on the lab findings, further surgery may be performed.

Treatment for Pleural Effusion

Small effusions that cause no symptoms, or only mild symptoms, may just be left and ‘observed’. Treatment is usually only needed if the effusion causes symptoms such as breathlessness.

A large pleural effusion that makes you breathless can be drained. This is called a pleural fluid aspiration or pleural tap. It is usually done by inserting a needle or tube through the chest wall. A local anaesthetic is injected into the skin and chest wall first to make the procedure painless. This may be a ‘one-off’ procedure to relieve symptoms.

However, in many cases, unless the underlying cause can be treated, an effusion is likely to return within a few weeks. Repeated draining of the fluid, when symptoms become troublesome, is one option.

Depending on the underlying cause, other treatment options that are sometimes considered include:

Pleurodesis: In this procedure, a special chemical (a sclerosant) is injected into the pleural space. This causes inflammation of the pleural membranes and helps them to ‘stick’ together.

This helps to prevent fluid building up again into an effusion. Sclerosing chemicals that are commonly used include tetracycline, sterile talc and bleomycin. Pleurodesis is most often used in the treatment of repeated (recurrent) effusions caused by cancer.

Leaving a permanent drain in place so the fluid can drain out as and when it forms.

An operation to insert a shunt (like an internal drain) to allow the fluid to drain out from the chest into the tummy (abdominal) cavity. This is called a ‘pleuroperitoneal shunt’. It is only occasionally used.

Pleurectomy: This is an operation to remove the pleura. It is sometimes used in people with effusions due to cancer when other treatment options have failed.

Prevention

In some cases, you can prevent pleurisy by preventing the medical condition that causes it. For example, some types of pneumonia can be prevented by vaccination. The risk of lung cancer is reduced by not smoking.

Not all cases of pleurisy can be prevented.

Nigeria: Terrorist attack Kuje prison while president jet out to Senegal for meeting.

According to Homepage news channel, On Wednesday, the president, Major General Muhammadu Buhari (ret. ), traveled from Abuja to Dakar, the capital of Senegal, to attend the summit of the International Development Association for Africa.

Buhari is anticipated to participate in an open dialogue on development challenges, priorities, and transformational efforts during the summit with other African leaders, which will result in the Dakar Declaration as the final product.

The President left for the trip just 24 hours after a terrorist attack targeted a convoy of automobiles carrying his advance team of security guards, protocol officers, and media officers ahead of his travel to Daura for Sallah.

Additionally on Tuesday, bandits stormed the Kuje Correctional Center in Abuja, which allowed detainees to flee the building. In the aftermath of the attack, 64 suspected Boko Haram militants managed to escape from the detention facility.

Buhari would be going to Dakar, according to Senior Special Assistant to the President on Media and Publicity, Garba Shehu, who made the announcement shortly after word of the incidents broke.

The President has traveled 11 times this year, with four of those trips occurring in June alone.

Prior to the 35th Ordinary Session of the Assembly of the Heads of State and Government of the African Union, the President landed in Addis Abeba, Ethiopia, on February 4.

On the 15th of the same month,  Buhari left Abuja to attend the 6th European Union-African Union Summit in Belgium.

On March 1, Buhari left Nigeria for Kenya to attend the 50th anniversary of the United Nations Environmental Programme. In the same month, Buhari went on a medical trip to the United Kingdom where he spent two weeks.

Not embarking on any travels in April, Buhari resumed international travels when he visited Cote D’Ivoire to attend the 16th Session of the Conference of the Parties of the United Nations Convention To Combat Desertification which held on May 9. He also visited the United Arab Emirates to console with the country over the death of Sheikh Khalif bin Zayed Al-Nahyan.

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Hirschsprung’s disease is a rare birth defect that affects the large intestine (bowel).

Definition

Hirschsprung’s disease is a rare birth defect that affects the large intestine (bowel). It is caused by missing some or all of the nerve cells in your intestine. Nerve cells are important because they tell your intestine to push stools through your bowel.

This condition occurs while a baby is in the womb. Cells may be missing from a small part of the intestine near the bottom (anus). Or cells may be missing from a large part of the intestine. This causes stools to back up in the intestine. It causes severe constipation, blockage, and colon infections.

Epidemiology

Hirschsprung’s disease affects approximately 1:5000-8000 live births. In short segment disease, there is a significant predilection for males (M: F of 3.5:1), which reduces with the increasing length of involvement. Interestingly, it is almost never seen in premature infants.

Pathophysiology of Hirschsprung’s disease

Hirschsprung’s disease is where ganglionic cells of the myenteric and submucosal plexuses in the bowel aren’t present proximally from the anus to a variable-length along the large intestine.

The enteric nervous system is derived from the vagal segment of neural crest cells which migrate along the vagus nerve to enter the foregut mesenchyme in a cranial to caudal direction.

The most common accepted aetiology of this disease is due to the arrest of the neuroblast, derived from neural crest cell migration in fetal development between week 8 to 12. It is also accepted that sometimes normal cell migration occurs but the neuroblast fails to properly develop due to apoptosis, improper differentiation, or failure in proliferation.

The aganglionic segment remains in a tonic state leading to failure in peristalsis and bowel movements. Feces in the rectum fail to trigger relaxation of the internal anal sphincter, due to aganglionosis. The accumulation of feces in the rectosigmoid region is responsible for the functional obstruction, which is the cause of many of the symptoms.

It can lead to proximal bowel dilatation which can present as abdominal distension. Increased intraluminal pressure can lead to decreased blood flow and deterioration in the mucosal layer. This stasis can lead to bacterial proliferation and the subsequent complication of Hirschsprung’s enterocolitis, which has a mortality rate of 25-30%. If not recognized early this can lead to sepsis and death.

Types of Hirschsprung’s disease

The two main types of Hirschsprung disease are:

Short-segment: This type is the most common. It affects 80% of people (mostly males) with the disease. Nerve cells don’t form in the last segment of the large intestine.

Long-segment: This type affects males and females equally. It occurs when nerve cells are missing from the large intestine.

Rarely, nerve cells don’t form in the large intestine and part of the small intestine. Healthcare providers call this condition total colonic aganglionosis. A very small number of children have no nerve cells in the intestines at all. This condition is known as total intestinal aganglionosis.

Hirschsprung’s disease causes

Hirschsprung disease occurs when nerve cells in the intestines don’t develop normally before an infant is born. Experts are still studying factors that may cause problems with how these nerve cells grow.

Certain genes increase the chance that a child will have Hirschsprung disease. Experts think that several different genes may play a role.

Risk factors

Factors that may increase the risk of Hirschsprung’s disease include:

Having a sibling who has Hirschsprung’s disease: Hirschsprung’s disease can be inherited. If you have one child who has the condition, future biological siblings could be at risk.

Being male: Hirschsprung’s disease is more common in males.

Having other inherited conditions: Hirschsprung’s disease is associated with certain inherited conditions, such as Down syndrome and other abnormalities present at birth, such as congenital heart disease.

Symptoms of Hirschsprung’s disease

An early symptom in some newborns is failure to have a first bowel movement within 48 hours after birth. Other symptoms may include

Green or brown vomit

Explosive stools after a doctor inserts a finger into the newborn’s rectum

Swelling of the abdomen

Diarrhea, often with blood

Failure to pass their first bowel movement (meconium)

Constipation

Nausea and vomiting

Abdominal pain or distension

Diarrhea

Aganglionic megacolon

Intestinal obstruction

Weight loss

Adducted thumb

Failure to thrive in infancy

Sepsis

Enterocolitis

Symptoms of Hirschsprung disease in toddlers and older children may include

Not being able to pass stools without enemas or suppositories. An enema involves flushing liquid into the child’s anus using a special wash bottle. A suppository is a pill Placed into the child’s rectum.

Swelling of the abdomen.

Diarrhea, often with blood.

Slow growth.

Intellectual disability

Complications

In Hirschsprung disease a part of the large intestine lacks normal nerve cells. This means that digested food and stool can’t move forward through that part of the digestive tract. The large intestine becomes blocked with stool. Your baby will be constipated, or unable to have normal bowel movements.

The blockage creates pressure on the inside of the intestine. This causes part of the intestinal wall to wear thin. Over time, a bacterial infection called enterocolitis can develop in the digestive tract. This is very serious. Symptoms of enterocolitis include:

Fever

Swollen belly

Vomiting

Diarrhea

Bleeding from the rectum

Lack of energy

Call your child’s healthcare provider right away if your child has any signs of enterocolitis.

Diagnosis

A physician will examine your child and obtain a medical history. Other tests may be done to evaluate whether your child has Hirschsprung disease. These tests may include:

Rectal biopsy: This is the only definitive test for Hirschsprung disease. For this test, a very small piece (1-2 mm) of the rectal lining is obtained using a small instrument passed through the anal opening. Sometimes a full-thickness rectal biopsy under anesthesia is recommended, but at other times a rectal suction biopsy without anesthesia is appropriate. There is no sensation for cutting inside the colon so pain-relieving medicines are not needed for the biopsy.

Abdominal X-ray: An X-ray of the child’s abdomen allows physicians to determine if there is a lack of stool at the end of the large intestine near the anus and dilated segments of the large and small intestine. This test may aid in confirming a diagnosis of Hirschsprung disease.

Barium enema: For this test, a fluid called barium (a chalky liquid used to coat the inside of the bowel so that it will show up on an X-ray) is given into the rectum as an enema. An abdominal X-ray of children with Hirschsprung disease should show that the end of the bowel is narrower than areas of the bowel close to the mouth. This test may also show other problems including strictures (narrow areas). Barium enema may appear normal in about 10 percent of children with Hirschsprung disease and is less reliable in infants than in older children.

Anorectal manometry: This test measures nerve reflexes that are missing in Hirschsprung disease.

Treatment for Hirschsprung’s disease

Treatment for Hirschsprung disease depends on the severity of the disease and your child’s overall health. All children with the condition undergo procedures that help eliminate static stool, and ultimately, surgery to remove the portion of the intestine that is malfunctioning.

Most children who have treatment and surgery do very well and go on to have normal, healthy bowel function.

Irrigation to Flush the Intestines

As a first step, doctors will irritate the bowel to remove trapped waste and gas. The irrigation is done by inserting a tube through the anus to flush out the colon. In most cases, this successfully clears the intestines and provides immediate relief to the child. These irrigations are continued until surgery can be done.

Sodium Supplementation

Some patients, particularly those whose entire colon is affected by Hirschsprung disease, may need an ileostomy for many months and sometimes years.

The ileostomy can cause the body to lose sodium, something that your child needs in order to absorb nutrients from what they eat. For that reason, children with an ileostomy are often prescribed sodium supplements by their doctor. Doctors will also monitor your child’s growth carefully to make sure that they are getting the nutrients they need to develop normally.

Surgery

The goals of surgery are to remove the abnormal part of the intestine and to reconnect a healthy part of the intestine to the anus so that the body can pass waste normally. In most cases, the rectum and the sigmoid colon (the last parts of the large intestine) will need to be removed. A very key technical aspect is to preserve the anal canal and anal sphincters which comprise the continence mechanism.

Surgery is typically performed on a healthy infant that has responded well to bowel irrigations. The operation performed while your child is under anesthesia involves both laparoscopic and transanal surgery. This allows the surgeon to reach the bowel through three or four tiny (3-5 mm) incisions on the abdomen (stomach area). This approach is minimally invasive and helps reduce recovery time.

In rare cases, if a child is very ill from enterocolitis (intestinal infection), and irrigations are not working, an ileostomy or colostomy is performed. These ostomy procedures collect waste from the small or large intestines and channel it into a bag attached to the child’s side. This is a temporary measure (lasting from a few months to a few years) that allows the child’s intestines to heal until surgery can be performed.

Your doctor will give you detailed information about how to manage your child’s ostomy.

Prevention

Since the cause of Hirschsprung’s disease is unknown, you cannot prevent or avoid it. However, parents who have the disease can unknowingly pass it on to their children.

Lipodystrophy syndromes are rare disorders marked by a lack of body fat just beneath the skin’s surface.

What is Lipodystrophy?

Lipodystrophy syndromes are rare disorders marked by a lack of body fat just beneath the skin’s surface. Which type a person has is determined by the patterns of fat loss on the body and whether the disease is acquired or genetic. Due to the body’s inability to process fat, lipodystrophy leads to severe metabolic, hormonal, cardiovascular and fat storage disorders. Because the disease affects an individual’s outward appearance, it can also have deep psychological effects that may need to be addressed by a mental health care professional.

People who have lipodystrophy can end up with extra fat deposits in their legs, on the face, the back of the neck, or abdomen, and within the liver (called non-alcoholic fatty liver disease). This happens because the body is unable to maintain a layer of fat beneath the skin’s surface, so the fat he or she consumes concentrates elsewhere in the body.

Pathophysiology of Lipodystrophy

The metabolic alterations seen in patients with lipodystrophies result from an overall decrease in adipose tissue burden. Adipose is a metabolically active tissue that has many physiologic functions. Adipose tissue not only provides insulation but also serves to mediate inflammation and secrete many hormones involved in endocrine regulation. In patients with lipodystrophies, a reduction in adipose leads to a deficiency in certain hormones.

In particular, a decrease in leptin has been described in many lipodystrophy patients. Leptin is a proinflammatory adipokine secreted by adipose tissue. It is often referred to as the “satiety hormone” because it regulates mediators of appetite and energy expenditure. A decrease in leptin, whether in the setting of intrinsic leptin deficiency or lipodystrophy, leads to the downstream complications of hyperinsulinemia, insulin resistance with possible progression to diabetes, hypertriglyceridemia, and hepatic steatosis.

Prevalence

The prevalence in adults varies from 2-60% but for UK adults a recent paper quotes a prevalence of 17%. With increased awareness there are now fewer new cases of LDHIV. A prevalence of 33% has been quoted for highly active antiretroviral therapy (HAART) treated HIV children.

Types of Lipodystrophy

The first type of lipodystrophies includes those that are present at birth:

Congenital generalized lipodystrophy (CGL) is one of the most well-studied lipodystrophy syndromes with over 300 reported cases in the literature. Affected subjects have extreme paucity of adipose tissue from birth, leading to a muscular appearance with prominent veins and an acromegaloid appearance, along with severe acanthosis nigricans, hepatosplenomegaly, and early onset of diabetes, hypertriglyceridemia and steatohepatitis. Marked hypoleptinemia is a universal feature of this disease.

Patients with familial partial lipodystrophy (FPL) have normal fat distribution at birth but usually develop fat loss from the extremities after puberty. The well-studied type of FPL is the Dunnigan variety (FPLD) in which fat loss from the limbs and often the trunk, especially anteriorly and over the breasts, is usually accompanied by excess fat deposition over the face and neck. Metabolic abnormalities are similar to those seen in patients with CGL but may not be as severe in some patients. Since the extent of fat loss is not uniform, circulating leptin levels may also vary from low to normal in these patients.

The second type of this disease includes patients who acquire it during their lifetime:

Acquired lipodystrophies may occur in association with other autoimmune disorders or panniculitis, or may be idiopathic. Patients with acquired generalized lipodystrophy (AGL) may show severe metabolic complications similar to patients with CGL, while patients with acquired partial lipodystrophy (APL), also known as Barraquer-Simons syndrome, who invariably have well-preserved lower body fat, have lesser incidences of metabolic complications compared with other lipodystrophy syndromes. Rare forms of acquired partial lipodystrophy associated with autoinflammatory syndromes may however be associated with significant metabolic abnormalities.

Causes

Inherited lipodystrophy is a genetic disorder, meaning one or both parents typically pass the abnormal gene to their child. The type of lipodystrophy your child has depends on which genes she or he inherited.

Acquired lipodystrophy is not passed down from parent to child, but develops at some point in childhood, adolescence or adulthood. It can be caused by:

Medications

An infection such as measles or pneumonia

An autoimmune disease such as rheumatoid arthritis

The cause of an acquired lipodystrophy can also remain unknown.

Risk factors

Experts do not know the exact cause of many cases of acquired lipodystrophy. Certain medications and autoimmune conditions may increase a person’s risk.

Related autoimmune conditions, beyond those listed just above, and may include:

Autoimmune thyroiditis

Autoimmune hepatitis

Autoimmune hemolytic anemia

Rheumatoid arthritis

Sjogren’s syndrome

Sicca syndrome

The following infections may also increase the risk of developing acquired lipodystrophy:

Chickenpox

Measles

Infectious mononucleosis

Diphther osteomyelitis

Pneumonia

HIV

Lipodystrophy symptoms

Lipodystrophy can affect all or part of the body. Signs and symptoms can include areas of the body that appear thin while other areas may appear to be enlarged. Children with the genetic form of lipodystrophy may have symptoms and signs including

Prominent musculature

Protruding belly

Voracious appetite

Early puberty

Cognitive deficiencies

Growth faster than their peers

Increased bone age

Complications

Metabolic complications in lipodystrophy can be severe and life-threatening. They are more common in those with a greater extent of fat loss, especially the generalised lipodystrophies. CGL and AGL feature reduced levels of leptin, causing over-feeding which results in the abnormal deposition of fat in other organs with associated metabolic complications, such as:

High insulin levels resulting in insulin-resistant diabetes

Hypertriglyceridemia causing recurrent attacks of pancreatitis

Polycystic ovarian syndrome, infertility, and masculinisation in females

Fatty liver disease, which can progress to liver cirrhosis and eventual liver failure

Enlargement of the liver and spleen

Hypertension and cardiovascular disease.

Other complications include:

Kidney disease

Peripheral T-cell cutaneous lymphoma, occuring in 7% of AGL

Significant psychological distress resulting from the appearance of fat loss

Physical discomfort arising from the loss of fat pads from the buttocks and feet.

Lipodystrophy diagnosis

Doctors typically diagnose lipodystrophy by performing a physical exam and asking about symptoms. They may try to eliminate the possibility of other conditions, such as Cushing’s syndrome or anorexia.

The following diagnostic tests can help support the diagnosis and rule out other conditions:

Genetic tests

Tests to check blood chemical profiles

Whole-body MRI to check for patterns of fat loss characteristic of acquired lipodystrophy

Renal biopsy to check for kidney involvement in cases of suspected APL

Treatment

Currently, there is no specific treatment that will permanently replace adipose tissue.

Cosmetic

The reduced amount of adipose tissue and its impact on appearance is distressing to some people. In certain cases, cosmetic interventions and surgery have been used. The best approach to cosmetic surgery in lipodystrophy has not been formally studied.

Dietary

The main treatment focus in lipodystrophy is on a low-fat or low energy diet. The aim of dietary therapy is to reduce the amount of ectopic fat in organs which contributes to insulin resistance and the development of metabolic complications of lipodystrophy. Dietary intervention can produce major improvements in many of the established complications that are associated with ectopic fat such as insulin resistance, high blood fat and liver inflammation.

Leptin

In certain types of lipodystrophy Leptin replacement therapy may be prescribed. Leptin is a hormone which is made by adipocytes (fat cells). Leptin regulates appetite. In patients affected by lipodystrophy leptin levels can be low due to the reduced number of adipocytes and when this happens it may increase appetite. Increased appetite and excess food intake will have a negative effect on metabolism. In such cases Leptin therapy can reduce appetite and improve some of the metabolic complications of the condition. It is most often used in cases of generalised lipodystrophy where there is a near absence of fat.

Psychological support

As a chronic condition and one that is appearance-altering, lipodystrophy can be associated with psychological distress. Some patients may benefit from accessing a variety of psychological support services.

Medication

Where specific complications arise such as diabetes and high triglycerides in the blood, medical therapy specific to those conditions may be recommended. It is not uncommon for lipodystrophy patients with diabetes to require high doses of insulin, in that instance concentrated forms of insulin therapy or sometimes insulin pump therapy may be needed. All of these medical interventions are prescribed in conjunction with a low energy/low-fat diet.

Metabolic Surgery

Among patients with partial lipodystrophy and metabolic complications that are not adequately controlled using conventional therapy, metabolic surgery, specifically has been used effectively. Roux-en-Y gastric bypass reduces the size of the upper stomach and thus supports weight loss. In addition, it improves general metabolic problems like reduction of high blood sugar levels and amelioration of insulin sensitivity in muscles, liver and fat tissues.

Prevention of Lipodystrophy

It is important, having identified aetiological factors, to try to prevent LDHIV. Efforts in this direction are being targeted on:

Increasing awareness amongst doctors and patients, together with regular assessment, may help early identification. Early identification may be assisted by techniques such as MRI.

Choice of antiretroviral regimen, avoiding combinations of PIs and certain NRTIs.

Early treatment and interventions for metabolic changes (as these may promote the LDHIV).

General advice on diet and exercise. This may include the use of supplements, high fiber, and omega 3, etc.

Earlier treatment of the HIV infection may help prevent LDHIV (before AIDS develops and before the CD4 count falls below 200/mm3).