Zollinger-Ellison syndrome (ZES) is a rare digestive disorder.

Definition

Zollinger-Ellison syndrome (ZES) is a rare digestive disorder. If you have ZES, you likely have one or more tumors in the first part of the small intestine, the pancreas, or both. These tumors, called gastrinomas, release the hormone gastrin.

This causes the stomach to release too much acid. Stomach acid is needed to break down food. But, too much acid can cause painful peptic ulcers inside the lining of your stomach and intestine. While gastrinoma tumors do cause health problems, they are typically not cancerous tumors.

Epidemiology of Zollinger-Ellison Syndrome

Gastrinomas are the most common islet cell tumours

Approximately 45% of gastrinomas originate in the pancreas, but as many as 35–40% originate in the duodenum and about 10–15% primarily in peripancreatic lymph nodes

Gastrinomas can also occur in the stomach, lung, liver and ovary

In approximately 25% of patients, the gastrinoma is part of MEN-I (in MEN-I, the gastrin-producing tumour is then often found in the duodenum)

Pathophysiology of Zollinger-Ellison Syndrome

In Zollinger-Ellison syndrome, hypertrophy of the stomach mucosa is caused by hypergastrinemia, which increases the number of parietal cells and leads to maximal output of acid.

Acid secretion also is stimulated by gastrin, leading to increased secretion of basal acid. Complications may result from increased acid, such as ulcerations of the gastrointestinal mucosa, malabsorption, and diarrhea. Malabsorption is caused by many factors, such as hypersecretion of gastric acid, inactivated pancreatic enzymes, and precipitation of bile salts.

Risk factors

The following are some known risk factors for an individual developing Zollinger-Ellison Syndrome:

A family history of ulcers or a certain type of tumors

An inherited genetic condition called multiple endocrine neoplasia type 1 (MEN-1) syndrome: MEN-1 is characterized by gastrinomas, increasing susceptibility to Zollinger-Ellison syndrome; about 50% of individuals who inherit MEN-1 are reported to be susceptible to Zollinger-Ellison Syndrome

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one’s chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Causes of Zollinger-Ellison Syndrome

Zollinger-Ellison syndrome is caused by tumors. These growths are most often found in the head of the pancreas and the upper small intestine. The tumors are called gastrinomas. High levels of gastrin cause the production of too much stomach acid.

Gastrinomas occur as single tumors or several tumors. One half to two-thirds of single gastrinomas are cancerous (malignant) tumors. These tumors often spread to the liver and nearby lymph nodes.

Many people with gastrinomas have several tumors as part of a condition called multiple endocrine neoplasia type I (MEN I). Tumors may develop in the pituitary gland (brain) and parathyroid gland (neck) as well as in the pancreas.

Zollinger-Ellison Syndrome Symptoms

Signs and symptoms of Zollinger-Ellison syndrome typically affect adults between 20 and 50 years old. Certain over-the-counter medications used to reduce acid production may mask initial symptoms, however, and delay diagnosis.

Abdominal pain

Diarrhea

Burning, aching, gnawing or discomfort in your upper abdomen

Acid reflux and heartburn

Nausea and vomiting

Bleeding in your digestive tract

Unintended weight loss

Decreased appetite

Complications            

The spread of the tumor to other organs (most often the liver and lymph nodes)

Failure to locate the tumor during surgery

Intestinal bleeding or perforation (hole) from ulcers in the stomach or duodenum

Severe diarrhea and weight loss

Diagnosis and test

If a thorough history and physical examination suggest that the patient may have Zollinger-Ellison syndrome, testing may include one or more of the following:

Blood tests to look for abnormal levels of gastrin

Imaging tests, such as a specialized ultrasound called EUS, CT scan, MRI imaging test, or a specialized scan called somatostatin receptor scintigraphy (sometimes called an Octreotide scan)

Upper endoscopy: A physician uses an endoscope (a long, thin, flexible instrument with a camera) to examine the inside of the upper digestive system, including the esophagus, stomach, and first portions of the small intestines

Treatment

Treatment of choice for Gastrinoma is to remove them surgically where possible. Peptic ulcers must be aggressively treated and controlled prior to surgery. The type of surgery for Gastrinoma depends on the location of the tumor. Since these tumors may frequently occur at more than one spot in the pancreas and the surrounding tissues more than one procedure may be required. The following operative procedures may be utilized to treat Gastrinomas.

Enucleation: Many small Gastrinoma’s in the pancreas may be treated by enucleation alone. This is a procedure of choice for patients that have small tumors (less than 1cm) where the tumor is located on the surface of the pancreas.

Resection of the pancreas: in patients with large tumors a distal pancreatectomy or a Whipple operation may be indicated depending on where the tumor is located in the pancreas.

Duodenal exploration: Gastrinomas often occur in the wall of the duodenum (first part of the intestine) and therefore opening duodenum and carefully feeling it to remove any tumors in this area is important.

Lymph nodes: In some patients, the tumor may be located in the lymph glands outside the pancreas therefore careful palpation and removal of these glands is important at the time of surgery

In some affected individuals with aggressively invasive Gastrinoma, recommended treatment may include the use of certain anticancer drugs (chemotherapy) to help reduce tumor mass and blood gastrin levels. Genetic counseling may be of benefit for affected individuals and their families. Another treatment for this disease is symptomatic and supportive.

Medications

A class of medications called proton pump inhibitors (PPIs) includes

Esomeprazole (Nexium)

Lansoprazole (Prevacid)

Pantoprazole (Protonix)

Omeprazole (Prilosec or Zegerid)

Dexlansoprazole (Dexilant)

PPIs stop the mechanism that pumps acid into the stomach, helping to relieve peptic ulcer pain and promote healing. A health care provider may prescribe people who have Zollinger-Ellison syndrome higher-than-normal doses of PPIs to control acid production. Studies show that PPIs may increase the risk of hip, wrist, and spine fractures when a person takes them long term or in high doses, so it’s important for people to discuss risks versus benefits with their health care provider.

Prevention of Zollinger-Ellison Syndrome

Most cases of Zollinger-Ellison syndrome are sporadic. This means that it does not stem from another condition, and it is not possible either to predict who will have it or ways to prevent it.

However, if there is a family history of MEN1 gene mutation, a doctor may recommend that a person undergoes predictive testing even if they do not have any symptoms.

At the age of 20 years, a person with the MEN1 gene mutation has a 50 percent chance of having symptoms. At the age of 40, the chance of developing symptoms rises to 95 percent, according to the Genetic and Rare Diseases Information Center (GARD).

If a person does not show symptoms by the age of 40 years, there is a good chance that they do not have a MEN1 mutation.

So far, there is no evidence that diet affects the risk of developing the syndrome.

Ataxia telangiectasia (AT) is a complex genetic neurodegenerative disorder.

What is ataxia-telangiectasia?

Ataxia telangiectasia (AT) is a complex genetic neurodegenerative disorder that may become apparent during infancy or early childhood. The disorder is characterized by progressively impaired coordination of voluntary movements (ataxia), the development of reddish lesions of the skin and mucous membranes due to permanent widening of groups of blood vessels (telangiectasia), and impaired functioning of the immune system (i.e., cellular and humoral immunodeficiency), resulting in increased susceptibility to upper and lower respiratory infections (sinopulmonary infections).

Individuals with AT also have an increased risk of developing certain malignancies, particularly of the lymphatic system (lymphomas), the blood-forming organs (e.g., leukemia), and the brain.

In those with AT, progressive ataxia typically develops during infancy and may initially be characterized by abnormal swaying of the head and trunk. As the disease progresses, the condition leads to an inability to walk (ambulation) by late childhood or adolescence.

Pathophysiology

The ATM gene encodes the protein kinase ATM, which is the key regulator of cellular response to double-strand breaks (DSB) in DNA. Therefore, ataxia-telangiectasia symptoms include all the possible consequences of the perturbations in DNA damage response (DDR).

One basic defect associated with the malady is the abnormal sensitivity of ataxia-telangiectasia cells to x-rays and certain radiomimetic chemicals but not to ultraviolet irradiation, which leads to chromosome and chromatid breaks.

Breakpoints are randomly distributed, but nonrandom chromosome rearrangements selectively affect chromosomes 7 and 14 at sites that are concerned with T-cell receptors and heavy-chain immunoglobulin coding and with the development of hematologic malignancies. Such disturbances could account for the frequency of infections and neoplasias.

As has been shown by Guerra-Maranhao et al, ataxia-telangiectasia patients are at high risk of having impaired responses to infection with pneumococci, which may be one of the causes of recurrent sinopulmonary infections in these patients. The authors analyzed the production of antibodies to polysaccharide antigens in patients with ataxia-telangiectasia and found that the levels of immunoglobulin G (IgG) antibodies to serotypes 1, 3, 5, 6B, 9V, and 14 of Streptococcus pneumoniae before and after immunization with 23-valent polysaccharide vaccine were significantly lower than in a healthy population.

Causes of ataxia telangiectasia

AT is caused by alterations (mutations) at specific areas within an individual’s genetic information. Each of us has a large amount of genetic information that is organized into smaller segments known as genes.

Genes provide the necessary instructions that our cells need to perform different functions within our bodies.
AT develops when there are alterations in a specific gene known as ATM, located on chromosome 11 at position q22.3. The ATM gene has the ability to produce an enzyme called a “serine/threonine kinase” that has several important functions:

It acts as a tumor suppressor — the enzyme keeps cells from growing and dividing too quickly and it promotes cell death.

It interacts with other proteins when DNA damage occurs following exposure to ionizing radiation.

The ATM enzyme coordinates DNA repair by activating other proteins that are essential for the repair process to occur.

It is believed that through a combination of these mechanisms, the ATM enzyme aids in the prevention of specific types of cancer, such as leukemia and lymphoma.

Patients with AT carry an alteration in both copies of the ATM gene in all the cells of their body. When both copies of the ATM gene are altered within the cells of an individual with AT, the altered gene copies will make less of the ATM protein or an ATM protein that does not function properly.

As a result, cells are hypersensitive to radiation and instead of repairing damaged DNA, the defective ATM proteins allow for alterations to accumulate in other genes because effective DNA repair is unable to occur. When this happens, individuals with AT are at an increased risk to develop leukemia and lymphoma. Additionally, altered ATM genes may allow cells to die inappropriately, particularly in the cerebellum. This can result in the neurological symptoms that occur in AT.

Risk Factors

Since A-T is an inherited genetic disease and symptoms often start in early childhood, lifestyle risk factors, or things you can change to lower your risk of developing a particular disease, aren’t applicable.

But having A-T raises the risk of developing cancer. Reducing lifestyle risk factors for cancer is even more important for people with the condition and their family members.

People with A-T have about a 40% risk of developing cancer, most likely leukemia or lymphoma. Those two cancers account for 85% of all cancers in people with A-T.1 People with A-T also have an increased risk of developing breast cancer, ovarian cancer, stomach cancer, skin cancer, and bone and soft tissue cancer.

The American Cancer Society says being a carrier of the ATM gene (inheriting one abnormal copy of the gene) has been linked to a high rate of breast cancer in some families. A 2021 review study identified a specific mutation or ATM variant associated with increased breast cancer risk called the V2424G mutation.

General risk factors for cancer include:

Older age

A personal or family history of cancer

Using tobacco

Obesity

Alcohol

Some types of viral infections, such as human papillomavirus (HPV)

Specific chemicals

Exposure to radiation, including ultraviolet radiation (UV) from the sun

Symptoms of ataxia telangiectasia

Symptoms include:

Decreased coordination of movements (ataxia) in late childhood that can include ataxic gait (cerebellar ataxia), jerky gait, unsteadiness

Decreasing mental development, slows or stops after age 10 to 12

Delayed walking

Discoloration of skin areas exposed to sunlight

Discoloration of skin (coffee-with-milk-colored spots)

Enlarged blood vessels in skin of nose, ears, and inside of the elbow and knee

Enlarged blood vessels in the whites of the eyes

Jerky or abnormal eye movements (nystagmus) late in the disease

Premature graying of the hair

Seizures

Sensitivity to radiation, including x-rays

Severe respiratory infections that keep coming back (recurring)

What are possible complications of ataxia telangiectasia?

Other problems that may occur with A-T include:

Weakened immune system

Increased risk for leukemia

Increased risk for lymphoma

Extreme sensitivity to radiation such as from X-rays

Pulmonary disease, growth hormone deficiency, and diabetes

Higher risk of leukemia and lymphoma

How is ataxia telangiectasia diagnosed?

A-T is suspected whenever a child develops signs of ataxia, meaning unsteady walking. Testing of the ATM gene is available, and genetic mutations can be identified in, about 90% of people with A-T. The following tests may be more helpful in determining if someone has A-T or another type of ataxia:

Immunoblotting (ATM protein testing). This is the best test to diagnose A-T. Nearly all individuals with A-T will have very low or no amounts of the protein made by the ATM gene.

Radiosensitivity assay. Since people with A-T have an increased sensitivity to radiation, removing some cells and treating the sample with radiation therapy can help make the diagnosis. It can take up to 3 months to get a result from this test.

ATM kinase activity. This test looks at the activity level of the protein made by the ATM gene. Little to no activity means that there is likely a mutation in the ATM gene.

How ataxia telangiectasia is treated?

Treatment for ataxia-telangiectasia (A-T) is geared toward preventing and managing symptoms. Common treatments include fighting respiratory infections and controlling the spread of and severity of dilated blood vessels.

Home Remedies and Lifestyle

A person with A-T should stay out of the sun, as direct sunlight causes worsening of the widened and dilated blood vessels in exposed areas (telangiectasia).

Nutritional therapies are being looked at for their potential in reducing symptoms associated with A-T including nutritional deficiencies and immune system dysfunction.

A particular nutritional supplement, myo-inositol (also called inositol), has been looked at with respect to its impact on blood sugar management and insulin sensitivity. Inositol is currently used in treating metabolic disorders like diabetes.

Diabetes is a common co-occurring disorder in people with A-T. Inositol may be useful for reestablishing balance in people with A-T who have type 2 diabetes or insulin-resistant diabetes.

Postural Drainage

Respiratory infections can make it even more challenging to clear the airways. Postural drainage is an at-home remedy that can help.

Postural drainage is when you put your head at a lower level than the rest of our body so your bronchial tubes and lungs can drain. You can do this by lying on your back, bending your knees, placing a pillow under your knees, and resting there until you feel relief.

Vitamin E Therapy

Vitamin E is an antioxidant that’s said to protect cells against free radical damage caused by things like environmental toxins, smoke, and pollution. In A-T, a person’s cells are under increased oxidative stress. For people with A-T, vitamin E may be used to provide temporary relief of symptoms.

Prescriptions

Prescriptions for A-T vary widely depending on the symptoms being treated. Prescriptions for A-T are used off-label, meaning there is no approved medication for A-T treatment.

Off-label prescriptions include certain anti-Parkinson’s and anti-epileptic drugs. Research on their usefulness in treating symptoms is limited due to small sample sizes.

Amantadine sulfate is one example. It may help in the treatment of motor symptoms (movement issues) in A-T. One study on 17 children treated with amantadine sulfate found that over 76% of patients had at least 20% symptom improvement after eight weeks.7

Other examples include:

Baclofen for ataxia-related muscle spasms or cramps

Clonazepam or Neurontin (gabapentin) and Lyrica (pregabalin) for dizziness and vertigo, tremors, and rest tremors

Prozac (fluoxetine) for speech and swallowing, and for uncontrolled laughing and crying

Metformin

Metformin lowers blood sugar levels (acting as an anti-hyperglycemic agent). It’s recommended as first-line therapy for type 2 diabetes, which can be a complication associated with living with A-T.

Valium: Valium (diazepam) can be used to help symptoms of slurred speech and involuntary muscle contractions.

Antibiotics: Antibiotics can be used to treat the respiratory infections common in persons with A-T.

Immune-Heightening Medications

Researchers are working in clinical trials to investigate how the following immune-heightening medications may help treat immune-related problems associated with A-T. To date, none have been approved in the treatment of A-T or A-T-specific symptoms.

Examples include:

Levamisole: This experimental cancer drug was pulled from the U.S. market in 2000 due to adverse side effects.

Interleukin-2: This drug regulates white blood cell activity involved in immune response.

Intron (interferon): This drug interferes with your immune system environment in such a way that it signals antiviral activity. It is FDA approved for treating certain cancers and hepatitis B.

Chemotherapeutic agents (e.g., cyclophosphamide): These suppress your immune system.

Antilirium (physostigmine): This drug works on the nervous system to alter signals between nerves and muscles.

Surgeries and Specialist-Driven Procedures

A-T can cause difficulties in drinking, chewing, and swallowing, and tremors can make holding utensils and eating particularly challenging. Treatments for such problems should be advised by a speech-language pathologist.

Dietitians can also help with specific changes like increasing calories and discussing the benefits and drawbacks of certain food supplements.

Gastronomy Tube

To prevent nutritional deficiencies and aspiration, a gastronomy tube (G-tube) can be inserted through the abdomen skin and into the stomach. With early placement, nutritional supplementation allows growth, improves stamina, and decreases the risk of lung damage from aspiration.6

G-tubes are recommended in the following situations:

A child cannot eat enough to grow.

A person of any age cannot eat enough to maintain weight.

Aspiration is problematic.

Mealtimes are stressful or too long, interfering with other activities.

Physical and Occupational Therapy

Physical therapy can be used to help with muscle strength and prevent tightening and shortening in the limbs.

Physical therapy, as well as speech therapy and occupational therapy, can be useful in maintaining certain functions, but cannot prevent their eventual progressive decline

Neurological deterioration eventually leads to needing to use a wheelchair and other adaptive devices. Children and teens may need adaptive equipment to attend school.

Genetic Counseling

Genetic counseling has been said to potentially be of benefit to people with A-T and their families. It can provide education for the patient and family members regarding the possibility and potential consequences of genetic testing for A-T relatives, including siblings. Genetic counseling may also be useful for families seeking support in interpreting results.

How can prevent ataxia telangiectasia?

Since ataxia telangiectasia is the result of a genetic mutation, there is no way to prevent it from occurring. You can take steps to reduce your risk of having a child with a genetic condition by avoiding smoking and avoiding exposure to chemicals. If you plan on becoming pregnant, talk with your healthcare provider about genetic testing to understand your risk of having a child with a genetic condition like ataxia-telangiectasia.

Congenital heart defects (also called CHDs) are heart conditions that a baby is born with.

Definition

Congenital heart defects (also called CHDs) are heart conditions that a baby is born with. These conditions can affect the heart’s shape or how it works or both.

CHDs can be mild or serious. CHDs are the most common types of birth defects. Birth defects are structural changes present at birth that can affect almost any part of the body. They may affect how the body looks, works or both. Birth defects can cause problems in overall health, how the body develops or how the body works.

Critical congenital heart defects (also called critical CHDs or critical congenital heart disease) are the most serious congenital heart defects. Babies with critical CHDs need surgery or other treatment within the first year of life. Without treatment, critical CHDs can cause serious health problems and death. Nearly 1 in 100 babies (about 1 percent or 40,000 babies) is born with a heart defect in the United States each year. About 1 in 4 babies born with a heart defect (about 25 percent) has a critical CHD. Some heart defects don’t need treatment or can be treated easily. But others, like some critical CHDs, may need surgeries over several years.

Types of Congenital Heart Defects

There are many different types of congenital heart defects. Some are commonly called a “hole in the heart” because they involve an abnormal connection between the heart’s chambers. But other types of heart defects involve:

Abnormal heart valves

Blood flow that’s blocked from entering or leaving the heart

Abnormal connections of the blood vessels to the heart

Parts of the heart that are too small to work properly

Types of congenital heart defects include:

Aortic stenosis

Atrial septal defect (ASD)

Atrioventricular canal defect

Bicuspid aortic valve

Coarctation of the aorta (COA)

Ebstein anomaly

Hypoplastic left heart syndrome

Patent ductus arteriosus (PDA)

Patent foramen ovale (PFO)

Pulmonary atresia

Pulmonary stenosis

Tetralogy of Fallot (TOF)

Total anomalous pulmonary venous return

Transposition of the great arteries

Tricuspid atresia

Truncus arteriosus

Ventricular septal defect (VSD)

Sometimes, a child can be born with more than one congenital heart defect.

Congenital Heart Defects Risk factors

Certain environmental and genetic risk factors might play a role in the development of congenital heart disease, including:

Genetics. Congenital heart disease appears to run in families (inherited). It’s associated with many genetic syndromes. For instance, children with Down syndrome often have congenital heart defects. Genetic testing can detect Down syndrome and some other genetic conditions while a baby is still in the mother’s womb.

German measles (rubella). Having rubella during pregnancy may affect how the baby’s heart develops while in the womb.

Diabetes. Having type 1 or type 2 diabetes during pregnancy also may affect a baby’s heart development. Gestational diabetes generally doesn’t increase the risk of congenital heart disease.

Medications. Taking certain medications while pregnant can cause congenital heart disease and other birth defects. Medications linked to heart defects include lithium for bipolar disorder and isotretinoin (Claravis, Myorisan, others), which is used to treat acne. Always tell your health care provider about the medications you take.

Alcohol. Drinking alcohol while pregnant has been linked to an increased risk of heart defects in the baby.

Smoking. If you smoke, quit. Smoking during pregnancy increases the risk of congenital heart defects in the baby.

Causes of Congenital Heart Defects

In around eight out of 10 cases, the reason for the congenital heart defect is unknown. Some of the known causes of CHD include:

Genes – 20 per cent of cases have a genetic cause

Other birth defects – A baby affected by certain birth defects, such as Down syndrome, is more likely to have malformations of the heart

Maternal illness – Illness of the mother during pregnancy (for example, rubella – now rare) may increase the risk of congenital heart disorders

Medication and drugs – Medication (over-the-counter or prescription) or illicit drugs taken by the mother during pregnancy may increase the risk of congenital heart disorders

Alcohol – A mother who drinks large amounts of alcohol during pregnancy may increase the risk of congenital heart disorders

Maternal health – Factors such as unmanaged diabetes and poor nutrition during pregnancy may increase the risk

Maternal age – Babies of older women are more likely to have a birth defect than babies of younger women.

Congenital Heart Defects Symptoms

The symptoms of congenital heart defects include:

Heart murmur

Rapid, fluttering, pounding heartbeats (heart palpitations)

Abnormal heart rhythms (arrhythmia)

Bluish tint to the skin, lips and fingernails (blue baby, cyanosis)

Cool, clammy skin

Fast breathing

Shortness of breath

Fainting

Poor feeding – especially in infants because they tire easily while nursing

Poor weight gain in infants

Fatigue during exercise or activity in older children

Irritability and/or prolonged crying

Abnormal rounding of the nail-bed (clubbing)

Stroke

Complications

Potential complications of a congenital heart defect include:

Congestive heart failure. This serious complication may develop in babies who have a significant heart defect. Signs of congestive heart failure include rapid breathing, often with gasping breaths, and poor weight gain.

Heart infections. Congenital heart defects can increase the risk of infection of the heart tissue (endocarditis), which can lead to new heart valve problems.

Irregular heart rhythms (arrhythmias). A congenital heart defect or scarring from heart surgery may cause changes in the heart’s rhythm.

Slower growth and development (developmental delays). Children with more-serious congenital heart defects often develop and grow more slowly than do children who don’t have heart defects. They may be smaller than other children of the same age. If the nervous system has been affected, a child may learn to walk and talk later than other children.

Stroke. Although uncommon, some children with congenital heart defects are at increased risk of stroke due to blood clots traveling through a hole in the heart and on to the brain.

Mental health disorders. Some children with congenital heart defects may develop anxiety or stress because of developmental delays, activity restrictions or learning difficulties. Talk to your child’s provider if you’re concerned about your child’s mental health.

Diagnosis and test

You or your baby may need one or more of the following tests to determine whether symptoms are caused by a congenital heart defect:

Echocardiography or echo is a painless test that uses sound waves to create moving pictures of the heart. It can help diagnose a heart defect or be used to follow progress over time. Echocardiography used during pregnancy (fetal echocardiography) can sometimes be used to help find a congenital heart defect before a baby is born

Electrocardiogram (EKG or ECG) to evaluate the rhythm of the heartbeat

Cardiac catheterization to measure the pressure and oxygen level inside the heart chambers and blood vessels. This can help the doctor figure out whether blood is flowing from the left side of the heart into the right side of the heart, instead of going to the rest of the body

Chest X-ray toshow whether the heart is enlarged. It can also show whether the lungs have extra blood flow or extra fluid, which can be a sign of heart failure

Genetic testing to determine if particular Genes or genetic conditions are causing the congenital heart defect. Your doctor may refer you or your child to a doctor who specializes in genetic testing

Cardiac MRI to diagnose a heart defect or follow progress over time

Pulse oximetry to estimate how much oxygen is in the blood

Treatment and medications

Depending on the severity and type of congenital heart defect involved, its treatment varies. Some babies may have mild heart attacks that get cured by themselves over time. Other children may have a more severe manifestation of defects that call for extensive treatment. In such cases, your options include:

Medications

Numerous medications can help the heart function more efficiently. Some may also be used to prevent the formation of blood clots and to control an irregular heartbeat.

Implantable Heart Devices

Certain devices such as pacemakers that help regulate an abnormal heart rate and implantable cardioverter defibrillators (ICDs) that can correct irregular heartbeats, can prevent some of the complications associated with congenital heart defects.

Catheter Procedures

These permit doctors to fix or repair congenital heart defects without opening the chest and heart surgically. Here, the doctor inserts a catheter through a vein in the leg and guide it up to the heart. Using small tools threaded through the catheter, s/he then proceeds to correct the defect. With advancement of technology most of heart defects can be closed in the Cathlab with catheter procedures. It decreases risks & complications of cardiac surgery.

Open-Heart Surgery

These may be required if catheter procedures are unable to repair a defect. These can close holes in the heart, widen blood vessels, or repair heart valves.

Heart Transplant

In cases where a congenital heart defect is too complex to be repaired, a heart transplant may be resorted to. During this procedure, a donor’s healthy heart replaces the patient’s heart.

Prevention of Congenital Heart Defects

How can I reduce the risk of a heart defect?

Scientists don’t have all the answers yet as to what causes heart defects other than random gene mutations. But, there are things that place you at higher risk (smoking, alcohol, certain medications, etc.) and these should be avoided during pregnancy. There are otherwise no proven strategies to prevent CHD.

You should follow your healthcare provider’s instructions during pregnancy, including:

Don’t use recreational drugs.

Get all recommended screening tests during pregnancy to detect problems as early as possible.

Manage any health conditions, such as diabetes and phenylketonuria.

Stop smoking and avoid secondhand smoke.

Avoid alcohol.

China had condemn Anthony Blinken comment in South Africa.

China has strongly condemned remarks made in South Africa on Monday by visiting United States Secretary of State Antony Blinken, who described China’s response to a recent visit to Taiwan by Speaker of the US House of Representatives Nancy Pelosi as “overreacting.

Responding to a question during an interview with eNCA, Blinken said: “For many countries around the world, like the US and South Africa, we have very complicated consequential relationships with China.

Our purpose is not to say you have to choose between the United States and China.

The real issue on the Taiwan visit has been to note the reaction, or overreaction, by China during a peaceful visit by a member of our legislative branch, taking into account that many members of our Congress have visited Taiwan in the past.

And then, a military response from China sending ballistic missiles which finally landed in the sea of Japan.

In a statement, the Chinese embassy to South Africa said Pelosi was “the second in line to the US presidency, with her visit to Taiwan constituting a serious violation of the US government’s commitment to the one-China policy.

China said the visit was a major political provocation to upgrade US official exchanges and substantive relations with Taiwan.

She was flown in a military aircraft and escorted by US Navy ships for what she acknowledged herself as an official visit.

The so-called ‘unofficial visit’ and ‘peaceful visit’ argued by the US side, is self-deceptive and preposterous.

Pelosi’s illegitimate visit to Taiwan has grossly infringed on China’s sovereignty, interfered in China’s internal affairs, violated the commitments made by the US side and jeopardised peace and stability across the Taiwan Straits.

China has made it clear many times that we are firmly opposed to Pelosi’s visit.

We had warned the US side that if it continued to go down the wrong path, it would have to bear all the consequences arising from it.

The US made a malicious provocation first and China has been compelled to act in self-defence.

The false accusation of ‘overreaction of the Chinese side’, is nothing but a despicable way of distorting facts and shifting blames, read the statement.

The Chinese government said the counter-measures taken were “justified, necessary and appropriate.

China added: The series of military counter-measures carried out by the Chinese military in waters near the island of Taiwan, are necessary and legitimate to deter the ‘Taiwan independence’ separatist forces to safeguard China’s national sovereignty and territorial integrity.

Meanwhile, China has announced a series of countermeasures against the United States, including sanctions against Pelosi and her immediate family members.

It warned: The US, as the deliberate perpetrator of this crisis and Pelosi as the troublemaker, should and must take full responsibility for this.

China’s measures are also necessary for upholding regional peace and stability, as well as international law and basic norms governing international relations.

If China does not firmly oppose the irresponsible and provocative actions by the US, the purposes of the UN Charter, such as the respect for sovereignty and territorial integrity and other basic norms governing international relations will be reduced to nothing but words on paper.

The vast developing world, which accounts for over 80% of the world’s population, could well become the next target, at any time.

China has described the standoff as a fight against hegemony, against interference and against secession.

Commenting on the Blinken visit, David Monyae, University of Johannesburg professor of political science and international relations and director for the Centre for Africa-China Studies, said The United States is being disingenuous playing double standards.

The recent Nancy Pelosi visit to Taiwan is an unnecessary, provocative and an offensive trip.

It undermined China’s sovereignty and territorial integrity meant to open floodgates for other countries to do the same by visiting Taiwan sharpening divisions with mainland China, said Monyae.

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Unknown gunmen allegedly captured two Ebubeagu militants in Imo state.

Two members of the Ebubeagu militia in Imo State were reportedly captured at the boundary between Izombe and Agwa communities in Oguta local council following a fierce gun battle Sunday.

The Ebubeagu operatives had reportedly positioned themselves on the way from Amakpuruedere Izombe village to Ogborafor Izombe market, trying to prevent the village from participating in the market outing of the yearly Okorosha Izombe festival.

A source confirmed to our correspondent that following an exchange of gunfire between the gunmen and the state militia, which lasted for about two hours, the operatives abandoned three of their vehicles and fled.

According to him, the operatives of Ebubeagu Security Outfit felt that the event would be an avenue for them to come and arrest the masterminds of Friday’s Agwa Police Station destruction where four policemen were killed and vehicles burnt with explosives, some whom they felt would be there because of the closeness of Izombe to Agwa.

On getting to the venue of the cultural festival, the Ebubeagu operatives made attempts to disrupt the event which was resisted by the people.

Unknown to them, some gunmen who got information about their presence in Izombe rushed down to the boundary between Izombe and Agwa where they laid ambush for them.

When they later came down to the boundary after their futile attempt to disrupt the festival, they were confronted by the unknown gunmen who engaged them in a gun duel.

This eventually led to the capture of not less than two Ebubeagu operatives by the unknown gunmen, while some scampered for safety, the eyewitness disclosed.

When contacted for comment, spokesman of the Imo State Police CSP Mike Abbattam was yet to confirm the incident as of press time.

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Peter Obi never a member of any fraternity as rumoured in social media.

Valentine Obienyem, media aide of Peter Obi, presidential candidate of the Labour Party (LP), says the former governor is not a member of the National Association of Seadogs (NAS), better known as the Pyrates Confraternity.

Obienyem responded to the claims of the membership of the LP presidential candidate in a terse text message on Monday.

Not at all, the media aide said after he was asked to respond to trending social media posts suggesting that Obi belongs to the confraternity.

In a now-deleted Facebook post, one Fejiro Oliver, who claimed to be a member of the association, described Obi as a “dedicated Pyrate who has led by the tenets we were baptised with.

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US on Monday released a new African policy to counter China and Russia.

The US on Monday released a new African policy to counter China and Russia’s presence in Africa.

The launch of the policy comes as US Secretary of State, Antony Blinken, visits South Africa, Rwanda and the Democratic Republic of Congo.

The new policy, US policy towards Sub-Saharan Africa, reframes the region’s importance to US national security interests and articulates a new vision for how and with whom the US engages, while identifying additional areas of focus.

It states that the world recognises Africa’s importance which is spurring several engagements on the continent and this presents new opportunities and challenges for US interests in the region.

However, China and Russia see the continent as an arena to challenge the rules-based international order and drive their personal interests, the policy states.

The People’s Republic of China (PRC), by contrast, sees the region as an important arena to challenge the rules-based international order, advance its own narrow commercial and geopolitical interests, undermine transparency and openness, and weaken US relations with African peoples and governments, the policy said.

On the other hand, Russia views the region as a permissive environment for parastatals and private military companies, often fomenting instability for strategic and financial benefit, the report states.

It adds that Russia uses its security and economic ties, as well as disinformation to undercut Africans’ principled opposition to Russia’s further invasion of Ukraine and related human rights abuses.

The document states that some of the longstanding approaches of the US have become insufficient to meet new challenges in a more contested and competitive world.

In order to advance its priorities through the new policy, the US will employ four objectives in Sub-Saharan Africa in the next five years.

These include fostering openness and open societies; delivering democratic and security dividends; advancing pandemic recovery and economic opportunity; and conservation, climate adaptation and a just energy transition.

Additionally, we will broaden our engagements, continuing to invest in the largest states while also deepening our relations with small and medium African states to advance our shared priorities, including the AU’s Agenda 2063.

The U.S. is likely to advance its objectives if Africa’s civil society, including journalists and activists, as well as multilateral bodies and democratic institutions, stand up for shared democratic values, such as transparency, accountability, diversity, equality and equity, women’s rights, and inclusion.

It will also elevate diaspora engagement to strengthen the dialogue between US officials and the diaspora in the United States and support the UN’s Permanent Forum for People of African Descent.

The United States will leverage all of our diplomatic, development, and defense capabilities, as well as strengthen our trade and commercial ties, focus on digital ecosystems, and rebalance toward urban hubs, to support these objectives, it said.

China has become the darling of many African countries with massive infrastructure projects and low-interest loans, all of which have helped grow Chinese influence in the continent.

In Nigeria, Africa’s largest economy, Chinese loans are being used to finance massive rail and road project, most of which are also being constructed by Chinese firms.

Russian influence on the continent manifested when most African countries refused to outrightly condemn Russian invasion of Ukraine or support sanctions against Russia for the invasion.

Russian private miltiary contractors, including the notorious Wagner group is also active in many African countries including the Central African Republic and Mali.

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PDP Presidential candidate Atiku Abubakar seen been guided to his vehicle.

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