What is Avoidant Personality Disorder?

Avoidant personality disorder is a personality disorder characterized by apparent avoidance in social situations and relationships because of an unwarranted fear of rejection by other people. People suffering from this personality disorder often manifest low self-confidence, mistrust toward other people and feelings that he or she is a failure.

Individuals who have avoidant personality disorder want to be in a relationship; however, they lack the ability and self-belief that are important in social relationships. So as to defend themselves from the probability of being criticized or ridiculed, they avoid other individuals. This withdrawal of social relations will likely isolate them from significant affairs and acts to strengthen their discomfort and anxiousness in social gatherings.

The actions of people suffering from avoidant personality disorder are marked by social isolation, introversion, mistrust and emotional aloofness.  These individuals are more likely to be careful when they talk and they carry a general feeling of uneasiness in their ways. Usually they are highly conscious and critical about their difficulties in social relationships.

Signs & Symptoms

The following are the seven diagnostic criteria to specify avoidant personality disorder:

1. The individual stays away from work activities that need social contact. They may turn down job interviews and promotions because they think that they lack the ability to fit the job description.
2. The individual is hesitant to involve him or herself in social gathering and activity with no apparent promise that he or she will be received. People who are suffering from this condition presume that people are not trustworthy until they prove that they are. Most people must give people suffering from avoidant personality disorder needs regular support and persuasion so that they will be encouraged to be involved in a social gathering or activity.
3. The individual is afraid of being criticized or ridiculed in social relationships. Therefore, people who have this disorder turn out to be really conscious on actions or behaviors that may indicate rejection. They will run away in a place wherein they assume that other people might disapprove them.
4. The individual always think of criticism or rejection. Much of his or her energy is given in worrying or evading circumstances they presume unsafe and will only cause them embarrassment.
5. The individual is withdrawn in new social interactions because of feelings of meagerness. Low self-confidence weakens their self-esteem in knowing and talking with new people.
6. The individual considers him or herself as socially incompetent. This self criticism is particularly evident when the person must have social encounters with people they don’t know. Individuals suffering from avoidant personality disorder see themselves as unpleasant or lesser to other people.
7. The individual is hesitant to make risks so as to prevent potential embarrassment. People with this disorder look for social interactions that guarantee the best likelihood of approval while lessening the probability of humiliation or criticism. They still may go to a party, for instance; however, they will stay in a corner talking with their dear friends than going to the party with a person they do not know that much.

Causes

The exact cause of avoidant personality disorder is not yet known and may be brought about by many factors like social, heredity and biological factors. The characteristics of this personality disorder usually show in childhood, characterized by extreme introversion and fear when he or she meets new people and circumstances. These signs are also common in normal children at this developmental stage, but if the symptoms persist into adulthood it can indicate a diagnosis of avoidant personality disorder.

The majority of people confirmed to have avoidant personality disorder have a past history of early and prolonged criticism and rejection from their parents. The necessity to have a relationship with their parents that reject them make the avoidant people desire for a social relationship, however, their yearning slowly turns into a defensive shield of protection versus the constant rejections they receive from their parents. Mockery or disapproval by friends farther strengthens their social isolation and adds to their fear of social connection.

Diagnostic Criteria (Tests)

An individual suffering from avoidant personality disorder may have the following:

Avoidant Personality Disorder picture

1. Be easily upset once a person or a group of people ridicule or reject them
2. Delay having extreme and close relationships
3. Hesitant to become connected with others
4. Will not choose jobs or activities that include socializing with people
5. Extremely shy during social gatherings because he or she is afraid to embarrass him or herself.
6. He or she will make possible problems look bad than they are
7. Perceived themselves as not good in social interactions, inferior or unlikable

Here are the following assessment tools beneficial in confirming avoidant personality disorder:

1. Thematic Apperception Test
2. Rorschach Psychodiagnostic Test
3. Millon Clinical Multiaxial Inventory
4. Minnesota Multiphasic Personality Inventory

Treatment Guidelines

Anti-depressant drugs

Anti-depressant drugs can usually make individuals less affected by rejection. Nevertheless, psychotherapy is believed to be the best treatment for avoidant personality disorder. Psychodynamic psychotherapy, which facilitates understanding on the patient’s thoughts and emotions together with cognitive behavioral therapy, can also treat avoidant personality disorder. A mixture of drugs and psychotherapy can be more beneficial compared to single way of treatment.

The prognosis of people suffering from this disorder is good with treatment. They may improve and acquire some social skills and the disorder can get better with proper treatment.

Avoidant Personality Disorder With Out Treatment

Without treatment, an individual suffering from avoidant personality disorder may suffer from total withdrawal and resignation to life. They can become substance abusers or may develop depression.

What is Budd Chiari Syndrome?

Budd Chiari syndrome is a rare disease produced by thrombotic or non-thrombotic hepatic venous outflow blockage. Dr. Budd explained the disease in 1845, and Dr. Chiari gave additional information regarding the primary pathologic explanation of the liver condition in 1899. The three main characteristics of Budd Chiari syndrome are enlargement of the liver, ascites, and sudden pain in the abdomen.

The majority of patients who have this syndrome usually also have the following underlying conditions:

1. thrombotic diathesis
2. polycythemia vera
3. paroxysmal nocturnal hemoglobinuria
4. pregnancy and use of birth control pills
5. tumors
6. chronic inflammatory diseases
7. clotting disorders
8. infections

Signs & Symptoms

The majority of the patients who have Budd Chiari syndrome have the three chief manifestations:

1. Ascites – abdomen becomes distended because of the fluid accumulation in the abdominal cavity
2. Pain in the abdomen – sudden in onset, accompanied by nausea and vomiting
3. Hepatomegaly – enlargement of the liver, blood can flow toward the liver, but not away from it

The accumulation of blood inside the liver can destroy the liver cells. This may subsequently lead to jaundice or yellowish discoloration of the eyes and skin and problems in the kidney.

Causes

Usually Budd Chiari syndrome was brought about by a blood disorder, for instance polycythemia vera or Sickle cell disease. Furthermore, it can also be brought about by the use of birth control pills, pregnancy, liver cancer, liver trauma, infection, and autoimmune diseases.

The causes or underlying conditions that can cause Budd Chiari Syndrome are the following:

1. hepatic vein thrombosis
2. hepatic vein compression caused by a tumor
3. pregnancy and the use of birth control pills
4. infection
5. blood disorders
6. congenital venous webs
7. inferior vena caval narrowing

Treatment

If there is no treatment provided, Budd Chiari Syndrome can seriously injure the liver.

Here are the following treatments for Budd Chiari Syndrome:

1. Drugs that can dissolve blood clots or lessen new blood clot formation can be prescribed.
2. Low sodium intake can regulate the growth of ascites.
3. Special surgeries can alleviate the blockage of blood in the liver.
4. A liver transplant can be a last resort if the liver is severely injured.

Pictures

Abdominal distension (ascites) in Budd chiari syndrome

Picture of Engorged veins in Budd chiari syndrome

What is Tracheoesophageal Fistula?

Tracheoesophageal fistula (TEF) is a condition in which there is an abnormal connection or passageway between the trachea and esophagus. This occurs when the trachea and the esophagus did not separate normally during the embryonic development. Often, this abnormality accompanies esophageal atresia (EA) in which there is no opening between the esophagus and stomach.

About 1 in 3,000 live births are born with this condition. Affected patients are most likely to be premature infants and those born to mothers diagnosed to have excessive amniotic fluid (polyhydramnios) during pregnancy.

Tracheoesophageal Fistula Symptoms

An infant born with TEF exhibits the 3C’s during feeding:

• Coughing
• Choking
• Cyanosis

Other manifestations include:

• Blowing bubbles of copious and frothy mucus in the mouth
• Excessive salivation and drooling
• Abdominal distention
• Respiratory distress

Types

EA with TEF on the lower portion of the esophagus

Approximately, 90% of diagnosed cases have this type. The upper segment of the esophagus ends in a blind pouch while the lower segment of the esophagus and stomach are connected by a fistula to the trachea.

TEF without EA

This type, often referred to as Type H, occurs at an incidence rate of 4.2 %.  The esophagus connects normally to the stomach. However, there is a fistula connecting the esophagus to the trachea.

EA with TEF on the upper portion of the esophagus

This type rarely occurs (0.8%). In here, the upper segment of the esophagus ends in a blind pouch and is connected by a fistula to the trachea.

EA with TEF to both upper and lower portion of the esophagus

Around 0.7% of cases have two fistulas present. The upper segment of the esophagus ends in a blind pouch and is connected by a fistula to the trachea.  There is another fistula which connects the lower segment of the esophagus and stomach to the trachea.

Diagnosis

Diagnosis of Tracheoesophageal fistula (TEF) is made by inserting a nasogastric catheter and obstruction is met as the catheter passes around 10 to 13 centimeter from the nostrils. There is also inability to aspirate gastric contents. On abdominal x-ray, the stomach is seen to be distended with air coming from the trachea.  Other diagnostic tests include barium swallow and bronchial endoscopy. If a radiopaque x-ray is done, the catheter is seen to coil around the upper esophagus.

Treatment and Repair

• Tracheoesophageal fistula (TEF) is corrected through a surgical repair. This involves the closing of the fistula and connecting or anastomosing the ends of the segments of the esophagus together.
• A portion of the colon may be taken and used to connect the segments of the esophagus if these are too far apart from each other. The surgeons may wish to complete the surgery in stages depending on the severity of the case. Simple fistulas may be repaired endoscopically with the use of fibrin glue to seal off the fistula.
• Early surgery lessens the chance for pneumonia to set in and improves the prognosis of the condition. Survivability of patients after corrective surgery of Tracheoesophageal fistula (TEF) can be as high as 90%.

Some important treatment points of Tracheoesophageal fistula (TEF) are

• Patients for Tracheoesophageal fistula (TEF) correction are usually not permitted to take anything by mouth in preparation for the surgery.
• Tracheoesophageal fistula (TEF) patients placed upright to avoid any aspiration.
• Intravenous fluids and antibiotics are prescribed for fluid balance and for any superimposed infections respectively.
• Feeding via a gastrostomy tube may be done 48 hours after the surgery while oral feedings are started 5 to 10 days thereafter.

What is Acute Intermittent Porphyria?

Porphyria is a group of diseases that entails defects in metabolism of heme resulting in too much secretion of porphyrin precursors and pophyrins. Acute intermittent porphyria (AIP), is one of those porphyrias. This hereditary type of porphyria is autosomal dominant. Acute intermittent porphyria is usually caused by decreased levels of porphobilinogen deaminase (PGBD), also called as hydroxymethylbilane synthase. Generally, the low levels of PGBD is insufficient to cause symptoms, but other factors that can activate it such as drugs, dietary changes, and drugs trigger the symptoms. It usually manifest in neuropathies, constipation and mostly, abdominal pain. Usually, patients does not develop symptoms, but the most common is abdominal pain. However, Acute intermittent porphyria patients do not develop symptoms like a rash unlike the majority types of porphyria.

Causes

Acute intermittent porphyria is a product of mutation in the enzyme gene of hydroxymethylbilane-synthase (HMB-synthase), that is also called  uroporphyrinogen I synthase or porphobilinogen deaminase.

Trigger Factors

There are also other triggering factors that are present, such as

1. hormones,
2. dietary changes, and
3. drugs.

Activating factors can sometimes be unidentified.

Acute attacks are often a result of provocation by means of drugs such as

1. sulfonamide antibiotics
2. barbiturates
3. anti-seizure drugs
4. metoclopramide
5. rifampin and
6. alcohol

Attacks may occur during later part of menstrual cycle or after ovulation in women. In an effort to lose weight, reduction of food intake, surgery, infections, and stressful situations may also trigger an attack.

Symptoms

People who inherited Acute intermittent porphyria gene; a mutation in PBGD, does not develop symptoms. Hence, the disease with a symptom could skip generations or is only documented in a single person in a family. The nonspecific symptoms account for the delay in diagnosis and there is high variability in its presentation.

Symptoms attack acutely, often in thirties or forties in a person’s life and are commonly seen in women more than in men. Acute intermittent porphyria attacks develop in hours or days and continue up to days or even weeks and it depends upon the precipitating factors as well as treatment. Skin manifestations are not present. However, there are rare exceptions that Acute intermittent porphyria patients with advanced renal failure develops plasma porphyrins elevation and blistering lesions.

Severe abdominal pain

In Acute intermittent porphyria, severe abdominal pain is the most common symptom. It is usually steady, severe, and poorly localized. However, it is occasionally cramping and it frequently is accompanied by constipation and other signs of ileus; abdominal distention, vomiting, nausea, and decrease in bowel sounds.

Other symptoms in Acute intermittent porphyria

1. Pain in arms and legs as well as in the back
2. Muscle weakness caused by damage in the nerves that supplies the muscles
3. Urinary retention
4. Confusion, seizures and hallucinations
5. Palpitation

Diagnosis

This disease is unusual and can copy symptoms of other common conditions, it usually is not suspected. On a side note, a false positive also happens. Misdiagnosis is common when there is an incorrect theory of having a porphyria.  A finding of Porphobilinogen (PBG) and Delta-aminolevulinic acid (ALA) levels that are increased in urine institutes that any of the acute porphyrias is present. The diagnosis of Acute intermittent porphyria is present if there is a deficiency in PBGD levels in a normal red blood cell. However, it is not a reliable marker of Acute intermittent porphyria because not all Acute intermittent porphyria patients illicit a deficient PBGD in their red blood cells.

If a known relative or direct family has been diagnosed with Acute intermittent porphyria, the enzyme PBGD is relatively low in the red blood cells. There are also cases in which low levels of the enzyme PBGD in red blood cells is transferred genetically to their offspring. Identified latent cases are advised to avoid  the cause and take specific precautions to prevent acute attacks. However, some families with AIP, has a deficient PBGD level in their liver and some tissues and is normal in red blood cells. False low values sometimes happen because of problems in collection and transport of the sample.

DNA, the cellular material that records all the information of the genes of an individual. A lot of mutations had been identified in the part of the DNA which entails the gene for PBGD. Almost every Acute intermittent porphyria family has a different kind of mutation in their gene. Within that family, there is an individual who inherits a same mutation of low levels of PBGD. It is in an advantage to know the exact kind of mutation in a certain family because that knowledge is the basis for identification of the gene carriers of AIP in a DNA test. This is a much precise method than measurement of PBGD enzyme activity in a red blood cell. During the present time, Acute intermittent porphyria and other porphyria testing through DNA tests is only available in some research laboratories.

Treatment

Hospitalization is needed for the acute attacks of Acute intermittent porphyria. Generally, close observation is required and medications for vomiting and nausea, and pain is given.

Diet Modification

To suppress the activity of the disease, high intake of carbohydrates or glucose are given and it can either be given intravenously or per orem.

Heme therapy

Heme therapy given intravenously is more effective in suppressing activity of the disease. It is much better if heme is started in an early stage of the attack and the response of the AIP patient is better. Thus, waiting for the effectivity of the glucose  therapy and delaying giving heme treatment is not advisable unless it is a mild attack.

Water & Salt balance

During an attack treatment, water and salt balance must be monitored and other potentially harmful drugs must be stopped. These medications are sulfonamides, barbiturates and many more. Attacks are often caused by low carbohydrate and calorie intake in an attempt to lessen weight meaning dietary measures are very important. Attacks of pre-menstruation resolves quickly by manipulating hormones.

Acute intermittent porphyria is dangerous if there is no confirmation of the diagnosis and the patient is given harmful drugs. Prognosis is usually better if diagnosis is done in early stages and treatment as well as preventive measures are started before there is some presence of nerve damage. Some people develop chronic pain even if the usual thing is that symptoms resolve when Acute intermittent porphyria attack happens. After a severe attack, progress in muscle weakness and nerve damage improves after several months or longer. Mental changes can also occur, but are not chronic.

A medic alert bracelet is also important as the disease is lifelong. However, it is advisable for Acute intermittent porphyria patients who had some attacks, but are not as important in latent cases. Other diseases can also develop and it may not always be related to porphyria.

Diet

Diet in Acute intermittent porphyria patients include intake of normal or high carbohydrates. As such, intake of carbohydrates must not be restricted even for a short period of time.

If AIP patient desires weight loss, it is advisable to consult to a  doctor and to a dietician to round up the normal caloric intake for the certain patient. It is appropriate to prescribe a diet 10% below normal caloric levels for the patient. Gradual weight loss will be attained and pophyria attacks are still prevented.