Treatment Options for Thalassemia Major

Before chronic transfusions are initiated, the diagnosis of B0-thalassemia should be confirmed and the parents counseled about this lifelong therapy. Initiating transfusion and chelation therapy can be difficult for parents to face early in their child’s life.

If there is the possibility of a bone marrow transplant, the blood should be negative for cytomegalovirus and irradiated.

Blood Transfusion therapy:

Transfusion therapy promotes general health and well-being and avoids the consequences of ineffective erythropoiesis. A transfusion program generally requires monthly transfusions, with the pretransfusion hemoglobin level >9.5 and <10.5 g/dL. In patients with cardiac disease, higher pretransfusion hemoglobin levels may be beneficial. Some blood centers have donor programs that pair donors and recipients, decreasing the exposure to multiple red cell antigens.

Transfusional hemosiderosis causes many of the complications of thalassemia major. Accurate assessment of excessive iron stores is essential to optimal therapy. The serum ferritin level is useful in assessing iron balance trends, but does not accurately predict quantitative iron stores. Undertreatment or overtreatment of presumed excessive iron stores can Continue reading “Treatment Options for Thalassemia Major”

Introduction to Liver Abscess

Liver AbscessA liver abscess occurs when bacteria or protozoa destroy hepatic tissue, producing a cavity, which fills with infectious organisms, liquefied liver cells, and leukocytes. Necrotic tissue then walls off the cavity from the rest of the liver.
Liver abscess occurs equally in men and women, usually in those older than age 50. Death occurs in 15% of affected patients despite treatment.
Underlying causes of liver abscess include benign or malignant biliary obstruction along with cholangitis, extrahepatic abdominal sepsis, and trauma or surgery to the right upper quadrant. Liver abscesses also occur from intra-arterial chemoembolizations or cryosurgery in the liver, which causes necrosis of tumor cells and potential infection.
The method by which bacteria reach the liver reflects the underlying causes.
Biliary tract disease is the most common cause of liver abscess. Liver abscess after intra-abdominal sepsis (such as with diverticulitis) is most likely to be caused by hematogenous spread through the portal bloodstream. Hematogenous spread by hepatic arterial flow may occur in infectious endocarditis. Abscesses arising from hematogenous transmission are usually caused by a single organism; those arising from biliary obstruction, by mixed flora. Patients with metastatic cancer to the liver, diabetes mellitus, or alcoholism are more likely to develop a liver abscess. Continue reading “Introduction to Liver Abscess”

Folic acid deficiency anemia

A common, slowly progressive megaloblastic anemia, folic acid deficiency anemia is most prevalent in infants, adolescents, pregnant and lactating females, alcoholics, elderly people, and people with malignant or intestinal diseases.
Folic acid deficiency anemia results from a decreased level or lack of folate, a vitamin that’s essential for red blood cell production and maturation. Causes include:
  • alcohol abuse (may suppress metabolic effects of folate)
  • inadequate diet (common in alcoholics, elderly people who live alone, and infants, especially those with infections or diarrhea)
  • impaired absorption (due to intestinal dysfunction from such disorders as celiac disease, tropical sprue, and regional jejunitis and from bowel resection)
  • bacteria competing for available folic acid
  • overcooking, which can destroy a high percentage of folic acids in foods
  • limited storage capacity in infants
  • prolonged drug therapy (with anticonvulsants and estrogens)
  • increased folic acid requirement during pregnancy, during rapid growth in infancy (common because of increased survival rate of preterm infants), during childhood and adolescence (because of general use of folate-poor cow’s milk), and in patients with neoplastic diseases and some skin diseases (chronic exfoliative dermatitis).
Signs and symptoms
Folic acid deficiency anemia gradually produces clinical features that are characteristic of other Continue reading “Folic acid deficiency anemia”

A Brief Introduction to Infertility

Definition of Infertility:
A. Primary infertility
1. Female partner has never been pregnant
2. Couple unable to conceive after unprotected intercourse for at least 1 year
B. Secondary infertility
1. Female partner has had at least one prior conception
2. Couple unable to conceive after unprotected intercourse for at least 1 year
Scope of Problem:
Approximately 15% of couples are unable to achieve a pregnancy within 1 year of attempting conception.Fertility declines are evident at least a decade prior to the menopausal transition.
Evaluation of the Infertile Couple:
A. History and physical
1. Menstrual cycle frequency and length
2. Gravidity, parity, pregnancy outcomes
3. Coital frequency and sexual dysfunction
4. Duration of infertility
5. Surgical history, particularly pelvic/abdominal
6. Medications, allergies
7. Tobacco, alcohol, drug history
8. History of sexually transmitted diseases
9. Family history of birth defects, reproductive difficulties, spontaneous abortions, early menopause, mental retardation Continue reading “A Brief Introduction to Infertility”

Chorionic Villus Sampling

It is one of the obstetric procedures and is summarized as follows:
-This is a diagnostic outpatient office procedure performed under ultrasound guidance
without anesthesia.
-The catheter is placed directly into the placental tissue without entering the amniotic cavity.
-Chorionic villi, which are placental precursors, are aspirated from a pregnant
uterus between 10 and 12 weeks’ gestation.
-The tissue is sent to the laboratory for karyotyping.
-The chromosomes of the villi are almost always identical to those of the embryo.
Nature of  Tissue Obtained:
The procedure can be performed either transcervically or transabdominally. Since the fetus and Continue reading “Chorionic Villus Sampling”

Factitious Disorder

A disorder characterized by the conscious production of signs and symptoms of both medical and mental disorders. The main objective is to assume the sick role and eventually hospitalization. Usually diagnosed with physical or psychological symptoms or both.
Called Factitious Disorder By Proxy if the signs and symptoms are faked for another person, as in
mother and child.
Seen more commonly in men and in hospital and health care workers. As children, many of the patients suffered abuse that resulted in frequent hospitalizations, thus their need to assume the sick role.
Physical and Psychiatric Presenting Symptoms.
• May have a gridiron abdomen from the multiple surgeries
• Typically demand treatment when in the hospital
• If tests return negative, they tend to accuse doctors and threaten litigation. Continue reading “Factitious Disorder”

Croup or Viral Laryngotracheobronchitis in Children

Definition and Epidemiology
  • Croup or viral laryngotracheobronchitis is an acute inflammation of the entire airway, mainly in the glottis and subglottic area, resulting in airway narrowing, obstruction, and voice loss. Therefore, it has generally been described as a triad of hoarse voice, harsh barking cough, and inspiratory stridor.
  • Typically, the condition affects younger children (6–36 months), with a peak incidence at 2 years of age. It is the most common cause of acute upper airway obstruction in young children; a reported 3% of children experience it before 6 years of age.
  • Seasonal outbreaks have been described in the fall and winter, although it may occur year round in some areas.
  • Males are more often affected than females.
Etiology and Pathophysiology
  • Viral infection is the predominant etiology; parainfluenza (types 1, 2, and 3) is the most common agent. Other common viral agents are respiratory syncytial virus (RSV) and influenza. Less commonly encountered viruses include adenovirus, rhinovirus, enterovirus, and measles virus.
  • Mycoplasma pneumoniae is one of the few bacterial microorganisms that has been reported as an etiologic agent.
  • In children, the larynx is very narrow and is comprised by the rigid ring of the cricoid cartilage; therefore, a viral infection causing inflammation of this area leads to airway edema and subsequent obstruction. This obstruction results in the classic symptoms of stridor and cough.
Clinical Presentation

Precautions While Performing Medical Procedures

Universal precautions should be used whenever an invasive procedure exposes the operator to potentially infectious body fluids. Not all patients infected with transmissible pathogens can be reliably identified. Because pathogens transmitted by blood and body fluids pose a hazard to personnel caring for such patients, particularly during invasive procedures, precautions are required for routine care of all patients whether or not they have been placed on isolation precautions of any type.

The CDC calls these universal precautions.

1. Wash hands before and after all patient contact.
2. Wash hands before and after all invasive procedures.
3. Wear gloves in every instance in which contact with blood or body fluid is certain or likely. For example, wear gloves for Continue reading “Precautions While Performing Medical Procedures”

Naltrexone For Addiction Treatment

Drugs used for addiction work in one of four ways. They either cause

1) the body to have a negative reaction to an ingested drug,

2) reduce the reinforcing effects of an ingested drug,

3) block the effects of the drug by binding to the receptor site, or

4) saturate the receptor sites with agonists that do not create the drug’s desired effect.

Naltrexone is known to be helpful for both opiate addiction and alcohol addiction. Naltrexone Continue reading “Naltrexone For Addiction Treatment”

Evaluating Patient With Abdominal Pain

The first priority when evaluating abdominal pain is to determine whether the pain is acute or chronic. Sudden and/or severe onset of pain should lead the clinician toward an emergent evaluation. Right lower quadrant pain is suspicious for an acute appendicitis, but by itself is not specific enough to warrant an emergent workup. A “gnawing” sensation is often described with ulcer disease, while pain that worsens after eating is associated with many conditions—pancreatitis, gallbladder disease, or even reflux. In the absence of hemodynamic instability, those causes are less likely to warrant emergent workup. Emesis with pain is not enough, by itself, to warrant emergent workup.

The location and radiation of pain is often helpful in determining the cause of abdominal pain. Pain from an Continue reading “Evaluating Patient With Abdominal Pain”