How the Red Cells Become Sphericle in Hereditary Spherocytosis?

The cells of hereditary spherocytosis are more permiable to sodium. As a result more sodium come in the cells. Water also find its way in to the cells down the osmotic gradient created by sodium influx. Influx of sodium irons also occurs in normal cells too, but the sodium potassium ATPase pump manage to send the excess sodium out of the cells. In hereditary sperocytosis, these this pump is overloaded, because the pump requires ATP to continue its function. The cell cannot provide enough ATP to meet this demand. As a result the cell cannot pump out all the sodium that comes in to the cell. Finally, water accumulates within the cell causing it to swell and become spherocytes.

How Osmotic Fragility Test Helps to Diagnose Hereditary Spherocytosis?

In hereditary spherocytosis the red cell membrane is more permeable to water. As a result, when these cells are suspended in water, they take in water and swell. The swollen cells finally lyse. 

Why everyone with Hereditary Sperocytosis doesn't have an Affected Parent?

Hereditary spherocytosis is a disease inherited in autosomal dominant manner. Therefore, theoretically everyone with this condition should have at least one affected parent, but this is not the case in 25% of the patients with this particular condition. This is because 25% of the patients who develop this condition get is as a result of spontaneous mutation of the genes responsible for the development of the red cell membrane.

How Long Does it Take to Vitamin B 12 Treatment to Take Effect?

Vitamin B 12 deficiency causes megaloblastic anemia. There are many people around the world who suffer from megaloblastic anemia. Megaloblasts form as a result of delayed maturation of the nuclear maturation of the erythroblasts. The proper maturation of nuclear materials needs Vitamin B 12 as well as folic acid. 

When a person is diagnosed to have megaloblastic anemia due to vitamin B 12 deficiency,, they are treated with vitamin B 12. People with vitamin B 12 deficiency develop many symptoms such as polyneuropathy, loss of vibration sense, etc.Once the diagnosis is made as vitamin B 12 deficiency, the patient is supplemented with the deprived agent. 

The most important question is 'how long will it take for these symptoms to dissappear?'. After 2 days of therapy, the patient may start to feel better. The investigations will show reticulocytosis 2-3 days after starting therapy. Some people with vitamin B12 deficienccy, has lower limb numbness indicating polyneuropathy, which might take 6-12 months to resolve.

Poisoning

Suicidal or homicidal poisoning with drugs over dosage is one of the common causes of morbidity and mortality in Asian countries. Acute poisoning accounts for an estimated 10-20% of acute medical admissions in United Kingdom.

When a patient with poisoning is bought to the ward, the following management steps are carried out.

·         assessment of the danger

·         gut decontamination using  activated charcoal

·         maintenance of fluid balance

·         air way control

Gastric levage is important if could be performed within first 60 minutes of ingestion of poisons. In some of the developing countries gastric lavage is carried out even after 2 hours. This is an incorrect method. In those countries the procedure is carried out by non medical personell with no training. This should never be happened. It put the patient’s life in danger due to aspiration.

Gastric larvage should never be performed on patients who does not give consents to do so.

MANAGEMENT

The following should be done in patients who present with a history, signs and symptoms of poisoning.

I)                    General Management

a.      Securing the airway, intubation,

b.      Assessment of breathing, oxygen saturation, and arterial blood gas analysis

c.       Circulation should be assed by measuring pulse rate, blood pressure, urinary output and peripheral perfusion

d.      estimate S. electrolytes, S. glucose and other parameters

e.      Challenge with glucose should be done to avoid irreversible brain injury due to hypoglycemia.

II)                   Specific Management

General Management involves the following steps.

B) Decontamination of body parts.