Hirschsprungs Disease

Hirschsprungs Disease

Short Bowel Syndrome

Malabsorption of water and electrolytes

Acid - Base Imbalance

Electrolyte Imbalance

Buffering by plasma proteins and hemoglobin

Hyperkalemia/Hypokalemia

Cardiac Arrest Hyperventilation/Hypoventilation

Water excretion/retention through urine Hirschsprung's disease is a blockage of the large intestine due to improper muscle movement in the bowel. It is a congenital condition, which means it is present from birth. Muscle contractions in the gut help digested materials move through the intestine. This is called peristalsis. Nerves in between the muscle layers trigger the contractions. In Hirschsprung's disease, the nerves are missing from a part of the bowel. Areas without such nerves cannot push material through. This causes a blockage. Intestinal contents build up behind the blockage, causing the bowel and abdomen to become swollen. One of the possible complications of Hirschsprungs disease is short bowel syndrome, a condition that can lead to malnourishment and dehydration.

In general, virtually all digestion and absorption is completed within the first 100-150 cm of jejunum in a healthy individual. In the absence of an intact colon, the minimum length of healthy bowel necessary to avoid parenteral nutrition is approximately 100 cm. Patients who have less than 100 cm of jejunum exhibit significant malabsorption. Although the ileum is limited in its capacity to form chylomicrons compared to the jejunum, studies have shown that the ileum has greater adaptive function as far as improving its absorptive function in the presence of short bowel syndrome. The jejunum cannot develop site-specific carriers for the transportation of vitamin B12 and bile salts, and, consequently, these are malabsorbed permanently in patients, following ileal resection. Furthermore, the loss of enteroglucagon and peptide YY cannot be underscored in the regulation of small-bowel motility. Because of this, acid-base imbalance and electrolyte imbalance happens. Maintenance of the internal environment in a state of dynamic equilibrium requires that the acid-base and electrolyte balance be maintained within the optimal physiologic range. Any deviation from this range can seriously disrupt enzymatic catalysis of intracellular chemical reaction and vital electrochemical process critical to nerve conduction, synaptic transmission and muscle contraction. Potassium, a cation thats the dominant cellular electrolyte, facilitates contraction of both skeletal and smooth muscles including myocardial contraction and figures prominently in nerve impulse conduction, acid-base balance, enzyme action, and cell membrane function. Because serum potassium level has such a narrow range (3.5 5 mEq/L), a slight deviation in either direction can produce profound clinical consequences. Paradoxically, both hypokalemia and hyperkalemia can diminish excitability and conduction rate of the heart muscle, which may lead to cardiac arrest. Furthermore, as cells use nutrients to produce energy, they produce byproducts. Two of these byproducts affecting acid-base balance are carbon dioxide and hydrogen. These two are the ones being regulated in order for the body to defend against fluctuations in pH (a measure of hydrogen ion concentration). There are three systems used by the body to defend against fluctuations in pH. These are the buffers in the blood, respiration through the lungs and excretion by the kidneys. Buffers are substances used by plasma proteins and hemoglobin to neutralize acids or bases. Bicarbonate, which is a base, and carbonic acid in the body fluids protect the body against change in acidity. These buffer systems serve as a first line of defense against changes in the acid-base balance. Respiratory system controls the pH by the rate and depth of respiration to increase or decrease the release of carbon dioxide. If the buffer system isnt able to maintain pH in its normal range, the respiratory system will adjust the frequency and depth of respiration to compensate. In an acidic environment (a low pH and an excess of hydrogen), hyperventilation will blow off excess carbon dioxide, in effect returning pH to normal. In an alkalotic environment (a high pH and a hydrogen deficit), decreasing

the depth and frequency of ventilation (hypoventilation) will cause the body to retain carbon dioxide, thus increasing hydrogen. The final line of defense against acid-base imbalance is the renal system. The kidneys are able to selectively change the amount of hydrogen secreted into urine and therefore, passed from the body. If the pH of the solution being filtered in the nephron is acidic, the kidneys increase the amount of hydrogen secreted into the urine, thereby decreasing the acidity and increasing pH in the blood. If the pH in the kidneys is alkalotic, the tubules in the nephrons will decrease the amount of hydrogen secreted, thereby increasing the acidity and lowering pH in the blood. While the kidneys are the best able to compensate for large variations in pH, their effect is not seen for hours or even days.