HYPERCHLOREMIC ACIDOSIS IN A PATIENT WITH ARGININOSUCCINATE LYASE DEFICIENCY
Normal physiological pH is between 7.35 and 7.45. A drop in pH below this range is called acidosis, and an increase in this range is called alkalosis. The most important pH buffering system in the human body is the bicarbonate/carbon dioxide (HCO3/CO2) chemical equilibrium system. The human body is very good at keeping ions in balance in most scenarios. As a result, the loss of bicarbonate (a negatively charged ion) moves the negatively charged chloride (Cl) ion into the extracellular space. This results in a narrow anion gap, an electrically neutral state without correcting the acidosis-induced pathology. Similarly, increased Cl can replace bicarbonate in the cell. To determine the exact etiology of the narrow anion gap, hyperchloremic acidosis requires another test, the urinary anion gap. The prevalence of hyperchloric acidosis is unknown. The exact frequency and distribution of the disease depend on the etiology. Similarly, morbidity and mortality depend on disease etiology. Hyperchloremic metabolic acidosis is a pathological condition due to loss of bicarbonate rather than acid production or retention. Bicarbonate depletion leading to hyperchloremic metabolic acidosis occurs in several ways. Gastrointestinal (GI) cause’s renal causes and extrinsic causes. Gastrointestinal loss of bicarbonate occurs with severe diarrhea, pancreatic fistula, nasojejunal gavage from the duodenum, and chronic use of laxatives. Extrinsic causes include ingestion of acids such as ammonium chloride or hydrochloric acid and volumetric resuscitation with 0.9% saline. Many exogenous causes of hyperchloric acidosis are logical evaluations. When substances such as ammonium chloride and hydrochloric acid are introduced into the body, they react with bicarbonate to buffer the pH. However, this depletes bicarbonate stores and leads to overly acidic conditions. Heavy resuscitation with normal saline leads to an overload of chloride ions in the blood. As mentioned earlier, chloride and bicarbonate work together to maintain the ionic balance in the cell space. Hyperchlorhydria forces bicarbonate into cells to maintain ionic balance and reduce available bicarbonate to the pH buffer system, resulting in net acidosis. Patients with hyperchloremia are necessarily unaffected because of hyperchloremia. However, acidosis can have many negative health effects. Headache, lack of energy, nausea, and vomiting are common symptoms, but as acidosis worsens, stupor, coma, myocardial instability, or cardiac arrest may occur. You would expect your breathing rate to increase as your body tries to reduce carbon dioxide. However, prolonged conditions can lead to muscle fatigue and respiratory failure. Physical examination shows altered mental status, tachycardia, tachypnea, use of accessory muscles for breathing, neurological deficits, muscle weakness, abnormal heart rhythm, heart murmurs, wheezing, rales, or rhonchi There are cases. As with any illness, a detailed medical history and physical examination are the most important first steps in evaluation. Hyperchloric acidosis due to gastrointestinal bicarbonate loss or drug ingestion is readily identified. A complete blood count (CBC) is helpful to assess the cause of infection with elevated white blood cell count and fluid status due to hemoglobin and hematocrit levels. A complete metabolic panel is important considering sodium, potassium, and chloride levels. This is because they can be used to calculate anion gap values.