Serious acid-base disorder or life-threatening arrhythmia in patients with ABO-incompatible liver transplantation who received therapeutic plasma exchange : A report of two cases

Background: Excessive citrate load during therapeutic plasma exchange (TPE) can cause metabolic alkalosis with compensatory hypercarbia and electrolyte disturbances. If TPE is required immediately before ABO-incompatible (ABOi) liver transplant (LT) surgery, metabolic derangement and severe electrolyte disturbance could worsen during LT anesthesia.Case: We report two ABOi LT cases who received TPE on the day of surgery because isoagglutinin titers did not be dropped below 1:8. One case had a surprisingly high metabolic alkalosis with a pH of 7.73 immediately after tracheal intubation because of hyperventilation during mask bagging. The other experienced sudden ventricular tachycardia and blood pressure drop after surgical incision accompanied with severe hypokalemia of 1.8 mmol/L despite supplementation with potassium.Conclusions: Special attention should be paid to patients who just completed TPE the operative day morning as they are vulnerable to severe acid-base disturbances and life-threatening ventricular arrhythmias in ABOi LT.

Prenatal and Postnatal Manifestations of Congenital Chloride Diarrhea Due to a Heterozygote Variant of the SLC26A3 Gene: A Case Report

Congenital chloride diarrhea (CCD) is caused by a recessive mutation in the SLC26A3 gene and characterized mainly by watery diarrhea, hypochloremia and metabolic alkalosis. Various different mutations in SLC26A3 are responsible for the disease. In the prenatal period, the symptoms of CCD may include polyhydramnios, preterm labor and abdominal distension. The main feature of CCD is chloride-rich diarrhea, which leads to excessive loss of fluid and salt immediately after birth and is followed by weight loss and dehydration. Hyponatremia and hypochloremia are soon accompanied by hypokalemia and metabolic alkalosis. Untreated CCD is fatal even in the first weeks of life. Diagnosis is made by high fecal chloride concentrations in patients with serum electrolytes corrected by salt substitution and confirmed using genetic testing of peripheral blood samples. Here, we detail prenatal and postnatal manifestations of a preterm infant, born via Caesarian section, who was suspected to suffer intrauterine bowel obstruction. Upper median laparotomy was performed and no intestinal abnormalities found. The course of the neonatal period was complicated by severe diarrhea with hypochloremia, hyponatremia and metabolic alkalosis. Based on the patient's clinical picture and stool examination, a diagnosis of CCD was established. Mutation of the SLC26A3 gene was confirmed using genetic testing.

Can newborn screening be cost benefit procedure if preventing serious complications of cystic fibrosis?

Cystic Fibrosis (CF) is a common inherited disease with reported mean prevalence of 0.737/10,000 in 27 EU countries (Farrell J Cyst Fibros. 2008). Still, many EU countries have not implemented CF in the Newborn Screening (NBS) programme, including our country. We report the case of a 7-month-old boy whose presenting signs of CF were life-threatening neurological symptoms caused by severe metabolic alkalosis and hypoelectrolytemia. By presenting this case, we argue hoping to persuade the authorities in any country that the available newborn screening for CF is the cost benefit procedure in preventing life treating consequences with the obvious impact on the long-term prognosis of this chronic disease.

Metabolic alkalosis following mitral valvuloplasty in a dog with preoperative acute kidney injury

CASE DESCRIPTION An 11-year-old sexually intact male Shih Tzu diagnosed with acute kidney injury and left-sided congestive heart failure that had nonelective mitral valve surgery. CLINICAL FINDINGS Metabolic alkalosis developed postoperatively, and plasma bicarbonate concentration peaked 2 days after surgery (40.2 mmol/L; pH, 7.550). TREATMENT AND OUTCOME Acetazolamide administration increased the urinary excretion of bicarbonate and contributed to the improvement of the dog’s acid-base status and oxygenation capacity. Metabolic alkalosis persisted for 4 days after surgery, and no treatment was required after resolution. Plasma urea nitrogen and creatinine concentrations normalized 2 days after surgery. CLINICAL RELEVANCE Severe metabolic alkalosis can occur as a complication following mitral valve surgery. Acetazolamide may be suitable for the treatment of severe metabolic alkalosis.

Treatment of Metabolic Alkalosis with Rice Broth

The available treatments of metabolic alkalosis should be given in the hospital and may be associated with serious adverse events. We aimed to use rice broth for treatment of metabolic alkalosis. Patients with metabolic alkalosis were received oral rice broth solution; serum electrolytes and acid-base parameters were measured before and after the treatment. The majority of patients were older and in the ICU. After treatment with rice broth solution, there was statistical significant decreased in blood PH and serum [HCO3-] from a mean of 7.45 ± 0.03 to 7.4 ± 0.009 and 34.5 ± 4.4 to 24.9 ± 1.1 mmol/l respectively and statistical significant increase in serum [Na+], serum [Cl-], serum [K+], serum [Ca+2], serum [PO4-2] and serum [Mg+2] from a mean of 132.5 ± 3.7 to 136.5 ± 1.9 mmol/l, 93.2 ± 5.4 to 101.9 ± 2.7 mmol/l, 3.1 ± 0.3 to 3.6 ± 0.2 mmol/l, 0.99 ± 0.1 to 1 ± 0.1mmol/l, 3.4 ± 0.5 to 3.6 ± 0.4 mg/dl and 1.88 ± 0.2 to 2.28 ± 0.17 mg/dl respectively. Rice broth is an effective oral treatment of metabolic alkalosis.

The Dietary Approach to the Treatment of the Rare Genetic Tubulopathies Gitelman’s and Bartter’s Syndromes

Gitelman’s (GS) and Bartter’s (BS) syndromes are rare, inherited autosomal recessive tubulopathies characterized by hypokalemia, metabolic alkalosis, renal sodium, chloride, and potassium and magnesium-wasting. While the treatment based on potassium, sodium, chloride, and magnesium supplementation in addition to other pharmacologic options are widely established, recommendations about the dietary approach to GS and BS still remain generic. In this review we focus on the dietary strategies to increase sodium, potassium, and magnesium intake in GS and BS patients. Potassium and magnesium-rich foods and supplements are considered together with those that may reduce through different mechanisms the potassium and magnesium plasma level. Magnesium supplementation is often poorly tolerated, causing abdominal pain and diarrhea in most patients. New formulations using liposome and, in particular, sucrosomial technology have been recently proposed for magnesium supplementation in order to increase magnesium supplement tolerability and intestinal absorption. The dietary approach to GS and BS may be very important in the therapeutic approach to these syndromes. Due to the relevance of the dietary approach to these syndromes, a nutritional counseling should always be recommended and the nutritionist should join nephrologists in the follow-up of GS and BS patient care.