An infant with alternating metabolic acidosis and alkalosis: answer
- نوع فایل : کتاب
- زبان : انگلیسی
- مؤلف : Nakysa Hooman & Daniel Jafari & Sahar Jalali-Farahani & Arash Lahouti Harahdashti
- چاپ و سال / کشور: 2011
Description
Cystic fibrosis is a monogenetic disorder with pulmonary and gastrointestinal manifestations and is conventionally diagnosed with abnormal levels of chloride in sweat [1]. Pediatric patients with extra-pulmonary symptoms have malnutrition as one of the most common presentations [2], whereas renal complications and pancreatic involvement generally manifest later in life [3]. The persistence of the infant’s failure to thrive and multisystem involvement pointed to an underlying systemic disease, rather than a renal-specific disorder, and the onset of symptoms early in life indicated genetic or metabolic disorders. Metabolic alkalosis on second admission was suggestive of various metabolic disorders or hormonal imbalances. However, low urinary levels of chloride (less than 10 mEq/l) rules out chloride-resistant forms of metabolic alkalosis [4], and since there was no history of significant gastrointestinal chloride loss (e.g., vomiting), CF was suspected. His mother later confirmed a salty taste while kissing the infant, a sign frequently associated with CF in infants [5]. Pancreatic insufficiency was reflected in abnormal stool tests; the patient was referred to a gastroenterology clinic, and was started on pancreatic enzyme supplements to address his stunted growth. Interestingly, Pseudomonas aeruginosa was later isolated from fecal samples. The colonization of gastrointestinal tract with Pseudomonas aeruginosa in CF patients has been documented before [6].
Pediatr Nephrol DOI 10.1007/s00467-011-1901-2 Received: 19 March 2011 / Accepted: 11 April 2011
