Chloride losing diarrhoea and metabolic alkalosis in an infant with cystic fibrosis.

Cystic fibrosis presenting with recurrent vomiting and metabolic alkalosis

“…Metabolic alkalosis may be the initial presenting feature in many of these reported infants [1,3,6,7,14,15]. Two siblings presenting with recurrent vomiting and metabolic alkalosis but without respiratory symptoms or initial steatorrhoea and ultimately diagnosed as CF, prompted us to re-investigate our patients with CF, especially those with metabolic alkalosis on initial presentation.…”

Congenital Chloride Diarrhoea: First Time Diagnosis in Bangladesh and its Management Difficulties

“…Congenital Chloride-Losing Diarrhoea (CCD) is the best-described congenital defect of large intestinal transport and characterized by watery diarrhoea with high levels of stool chloride, which exceed the sum of stool Sodium (Na) and Potassium (K), and low stool Bicarbonate (HCO 3 ) concentrations and PH. Since the first reports of CCD in 1945 [2] the defect in anion exchange leading to high stool chloride concentrations may theoretically due to either failure of chloride absorption or to active secretion of chloride into the intestine. The alkalosis probably develops partly through an associated increase in H + excretion and partly through an absence of HCO; secretion in the ileum and colon1.…”

“…Its Prevalence is more in Finland, Saudi Arabia, Kuwait, and Poland. Consanguinity of marriages is one of the main factors for high incidence like Saudi Arabia and Kuwait such as 1 in 5000 [2] but in Bangladesh, CCD incidence is not known and there was no previously any reported cases. Diarrhea starts at fetal life which causes polyhydramniosis and in most of the cases finally causes premature delivery [3] early diagnosis in infancy is essential for saving life and also prevent the mental and psychomotor impairment and the chronic contraction of the intravascular space leading to renal dysfunction and gout [3].…”

Alcalosis metabólica hipoclorémica como presentación de la fibrosis quística. Informe de dos casos

“…Velde (35) ilustró cómo la sudoración excesiva resultante de la abstinencia de heroína fue la única explicación lógica para la alcalosis metabólica que presentó un niño con FQ en un ambiente no caluroso (35). El cloro también se puede perder en cantidades importantes por la diarrea, en los niños con FQ, como se describió en dos pacientes con íleo meconial e ileostomía (20,36). Estas alteraciones en los electrólitos llevan a disminución del volumen extracelular, estimulando el sistema reninaangiotensina-aldosterona, la hormona antidiurética y el sistema simpático, lo que genera un aldosteronismo secundario y, por lo tanto, aumento de la reabsorción de Na + , Cly H 2 O en el túbulo colector y excreción de K + e H + , dando lugar a la alcalosis metabólica hipoclorémica hipokalémica.…”