Congenital chloride diarrhoea in Kuwaiti children

Abstract

Congenital chloride diarrhoea was diagnosed in 16 Kuwaiti children over a 7 year period (1980-1986) with an estimated incidence of 7.6 per 100,000 live births. The mean age at diagnosis was 3.2 months (range 1 week to 5 months). There were 9 boys and 7 girls with a mean age of 3 years 10 months (range 10 months to 7 years). All children had a shortened gestational period, abdominal distension and chronic diarrhoea. The serum electrolytes in all patients prior to treatment showed hyponatraemia, hypokalaemia, hypochloraemia and metabolic alkalosis. The diagnosis was confirmed by a stool chloride content that exceeded the sum of faecal sodium and potassium. Fifteen patients survived and showed catch-up growth with adequate replacement therapy and 1 died with renal failure.

Congenital Chloride Diarrhea: Is it a Common Disorder in Saudi Arabia?

“…The incidence was estimated to be one in 14,000 in a multicenter study from Kuwait. 4 A similar but smaller study of National Guard dependents from Riyadh estimated the incidence at one in 5500. 7 The true incidence in Saudi Arabia is probably somewhere in between these two numbers but is significantly higher than the one in 43,000 from Finland.…”

“…[2][3][4][5][6][7] In the 50 years since the first case reports of CCD were published, 8,9 more than 140 cases encompassing all races and from all parts of the world have been recognized. [2][3][4][5][6][7][8] The description of CCD as a "Finnish disease" based on the impression that this disease is most common in Finland, 10 should be revised in the context of more than 50 cases from the Arabian Peninsula compared to approximately 40 cases from Finland. The incidence was estimated to be one in 14,000 in a multicenter study from Kuwait.…”

Congenital Chloride Diarrhea: A Single Center Experience with Ten Patients

“…More than one-third of the reported cases have been from Finland but the disease has also been identified in several other European countries, 4 North and South America and the Far East 5,6 and more recently, from the Arabian Peninsula. [7][8][9][10] We report our experience in 10 patients with CCD to familiarize clinicians with the clinical and laboratory findings of this disease and to stimulate awareness of the gene prevalence in the region where there is a high incidence of consanguineous marriages. Early recognition of CCD allows for appropriate therapy and prevention of the complications that may result from chronic extracellular fluid volume depletion and metabolic alkalosis.…”

Congenital Chloride-Losing Diarrhea in Saudi Children

“…Our small number of cases does not allow for us to calculate the exact frequency in Saudi Arabia. CCD was reported from Kuwait 6 and we reported one other case from Saudi Arabia 7 but came across no report from elsewhere in the Arab world.…”