Pseudo-Bartter’s syndrome in patients with cystic fibrosis: A case series and review of the literature
Introduction. Pseudo-Bartter syndrome (PBS) is characterized by hyponatremic, hypochloremic metabolic alkalosis that mimics Bartter syndrome but with no pathology in the renal tubules. We present five patients with cystic fibrosis (CF) and PBS. Cases Outline. Four children aged between thre...
| Main Authors: | , |
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| Format: | Article |
| Language: | English |
| Published: |
Serbian Medical Society
2015-01-01
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| Series: | Srpski Arhiv za Celokupno Lekarstvo |
| Subjects: | |
| Online Access: | http://www.doiserbia.nb.rs/img/doi/0370-8179/2015/0370-81791512748V.pdf |
| Summary: | Introduction. Pseudo-Bartter syndrome (PBS) is characterized by hyponatremic,
hypochloremic metabolic alkalosis that mimics Bartter syndrome but with no
pathology in the renal tubules. We present five patients with cystic fibrosis
(CF) and PBS. Cases Outline. Four children aged between three and
five-and-one-half months with previously diagnosed CF and one aged 17 months
with previously undiagnosed disease, were hospitalized during the summer
season, with severe dehydration, oliguria, apathy and adynamia. Additionally,
one of them had an ileostomy due to meconium ileus after birth. All children
were on a diet without additional salt intake. Laboratory analysis on
admission showed hyponatremia (115-133 mmol/L, mean 122.4 mmol/L), high
plasma renin activity (229-500 pg/ml, mean 324 pg/ml) and metabolic alkalosis
(pH 7.5-7.6, mean 7.56) in all the patients, and in four of them high blood
level of aldosterone (74-560 pg/ml, mean 295.9 pg/ml), hypokalemia (2.3-2.8
mmol/L, mean 2.6 mmol/L), hypochloremia (59-71 mmol/L, mean 66 mmol/L) and
low urinary sodium (5-12 mmol/L, mean 9 mmol/L). After intravenous
rehydration followed by additional use of sodium and chloride in mean dosis
of 1.78 mmol/kg per day, all the patients made a complete recovery. With
advice for additional use of salt in the mentioned amount, the patients were
discharged from the hospital. Conclusion. PBS is one of CF complications,
especially in infants and young children in situations accompanied by
increased sweating and/or other causes of additional loss of sodium and
chlorine. Sometimes, as was the case with one of our patients, PBS may be the
initial presentation form of the disease. |
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| ISSN: | 0370-8179 2406-0895 |