scholarly journals Atypical presentation of cystic fibrosis: Obese adolescent with hypertension and pseudo-Bartter’s syndrome

2012 ◽  
Vol 69 (4) ◽  
pp. 367-369 ◽  
Author(s):  
Aleksandar Sovtic ◽  
Predrag Minic ◽  
Radovan Bogdanovic ◽  
Natasa Stajic ◽  
Milan Rodic ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Metabolic Alkalosis ◽  
Caloric Intake ◽  
Chloride Concentration ◽  
Plasma Renin ◽  
Holter Monitoring ◽  
Atypical Presentation ◽  
Bartter’S Syndrome ◽  
Bartter's Syndrome ◽  
Obese Adolescent

Introduction. Infants with cystic fibrosis may fail to thrive despite recommended caloric intake because of electrolyte disurbances caused by salt depletion resulting in hypochloremic metabolic alkalosis or pseudo-Bartter's syndrome. In most patients reported symptoms began in infancy, but it may be an initial presentation of disease in a previously healthy adolescent. Case report. A 15-year-old boy was admitted for evaluation of recurrent episodes of malaise associated with dehydration and acute renal insufficiency. Laboratory analysis showed hypochloremic metabolic alkalosis with hyponatremia and hypokalemia. On admission the boy was obese, with body weight of 95.5 kg (> P97), height 174 cm (> P75), and body mass index of 31.2 kg/m2 (> P95). Physical examination was inconclusive. Blood pressure holter monitoring proved significant systolic hypertension. Routine urinalysis, protein and electrolyte levels in urine were normal. Plasma renin and aldosteron were normal. Sweat chloride concentration was 63 mmol/L. Genetic testing confirmed the diagnosis of cystic fibrosis. Conclusion. To our knowledge, this is the first reported case of atypical presentation of cystic fibrosis in an adolescent presented with pseudo-Bartter's syndrome and signs of obesity and hypertension. We suggest that every patient with hypochloremic metabolic alkalosis should be evaluated for cystic fibrosis.

scholarly journals Case of Pseudo-Bartter's Syndrome: An atypical presentation of cystic fibrosis

2011 ◽  
Vol 31 (2) ◽  
pp. 121-123 ◽  
Author(s):  
Enayatollah Nemat Khorasani
Keyword(s):  
Cystic Fibrosis ◽  
Key Words ◽  
Metabolic Alkalosis ◽  
Atypical Presentation ◽  
Bartter’S Syndrome ◽  
Bartter's Syndrome ◽  
In The Beginning

A three months infant who in the beginning had disease cystic fibrosis was diagonosed with pseudo-bartter's syndrome. The disease began with coughing, diarrhoea, vomiting and weakness. Investigation revealed; electrolytes showin hyponatremia (110 mmol/L) and hypokalemic (2.6 mmol/L) and hypochloremic (63 mmol/L) metabolic alkalosis (HCO3=43 mmol/L). Key words: Pseudo Bartter's Syndrome; Cystic fibrosis; Metabolic alkalosis. DOI: 10.3126/jnps.v31i2.3911 J Nep Paedtr Soc 2010;31(2):121-123

Neo-Mull-Soy Metabolic Alkalosis: A Model of Bartter's Syndrome?

PEDIATRICS ◽  
1980 ◽  
Vol 66 (5) ◽  
pp. 784-786
Author(s):  
Vivian M. Reznik ◽  
William R. Griswold ◽  
Stanley A. Mendoza ◽  
Richard M. McNeal
Keyword(s):  
Metabolic Alkalosis ◽  
Chloride Transport ◽  
Chloride Concentration ◽  
Thick Ascending Limb ◽  
Loop Of Henle ◽  
Bartter’S Syndrome ◽  
Bartter's Syndrome ◽  
Hypokalemic Alkalosis

A case of Neo-Mull-Soy-induced metabolic alkalosis occurred in an 8-month-old child. This child had hypochloremic hypokalemic alkalosis as well as hyperreninemia. Initially, a diagnosis of Bartter's syndrome was made and treatment consisted of KCl replacement, indomethacin, and aspirin. In retrospect, the diagnosis of Neo-Mull-Soy induced metabolic alkalosis could have been suspected on the basis of the low chloride concentration in his urine. Proposed mechanisms for the etiology of Bartter's syndrome are reviewed. Neo-Mull-Soy induced metabolic alkalosis simulates Bartter's syndrome and supports the concept that the primary abnormality in Bartter's syndrome is chloride deficiency. The chloride deficiency in Bartter's syndrome results from a defect in active chloride transport in the thick ascending limb of the loop of Henle.

CASE STUDIES OF SIBLINGS WITH JUXTAGLOMERULAR HYPERPLASIA AND SECONDARY ALDOSTERONISM ASSOCIATED WITH SEVERE AZOTEMIA AND RENAL RICKETS—BARTTER'S SYNDROME OR DISEASE?

PEDIATRICS ◽  
1970 ◽  
Vol 46 (3) ◽  
pp. 344-361
Author(s):  
B. S. Arant ◽  
N. C. Brackett ◽  
R. B. Young ◽  
W. J. S. Still
Keyword(s):  
Renal Function ◽  
Normal Renal Function ◽  
Sodium Intake ◽  
Plasma Renin ◽  
Bartter’S Syndrome ◽  
Bartter's Syndrome ◽  
Cell Hyperplasia ◽  
Severe Renal Failure ◽  
Excretion Rates ◽  
Disease Present

In 1962 two cases of juxtaglomerular (J-G) hyperplasia and secondary aldosteronism without hypertension with otherwise near normal renal function were presented. We have followed, since birth, two male siblings (F.T., age 6; J.T., age 3), with many features of "Bartter's syndrome," but who progressed to severe renal failure (F.T. and J.T.) and in one child (F.T.) overt renal rickets. Metabolic alkalosis, high levels of plasma renin activity (PRA) not suppressed by Na loading were found, but renal biopsy (J.T.) and autopsy (F.T.) revealed a severe glomerular and interstitial nephritis with mild J-G hyperplasia (not the typical "invasive" J-G cell hyperplasia seen in Bartter's cases). Balance studies demonstrated renal sodium wasting in both J.T. and F.T. PRA and aldosterone excretion rates were inappropriately high for sodium intake. Severe bone disease present in F.T. was associated with an increase in the total hydroxyproline (HP): Cr ratio to 1.77 (N = .13). Free HP excretion was increased in J.T. in whom bones appeared normal. It is suggested that either the typical cases with "invasive" J-G hyperplasia and near normal renal function represent a distinct entity (?Bartter's disease) or that "Bartter's syndrome" must be extended to include uremia and renal osteodystrophy.

Long-Term Use of Propranolol, Ibuprofen, and Spironolactone in the Management of Bartter's Syndrome

PEDIATRICS ◽  
1979 ◽  
Vol 63 (5) ◽  
pp. 754-756
Author(s):  
Robert J. Cunningham ◽  
Ben H. Brouhard ◽  
Michael Berger ◽  
Tom Petrusick ◽  
Luther B. Travis
Keyword(s):  
Plasma Renin Activity ◽  
Linear Growth ◽  
Plasma Renin ◽  
Renin Activity ◽  
Bartter’S Syndrome ◽  
Bartter's Syndrome ◽  
Prostaglandin Synthetase ◽  
Catch Up

This report concerns two patients wter'sth Barter's syndrome who were treated with propranolol, spironolactone, and potassium supplements. When ibuprofen was added to this regimen, potassium supplements were no longer required. In both patients, plasma renin activity decreased, plasma volumes increased, and a "catch-up" in linear growth ensued. This report confirms others that indicate prostaglandin synthetase inhibitors are a useful adjunct in the therapy of Bartter's syndrome.

The effect of methyldopa on plasma renin activity in a child with Bartter's syndrome

1970 ◽  
Vol 77 (6) ◽  
pp. 1071-1074 ◽  
Author(s):  
Ronald G. Strauss ◽  
Shakil Mohammed ◽  
Jennifer M.H. Loggie ◽  
William K. Schubert ◽  
Alfred F. Fasola ◽  
...  
Keyword(s):  
Plasma Renin Activity ◽  
Plasma Renin ◽  
Renin Activity ◽  
Bartter’S Syndrome ◽  
Bartter's Syndrome

scholarly journals Cystic Fibrosis in An Infant: Presented as Pseudo Bartter Syndrome

2016 ◽  
Vol 15 (1) ◽  
pp. 57-59 ◽  
Author(s):  
Jubaida Rumana ◽  
Arpana Lyengar ◽  
Mohammed Maruf Ul Quader ◽  
Mohammad Hanif
Keyword(s):  
Cystic Fibrosis ◽  
Metabolic Alkalosis ◽  
Gastrointestinal Symptoms ◽  
Chloride Concentration ◽  
Bartter Syndrome ◽  
Term Survival ◽  
Multiple Organs ◽  
Sweat Chloride ◽  
History Of ◽  
Renal Tubular

Cystic Fibrosis (CF) can involve multiple organs although the most commonly affected systems are respiratory and gastrointestinal ones. In infancy it can also present as Pseudo Bartter Syndrome which is characterized by hyponatremic, hypochloremic, hypokalamic metabolic alkalosis without renal tubular pathology. We report a 5 month old boy who had history of recurrent episodes of dehydration due to vomiting and recurrent respiratory infection. His biochemical parameters suggestive of Pseudo Bartters Syndrome. Initial sweat chloride test was normal 16 mEq/L whereas the repeated test revealed Sweat chloride concentration of 107 mEq/L. The cystic fibrosis mutation analysis revealed F508 del homozygosity for the Cystic Fibrosis Transmembrane Regulator (CFTR) gene. Cystic fibrosis should be always considered in any infant with metabolic alkalosis and hyponatremic hypochloremic dehydration, whether or not there are association with pulmonary and /or gastrointestinal symptoms. Early diagnosis is essential in improving the prognosis and long term survival of these children.Chatt  Shi Hosp Med Coll J; Vol.15 (1); Jan 2016; Page 57-59

scholarly journals 17 Infants diagnosed clinically after false negative cystic fibrosis newborn screening may present with Pseudo-Bartter's-syndrome

2015 ◽  
Vol 14 ◽  
pp. S60
Author(s):  
A.C. Gjerstad ◽  
E. Bakkeheim ◽  
K. Handeland ◽  
E. Lundman ◽  
R.D. Pettersen ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Newborn Screening ◽  
False Negative ◽  
Bartter’S Syndrome ◽  
Bartter's Syndrome

scholarly journals Cystic fibrosis in an infant presenting with features of Pseudo Bartter's syndrome.

2018 ◽  
Vol 15 (4) ◽  
Author(s):  
Dinesh M Nayak ◽  
Chandrika D Nayak ◽  
Nagesha K M
Keyword(s):  
Cystic Fibrosis ◽  
Bartter’S Syndrome ◽  
Bartter's Syndrome
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