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CASE REPORT
Year : 2010 | Volume : 58 | Issue : 1 | Page : 106--108

Renal tubular acidosis presenting as respiratory paralysis: Report of a case and review of
literature
J Kalita, PP Nair, G Kumar, UK Misra
Department of Neurology, Sanjay Gandhi PGIMS, Lucknow, India

Correspondence Address:
J Kalita
Department of Neurology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Raebareily Road, Lucknow - 226 014
India

Abstract
Respiratory paralysis due to renal tubular acidosis (RTA) is rare. We report a 22-year-old lady who developed severe
bulbar, respiratory and limb paralysis following respiratory infection. She had hypokalemia (1.6 meq/L) and
hyperchloremic (110 meq/l) acidosis (pH 7.1). She was diagnosed as distal RTA by ammonium chloride test. She
improved following sodium bicarbonate and potassium supplementation. RTA should be differentiated from familial
periodic paralysis (FPP) because acetazolamide used in FPP aggravates RTA and sodium bicarbonate used in RTA
aggravates hypokalemic periodic paralysis.

How to cite this article:

Kalita J, Nair P P, Kumar G, Misra U K. Renal tubular acidosis presenting as respiratory paralysis: Report of a case and review of
literature.Neurol India 2010;58:106-108

How to cite this URL:

Kalita J, Nair P P, Kumar G, Misra U K. Renal tubular acidosis presenting as respiratory paralysis: Report of a case and review of
literature. Neurol India [serial online] 2010 [cited 2017 Aug 20 ];58:106-108
Available from: http://www.neurologyindia.com/text.asp?2010/58/1/106/60415

Full Text

Introduction

Familial periodic paralysis (FPP) and renal tubular acidosis (RTA) have similar clinical picture with rarity of facial,
ocular, bulbar and respiratory muscle weakness [1] although both these conditions are due to different
pathophysiological mechanisms. In FPP, there is redistribution of K+ across the cell membrane, which is attributed to
mutation in two genes encoding subunits of voltage-gated calcium or sodium channel (CACNA1S and SCN4A) of
skeletal muscles. [2] In RTA on the other hand, there is loss of K+ due to impaired handling of K+ in the renal
tubules resulting in impairment of acidification of urine. Excretion of other ions such as Na+ and Ca++ increases
bicarbonate loss. [3] Respiratory involvement and severe paralysis is regarded to be rare in RTA and only 15 patients
in English literature could be found. [1],[4],[5],[6],[7],[8],[9],[10],[11],[12],[13],[14],[15],[16] It is important to
differentiate RTA from FPP because the principles of management and prophylaxis are different. We report a rare case
of RTA who had respiratory paralysis and review the relevant literature.

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8/20/2017 Renal tubular acidosis presenting as respiratory paralysis: Report of a case and review of literature :<b>J Kalita, PP Nair, G Kumar, UK Misra</

Case Report

A 22-year-old postgraduate female student noted proximal muscle weakness in the morning and had difficulty in
getting up from squatting position. The weakness progressed over a few hours and she experienced difficulty in
turning in bed, swallowing and speaking. On the third day she developed breathing difficulty, drowsiness and
carpopedal spasm. She had suffered from fever and cough five days prior to this illness which had lasted for two
days. On examination patient was drowsy, dyspnoeic and was unable to move, swallow or speak. Her blood pressure
was 150/50 mm Hg, pulse 96/min irregular, respiratory rate 40/min and responded to command by eye movement
and closure. She had flaccid quadriplegia of Grade 0. Biceps, triceps, knee and ankle reflexes were absent. She was
intubated and ventilated. Chvostek and Trousseau signs were present. Her hemoglobin was 8.9 g/dl, leucocyte count
16400/mm 3 , erythrocyte sedimentation rate 31 mm for the first hour, fasting blood sugar 70 mg/ dl, BUN 12 mg/dl,
serum creatinine 0.9 mg/dl, uric acid 3.2 mg/dl, AST 880 IU/L, ALT 43 IU/L, creatine kinase 190 IU/L, bilirubin 0.6
mg/dl, calcium 6.9 mg/dl, phosphorous 1.9 mg/dl and potassium was 1.6 meq/l. Blood gas analysis revealed pH 7.1
(N = 7.35-7.45), serum potassium 2.1 (N = 3.5-4.5) mEq/L, serum sodium 136 (N = 135-148) mEq/L, PCO 2 52
(35-45) mmHg, PO 2 130 (80-100) mmHg, HCO 3 16.5 meq/L, chloride 110 mEq/L and an anion gap of 10
suggesting hyperchloremic normal anion gap metabolic acidosis. Electrocardiogram showed multiple premature
ventricular ectopics and U waves [Figure 1]a. She was treated with intravenous sodium bicarbonate 20 mEq, and KCl
20 mEq over 2 h following which her cardiac rhythm normalized. After administration of 160 mEq of potassium over
the next 10 h of hospitalization, her muscle power and respiration improved and she was extubated. Her serum
potassium at that point was 3.1 mEq/L. Within 48 h her blood pH and serum potassium were normalized. Ammonium
chloride test was later carried out by administering 0.1 mg/kg ammonium chloride orally after obtaining blood pH,
bicarbonate and urinary pH. These parameters were measured hourly for 6 h and revealed lack of acidification of
urine (pH = 8) in spite of increasing acidosis of blood from 7.42 to 7.29 suggesting distal RTA. Her 24-h urinary
protein was 280 mg, serum vitamin D level was 21.86 (normal 9-47) ng/ml and serum parathormone level 393.3
(normal 9-55) pg/ml. Urine and blood cultures were sterile. Radiographs of chest and pelvis were normal but plain
radiograph abdomen revealed bilateral renal calculi. Ultrasonography of abdomen revealed cholelithiasis and
intravenous pyelography bilateral hydronephrosis [Figure 2]. The patient was prescribed uriliser (potassium citrate
and ammonium citrate) 10 ml thrice daily, calcium carbonate 500 mg thrice daily and vitamin D 1 mcg daily. She has
been asymptomatic for the last three years on these treatments.

Discussion

Our patient suffered from severe hypokalemia resulting in bulbar, respiratory and limb weakness due to distal RTA.
The diagnosis of distal RTA was based on hyperchloremic metabolic acidosis and positive ammonium chloride test in
which increasing acidemia did not result in acidification of urine and urine pH remained higher than 5.5. In RTA
acidosis and hypokalemia can be life-threatening during stress or an intercurrent illness as in our patient. Severe
hypokalemia was associated with cardiac arrhythmia and respiratory paralysis resulting in medical emergency
necessitating artificial ventilation.

Only 16 patients including our patient of hypokalemia due to RTA have been associated with respiratory paralysis.
[1],[4],[5],[6],[7],[8],[9],[10],[11],[12],[13],[14],[15],[16] Their age ranged between five and 55 years and all but
two were females. Potassium level in these patients has ranged between 0.8 mEq/L to 2.09 mEq/L. The precipitating
factors included diarrhea in 12, glue sniffing and alcohol in one patient each. In our patient also the acute attack of
paralysis was triggered by infection. The associated systemic illnesses were Sjogren syndrome in five, [4],[5],[6],[7],
[8] chronic active hepatitis in two, [9],[10] interstitial nephritis in two [7],[10] and diabetes insipidus in one. [6] Only
one of these studies did the ammonium chloride test for confirmation of distal RTA. [5] Muscle weakness in RTA is
common but respiratory paralysis is rare and is a serious complication. The progression to respiratory paralysis may
be influenced by individual susceptibility and sensitivity towards hypokalemia and probably also transmembrane
potassium gradient of respiratory muscles. The majority of reported patients with RTA with respiratory paralysis were
females. With respiratory support and correction of metabolic abnormality all the patients had a good outcome.

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Patients with RTA with respiratory failure and ventricular fibrillation have been reported from Thailand. This condition
occurs in otherwise healthy adults and sometimes resulted in nocturnal death. [17]

Type I RTA may be familial and autosomal dominant is the commonest of inheritance. X-linked, autosomal recessive,
and sporadic form has also been reported. In our patient there was no family history. Mutation in chloride bicarbonate
exchanger gene has been found in the autosomal dominant form. Other diseases associated with RTA Type I are
galactosemia, Ehler Danlos's syndrome, Fabry's disease, Wilson's disease, hereditary elliptocytosis, Sjogren's
syndrome, hypogamaglobulinemia and chronic active hepatitis. [18] It is important to differentiate FPP from RTA as
both the diseases may have a similar clinical picture; however, presence of tetany, cardiac arrhythmia and
hypokalemia during the interictal period suggests secondary hypokalemia. It is important to differentiate RTA from
FPP because during acute attack, administration of sodium bicarbonate in FPP would facilitate intracellular potassium
flux and fatal hypokalemia may occur. [1] In FPP oral potassium and acetazolamide are useful in preventing the
attacks but acetazolamide is contraindicated in RTA as it produces acidosis.

Acknowledgment

We thank Mr. Rakesh Kumar Nigam for secretarial help.

References
1 Christensen KS. Hypokalemic periodic paralysis secondary to renal tubular acidosis. Eur Neurol 1985;24;303-5
2 Sternberg D, Maisonobe T, Jurkat-Rott K, Nicole S, Launay E, Chauveau D, et al. Hypokalaemic periodic
paralysis type 2 caused by mutations at codon 672 in the muscle sodium channel gene SCN4A. Brain
2001;124:1091-9.
3 Owen EE, Verner JV Jr. Renal tubular disease with muscle paralysis and hypokalemia. Am J Med 1960;28:8-21.
4 Reddy KS, Jha V, Nada R, Kohli HS, Sud K, Gupta KL, et al. Respiratory paralysis in Sjogren syndrome with
normal renal function. Natl Med J India 2003;16:253-4.
5 Fujimoto T, Shiiki H, Takahi Y, Dohi K. Primary Sjogren's syndrome presenting as hypokalaemic periodic
paralysis and respiratory arrest. Clin Rheumatol 2001;20:365-8.
6 Chang YC, Huang CC, Chiou YY, Yu CY. Renal tubular acidosis complicated with hypokalemic periodic paralysis.
Pediatr Neurol 1995;13:52-4.
7 Poux JM, Peyronnet P, Le Meur Y, Favereau JP, Charmes JP, Leroux-Robert C. Hypokalemic quadriplegia and
respiratory arrest revealing primary Sjogren's syndrome. Clin Nephrol 1992;37:189-91.
8 Ohtani H, Imai H, Kodama T, Hamai K, Komatsuda A, Wakui H, et al. Severe hypokaemia and respiratory arrest
due to renal tubular acidosis in a patient with Sjogren syndrome. Nephrol Dial Transplant 1999;14:2201-3.
9 Koul PA, Saleem SM. Chronic active hepatitis with renal tubular acidosis presenting as hypokalemic periodic
paralysis with respiratory failure. Acta Paediatr 1992;81:568-9.
10 Bridi GS, Falcon PW, Brackett NC Jr, Still WJ, Sporn IN. Glomerulonephritis and renal tubular acidosis in a case
of chronic active hepatitis with hyperimmunoglobulinemia. Am J Med 1972;52:267-78.
11 Gombar S, Mathew PJ, Gombar KK, D'Cruz S, Goyal G. Acute respiratory failure due to hypokalemic muscular
paralysis from renal tubular acidosis. Anaesth Intensive Care 2005;33:656-8.
12 Kao KC, Tsai YH, Lin MC, Huang CC, Tsao CY, Chen YC. Hypokalemic muscular paralysis causing acute
respiratory failure due to rhabdomyolysis with renal tubular acidosis in a chronic glue sniffer. J Toxicol Clin
Toxicol 2000;38:679-81.
13 Herrmann RA, Mead AW, Spritz N, Rubin AL. Hypopotassemia with respiratory paralysis. Case due to renal
tubular acidosis. Arch Intern Med 1961;108:925-8.
14 Peltz S, Hashmi S. Severe hypokalemia and ventilator-dependent, generalized paralysis. NY State J Med
1991;91:32-3.
15 Alvarez J, Low RB. Acute respiratory arrest due to hypokalemia. Ann Emerg Med 1988;17:288-9.
16 Feest TG, Wrong O. Potassium deficiency and sudden unexplained nocturnal death. Lancet 1991;338:1406.
17 Brenner BM. The Kidney. 7 th edn. Vol 1 Saunders, Philadelphia 2004. p. 950.
18 Nimmannit S, Malasit P, Chaovakul V, Susaengrat W, Vasuvattakul S, Nilwarangkur S. Pathogenesis of sudden

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8/20/2017 Renal tubular acidosis presenting as respiratory paralysis: Report of a case and review of literature :<b>J Kalita, PP Nair, G Kumar, UK Misra</

unexplained nocturnal death (lai tai) and endemic distal renal tubular acidosis. Lancet 1991;338:930-2.

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