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Renal Tubular Acidosis James C.M

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Renal Tubular Acidosis James C.M consultation with the specialist Renal Tubular Acidosis James C.M. Chan, MD,*† The infant’s paternal grandfather Jon I. Scheinman, MD,‡ died at age 61 from bronchitis and Karl S. Roth, MD*† heavy smoking. The 60-year-old pa- ternal grandmother was healthy and well. There was no family member Objectives After completing of short stature. The 38-year-old this article, readers should be able mother (162.6 cm) and the 46-year- to: old father (175.3 cm) both were in good health. The maternal grandfa- 1. Describe the important presenting ther, age 72, had a history of renal characteristics of renal tubular stones. The 67-year-old maternal acidosis (RTA). grandmother (157.5 cm) had a his- 2. Delineate the mechanisms of the tory of gallstones. No one in the growth failure commonly family was on dialysis or had kidney encountered in RTA. diseases except for the maternal grandfather’s renal stones. 3. Characterize the various types of At 2 months of age, the infant had primary RTA. persistent failure to thrive and a 4. Describe diagnostic tests and 1-day history of irritability and vom- treatment modalities available for iting and was readmitted for diagnos- RTA. tic evaluation. Serum bicarbonate 5. Delineate the conditions giving level was 12 mEq/L (12 mmol/L), rise to secondary distal and and she was tachypneic, with a respi- ratory rate of 60 breaths/min and proximal RTA. intercostal retraction. Her height was 48.5 cm (Ͻ5th percentile) and her weight was 3.45 kg (Ͻ5th percen- Case Presentation tile). Blood pressure was 81/48 mm A 2-month-old Caucasian female Hg. She was alert and calm, with presented for failure to thrive. She normal skin turgor. Chest showed was born at 33 weeks’ gestation via equal expansion and clear breath primary cesarean section for preg- sounds, with no rales or wheezes. nancy-induced hypertension to a 38- There were distinct heart sounds, a year-old G1P0 mother. Her birth- regular rhythm, and no murmur. weight was 1,430 g, making her The abdomen was soft and non- small for gestational age. Apgar tender, had normally active bowel scores were 7 and 9 at 1 and 5 min- sounds, and had no masses or utes, respectively. Newborn meta- hepatosplenomegaly. Pulses were full bolic screen results were negative. At and equal. She had good muscle tone 3 weeks of age, with good oral intake and spontaneous movement of all of formula, alternating with breast- extremities. feeding, the infant was discharged Laboratory values on admission from the hospital. were: blood pH, 7.28; serum so- dium, 138 mEq/L (138 mmol/L); From the Department of Pediatrics* and the potassium, 5.2 mEq/L (5.2 mmol/ Department of Biochemistry Molecular Biophysics†, L); chloride, 113 mEq/L (113 Virginia Commonwealth University, Richmond, VA, and the Department of Pediatrics at the University mmol/L); bicarbonate, 12 mEq/L of Kansas‡, Kansas City, KS. (12 mmol/L); urea nitrogen, 3 Pediatrics in Review Vol.22 No.8 August 2001 277 consultation with the specialist mg/dL (1.07 mcmol/L); and creat- Table 1. Normal Indices of Urinary Excretion of inine, 0.2 mg/dL (17.7 mcmol/L). Urinalysis showed: pH, 7; specific Citrate and Other Variables gravity, 1.003; and no hematuria or Index Measurement Normal Values albuminuria. Diagnostic evaluation for sepsis was negative, and ceftriax- Calcium <4 mg/kg per 24 h one was discontinued on the sixth Urinary calcium/creatinine ratio: <0.21 mg/mg (adults) hospital day. <0.41 mg/mg (19 mo to 6 y) Ultrasonography of the kidneys <0.60 mg/mg (7 to 18 mo) showed diffuse nephrocalcinosis bi- <0.86 mg/mg (<7 mo) laterally. The right kidney was 5.1 cm Oxalate <50 mg/1.73 m2 per 24 h (<2 mg/kg per 24 h) 2 and the left kidney 4.8 cm. They Uric acid <815 mg/1.73 m per 24 h (35 mg/kg per 24 h) Cystine <75 mg/g creatinine appeared normal for age, exhibited Citrate >180 mg/g creatinine normal echogenicity and normal Creatinine Newborns: 8 to 10 mg/kg per 24 h preservation of parenchyma, and had Children: 12 to 10 mg/kg per 24 h no hydronephrosis or hydroureters. Adults: F 12 to 15 mg/kg per 24 h The alkaline urine pH consistently M 15 to 20 mg/kg per 24 h above 5.5 in the presence of meta- From Laufer J, Biochis H. Urolithiasis in children: current medical management. Pediatr Nephrol. 1989;3:317–331; Sargent JD, Stukel TA, Kresal J, Klein RZ. Normal values for random urinary bolic acidosis and the presence of calcium to creatinine ratios in infancy. J Pediatr. 1993;123:393–397; and Manz F, Kehrt R, Lausen nephrocalcinosis without a history of B, Merkel A. Urinary calcium excretion in healthy children and adolescents. Pediatr Nephrol. diuretic having been used suggested 1999;13:894–899. the diagnosis of renal tubular acidosis (RTA). The urinary calcium-to-creatinine ratio was 0.73 mg. The urinary ci- was discharged from the hospital on about 0.08 mg/mg. The bilateral trate was 80 mg/g creatinine (nor- the eighth day. nephrocalcinosis was followed with mal, Ͼ180 mg/g creatinine, Table She was evaluated every month ultrasonography of the kidneys at 13 1). Urinary oxalate was 1.1 mg/kg for the first 5 months, during which months, 20 months, 2 years, 4 years, per day (normal, Ͻ2 mg/kg per day, Bicitra (1 mLϭ1 mEq) was increased and 5 years of age. The kidneys con- Table 1). to 8 mL tid. At 10 months of age, the tinued to grow, but the nephrocalci- After intravenous infusion of dose was adjusted to 12 mL qid and nosis in the medullary pyramids per- 4 mEq/kg per day of sodium bicar- at age 13 months was modified to a sisted. At 5 years of age, the right bonate, the metabolic acidosis was daily divided dosage of 12 mL, 12 kidney was 7.23 cm and the left kid- corrected and the tachypnea re- mL, and 24 mL, with the larger dose ney 8 cm, which was within normal solved. The diagnosis of RTA was at bedtime. The rationale was that values. The urine pH was 8 and spe- confirmed when the urine minus growth hormone secretion is highest cific gravity was 1.001, and the urine blood partial pressure of CO2 was at night and she would benefit from was negative for blood and protein. found to be less than 17 mm Hg better correction of metabolic acido- The Bicitra therapy was modified initially and 10.7 mm Hg on repeat sis with a larger dose at bedtime. At at 5 years of age to 24 mL in the measurement (normal, Ͼ20 mm Hg, 20 months of age, her height was morning, 12 mL at noon, and 36 mL Fig. 1). The child was started on 79 cm (10th percentile) and her at night (total, 5 mEq/kg per day). Bicitra brand of sodium citrate and weight was 9.23 kg (5th percentile). The corresponding electrolyte pattern citric acid oral solution 5 mL (5 The average dose of alkali therapy was sodium, 139 mEq/L (139 mmol/ mEq) qid. was 5 to 6 mEq/kg per day. She L); potassium, 4.3 mEq/L (4.3 mmol/ During the admission, the infant subsequently was followed every 3 L); chloride, 105 mEq/L (105 mmol/ alternately breastfed and was given months for the third year of life and L), CO2, 23 mEq/L (23 mmol/L); 45 mL of iron-fortified forumla every 6 months thereafter. serum urea nitrogen, 11 mg/dL (20 kcal/oz) every 3 to 4 hours. On Urine calcium-to-creatinine ratio (3.9 mmol/L); and creatinine, the fourth hospital day, a soy-based was monitored at each follow-up visit 0.5 mg/dL (44.2 mcmol/L). formula was substituted. She toler- and was documented at normal val- The last ultrasonography was ob- ated Bicitra added to the formula and ues of less than 0.20 mg/mg, usually tained at 6 years of age. Bilateral 278 Pediatrics in Review Vol.22 No.8 August 2001 consultation with the specialist Case Discussion The initial growth retardation of the child described in the case is related to the 33-week gestation, but the RTA also plays a significant role. Even with the increased therapeutic dose of Bicitra at 5 mEq/kg per day, growth did not increase significantly until the second year, when a double dose began to be administered be- fore bedtime. The presence of severe metabolic acidosis with serum bicarbonate less than 12 mEq/L (12 mmol/L) clini- cally associated with tachypnea illus- trates the logical steps to pursue the diagnosis of RTA (Table 2). In many patients, RTA may present with poly- uria, constipation, and failure to thrive. The polyuria of RTA is in- duced partly by the hypercalciuria, but the chronic hypokalemia from potassium wasting (vide infra) also contributes to the lack of urine con- centrating ability. Damage to the pa- pillae and their collecting systems caused by interstitial nephritis of chronic hypokalemia also may con- tribute. The urine specific gravity of this child was consistently low and at Figure 1. A. Minimum urine pH versus fractional excretion of the latest follow-up was 1.000. The bicarbonate (%). Closed squares and closed circles represent constipation seen in patients who healthy individuals and patients who have proximal, type 2 RTA, have RTA is due to the muscle weak- respectively.
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