Introduction: Hyperkalemia renal tubular acidosis (type IV) occurs secondary to impaired renal responsiveness to aldosterone. This can occur chronically in infants and children with a history of obstructive uropathy. The aim of this study was to assess of prognostic factors in children RTA type IV overwhelmed by bilateral obstructive uropathy.Materials and Methods: In this study, we recruited and observed 48 male patients with both bilateral obstructive uropathy at the urinary bladder outlet and RTA type IV for two years. In this period, we registered patients’ demographic data; also, children’s growth, sonographic data, renal function tests and serum electrolytes underwent serial assessment and in if clinically indicated, the patients were treated with drugs like citrate sodium and Kay oxalate.Results: Frequent urinary tract infection (p=0.0011), abnormal &lt;20 weeks gestation sonography results like bilateral hydronephrosis (p=0.00001), birth weight below 2500 gr (LBW) (p=0.0014), preterm delivery (p=0.001), maternal age at birth below 20 years (p=0.0018), pregnancy more than 2 times (p=0.004), admission due to respiratory problems during infancy (p=0.003) and gestational diabetes (P=0.001) were significantly associated with a poor prognosis.Conclusions: Considering the results of this study, it seems logical to consider abortion in case of renal hydronephrosis and dysplasia in gestational age below 20 weeks.  Keywords: Hyperkalemia; Urologic Diseases; Renal Tubular Acidosi

Sharafkhah, Mojtaba

Yousefichaijan, Parsa

English

Journals Portal, Shahid Beheshti University of Medical Sciences

 Prognostic Factors in RTA Type IV – Yousefichaijan P et al                                                  Journal of Pediatric Nephrology | Volume 1 | Number 2| October 2013    61  Research Article  J Ped. Nephrology 2013 Oct;1(2):61-64                http://journals.sbmu.ac.ir/jpn Assessment of Prognostic Factors in Children with Renal Tubular Acidosis Type IV Overwhelmed by Bilateral Obstructive Uropathy  How to Cite This Article: Yousefichaijan P, Sharafkhah M. Assessment of Prognostic Factors in Children with Renal Tubular Acidosis Type IV Overwhelmed by Bilateral Obstructive Uropathy. J Ped. Nephrology 2013 Oct 1(2):61-64.  Parsa Yousefichaijan,1 Mojtaba Sharafkhah 2*  1 Associate Professor of Pediatric Nephrology, Department, School of Medicine, Arak University of Medical Sciences, Arak, Iran 2 Students Research Committee, School of Medicine, Arak University of Medical Sciences, Arak, Iran   * Corresponding author Mojtaba Sharafkhah  Students Research Committee, Arak University of Medical Sciences, Arak, Iran. E-mail: sharafkhah@arakmu.ac.ir  Introduction: Hyperkalemia renal tubular acidosis (type IV) occurs secondary to impaired renal responsiveness to aldosterone. This can occur chronically in infants and children with a history of obstructive uropathy. The aim of this study was to assess of prognostic factors in children RTA type IV overwhelmed by bilateral obstructive uropathy. Materials and Methods: In this study, we recruited and observed 48 male patients with both bilateral obstructive uropathy at the urinary bladder outlet and RTA type IV for two years. In this period, we registered patients’ demographic data; also, children’s growth, sonographic data, renal function tests and serum electrolytes underwent serial assessment and in if clinically indicated, the patients were treated with drugs like citrate sodium and Kay oxalate.  Results: Frequent urinary tract infection (p=0.0011), abnormal <20 weeks gestation sonography results like bilateral hydronephrosis (p=0.00001), birth weight below 2500 gr (LBW) (p=0.0014), preterm delivery (p=0.001), maternal age at birth below 20 years (p=0.0018), pregnancy more than 2 times (p=0.004), admission due to respiratory problems during infancy (p=0.003) and gestational diabetes (P=0.001) were significantly associated with a poor prognosis. Conclusions: Considering the results of this study, it seems logical to consider abortion in case of renal hydronephrosis and dysplasia in gestational age below 20 weeks.   Keywords: Hyperkalemia; Urologic Diseases; Renal Tubular Acidosis   Received: June-2013  Revised: July-2013 Accepted: Sep-2013 Running Title: Prognostic Factors in RTA Type IV   Introduction Type IV renal tubular acidosis (RTA) occurs secondary to impaired renal responsiveness to Aldosterone (pseudohypoaldosteronism). Acidosis is the inevitable result since Aldosterone has a direct effect on H+/ATPase responsible for hydrogen secretion [1]. Moreover, Aldosterone is a potent stimulant for potassium secretion in the collecting tubule; therefore, lack of Aldosterone   results in hyperkalemia in children. Aldosterone unresponsiveness is usually a common cause of type IV RTA. This can occur transiently, during an episode of acute pyelonephritis or acute urinary obstruction in children. It may occur chronically, particularly in infants and children with a history of obstructive uropathy [2]. The latter patients can have significant hyperkalemia, even in instances when renal function is normal or only mildly  Prognostic Factors in RTA Type IV – Yousefichaijan P et al                                                  Journal of Pediatric Nephrology | Volume 1 | Number 2| October 2013    62  impaired [3]. Patients with type IV RTA, like those with type I and II RTA, can present with growth failure in the first few years of life. Polyuria and dehydration (from salt wasting) are common [3]. Rarely, patients (especially those with pseudohypoaldosteronism type 1) present with life-threatening hyperkalemia. Patients with obstructive uropathy can present acutely with signs and symptoms of pyelonephritis, such as fever, vomiting, and foul-smelling urine [3]. Urinary tract obstruction can result from congenital (anatomic) lesions or can be caused by trauma, neoplasia, calculi, inflammatory processes, or surgical procedures although most childhood obstructive lesions are congenital [2]. Obstructive lesions occur at any level from the urethral meatus to the calyceal infundibulum. The pathophysiologic effects of obstruction depend on its level, the extent of involvement, the child's age at onset, and whether it is acute or chronic [2].  Obstruction of the urinary tract generally causes hydronephrosis, which is typically asymptomatic in its early phases. An obstructed kidney secondary to a ureteropelvic junction (UPJ) or ureterovesical junction obstruction can manifest as a mass or cause upper abdominal or flank pain on the affected side [2]. Pyelonephritis can occur because of urinary stasis. An upper urinary tract stone can occur, causing abdominal and flank pain and hematuria. With bladder outlet obstruction, the urinary stream may be weak; urinary tract infection is also common. Many of these lesions are identified by antenatal ultrasonography; an abnormality involving the genitourinary tract is suspected in as many as 1/100 fetuses[2]. In our study, we focused on the assessment of prognostic factors in children RTA type IV overwhelmed by bilateral obstructive uropathy. Materials and Methods In this study, we recruited and observed 48 male patients with obstructive uropathy and a diagnosis of RTA type IV at birth or during infantile period who had hydronephrosis, dysplasia, hydrouretronephrosis or urinary bladder dilation in fetal sonography. The patients were observed for two years. We registered patients’ demographic data about birth weight, term situation at birth, maternal age at birth, gestational diabetes, number of pregnancies, admission due to respiratory problems during infancy, and the results of paraclinical tests like serum urea, creatinine, sodium, potassium, bicarbonate, urine analysis and culture and serial sonopgraphy (for kidney growth). Poor prognosis was suggested if three or more of the fallowing criteria were observed simultaneously: 1. GFR (Glomerular filtration rate) modifications 2. Impairment of growth and development 3. DMSA (dimercaptosuccinic acid) scan modifications 4. DTPA (Diethylene Triamine Pentacaetic Acid) scan modifications 5. Continued electrolyte abnormalities (hyperkalemia, acidosis) 6. Death Cases with a need for surgery were referred. Upon clinical indication, prophylactic antibiotics with 1/3 of the therapeutic dose, citrate sodium, and Kay oxalate were administered. In this period, the child’s growth, height, weight, head circumference and development were observed. The data was analyzed with SSPS version PASW statistics 18 (SPSS Inc., Chicago, IL, USA) using odds ratio, chi square and logistic regression test. Results Of 48 children in our study, 39 (81.25%) had the criteria of poor prognosis. The majority of the children had a history of admission due to respiratory problems during infancy. As a result, a significant relationship was found between admission due to respiratory problems during infancy and poor prognosis (P=0.003). Our 2-year study showed that of 48 children, 37 (77.08%) had a history of frequent UTI; these children also had the criteria of poor prognosis (P=0.0011). Regarding gestational diabetes, our results showed that of 39 children with poor prognosis, 33 (84.6%) had a history of gestational diabetes; therefore, this variable had a significant relationship with poor prognosis in our study population (P=0.001). Moreover, the results showed that birth weight below 2500 gr (LBW) (p=0.0014), infants and children with abnormal <20 weeks gestation sonography results like bilateral hydronephrosis (p=0.00001), preterm delivery (p=0.001), maternal age at birth below 20 years (p=0.0018) and pregnancy more than 2 times (p=0.004) were significantly associated with poor prognosis criteria. (Table 1)       Prognostic Factors in RTA Type IV – Yousefichaijan P et al                                                  Journal of Pediatric Nephrology | Volume 1 | Number 2| October 2013    63  Table 1. Correlation between demographic data and poor prognosis criteria in patients Key: VLBW= Very Low Birth Weight (<1500 g.), LBW= Low Birth Weight (1500-2500 g.), NL: Normal (2500-4000 g.) w= week, PV= P. value<0.05 is considered as the level of statistical significance.  Discussion Considering the results of this study, it seems logical to consider abortion in case of renal hydronephrosis and dysplasia in gestational age below 20 weeks. Moreover, medical care during pregnancy for a safe and term delivery with suitable fetus weight can considerably eliminate gestational complications and hypoxia seen in pre-term deliveries and low birth weight infants. On the other hand, since conception before 20 years of age is more likely to result in pre-term delivery, we recommend postponing conception to after this age to reduce pre-term delivery related complications. Studies dealing with obstructive uropathy have not cited RTA IV related complications. Nevertheless, in a study by Rothsterin M [4], RTA IV was found to be more common in patients with urinary outlet obstruction than other causes of bilateral obstruction. In other study performed by Kuymars [5], obstructive uropathy with low volume of amniotic fluid was associated with poor prognosis. In a study conducted by Gargah T [6], children with PUV obstruction who had renal dysplasia renal scar in fetus or infantile period had worse outcomes. Nasir AA [7], similar to us, concluded that dysplasia before 20 weeks gestation and persistence of serum creatinine above 0.8 heralded poor prognosis. Narasimhaakl [8] did not report any favorable prognostic factor. Similar to our findings, a study by Becher A [9] showed that sonography problems before 20 weeks gestation were associated with a poor prognosis. In another study by Salam MA [10], surgical intervention during pregnancy to reduce the pressure on renal parenchyma showed better results than non-surgical methods. Conclusions None of these studies focused on RTA type IV; therefore, it is prudent to lunch similar studies with more patients in different centers and longer observation periods to assess the overall prognosis in such patients.  Conflict of Interest None declared Financial Support None declared  References 1. Yousefi P, baghcheghi N, koohestani H.R. Renal diseases in children. 2012 - payam digar  2th edition  Arak University of Medical Sciences iran. 2. Gack S. Eider, Obstruction of the urinary tract kliegman, Stanton. geme, schor Behrman , Nelson Textbook of pediatrics 19th edition , Elsevier saunders, 2011 chapter S34. P1838-1846.  3. Ragasree sreedharan, Ellis d. Avener, Hyperkalemic (type Iv) renal tubular acidosis. In: kliegman, Stanton, St. Geme , Schor , Behrman , Nelson Textbook of pediatrics 19th edition Elsevier saunders, 2011, chapter 523/3.  P1810 – 1811.  4. Rothstcin M, Obialo c. H , Uslea La , Renal Tubular acidosis Endocrinol Metab Clin North Am 2007 Dec 19(4):869 – 89.  5. Humar S, Walia S, lhpeme O, et al. Postnatal outcome of prenatally diagnosis sever fetal renal pelvic dilatation. Prenat Diagn 2012 jun 32(6):519 – 22. 6. Gargah T, Gharbi Y, Ben Maussa M, Kaabar N, Lakgoua MR. Posteriar uretral valves . About a series of 44 cases. Tunis Med 2010 Aug: 88 (8):57 – 62.  7. Nasir AA, Ameh EA, Abdur – Rahman LO Adeniran go, Abrhcm Mh .  Posterior urethral value. World j pediatr , 2011 Aug 7 (3):205 -16.  Definition Number of patients Number of  Poor prognosis patients Statistical test PV Birth weight VLBW LBW Nl  >4000 g 4 20 21 3 0 (0%) 19 (95%) 2 (9.5%) 0 (0%)  0.0014 total 48 (100%) 21 (43.7%)  Term situation at birth Term: 37-40 w Pre term <37 w Post term>40w 29 19 0 2 (6.8%) 16 (84.2%) 0 (0%)  0.001 total 48 (100%) 18 (37.5%)  Mother's age at birth <20 years 20-30 years >30 years 19 24 5 18 (94.7%) 3 (12.5%) 0 (0%)  0.0018 total 48 (100%) 21 (43.7%)  Number of pregnancy 1 time 2 times >2 times 13 21 14 0 (0%) 2 (9.5%) 13 (92.8%)  0.004 total 48 (100%) 15 (31.2%)   Prognostic Factors in RTA Type IV – Yousefichaijan P et al                                                  Journal of Pediatric Nephrology | Volume 1 | Number 2| October 2013    64  8. Narasimhan KL, Choudhary SK, KaurB. Anterior urethral Valves. indian pediatr 2005 jul 42 (7):708 – 10.  9. Becher A, Baun M, Obstructive uropathy Early Hum Dev 2006 jan 82 (1):19-22.  10. Salam MA. Posterior urethral Valve: outcome of antenatal intervention. Int j urol 2006 oct 13 (10):1317– 22.  

Assessment of Prognostic Factors in Children with Renal Tubular Acidosis Type IV Overwhelmed by Bilateral Obstructive Uropathy

https://journals.sbmu.ac.ir/jpn/article/download/4534/4620

Assessment of Prognostic Factors in Children with Renal Tubular Acidosis Type IV Overwhelmed by Bilateral Obstructive Uropathy

Abstract

Similar works

Full text

Available Versions

Journals Portal, Shahid Beheshti University of Medical Sciences