hyponatremic hypertensive syndrome in a preterm infant with twin anemia polycythemia sequence

M ANIP T Hyponatremic hypertensive syndrome in a preterm infant with twin anemia polycythemia sequence Yun jeong Lee1 , Seung hyun Shin1 , Sae Yun Kim2 , Seung Han Shin1 , Young hoon C

Hyponatremic hypertensive syndrome in a preterm infant with twin anemia

polycythemia sequence

Yun jeong Lee, Seung hyun Shin, Sae Yun Kim, Seung Han Shin, M.D, Young hoon

Choi, Ee-Kyung Kim, Han-Suk Kim

PII: S1875-9572(16)30242-X

DOI: 10.1016/j.pedneo.2016.08.003

Reference: PEDN 616

To appear in: Pediatrics & Neonatology

Received Date: 18 May 2016

Revised Date: 25 July 2016

Accepted Date: 26 August 2016

Please cite this article as: Lee Yj, Shin Sh, Kim SY, Shin SH, Choi Yh, Kim E-K, Kim H-S, Hyponatremic

hypertensive syndrome in a preterm infant with twin anemia polycythemia sequence, Pediatrics and

Neonatology (2016), doi: 10.1016/j.pedneo.2016.08.003.

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Hyponatremic hypertensive syndrome in a preterm infant with

twin anemia polycythemia sequence

Yun jeong Lee1 , Seung hyun Shin1 , Sae Yun Kim2 , Seung Han Shin1 , Young hoon Choi3 , Ee-Kyung Kim1 , Han-Suk Kim1

Author Affiliations:

1

Department of Pediatrics, Seoul National University College of Medicine, Seoul, Korea

2

Department of Pediatrics, Kangwon National University Hospital, Chuncheon, Korea

3

Department of Radiology, Seoul National University College of Medicine, Seoul, Korea

Address correspondence to:

Seung Han Shin M.D

Department of Pediatrics, Seoul National University College of Medicine,

101 Daehak-ro Jongno-gu, Seoul 110-799, Korea

Tel: +82-2-2072-3555

Fax: +82-2-2072-0590

E-mail: revival421@snu.ac.kr

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Brief Communication

Hyponatremic hypertensive syndrome in a preterm infant with twin anemia-polycythemia sequence

Running title: HHS in a preterm infant with TAPS

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Abstract

We report a preterm infant with twin anemia-polycythemia sequence (TAPS) who experienced hyponatremic hypertensive syndrome (HHS) in early neonatal period Sudden irritability and aggravated apnea developed at the twelfth day after birth with hypertension and polyuria Hyponatremia, hypernatriuria, and hypocalcemia were accompanied In HHS, excessively stimulated renin-angiotensin-aldosterone system induced by ischemic kidney is responsible for polyuria and renal electrolyte loss from normal kidney through pressure diuresis and natriuresis The baby had umbilical arterial catheter and was donor of TAPS, which could contributed to transient unilateral renal ischemia He received fluid therapy and antihypertensive medication, and HHS was improved in a few days Early recognition and targeted prompt management resulted in favorable outcome

Key words

Hypertension, Hyponatremia, Preterm infant

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Introduction

Hyponatremic hypertensive syndrome (HHS), in which the unilateral ischemic kidney

induces the activation of renin-aldosterone-angiotensin system (RAAS) which affects the contralateral normal kidney that excretes water and sodium, is rarely reported in preterm infants.1,2

Twin anemia-polycythemia sequence (TAPS) occurs in 3–5% of the monochorionic twins, and it is characterized by large inter-twin hemoglobin differences without signs of twin oligo-polyhydramnios sequence.3 As in case of the donor of twin-to-twin transfusion syndrome (TTTS), the donor of TAPS could experience decreased renal function anecdotally Herein,

we present the first case report of HHS in the donor of TAPS among preterm triplets

Case report

A preterm boy was delivered at a gestational age of 31+1 weeks with birth weight of 1,290 g

as the third baby among triplets His mother underwent an emergency cesarean section due to severe preeclampsia Among the triplets, the second (male, birth weight 1,520 g) and the third babies developed TAPS and they shared the same chorion The third baby was the donor part who developed anemia (hemoglobin: 9.1 g/dL, hemoglobin level of the 2nd baby: 28.4 g/dL) and low blood pressure An umbilical arterial catheter (UAC) was inserted for blood pressure monitoring and it was removed after three days

At the twelfth day after birth, sudden irritability and mottled skin developed with aggravation of apnea Laboratory findings revealed hyponatremia, hypocalcemia, and hypernatriuria Renin and aldosterone levels were elevated (Table 1) Hypertension was detected on the same day (mean blood pressure of 86 mmHg, above the 95th percentile for his age), and polyuria up to 9.4 mL/kg/hour developed subsequently Kidney ultrasonography revealed a perfusion defect in the mid to lower portion of the right kidney without detectable

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vascularity (Supplementary Fig 1A)

Fluid therapy targeting the cutting off the vicious cycle of volume depletion and continuous infusion of intravenous nicardipine for blood pressure control was provided to the baby With stabilization of blood pressure, polyuria subsided, and sodium levels in the blood and urine were normalized within three days (Supplementary Fig 2) At the nineteenth day after birth, all intravenous fluids were discontinued and follow-up kidney ultrasonography at the twenty-seventh day showed partial improvement of the perfusion defect in the right kidney (Supplementary Fig 1B) There were no seizures, and the amplitude-integrated electroencephalogram and serial brain ultrasonography revealed normal findings

He was discharged from the neonatal intensive care unit on oral amlodipine on the forty-fifth day of life At three months of age, his blood pressure had normalized and amlodipine was discontinued He had no abnormal neurological signs or developmental delay at twelve months

Discussion

In this case report, we described a preterm infant who was a donor of TAPS and who experienced HHS during the early neonatal period In HHS, the excessively stimulated RAAS induced by the unilateral ischemic kidney is responsible for polyuria and renal electrolyte loss from the contralateral normal kidney through pressure diuresis and natriuresis It results in severe volume depletion which stimulates secretion of the antidiuretic hormone (ADH), and it further aggravates hyponatremia together with RAAS Proteinuria, glycosuria, and hypercalciuria can be present due to glomerular hyperfiltration.2,4 Our case showed increased renin and aldosterone activity, which decreased with improvement of symptoms Elevated

ADH and B-type natriuretic peptide levels were also detected

The baby had received an UAC and was a donor of TAPS, which could have contributed to

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transient unilateral renal ischemia UAC is a well-known cause of hypertension in neonates and is frequently associated with thrombosis.5 Decreased renal blood flow in the donor of TTTS subsequent to hypovolemia could occur, followed by renal dysfunction.6 TAPS is considered as a form of TTTS, and a recent study showed that the donor of TAPS had higher creatinine levels than the recipient because of chronic inter-twin transfusion.7

In preterm infants, the symptoms of HHS are vague and nonspecific, which make it difficult

to detect the problem.2 In our case, the boy also showed sepsis-like clinical presentation Treatment of HHS should be based on understanding of the underlying pathophysiology, combination of early management of hypovolemia, correction of electrolyte imbalance and control of hypertension With normalization of blood pressure, symptoms of HHS can be

resolved

In conclusion, HHS might be considered in neonates who have hyponatremia and

hypertension Risk factors, such as a history of indwelling UAC or unstable hemodynamics including TTTS or TAPS, should be reviewed Early recognition is important, and vicious

cycle of abnormally activated RAAS should be broken by prompt and targeted management

There is no conflict of interest

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References

1 Bourchier D Hyponatraemic hypertensive syndrome in two extremely low

birthweight infants J Paediatr Child Health 2003;39:312-4

2 van Tellingen V, Lilien M, Bruinenberg J, de Vries WB The hyponatremic hypertensive syndrome in a preterm infant: A case of severe hyponatremia with neurological

sequels Int J Nephrol 2011;2011:406515

3 Slaghekke F, Kist WJ, Oepkes D, Pasman SA, Middeldorp JM, Klumper FJ, et al Twin anemia-polycythemia sequence: Diagnostic criteria, classification, perinatal

management and outcome Fetal Diagn Ther 2010;27:181-90

4 Nicholls MG Unilateral renal ischemia causing the hyponatremic hypertensive

syndrome in children more common than we think? Pediatr Nephrol 2006;21:887-90

5 Revel-Vilk S, Ergaz Z Diagnosis and management of central-line-associated

thrombosis in newborns and infants Semin Fetal Neonatal Med 2011;16:340-4

6 Mahieu-Caputo D, Dommergues M, Delezoide AL, Lacoste M, Cai Y, Narcy F, et al

Twin-to-twin transfusion syndrome Role of the fetal renin-angiotensin system Am J Pathol

2000;156:629-36

7 Verbeek L, Slaghekke F, Favre R, Vieujoz M, Cavigioli F, Lista G, et al Short-term

postnatal renal function in twin anemia-polycythemia sequence Fetal Diagn Ther 2016;39:

192-7

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Table 1 Laboratory values of the case (plasma and urine)

Urine (Spot collection)

*

Reference range of neonates or children

BNP (B-type natriuretic peptide), ADH (Antidiuretic hormone)

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Figure legends

Supplementary figure 1 (A) Initial renal Doppler ultrasonography obtained on the thirteen day showed a perfusion defect in the mid to lower portion of the right kidney (arrow) (B)

Follow-up ultrasonography performed on the twenty-seven day showed improved but slightly decreased perfusion in the same area (arrow).

Supplementary figure 2 Changes in blood pressure, sodium concentration (capillary) and

urine output