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 Table of Contents  
Year : 2015  |  Volume : 12  |  Issue : 1  |  Page : 39-41

Nephropathy in a 5-year-old Nigerian child with cyanotic congenital

Department of Paediatrics, Aminu Kano Teaching Hospital, Bayero University, Kano, Nigeria

Date of Web Publication5-Jan-2015

Correspondence Address:
Ibrahim Aliyu
Department of Paediatrics, Aminu Kano Teaching Hospital, Bayero University, Kano, Kano State
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0189-7969.148486

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Nephropathy in cyanotic congenital heart disease is common and the dominant feature is glomerular damage which is related to the duration of cyanosis and the extent to which the hematocrit is elevated. Previous reports have been mostly on adult Caucasians and a few case reports in Nigerian children. However nephrotic syndrome has been rarely reported and rarer still gross hematuria in a child. I report a case of a 5-year-old Nigerian boy with tetralogy of Fallot with recurrent gross hematuria and nephrotic syndrome.

Keywords: Cyanotic congenital heart disease, nephrotic syndrome, recurrent gross hematuria, tetralogy of Fallot

How to cite this article:
Aliyu I. Nephropathy in a 5-year-old Nigerian child with cyanotic congenital. Nig J Cardiol 2015;12:39-41

How to cite this URL:
Aliyu I. Nephropathy in a 5-year-old Nigerian child with cyanotic congenital. Nig J Cardiol [serial online] 2015 [cited 2023 May 30];12:39-41. Available from: https://www.nigjcardiol.org/text.asp?2015/12/1/39/148486

  Introduction Top

Complications associated with congenital cyanotic heart disease are infective endocarditis, cerebral abscess; others are stroke, iron deficiency and thrombocytopenia, but renal complications are rare. [1],[2] Most of the findings of renal complications had been in adult though few case reports have been documented among Nigerian children. [3],[4] Proteinuria is the commonest renal complication in children with congenital heart disease and rarely does it progress to nephrotic syndrome (NS) and there is a dearth of information on its association with gross hematuria. Therefore a case of a 5-year-old boy with tetralogy of Fallot who presented with recurrent gross hematuria and NS is reported.

  Case Report Top

This paper reports a case of a 5-year-old boy presented with a recurrent difficulty in breathing and cyanosis since age of 6 months. These had been progressively increasing in severity and worsened on crying and playing. There was also history of squatting. There was no history of fever. He was the 4 th child in a monogamous non-consanguineous family of four children. There was no family history of diabetes mellitus or hypertension. The pregnancy and delivery were not adversely eventful. There was no history of maternal alcohol ingestion and no similar problem in any other members of the family. At the 3 rd year of life, parents noticed bilateral eye swellings worse in the morning, which later became generalized body swelling associated with the passage of reddish urine.

On examination, he was afebrile, had anarsaca [Figure 1], conjunctival plethora [Figure 2], central cyanosis [Figure 3] with grade III finger clubbing [Figure 4]. He had oxygen saturation of 85% in room air, full volume regular peripheral pulses, pulse rate: 60 bpm, blood pressure: 90/70 mmHg right arm supine, with apex beat at the 4 th left intercostal space mid-clavicular line; heart sounds were S1 and single S2 with a Grade 3/6 ejection systolic murmur maximal at the left upper sternal edge radiating upwards. The liver and spleen were not palpably enlarged, while the kidneys were not ballotable. He had proteinuria of 3 + on 3 consecutive days with serum albumin of 20 g/L (32-51 g/L); total protein of 60 g/L (59-86 g/L) g/dL and protein clearance of 3.8 g/m 2 ; the urea was 18 mmol/L (2.1-6.9 mmol/L); Na + of 128 mmol/L (130-146 mmol/L); K + of 4.5 mmol/L (3.0-5.6 mmol/L); Hco 3 of 19 mmol/L (20-28 mmol/L); Cl of 85 mmol/L (94-108 mmol/L); and creatinine of 300 umol/L (30-111 umol/L); the urine microscopy showed numerous dysmorphic red blood cells. Renal biopsy was not done.
Figure 1: Peri-orbital and facial puffiness with abdominal swelling

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Figure 2: Conjunctival plethora

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Figure 3: Bluish tongue

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Figure 4: Digital clubbing

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Full blood count showed packed cell volume of 68%, with hypochromic microcytic red blood cells, and normal platelet counts. Chest radiograph showed boot shaped heart and pulmonary oligaemia [Figure 5]. Electrocardiogram showed atrioventricular dissociation with ventricular rate of 60/min in keeping with third degree heart block with accelerated idioventricular rhythm [Figure 6] Transthoracic 2-D echocardiography showed right ventricular hypertrophy, a large ventricular septal defect with overriding of the aorta and thickening and doming of the pulmonary valves with narrowing of the infundibulum. The diagnosis of tetralogy of Fallot with infundibular stenosis, NS; third degree heart block with accelerated idioventricular rhythm was made. He had partial exchange with normal saline and was placed on captopril; the heart rate progressively declined despite being on atropine. Facility for cardiac pace-maker was not available; bradycardia worsened and the patient died on the 4 th day of admission.
Figure 5: Boot-shape heart with pulmonary oligaemia

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Figure 6: Electrocardiogram showing third degree heart block

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  Discussion Top

Renal damage occurs in congenital cyanotic heart disease; [5] this may involve glomerular and tubular injuries; though glomerular injury is commoner and may manifest as proteinuria and rarely progressing to NS. Factors implicated in its pathogenesis include hypoxia, polcythaemia, and hyperdynamic circulation. [6] Renal biopsy often shows glomerulomegaly, capillary dilatation, thickening of the capillary walls, focal or diffuse proliferation of mesangial cells and segmental/global glomerulosclerosis. [6] NS is characterized by massive loss of urinary protein (primarily albuminuria) leading to hypoproteinemia (hypoalbuminemia) resulting in edema. Hyperlipidemia, hypercholesterolemia, and increased lipiduria are usually associated. Although not commonly thought of as part of the syndrome, hypertension, hematuria, and azotemia may occur. Though most of the reports have been among Caucasian adults, [7] three cases have been reported among Nigeria children, which highlights the fact that; renal complication may set in very early in life and that its not been reported among Nigerian adults could mean that most of these children do not survive to adulthood due to lack of surgical intervention. Furthermore, our case presented with complaints of recurrent body swellings since the age of 3-years earlier than those reported by Adedoyin and Afolabi [3] and Ogunkunle et al., [4] (their patients were diagnosed at the age of 9-years; and 7 and 12-years respectively), which means the age of onset of nephropathy maybe earlier. Recurrent gross hematuria before now had not been reported as a major complaint among those with cyanotic congenital heart disease-associated nephropathy, which makes this case unique; therefore the spectrum of its clinical presentation is extensive.

The role of iron deficiency in the development of renal complication is not clear unlike in the pathogenesis of stroke, hypercyanotic spell and cerebral abscess in cyanotic heart disease. [8] This patient presented late in the course of his illness and despite partial exchange transfusion and been on angiotensin-converting enzyme (ACE) inhibitor [9],[10] therapy patient's renal status progressively deteriorated with worsening edema. Therefore early detection and institution of treatment should be the keyword and health-care facilities should be strengthened to cater for such patients.

I advocate that it should be routine practice to evaluate patients with congenital heart disease for proteinuria because early detection and commencement of treatment with ACE inhibitors will reduce the risk of progression to severe renal damage, overt NS and renal failure.

  Conclusion Top

Nephropathy occurs in children with cyanotic congenital heart disease and the spectrum of clinical presentation is variable; while proteinuria is the most common manifestation, NS, recurrent gross hematuria may also occur and clinicians should routinely exam the urine of patients with cardiac disease to ensure early detection and treatment of the renal complications.

  References Top

Perloff JK, Marelli AJ, Miner PD. Risk of stroke in adults with cyanotic congenital heart disease. Circulation 1993;87:1954-9.  Back to cited text no. 1
Kurlan R, Griggs RC. Cyanotic congenital heart disease with suspected stroke. Should all patients receive antibiotics? Arch Neurol 1983;40:209-12.  Back to cited text no. 2
Adedoyin OT, Afolabi JK. Sudden deterioration in the renal function of an African child with cyanotic congenital heart disease. J Natl Med Assoc 2006;98:287-9.  Back to cited text no. 3
Ogunkunle OO, Asinobi AO, Omokhodion SI, Ademola AD. Nephrotic syndrome complicating cyanotic congenital heart disease: A report of two cases. West Afr J Med 2008;27:263-6.  Back to cited text no. 4
Agras PI, Derbent M, Ozcay F, Baskin E, Turkoglu S, Aldemir D, et al. Effect of congenital heart disease on renal function in childhood. Nephron Physiol 2005;99:p10-5.  Back to cited text no. 5
Hida K, Wada J, Yamasaki H, Nagake Y, Zhang H, Sugiyama H, et al. Cyanotic congenital heart disease associated with glomerulomegaly and focal segmental glomerulosclerosis: Remission of nephrotic syndrome with angiotensin converting enzyme inhibitor. Nephrol Dial Transplant 2002;17:144-7.  Back to cited text no. 6
Flanagan MF, Hourihan M, Keane JF. Incidence of renal dysfunction in adults with cyanotic congenital heart disease. Am J Cardiol 1991;68:403-6.  Back to cited text no. 7
Rose SS, Shah AA, Hoover DR, Saidi P. Cyanotic congenital heart disease (CCHD) with symptomatic erythrocytosis. J Gen Intern Med 2007;22:1775-7.  Back to cited text no. 8
Takahashi S, Ooya K, Wada N, Inagaki T, Takana Y, Saito A, et al. Partial exchange transfusion improved renal function and proteinuria in a patient with cyanotic heart disease nephropathy. Clin Nephrol 2003;60:222-3.  Back to cited text no. 9
Fujimoto Y, Matsushima M, Tsuzuki K, Okada M, Shibata M, Yanase Y, et al. Nephropathy of cyanotic congenital heart disease: Clinical characteristics and effectiveness of an angiotensin-converting enzyme inhibitor. Clin Nephrol 2002;58:95-102.  Back to cited text no. 10


  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]


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