Chapter 13 Cathy Poole The aim of this chapter is to help the reader develop an understanding of diseases of the renal system and the care required to help children and young people. Some disorders of the renal system are common, such as urinary tract infections, while others are less so, bladder exstrophy, for example. This chapter will discuss the most common disorders seen in the general children’s nursing environment and will also introduce you to some of the more complex and rarer disorders. When referring to renal disorders the reader should consider the urinary tract as a whole, kidneys, ureters, bladder, and the urethra (Fig. 13.1). Disorders of the urinary system are often referred to as either upper or lower urinary tract disorders (Peate & Gormley‐Fleming, 2015). This chapter is divided into disorders of the upper urinary tract and disorders of the lower urinary tract. In addition to this, management of disorders of the urinary system can be either medical or surgical, and in some cases a combination of both. The nursing care of the infant, child or young person with a medical or surgical disorder of the entire urinary system will be outlined. Prior to the advent of antenatal ultrasound screening few congenital malformations of the renal system were diagnosed until they caused symptoms in infancy, childhood, or later in adult life. The importance of early diagnosis is well recognised and a section in this chapter will be dedicated to renal anomalies that can be detected during the antenatal period. The value of undertaking specific renal nursing assessment will also be outlined, providing a renal system‐focused approach. Fig. 13.1 provides an illustration of the upper and lower urinary tract. Early identification of renal system anomalies is important as they may be associated with abnormal renal development and function. They may also predispose to postnatal infection and involve urinary tract obstruction, which may require surgical intervention (Lissauer & Clayden, 2007). Early detection, therefore, has the potential for early intervention and affords the opportunity to reduce, minimise or even prevent progressive renal system damage. However, a concern with this early detection is that very minor anomalies are also detected, the most common being renal pelvic dilatation (RPD). RPD rarely requires intervention, but does require follow‐up during the antenatal period and potentially further investigations postnatally depending on the degree of dilatation. This can consequently lead to unnecessary anxiety for the parents. It is estimated that antenatal anomalies of the urinary system are identified in 1 in 200–400 births (Lissauer & Clayden, 2007). Table 13.1 provides an insight into these anomalies. Table 13.1 Antenatal urinary tract abnormalities Source: Adapted from Lissauer & Clayden 2007, and infoKID; www.infokid.org.uk/ (accessed 6 December 2016). In summary, kidney and urinary tract anomalies are the most common antenatal abnormality detected. The use of antenatal ultrasound scanning is recommended during all pregnancies to aid early diagnosis of urinary tract abnormalities, thus establishing plans for postnatal care to optimise the outcome and reduce or delay the progress of renal function deterioration. Nursing assessment is a key component of nursing practice, which forms the foundation for planning and provision of patient‐ and family‐centred care. The Nursing and Midwifery Council (NMC) (2015) states that nurses must ‘make sure that people’s physical, social and psychological needs are assessed and responded to’. It is therefore really important when undertaking a holistic nursing assessment that criteria relating to the renal system are taken into account (see Table 13.2). Table 13.2 Assessment of the renal system Source: Adapted from The Royal Children’s Hospital Melbourne 2014. Commonly, infants, children and young people who present with symptoms of a urinary tract disorder will be required to undergo further tests to confirm the diagnosis. These tests may include the following; For a successful outcome to these specialist investigations, the child and family need full preparation, explanation and, where required, provide informed consent. The Great Ormond Street Hospital Manual of Children’s Nursing Practices, Chap. 14 (Macqueen, Bruce & Gibson, 2012), provides a comprehensive account of the nursing implications for these investigations as does infoKID, a web resource for parents and carers of children with renal disorders (www.infokid.org.uk). In summary, while this section has focused on renal‐specific assessment skills, these should form part of the full holistic assessment of all infants, children and young people for whom you care. Proteinuria is leakage of protein from the blood into the urine. Normally, significant amounts of protein do not pass into urine when the blood is filtered in the glomeruli, because protein molecules are too large to pass through the tiny holes (fenestrae) in the kidney filters. However, the glomeruli can be damaged in renal disease, which allows protein to pass into the urine. Most proteinuria is identified by urinalysis using a dipstick. It is quite normal for small amounts of protein to be detected in urine during febrile illnesses or after excessive exercise and this would not require further investigation. Additional investigations would, however, be required if proteinuria continues as there are several causes, and the management would be determined dependent upon the diagnosis (Table 13.3). Table 13.3 Some causes of proteinuria Source: Adapted from infoKID; www.infokid.org.uk/ (accessed 6 December 2016). Nephrotic syndrome can occur at any age and it affects more boys than girls. It often starts when a child is between 2 and 5 years old. There is a congenital form of nephrotic syndrome, Finnish type which appears during the first year of life; however, it is very rare. There are different types of nephrotic syndrome which are classified according to whether steroid drugs treat and resolve the symptoms, or what the causes of the symptoms are. Sometimes it is not possible to know what type of nephrotic syndrome children have until they have tried a course of steroid treatment. Children with nephrotic syndrome present with classical clinical signs: The amount of protein in the urine can be so large that the urine appears ‘frothy’ and is so full of albumin that it looks like the white of an egg. For 85–90% of children, the proteinuria resolves with corticosteroid therapy, the so‐called steroid‐sensitive nephrotic syndrome. Complications, for example, hypovolemia, thrombosis and infection, require close monitoring and the nursing team should be mindful of the following assessment needs: It may be necessary to undertake vital signs more frequently depending on the child’s clinical status. In summary, the majority of children who have nephrotic syndrome respond to steroid therapy, and while they may have relapses, they usually grow out of it. For further detail on some general renal nursing principles, please see the section on this later in the chapter. Glomerulonephritis is a type of kidney disease that involves the glomeruli, which sit in the Bowman’s capsule in the nephron (Fig. 13.3). During glomerulonephritis, the glomeruli become inflamed, which impacts on the kidney’s ability to filter urine normally. There are many causes of glomerulonephritis, for example: Children who present with glomerulonephritis can have a wide variety of signs and symptoms. The most common are listed in Table 13.4. Table 13.4 Possible signs and symptoms of glomerulonephritis Source: Adapted from Children’s Hospital of Philadelphia n.d. (accessed 6 December 2016). Diagnosis is made on the history and physical examination, supported by a variety of tests, which may include the following; The management and nursing care of children with glomerulonephritis is very much dependent upon the presenting symptoms and the underlying cause. Some children may need to have some dietary restrictions, for example, a no added salt diet, or a fluid restriction. Others may need to take medication, for example, antibiotics, antihypertensives and diuretics. On rare occasions children may be prescribed immunosuppressive drugs, and in even rarer cases may require dialysis. In summary, there are several causes of glomerulonephritis, the most common of which is associated with a streptococcal infection of the throat. For the majority of children the disease is mild, responds well to treatment and does not cause any long‐term renal disease. Henoch–Schönlein purpura (HSP) is a condition that can affect people of all ages; however, it is most often seen in children between the ages of 3 and 10 years, and is twice as common in boys. It is more common during the winter months and is frequently preceded by an upper respiratory tract infection. Children with HSP often present with a fever, and have a rash that is characteristically symmetrically distributed over the buttocks, feet and ankles, backs of legs, lower back and arms. Children with HSP also commonly complain of pain in their joints and abdominal colic‐like pain. Intussusception (see Chapter 14) can occur. Although renal involvement is common, it is not usually the first symptom (Lissaur & Clayden, 2007). Most children with HSP do not need special treatment, and many will be monitored as outpatients. The mainstay of treatment is symptom management, i.e., analgesia for joint pain. After several days or a few weeks, the majority of children begin to feel better and the rash and other symptoms disappear. HSP sometimes comes back, usually within a few months, and may need further treatment. A few children have long‐term problems, especially when their kidneys are affected. They will need to be monitored and may need specialist paediatric nephrology follow‐up. Haematuria indicates the presence of red blood cells in the urine. Urine does not normally contain red blood cells because these are too large to pass through the tiny holes (fenestrae) in the kidney filters. However, the glomeruli can be damaged in renal disease, which allows blood to pass into the urine. Microscopic haematuria means that the blood can only be detected during microscopy. Gross haematuria means the urine appears red in colour. Microscopic haematuria in healthy children does not usually need to be investigated unless it is present in at least three urine tests over several months. However, if a child has other symptoms, for example, hypertension or proteinuria, further investigations are warranted. Haematuria is a common finding in children and has many causes, which include the following: In summary, care and management of children with haematuria may be minimal to complex depending on the underlying cause. Chronic kidney disease (CKD) is characterised by permanent deterioration of renal function that gradually progresses to end‐stage renal disease (ESRD). Congenital disorders, including congenital anomalies of the kidney and urinary tract, are responsible for about 66% of all cases of CKD in children in developed countries. All data on CKD in adults and children is reported to the UK Renal Registry by the specialist renal units across the UK and Ireland. An annual report is published by the UK Renal Registry, a section being dedicated to an analysis of CKD data in children. The last UK Renal Registry (2015) reported that a total of 917 children and young people under 18 years with established renal failure (ERF) were receiving treatment at paediatric nephrology centres in 2014. This clearly demonstrates that CKD is very rare in children and out of these, 79.3% had a functioning kidney transplant, 11.2% were receiving haemodialysis, and 9.5% were receiving peritoneal dialysis (UK Renal Registry, 2015). There are a variety of causes of CKD (Table 13.5), the most common primary renal diagnosis being renal dysplasia with reflux. Table 13.5 Causes of CKD in children Source: UK Renal Registry 2015. Understanding normal renal function (Peate & Gormley‐Fleming, 2015) will enable you to appreciate the physiological consequences of CKD (Table 13.6), and to recognise the need for multidisciplinary specialist paediatric nephrology services. CKD is classified into five stages (Table 13.6), which are defined by the GFR. The GFR measures the volume in millilitres (mL) filtered by the kidneys each minute (min), which is an indication of kidney function. This is adjusted for children against a standard adult body size, which has a surface area of 1.73 square metres (m2). Table 13.6 Consequences of CKD Table 13.7 describes the stages of CKD and GFR. Table 13.7 Stages of CKD and GFR Source: infoKid; www.infokid.org.uk/ (accessed 6 December 2016). Unfortunately there is no cure for CKD. Treatment options include the following: In summary, CKD is rare in children. It has many causes, the most common of which is associated with structural congenital malformations, which require the support of a specialist paediatric renal team to provide holistic family‐centred care. There is no cure for CKD; however, with dialysis and transplantation many children with CKD now find themselves being transferred to adult renal units in their late teens. Acute kidney injury (AKI) can be defined as ‘an abrupt decline in renal function resulting in loss of water, electrolyte and acid–base regulation, with collection of nitrogenous waste material in the body’. This produces disturbance of water, electrolyte, acid–base balance, and nitrogenous waste products and blood pressure. In order to understand the consequences of AKI you need to fully understand normal renal function as described by Peate & Gormley‐Fleming (2015). Like CKD, AKI is classified into stages (Table 13.8). AKI is much more common in children than CKD, and there are many causes. These are classified as follows:
Disorders of the renal system
Aim
Introduction
Antenatal detection of renal anomalies
Antenatal anomaly detected
Brief description of anomaly
Renal agenesis (Potter syndrome)
Absence of both kidneys, associated with reduced volume of amniotic fluid (oligohydramniosis), lung hypoplasia, low‐set ears, beaked nose, downward eye slants, and limb abnormalities. Incompatible with life. Affects 1 in 4000 pregnancies.
Solitary kidney
Solitary kidney is a condition in which children have a single kidney instead of two kidneys.
Horseshoe kidney
During embryonic development the kidneys fuse together to form a horseshoe‐shape. Reported to affect about 1 in 600 children.
Duplex kidney
Children with a duplex kidney (also called a duplicated collecting system) have two ureters coming from a single kidney. Duplex kidneys can occur in one (unilateral) or both (bilateral) kidneys (Fig. 13.2).
Ectopic kidney (or renal ectopia)
An ectopic kidney does not grow in the proper location. It occurs in about 1 in 900 births. Infants, children and young people with an ectopic kidney usually have no symptoms, but sometimes it may cause urinary problems, e.g., urine blockage, infection, or urinary stones.
Hydronephrosis
One or both kidneys are stretched and swollen (dilated). It is quite common, affecting about 1 in 100 pregnancies. Most cases are not serious.
Multicystic dysplastic kidney (MCDK)
The kidneys have bundles of cysts in them, which are like sacs filled with liquid. MCDK can be unilateral or bilateral. The cysts can be very large initially; however, over time they become smaller and the kidney gradually shrinks.
Posterior urethral valves (PUV)
Affects only boys as only boys have a posterior urethra. PUV are extra flaps of tissue in the urethra. Babies with PUV may not be able to micturate normally, during fetal development and after they are born.
Assessment of the renal system
Urinary symptoms: enuresis (bed wetting), new‐onset incontinence, frequency, urgency, quantity, dysuria and its timing during voiding (at beginning or end, throughout), change in colour and odour of urine, haematuria, presence of stones or sediment in the urine, toilet‐training problems, reduced urine output.
Hydration status, including blood pressure, fluid balance, and weight, ankle and eye oedema (periorbital oedema).
Growth and feeding, diet or fluid restrictions, sudden weight loss.
Urine output (neonates 1–3 mL [kg/h]; children <2 yrs 2–3 mL [kg/h]; >2 yrs 0.5–1 mL [kg/h]).
Urinalysis (pH, ketones, protein, blood, leukocytes, nitrite, glucose, specific gravity). Proteinuria and haematuria are early signs of disorders of the renal system.
Review blood chemistry results, urea, creatinine, electrolytes, albumin and haemoglobin, and estimated GRF (glomerular filtration rate).
Skin condition: temperature, turgor and moisture, colour, evidence of any rashes, enlarged painful nodes (axilla, groin).
Inexplicable crying, holding genitalia, discolouration of external genitalia, pain or swelling of the testicles, penile discharge, itching or swelling of the genitalia, urethral or vaginal discharge.
Location of pain, flank, suprapubic, genital, groin or low back pain.
Medication: prescribed, over‐the‐counter or herbal remedies and supplements.
Medical disorders of the upper urinary tract
Proteinuria
Nephrotic syndrome
Glomerulonephritis
Renal dysplasia
Vesicouretric reflux (VUR)
Vasculitis
Orthostatic proteinuria
Acute kidney injury (AKI)
Renal tubular diseases – very rare in children, Fanconi syndrome, for example
Hypertension
Diabetes – rare in children very common in adults, known as diabetic nephropathy
Nephrotic syndrome
Glomerulonephritis
Haematuria and proteinuria
Urine looks dark brown in colour
Fluid overloaded
Hypertensive
Increase in body weight
Oedema
Headaches
Seizures
Reduced urine output (oliguria)
Increased respiratory rate
Changes in physical appearance
Pale skin colour
Skin rash, especially over the buttocks and legs
Lethargic
Other
Sore throat
Decreased appetite
Henoch–Schönlein purpura
Haematuria
Chronic kidney disease
Renal dysplasia with reflux
Obstructive uropathy
Glomerular disease
Congenital nephrotic syndrome
Tubulo‐interstitial disease
Renovascular disease
Polycystic kidney disease
Metabolic
Malignancy and associated disease
Functions of the kidney
Clinical consequence of CKD
Fluid balance
Reduced/no urine output (oliguria/anuria) leading to fluid overload, peripheral oedema, pulmonary oedema, shortness of breath.
Removal of waste
Uraemia which causes nausea, vomiting and loss of appetite.
Persistent itchy skin (pruritus) due to uraemia.
Renin and angiotensin secretion
Reduced blood supply (hypoperfusion) to damaged nephrons causes increased secretion of renin, angiotensin and aldosterone causing hypertension.
Erythropoietin (EPO) secretion
Reduced production of EPO and the circulation of toxic waste substances cause renal anaemia.
Anorexia and lethargy, failure to thrive.
Vitamin D synthesis
Without vitamin D synthesis phosphate is retained and calcium levels drop (hypocalcaemia), which results in secondary hyperparathyroidism. If left untreated bones become painful, brittle and are prone to fracture (rickets, renal bone disease).
Red eyes of uraemia, due to high plasma phosphate levels.
Acid–base balance
Reduction in the secretion of hydrogen ions culminates in metabolic acidosis.
Electrolyte balance
Hyperkalaemia causing heart arrhythmia.
Kidneys normally excrete 90% of daily potassium intake.
Stage
GFR
Kidney function
What this means
1
90 or higher
Normal, but other signs of kidney disease
Normally no symptoms
2
60–89
Mildly reduced
Normally no symptoms
3a
45–59
Moderately reduced
Normally no symptoms
3b
30–44
Moderately reduced
Children may start to have symptoms of CKD
4
15–29
Severely reduced
Many children have more symptoms of CKD.
Start to plan for treatment options for next stage
5
Less than 15
Very severely reduced and cannot support the body
This is also called end‐stage renal failure (ESRF) or established renal failure.
Children are started on treatments, including dialysis and kidney transplantation
Acute kidney injury