1. Pediatric Nursing Unit Ten Child with Genitourinary Disorders

2. Introduction: The genitourinary system is responsible for maintaining metabolism and for expelling waste products from the body. The genitourinary system consists of the kidneys, nephrons, distal tubules, collecting ducts, renal pelvis, ureter, bladder, urethra, and sphincter.

3. Developmental and Biological Variances: All nephrons are present at birth. Kidneys and tubular system mature throughout childhood reaching full maturity during adolescence. During first two years of life kidney function is less efficient. Bladder: – Bladder capacity increases with age. – 20 to 50 ml at birth. – 700 ml in adulthood.

4. Urinary Output: – Urinary output per kilogram of body weight decreases as child ages because the kidneys become more efficient. Infants1-2 mL/kg/hr Children0.5 – 1 mL/kg/hr Adolescents40 – 80 mL/hr Developmental and Biological Variances:

5. Growth and Development: – Newborn = loss of the perfect child. – Toddler = toilet training. – Pre-school = curiosity. – School age = embarrassment. – Adolescent = body image / sexual function. Developmental and Biological Variances:

6. Focused Health History Single umbilical artery. Chromosomal abnormality. Congenital anomalies. Ear tags. Toilet training history. Family history. Growth patterns.

7. Toilet Training Readiness: 12 months no control over bladder. 18 to 24 months some children show signs of readiness. Some children may not be ready until around 30 months.

8. Diagnostic Tests: Urinalysis: – Protein. – Leukocytes. – Red blood cells. – Casts. – Specific Gravity. – Urine Culture for bacteria

9. Urine Specific Gravity: – Normal value = 1.010. – Increased Urine SG: Dehydration – diarrhea – excessive sweating – vomiting. – Decreased Urine SG: Excessive fluid intake – pyelonephritis – nephritis Diagnostic Tests:

10. Laboratory Values: – CBC with WBC count. – Hemoglobin / hematocrit. – Clotting studies. – Urea or BUN: Urea is normally freely filtered through the renal glomeruli, with a small amount reabsorbed in the tubules and the remainder excreted in the urine. Decrease or increase in the value does not tell the cause: pre-renal, post-renal or renal. Elevated BUN just tells you the urea is not being excreted by the kidney not why. Diagnostic Tests:

11. Laboratory Values: – Creatinine: Creatinine is a very specific indicator of renal function. If kidney function is decreased / creatinine level with be increased. Conditions that will increase levels: glomerulonephritis, pyelonephritis or urinary blockage. – Cholesterol. – Erythrocyte sedimentation rate (ESR). – C-Reactive protein (CRP). Diagnostic Tests:

12. Ultrasound. VCUG – Voiding cysto urethrogram. IVP – Intravenous pyelogram. Cystoscopy. CT Scan. Renal Biopsy Diagnostic Tests:

16. Treatment Modalities: Urinary diversion: – Stents. – Drainage tubes. Intermittent catheterization: – Watch for latex allergies. Pharmacological management: – Antibiotics. – Anticholinergic for bladder spasm.

17. Peritoneal Dialysis: – The child’s own peritoneal cavity acts as the semi- permeable membrane across which water and solutes diffuse. Treatment Modalities:

18. Peritoneal Dialysis: – Often initiated in the ICU. – Dialysis set-ups are available commercially. – Soft catheter is used to fill the abdomen with a dialysis solution. – The solution contains dextrose that pulls waste and extra fluid into the abdominal cavity. – Dialysis fluid is then drained. Treatment Modalities:

19. Peritoneal Dialysis: – Dialysis fluid: High glucose concentrate: 2.5 to 4.25%. The osmotic pressure of the glucose in solution draws the fluid from the vascular spaces into the peritoneum, making available for exchange and elimination of excess fluid. Treatment Modalities:

20. Peritoneal Dialysis: – Complications of Peritoneal Dialysis: Peritonitis Pain during infusion of fluids Leakage around the catheter Respiratory symptoms: – Abdominal fullness from too much fluids – Leakage of fluid to chest from hole in diaphragm Treatment Modalities:

21. Hemodialysis: – Used in treatment of advanced and permanent kidney failure. – Blood flows through a special filter that removes waste and extra fluids. – The clean blood is then returned to the body. – Done 3 times a week for 3 to 5 hours. Treatment Modalities:

22. Hematuria: Hematuria means blood in urine, it is caused by several conditions. In children, hematuria is usually caused by urinary tract infections, usually cystitis. The congenital conditions associated with hematuria are hypercalcinuria and hereditary nephritis. The acquired conditions are poststreptoccocal glomerulonephritis, urinary calculi, systemic conditions, hemolytic uremia syndrome…etc. The appropriate treatment of hematuria is based on the treatment of the underlying cause.

23. Proteinuria: Proteinuria is the presence of protein in the urine. It can be persistent or intermittent. Proteinuria can be a symptom of a disease or a self- limiting condition. The persistent proteinuria may be secondary to nephritic syndrome, glomerulonephritis, or acute or chronic renal failure.

24. The most common type of protreinuria in children is orthostatic proteinuria. The child with this condition excrete an abnormal amount of protein in urine when upright, but a normal amount when lying down. Other forms of proteinuria are exercise-induced proteinuria, which is seen after the child has exercised vigorously, and febrile proteinuria which accompanies the disease anytime the child has an elevated temperature because of a viral or infectious process. Proteinuria:

25. Urinary Tract Infection: Most common serious bacterial infection in infants and children. Highest frequency in infancy. Uncircumcised males have a ten-fold incidence. E-coli most common organism 85%

26. Etiology: – Anatomic abnormalities. – Neurogenic bladder – incomplete emptying of bladder. – In the older child: infrequent voiding and incomplete emptying of bladder or constipation. – Most common in females - short urethra. Urinary Tract Infection:

27. Assessment: – Neonate: jaundice, fever, failure to thrive, feeding, vomiting. – Infant: irritability, poor feeding, vomiting, diarrhea, strong odor to urine. – Childhood: vomiting, diarrhea, abdominal or flank pain, fever, enuresis, urgency, frequency, strong odor to urine. Urinary Tract Infection:

28. Diagnosis: – Urinary Tract Infection: Pyuria – white blood cells in urine. Culture of urine – grows out bacteria. – Urosepsis: Blood culture and urine culture grow out the same organism. – Pyelonephritis: Elevated white blood cell count. Elevated C-reactive protein and erythrocyte sedimentation rate. Urinary Tract Infection:

29. Management and Nursing Interventions: – Antibiotic therapy for 7 to 10 days: Amoxicillin or Cefazol or Bactrim or Septra. – Increase fluid intake. – Frequent voiding. – Acetaminophen for pain. Urinary Tract Infection:

30. Management and Nursing Interventions: – The main nursing intervention is teaching parents how to prevent recurrent infections. Teaching should include: Compliance with medication. The importance of fluid to the child. Teaching the child and parents about appropriate hygiene. Follow-up cultures are important in order to determine if the infection has been eradicated. Urinary Tract Infection:

31. Acute Glomerulonephritis: An acute, specific, self limited disease affecting glomeruli as an auto-immune response to infections caused by nephritogenic strains of group-A, beta- hemolytic streptococci. Symptoms appear 1 to 2 weeks after a Strep A skin or throat infection.

32. Clinical Manifestations: – Hematuria: It is usually the first feature. – Facial Edema: Usually mild pitting edema. Puffy eye-lid to peri-orbital edema, is especially marked early at morning. – Oliguria: Urine volume less than 180ml per day. In severe cases anuria may be present. Acute Glomerulonephritis:

33. Clinical Manifestations: – Hypertension: A diastolic pressure >90mm Hg (for child younger than 10 years), or >100mm Hg (for child over 10 years old. This leads to headache, vomiting and blurring of vision. – Mild proteinuria. Prognosis: – 90% complete recovery. – 10% progress to chronic nephritis, nephritic syndrome, or death in acute stage. Acute Glomerulonephritis:

34. Treatment and nursing care: – Management is supportive and early treatment is necessary to avoid complications and include: Anti-hypertensive drugs. Diuretics. Antibiotics if + throat culture or blood culture. 24 hour urine for Creatinine clearance. Bed rest is recommended in the acute phase. Low sodium / high protein. Acute Glomerulonephritis:

35. Treatment and nursing care: – The major nursing interventions are: The assessment of the progression of the disease: – Regular monitoring of vital signs is essential since the blood pressure is the measurement that determines the progression of the disease. The alertness to prevent complications. Supportive care for the child and the family, especially during the acute phase. Acute Glomerulonephritis:

36. Treatment and nursing care: – The major nursing interventions are: Teaching: – Culture sore throats. – Take antibiotics for full course prescribed. – Do not share medications with others in family. Acute Glomerulonephritis:

37. Nephrotic Syndrome / nephrosis: Nephrotic syndrome is a nonspecific disorder in which the kidneys are damaged, causing them to leak large amounts of protein. Nephrotic syndrome is not a disease itself, but the manifestation of many different glomerular diseases. These diseases might be: – Acute and transient, such as postinfectious glomerulonephritis. – Chronic and progressive, such as focal segmental glomerulosclerosis (FSGS). – Relapsing and remitting, such as minimal change nephrotic syndrome (MCNS).

38. Etiology: – Primary nephrotic syndrome (PNS): Also known as idiopathic nephrotic syndrome (INS). It is associated with glomerular diseases intrinsic to the kidney and not related to systemic causes. It accounts about 90% of cases of nephritic syndrome during childhood. Onset age: most commonly between 2-7 years, more in males. Good response to corticosteroids (prednisolone). Nephrotic Syndrome / nephrosis:

39. Etiology: – Secondary nephrotic syndrome: Refers to an etiology extrinsic to the kidney Infections: – Congenital syphilis, toxoplasmosis, cytomegalovirus, rubella, hepatitis B and C, HIV/acquired immunodeficiency syndrome (AIDS), Malaria.syphilistoxoplasmosiscytomegalovirusrubellaMalaria Drugs: – Penicillamine, gold, nonsteroidal anti-inflammatory drugs (NSAIDs), interferon, mercury, heroin, pamidronate, lithium Nephrotic Syndrome / nephrosis:

40. Etiology: – Secondary nephrotic syndrome: Systemic disease: – Systemic lupus erythematosus, malignancy - Lymphoma, leukemia, vasculitis -Wegener granulomatosis, Churg-Strauss syndrome, polyarteritis nodosa, microscopic polyangiitis, Henoch-Schönlein purpura (HSP).polyarteritis nodosa – Congenital Nephrotic Syndrome. Nephrotic Syndrome / nephrosis:

41. Assessment: – Generalized edema. – Edema is worse in scrotum and abdomen (results in ascites). – Dramatic weight gain. – Pale, fatigue, anorexic. – Urinary output decreased. – Urine foamy and frothy with elevated SG. Nephrotic Syndrome / nephrosis:

42. Diagnostic evaluation: – Proteinuria: 4+ urine in urine. – Hypoalbuminemia. – Hypercholesterolemia: Fat cells in blood. – BUN and Creatinine normal unless renal damage Nephrotic Syndrome / nephrosis:

43. Interventions: – Diuretics (during acute phase lasix would be given after IV albumin). – Fluid restriction if edema severe. – Low sodium / high protein diet. – Daily weights. – Strict intake and output. Nephrotic Syndrome / nephrosis:

44. Interventions: – Corticosteroid Therapy: Is started as soon as the diagnosis has been confirmed. High dose prednisone. Taper when protein loss in urine decreases. Current recommendations to keep on low dose every other day for up to 6 months. If relapse or remission not obtained will try cytotoxic medications. Nephrotic Syndrome / nephrosis:

45. Interventions: – Corticosteroid Therapy: Side Effects of Cortisone Therapy: – Hirsutism. – Moon face with ruddy cheeks. – Acne. – Dorsocervical fat pads. – Ecchymosis (easy bruising). – Truncal obesity. – Mood swings – inability to sleep. – Increase appetite. Nephrotic Syndrome / nephrosis:

46. Interventions: – Corticosteroid Therapy: Nursing Interventions for long tern use: – Prednisone prescribed every other day. – Instruct to take in the morning. – Take with food: can cause GI upset. – Do not stop taking medication until instructed to do so. – Medication needs to be tapered. – Monitor for infection Nephrotic Syndrome / nephrosis:

47. Prognosis: – The overall mortality of INS has decreased dramatically from over 50% to approximately 2-5%. – Despite the improvement in survival, INS is usually a chronic, relapsing disease (relapses in >60% of patients) and most patients experience some degree of morbidity. – There is a potential for progression to chronic kidney failure and end-stage kidney failure (ESKD). Nephrotic Syndrome / nephrosis:

48. Acute Renal Failure (ARF): Is an abrupt decline in the renal regulation of water, electrolytes, and acid-base balance, which result in the retention of nitrogenous waste. It could be: – Pre-renal, resulting from impaired blood flow to or oxygenation of the kidneys. – Renal, resulting from injury to or malformation of kidney tissues. – Post-renal, resulting from obstruction of urinary flow between the kidney and urinary meatus.

49. Etilogy: – Newborn causes: Congenital anomalies. Hypotension. Complication of open heart surgery. – Childhood causes: Dehydration. Glomerular nephritis / Nephrotic Syndrome. Nephro-toxicity / drug toxicity. Acute Renal Failure (ARF):

50. Assessment: ARF – Sudden onset. – Oliguria: Urine output less than 0.5 to 1 mL/kg/hour. – Volume overload due to retained fluid: Hypertension, edema, shortness of breath. – Acidosis. – Electrolyte imbalance and dehydration. Acute Renal Failure (ARF):

51. Diagnostic Tests: – Decrease RBC due to erythropoietin. – Urea and Creatinine elevated. – GFR (glomerular filtration rate) most sensitive indicator of glomerular function. Goals of Treatment: Acute Renal Failure – Reduce symptoms. – Supportive care until renal function returns. – Medications – corticosteroids. – Dietary restrictions – sodium. – Dialysis if indicated. Acute Renal Failure (ARF):

52. Enuresis: Involuntary discharge of urine after the age by which bladder control should have been established, usually considered to be age of 5 years. – Nocturnal = at night. – Diurnal = during the day. – Secondary = relapse after some control. Familial history: – Males outnumber females 3:2. – 5 to 10% will remain enuretic throughout their lives. – Rule out UTI, ADH insufficiency, or food allergies.

53. Pharmacologic Interventions: – Desmopressin synthetic vasopressin acts by reducing urine production and increasing water retention and concentration. – Tofranil: anticholinrgic effect – FDA approval for treatment of enuresis. Side effect may be dry mouth and constipation. Some CNS: anxiety or confusion. Need to be weaned off. Enuresis:

54. Interventions: – Diet control: Reduce fluids in evening. Control sugar intake. – Bladder training: Praise and reward. Behavioral chart to keep track of dry nights. Alarm system Enuresis:

55. Ureteral Reflux: Males 6 to 1. Genetic predisposition. Present as UTI or FTT. Diagnostic tests. Antibiotics if indicated. Surgery to re-implant ureters.

56. Hydronephrosis: Water on kidney. Due to obstruction. Congenital anomaly. Goals of care to maintain integrity of kidney until normal urinary flow can be established.

57. Hypospadias: Most common anomaly of the male phallus. Incomplete formation of the anterior urethral segment. The opening of the urethral meatus is at any site on the ventral surface of the penis. Cordee – downward curve of penis.

58. Management: – Newborn: Circumcision not recommended. Foreskin may be needed for reconstructive surgery. – Surgical Interventions, Repair: Release of tight chordee. Placement of urethra opening at head of penis. Surgery recommended at around six to nine months of age. Hypospadias:

59. Management: Hypospadias:

60. – Long term outcomes: Leaking at the site. Body image. Hypospadias:

61. Hydrocele: Hydrocele is a scrotal swelling due to accumulation of serous fluid in the scrotal sac. Unlike an inguinal hernia, a hydrocele generally is not painful and does not have noticeable symptoms. (An inguinal hernia is tender and causes intestinal symptoms.) The hydrocele may close spontaneously in the first year of life. For this reason, operation is usually delayed unless the swelling causes discomfort.

62. Types of hydroceles: – Communicating Hydrocele: This is a hydrocele that has contact (or communication) with the fluids of the abdominal cavity. Hydrocele:

63. Types of hydroceles: – Non-communicating Hydrocele: This condition might be present at birth or might develop years later for no obvious reason. A non-communicating hydrocele usually remains the same size or has a very slow growth. Hydrocele:

64. Treatment: – A non-communicating hydrocele usually does not need to be surgically repaired, since it usually goes away spontaneously within six to 12 months. – A communicating hydrocele needs to be surgically repaired to prevent further complications. – Aspiration of fluid is useless. Hydrocele:

65. Epispadius: The opening of the urethral meatus is at any site on dorsal surface of the penis.

66. Undescended Testes (Cryptorchidism): A condition seen in newborns when one or both of the male testes have not passed down into the scrotal sac. Thirty percent of cases are bilateral (involve both testes). More commonly seen in premature males because the testes do not descend from the abdomen to the scrotal sac until the seventh month of fetal development. Occurs in 3 percent to 4 percent of full-term infants.

67. Causes: – P rematurity, twenty-one percent of premature male infants have an undescended testis. – Hormonal disorders. – Spina bifida. – R etractile testes (a reflex that causes a testicle to move back and forth from the scrotum to the groin). – T esticular absence. Undescended Testes (Cryptorchidism):

68. Symptoms: – A non-palpable testis (unable to feel upon examination) is the most common symptom of cryptorchidism. Treatment: – Undescended testes usually resolves without any intervention by the time that the infant is 6 months old. – Hormonal therapy. Undescended Testes (Cryptorchidism):

69. Treatment: – Surgical intervention: The recommended treatment. The surgery, called orchiopexy, is usually performed between months 6 and 18, and is successful in 98 percent of children with this condition. Undescended Testes (Cryptorchidism):

70. Complications: (If not treated) – Infertility (most common in bilateral cases) – Risk of testicular cancer increases considerably by age 30 or 40. – Inguinal hernia. – Testicular torsion (a painful twisting of the testes that can decrease blood supply to the testes). – Psychological consequences of an empty scrotum. Undescended Testes (Cryptorchidism):

71. Testicular Torsion: Test torsion is an acute, painful condition in which the spermatic cord is twisted just above the testicle, blood flow is compromised to the testicle, and the child exhibits muscle spasms. Left testicle affected more; Longer cord on left side. Torsion occurs at any age but is most prevalent in adolescence, with a peak at about 14 years of age.

72. The diagnosis is made by using Doppler ultrasound. The condition is treated as an emergency in order to avoid testicular atrophy and loss of the testis. Ideally, surgery should be done within 6 hours after the torsion has occurred. Testicular Torsion:

73. Wilms tumor: Wilms' tumor or nephroblastoma is cancer of the kidneys that typically occurs in children, rarely in adults. It is a malignant tumor. Its common name is an eponym, referring to Dr. Max Wilms, the German surgeon (1867–1918) who first described this kind of tumor It is the most common childhood abdominal malignancy.

74. In 5-10% of patients, both kidneys are affected at the same time or one after the other. The majority (75%) occur in otherwise normal children; a minority (25%) is associated with other developmental abnormalities. The median age at diagnosis is approximately 3.5 years. Wilms tumor:

75. Signs and symptoms: – Asymptomatic palpable abdominal mass; occurs in 80% of children at presentation. – Abdominal pain or hematuria occurs in 25%. – Urinary tract infection and varicocele. – Hypertension. – Fever. Wilms tumor:

76. Diagnosis: – Laboratory Studies: Include: CBC count, chemistry profile (including kidney function tests and routine measurements of electrolytes and calcium), urinalysis, coagulation studies, and cytogenetics studies. – Imaging Studies: Include: renal ultrasonography, CT scanning, MRI scanning, and four-field chest radiography (images may depict lung metastases). Wilms tumor:

77. Treatment: – The usual approach in most patients is nephrectomy followed by chemotherapy with or without postoperative radiotherapy. – Diet: No special diet is recommended. – Activity: the patient has only one kidney after therapy. Activities that carry an inherent risk of kidney injury, such as boxing and hockey, should be avoided. Wilms tumor:

78. Complications: – Nephrectomy leaves the child with one functional kidney. In almost all patients, the remaining kidney can compensate and maintain adequate renal function. – Specific complications are as follows: Impaired renal function. Impaired cardiac function. Wilms tumor:

79. Complications: – Specific complications are as follows: Impaired pulmonary function. Impaired hepatic function. Impaired musculoskeletal function. Second malignant neoplasms. – Relapses: The lungs are the most common site of relapse. Wilms tumor:

80. Prognosis: – Survival rates of children with this neoplasm are approximately 85-90% surviving at least five years. – The prognosis for patients who have a relapse is not as good as newly diagnosed Wilms tumor, with 40-80% expected to survive after salvage therapy. Wilms tumor: