Genitourinary disorders

Introduction
• The urinary tract includes the organ system primarily
responsible for cleaning and filtering excess fluid and
waste material from the blood.
• Kidneys, Ureters, Bladder, Urethra
• In children, problems of the urinary system include
acute and chronic kidney failure, urinary tract
infections, obstructions along the urinary tract, and
abnormalities present at birth
 Nephrotic Syndrome

• Nephrotic syndrome is a clinical state characterized by

proteinurea, hypoalbuminemia, hyperlipedemia, edema .

 Incidence
• Occurs at all ages but is most prevalent in
children between the ages 1.5-6 years
• It affects more boys than girls, 2:1 ratio
• Most studies put the incidence at 2-7 per
100,000 population
 Etiology
• This disorder can occur as,
– Primary Nephrotic syndrome
– Secondary Nephrotic syndrome
– Congenital Nephrotic syndrome
 Etiology
• Primary Nephrotic syndrome
– Primary disease known as idiopathic nephrosis,
childhood nephrosis or minimal change nephrotic
syndrome (MCNS)
– 90% belong to this catogory
 Etiology
• Secondry nephrotic syndrome
– When the nephrotic syndrome occurs as a part of a
organized systemic disease or result from some evident
causes,
• SLE (Systemic lupus erythematosus)
• Infection
– HIV
– Hepatitis B and C
– Malaria
– Syphilis
• Obesity – generally lower proteinuria and less edema
• Drug exposure
– NSAID (nonsteroidal anti-inflammatory drugs)
 Etiology
• Congenital nephrotic syndrome
– It is inherited as an autosomal recessive disorder
– This disorder is characterized by increased
glomerular permeability to plasma protein which
result in massive urinary protein loss.
 Types of nephrotic syndrome
1. Minimal change disease.
• Affects between 85 and 95 percent of children
• Usually responds to steroids
• Tends to relapse less and less frequently as the
child grows older
• Usually doesn’t affect kidney function
• Rarely results in a child needing dialysis or
kidney transplant
 Etiology
2. Focal segmental glomerulosclerosis (FSGS)
• Characterized by scattered scarring of some of the
glomeruli, which may result from another disease or a
genetic defect or occur for no known reason.
• More aggressive form of nephrotic syndrome
• usually doesn’t respond to steroid therapy
• may require multiple biopsies to diagnose
• often result in the child needing kidney dialysis and/or
kidney transplant
 Pathophysiology
Renal glomerular
damage

proteinuria

Increased hepatic
synthesis of
hypoproteinemia
protein and lipids

hypovolemia Decreased
oncotic
hyperlipedemia
Decresed Increased pressure
renal blood secretion of
flow ADH &
aldosterone

Renin Na+ & H2O

release Reabsorption edema
Increased
vasoconst hydrostatic
ruction pressure
 Clinical manifestation
• Weight gain
 Clinical manifestation
• Puffiness of face
– Especially around the eyes
– Arising in the morning
– Subside during the day
 Clinical manifestation
• Abnormal swelling (ascites)
 Clinical manifestation
• Labial or scrotal swelling
• Ankle or leg swelling
 Clinical manifestation
• Edema of intestinal mucosa possibly causing,
– Diarrhea
– Anorexia
– Poor intestinal absorption
 Clinical manifestation
• Blood pressure normal or slightly decreased
• Urine alterations
– Decreased volume
– Frothy
 Diagnostic evaluation

• History & Clinical manifestations

• Urine analysis
– Proteinurea (higher than 3+ on urine dipstick)
– Hyaline casts, oval fat bodies and few RBC
– Hematuria
• GFR is usually normal or high
 Diagnostic evaluation
• Total serum protein concentration is low with
serum albumin reduced and plasma lipids
elevated.
• Platelets count may be elevated
• Hb & hematocrit may be normal or elevated
• Renal biopsy may be need to distingush
between the other types of nephrotic
syndrome.
 Therapeutic management
• Corticosteroid are the first line therapy for
MNCS.
– Prednisone 2mg/kg/day in one or more divided
doses for 4-6 weeks.
 Consider steroid-sparing agents
Cyclophosphamide
• Recommended dose 2 mg/kg for 8-12 wks •
• More likely to be effective in frequent relapsing
than in steroid-dependent NS
 Consider steroid-sparing agents
Levamisole
• Anti-helminthic agent
• Immuno-modulator – mechanism unknown
• More anecdotal beneficial effect than in well-
designed studies
• Dose – 2-2.5 mg/kg every second day
 Consider steroid-sparing agents
Calcineurin inhibitors - Cyclosporine
• Blocks T-cell activation
• Induces and/or maintains remission in frequent-
relapsing and in steroid-dependent NS
• Prolonged treatment is mandatory as high rate
of relapse following discontinuation of CsA
• Monitor blood levels (trough 100-200ng/mL)
 Consider steroid-sparing agents
• Mycophenolate mofetil (MMF)
• Inhibits T- and B-cell proliferation
• Small studies show beneficial effects
• Less toxicity compared to calcineurin inhibitors
 Therapeutic management
• Antibiotic may be used to treat infections
• Diuretics are used to relieve edema
• Abdominal paracentesis is done to releave
pressure which causes breath lessens
 Nursing management
• Bed rest is required during the stages of
edema
• Comfortable poisoning
• Care of edema skin
 Nursing management
• Diet should be low sodium and high protein
• Nutritional needs should be met.
 Nursing management
• Fluid intake requirement is calculated
according to the output and weight of the
child
 Nursing management
• Monitor vital signs and observation of early
signs of infection
 Nursing management
• Support of the child and family.
ACUTE GLOMERULONEPHRITIS
 Introduction

• The glomeruli are structures in kidneys made up

of tiny blood vessels.
• These knots of vessels help filter blood and
remove excess fluid.
• If glomeruli are damaged, kidneys will stop
longer work properly, and body can go into
kidney failure.
 Definition
• Glomerulonephritis is a group of diseases that
injure the part of the kidney that filters
blood (called glomeruli).
• The common features include oliguria, edema,
hypertension, hematuria and proteinuria.
 Incidence
• Acute post streptococcal glomerulonephrits
(APSGN) is the most common of the post
infectious renal disease in children.
• APSGN occurs at any age but affects primarily
early school children (6-7years)
• It is uncommon in children younger than two
years
• Males outnumber females 2:1
 Etiology
• APSGN is an immune complex disease it
occurs after streptococcal infection (group A
beta hemolytic streptococcus) .
• Secondary to Streptococcal pharyngitis.
• systemic lupus erythematosus (SLE or lupus)
 Pathophysiology
• The pathophysiology of APSGN is still uncertain.

Immune complexes are deposited in the glomerular basement membrane

Glomeruli became edematous and infiltrated with polymorphonuclear

leukocytes which occlude capillary lumen

Decrease plasma filtration

Excessive accumulation of water and retention of sodium

Circulatory congestion and edema

 Clinical manifestations
• Edema
– Especially preriorbilal
– Facial edema (in morning) and spreads during the
day to involve extremities and abdomen
 Clinical manifestations
• anorexia
 Clinical manifestations
• Urine;
– Cloudy, smoky brown (Tea or cola)
– Severely reduced volume (urine output is < 400
ml/day)
 Clinical manifestations
• Pallor, irritability and lethargy
• Child appearing ill and seldom express specific
complaints.
 Clinical manifestations
• Older children complaints of ,
– Headaches
– Abdominal discomforts
– dysuria
 Clinical manifestations
• Mild to moderately elevated BP
 Diagnostic evaluation
• Urine examination
– Hematuria
– High specific gravity
– Presence of WBC & epithelial cell
 Diagnostic evaluation
• Blood examination
– Urea nitrogen and creatinine is elevated
– ESR increased
 Diagnostic evaluation
• Anti streptolysine O titer ( ASO titer)
• Renal biopsy if child is not responding to the
treatment
 Diagnostic evaluation
• Chest X ray
– Cardiac enlargement
– Pulmonary congestion
– Pleural effusion
 Therapeutic management
• Management consist of general supportive
measure and early recognition and treatment
of complications.
• Child with normal BP and satisfactory urine
out put can be treated at home.
• Child with edema, hypertension, hematuria,
oliguria should be hospitalized
 Therapeutic management
• The patients with more than 30 percent
crescents on renal biopsy are often treated
with methylprednisolone pulses.
• Supportive treatment
– Antibiotics – long acting penicillin may be given to
treated infection
– anti-hypertension medications and diuretics are
used to control hypertension.
 Therapeutic management
• Dietary restriction of sodium & fluid
• Substantial amount of potassium are generally
restricted during the period of oliguria.
 Nursing management
• Bed rest and restrict the activities
 Nursing management
• Regular measurement of vital signs, body
weight, intake and out put is essential for
monitoring the progress of desease
 Nursing management
• Small and frequent diets and fluids should be
supplied according to prescription
 Wilms
tumour
 Introduction
• It is the most common child hood malignancy
over the past 5 decades
• Multidisciplinary approach to this tumor has
became an example for successful treatment
of cancer
• Discovered by max wilms in 1899
 Definition
• Wilms' tumor is a kidney cancer that primarily
affects children.
• Wilms' tumor most often occurs in just one
kidney, though it can sometimes be found in
both kidneys at the same time.
 Incidence
• Most common in children less than 15 years
old.
• Peek in 2-4 years of age.
• It occurs three times more in Africans and
Americans than in east Asian
• It occurs more in boys & girls equally
• 1-2.5% have familial origin
 Etiology
• Wilms tumor is thought to be caused by
alterations of genes responsible for normal
genitourinary development.
• Examples of common congenital anomalies
associated with Wilms tumor are
cryptorchidism, a double collecting system,
horseshoe kidney, and hypospadias.
• Environmental exposures
 Etiology
• WT1 gene: a genetic model for the development
of Wilms tumor.
• WT1, the first Wilms tumor suppressor gene at
chromosomal band 11p13.
• Additional genetic loci: A second gene that
predisposes individuals to develop the Wilms
tumor has been identified (but has not yet been
cloned) telomeric of WT1, at 11p15
• WAGR syndrome
• denys-drash syndrome
• beckwith-wiedemann syndrome
 Pathophysiology
• It develop in the renal parenchyma in either
the central or polar location.
• The entire renal parenchyma appear replaced
by tumor with only rim of compressed renal
tissue remaining
• The majority of tumors present as a single
mass surrounded by a pseudo capsule of
connective tissue that appears to separate
kidney and tumor.
 Pathophysiology
• The lesion are often vascular, soft, gelatinous,
and necrotic in the centre.
• The lesion are composed of mesenchymal and
epithelial elements in various ratios and
various stages of maturity.
• The mesenchymal elements may show
differentiation in to striated muscle, cartilage,
and bone
Pathophysiology
 Stages
• Stage I. The cancer is found only in one kidney,
and generally can be completely removed
with surgery.
• .
 Stages
• Stage II. The cancer has spread to the tissues
and structures near the affected kidney, such
as fat or blood vessels, but it can still be
completely removed by surgery.
 Stages
• Stage III. The cancer has spread beyond the
kidney area to nearby lymph nodes or other
structures within the abdomen, and it may not
be completely removed by surgery.
 Stages
• Stage IV. The cancer has spread to distant
structures, such as the lungs, liver, bones or
brain.
• Stage V. Cancer cells are found in both kidneys
 Clinical features
• Abdominal mass or enlarging abdomen
• Urethral obstruction signs
• Less common hypertension, hematuria, and
anorexia.
• Fever
 presentation
• most patients have a complaint
– abdominal mass (~ 80%)
– abdominal pain (~ 40%)
– fever (~ 20%)
– hematuria (~ 20%)
• congenital anomalies
– ~ 10% cases syndromic
 Diagnosis
• A physical examination. look for possible
signs of Wilms' tumor.
• Blood and urine tests. Blood tests can't detect
Wilms' tumor, but they can provide child's an
overall assessment of health.
• Bone marrow aspiration may be performed
 Diagnosis
• Imaging tests. Imaging tests that create
pictures of your child's kidneys helps to
determine whether the child has a kidney
tumor.
• Imaging tests may include ultrasound,
computerized tomography (CT) and magnetic
resonance imaging (MRI).
 Therapeutic management
• Treatment will depend on a number of factors
including how the cells appear under the
microscope (histology) and the stage of the
tumor.
• Treatment may include chemotherapy,
radiotherapy or surgery.
 Surgical management
• Surgery may be scheduled as soon possible
after conformation of renal mass, usually with
in 24 to 48 hours.
• The tumor , affected kidney and adjacent
adrenal gland are removed.
• Care should be taken care for preventing
rupture of encapsulated tumor intact.
• lymph node sampling
 Nursing management
• Pre operative care:
– Prepare the child and parent for all laboratory and
operative procedures.
– Pre operative observations
– Do not palpate the tumor unless absolute
necessary
– Information about radiation and chemotherapy
immediately after surgery
 Nursing management
• Post operative care:
– Recovery is usually rapid
– Care full monitoring of GI activities
– Post operative monitoring
– Family support
Congenital defects
 Hypospadiasis
• It is a condition when the site of urethral
meatus is on the penile ventral surface,
Instead of opening at the tip of the glans of
the penis.
 Causes
• The exact reason this defect occurs is unknown.
• Sometimes hypospadias is inherited
• may be an increased risk for In vitro fertilization
(IVF) to conceive .
 Pathophysiology
• As the penis develops in a male fetus, certain
hormones stimulate the formation of the
urethra and foreskin.
• Hypospadias results when a malfunction occurs
in the action of these hormones, causing the
urethra to develop abnormally.
 Clinical manifestation
• The appearance of the penis is altered.
• Inability of the boy to stand and urinate, he
must sit and void.
 Therapeutic management
• Treatment involves surgery to reposition the
urethral opening and, if necessary, straighten
the shaft of the penis.
• Using tissue grafts from the foreskin or from
the inside of the mouth to reconstruct the
urinary channel in the proper position,
correcting the hypospadias
• Surgery is best done at an early age — usually
between ages 4 months and 12 months.
Surgical repair
 Epispadias
• An epispadias is a rare type of malformation
of the penis in which the urethra ends in an
opening on the upper aspect (the dorsum) of
the penis.
•
 Epispadias
• It can also develop in females when the
urethra develops too far anteriorly
 Phimosis
• It is condition in which the fore skin is narrowed
and cannot be retracted over the glans
• Physiologic phimosis occurs naturally in
newborn males.
• Pathologic phimosis defines an inability to
retract the foreskin after it was previously
retractable or after puberty, usually secondary
to distal scarring of the foreskin.
Phimosis
 Pathophysiology
• Physiologic phimosis results from adhesions
between the epithelial layers of the inner
prepuce and glans.
• These adhesions spontaneously dissolve with
intermittent foreskin retraction and erections,
so that as males grow, physiologic phimosis
resolves with age.
Physiologic phimosis
 Pathophysiology
• Poor hygiene and recurrent episodes of
balanitis lead to scarring of preputial orifices,
leading to pathologic phimosis.
• Forceful retraction of the foreskin leads to
microtears at the preputial orifice that also
leads to scarring and phimosis.
 Pathophysiology
• Patients with phimosis, both physiologic and
pathologic, are at risk for developing
paraphimosis.
• When the foreskin is forcibly retracted past
the glans and/or the patient or caretaker
forgets to replace the foreskin after retraction.
• Penile piercings increase the risk of developing
paraphimosis if pain and swelling prevent
reduction of a retracted foreskin.
 Paraphimosis
• Paraphimosis is the entrapment of a retracted
foreskin behind the coronal sulcus.
• Paraphimosis is a disease of uncircumcised or
partially circumcised males.
Paraphimosis
 Clinical manifestation
• In Pathologic phimosis may be detected in
males who report painful erections,
• hematuria,
• recurrent urinary tract infections,
• preputial pain, or a weakened urinary stream.
 Clinical manifestation
• Paraphimosis classically presents with a
painful, swollen glans penis in the
uncircumcised or partially circumcised patient.
• A preverbal infant may present only with
irritability.
 Theraputic management
• Mild cases manual retraction of foreskin and
proper cleaning of area.
• Severe cases circumcision or vertical division
and transverse suturing of foreskin.
 Theraputic management
• Recommend 0.1 -0.05% betamethasone
dipropionate applied to the preputial orifice
twice a day for 4-6 weeks.
• Betamethasone valerate 0.1%, 0.2%, and 0.2%
in combination with hyaluronidase has also
been shown to be effective.
 Undecended testis (cryptorchidism)
• Undescended testicle (cryptorchidism) is a
testicle that hasn't moved into its proper
position in the bag of skin hanging below the
penis (scrotum) before birth.
 Causes
• The exact cause of an undescended testicle
isn't known.
• A combination of genetics, maternal health
and other environmental factors might disrupt
the hormones, physical changes and nerve
activity that influence the development of the
testicles.
 Risk factors
Factors that might increase the risk of undescended
testicle in a newborn include:
• Low birth weight
• Premature birth
• Family history of undescended testicle
• Alcohol use by the mother during pregnancy
• Cigarette smoking by the mother or exposure to
secondhand smoke
• Obesity in the mother
• Parents' exposure to some pesticides
 Pathophysiology
• Testicles form in the abdomen during fetal
development.
• During the last couple of months of normal fetal
development, the testicles gradually descend
from the abdomen through a tube-like
passageway in the groin (inguinal canal) into the
scrotum.
• With an undescended testicle, that process stops
or is delayed.
 Pathophysiology
• Generally undescent testis are found in the
abdominal cavity near the pubic tubercle,
inguinal canal, and retroperitonial space
• Undescent testis are at the higher temperature
than the scrotal temperature there for the
spermforming cells are damaged .
• If both the testis are undescended sterility
occurs
 Clinical manifestation
• Not seeing or feeling a testicle where you
would expect it to be in the scrotum is the
main sign of an undescended testicle.
 Theraputic management

• An undescended testicle is usually corrected

with surgery.
• The surgeon carefully manipulates the testicle
into the scrotum and stitches it into place
(orchiopexy).
• This procedure can be done either with a
laparoscope or with open surgery.
• It is recommend doing the surgery after 3 to 6
months old and before he is 12 months old.
 Theraputic management
• Hormone treatment
Hormone treatment involves the injection of
human chorionic gonadotropin (HCG).
• This hormone could cause the testicle to move
to your son's scrotum.
• Hormone treatment is not usually recommended
because it is much less effective than surgery.
 Complications
• Testicular cancer.
• Fertility problems
• Testicular torsion
• Trauma.
• Inguinal hernia
Thank you