>Nephroti c Syndrome

>Wi l m s tumor
>Management
GENITOURINARY
PROBLEM
Ki dney Di sease i n Chi ldren - Chi ldhood Nephroti c Syndrome
Nephrotic Syndrome
Nephrotic syndrome i s a set of si gns or symptoms that may
poi nt to ki dney probl ems.
Nonspecific syndrome i s al tered gl omerular permeabil ity due
to damage of the gl omeruli causing abnormal l oss of protein.
Chi l dhood nephrotic syndrome can occur at any age but i s
most common between the ages of 1 and 5 years.
Di et i s l ow- sodium
Inci dence
The i nci dence of Nephrotic syndrome worl dwide:
Chi l dren: 2 per 100, 000 persons per year

NEPHROTIC SYNDROME
The incidence of Nephrotic syndrome worldwide:
Children: 2 per 100,000 persons per year


INCIDENCE
PATHOPHYSIOLOGY
For about 80% of chi l dren wi th nephroti c syndrome, the cause i s
unknown. Thei r ki dneys wi l l appear normal or nearl y normal after
tests are done. Doctors refer to thi s as "mi ni mal change
di sease". Mi ni mal change di sease resul ts i n abnormal ki dney
functi on, but when the ki dney ti ssue i s exami ned under a
mi croscope, i t appears normal or nearl y normal . The cause of the
abnormal functi on typi cal l y can' t be determi ned.
Wi th mi ni mal change di sease, there are
Very ti ny physi cal changes i n the ti ny fi l ters (cal l ed gl omerul i ) i n
the ki dneys.
Can usual l y be treated wi th a medi ci ne cal l ed predni sone, but
nephroti c syndrome can come back. Predni sone stops the
movement of protei n from the bl ood i nto the uri ne.
Most chi l dren wi l l grow out of mi ni mal change di sease i n thei r
teen years

Mani festati ons
The mai n symptoms of nephroti c syndrome are
Swel l i ng
Protei ns are needed i n the bl ood to hel p hol d water i n the bl ood
vessel s. Swel l i ng i s usual l y fi rst noti ced around the eyes and then
around the l ower l egs and rest of the body.
Infecti ons
Speci al i sed protei ns cal l ed anti bodi es pl ay an i mportant rol e i n
fi ghti ng i nfecti on. Poor growth and devel opment
Protei nuri a
Increase gl omerul ar permeabi l i ty l eads to protei n l oss i n the uri ne,
and subsequentl y
Hypoal bumi nemi a
Low serum al bumi n l evel
Hyperl i pi demi a
Occurs because the l i ver i ncreases producti on of l i poprotei ns to try
to compensate for protei n l oss.

MANIFESTATIONS
Nursing Diagnosis
According to Pilliteri A., Nursing diagnosis for
Nephrotic syndrome are:
Imbalanced nutrition, less than body requirements,
related to restricted diet, and protein loss
Risk for impaired skin integrity related to edema
Knowledge deficit related to chronic illness

NURSING DIAGNOSIS
Medi cal Management
Nephroti c Syndrome can be di agnosed through uri ne sampl e for
uri ne checki ng.
There i s no speci fi c treatment for nephroti c syndrome, but the
symptoms can be managed wi th medi cati on and changes to the
di et.
Steroi ds. Your chi l d wi l l normal l y be prescri bed the steroi d
medi ci ne predni sone when they are fi rst di agnosed.
Di ureti cs. Di ureti cs are not commonl y used to reduce the edema
because they tend to decrease bl ood vol ume, whi ch i s al ready
decreased. Chi l dren who respond poorl y to predni sol one al one,
however, may need di ureti c therapy wi th a drug such as
furosemi de. But there i s al ways a danger that too much
potassi um wi l l be excreted, causi ng hypokal emi a.
Peni ci l l i n. Peni ci l l i n may be prescri bed to prevent i nfecti on.
Di etary Changes. Your chi l d wi l l need to fol l ow a stri ct l ow- sal t
di et, to prevent further water retenti on and edema.
Ki dney transpl antati on may be necessary to sustai n l i fe
MEDICAL MANAGEMENT
Nursi ng Management
Consul t a di eti ti an or nutri ti onal team to i mpl ement
i nterdi sci pl i nary team management and hel ps determi ne
nutri ti onal needs
Use fl avouri ng agents (e. g. , l emon and herbs) i f sal t i s restri cted
to enhance food sati sfacti on and sti mul ate appeti te
Emphasi ze i mportance of wel l - bal anced, nutri ti ous i ntake.
Provi de i nformati on regardi ng i ndi vi dual nutri ti onal needs to
promote wel l ness
Encourage semi - Fowl er s posi ti on to reduce peri orbi tal edema
Identi fy i nformati on that needs to be remembered to faci l i tate
l earni ng
Provi de support to fami l y

NURSING MANAGEMENT
Al so known as nephroblastoma
Rare ki dney cancer that pri marily af fects chi l dren ages 3 to 4
Doctors bel ieve that the tumor begins to grow as a fetus
develops i n the womb, wi th some cel l s that are destined to
form i nto the ki dneys mal functi oning and formi ng a tumor
Some chi l dren wi th the di sorder have a del etion on
chromosome 11
The tumor was named af ter Dr. Max Wi l liams (1867- 1918), a
German surgeon who fi rst described i t.

WILMS TUMOR (NEPHROBLASTOMA)
Uni ted States: 10 chi l dren and adol escents per 1 mi l l ion
before age 15 years
Worl dwide: one i n every 8, 000

INCIDENCE
It s not cl ear what causes Wi l m s tumor but doctors bel ieve
that there s a DNA error

PATHOPHYSIOLOGY
Mani festations
Wi l m s tumor doesn t al ways cause si gns and symptoms.
Di scovers a fi rm, smooth
MANIFESTATIONS
Nursing Di agnosis
Anxi ety rel ated to threat to death

NURSING DIAGNOSIS
Observe behavior to poi nt the cl i ent s l evel of anxi ety
Establ ish therapeutic rel ati onshi p, conveying empathy and
uncondi tional posi tive regard; Be avai lable to cl i ent for
l i stening and tal king; Encourage cl i ent to acknowl edge and
express feel ing to i dentify feelings and begin to deal wi th
probl ems
Cl arify meaning of feel ings and acti ons by provi ding feedback
and checking meani ng wi th the cl i ent
Support and provi de care

NURSING MANAGEMENT
Wi l m s tumor i s di agnosed through:
Abdominal ul trasound scan, Abdominal CT scan
Chest X- ray
Bi opsy
Staging
Stage 1 Cancer is limited to the kidney. Can be completely
removed
Stage 2 Cancer reached nearby tissues, fats, and blood vessels
close to kidney. Can be completely removed
Stage 3 Cancer spread further and reached nearby lymph nodes or
other parts of the abdomen. Removing surgically may not be possible
Stage 4 Cancer spread further still to other parts of body, including
brain, liver.
Stage 5 both kidneys have cancer cells

MEDICAL MANAGEMENT
Treatment and Drugs
Hi stol ogy - Cancer cel l s observed under mi croscope
Favorable good chance of cure
Unfavorable more aggressive & difficult to cure
Nephrectomy surgical removal of ki dney ti ssue
Simple whole kidney is surgically removed. The other kidney is
enough to maintain the patient in good health.
Partial - the tumor and part of the kidney tissue that surrounds it is
surgically removed. This type of surgery is done if the other kidney is
not 100% healthy
Radical - the whole kidney, nearby adrenal gland and lymph nodes,
as well as other surrounding tissue are surgically removed.
Ki dney Transplant
Chemotherapy
Radi otherapy