Case Study Nephrotic Syndrome

OBJECTIVES General: This study aims to broaden the knowledge of the student – nurses as well as their superiors about the disease, to be able to respond and intervene with client's correctly, render accurate nursing care needed in order to provide an effective nursing management in a hospital set – up until the client is ready for discharge. Specific:

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To identify the major disease manifestations, risk factors and etiology.

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To understand the pathophysiology of Nephrotic Syndrome in relation to the anatomy and physiology.

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To assess a patient with Nephrotic Syndrome.

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To know the nature of the drugs administered with compliance to nursing responsibilities.

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To formulate a nursing care plan for Nephrotic Syndrome

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To implement effective nursing intervention.

Introduction Definition • • •

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Nephrotic syndrome is a group of symptoms including protein in the urine (more than 3.5 grams per day), low blood protein levels, high cholesterol levels, high triglyceride levels, and swelling Autoimmune process leading to structural alteration of glomerular membrane that results in increased permeability to plasma proteins, particulary albumin. Nephrotic syndrome is a disorder of the glomeruli (clusters of microscopic blood vessels in the kidneys that have small pores through which blood is filtered) in which excessive amounts of protein are excreted in the urine. This typically leads to accumulation of fluid in the body (edema) and low levels of the protein albumin and high levels of fats in the blood. Nephrotic syndrome is not a specific glomerular disease but a cluster of clinical findings, including:Marked increase in protein (particularly albumin) in the urine (proteinuria), Decrease in albumin in the blood (hypoalbuminemia), Edema, High serum cholesterol and low-density lipoproteins (hyperlipidemia).

CLASSIFICATION Idiopathic nephrotic syndrome (90% of cases) Minimal change nephrotic syndrome Nephrotic syndrome with mesangial proliferation Nephrotic syndrome with focal sclerosis Nephrotic syndrome secondary to glomerulonephritis (10% of cases) Membranous glomerulopathy MPGN OTHRS as SLE and HSP Congenital nephrotic syndrome AR presenting at birth or during the 1st 6 months Causes: Nephrotic syndrome has many causes and may either be the result of a disease limited to the kidney, called primary nephrotic syndrome, or a condition that affects the kidney and other parts of the body, called secondary nephrotic syndrome. Primary causes of nephrotic syndrome are usually described by the histology, i.e., minimal change disease (MCD), focal segmental glomerulosclerosis (FSGS) and membranous nephropathy (MN), sickle cell disease, diabetes mellitus and malignancy such as leukemia.

PREDISPOSING FACTO RATIONALE R Age Children ages 1 1⁄2 and 4 yr are predisposed in having nephrotic syndrome. Sex Males are more predisposed than males in acquiring nephrotic syndrome. Genetic People with family history of nephrotic syndrome increases likelihood of s developing nephrotic syndrome. PRECIPITATING FACTOR Focal segmental glomeruloscleros is - HIV/AIDS - Nephrectomy Membranous nephropathy – Hepatitis B infection – SLE – Cancer Minimal change disease Diabetes Mellitus Drugs – Heroin – NSAID – Gold – Penicillamine

RATIONALE Most common cause of idiopathic NS among adults. May be secondary to HIV/AIDS infection or loss of nephrons.

Deposition of immune complexes on the glomerular basement membrane causing it to thicken. It can be secondary to certain cancers, Hepatitis B infections and autoimmune disorders such as SLE.

Causes 80 to 90% of childhood nephrotic syndrome in children 4 to 8 years of age idiopathic in nature. Prolonged elevated blood glucose levels alters glomerular base membranes thereby causing impaired renal function. These drugs can contribute to the development of focal segmental glomerulosclerosis, membranous nephropathy and minimal change disease which in turn precipitate occurrence of nephrotic syndrome.

Human anatomy (KIDNEY)

NORMAL KIDNEY SIZE - The normal kidney size of an adult human is about 10 to 13 cm (4 to 5 inches) long and about 5 to 7.5 cm (2 to 3 inches) wide. It is approximately the size of a conventional computer mouse. NORMAL KIDNEY COLOR The kidneys are dark-red, bean-shaped organs. One side of the kidney bulges outward (convex) and the other side is indented (concave) NORMAL KIDNEY LOCATION – towards the back of the abdominal cavity, just above the waist. One kidney is normally located just below the liver, on the right side of the abdomen and the other is just below the spleen on the left side. – Kidney anatomy and excretion The most basic structures of the kidneys, are nephrons. They are responsible for filtering the blood. The renal artery delivers blood to the kidneys each day. Over 180 liters (50 gallons) of blood pass through the kidneys every day. When this blood enters the kidneys it is filtered and returned to the heart via the renal vein. The process of separating wastes from the body fluids and eliminating them, is known as excretion. The urinary system is one of the organ systems responsible for excretion. The

kidneys are the main organs of the urinary system. Kidney anatomy and blood vessels The kidney is full of blood vessels. Every function of the kidney involves blood, therefore, it requires a lot of blood vessels to facilitate these functions. Together, the two kidneys contain about 160 km of blood vessels. Renal capsule is a tough fibrous layer surrounding the kidney and covered in a thick layer of adipose tissue. It provides some protection from trauma and damage Renal cortex is the outer portion of the kidney between the renal capsule and the renal medulla. In the adult, it forms a continuous smooth outer zone with a number of projections (cortical columns) that extend down between the pyramids. Ultrafiltration occurs. Renal medulla is the innermost part of the kidney split up into a number of sections, known as the renal pyramids contains the structures of the nephrons responsible for maintaining the salt and water balance of the blood is hypertonic to the filtrate in the nephron and aids in the reabsorption of water

Patient's Profile a. Demographic Data Name: Baby X Age: 3 years old Sex: M Nationality: Filipino Religion: Catholic Physician: Joselito Mattheus, MD Informant: Minerva Flores (mother) Mother: Minerva Del Valle Flores Father: Christian Flores Date of Arrival: February 4, 2011 5:55 pm b. Chief Complaint: Periorbital Edema c. History of Present Illness: Was diagnosed with Nephrotic Syndrome last July 2010. Three days Prior to Admission, the patient had a non productive cough but with no other sign and symptoms of the illness. The patient noted to have periorbital edema upon waking up. And one day prior to admission, the patient was to have bipedal edema. This prompted the mother to seek medical attention. d. Past Medical History Baby X haven't undergone any surgery but was hospitalized last July 2010 in this institution due to the illness, Nephrotic Syndrome. He doesn't have any history of other disease. But the patient was already taking Prednisone even before. He doesn't have also any other childhood illness. Baby X had her immunization of BCG, Hepa (I, II, III), MMR, DPT (I, II, III), and OPV (I, II, III). e. Family History Mother HPN (+) Cancer (-) TB (-) Asthma (-) Diabetes Mellitus (-)

Father (+) (-) (-) (-) (-)

f. Maternal and Perinatal History Born to a 21 year old with a G2P1 in the year 2002. The mother had UTI during the second semester. Was a normal delivery in a lying-in by a midwife. Baby X was breastfed, and then shifted to bottled milk. Baby X started having solid food when he was 9 month old.

Laboratory

Total Protein Albumin Globulin Result

Values

Analysis

Interpretation

Total Protein

31.99

Adult:66-8 1-18y/o: 57-80 Newborns (128days): 41-63

Decreased

Due to low Albumin level

Albumin

10.21

35-50

Decreased

Dehydration,kid ney losses

Globulin

22.00

11-35

Normal

Normal

A/C Ratio

0.47

1.1-2.2

Decreased

Liver dysfunction

Result

Values

Interpretation

Analysis

Creatinine

20.77 mmol/L

Adults 45104 Neonate 2787 Infant 14-34 Child 23-68

Normal

Normal

Potassium

3.64 mmol/L

3.6-5.5

Normal

Potassium is the most abundant intracellular cation, much smaller accounts are found in the blood. K is essential for the transmission of electrical impulses in cardiac and skeletal muscle. In addition, it helps to maintain the osmolality and electroneutrality of cells, functions in enzyme reactions that transform glucose into energy and amino acids into proteins and participates in the maintenance of acid base balance.

Na

130.30 mmol/L

135-145

Decreased

Sodium is the most abundant electrolytes in ECF, its concentration

ranges from 135145 mmol/L. Hyponatremia refers to a serum Na level that is below normal (less than 135 meq/L) Na maybe lost by way of vomiting, use of diuretics particularly in combination with a low salt diet. Cl

102.20

100-111

Normal

Normal

Calcium

1.89 mmol/L

2.20-2.65

Below the normal range

Low blood calcium (or hypocalcemia) can have various causes, including: bone problems, low levels of the blood protein albumin, inflammation of the pancreas, kidney disease, malfunction of the parathyroid gland (hypoparathyroidism) and improper absorption of food or calcium.

URINALYSIS

A urinalysis is usually ordered when a doctor suspects that a child has a urinary tract infection or a health problem that can cause an abnormality in the urine. This test can measure:

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the the the the the

number and variety of red and white blood cells presence of bacteria or other organisms presence of substances, such as glucose, that usually shouldn't be found in urine pH, which shows how acidic or basic the urine is •

Color

RESULT Yellow

the concentration of the urine Normal Amber pale to dark yellow

Intepretation Normal

Analysis Urine is normally clear yellow or amber in color. The color of urine is

Transparenc y

Slightly Turbid

Clear

Normal

Reaction (ph) Specific Gravity

6.5

5-6

Increase

1.025

1.010-030

Normal

Sugar

(-)

Negative

Protein

(+)

Negative

EXAM NAME RBC WBC Crystals

Epithelial cells

RESULT 6-9 20-25 Amorphous Urates – Few

Few

mainly a result of the presence of the pigment urochrome, which is produced through endogenous metabolic processes. Transparency refers to the clarity of the urine. Acidic, may indicate infection. Specific gravity is an indicator of urine concentration of the amount of solutes (metabolic waste and electrolytes) present in the urine. ↑-diabetes mellitus, renal glycosuria (excretion of glucose), nephrotoxic chemicals (carbon monoxide) -

NORMAL VALUE 0-2/hpf 0-5/hpf None

INTEPRETATION

ANALYSIS

Normal Normal

Few

Normal

Some crystals are found exclusively in acid urine. Healthy people often have only a few crystals in their urine. A large number of crystals, or certain types of crystals, may mean kidney stones are present or there is a problem with how the body is using food (metabolism). Epithelial cells are found in urine samples are derived a.) the lining of female lower urethra and the vagina. b.) linings of the renal pelvis bladder. c.) renal

Bacteria cast Casts

Few Hyaline cast 1-2/hpf

Few None

Normal

tubules themselves Hyaline casts are formed in the absence of cells in the renal tubular lumen. They have a smooth texture and a refractive index very close to that of the surrounding fluid. When present in lower numbers (0-1/LPF) in concentrated urine of otherwise normal patients, hyaline casts are not always indicative of clinically significant disease.

Complete Blood Count The complete blood count (CBC) is a common blood test that evaluates the three major types of cells in the blood: red blood cells, white blood cells, and platelets. A CBC may be ordered as part of a routine checkup, or if your child is feeling more tired than usual, seems to have an infection, or has unexplained bruising or bleeding. The CBC can also test for loss of blood, abnormalities in the production or destruction of blood cells, acute and chronic infections, allergies, and problems with blood clotting.

Result

Values

Interpretation

Analysis

Hgb

12.6

12-14 g/L

Normal

Hgb is the main intracellular protein. Its primari

Hct

0.403

0.37-0.47

Normal range

Hct or packed RBC volume measure the proportion of RBC's in a volume of whole blood and is expressed as a percentage.

RBC

4.88

4.7-6.1 x10^9/L

Normal

Erythrocytes are the most

abundant cells of the blood. Its primarily responsible for tissue oxygenation thus, a decreased level of RBC indicates anemia. MCV

82.4

80-95fL

Normal

MCH

25.8

27-31pg

Below the normal range

31.3

32-36 g/dL

MCHC

Mean corpuscular volume indicates the volume/size of the hgb each RBC. Very useful in differentiatin g anemia.

Below the normal MCHC is the range proportion of the hgb contained in each RBC. Indication of hgb deficiency and of the oxygen carrying capacity of the individual erythrocytes.

MEDICAL MANAGEMENT Fluid and sodium restriction, oral or intravenous diuretics, and angiotensinconverting enzyme inhibitors. • Fluid and sodium restrictions Creating a negative sodium balance will help reduce edema, presumably as the underlying illness is treated or as renal inflammation slowly resolves. Patients should limit their sodium intake to 3 g per day, and may need to restrict fluid intake (to less than approximately 1.5 L per day). Large doses (e.g., 80 to 120 mg of furosemide) are often required and these drugs typically must be given intravenously because of the poor absorption of oral drugs caused by intestinal edema. Low serum albumin levels also limit diuretic effectiveness and necessitate higher doses. Thiazide diuretics, potassium-sparing diuretics, or metolazone (Zaroxolyn) may be useful as adjunctive or synergistic diuretics. • Diuretics "Diuretics are the mainstay of medical management; however, there is no evidence to guide drug selection or dosage," Dr. Kodner writes. "Based on expert opinion, diuresis should aim for a target weight loss of 1 to 2 lb (0.5 to 1 kg) per day to avoid acute renal failure or electrolyte disorders. Loop diuretics, such as furosemide (Lasix) or bumetanide, are most commonly used." • Angiotensin-converting enzyme In persons with nephrotic syndrome, angiotensin-converting enzyme inhibitors have been shown to decrease proteinuria and lower the risk for progression to renal disease. Angiotensin-converting enzyme (ACE) inhibitors have been shown to reduce pro-teinuria and reduce the risk of progression to renal disease in persons with nephrotic syndrome.15,16 One study found no improvement in response when corticosteroid treatment was added to treatment with ACE inhibitors.17 The recommended dosage is unclear, and enalapril (Vasotec) dosages from 2.5 to 20 mg per day were used. Most persons with nephrotic syndrome should be started on ACE inhibitor treatment to reduce protein-uria, regardless of blood pressure. * Although corticosteroid treatment may benefit some adults with nephrotic syndrome, research evidence supporting this therapy is limited. At present, intravenous albumin, prophylactic antibiotics, and prophylactic anticoagulation are not advised. ALBUMIN Intravenous albumin has been proposed to aid diuresis, because edema

may be caused by hypoalbuminemia and resulting oncotic pressures. However, there is no evidence to indicate benefit from treatment with albumin, and adverse effects, such as hypertension or pulmonary edema, as well as high cost, limit its use.

CORTICOSTEROIDS Treatment with corticosteroids remains controversial in the management of nephrotic syndrome in adults. It has no proven benefit, but is recommended in some persons who do not respond to conservative treatment. Treatment of children with nephrotic syndrome is different, and it is more clearly established that children respond well to corticosteroid treatment. Family physicians should discuss with patients and consulting nephrologists whether treatment with corticosteroids is advisable, weighing the uncertain benefits and possibility of adverse effects. Alkylating agents (e.g., cyclophosphamide [Cytoxan]) also have weak evidence for improving disease remission and reducing proteinuria, but may be considered for persons with severe or resistant disease who do not respond to corticosteroids.

LIPID-LOWERING TREATMENT A Cochrane review is underway to investigate the benefits and harms of lipid-lowering agents in nephrotic syndrome. Some evidence suggests an increased risk of athero-genesis or myocardial infarction in persons with nephrotic syndrome, possibly related to increased lipid levels. However, the role of treatment for increased lipids is unknown and, at present, the decision to start lipidlowering therapy in persons with nephrotic syndrome should be made on the same basis as in other patients.

ANTIBIOTICS There are no data from prospective clinical trials about treatment and prevention of infection in adults with nephrotic syndrome. Given the uncertain risks of infection in adults with nephrotic syndrome in the United States, there are currently no indications for antibiotics or other interventions to prevent infection in this population. Persons who are appropriate candidates should receive pneumococcal vaccination.

ANTICOAGULATION THERAPY There are currently no recommendations for prophylactic anticoagulation to prevent thromboembolic events in persons with nephrotic syndrome who have not had previous thrombotic events, and clinical practice varies. A Cochrane review is in process. Physicians should remain alert for signs or symptoms suggesting thromboembolism and, if it is diagnosed, these events should be treated as in other patients. Persons who are otherwise at high risk of thromboembolism (e.g., based on previous events, known coag-ulopathy) should be considered for prophy-lactic anticoagulation while they have active nephrotic syndrome. NURSING MANAGEMENT • • • • • • • • •

Assess and document the location and character of the patient's edema. Weigh the patient each morning after he voids and before he eats, make sure he's wearing the same amount of clothing each time you weigh him. Measure blood pressure with the patient lying down and standing. Immediately report a decrease in systolic or diastolic pressure exceeding 20 mm Hg. Monitor intake and output Ask the dietitian to plan a low-sodium diet with moderate amounts of protein. Frequently check urine for protein Provide meticulous skin care to combat the edema that usually occurs with nephrotic syndrome Use a reduced-pressure mattress or padding to help prevent pressure ulcers. To prevent the occurrence of thrombophlebitis, encourage activity and exercise,

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and provide antiembolism stockings as ordered Give the patient and family reassurance and support, especially during the acute phase, when edema is severe and the patient's body image changes.

DISCHARGE PLANNING MEDICATION •

Advice patient to continue the medications prescribed by the physician.

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Emphasize the strict adherence to dosage and the frequency for desired therapeutic effect

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Explain the possible consequences of not adhering the medication and the possible side effects while taking the medications.

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Caution patients who are receiving steroid therapy to take the dosages exactly as prescribed; explain that skipping doses could be harmful or lifethreatening.

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In cases of long-term steroid therapy, explain the signs of complications, such as GI bleeding, stunted growth (children), bone fractures, and immunosuppression.

EXERCISE •

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Advice the client to perform light exercise such as: stretching of the upper and lower extremities and carrying out some simple chores as form of exercise for good circulation. To prevent atrophy of the muscles especially on the affected part of the body and body weakness Do not restrict on activity, unless the client is severely edematous

TREATMENT •

Encourage patients with hypercoagulability to maintain hydration and mobility and to follow the medication regimen.

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Explain that they need to monitor the urine daily for protein and keep a diary with the results of the tests.

HEALTH TEACHING

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Teach the patient and family about the disease process, prognosis, and treatment plan.

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Instruct the patient and family to avoid exposure to communicable diseases and to engage in scrupulous infection control measures such as frequent hand washing.

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Teach family members to report even mild signs of infection.

OUT – PATIENT •

Inform about possible check – ups and treatment especially if fever and abdominal pain recur.

DIET •

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Advise the mother of the client to avoid adding extra salt to food at the table and try to reduce the intake of processed foods such as: chips, canned goods, tocino, instant noodles, seasoning, etc. Reduce sodium intake to 1000-2000mg daily, sodium should be less than or equal to calories per serving Instruct the mother when giving her child of fluids; it should be less than 1000 liter per day. Limit on intake of fluid to avoid edematous

SPIRITUALITY •

Encourage creative expression, as in art, music and writing. This keeps the imagination alive and serves to regenerate the body, mind and spirit.