PEDIA Case 1.2. Nephrotic Syndrome

8/16/2019 PEDIA Case 1.2. Nephrotic Syndrome

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Pangilinan, JunevePascua, Krinzel

Perez, WilliamPescante, Ma Nina

Pediatrics IIA Case presentation


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GENERAL DATA

Patient: D.D Age: 6 y/o Filipino

Male Currently lives in San Andres Bukid, Manila Birth date: February 7, 2008 3rd time admission at Ospital ng Maynila Medical

Center on June 23, 2014 at around 9:30 pm Informant: Father Reliability: 90%


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CHIEF COMPLAINT

Difficulty opening his eyes


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HISTORY OF PRESENT ILLNESS


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16 MONTHS PTA

Patient manifested with periorbital edema in the morning.

Swelling progressed to his face then to his abdomen andlower extremities.

Edema was described to be nonpitting which lasted for 4days.

Tea-colored urine was also noted as well as a remarkableincrease in the patient’s weight from 30 kg to 40 kg.

No medication was taken but patient sought consult toOMMC and was admitted for the first time


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16 MONTHS PTA

During admission, patient’s highest BP was 120/90 mmHgcompared to his normal BP of 90/60 mmHg.

Patient was given with IV dextrose and albumin withunrecalled dosage and swelling subsided.

It took 4 days before edema was noted to have completelyresolved thus the patient was discharged.


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16 MONTHS PTA

Patient was prescribed with the ff medication: prednisone 5 mL syrup once every other day

isoniazid (unrecalled dosage)

Patient was compliant in taking the medications.

They were advised that the patient should avoid salty foods,chocolates and juice.

They were advised that the patient should avoid sick people


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Patient was apparently well after 1st admission.

Patient had a regular consult every 2 weeks at OMMC OPD.

Urine dipstick test was done every checkup and level ofalbumin was regularly monitored.


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Father of the patient noticed that patient easily contractsrespiratory infections almost every 3 months.

Patient usually manifests with undocumented fever, clearnasal discharge and cough which last for a week.

Patient was given amoxicillin (250mg/5mL) syrup for 7 daysevery time he was suffering from respiratory infections.

Patient was compliant in taking the medication and

symptoms usually resolved after treatment.


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6 MONTHS PTA

Patient manifested again with facial edema and abdominalswelling of the same quality and pattern which promptedsecond admission at OMMC

Same medications were given and patient stayed in the

hospital for a week.

Patient continued taking his medication after discharge andwas able to return to school after hospital admission.

Patient was apparently well after the 2nd

admission until 1month PTA


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1 MONTH PTA

Patient missed a checkup because he was in the province.

On his next check up, patient had urine dipstick test

Results showed cloudy urine and +4 albumin.

In addition to his maintenace drug (prednisone), patient wasprescribed with enalapril with unrecalled dosage.

Patient was compliant together with other medication.

No other symptoms were reported until 1 week PTA


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1 WEEK PTA

Patient woke up with periorbital and facial edema butdismissed it as “taba sa mukha”

It was nonpitting without accompanying redness, tenderness,itchiness or any discomfort.

Tea-colored urine described as “iced tea” in color was alsonoted.

No other accompanying symptoms were noted like fever,dysuria or flank pain.

No other medication was taken aside from prednisone andenalapril.

Swelling did not resolve.


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3 DAYS PTA

Presence of periorbital and facial edema persists. Abdominal swelling and bilateral pitting sacral edema were

also noted.

Same urine color was noted but no changes in urinefrequency, volume and urgency were observed.

No other medications were taken. Consult was not done.


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2 HOURS PTA

Swelling of the abdomen was still apparent and swelling onhis legs below the knee were noted.

Presence of periorbital and facial edema persists but patientcomplained of difficulty opening his eyes so they sought

consult to OMMC hence the admission.


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REVIEW OF SYSTEMS

Constitutional (-) loss of appetite

Skin and nails (-) rashes (-) changes in skin color

Head (-) headache (-) lightheadedness (-)dizziness (-) syncope

Eyes

(-) eye pain (-) blurred vision (-) discharge

Ears (-) discharge (-) hearing problem

Nose and sinuses (-) tenderness (-) epistaxis

Mouth and throat (+) sore throat (-) dysphagia

Respiratory (-) dyspnea (-)orthopnea (-) PND

Cardiovascular (-) chest pain (-) palpitations

Gastrointestinal (+) vomiting (+) diarrhea (-)hematemesis (-) hematochezia


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REVIEW OF SYSTEMS

Genitourinary (-) flank pain

Endocrine (-) excessive sweating

Nervous/Behavioral (-) paresthesia (-) numbness

Musculoskeletal (-) muscle pain

Hematologic (-) pallor (-) bruising (-) overt bleeding


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PAST MEDICAL HISTORY

(-) allergies in food and medication (+) measles and chickenpox

Immunization History

Immunizations taken: 1 dose BCG

3 doses DPT 3 doses OPV

3 doses hepatitis B

1 dose measles.


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PERSONAL HISTORY

Feeding History

Patient has good appetite, eats regularly and does notskip meals

Eats 3 full meals a day with snacks in between MEAL: 1 cup of rice, fried egg white, 1 serving of meat (fried chicken

without skin, fish or pork)

SNACKS: sandwiches/ biscuits and mineral water

Seldom eats leafy and non-leafy vegetables

Preference for fruits, milk, and sweet foods

3-4 glasses of mineral water a day

Does not take vitamins nor food supplements.


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PERSONAL HISTORY

Family HistoryFather (39), works at an upholstery cleaning

service and Mother (36), housewife, both with no

known illnessPatient had a 3-mo old sibling with no known

illnesses.

Reported history of HPN on father’s side and DMon mother’s side. No family history of asthma,kidney disease, cardiac disease, blood disorders andcancer.


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Socioeconomic History lives with parents, sister and aunt (mother’s side) in one

floor of a multi-storey residence in San Andres, Bukid,Manila for 6years.

Father and aunt work for their living

Environmental History Patient often stays at home, otherwise plays with friends at

school and neighbourhood. No exposure to cigarette smoke at home.

Drinking water is mineral type

Regular medications: prednisone and enalapril; amoxicillinand cough syrups

PERSONAL HISTORY


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PHYSICAL EXAM


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GENERAL SURVEY

Lying on bedWith oxygen mask

Regulated at 3L per minute

Conscious

Coherent

Slightly irritable but cooperative


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Vital Signs

PR: 154 bpmRR: 20 breaths per min Regular rhythm and depth

BP: 100/60 Over right brachial artery, supine

Temp: 37.7°C, axillary


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Anthropometric

Weight: 24.9 kgHeight: 108 cm

BMI: 21.35Abdominal circumference: 75 cm


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Anthropometric


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SKIN

Moist and warmNo jaundice or cyanosis

Good skin turgor No suspicious nevi, rash, petechiae,

eccyhmoses

No clubbing on fingernails


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EAR

Symmetric without swelling, redness, ordischarge

Non tender

Intact tympanic membrane on both sides

Heard sound on whisper test at 2ft. on left

and right ears Weber and Rinne test not done


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NOSE AND SINUSES

Symmetric without any deformities,obstruction, lesions, exudates or

discharge.Nasal mucosa was pink

Septum in the midline

No frontal and maxillary sinustenderness


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MOUTH AND THROAT

No circumoral pallor Lips were pink and moist without any sores or

lesions Gums without swelling or ulceration Oral mucosa was pinkish without ulcers, white

patches or nodule Impacted first molar of all quadrants of the mouth

Tongue is pinkish, moist and in the midline withoutlesions. Uvula in the midline Tonsils are pink, Grade 1


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NECK

Trachea was in the midline Nonpalpable and nontender cervical lymph nodes

Thyroid gland not enlarged

Carotid pulsations and jugular vein pulsation notprominent


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THORAX AND LUNGS

Symmetric chest without deformities Transverse diameter > anteroposterior diameter

No retractions and respiratory lag

No use of accessory respiratory muscles

Equal chest expansion

Tactile fremitus not performed

Bronchovesicular breath sounds were heard onmajority of intercostal spaces on both lung fields

No adventitious breath sounds


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CARDIOVASCULAR SYSTEM

Adynamic precordium PMI at 4th ICS left midclavicular line

No heaves, lifts, or thrillsS1 louder than S2 at the apex

S2 was louder than S1 at the base

No murmurs


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ABDOMEN

Protuberant No scars, lesions, engorged blood vessels No bulging of flanks Skin on the abdomen pitted when the diaphragm of

the stethoscope was placed over it No bruits on the epigastric area and hypogastric

area

Normoactive bowel sounds at 40/min (-) fluid wave No mass or tenderness


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Genitourinary

(+) scrotal swellingTea-colored urine


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EXTREMITIES AND

PERIPHERAL VASCULAR

Bilateral pitting edemalevel below the knee

FeetFace

Edema grade 2+ lasts for 37-40 seconds

No varicosities, deformities, visible joint swelling and redness


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NEUROLOGIC

ConsciousCoherent

Slightly irritableCooperative

Clear speech

Folstein MMSE was not done


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MOTOR EXAM TESTS FOR

COORDINATION REFLEXES

SENSORY EXAMINATION

Not done


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SALIENT FEATURES

CC: Difficulty opening the eyes6 years old maleFacial swelling Periorbital swellingScrotal swellingTea-colored urineGrade 2+ bipedal edema below the knee Cloudy urine+4 albumin in the urine Irritability


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APPROACH TO

DIAGNOSIS

The presenting manifestation of the patientis

EDEMA

. Approach to diagnosis will bebased on the presenting manifestation thatpoints to a

GROUP OF DISEASE OR

DISORDERS


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DIFFERENTIAL

DIAGNOSIS


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DIFFERENTIAL DIAGNOSIS

Patient’s Signs &Symptoms

1. IdiopathicNephrotic Syndrome

(INS)

2. AcuteGlomerulonephritis

(AGN)

3. Protein-losingNephropathy

(PLN)

Edema (+) (+) (+)

Proteinuria (+) (+) (+)

Hypertension(once)

rare prominent prominent

Tea-coloredurine

(+) ~20% presentswith microhematuria

(+) usually grosshematuria

(+)

Cloudy urine (+) (+) (+)

Weight gain (+) (-) (-)

Others o Most common inmales aged 2-6years old

o Usually presents withcloudy urine which

was seen in thepatient

Patient did not present

with the following

characteristic symptoms

of AGN:

o Dyspnea

o Oliguria

o Headache

o Flank pain

Patient did not present

with the following

characteristic symptoms

of PLN:

o Abdominal pain

o Signs of liver

disease (ie.

jaundice,splenomegaly etc.)

Decision Cannot be ruled out Ruled out Ruled out


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DIAGNOSTIC

WORK-UP


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URINE PROTEIN MEASUREMENT

Measured by a timed collection done over a24-hour period (starting at 7am andfinishing the next day at the same time) or a

single spot collection In healthy individuals, there are no more

than 150 mg of total protein in a 24-hoururine collection.


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URINE PROTEIN MEASUREMENT

When the ratio of urine protein to urinecreatinine is greater than 2 g/g, thiscorresponds to 3 g of urine protein per dayor more

The exact type of urine protein is ofpotential interest and can be tested by urine

protein electrophoresis This differentiates nephrotic syndrome from

other protein-secreting diseases other than

albumin


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SEROLOGIC TESTS

Serum albumin level is classically low in nephroticsyndrome, being below its normal range of 3.5-4.5 g/dL

In a study, frequency of focal and segmental

glomerulosclerosis increased with elevations in serumalbumin from 26% in patients with serum albumin < 30 g/L to

74% in patients with serum albumin of 35 g/L or higher

Serum cholesterol and triglyceride are usually elevated.Checking this will allow adjustment in treatment.


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SEROLOGIC TESTS

Serum creatinine will be normal ranged inuncomplicated nephrotic syndrome but itmay be increased due to diminished renal

perfusion and may indicate atypicalnephrotic syndrome that may warrantreferral to pediatric nephrologist.

Serum electrolyte balance should bemonitored.


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Management


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NONPHARMACOLOGIC

MANAGEMENT

Diet should provide adequate energy (caloric intake) andadequate protein (1-2 g/kg/d)

SALT/FLUID RESTRICTION

Sodium intake should be reduced by the initiation of a

low-sodium diet and may be normalized when the childenter remission.

1 to 3 years 2g salt per day (0.8g sodium)

4 to 6 years 3g salt per day (1.2g sodium)

7 to 10 years 5g salt per day (2g sodium)

11 and over 6g salt per day 2.4g sodium) www.clinical guidelines.scot.nhs.uk


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NONPHARMACOLOGIC

MANAGEMENT

Fluid restriction maybe necessary if the child is hyponatremic.

A gentle fluid restriction is also usually beneficial to minimizeedema. Suggested fluid intake: (Christian, 2013)

5 yrs = 1 litre/day

A swollen scrotum may be elevated with pillows to enhancethe removal of fluid by gravity.


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NONPHARMACOLOGIC

MANAGEMENT

Close monitoring of volume status, serum electrolyte balance and renal function is necessary.

Daily weight monitoring and daily urine dipstick.

There are no activity restrictions for patients with Nephrotic

Syndrome. Ongoing activity rather than bedrest will reducethe risk of blood clots.


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PHARMACOLOGIC

MANAGEMENT

STEROID THERAPY (INITIAL THERAPY) Prednisone (12-week initial course): 60 mg/m2/ day [2 mg/kg/day] (minimum daily

dose, 80 mg divided into 2-3 doses) for 4-6

consecutive weeksAfter initial 6-week course, Prednisone dose should be

tapered to 40 mg/m2/day [1.5 mg/kg] given everyother day as single morning dose for 6 weeks

The alternate-day dose is then slowly tapered anddiscontinued over the next 2-3 months Reduce dose by 5-10mg/m2 each week


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PHARMACOLOGIC

MANAGEMENT

RESPONSE TO TREATMENTMost respond to steroids within 2-4 weeks

Children who continue to have proteinuria (2+ orgreater) after 8 weeks of steroid therapy areconsidered RESISTANT and a diagnostic renalbiopsy should be performed.


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PHARMACOLOGIC

MANAGEMENT

STEROID-DEPENDENT NEPHROTICSYNDROME THERAPY

CYCLOPHOSPHAMIDE

2-3 mg/kg/24 hrs given as a single dose for a totalof 8-12 weeks

Prolongs the duration of remission and reduces thenumber of relapses in children with frequentlyrelapsing and steroid-dependent nephroticsyndrome.


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PHARMACOLOGIC

MANAGEMENT

METHYLPREDNISONE 30 mg/kg bolus (maximum 1g)

First 6 doses given every other day followed by a taperingregimen for periods up to 18 mos

Given for children with Complicated NephroticSyndrome.

CYCLOSPORINE

3 – 6 mg/kg/24 hr divided q 12 hr

TACROLIMUS

0.15 mg/kg/24 hr divided q 12 hr


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PHARMACOLOGIC

MANAGEMENT

ANTIHYPERTENSIVE THERAPYOnly when hypertension is present and particularly if

it persists

ACE Inhibitors

ARBs

Ca channel blockers

Beta blockers


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REFERENCES

Fauci AS, Kasper DL, Longo DL, Braunwald E, Hauser SL, Jameson J.(2008). Harrison's Principles of Internal Medicine. Philadelphia: McGrawHill Companies.

Gipson DS, Massengill SF, Yao L, Nagaraj S, Smoyer WE, Mahan JD,Wigfall D, Miles P, Powell L, Lin JJ, Trachtman H, Greenbaum LA.

(2009). Management of Childhood Onset Nephrotic Syndrome.Pediatrics 124: 747-757

Kliegman, R. (2007). Nelson textbook of pediatrics. — 18th ed. Philadelphia,PA: Saunders Elsevier.

Lane JC, Langman CB, Finberg L. (2014). Pediatric NephroticSyndrome Treatment & Management. Retrived on 6 July, 2014 fromhttp://emedicine.medscape.com/article/982920-treatment#showall


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REFERENCES

Cohen, Eric P. and Vecihi Batuman. 2014. NephroticSyndrome Diagnostic Workup. Accessed on 29 June, 2014from http://emedicine.medscape.com/article/244631-workup.

Fauci, A. S., Kasper, D. L., Longo, D. L., Braunwald, E.,Hauser, S. L., Jameson, J., et al. (2008). Harrison's Principles ofInternal Medicine. Philadelphia: McGraw Hill Companies.

Kliegman, R. 2007. Nelson textbook of pediatrics. 18th ed.

Philadelphia, PA: Saunders Elsevier. p. 3564-3569.

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PEDIA Case 1.2. Nephrotic Syndrome

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