CASE PRESENTATION

Nephrotic syndrome
FASL GOLDANNE BUENAFLOR
4TH YR MEDICAL CLERK

OUTLINE
Present a case of Nephrotic Syndrome  Diagnosis  Pathophysiology  Management  Complications  Prognosis  Summary


GENERAL DATA
G.J.  2 yrs old  Male  South Cotabato  Admission Date: July 25, 2011  Informant: mother


CHIEF COMPLAINT


generalized edema

HISTORY OF PRESENT ILLNESS

 Px

is a diagnosed case of Nephrotic Syndrome since March 2011

 Recurrent onset of
 Initially on

4 days PTA

edema

the eyes  Then, abdomen and extremities



Consultation with AP done

 Referred to our  Deferred

institution

HISTORY OF PRESENT ILLNESS

1 day PTA

 New onset of
 On and

fever

off  Moderate grade  Partially relieved by PCM



Associated with nonproductive cough and coryza signs and symptoms prompted this admission

Few hrs PTA

 Persistence of

PAST MEDICAL HISTORY

HOSPITALIZATIONS:


March 2011 NS and BPN (7days)



Unrecalled antibiotic, Furosemide, Prednisone Prednisone Prednisone

May 2011 NS x 4days



June 2011 NS x 2 days



FAMILY HISTORY
DM = maternal side HPN = both sides Asthma = maternal side Nephrotic Syndrome = (-)

MATERNAL & OBSTETRIC HISTORY

st 1

child  24 years old G2P2  Good prenatal follow up  No history of antenatal infection  Non smoker  Non alcoholic beverage drinker

BIRTH AND NEONATAL HISTORY

Delivered via NSVD 7 months AOG Preeclampsia Icterisia admitted for 20 days

GROWTH AND DEVELOPMENT

Social smile = 2 mos Able to hold head = 3 mos Rolls over = 5 mos Babbles = 6 mos Sits without support = 7 mos Crawls = 8 mos Stands with support = 10 mos Walks = 14 mos

IMMUNIZATION HISTORY
BCG x 1 Hepatitis B x 3 OPV x 3 DPT x 3 Measles x 1

DIET/NUTRITIONAL HISTORY
Breast fed = first 3-4 wks 3 Bottle fed with PediaSure  Complementary feeding @ 6 mos  Rice, Fish, Vegetables, etc


PERSONAL and SOCIAL HISTORY

Parents are both Filipinos  Lives with both his parents and 1 sibling  No history of recent travel


ENVIRONMENTAL HISTORY
No smoker in the house  No pets  No exposure to anyone who is ill  No epidemic dse in community  Proper waste disposal


REVIEW OF SYSTEMS
(-) significant weight change  (-) nausea/vomiting  (+) cough  (-) skin lesions  (-) seizure


PHYSICAL EXAMINATION
>awake, alert and responsive; afebrile; not in respiratory distress

Vital Signs: PR: 128 RR: 39 T: 36.6 BP: 100/60

Anthropometric Measurements: Weight = 13 kg Length = 82 cm BSA = 0.54

PHYSICAL EXAMINATION


SKIN: Warm to touch with good turgor and

mobility. No rashes nor jaundice noted.

HEENT:
Head normocephalic; (+) facial swelling  Eyes anicteric sclerae, pinkish palpebral conjunctivae; briskly reactive pupils; (+) periorbital sweling  Ears (-)discharge, (-)tenderness ( Nose (-)discharge, nasal septum in midline  Throat pinkish buccal mucosa


Figure 1.

Nephrotic edema.

PHYSICAL EXAMINATION


NECK: No lesions were noted. Neck is supple and thyroid is non-palpable. nonLYMPH NODES: No palpable lymph node was noted. LUNGS and THORAX:
   

I: symmetrical chest wall; (-) tachypnea; (-) ((retraction P: equal chest expansion P: resonant on both lung fields A: (+) crackles; (+) harsh breath sounds

PHYSICAL EXAMINATION


CVS:
I: adynamic precordium  P: PMI at 5th ICS MCL  A: Regular cardiac rate and rhythm


ABDOMEN:
I: globular abdomen  A: normoactive bowel sounds  P: tympanitic on all quadrants  P: (+) distention; (-)tenderness; (-) organomegaly


PHYSICAL EXAMINATION


BREASTS: symmetrical with no lesions or discharge GENITOURINARY: (-)discharge; (-)dysuria; (+) ((minimal scrotal swelling EXTREMITIES: equal, full pulses; (+) Grade IV pitting bipedal edema MUSCULOSKELETAL: (-)deformity; (-) ((tenderness

SALIENT FEATURES
HISTORY: PHYSICAL EXAMINATION:  (+) facial swelling, periorbital edema  Male sex  (+) crackles, harsh breath sounds  Age 2 yrs  (-) murmurs, tachycardia  (-) history of:
(+) distended abdomen  CV Dse  (-) fluid wave/shifting dullness  Liver Dse  (-) organomegaly  Renal Failure  (+) minimal scrotal swelling  (+) Gr. IV bipedal edema


OUTLINE
Present a case of Nephrotic Syndrome  Diagnosis  Pathophysiology  Management  Complications  Prognosis  Summary


BASIC APPROACH TO A PX WITH GROSS EDEMA

HEART FAILURE LIVER FAILURE GROSS EDEMA ACUTE KIDNEY INJURY

CANCER METZ

NEPHROTIC SYNDROME

NEPHROTIC SYNDROME
CRITERIA:  Heavy Proteinuria  Hypoalbuminemia  Edema  Hyperlipidemia Age of incidence: all ages but most between 1-5 yrs old 1 M>F


TYPES


Clinical
Pure or Simple  Mixed or Complex


Response to Steroid Therapy

Steroid Responsive (Complete or Incomplete)  Steroid Resistant


Pathologic
Minimal Change Disease  Non-Minimal Change Disease (MGN,FSGS, NonMPGN)


CAUSES


Primary
MCD  FSGS  MGN  MPGN


Secondary
Diabetes Mellitus  SLE  Amyloid  HIV  Drugs: NSAIDs, Gold, etc.


In children under age 5 years the disease usually takes the form of idiopathic (primary) NS of childhood (nil disease, lipoid nephrosis).

OUTLINE
Present a case of Nephrotic Syndrome  Diagnosis  Pathophysiology  Management  Complications  Prognosis  Summary


Proteinuria
At least ++++ urine protein or >3.5g/24hr (adult), or >40/mg/m2/hr (child)  Causes: systemic overproduction, tubular dysfunction, or glomerular dysfunction


UA (7/25/11) Urine protein ++++

Heavy proteinuria (albuminuria)

Hypoalbuminemia
 

Serum albumin <2.5mg/dl D/t urinary protein loss

Blood Chemistry (7/26) S. albumin 2mg/dl

Edema


Generalized pitting edema

Grading of Pitting Edema Grade I: slight pitting, 2mm, disappears rapidly Grade II: little deep pit, 4mm, disappears in 10-15s Grade III: deep pitting, 6mm, may last more than a minute, extremities swollen Grade IV: very deep pitting, 8mm, pit lasts 25mins, extremity grossly swollen

Starling Formula

Hyperlipidemia


Most nephrotic patients have elevated levels of total and low-density lipoprotein (LDL) lowcholesterol. Nephrotic patients often have a hypercoagulable state and are predisposed to deep vein thrombophlebitis, pulmonary emboli, and renal vein thrombosis.

7/25/11 Cholesterol 17.18 (<5.2) VLDL 2.66 (0-0.43) (0TG 5.86 (0-1.95) (0-

OUTLINE
Present a case of Nephrotic Syndrome  Diagnosis  Pathophysiology  Management  Complications  Prognosis  Summary


TREATMENT
A) General measures (supportive/symptomatic)  Diuresis to relieve edema  Monitoring kidney function  Treat dysliidemias  Albumin infusions are generally not used because their effect lasts only transiently.

TREATMENT


Initial attack: Prednisone 60mg/m2/day or 2mg/kg/day in divided doses for 4wks Relapse and recurrence:
Increase corticosteroid  Use immunosuppresive agents (e.g. cyclophosphamide, chlorambucil, clyclosporin A)


OUTLINE
Present a case of Nephrotic Syndrome  Diagnosis  Pathophysiology  Management  Complications  Prognosis  Summary


Complications
    

Thromboembolic Disease (Renal V Thrombosis) Infection Acute Renal Failure Pulmonary Edema Growth Retardation

OUTLINE
Present a case of Nephrotic Syndrome  Diagnosis  Pathophysiology  Management  Complications  Prognosis  Summary


PROGNOSIS
Depends on cause of NS  Most cases of MCD remit permanently  Good in children d/t steroid responsiveness  FSGS ESRD


OUTLINE
Present a case of Nephrotic Syndrome  Diagnosis  Pathophysiology  Management  Complications  Prognosis  Summary


SUMMARY
NS: hypoproteinemia, heavy proteinuria, hypoproteinemia, proteinuria, edema, hyperlipidemia  Dx: clinical and pathologic Dx:  Mgt: symptomatic and immunosuppresion  Prognosis: generally good


Thank you

Table 1. STAGES OF CHRONIC KIDNEY DISEASE*

STAGE DESCRIPTION 1 2 3 4 5 Kidney damage with normal or Kidney damage with mild or Moderate Severe GFR GFR GFR (mL/min/1.73m2) 90 6060-89 3030-59 1515-29 <15 (or dialysis)

GFR

GFR

Kidney failure

* Chronic kidney disease is defined as either kidney damage or GFR < 60mL/min/1.73m2 for 3months. Kidney damage is defined as pathologic abnormalities or markers of damage, including abnormalities in blood or urine tests or image studies.