Question

template for nephrotic syndrome and heart failure in pediatric

Answer 1

NEPHROTIC SYNDROME ;

THE CHILD IS HAVING HEAVY PROTEINURIA + HYPOALBUMINEMIA+ EDEMA (PITTING) +HYPERLIPIDEMIA

1.GLOMERULAR SYNDROME INVOLVING -

DECREASED GFR

ANEMIA

INCREASED INFLAMATORY MARKERS

ECF VOLUME REPLETE /OVERLOAD

ETIOLOGY

PRIMARY OR IDIOPATHIS NS 90% SECONDARY NS 10% CONGENITAL NS

=MINIMAL CHANGE DISEASE (MCD) = INFECTIONS -HIV,HBV,HCV = FINNISH TYPE

=SIGNIFICANT GLOMERULAR = ATOIMMUNE DISORDER = DIFFUSE MESA-

HISTOLOGICAL LESION Eg; SLE,HSP NGIAL SCEROSIS

Eg;MESANGIAL POLIFERATION =ALLERGIES =CONGENITAL INFECTION

=DRUGS-NSAID Eg;TORCHS,HBV

PATHOPHYSIOLOGY

=INCREASE GLOMERULAR CAPILLARY WALL PEREABILITY FOR PROTEINS

DUE TO

LOSS OF NEGATIVE CHARGED GLYCOPROTEIN IN MCD

DUE TO

MUTATION IN PODOCYTE PROTEINS (PODOCIN,BETA-ACTININ 4 )IN NON-MCD

LEAD TO

MASSIVE PROTEINURIA

HYPOALBUMINEMIA

LEADS

STIMULATE LIVER PROTEIN AND LIPID SYNTHESIS

AND

INCREASE SERUM LIPID LEVEL(TG,CHOLESTROL

HYPERLIPIDEMIA

HYPOALBUMINEMIA

LEADS

DECREASE PLASMAONCOTIC PRESSURE

AND

FLUID MOVE FROM INTRAVASCULAR TO INTERSTITIAL SPACE

CAUSES

EDEMA AND HYPOVOLEMIA

DUE TO

DECREASE RENAL PERFUSION PRESSURE

STIMULATE

RELEASE OF ADH AND ALDOSTERONE LEADS TO WATER AND NA RETENSION

CLINICAL MANIFESTATION OF IDIOPATHIC NS

AGE OF ONSET-MOST COMMONLY APPEARS BETWEEN 2-6 YEARS

MALE ; FEMALE=2:1

EDEMA STARTS AROUND EYES (PERIORBITAL AND FACIAL PUFFINESS THEN GRADUALLY PROGRESSES OVER EXTREMITIES,GENITALS AND WITH DEVELOPMENT OF PLEURAL EFFUSIONS AND ASCITES (ANASARCA)

NONSPECIFIC CLINICAL MANIFESTATION

FATIQUE AND LETHARGY

ANOREXIA ,IRRITABILITY

NAUSEA AND VOMITING ,ABDOMINAL PAIN,DIARRHOEA

BODY WEIGHT INCREASE DUE TO EDEMA

DECREASE URINE OUTPUT

DIFFICULTY IN BREATING

HOW TO INVESTIGATE

URINE ANALYSIS -PROTEINURIA,24 HRS URINE TEST FOR PROTEIN EXCRETION,SPOT URINE PROTEIN/ CREATININE RATIO AND

CULTUE AND SENSITIVITY

BLOOD TEST - SERUM PROTEIN, CHOLESTROL,ELECTROLYTES ,RFT,ESR,

OTHERS- CHEST X RAY ,USG -KUB, RENAL BIOPSY-NOT RUTINELY INDICATED( IF AGE <1 OR >8 YRS)

TREATMENT

SUPPORTIVE - DIET LOW SALT AND HIGH PROTEIN

DIURETICS (FRUSEMIDE ,HYDROCHLORTHIAZIDE,METOLAZONE ,SPIRONOLACTONE

20% SALT FREE ALBUMIN

SPECIFIC - INITIAL THERAPY - PREDNISOLONE ,CYCLOPHOSPHAMIDE ,CHLORAMBUCIL,CYCLOSPORINE,MICROPHENOLATE MOFETIL,ENDOXAN,TARCOLIMUS,RITUXIMAB

HELP TO TREAT -TREATMENT OF INFREQUENT AND FREQUENT RELAPSE NS

-TREATMENT OF STEROID DEPENDENT NS

-TREATMENT OF STEROID RESISTANCE NS

COMPLICATION

INFECTIONS

HYPERCOAGULABILITY

CARDIOVASCULAR DISEASE

HYPOVOLEMIC SHOCK

GROWTH RETARDATION

  

HEART FAILURE

DEFINITION _SYSTOLOC HEART FAILURE -INABILITY OF THE HEART TO MAINTAIN AN OUTPUT TO MEET THE  METABOLIC NEEDS OF THE BODY .

- DIASTOLIC HEART FAILURE -INABILTY TO RECEIVE THE BLOOD INTO THE VENTRICLES

CAUSES  

VENTRICULAR PUMP DYSFUNCTION

VOLUME OVERLOAD

PRESSURE OVERLOAD (INCREASED AFTERLOAD)

FETUS- ANEMIA,SVT ,VT

PRETERM BABY- FLUID OVERLOAD

INFANTS -CONGENITAL HEART DISEASES

CHILDREN- RHEUMATIC FEVER ,SYSTEMIC HTN

CLASSIFICATION

CLASS 1-MILD =NO SYMPTOMS

CLASS 2-MILD= SLIGHT LIMITATION OF PHYSICAL ACTIVITY

CLASS 3-INTERMEDIATE=LIMITATION OF PHYSICAL ACTIVITY

CLASS 4= SEVERE =SYMPTOMS MAY BE PRESENT AT REST

CLINICAL MANIFESTATION

SYMPTOMS

INFANT-POOR WEIGHT GAIN

POOR FEEDING

SWEATING OF FOREHEAD DURING FEEDING

CHILD-TACHYPNOEA, PERSISTANT COUGH ,WHEEZE

SIGNS

RIGHT SIDE-FACIAL EDEMA ,PEDAL EDEMA,HEPATOMEGALY

LT SIDE HEART FAILURE -INCREASE HR,INCREASE RR, COUGH ,WHEEZE, RALES IN CHEST

BOTH RT AND LT HEART FAILURE- LACK OF WEIGHT GAIN,SMALL VOLUME PULSE,PERIPHERAL CYNOSIS,CARDIAC ENLARGEMENT,GALLOP RHYTHM

PATHOPHYSIOLOGY

MYOCYTES LOSS FIBROSIS

LEADS

HEART FAILURE

STIMULATE

RENIN ANGIOTENSIN SYSTEM

LEADS

INCREASE NA AN H2O

DUE TO

INCREASE INTRAVASCULAR VOLUME

LEADS

INCREASE BLOOD PRESSURE

AND

INCREASE CARDIAC WORLK LOAD

LEADS TO

MYOCYTES LOSS FIBROSIS

INVESTIGATION WHY=

CONFIRM THE DIAGNOSIS -2D ECHO,CHEST X RAY

DETECT THE CAUSE =BLOOD TEST-HB,ABG ANALYSIS,SERUM CALCIUM,TFT

TO KNOW THE COMPLICATION

TREATMENT

REDUCTION IN CARDIAC OVERLOAD -BED REST ,PROPER POSITION

VASODILATORS,ACE INHIBITORS ,BETA -BLOCKERS ,ARBs

INCRESE CARDIAC CONTRACTION- -INOTROPICS(DOPAMINE ,DIGOXIN ,DOBUTAMINE ,EPINEHRINE

PRELOAD REDUCTION- DIURETICS (HYDROCHLOROTHIAZIDE,FRUSEMIDE )

TREATMENT OF THE CAUSES -ANEMIA -BLOOD TRANSFUSION

ANTICOAGULANTS - CARDIOVERSION

CONGENITAL PROBLEM - SURGICAL CLOSURE (PDA ,VSD)