NEPHROTIC SYNDROME

• FACILITATOR;F.FURIA
• PRESENTER;ABDALLAH MAURICE
 OUTLINES
• DEFINITION
• EPIDEMIOLOGY
• CAUSES
• TYPES
• CLINICAL FEATURES
• INVESTIGATIONS
• COMPLICATIONS
• MANAGEMENT
 DEFINITION
• Is the loss of 3 grams or more per day of
protein into urine or on a single spot urine
collection,the presence of 2 g of protein per
gram of creatinine. It is characterised by
>Massive proteinuria
>Hypoalbuminemia
>Hyperlipidemia(hypercholerosterelimia)
>oedema
 EPIDEMIOLOGY
• Affects adults and children of any sex and race,although is mainly found in
adults with a ratio of adults to children 26 to 1.Syndrome presents in
different ways between adults and children,in children minimal change
disease(66% of cases),followed by other types.In adults the most common
disease is membranous glomerulonephritis(30-40%) followed by focal
segmental glomeruloslerosis(15-25%) and minimal change disease.
• Disease is more common in men than women by a ratio of 2 to 1.
• Spontaneous remission occurs in up to 20-30% of cases during the first year
of illness,however this improvement is not definitive as some 50-60% of
patients die or develop renal failure 6 to 14 years after this remission.On the
other hand 20-30% of patients have continous episodes of remissions and
relapses without dying or kidney damage.the main cause of death
cardiovascular as a ressults of chronicity of the syndrome and
thromboembolic accidents.
 CAUSES
• Primary causes/Idiopathic>80%-caused by
glomerular diseases intrinsic to the kidney and not
related to systemic causes.Its sub categories is divided
based on histological descriptions,these includes
>Minimal change disease(between 1-10 yrs)
>Focal segmental glomerulosclerosis
>Membraneous nephropathy
>Membranoproliferative glomerulonephritis
>Iga nephropathy
 CAUSES CONT
.Secondary causes
>Autoimmune and vasculitic diseases,henoch schonmlen purpura,systemic lupus
erthematosus.
>Infectioous diseases
-viral:HIV,HBV,HCV
-bacterial:streprococcal
-protozoal:malaria
>drugs:NSAIDS,Ampicillin,penicillamine,gold,captopril,lithium
>Systemic conditions
-autoimmune:vasculitis,SLE,henoch schonmlen purpura
-metabolic:diabetes,amyloidosis
-malginancies:leukemia,lymphoma
-haematologic:SCA
-obesity
• Genetic causes(hereditary)
 TYPES OF NEPHROTIC SYNDROME
• Nephrotic syndrome is divided based on underlying
cause ,ie
>Primary
>secondary
Oftenly is classified based on histologic pattern
>minimal change disease
>focal segmental glomerulosclerosis
>membraneous glomerulonephritis
>membranoploliferative glomerulonephritis
 CLINICAL FEATURES
• Pitting edema in 95 of children
• Respiratory tract infection
• Allergy
• Microhematuria
• Symptoms of
infection,fever,lethargy,irritability or
abdominal pain due to sepsis or peritonitis
• tachypnea
 CLINICAL FEATURES
• Seizures due to cerebral thrombosis
• Diarrhoea
• Fatique
• Hypertension
• anorexia
 INVESTIGATIONS
• Urine evaluation
-dipstick
-Urine protein quantification(24 hr urine
protein/urine protein creatinine ratio
• Serum albumin,cholesterol
.Complete blood count
.serum electrolyteS,calcium,phosphorus,ionized
calcium,BUN,creatinine
 INVESTIGATIONS CONT

• Hepatitis B and C test,HIV

• Kidney ultrasonograpphy
• Chest radiography
• Kidney biopsy
 COMPLICATIONS
• Edema
• Hypertension
• Thrombosis
• Infection
• Acute kidney failure
• Medication adverse effects
• Hypothyroidism
• Hypocalcaemia
• Microcytic hypochromic anaemia
• Protein malnutrition
• Vitamin D defficiency
• Hypovolemia
• Chronic renal failure
 MANAGEMENT
.Supportive management
-Diet: (130-140%) of recommended daily intake
-low salt intake
-fluid restriction
-hypovolemia:monitoring of BP
-Diuretics;massive oedema
-thromboembolism
>heparin and warfarin
-hypertension
>ACEi and ARBs
-osteoporosis
>calcium carbonate and vitamin D
 MANAGEMENT CONT
.High dose steroids
-2mg/kg daily for 6 weeks(max 60)
-1.5mg/kg(max 40) alternating days for 6 weeks but if the patient
attained remission after 4 weeks
.Relapse management
.steroid dependant and frequent relapses
 REFFERENCES

• >Nelson textbook of pediatrics,2 volume set,7th edition,Robert

kliegman,Bonita stanton,Joseph st.Geme,Nina
• >http//emedicine.mediscape.com/article/982920(pediatric
nephrotic syndrome)
• >Goldstein,Adam;Tratchman,Howard(pediatric nephrotic
syndrome)
• >Clardy,Chris(may 2000)nephrotic syndrome in
children,pediatric nephrology handout.
• >Adult nephrotic syndrome-National institute of diabetes and
digestive and kidney diseases(NIDDK)