GENERAL DATA G.E.L. male 3 yo Roman catholic Brgy. Dungon C. Mandurrio Iloilo City

Chief Complaint

Puffy eyelids and swelling of both lower extremities

History of Present Illness

6 days PTA- Periorbital swelling of both eyes upon waking up.- regression of swelling was noted in the

afternoon- no other associated signs and symptoms

( fever, tea colored urine, cough, abdominal pain and vomiting)

- No medications given and no consultations was done

1day PTA swelling of the face still persisted.

associated with swelling of both lower extremities from legs to feet, noted in the afternoon.

abdominal distension was also noted

On the Day of Admission still with above signs and symptoms with

abdominal girth of 50 cm Consult was done And was advised for admission

Past Medical History Patient was diagnosed to have Bronchial

Asthma and pneumonia and was given with unrecalled antibiotics. He was discharged after 7 days.

Patient started to experienced on and off tonsillitis at the age of 9 months old until now.

Family History history of DM in paternal side (-) HPN (-) asthma (-) allergies

PERSONAL HISTORY

Prenatal History Pregnancy was planned and wanted Prenatal check up started at 1 and

1/2month AOG at a local hospital No known complications noted

(UTI, DM, and HPN) TT4 Non smoker and non alcoholic drinker experienced ruptured BOW for 4 days

Natal Period Delivered full term via NSVD at cephalic

presentation pinkish and had a good cry Strong motor activity (-) complications and (-) congenital

deformities wt: 2.8 kg

Neonatal patient was treated with unrecalled

antibiotics for 7 days and was discharged in good condition

Nutrition/feeding Patient was exclusively breastfeed up to two

years old. Currently, patient was in enfagrow with 1:2

dilution At 7 months old patient’s mother started

giving solid foods.

Immunization 1 dose of BCG 3 doses of HEP B3 dose of DPT 3 doses of polio1 dose of measles 1 dose of MMR3 doses of Hib vaccine 1 dose of typhoid 1 Rota virus vaccine

Physical Examination Armborne, awake, febrile Vital signs:

CR:108bpmRR: 25cpmTEMP: 37° C

Anthropometric Data:Weight: 19 kg with z score of above + 2 SD defines

normal range

Physical examination: Skin: (+) skin lesions on lower extremities, no

pallor on skin, with good capillary refill HEENT: normocephalic, anicteric sclerae,

pinkish conjunctivae, non hyperemic tonsils CHEST: SCE, (-) rales, (-) wheeze HEART: AP, NCRRR, no murmurs noted

ABDOMEN: globular, distended with 50 cm ab girth, normoactive bowel sounds, non tender

extremities: (+) slight swelling of both lower extremities

Socioeconomic HistoryPatient lives in a congested urban area

House is made of mixed materials, with 3 rooms and 1 comfort room (flushed type) Patient’s source of water is deep well, and for drinking, through mineral refilling stations

OBJECTIVES:

1. Define 2. Clinical Manifestations3. Diagnosis 4. Management and Treatment

N E P H R O T I C S Y N D R O M E

Etiology and Epidemiology A small amount of protein is found in the

urine of healthy children ( < 4 mg/m2/hour or U pr/cr

< 0.2).

Nephrotic proteinura in children protein greater than 40mg/m2/hour or

Proteinuria between these two levels: mildly

to moderately elevated but not nephrotic

Characterized by: Nephrotic range proteinuria - defined as protein excretion of > 40 mg/m2/hr or a first morning protein : creatinine ratio of >2-3 : 1

TRIAD:

hypoproteinemia (serum albumin

< 3.0 g/dL)

hypercholesterolemia (>250

mg/dL)

edema

PATHOGENESIS:

HYPOALBUMINEMIA

Increased hepatocytemetabolism of VLDLand albumin

↑ inhibition of lipoprotein lipase↑ urine loss of Apo C II

↓oncotic pressure

↓intravascular volume

AldosteroneADH

Na and water retention

↑interstitial fluid volume

HYPERCHOLESTEROLEMIA

EDEMA

Fluid shift from vascular to interstitial compartment

Types of Nephrotic Syndrome PRIMARY OR IDIOPATHIC (associated with primary

glomerular disease without evidence of a specific systemic cause.)

- Minimal change nephrotic syndrome (MCNS)

- Focal segmental glomerulosclerosis (FSGS)

- Membranoproliferative glomerulonephritis (MPGN)

- Membranous nephropathy

- Congenital nephrotic syndrome

SECONDARY NEPHROTIC SYNDROME

– Allergic reactions– Diabetes– Amyloidosis– Malignancies– CHF– Constrictive pericarditis

– SLE– Henoch Schonlein purpura– Infections

• Hepatitis B, hepatitis C, malaria

– Wegener and other vasculitides

suspected in patients :- >8 yr - those with hypertension- Hematuria- renal dysfunction- extrarenal symptoms (rash, arthralgias, fever)- depressed serum complement levels.

Minimal Change Nephrotic Syndrome (MCNS)

Most common histologic form of primary NS (85%)

the glomeruli appear normal or show a minimal increase in mesangial cells and matrix.

>80% of children < 7 y/o Children 7-16 y/o : 50% chance of MCNS Male:female 2:1

Mesangial proliferation characterized by a diffuse increase in mesangial

cells and matrix on light microscopy Immunofluorescence microscopy might reveal

trace to 1+ mesangial IgM and/or IgA staining. Electron microscopy reveals increased numbers

of mesangial cells and matrix as well as effacement of the epithelial cell foot processes

Focal Segmental Glomerulosclerosis (FSGS)

10-20% of children with primary NS; 33% progress to renal failure

The lesions consist of mesangial cell proliferation segmental scarring on light microscopy

Immunofluorescence microscopy is positive for IgM and C3 staining in the areas of segmental sclerosis.

Electron microscopy demonstrates segmental scarring of the glomerular tuft with obliteration of the glomerular capillary lumen.

glomeruli show lesions that are both focal (present only in a proportion of glomeruli) and segmental (localized to ≥1 intraglomerular tufts).

Membranous nephropathy Histologic diagnosis <5% of children with primary NS Common in adolescents and children with systemic

infections eg hepatitis B, syphilis, malaria, toxoplasmosis Common in children receiving drugs eg gold salts,

penicillamine

Membranoproliferative glomerulonephritis (MPGN)

Hypocomplementemia with signs of glomerular renal disease

5-15% of children with primary NS have MPGN

Persistent, progress to renal failure

Congenital nephrotic syndrome manifesting at birth or within the first 3 mo of life may be classified as primary or as secondary Finnish type: autosomal recessive disorder due to

mutation in nephrin protein component in glomerular filtrayion slit

Heterogenous group: diffuse mesangial sclerosis;, associated with drugs or infections

Prenatal onset: elevated levels of maternal alpha-fetoprotein

CLINICAL MANIFESTATION Children usually present with mild edema,

which is initially noted around the eyes and in the lower extremities.

Ascites : Anorexia, irritability, abdominal pain, Respiratory distress: pleural effusions,

Pulmonary edema diarrhea: Due to interstitial edema genital edema

DIAGNOSIS urinalysis reveals 3+ or 4+ proteinuria, spot urine protein:creatinine ratio exceeds 2.0 urinary protein excretion exceeds 40 mg/m2/hr The serum albumin level is <2.5 g/dL serum cholesterol and triglyceride levels are

elevated

TREATMENT MCNS: Steroid treatment

- May be initiated even without renal biopsy since >80% of children under 13 y/o are steroid-responsive eg MCNSPrednisone 2 mg/kg/day (60 mg/m2/24 hours), max 60 mg/day x 12 weeks- Indications for renal biopsy

Nonresponders to steroid treatment- Immunotherapy for frequent relapsers or steroid resistant

FSGSNo clear effective therapy35% respond to steroidsOthers respond to immunosuppressive therapy

MPGN and membranous GNChronic steroid or immunosuppressive therapyDo not reliably remit with standard NS steroid therapy

Familial congenital NSAggressive medical therapyEarly nephrectomyDialysistransplantation

Treatment of co-morbidities Edema

Restriction of salt intakeLoop diuretics25% albumin + IV loop diuretic

Albumin is excreted rapidly thus salt restriction and diuretics must be continued

pleural effusiondrainage

Acute hypertensionB blockersCa channel blockers

Persistent hypertensionAngiotensin converting enzyme inhibitors (ACE inhibitors)

Acute Glomerulonephritis

Acute GlomerulonephritisInterpretation:Glomerular disease

urine

• red

Dipstick test

• blood

Acute Glomerulonephritis

are the commonest non suppurative diseases affecting the kidney.

Refer to those diseases that affect the filtering structure of the nephron called the glomerulus

Acute Glomerulonephritis

Acute glomerulonephritis (AGN) refers to a variety of renal diseases characterized by sudden onset of:- Edema- hypertension,- gross or significant microscopic hematuria- proteinuria- oliguria & azotemia

Common ClassificationsAs to etiology

- Post-infectiousPost-streptococcal (PSAGN)Non-post streptococcal (other bacteria, viruses, parasites, fungi)

- Non-infectious

Common Classifications As to clinical manifestations

- PrimaryKidneys are exclusively involvedDisorder that afflict the glomeruli primarily

- SecondaryRenal involvement is attributable to a systemic disease eg Henoch-Schonlein purpura (HSP) or systemic lupus erythematosus (SLE)

Acute Poststreptococcal Glomerulonephritis

This disease is a classic example of the acute nephritic syndrome characterized by :

- Sudden onset of gross hematuria- Edema- Hypertension- Renal insufficiency.

Etiology and Pathogenesis

Acute poststreptococcal glomerulonephritis follows infection of the throat or skin by certain nephritogenic strains of group A ß-hemolytic streptococci.

commonly follows streptococcal pharyngitis during cold weather months

streptococcal skin infections or pyoderma during warm weather months

Strep pharyngitis or impetigo (grp A streptococcus)

Immunologic reaction to bacterial proteins

trapped in glomerula capillary wall

Activate complement

system

Glomerular injury

PSGN

Immune complex mediated disease - Findings IgG and a complement C3 in the GBM.

- rise in the streptococcal enzymes titer and a drop in serum complement system

Typical Clinical Course

Typical Clinical Course Post streptococcal glomerulonephritis is most

common in children aged 5-12 yr and uncommon before the age of 3 yr.

The typical patient develops an acute nephriticsyndrome 1-2 wk after an antecedent

streptococcal pharyngitis or 3-6 wk after a streptococcal pyoderma

Latent period 1-2 weeks post pharyngitis, longer post-pyoderma

Followed by acute nephritic signs Hematuria (dark-brown, rusty, coke-colored, tea-colored urine),edema

Oliguric period 7-10 days

Heralded by signs of salt/fluid retention (edema, hypertension) followed by disturbed kidney function (oliguria, azotemia)

Complications of hypertensive encephalopathy, congestive heart failure, acute renal failure are anticipated

Oliguric period Edema and hematuria are the commonest

presentation. hypertension and oliguria

Oliguric period Edema starts periorbitally, noticeable upon

waking up and it gravitates to lower extremities on prolonged standing.

Is taut but pits in pressure Hypertension is suspected in the presence of

headache, nuchal pain, vomiting or transient visual loss.

Transient anemia- due to hemodilution due to hypervolemia.

Diuretic period

Another 7-10 days Spontaneous voiding Progressive clinical improvement Gross hematuria may persist longer BP normalizes and the child starts

feeling better.

Convalescent period Further 7-10 days Increased well-being All alarming indices seen in the oliguric phase

are gone

Convalescent period The acute phase generally resolves within 6-8

wk. Urinary protein excretion and hypertension

usually normalize by 4-6 wk after onset, Persistent microscopic hematuria may persist

for1-2 yr after the initial presentation

Clinical Manifestations

Post streptococcal glomerulonephritis is most common in children aged 5-12 yr and uncommon before the age of 3 yr.

The typical patient develops an acute nephriticsyndrome 1-2 wk after an antecedent

streptococcal pharyngitis or 3-6 wk after a streptococcal pyoderma

Diagnosis

Urinalysis demonstrates red blood cells (RBCs),frequently in association with RBC casts, proteinuria, and polymorphonuclear leukocytes.

mild normochromic anemia may be present from hemodilution and low-grade hemolysis.

The serumC3 level is usually reduced in the acute phase and returns to normal 6-8 wk after onset

A rising antibody titer to streptococcal antigen(s)confirms a recent streptococcal infection.

Anti streptolysin O titer is commonly elevated after a pharyngeal infection

Anti Dnase B and anti hyaluronidase is elevated in skin infrction like empetigo

Confirmatory Observation/Investigation- The typical clinical course occurring in previously well child is very important diagnostic finding-Bacteriologic demonstration of Grp A B-hemolytic streptococci on throat & skn cultures

THERAPY Supportive measures:- Bed rest and limitation of physical activities- Dietary sodium restriction of 2 gm/day- Fluids are limited to insensible water loss

replacement ( 20 ml/kg/day) plus volume per volume replacement of urine output

TREATMENTantibiotics- Penicillin V 50-100KU/kg/day x10 days- Erythromycindiuresis(Furosemide 1 mkdose)correct hypertension- Nifedipine 0.25-0.5 mkday TID- Captopril0.5-2.0 mkday TID- Sodium nitroprusside 0.5-1.0 mcg/kg/min- Labetolol 0.2-1.0 mkdose- Hydralazine 0.2-0.4 mkdose

END.