PEDIATRICS MCQS, TOACS, PEARLS & UPDATES: Pediatric SEQs

PEDIATRICS MCQS, TOACS, PEARLS & UPDATES: Pediatric SEQs: Pediatric SEQs A 12‐year‐old boy presented with obesity. He had been floppy and a poor feeder as an infant with recurrent fits until t...

Pediatric SEQs

Pediatric SEQs

A 12‐year‐old boy presented with obesity. He had been floppy and a poor feeder as an infant with recurrent fits until the age of seven years. On examination, he had developmental delay and special educational needs. His height was on the 50th centile and weight well above the 97th centile.

The following measurements were obtained on a basal blood sample:

Calcium 1.95mmol/L [7.82 mg/dL]

Albumin 41 gL [4.1 g/dL]

Phosphate 1.90mmol/L [5.9 mg/dL]

Creatinine 78μmol/L [0.9 mg/dL]

Alkaline phosphatise 202 IU/L (reference range 100–400)

PTH 14.5pmolL (reference range 0.9–5.5) [138 pg/ml (reference range 10–65)] 25‐OH‐cholecalciferol 16.1 ng/ml (reference range 8–50)

Free T4 11pmol/L (reference range 9.8–23.1) [0.88 ng/dL (reference range 0.8–2.4)]

TSH 5.7mU/L (reference range 0.35–5.5)

Questions and Answers

1.      What do these results demonstrate?

2.      What is the most likely diagnosis?

3.      What additional investigations may clarify the diagnosis?

PEDIATRICS MCQS, TOACS, PEARLS & UPDATES: PEDIATRICS PEARLS: Indications for use of immune g...

PEDIATRICS MCQS, TOACS, PEARLS & UPDATES: PEDIATRICS PEARLS: Indications for use of immune g...: Indications for use of im m une globulin intravenous (IGIV): §   Kawasaki disease §   Acute bacterial or viral infections in immunocom...

PEDIATRICS PEARLS: Indications for use of immune globulin intravenous (IGIV)

Indications for use of im m une globulin intravenous (IGIV):

§  Kawasaki disease

§  Acute bacterial or viral infections in immunocompromised or neutropenic patients.

§  Immune-mediated thrombocytopenia

§  Primary immunodeficiencies

§  Chronic inflammatory demyelinating polyneuropathy

§  Guillain-Barre syndrome

§  HIV infection:

-        Two bacterial infections within 1 year

-        Unable to make antigen -specific antibodies.

-        Hypogammaglobulinemia.

TOACS STATION

TOACS STATION

QUESTIONS:

1.    What is the mode of inheritance?

2.    Name two conditions related to this inheritance

3.    What is the risk of (a) to transmit this disease to their sons and daughters?

PEDIATRICS MCQS, TOACS, PEARLS & UPDATES: PEDIATRICS PEARLS: AGE AND APPEARANCE OF SINUSES

PEDIATRICS MCQS, TOACS, PEARLS & UPDATES: PEDIATRICS PEARLS: AGE AND APPEARANCE OF SINUSES: AGE AND APPEARANCE OF SINUSES At birth: maxillary, anterior and posterior ethmoidal sinuses. They are present in utero. Only ethmoidal...

PEDIATRICS PEARLS: AGE AND APPEARANCE OF SINUSES

AGE AND APPEARANCE OF SINUSES

At birth: maxillary, anterior and posterior ethmoidal sinuses. They are present in utero. Only ethmoidal sinuses are pneumatized at birth.

5-6 years: frontal and maxillary sinuses. They begin to develop by age 1-2 years, but are not seen radiographically until 5-6 years of age. 

Frontal sinuses begin to develop at 7 -8 years of age and completely develop in addescence.

In a 4-year-old girl, all sinuses are present except frontal and sphenoidal. The maxillary sinuses are not pneumatized until 4 years of age.

PEDIATRICS MCQS, TOACS, PEARLS & UPDATES: Pediatrics MCQs

PEDIATRICS MCQS, TOACS, PEARLS & UPDATES: Pediatrics MCQs: MCQS MCQ#01 A 14-year-old boy is diagnosed with diabetes. He is receiving insulin injection. The recommendation of insulin therapy f...

Pediatrics MCQs

MCQS

MCQ#01

A 14-year-old boy is diagnosed with diabetes. He is receiving insulin injection. The recommendation of insulin therapy for a regular exercise:

A. Increase the dose by 10% 

B. Increase the dose by 25% 

C. Increase the dose by 50% 

D. Decrease the dose by 25% 

E. Decrease the dose by 10%

MCQ#02

A 7-year-old girl had tonsillectomy 1 month ago. The true statement after tonsillectomy: 

A. Reduction in nasal allergy

B. Reduction of rheumatic fever

C. Reduction of number of infections 

D. Definitive improvement in nutrition

E. Definitive improvement in respiratory infections.

MCQ#03

The side-effect of rotavirus vaccine: 

A. Flu-like symptoms

B. Hepatitis

C. Intussuception

D. Localized abscess 

E. Mal-rotation

MCQ#04

A 15-month-old healthy boy is due to for his MMR vaccination. His parents are healthy. His paternal uncle has a HIV infection and he lives with them. 

The next step in the management:

A. MMR vaccine should not be given.

B. His uncle should look for another place to live before MMR is given. 

C. Give MMR in 18 months of age.

D. MMR should be given.

E. Isolate the child from his uncle.

MCQ#05

A 7-year-old boy is diagnosed to have a obstructive lung disease. 

All of the following are abnormal except: 

A. Residual volume 

B. Vital capacity

C. Total lung capacity

D. Functional residual capacity

E. Diffusing capacity of carbon monoxide

PEDIATRICS MCQS, TOACS, PEARLS & UPDATES: TOACS STATION

PEDIATRICS MCQS, TOACS, PEARLS & UPDATES: TOACS STATION: TOACS QUESTIONS: 1.    What’s the name of this test? 2.   What’s your next test?

PEDIATRICS MCQS, TOACS, PEARLS & UPDATES: PEDIATRIC PEARLS: How to differentiate pyloric ste...

PEDIATRICS MCQS, TOACS, PEARLS & UPDATES: PEDIATRIC PEARLS: How to differentiate pyloric ste...: How to differentiate pyloric stenosis from adrenal insufficiency?  In pyloric stenosis there is hypochloremic metabolic alkalosis; low ...

PEDIATRIC PEARLS: How to differentiate pyloric stenosis from adrenal insufficiency?

How to differentiate pyloric stenosis from adrenal insufficiency? 

In pyloric stenosis there is hypochloremic metabolic alkalosis; low Na, low cl, normal K (total body K is low); high pH , high CO2, high HCO3 (> 30 mEq/dL).

In adrenal insufficiency, patients have vomiting, but no palpable abdominal mass and there is presence of metabolic acidosis, high serum K, low serum Na, and elevated urinary Na excretion.

TOACS STATION

TOACS

QUESTIONS:

1.  What’s the name of this test?

2.  What’s your next test?

PEDIATRICS MCQS, TOACS, PEARLS & UPDATES: Pediatric MCQs

PEDIATRICS MCQS, TOACS, PEARLS & UPDATES: Pediatric MCQs: MCQS MCQ#01 A newborn CBC reveals hemoglobin (11.0), MCV (106), WBC (12,600), platelet (296,000), and reticulocyte (2%).  Most like...

Pediatric MCQs

MCQS

MCQ#01

A newborn CBC reveals hemoglobin (11.0), MCV (106), WBC (12,600), platelet (296,000), and reticulocyte (2%). 

Most likely diagnosis:

A. Anemia of prematurity 

B. Iron deficiency anemia 

C. G6PD deficiency

D. Normal

E. ‘ABO’ incompatibility and Coombs positive

MCQ#02

A 4-year-old boy appears with severe watery diarrhea and recurrent, vague, crampy mid-abdominal pain for the last 2 days. The boy looks healthy. His mother is unsure of any obvious relation with milk ingestion. Physical examination reveals abdominal distension, flatulance, and borborygmi.

Most likely diagnosis: 

A. Lactase deficiency 

B. Sucrase deficiency

C. Galactase deficiency

D. Sucrase-isomaltase deficiency

E. Glucose-galactose malabsorption

MCQ#03

A pregnant mother took valproic acid in early first trimester. She was not sure about her pregnancy at that time. She is worried. Most likely complication:

A. VSD

B. Limb defects 

C. Spina bifida

D. Hydrops fetalis 

E. Hypoplastic nose

MCQ#04

A 24-week gestational age premature infant (550 grams birth weight) developed BPD (bronchopulmonary dysplasia). She has moderate to severe BPD changes in lungs. At present, the girl is 2 months old and still requiring oxygen by nasal canula. Mother wants to know the usual time of recovery in BPD patients:

A. 1 – 3 months 

B. 3 – 6 months 

C. 6 – 12 months 

D. 12 – 15 months 

E. 15 – 24 months

MCQ#05

All of the following statements are true about pertussis except:

A. Both disease and immunization against pertussis provides complete and lifelong immunity against disease or reinfection.

B. Erythromycin for 14 days is drug of choice.

C. Erythromycin can cause hypertrophic pyloric stenosis.

D. A severely ill infant may appear completely normal between episodes.

E. Prophylactic antibiotic is not recommended for exposed health care workers.

PEDIATRICS MCQS, TOACS, PEARLS & UPDATES: Pediatrics TOP-UPS: BMI and Classifications

PEDIATRICS MCQS, TOACS, PEARLS & UPDATES: Pediatrics TOP-UPS: BMI and Classifications

Pediatrics TOP-UPS: BMI and Classifications

PEDIATRICS MCQS, TOACS, PEARLS & UPDATES: Pediatric TOACS

PEDIATRICS MCQS, TOACS, PEARLS & UPDATES: Pediatric TOACS: TOACS MCQ#01 A newborn male appears with jaundice due to Rh-hemolytic disease. The most common antigen responsible for Rh-hemolyti...

Pediatric TOACS

TOACS

MCQ#01

A newborn male appears with jaundice due to Rh-hemolytic disease. The most common antigen responsible for Rh-hemolytic disease:

A. A 

B. B 

C. C 

D. D 

E. Null

MCQ#02

The best diagnostic study for a congenital toxoplasmosis: 

A.  Urine culture

B.  Blood culture

C.  IgG for toxoplasmosis

D. IgM specific for toxoplasma by ISAGA (immunosorbent agglutination assay) E.  IgM-ELISA (enzyme-linked immunosorbent assay)

MCQ#03

A 12-month-old boy is diagnosed with iron-deficiency anemia. He is drinking cow’s milk. The laboratory findings include all of the following except:

A. Reduced serum ferritin 

B. Reduced MCV

C. Normal TIBC

D. Elevated platelet counts 

E. Normal WBC counts

MCQ#04

A 5-year-old girl went to a clinic for routine check up. CBC test results reveal hemoglobin (12.0), hematocrit (36.0), MCV (55), and reticulocyte count (2%). Most likely diagnosis:

A. Iron deficiency anemia 

B. Folic acid deficiency 

C. Thalassemia major

D. Thalassemia minor 

E.  Normal

MCQ#05

A 12-month-old boy is admitted with meningitis. The boy is treated with third generation cephalosporin. The boys is kept NPO and intravenous fluid is given. After 36 hours, the boy drinks 240 mL of orange juice at a time. About 2 hours later, the boy developed a generalized convulsion for 30 seconds. The serum electrolytes result reveals Na 122, K 4, Cl 92, glucose 76, BUN 4, and creatinine 0.4. Urine specific gravity is 1027. Urine output is 0.2 mL/kg/hour. Most likely diagnosis:

A. Increased ADH secretion 

B. Diabetes insipidus

C. Pseudohyponatremia 

D. Laboratory error

E. Hyponatremic dehydration

PEDIATRICS TOACS

TOACS

MCQ#01

A boy is diagnosed with proximal renal tubular acidosis (RTA) type II. Subsequently he developed rickets.

Most common cause of rickets in this patient

A. Loss of vitamin D in urine

B. Primary hypoparathyroidism

C. Loss of calcium in stool

D. Loss of bicarbonate in stool

E. Phosphaturic hypophosphatemia

MCQ#02

Preferred diagnostic test for DDH (developmental dysplasia of hip) or CDH (congenital dislocation of hip):

A. X-ray hip joints

B. Nuclear bone scan

C. Ultrasonography

D. CT-scan

E. Ortalani test

MCQ#03

A 12-year-old child died suddenly. He was taking which of the following medication:

A. Phenobarbital

B. Phenytoin

C. Tricyclic antidepressant

D. Lorazepam

E. Propranolol

MCQ#04

Most common congenital foot deformity:

A. Talipes equinovarus

B. Developmental dysplasia of hip

C. Metatarsus adductus

D. Metatarsus abductus

E. Wide separation of first and second toes

MCQ#05

Management of a patient younger than 1 year of age with a foreign body aspiration. First to last sequence should be:

A. Abdominal thrusts, back blows, mouth open and remove foreign body.

B. Chest thrusts, back blows, mouth open and remove foreign body.

C. Back blows, chest thrusts, mouth open and remove foreign body.

D. Back blows, abdominal thrusts, mouth open and remove foreign body.

E. Abdominal thrusts, back blows, mouth open and remove foreign body.

PEDIATRICS FCPS MCQS AND TOACS: BENIGN NOCTURNAL PAINS OF CHILDHOOD

PEDIATRICS FCPS MCQS AND TOACS: BENIGN NOCTURNAL PAINS OF CHILDHOOD: BENIGN NOCTURNAL PAINS OF CHILDHOOD Introduction: §   Usually call "Developing PAINS' §   Most common cause of episodic m...

BENIGN NOCTURNAL PAINS OF CHILDHOOD

BENIGN NOCTURNAL PAINS OF CHILDHOOD

Introduction:

§  Usually call "Developing PAINS'

§  Most common cause of episodic musculoskeletal pain in children

§  Peak onset at ages 3 to 12 years

§  Does not really match with development spurt; along these lines the term 'developing pains' is a misnomer.

Clinical presentation:

§  Episodic, bilateral leg pain occurring primarily at night

-        Localized to the calf, shin, behind the knees, or sometimes thights.

-        Responds to massage or over-the-counter analgesics

-        Always disappears by morning 

§  Children are normally active and pain-free during the day in between episodes

-        Pain episodes may occur after days with increased physical activity

-        Motor development is normal 

§  Symptoms usually resolve by late childhood

Diagnosis and evaluation

§  Physical examination is normal

§  Children with flat feet (pes planus) or hypermobility may be predisposed.

§  Radiographs, if obtained, are also normal.

Treatment:

§  Supportive care.

§  Warms baths before bedtime, massage, or over-the-counter analgesics as needed.

§  Education and reassurance that the pains are benign and self-limited.

PEDIATRICS FCPS MCQS AND TOACS: Basic 7 Steps of Short Case

PEDIATRICS FCPS MCQS AND TOACS: Basic 7 Steps of Short Case: Basic 7 Steps of Short Case 1. Approach “Approach” 2. Hand off Exam 3. Hand on Exam 4. Finalization “Structure” 5. Interpre...

Basic 7 Steps of Short Case

Basic 7 Steps of Short Case

1. Approach “Approach”

2. Hand off Exam

3. Hand on Exam

4. Finalization “Structure”

5. Interpretation

6. Presentation

7. Discussion “Formulation”

Remember it is only 9 minutes to show yourself in competition.

Time management is of extreme importance.

Basic 7 Steps of Short Case

1. Approach: Few seconds

§  Greeting, warm smile

§  Introduce yourself

§  Permission

§  Put child at ease

§  Explain what you are going to do

§  Positioning

§  Undress

§  Play and chat with the child.

2. Hand off Exam: 30-60 seconds

§  Observe from end of the bed

§  Show observing skills

§  Count respiratory rate if appropriate

§  Compare both sides.

§  Observe closely; face & head or skin stigma.

§  Don’t miss the mouth, all backs & nappy area.

3. Hand on Exam: Maximum 5 minutes

§  Do all or some of Anthropometric measures in growth & nutrition stations “or offer to measure in other cases”

§  Prepare to touch; ask if anything hearting, warm your hands, go to child level, chat with the child, look to the child’s face.

§  Start away from target area and close gradually

§  Use your hands first, then any tool “Stethoscope, Otoscope, “Warm it”

§  Distract the child or simulate on toy, caregiver or yourself before touch. Mother may help you.

4. Finalization: Few seconds

Say; to complete my examination, I would like to do ….. (Package of offers). This makes your image more colorful during very limited time of examination.

 5. Interpretation: Few seconds

Before you turn to the Examiner, you should reconstruct “in your mind” your findings; positive and negative and formulate a complete bright picture by joining and connecting the important pieces of information together.

6. Presentation: 30-60 seconds

The best way is to divide your presentation in to THREE phases:

A.      Start talking after the first circuit by the child’s name and basic 4 Ds.

B.      Talk through the second circuits “Findings of general examination”. This is best done by running commentary. Mention the positive and important negative findings. “Commonly you may miss important signs you already observed, if you left it to the end”. The Examiner considers that you didn’t notice it even if it was so clear. 

C.      Mention your findings of the third circuit after you complete it. Turn to the Examiner and talk confidently.

D.     Summarize the overall examination and raise your conclusion. “You may say: "all of these finding are consistent with...and I would like to..."

The only exceptions are general and developmental stations. Use running commentary all through stations and use statement of “child demonstrates” rather than “child can do” in developmental stations.

7. Discussion: 1-2 minutes

§  Listen carefully to the examiner’s question and statement.

§  Respond appropriately to the question.

§  Show confident look and clear voice with appropriate body language

§  Don’t dig a black hole to express your extended knowledge

§  Don’t go very deeply in details but, hit an important points as list of headlines.

§  Give a clear diagnosis if you are confident about it.

§  Alternatively, stick to findings and appropriate top priority 2-3 differential diagnosis only related to the patient.

§  Start broadly and focus gradually to specific investigations or treatment

4-D: Disease (Well /unwell), Distress (Count RR), Dimensions (fatness, stature), Dysmorphism (Syndromic..) 

Pediatric TOACS

TOACS

IMMUNIZATION RECOMMENDATION IN HIV

IN RESPECT TO

1.    After exposure to measles

2.    When to receive varicella vaccine

3.    Regarding inactive vaccines

4.    IPV or OPV

PEDIATRICS FCPS MCQS AND TOACS: Pediatrics TOACS:

PEDIATRICS FCPS MCQS AND TOACS: Pediatrics TOACS:: TOACS QUESTIONS: 1.     Identify it. 2.      Enlist its use and efficacy 3.      What blue and yellow beads signify?

Pediatrics TOACS:

TOACS

QUESTIONS:

1.    Identify it.

2.    Enlist its use and efficacy

3.    What blue and yellow beads signify?

Pediatric NEPHROLOGY: How to prepare NEPHROTIC SYNDROME for part II examine.

MANAGEMENT OF CHILDHOOD NEPHROTIC SYNDROME

Useful definitions:

Nephrotic syndrome	Presence of generalized edema, heavy proteinuria and hypoalbuminaemia.  Hypercholesterolemia (>200mg/dL), invariably present, is not conventionally included as a part of the diagnosis.
Heavy proteinuria and hypoalbuminaemia
Remission	Loss of edema and urine protein/creatinine ratio <0.02 or urinary total protein excretion <4 rng/rn2/hr or early morning (first void) urine dipstick negative/trace for 3 consecutive days.
Steroid responsive	Remission achieved with steroid therapy alone.
Relapse	Sustained proteinuria of urine protein/creatinine ratio >0.2 or early morning (first void) urine dipstick ≥2+ for 3 consecutive days (partial biochemical relapse) ± generalized edema + hypoalburnlnaemia (<2.5g/dL (full clinical relapse), having previously been in remission.
Infrequent relapses	Relapses after the first episode, but <2 episodes within first 6 months or <4 episodes within any subsequent one year period.
Frequent relapses	Relapses after the first episode, with ≥2 episodes within first 6 months or ≥4 within any subsequent one-year period.
Steroid dependence	Frequent relapsers with 2 consecutive relapses while on steroid therapy or within 2 weeks of cessation of steroid therapy.
Steroid resistance	Failure to achieve remission despite 8 weeks of daily high dose (60mg/m2/day) prednisolone therapy.

Classification:

A.      Congenital

B.      Acquired

a                  Primary

b                 Secondary

Acquired (Primary)

Subtypes	ISKDC Classification
Minimal change nephrotic syndrome (MCN)	76%
Focal segmental glomerulosclerosis (FSGS)	07%
Focal global sclerosis (FGS)	02%
Mesangial proliferative glomerulonephritis (MPG)	04%
Membranoproliferatlve glomerulonephritis (MPGN)	08%
Membranous nephropathy	02%
Chronic glomerulonephritis	01%

Acquired (Secondary)

A.      Autoimmune disease:  systemic lupus erythematosus and Henoch-Schonlein purpura

B.      Infections: Hepatitis A and B, Human immunodeficiency virus

C.      Hereditary nephritis: Alport syndrome

D.     Drugs: Hevy metal like mercury and penicillamine therapy

E.      Metabolic: Diabetic nephropathy

Clinical evaluation

§  History and clinical signs of systemic involvement:

Pallor

Rash

Petechiae

Arthritis

Hepatosplenomegaly

Hemoptysis

§  Family history of nephrotic syndrome, renal failure and hearing defects,

§  Drug history especially of traditional medicine.

Investigations to evaluate etiology

1. Step 1 (uncomplicated presentation):

§              Full blood count

§              Serum complements (C3, C4)

§              Anti-nuclear antibodies or anti-double-stranded DNA antibodies

§             Hepatitis B surface antigen

2. Step 2 (atypical presentation):

§             Hepatitis C antibody (indicated in nephritic-nephrotic syndrome)

§             ANCA (indicated in nephritic-nephrotic syndrome or evidence of leukocytoclastic vasculitis)

§             C3 nephritic factor (indicated in nephritic-nephrotic syndrome)

§              Serum immunoglobulins (lgG, IgM, IgA)

3. Step 3: Mutational analysis

       Congenital and infantile onset nephrotic syndrome:

•       WT1 exons 8 and 9 (Wilms tumor 1 protein)

•       NPHS1 (nephrin)

•       NPHS2 (podocin)

•       LAMB2 (Iaminin subunit beta-2) (congenital and infantile onset)

•       PLC£1 (phospholipase C, epsilon 1) (congenital and infantile onset)

§               Childhood onset steroid-resistent or steroid-dependent nephrotic syndrome:

•       WT1 exons 8 and 9 (Wilms tumor 1 protein)

•       NPHS1 (nephrin)

•       NPHS2 (podocin)

§              Adult onset FSGS

•       NPHS2 (podocin)

•       TRPC6 (transient receptor potential cation channel C, member 6)

•       ACTN4 (a-actinin4)

•       CD2AP (C02-associated protein)

•       MYH9 (myosin, heavy chain 9, non-muscle)

•       INP2 (inverted formil) 2)

4. Step 4 (steroid-resistant collapsing FSGS):

§             HIV antibody

§             Parvovirus IgM, IgG and PCR

Indications for renal biopsy

1.        Steroid resistance

2.       Age <1 year

3.       Age >10 years if steroid-dependent or resistant

4.      Hypertension

5.       Gross hematuria

6.      Hypocomplementemia

7.       Renal failure

8.      Family history of renal failure and deafness

Treatment

1.        Prednisolone

Indications: First episode of nephrotic syndrome and subsequent relapses.

Protocol

•       Children experiencing their first episode of nephrotic syndrome should be treated with prednisolone at 60 mg/m²/day (maximum of 60 mg/day) for 06 weeks followed by 40 mg/m² of prednisolone every alternate day for 06 weeks and gradual taper over 4 weeks for a total treatment course of 3-6 months.

•       Prednisolone should be given as single morning dose to minimize the side effects.

•       Children with first relapse and infrequent relapses should be treated with prednisolone 60 mg/m² /day (maximum 60 mg) minimum 14 days or until 3 days proteinuria free, then prednisolone 40 mg/m²/EOD for 4 weeks, tapering off over 3 months.

•       Children with frequently relapsing nephrotic syndrome should be placed on maintenance therapy after treatment of relapse, with alternate-day prednisolone 0.1-0.5 mg/kg/EOD for 3 to 6 months.

•       Children with steroid-dependent nephrotic syndrome should be placed on maintenance therapy with prednisolone 0.1-0.5 mg/kg/EOD for 9-12 months.

•       In children on maintenance alternate day prednisolone for 06 months, tapering of prednisolone is recommended, according to the difficulty in attaining remission.

•       If patient has proteinuria ≥2+ for more than 5 days ± having mild/gross edema and on EOD regime, consider increasing prednisolone to daily dose (02mg/kg/day) until 3 days proteinuria free, before reducing to EOD at the same dose, and continue tapering protocol.

•       Note: Prednisolone dose should not be reduced unless the patient has been proteinuria free for the entire period, i.e. negative or trace.

•       Monitor height, weight, blood pressure at each clinic visit.

•       Ophthalmologic screen for posterior subcapsular cataracts should be done at least on a yearly basis for those that have been on ≥6 months of prednisolone therapy.

•       Consider DEXA scan for bone mineral density in children on long-term (>1 year) daily prednisolone. Consider starting on calcium and vitamin D supplements.

 2.       Levamisole

Indications: Frequently relapsing or steroid-dependent nephrotic syndrome with unacceptable steroid side-effects, due to MCNS.

Protocol:

§                 Maintain prednisolone at 60 mg/m²/day until proteinuria tree for 2 weeks.

§                Start levamisole 2.5 mg/kg/EOD for 6-12 months.

§                Wean prednisolone to 40 mg/m²/EOD and taper over 8 weeks.

§                Monitor total white count (TLC) weekly for 1st 4 weeks, then every 2 weeks for the next 12              weeks, then monthly.

§                Main side effect is agranulocytosis.

§               Stop drug if:

•       TLC <3.0 x10⁹/L or absolute neutrophil count (ANC) <1.5 X10⁹/L.

•       Note: neutropenia tends to occur within the 1^st  3 months.

•       Purpuric rash develops (do not re-start).

§               Note: Levamisole tablet= 50 mg (Ketress )

3.       Cyclophosphamide

Indications: Frequently relapsing or steroid-dependent nephrotic syndrome with unacceptable steroid side-effects, due to MCNS.

Protocol:

§               Maintain prednisolone at 60 mg/m²/day (maximum 60 mg) until proteinuria free for at least 3         days. While on cyclophosphamide, the prednisolone dose should not exceed 60 mg.

§            Start cyclophosphamide 2.0-2.5 mg/kg/day preferably when patient is in remission. Continue·          for 8 weeks (frequent relapser) to 12 weeks (steroid-dependent).

§       If patient is steroid-resistant and edematous, ensure good urine output using albumin or              diuretics before starting cyclophosphamide, to prevent hemorrhagic cystitis.

§           Wean prednisolone to 40 mg/m2/EOD and taper off over 8-12 weeks.

§       Monitor total White Count (TLC) weekly for first 04 weeks, then every 02 weeks for next 08     weeks.

§  Stop drug if:

•       Febrile illness.

•       TLC <3000/dL or absolute neutrophil count (ANC) <1500/dL.

•       Platelet count <100 x10⁹/L.

§  Parents and patients should be informed of the potential risks of:

•       Sterility, if the cumulative dose exceeds 250 mg/kg, especially in the post-pubertal patient. Post-pubertal adolescent boys should be advised to consider sperm storage.

•       Malignancy (very low risk, unless the patient is given repeated courses for more than a year).

§  Note: Cyclophosphamide table= 50 mg (Imuran)

4.      Cyclosporine (CsA)

Indications: Steroid-resistant and steroid-dependent patients with MCNS who has failed cytotoxic therapy with alkylating agents such as cyclophosphamide AND with normal renal function. Nephrotic syndrome due to FSGS WITH normal renal function.

Protocol:

§  Check serum urea, creatinine, electrolytes, and liver function test before starting on CsA.

§  Give CsA 3-8 mg/kg/day given as BD dose to attain trough CsA levels at 100-300 mcg/L.

§  Monitor CsA levels and serum creatinine 2x for 1^st  week, then once in 2 weeks for 1^st  month, then 4-6 weekly.

§  If serum creatinine increases by 25% and CsA nephrotoxicity is suspected, reduce CsA dose by 20% to determine if this is the cause.

§  Use diltiazem 15-30 mg 12H to increase CsA levels increasing by 15 mg/dose until a maximum of 3 mg/kg/dose 8-12H (maxirnum dose per day = 60 mg 8H), Effect of diltiazem should be seen by 3 weeks.

§  Precaution: Stop diltiazem if heart rate s 60 beats/min, as diltiazem has been associated with sino-atrial block, Check ECG,

§  Response should be seen in 2 weeks to 1 month. If no response after 2 months, consider adding mycophenolate mofetil or changing to tacrolimus.

§  Taper prednisolone dosage as patient goes into remission.

§  If patient responds to CsA:

§  In MCNS, continue for 1 to 2 years before tapering off the CsA. If patient relapses again, re-start on CsA if they are steroid-dependent, and consider changing to mycophenolate mofetll especially for those with evidence of CsA toxicity on renal biopsy.

§  In FSGS, continue for up to 5 years before considering tapering the dose, and changing to mycophenolate mofetil.

•       Repeat renal biopsy should be considered at the end of 2 years to assess for nephrotoxic effects, especially in those where continuation of CsA beyond 2 years has been planned.

•       Advise on good dental hygiene to prevent gum hyperirophy. Refer to Dental service if there is severe gum hypertrophy.

§  Consider posterior reversible encephalopathy syndrome if patient has neurological symptoms.

§  Drug interactions:

•       Drugs decrease CsA levels: Phenytoin, isoniazid, carbamazepine, phenobarbitone, rifampicin, co-trimoxazole, warfarin.

•       Drugs increase CsA levels: Erythromycin, ketoconazole, cimetidine, methylprednisolone, diltiazem, verapamil, metoclopramide, fluconazole, vancomycin, imipenem, co-trimoxazole, grapefruit and grapefruit juice.

•       Drugs that potentiate nephrotoxicity when used with CsA: Aminoglycosides, amphotericin, acyclovir, co-trimoxazole, Nonsteroidal anti-inflarnmatory drugs (NSAIDs), phenobarbitone, rifampicin, captopril.

•       Other interactions:

Irnipenern: Central nervous systern disturbance including seizures

Digoxin: Increase in Digoxin levels

Furosernide: Decrease nephrotoxicity

HMG CoA reductase inhibitor: Rhabdornyolysis, rnyalgia and muscle weakness

5.       Mycophenolate Mofetil (MPA)

Indications: Patients who are still relapsing while on prednisolone and CsA Alternative drug In those with CsA toxicity,

Protocol:

§  MPA Dose: 600 mg/m²/dose 12H (15-23 mg/kg/dose 12H), Maximum dose:1 gm 12H

§  MPA + CsA Dose: 600 mg/m²/dose 12H (15-23 mg/kg/dose),Maximum dose:1 gm 12H

§  MPA + Tac. Dose: 300 mg/m²/dose 12H (08-12 mg/kg/dose),Maximum dose: 500 mg 12H

§  Dose adjustments required for renal impairment. Drug is not removed by hemodialysis.

§  Monitor full blood count (FBC) in a month and 3-monthly thereafter. Do a serum MPA level in one month. Trough levels range from 2-4 mg/L.

§  Omit if absolute neutrophil count <1.5 x1 0⁹/L.

§  Side effects:

•       Neutropenia (especially at 1-6 months), anemia, thrombocytopenia (rare)

•       Gastritis, peptic ulceration, abdominal colic, diarrhea, gastrointestinal hemorrhage and perforation.

•       Start ranitidine prophylactically at 3 mg/kg/dose nightly (maximum 150 mg). Increase the dose to BD if the patient complains of gastric pain. Alternatively, start omeprazole 1 mg/kg/dose nightly or twice daily (maximum 120 mg three times a day).

§  Drug interactions: Acyclovir and ganciclovir increase MPA levels. Antacids decrease MPA levels. Do not administer drugs simultaneously.

6.      Tacrolimus (Tae)

Indications: Patients who fail to respond within 2 months to GsA ± MPA therapy.

Protocol:

§  Check serum urea, creatinine, electrolytes, and liver function test before starting on Tac.

§  Dose: 0.15 mg/kg 12H, increasing to achieve trough Tac levels of 5-15 mcg/L.

§  Monitor Tac levels and serum creatinine 2x for 1^st week, then once in 2 weeks for 1^st  month, then 4-6 weekly.

§  If serum creatinine increases by 25% and Tae nephrotoxicity is suspected, reduce Tac dose by 20% to determine if this is the cause.

§  Consider using diltiazem to increase Tae levels.

§  Response should be seen in 2 weeks to 1 month. If no response after 2 months, consider adding mycophenolate mofetil.

§  Repeat renal biopsy should be considered at the end of 2 years to assess for nephrotoxic effects, especially in those where continuation of Tae beyond 2 years has been planned.

§  Major side effects include tubulopathy with hypomagnesemia, diabetes mellitus and posterior reversible encephalopathy syndrome.

§  Monitor 3-monthly serum magnesium levels and fasting blood glucose levels. Monitor 6-monthly HbA 1 c levels.

§  Drug interactions:

§  Agents that decrease Tac concentrations:  Phenytoin, isoniazid, carbamazepine, Dexamethasone, phenobarbitone, rifampicin, sodium bicarbonate, aluminum hydroxide.

§  Agents that increase Tac concentrations: Cimetidine, danazol, erythromycin, ketoconazole, methylprednisolone, diltiazem, fluconazole, itraconazol, co-trimoxazole, verapamil, grapefruit and grapefruit juice

7.       Rituximab

Indications: Steroid-dependent and steroid-resistant nephrotic syndrome not responding well to conventional treatment.

Protocol:

§  Pre-infusion Monitoring: FBC, liver function test, urea, creatinine, sodium, potassium, chloride, bicarbonate, calcium, phosphate, uric acid, fasting glucose, triglycerides, total cholesterol, LDL-cholesterol, HDL-cholesterol, serum IgG, IgM, IgA, varicella-zoster IgG, Hepatitis BsAg, 24-hour urinary protein excretion, creatinine clearance, urine microscopy, urine protein/creatinine ratio, serum complements C3, C4, lymphocyte subsets: CD3, CD4, CD8, CO19, CD20, CD25 on CD3

§  Doses:

Steroid-dependent	IV Rituximab 375mg/m2 2-weekly X 02doses
Steroid-resistant	IV Rituximab 375mg/m2 2-weekly X 04doses

§  Pre-medication 30 minutes prior to infusion

•       Paracetamol10-15 mg/kg (maximum 1 g)

•       IV Hydrocortisone 4-10 mg/kg (maximum 500 mg)

•       IV Diphenhydramine 1 mg/kg (maximum 50 mg)

§  IV Rituximab infusion:

•       Start at 50 mg/hr and increase by 50 mg every 30 minutes as tolerated by blood pressure (BP) and heart rate (HR).

•       Maximum infusion rate 400 mg/hour.

•       Monitor heart rate, respiratory rate (RR), blood pressure, temperature, oxygen saturation (Sp02) every 15 minutes for 1^st hour. Then hourly if no problems.

•       If BP falls <100/60 mm Hg, HR >120/minute, Sp02 <95% on room air, stop infusion and recheck. Inform Pediatric Nephrologist on-call immediately.

•       When parameters stabilize, restart infusion at previous tolerated rate.

§  Post-Rituximab Monitoring:

•       Lymphocyte subsets CD3, CD4, CD8, CD19, CD25 on CD3, on D1, D3, then monthly till CD 19 recovery

•       Daily urine protein dipstick monitoring

•       Monthly urine protein/creatinine ratio

•       Monthly 24-hour urine protein excretion until <0.3 g/day/1 .73m2

•       Full blood count on day 1, then weekly for 2 weeks, then monthly Liver function test, serum urea, creatinine, sodium, potassium, chloride, bicarbonate, calcium, phosphate, uric acid rnonthly

•       Fasting triglycerides, total cholesterol, LDL-cholesterol, and HDL-cholesterol 3-monthly

•       Creatinine clearance 3-monthly

§  Complications:

Acute: Monitor patients for onset of cytokine release syndrome characterized by severe dyspnea, often accompanied by bronchospasm and hypoxia, in addition to fever, chills, rigors, urticaria/rash, nausea, vomiting, fatigue, headache, throat irritation, rhinitis, flushing and angioedema. The syndrome usually manifests itself within one to two hours of starting the first infusion.

Stop infusion immediately and give an analgesic/antipyretic, an antihistamine and occasionally oxygen, intravenous saline or bronchodilators and corticosteroids if required.

Resuming infusions after adverse reactions: Infusion can be initially resumed at not more than one-half the previous rate upon complete resolution of all symptoms, normalization of laboratory values and chest x-ray findings. If the same severe adverse reactions occur for a second time, the decision to stop the treatment should seriously be considered.

Chronic:

Progressive multifocal leukoencephalopathy

Interstitial lung disease: Diagnosis should be considered in patients who present with fever, cough and breathlessness, with no clear evidence of infection. Prompt diagnosis and early institution of corticosteroid therapy is essential.

Erythema nodosum.

§  Pneumocystis jirovecii Prophylaxis: Co-trimoxazole oral 3 mg/kg/dose alternate day starting after the first dose of Rituximab, and continuing for 3 months after the last dose of Rituximab. Check G6PD status and exclude in G6PD-deficient patients.

8.      General Management

§  Diet:

•       Children on steroids: Normal calorie, low saturated fats, no refined sugars (10·14% protein, 40-50% poly- and monounsaturated fats, 40-50% carbohydrate).

•       Salt restriction only if edema is present.

•       Fluids as desired up to 1.5x requirem.ents. Restrict to 50% of maintenance if edema present

•       Protein requirements: High protein intake: no evidence of benefit unless there is massive loss preventing growth To avoid malnutrition, increase protein intake to compensate for protein loss.

§  Decrease proteinuria:

•       Angiotensin converting enzyme inhibitor (ACE inhibitor): Start on enalapril 0.1-1.0 mg/kg daily as 12H or 24H dose (maximum 40 mg daily) or ramipril 0.05-0.2 mg/kg once daily (maximum 10 mg). Consider giving the dose nightly if patient's blood pressure is low and patient has dizziness during the day

•       Angiotensin /I receptor blocker: Consider adding losartan 0.6-2.0 mg/kg daily (maximum 100 mg) if the proteinuria is still Significant

•       Aim to decrease proteinuria to at least less than nephrotic range, i.e. <2 g/day1.73m2 or protein : creatinine ratio <0.2 , and to increase serum albumin >30 g/L

•       Consider use of NSAIDs if patient remains nephrotic and preservation of renal function is not an issue

§  Decrease hypercholesterolemia:

•       Dietary restriction as above

•       HMG CoA reductase inhibitors:

-        Lovastatin 0.4-0.8 mg/kg nightly. Dose can be increased monthly, to a maximum of 12H dosing (maximum 40 12H)

-        AtoNastatin 0.2-1.6 mg/kg nightly. Dose can be increased monthly (maximum 80 mg nightly)

-        Lovastatin in combination with calcineurin inhibitors has the lowest risk of rhabdomyolysis Simvastatin has the highest risk of rhabdomyolysis

-        Monitor liver function test and serum creatine kinase monthly for first 3 months then 3-monthly

§  Decrease hypercoagulopathy

•       Avoid hypovolemia

•       Aspirin 3-5 mg/kg (maximum 100 mg) daily or anti-platelet agents such as dipyridamole 1·2 mg/kg (maximum 100 mg) 8H

•       Heparin or warfarin (if there has been a thrombotic event) Note: Stop aspirin, dipyridamole, heparin or warfarin at least one week prior to renal biopsy. Ensure bleeding time, prothrombin time and partial thromboplastin time are normal before proceeding with the procedure.

§  Control of Edema

•       Albumin infusion 20% 1 g/kg over 4 hours followed by IV furosemide 1-2 mg/kg mid-way and at end of infusion'

•       Consider 'chronic diuretic therapy’ in patients with intractable edema, using combination of:

-        Furosemide 1·2 mg/kg OM or BD

-        Spironolactone

-        0-10 kg: 6.25 mg BD

-        11-20 kg: 12.5 mg BD

-        21-40 kg: 25 mg BD

-        >40 kg: 25 mg 8H

•       Bumetanide 25-50 mcg/kg (maximum 3 mg) daily, increasing to 8-12H

Algorithm Management of Steroid-Resistant Nephrotic Syndrome

Algorithm Management of steroid-sensitive Nephrotic Syndrome