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Case 1 The child presented with history of cyanosis since birth.

Look at the picture & answer the questions.

1. What is this condition called?

2. In which disease it is seen?

3. How will you treat the attack seen in the picture?


Key

1. Tet Spell (Paroxysmal Hypercyanotic Spell)

2. Tetralogy of Fallot (TOF)

3. Treatment plan is as;

Hospitalization

Place the child in lateral knee-chest position

Oxygen inhalation

Subcutaneous Morphine sulphate

Propranolol

I/V Na-Bicarbonate to correct acidosis

Note 1. Tet spell is a problem during first 2 years of life in TOF. The infant becomes hyperapneic

& restless. Cyanosis increases and grasping respiration occurs. If severe may lead to

Syncope. The spell mostly occurs when child wakes up in the morning or after sleep or

during crying. During the attack, systolic murmur disappears.

2. Cardio-protective shunts (improve oxygenation if along TOF) like ASD, PDA.


Case 2 The child with known case of Tetralogy of Fallot (TOF) developed severe dyspnea &

respiratory rate more than 42/min. On examination, there was marked cyanosis & child was

restless.

1. What is your diagnosis?

2. Give its emergency management.

Key

1. Tet Spell (Paroxysmal Hypercyanotic Spell)

2. The emergency management is;

Hospitalization

Place the child in lateral knee-chest position

Oxygen inhalation

Subcutaneous Morphine sulphate

Propranolol

I/V Na-Bicarbonate to correct acidosis


Case 3

1. Give findings on above x-ray?


3. Give 3 complications of this disease?


Key

1. The findings on x-ray are;

Boot shaped heart (with up tilted apex due to RVH)

Oligemic lung fields (diminished pulmonary vascular markings)

Pulmonary artery bay


3. The complications of this disease are;

Cerebrovascular accident (CVA)

Brain abscess

Bacterial endocarditis

Polycythemia

Relative anemia

Failure to thrive

Psychological problems


Case 4 Picture of child’s hand who presented with history of cyanosis since 12 hours after birth is

shown here;

1. What is obvious findings in above photograph?

2. Give your most likely diagnosis?

3. Name 3 congenital cyanotic heart diseases?


Key

1. Clubbing of fingers (Hippocratic fingers)


3. Congenital cyanotic heart diseases are

Tricuspid atresia

Transposition of great arteries (TGA)

Truncus arteriosus

Pulmonary atresia

Tetralogy of Fallot (TOF)

Note

In Tetralogy of Fallot (TOF);

1. Harsh ejection systolic murmur along left sternal border in 3rd intercostal space

2. Left parasternal heave due to RVH

3. Failure to thrive is there


Case 5

1. Which anomaly is obvious in figure B?

2. Give its two risk factors?

3. Name the murmur heard in this disease?

4. At which site in the precordium, the murmur would be best heard?

Key 1. Patent ductus arteriosus

2. Risk factors are;

Maternal rubella infection

Premature infant

Female sex

3. Continuous (Machinery murmur)

4. Left infra-clavicular area & radiating to the back


Case 6 A 5 month girl is brought to the office by her mother, who states that the girl had an episode

following feeding during which she began to breathe deeply, became blue & then lost

consciousness. The mother states that she picked her up & held her & the infant regained her

usual color & become alert. Physical examination revealed a harsh systolic murmur. The

remainder of physical examination is unremarkable.


2. Enumerate the components of this disease?

3. Name the palliative surgery used in the treatment of this disease?

Key 1. Tetralogy of Fallot (TOF)

2. The features of this disease are;

Pulmonary infundibular stenosis

Ventricular septal defect (VSD)

Overriding aorta

Right ventricular hypertrophy (RVH)

3. Blalock Taussing shunt


Case 7 A 5 years old girl from Okara is brought with a 3 day history of fever & intermittent joint pain.

She is generally healthy but according to her mother, she had a cold about a month ago. On

physical examination, her temperature is 103.2 0F, B.P is 94/60 mmHg, pulse is 114/min &

respiration is 22/min. There is a grade III/6 murmur best heard at the apex. Multiple fine pink

macules are noted on her trunk. The macules are blanching in the middle.


2. What ECG findings will you find in this patient?

3. How will you manage this case?

4. How will you prevent this disease?

Key 1. Acute rheumatic fever

2. Reversible prolongation of R-R interval

3.

Management of Disease

General measures Strict bed rest Salt restriction

Medical measures Salicylates Benzathine penicillin Corticosteroids

4.

Prevention of Disease Primary prophylaxis Treat streptococcal pharyngitis by

benzathine penicillin for 10 days Secondary prophylaxis

Prevent recurrence by continuous chemoprophylaxis


Case 8 A 4 months old boy gets cyanosed on crying. Examination revealed single soft 2nd heart sound

and an ejection systolic murmur close to left upper sternal border. X-ray chest reveals reduced

vascular shadows in the lungs bilaterally.

1. What does the heart sound & murmur indicate?

2. What is most likely diagnosis?

3. How will you confirm your diagnosis?

Key

1. These indicate pulmonary stenosis as 2nd heart sound is soft & there is ejection systolic

murmur.


3.

Confirmation of Diagnosis Blood count Hb RBCs

Chest X-ray Boot shaped heart Oligemic lung fields

ECG Right axis deviation & RVH

Echocardiography RVH, pulmonary stenosis, VSD, overriding aorta


Case 9 A 7 days old pre-term baby is admitted in the nursery. He is receiving intravenous fluids. Being

previously well, he developed bilateral fine crepitation and hepatomegaly. A continuous

machinery murmur is heard at the left upper sternal margin.

1. What is most likely diagnosis?

2. Give one non-invasive investigation to confirm the diagnosis?

3. Enlist three management steps?

Key

1. Patent ductus arteriosus (PDA) / large one

2. Trans-thoracic echocardiography

3.

Steps of Management Medical measures to close the duct Indomethacin (0.2 mg/kg IV 3 doses every 8-

12 hours) RBCs increased

Treat Cardiac Failure Restrict fluids Diuretics (furosemide)

Surgery 1 year of age using Coil or occlusion device via cardiac catheter If < 1 year, ligation and division

Note Choice of surgery;

Small PDA: close to prevent endocarditis

Moderate PDA: close to prevent CCF & pulmonary disease


Case 10 A 2 years old boy is brought into the emergency room with complaint of fever for 6 days &

the development of the limp. On examination, he is found to have an erythematous macular

exanthema over his body as shown in the image. Bilateral conjunctivitis, dry & cracked lips, a

red throat & cervical lymphadenopathy. There is grade 2/6 vibratory ejection murmur at the

lower left sternal border. WBCs & differential show predominant neutrophils with increased

platelets. He later developed desquamation of skin of palms & sole around the finger tips with

edema.


2. Give the diagnostic criteria of this disease?

3. How will you manage this case?


Key 1. Kawasaki disease

2. Fever for at least 5 days + 4 of following 5 criteria

Steps of Management

Mucosal involvement Dry cracked lips, strawberry tongue, injected pharynx

Hands & Feet Edema, desquamation, redness

Eye Non-purulent bilateral conjunctivitis

Adenopathy Unilateral cervical lymphadenopathy > 1.5 cm

Rash Polymorphous truncal erythematous scarlitiform rash

3.

Supportive

Take blood culture to rule out other bacterial infections

IV gamma globulin high dose

High dose aspirin until platelet count start to decrease

Long term aspirin + Dipyridamole for coronary abnormalities

If myocardial infarction or ischemia present, do early cardiac catheterization & bypass surgery

Note 1. Kawasaki disease is the vasculitis of all blood vessels especially coronary arteries.

2. Diagnostic criteria is remembered with pneumonic My HEART

Mucosal involvement

Hands & Feet

Eyes

Adenopathy

Rash

Temperature

3. Complications include myocarditis, pericarditis, coronary aneurysm & coronary vasculitis

4. Cause of death is rupture of coronary aneurysm & myocardial infarction due to vasculitis

5. ESR, WBC & Platelets es while Hb on blood work up.

6. Echocardiography shows coronary aneurysm


Case 11 A 6 years old boy presents with colicky abdominal pain, bloody diarrhea & pain in the knee

joints. History reveals upper respiratory tract infection one week ago. On examination, a

maculopapular, non-blanching rash was observed on the legs as shown here.


2. Which renal complication can develop in this child?


Key

1. Henoch Schönlein Purpura

2. Following renal complication may occur;

Glomerulonephritis / IgA nephropathy

Nephrotic syndrome

Note 1. IgA forms in response to infections (upper respiratory) etc. & gets deposited in small

blood vessels & kidneys leading to vasculitis & glomerulonephritis.

2. Involvement of GIT blood vessels leads to colicky pain & bloody diarrhea & sometimes

intussusception.

3. In adults, skin & joint involvement and severe kidney diseases are more likely but GIT

symptoms are less. Also myocardial involvement is more common in adults.

4. Skin biopsy will show leckocytoclastic vasculitis with IgA and C3.

5. Palpable purpura is the most common and earliest feature. Later petechial lesions,

cutaneous nodules, sub-cutaneous edema and arthritis.


Case 12


2. What is genetic basis of this disease?

3. Which malignancy is predisposed if a child has this disease?


Key

1. Down’s syndrome

2. Genetic basis of this disease is;

Meiotic non-disjunction (95%): Trisomy 21

Robertsonian translocation (4%)

Mosaicism due to meiotic non-disjunction during embryogenesis (1%)

3. Leukemia especially acute lymphocytic leukemia (ALL)

Note

Down syndrome may lead to following in the later life;

Hearing loss

Visual impairment

Leukemia

Atlantoaxial instability

Hypothyroidism

Celiac disease

Epilepsy

Alzheimer’s disease


Case 13 A 28 years old mother gave birth to a child. On physical examination, the child is alert and

moves all extremities well but is hypotonic. Abdominal examination shows umbilical hernia.

Karyotype is shown here;


2. Give 5 physical clinical features of this disease?

3. Name the abnormalities of other organs most likely found in this disease?


Key

1. Down syndrome

2. Clinical features in this disease are;

Hypotonia

Epicanthic folds

Upslanted palpebral fissures

Brush field spots in iris

Flat face

Protruded tongue

Small ears

Flat occiput

Simian crease & incurved 5th finger

Wide scandal gap b/w 1st & 2nd

toes.

Short neck with excessive skin fold

3.

Abnormalities in Other Organs Heart (40%) Endocardial cushion defects

VSD Tetralogy of Fallot (TOF)

Intestine Duodenal atresia Hirschsprung disease

Endocrine Hypothyroidism

Eye Cataract Strabismus

Ears Hearing loss


Case 14 At the time of delivery, a woman is noted to have a large volume of amniotic fluid. At 6

hours of age, her baby begins regurgitating small amount of mucous & bile stained fluid.

Abdominal x-ray is shown here;


2. Which syndrome is associated with this disorder?

3. Give two pre-natal screening methods to identify this syndrome during gestational

period?

Key 1. Duodenal atresia

2. Down syndrome

3. Pre-natal screening methods

Pre-natal Screening Methods

Chromosomal Analysis Amniocentesis Chorionic villi sampling & FISH

Ultrasound (1st trimester) Shows increased nuchal translucency


Case 14

1. Give findings in above photograph?


3. What is most common genetic abnormality found in this disease?

Key 1. Following findings are there;

Flat nasal bridge

Up slanting palpebral fissure

Lower set ears

Short & broad hands

Single transverse palmer crease (Simian crease)

Epicanthic folds

2. Down syndrome

3. Meiotic non-disjunction leading to trisomy 21


Case 15 A mother brings her two & half week infant to you and tells that he sleeps most of the day

and he does not feed well. On physical examination, the infant has normal weight and length

but has enlarged head and enlarged tongue. Heart rate is 70 beats per minute. The child is

still jaundiced and has wide anterior and posterior fontanels, distended abdomen and

umbilical hernia.


2. How will you investigate?

3. How will you treat this child?

Key 1. Congenital hypothyroidism (Cretinism)

2. Investigation are in the table;

Investigations Screening Thyroid functions tests

TSH increased T4 decreased

Imaging X-ray Long Bones Absence of epiphyseal center Epiphyseal dysgenesis Large fontanelle Wormian bone

Thyroid scan Difference b/w agenesis & ectopic thyroid

Endocrine Hypothyroidism

ECG Decreased P, T & QRS

EEG Low voltage

3.

Lifelong thyroxine replacement

Use T4 only (Sodium L-Thyroxine)

Single daily dose

Neonate: 10-15 ug/kg/day

Child: 4 ug/kg/day


Case 17 This child was born at 42nd weeks of gestation and presented with acrocyanosis and

prolonged jaundiced after birth.


2. Give etiology of this disease?

3. What do you mean by Pendred syndrome?


Key

1. Congenital hypothyroidism (Cretinism)

2.

Dysgenesis or aplasia of thyroid gland

Dyshormonogenesis (Autosomal recessive)

Ectopic thyroid gland

Iodine deficiency (Endemic goiter)

End organ resistance to thyroxine

Trans-placental passage of drugs

Use of propylthiouracil during pregnancy

Maternal autoimmune thyroid disease

3. It is an autosomal thyroid dyshormonogenesis defect having defect in Organification

and is associated with Sensorineural hearing loss.

Note For congenital hypothyroidism;

1. History: Prolonged jaundice or poor feeding

2. Symptoms: Lethargy and constipation

3. Examination: Larger anterior & posterior fontanelle, umbilical hernia

4. Commonest enzyme affected in dyshormonogenesis is Thyroid Peroxidase.


Case 18 This child was presented with constipation. History revealed poor feeding and prolonged

physiological jaundice after birth.


2. Name the drug used to treat this disease?

3. How this drug is given to child?

4. Give two important complications in untreated baby?

Key 1. Congenital hypothyroidism (Cretinism)

2. L-thyroxine tablet

3. After adjusting the dose, the tablet is crushed & given to child in small amount of water

or milk.

4.

Mental retardation

Impaired physical growth

Epiphyseal dysgenesis

Short stature


Case 19


2. Name two diseases which might be associated with it?

3. What are its possible complications?

Key 1. Umbilical hernia

2.

Congenital hypothyroidism

Down syndrome

Mucopolysaccharide storage disease

Beckwith Wiedemann syndrome

3.

Obstruction of bowel loops

Strangulation

Ischemia

Necrosis

Sepsis

Hemorrhage


Case 20 A 15 years old girl is referred for evaluation of short stature. Her bowel habits are not

satisfactory and she has constipation off and on. She is not performing well in school.

Menarche has not yet started. Weight is 30 kg & height is 135 cm. Her neck seems short as

her low hairline posteriorly.

1. What other three clinical findings you will look for in this girl?

2. What is your clinical diagnosis?

3. Enlist three investigations you will carry out to confirm your diagnosis?

Key 1.

Webbed neck and redundant skin on nape of neck

Shield check and widely spaced nipples

Cubitus valgus (increased carrying angle)

Multiple pigmented nevi

Signs of coarctation of aorta (Radio-femoral delay, notching of ribs)

Streaked ovaries on ultrasound

2. Turner syndrome

3.

Hormonal levels (FSH, LH, TSH) FSH raised

Chromosomal analysis (Karyotyping)

X-ray wrist (Bone age = chronological age)

Echocardiography (for coarctation of aorta)

Pelvic ultrasound scan (to see uterus etc.)


Case 21 A 12 years old girl who has short stature and delayed puberty presents to your clinic. After

doing physical examination, tests were done to identify the karyotype which is shown here;


2. What are the chromosomal abnormalities in this karyotype?

3. Give five features of this disease?


Key 1. Turner syndrome

2. X0

3.

Short stature

Low posterior hairline

Webbed neck

Shield chest

Widely spaced nipples

Cubitus valgus (increased carrying angle)

Swelling of hands and feet (Lymphedema)

Steaked ovaries

Multiple pigmented nevi

Signs of Coarctation of aorta (Radio-femoral delay, notching of ribs)

Redundant skin at the nape of the neck

Renal abnormalities (Horse shoe kidneys)


Case 22

1. Name the apparatus shown in the photograph?

2. Give indications for its use?

3. Give three complications of this procedure?


Key

1. Phototherapy machine

2.

Neonatal Pathological Unconjugated Hyperbilirubinemia

Prophylactically for low birth weight babies & severely bruised babies

In hemolytic disease of newborn

3.

Loose stools

Erythematous macular rash

Hyperthermia

Dehydration

Bronze baby syndrome

Retinal damage

Masking of cyanosis


Case 23

1. Name the apparatus shown in the photograph?

2. What its mechanism of action?

3. At which level of indirect bilirubin, this procedure is recommended?

4. What do you mean by Breast Milk jaundice?


Key 1. Phototherapy machine

2. Blue light (425-475 nm) is used which;

Converts unconjugated bilirubin into water soluble Lumirubin excreted in urine

Converts 4Z and 15Z unconjugated bilirubin by E-isomerization into water soluble

compounds.

3.

Term infants: 16-18 mg/dl

Pre-term: at somewhat low level

4. Inhibitors of bilirubin conjugation (glucuronidase, pregnanediol, free fatty acids, and

steroids) are present in some breast milk and it causes unconjugated

hyperbilirubinemia during second week of life.

Note 1. 1 g Hb produces = 34 mg of Bilirubin

2. Physical evidence of jaundice becomes visible when bilirubin = (5-10) mg/dl

Physiological Jaundice Pathological Jaundice

Appears on 2nd to 3rd day 1st day Peak bilirubin Term: 10-12 mg/dl Pre-term:15 mg/dl

Greater

Rate of bilirubin < 5 mg/dl per day

> 5 mg/dl per day

3. Kernicterus occurs if bilirubin levels are;

Term > 25 mg/dl

Pre-term, sepsis, hemolysis, asphyxia, hypoglycemia, sulfa drugs: < 20 mg/dl


Case 24

1. Which Neonatal reflex is being elicited in this picture?

2. Name different components of this reflex?

Key 1. Moro Reflex

2. Different components are;

Abduction

Extension

Adduction

Flexion

Opening of hands

Note 1. Moro reflex is obtained after 28 weeks of life

2. It disappears after 4-6 months

3. If Absent: Sedation, asphyxia, hypotonia, < 28 weeks delivery

4. If asymmetrical: Erb’s paralysis

5. If exaggerated: Cerebral palsy/irritability


Case 25

1. Identity the instrument?

2. What is it use?

Key

1. Harpenden infantometer

2. To measure the length of an infant


Case 26

1. Give the positive findings in the above photograph of an infant?

2. How would you differentiate it from Caput succedaneum?

3. Should it be drained?

Key 1. Cephalhematoma

2.

Cephalhematoma Caput succedaneum

Sub-periosteal hematoma Bruising and edema of presenting part

Confined to sutures lines & one part of the skull Irregular, not confined

3. Drainage is contra-indicated as it may lead to infection.

Note Causes of Cephalhematoma: Birth injury, coagulation and platelet disorder

Complications of Cephalhematoma: Anemia, jaundice, kernicterus


Case 27

1. Identify the obvious findings in this photograph?

2. Which vitamins deficiency leads to this condition?

Key 1. Angular Stomatitis

2.

Vitamin B2 (Riboflavin)

Vitamin B6 (Pyridoxine)


Case 28

1. Give findings in this photograph?


3. X-ray of which part of the body is very helpful in establishing diagnosis in this disease?

Key

1. Rachitic Rosary (beading of ribs, prominent costo-chondral junction)

2. Rickets

3. Wrist x-ray


Case 29

1. Give findings in this photograph?


3. Give two complications of this disease?

Key 1. Harrison’s sulcus (depression above the sub-costal margin at the site of attachment of

diaphragm)

2. Rickets

3.

Respiratory infections (bronchitis, bronchopneumonia)

Pulmonary atelectasis

Anemia


Case 30

6 years old boy with the history of frequent admission due to cough and breathing difficulty.

1. Name the abnormality obvious in this photograph?

2. Name two diseases in which this abnormality can be seen?

Key 1. Harrison’s sulcus (depression above the sub-costal margin at the site of attachment of

diaphragm)

2.

Rickets

Asthma


Case 31


2. Give x-ray findings?

3. Name important investigations for this disease?

Key 1. Rickets

2. X-ray of wrist showing;

Cupping

Widening

Fraying

Flaring

3. Important investigations are;

Serum calcium

Serum phosphate

Plasma alkaline phosphate

25-hydroxyvitamin D (< 20 ng)

of distal ulna and ulna


Case 32

1. Identify the disease?

2. Which nutrient is deficient in this disease?

3. Name one skin lesion found in this disease?

Key 1. Kwashiorkor

2. Protein deficiency

3. Skin lesions found are;

Flaky paint dermatitis

Ulcers

Signs of vitamin deficiency

Hypo or hyperpigmentation of covered areas


Case 33

Case 35


Case 36

Case 37


Case 38

Case 39


Case 40

Case 41


Case 42

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Case 44


Case 44

Case 45


Case 46

Case 47


Case 48

Case 49


Case 50

Case 51


Case 50

Case 52

OSPE Peads Medicine SurgicoMed.com OSPE … Peads Medicine SurgicoMed.com OSPE PEADS ... Nephrotic...

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