PEDIATRICS MCQS, TOACS, PEARLS & UPDATES: FCPS MCQS FOR IMM AND FCPS FINAL PART 2

PEDIATRICS MCQS, TOACS, PEARLS & UPDATES: FCPS MCQS FOR IMM AND FCPS FINAL PART 2: MQC#01 A 4-day-old 26 weeks gestational age preterm infant on a mechanical ventilator developed PDA murmur. The fluid was restricted. How...

FCPS MCQS FOR IMM AND FCPS FINAL PART 2

MQC#01

A 4-day-old 26 weeks gestational age preterm infant on a mechanical ventilator developed PDA murmur. The fluid was restricted. However, the murmur was audible and PDA was confirmed by an echocardiogram.

Indomethacin therapy is contraindicated when:

a) BUN value is 18 mg/dL

b) Platelet count is 55,000

c) Creatinine level is 1.9 mg/dL

d) Necrotizing enterocolitis

e) Urine output is 1.1 mL/Kg/hour

MQC#02

A 6-week-old 26 weeks gestational age preterm infant is on a mechanical ventilator. The ventilator setting is IMV 30, PIP 20, PEEP 5, FiO2 35%, inspiratory time 0.45 second, and flow rate 8 L/minute. ABG reveals pH 7.50, PCO2 59, PO2 80, HCO3 34, base excess +3.5.

Most likely diagnosis:

a) RDS (RD syndrome)

b) Pneumothorax

c) Pulmonary hemorrhage

d) PIE (pulmonary interstitial emphysema)

e) BPD (bronchopulmonary dysplasia)

MQC#03

A pregnant mother is diagnosed with HIV positive. She delivered a 3.4 kg healthy male newborn.

The next step in management:

a) Start zidovudine 6 mg/kg/day divided Q.6.hours, orally for first 6 weeks.

b) Start zidovudine 8 mg/kg/day divided Q.6.hours, orally for first 6 weeks

c) No therapy is indicated.

d) Perform a HIV test for the newborn and wait for the result.

e) Start zidovudine 8 mg/kg/day divided Q.6.hours, orally for first 1 month

MQC#04

The best diagnostic study in HIV infection:

a) HIV RNA PCR

b) HIV DNA PCR

c) HIV culture

d) P 24 antigen

e) IgM anti-HIV

MQC#05

A pregnant mother has been receiving an anticonvulsant medication throughout this pregnancy. The newborn P/W hypertelorism, flattened nose, and hypoplastic nails.

The anticonvulsant medication is:

a) Phenobarbital

b) Carbamazepine

c) Lorazepam

d) Paraldehyde

e) Phenytoin

For answers, please click at 
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ANSWERS of MCQS dated 23/3/2020

MCQ#01
c
Indomethacin is contraindicated in patients with bleeding disorder, NEC, oliguria (less than 1 mL/kg/hr), elevated creatinine level (more than 1.8 mg/dL), and thrombocytopenia (less than 50,000/mm3).


MCQ#02
c


MCQ#03
a



MCQ#04
b
HIV DNA PCR.


MCQ#05
b

PEDIATRICS MCQS, TOACS, PEARLS & UPDATES: Serum-ascites albumin gradient (SAAG)

PEDIATRICS MCQS, TOACS, PEARLS & UPDATES: Serum-ascites albumin gradient (SAAG): S erum-ascites albumin gradient (SAAG) . SAAG = serum albumin–ascites albumin • SAAG value (>1.1 g/dL) indicates ascites from portal...

ABO incompatibility

1. E
2. E.
3. B 


ABO incompatibility is the most likely diagnosis. Mother is group O, baby group A: this is the most common ABO incompatibility. Maternal blood will contain group A antibodies, which cross into the baby and haemolyse red blood cells. Prolonged rupture of membranes may increase the likelihood of neonatal sepsis: however, lack of signs on examination, normal white count, differential and platelet count mitigate against this diagnosis.
Breast milk jaundice is a diagnosis of exclusion only when a well baby is in the phase of prolonged unconjugated jaundice.
Most bilirubin will be unconjugated at this stage. Serum haptoglobin level is not indicated. In a well baby at 32 h of age, urea and electrolyte values are likely to reflect maternal electrolyte levels just before birth and there is no history suggesting that these might have become deranged. Glucose-6-phosphate dehydrogenase deficiency is X-linked co-dominant and less likely to manifest in female babies. Osmotic fragility test is an outmoded test for hereditary spherocytosis.
Double phototherapy and antibiotic cover are appropriate in this case although infection is less likely than ABO incompatibility. The bilirubin rise was steep and contingency plans might be made for an exchange transfusion, but in this baby’s case, ‘double phototherapy’ interrupted the rise in bilirubin and no exchange transfusion was needed.
Adequate hydration must be maintained during phototherapy. While some neonatologists would give oral dextrose, or formula supplementation (after discussion with parents), others might give some intravenous supplementation until breast milk comes in. The fluid prescription proposed here is inappropriate in volume and composition.

Neurology SEQ for those pediatricians who wants to learn PEDIATRIC NEUROLOGY

Neurology SEQ for those pediatricians who wants to learn PEDIATRIC NEUROLOGY

A healthy 9-year-old girl is brought to the emergency room after her parents witnessed her having an unusual event shortly after going to bed. They reported hearing gurgling noises coming from her room. When they found her, she was sitting up in bed and unable to speak. She had jerking of her right lower face with excessive drooling. She appeared awake during the spell. It stopped spontaneously after 2 minutes. She has had a cold but otherwise has been well recently.

General Examination: Normal. 

Neurologic Examination:

Mental Status: Alert and cooperative. 

Language: Fluent without dysarthria. 

Cranial Nerves: II through XII intact. 

Motor: She has normal bulk and tone with 5/5 strength throughout. 

Coordination: There is no dysmetria on finger-to-nose testing. Sensory: No deficits are noted. Gait: She has a normal heel, toe, flat, and tandem gait.

Reflexes: 2+ throughout with bilateral plantar flexor responses.

QUESTIONS:

1. Briefly summarize this case.

2. Localize the examination findings.

3. Give the most likely diagnosis and provide a differential diagnosis.

4. Discuss an appropriate diagnostic work-up.

5. Discuss the management of this patient.

For answers, please click at 

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Answer to questions raised in PEDIATRIC NEUROLOGY SEQ

Answer to questions raised in PEDIATRIC NEUROLOGY SEQ

DIAGNOSIS:

Benign childhood epilepsy with centrotemporal spikes.

Benign childhood epilepsy with centrotemporal spikes (BECTS) or benign rolandic epilepsy is the most common form of idiopathic epilepsy in childhood.

The peak ages of onset are between 5 and 10 years. However, onset may occur in children as young as 3 years or as old as 13 years. Affected children are typically developmentally and intellectually normal. There is a strong genetic predisposition to epilepsy in BECTS. A gene on chromosome 15q14 has been implicated in some families.

The seizures in BECTS are brief and infrequent. Approximately 10% of children will experience only one seizure. In the majority of children (70%), seizures will occur only two to six times. Twenty percent of patients will experience more frequent seizures. In approximately 70% of children, seizures occur only at night. The seizures in BECTS have a characteristic semiology, involving hemifacial clonic movements, speech arrest, dysarthria, and excessive drooling. Preceding paresthesias around the mouth, gums, cheek, or lips may occur. During the seizure, there may be involvement of the ipsilateral arm or leg, as well as secondary generalization.

The seizures usually last 1 to 2 minutes.

The EEG background in BECTS is normal in the awake and sleep states. Epileptiform paroxysms are described as focal, diphasic spike-and-slow-wave activity of medium to high voltage located over the rolandic or centrotemporal regions. Epileptiform discharges may occur either unilaterally or independently and bilaterally. A horizontal dipole with maximum spike negativity over the central and temporal regions with positivity in the frontal regions is also a classic EEG finding.

Characteristically in this disorder, spike-and-wave activity increases in frequency and amplitude during drowsiness and nonrapid eye movement (REM) sleep. Centrotemporal spikes may occur only during sleep in approximately 30% of children.

Spontaneous resolution of seizures by adulthood (18 years of age) occurs in almost all patients with BECTS and, thus, it is given the designation of a “benign” epilepsy. Some academic and behavioral problems have been reported in children with BECTS, but the overall prognosis for intellectual and neurologic outcome is excellent.

The patient is a healthy 9-year-old girl who presents after a simple partial seizure involving right facial jerking, drooling, and speech arrest. Her neurologic exam is normal.

Right facial twitching as well as speech arrest localize to the left centrotemporal, rolandic region.

The patient in this case presents with the classic features of a focal seizure in the setting of BECTS, but the diagnosis of BECTS can be made only when both the clinical history and the EEG findings are consistent with this syndrome. Other localization-related epilepsies caused by underlying structural lesions (trauma, cerebrovascular disease, tumor, neuronal migration abnormalities, mesial temporal sclerosis) should also be considered.

For a patient who presents to the emergency room with a new-onset focal seizure, a head CT scan, as well as basic laboratory work (basic chemistries, CBC, toxicology screen), may be warranted. A referral (inpatient or outpatient) for an awake and sleep EEG is indicated to evaluate for focal epileptiform activity. The diagnosis of BECTS may be assigned only when the clinical history as well as the EEG are consistent.

The role of neuroimaging in a patient with BECTS is controversial. If the clinical

history is consistent and classic bilateral, independent centrotemporal spikeand-

wave activity is found on the EEG, a brain MRI is not always performed.

However, if there are atypical clinical features or the EEG shows unilateral or atypical epileptiform activity, a brain MRI is typically recommended to rule out structural abnormalities.

Treatment with anticonvulsants is often unnecessary in BECTS as seizures are typically infrequent and occur primarily in sleep. However, when seizures are more frequent or occur during wakefulness, they may be disruptive to a child’s quality of life. A variety of anticonvulsants have been used in the treatment of BECTS and most are efficacious. However, carbamazepine, oxcarbazepine, and gabapentin have tolerable side effects and are most commonly used in clinical practice.

PEDIATRICS MCQS, TOACS, PEARLS & UPDATES: Classic Imaging Signs of Congenital Cardiovascular...

PEDIATRICS MCQS, TOACS, PEARLS & UPDATES: Classic Imaging Signs of Congenital Cardiovascular...: Classic Imaging Signs of Congenital Cardiovascular Abnormalities Transposition of the Great Arteries and Egg-on-a-String Sign TAPVR ...

Classic Imaging Signs of Congenital Cardiovascular Abnormalities

Classic Imaging Signs of Congenital Cardiovascular Abnormalities

Transposition of the Great Arteries and Egg-on-a-String Sign

TAPVR and Snowman Sign

Partial Anomalous Pulmonary Venous Return and Scimitar Sign

Tetralogy of Fallot and Boot-shaped Heart

Aortic Coarctation, Figure of Three, and Reverse Figure of Three

Ebstein Anomaly and Box-shaped Heart

Inadequate virilization of male infants (male pseudohermaphroditism)

Inadequate virilization of male infants 

(male pseudohermaphroditism)

This condition is caused by inadequate androgen production or incomplete end-organ response to androgen. 

These patients have a 46,XY karyotype and exclusively testicular tissue and majority of these patients require extensive laboratory investigation before an exact diagnosis can be determined.

1. Decreased androgen production: This can be caused by one of several rare enzyme defects that are inherited (autosomal recessive). Some of these defects also cause cortisol deficiency and nonvirilizing adrenal hyperplasia, and

others are specific to the testosterone pathway. Other causes of decreased androgen production include deficiency of Müllerian-inhibiting substance (the most common presentation is a male infant with inguinal hernias that contain a uterus or fallopian tubes); testicular unresponsiveness to human chorionic gonadotropin (hCG) and luteinizing hormone (LH); and anorchia (absent testes caused by loss of vascular supply to the testis during fetal life). Presence of microphallus/micropenis and hypoglycemia suggests a pituitary deficiency with absence of gonadotropins, ACTH, and/or growth hormone.

2. Decreased end-organ response to androgen: Also referred to as testicular feminization, can be caused by a defect in the androgen receptor or an unknown defect with normal receptors. It can be total (labial testes with otherwise normal appearing female genitalia) or, more commonly, partial (incomplete virilization of a male).

3. 5α-Reductase deficiency: Results in failure of the external genitalia to undergo male differentiation because of the lack of DHT . The outcome is a neonate with a 46,XY karyotype and female or atypical genitalia with internal testes and male ducts.Diagnosis

Laboratory studies:

Normal 46,XY karyotype. 

The differential diagnosis of an incompletely virilized genetic male is extremely complex and includes in utero testicular damage, defects of testosterone synthesis, end-organ resistance, and an enzymatic defect in the conversion of testosterone to DHT. The laboratory evaluation is correspondingly complex and usually proceeds through a number of steps.

a. Testosterone and dihydrotestosterone: 

These hormone levels should be measurable and are higher in newborns than later in childhood. In the male pseudohermaphrodite, testosterone is low in any defect in testosterone production. The testosterone-to-DHT ratio should be between 5:1 and 20:1 when expressed in similar units. A high testosterone-to-DHT ratio suggests 5α-reductase deficiency. Androstenedione levels are measured to diagnose 17-ketosteroid reductase deficiency.

b. Luteinizing hormone and follicle-stimulating hormon: 

These hormones are also higher in infancy than they are in childhood. A diagnosis of gonadotropin deficiency is suspected if these values are low in a reliable assay but can be confirmed in infancy only if there are other pituitary hormone deficits. Note that growth hormone and ACTH deficiency may manifest in the newborn period as hypoglycemia. In primary gonadal defects and some androgen-resistant states, LH and follicle-stimulating hormone are elevated.

c. Human gonadotropin stimulation test: 

Administration of hCG to assess the stimulation of gonadal steroid production when testosterone values are low (as in gonadotropin deficiency or a defect in testosterone synthesis).

Gonadal response to hCG is assessed: a rise in the testosterone level confirms the presence of Leydig cells and, by implication, testicular tissue. 

In patients with 5α-reductase deficiency, the basal testosterone-to-DHT ratio may be normal but elevated after hCG stimulation. 

d. Assessment of pituitary function: 

If gonadotropin deficiency due to impairment of pituitary function is suspected (eg, microphallus/micropenis combined

with hypoglycemia), thyroid function tests, growth hormone levels, ACTH stimulation test, and imaging studies of the pituitary gland may be indicated.

Dexamethasone: Idiopathic Thrombocytopenic Purpura in Children and Adolescents

Indication: 

Children and adolescents (age range, 3 to 17 years) with persistent or chronic refractory ITP.

Dosage and duration: 

Guidelines have provided varied oral dosage regimens for dexamethasone in the management of ITP, including 28 to 40 mg/m2/day, without specifying duration or cyclic high-dose regimens (0.6 mg/kg/day for 4 days every 4 weeks for 6 cycles).

Small prospective trials have documented oral dosage regimens as 20 mg/m2 daily (in 2 divided doses) for 4 days every 4 weeks for a total of 6 cycles/courses or 0.6 mg/kg daily for 4 consecutive days once a month for 6 courses.

Intravenous

Administered as 20 mg/m2 daily for 4 days. A cycle was administered every 15 days for a total of 4 courses.

You can read full article at 

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3839481/

PEDIATRICS MCQS, TOACS, PEARLS & UPDATES: Wilson’s disease: Asymptomatic patients treatment ...

PEDIATRICS MCQS, TOACS, PEARLS & UPDATES: Wilson’s disease: Asymptomatic patients treatment ...: Wilson’s disease Asymptomatic patients treatment protocol: Initial treatment (duration ranges from 6 months to 5 years):  - T...

Wilson’s disease: Asymptomatic patients treatment protocol

Wilson’s disease

Asymptomatic patients treatment protocol:

Initial treatment (duration ranges from 6 months to 5 years): 

- Trientine 750– 1500mg/ day in two or three divided doses; or penicillamine 250– 500mg/ day, increasing by 250mg every 4– 7 days to 1000– 1500mg/ day in two divided doses;

- zinc 50mg tds if chelator not tolerated or declined.

- A djunctive treatment: avoid copper- rich foods (shellfish, nuts, chocolate, mushrooms, organ meats), especially during year 1; pyridoxine 25– 50mg daily (penicillamine inactivates pyridoxine).

-Maintenance (lifelong):

• zinc 50mg tds or lower dose (approximately two- thirds initial dose) of chelating agent.

Pediatric MCQs of the WEEK

Pediatric MCQs of the WEEK

An 8-day-old male infant is brought to clinic for a routine post-delivery evaluation. On examination, the child weighs 6 kg. Multiple pits are present on the posterior helix of the ears. The neonate is excessively jittery. Pregnancy history, labor, and vaginal delivery were unremarkable. The mother states the baby is feeding well.

MCQ#01

Evaluation of which of the following would be most beneficial in an infant with these physical

findings and history?

(A) Calcium

(B) Thyroid

(C) Magnesium

(D) Glucose

(E) Sodium

MCQ#02

Additional family history is obtained and the siblings examined. There is a history of mental

retardation in the family. Some of the siblings are large for their age and have the same lines or pits seen on the posterior helix of their ears.

Which of the following genetic disorders does this family most likely have?

(A) Prader-Willi syndrome

(B) Neurofibromatosis

(C) 22q11 deletion syndrome

(D) Sotos syndrome

(E) Beckwith-Wiedemann syndrome

MCQ#03

Based on your diagnosis, the most likely etiology of this child’s jitteriness and abnormal laboratory study is which of the following?

(A) Hyperinsulinism

(B) Thyrotoxicosis

(C) SIADH

(D) Adrenal insufficiency

(E) Excess growth hormone

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ANSWERS: Pediatric MCQs of the WEEK

ANSWERS: Pediatric MCQs of the WEEK

MCQ#01

Correct Answer: (D) 

Although jitteriness in neonates can result from abnormalities of each of the possible answers, this infant has qualities that make hypoglycemia the most likely cause. The size of the infant is well above the 90th percentile for newborn males. Many causes of macrosomia (eg, previous large-for-gestational age infants and maternal diabetes) are associated with abnormal glucose metabolism. The additional findings of ear anomalies could implicate a calcium abnormality (eg, DiGeorge anomaly/sequence), but the child has no heart murmur and is not failing to thrive at this point, making this a less likely diagnosis.

MCQ#02

Correct Answer: (E) 

There is a well-known association of Beckwith-Wiedemann syndrome (BWS) with hypoglycemia affecting up to 50% of children with the disorder. Physical findings are characterized by macrosomia, microcephaly, visceromegaly, and macroglossia. Other associated anomalies are an increased incidence of hemihypertrophy, omphaloceles, cryptorchidism, and renal tumors. The ear pits or creases are not always present in affected patients but are highly characteristic of BWS. Transmission is often from a sporadic mutation on chromosome 11p15, although autosomal dominant inheritance is also seen. The frequency of occurrence is 1:15,000, with variable expression. Mild to moderate mental deficiency has been reported in this disorder and is thought to be related to neonatal hypoglycemia. The other syndromes are not associated with hypoglycemia.

MCQ#03

Correct Answer: (A) 

The exact etiology of hypoglycemia in a neonate can be difficult to determine and involves a differential that is very different from that seen in an older infant, child, or adult. Small–for-gestational-age infants and premature infants both have increased incidence of symptomatic hypoglycemia. Amajority of their glucose problems are related to deficient glycogen stores, muscle protein, and body fat needed for metabolization to meet energy requirements.

Infants born to diabetic mothers also experience an increased incidence of hypoglycemia. However, hypoglycemia in infants of diabetic mothers is not due to insufficient stores, but is due to hyperinsulinemia and low glucagon levels. Beckwith-Wiedemann syndrome infants also experience hyperinsulinemia which causes hypoglycemia. Their increased insulin secretion is caused by pancreatic islet cell hypertrophy.

Treatment for these infants is the same as for other causes of hyperinsulinism; supportive administration of intravenous glucose at a rate of 6–8 mg/kg/min. At times, more aggressive treatments are warranted (eg, increased rates of glucose administration and supplementation of regulatory hormones by injections of steroids and growth hormone).

Facial Measurements (DYSMORPHOLOGY) for PEDIATRICIANS

Facial Measurements 

Antituberculous Medications and Development of Jaundice/Abnormal Liver Function Tests

IF A CHILD ON ATT HOW TO FOLLOW:

1. CHECK BASELINE LFTs after 2-4 weeks of ATT

    LFTs are normal—Normal LFT no follow up needed.

    LFTs are abnormal—Check LFTs every 2 weekly for 8 weeks.

2. If ALT >2 time UNL ---------------------Check LFT 2 time/weekly till normal.

3. If ALT >3 time UNL With symptoms/ >5 time UNL without symptoms then STOP ATT 

    ( all drugs) & START 2nd LINE ATT—till normalization of LFT(ALT<2 time normal).

4. When LFTs are normalized (normalized ALT <2 time UNL), then RESTART RIFAMPICIN (NOTE: start with low dose of rifampicin then gradually increase over a week)

(LOOK FOR RIFAMPICIN TOLERANCE)

5. AFTER 3-7 DAYS RESTART INH ---------IF SYMPTOM REAPPEAR REMOVE LAST DRUG.

6. IN CASE OF PROLONGED SEVERE HEPATOTOXICITY DO NOT USE PZA BUT ADD OTHER DRUG LIKE CYCLOSERINE, ETHAMBUOL.

7. (NO DERRANGED LFTS) CONTINUE Rx