Hyperosmolar non-ketotic coma

Criteria to diagnose Hyperosmolar non-ketotic coma (Hyperglycemic hyperosmolar state; HHS)

1. Plasma glucose concentration >33.3 mmol/L (600 mg/dL)
2. Arterial pH >7.30
3. Serum bicarbonate >15 mmol/L
4. Small ketonuria, absent to mild ketonemia
5. Effective serum osmolality >320 mOsm/kg
6. Clinically : Stupor or coma, severe dehydration

Disproportionate Short Stature:

A. With short limbs e.g.

- Achondroplasia

- Rickets

- Osteogenesis imperfecta

B. With short trunk e.g.

- Skeletal dysplasia e.g. Ectodermal dysplasia and Morquio s syndrome

- Fanconi anemia

Diagnostic triad of Wilson Disease: 
- Serum ceruloplasmin (< 20 mg/dL).
- Urinary copper excretion after loading dose of D-penicillamine
- Liver biopsy: hepatic copper accumulation.

Rheumatic Fever: Arthritis Treatment

Treatment of Migratory Arthritis in Rheumatic fever:

1.       Bed rest for 2 weeks

2.       Anti-inflammatory drug: Salicylates

a.      Dose: 50-70 mg/kg/day (max = 6 gram /day) For 3-5 days

b.      Then 50 mg/kg/d for 3 weeks.

c.       Then gradual withdrawal monitored by decline in ESR & CRP

d.      Side effects: Gastritis, GI bleeding, Reye syndrome

3.      Daily examination is vital to pick carditis that can present within 2 weeks of the onset

Pediatric Endocrinology

A 3-year-old girl is brought to the emergency department after her parents had found that she had difficulty in waking this morning. She has been ill with an upper respiratory tract infection for the past 2 days, so has not been eating very well. She did not eat any of her dinner yesterday. Her past medical history is significant for short stature and slow weight gain. She is not on any medication. She is sleepy and difficult to arouse. The physical examination findings are otherwise unremarkable. A finger-stick glucose level is 36 mg/dL (2.0 mmol/L). Blood is drawn at the time of hypoglycemia. She is treated with 1 g/kg of intravenous dextrose, after which she becomes alert and talkative.

Laboratory results are shown:

Laboratory test Result

Blood

Glucose:                            32 mg/dL (1.8 mmol/L)

β-Hydroxybutyric acid:       10 mg/dL (1720 μmol/L) 

                                        (Reference range, fasting<4.2 mg/dL [403 μmol/L])

Fatty acids (nonesterified): 80 mg/dL (2.8 mmol/L) 

                                        (Reference range, 17-42mg/dL [0.60-1.49 mmol/L])

Cortisol:                           8 μg/dL (772 nmol/L) 

                                (Reference range, AM 6.2-19.4 μg/dL [171-535 nmol/L])

Growth hormone:      14 ng/mL (14 μg/L) 

                                (Reference range, > 10 ng/mL [10 μg/L] with stimulation)

Lactate:                    10.8 mg/dL (1.2 mmol/L) (Reference range, 4.5-20 mg/dL [0.5-2.2 mmol/L])

Toxicology screen:                                    Negative

Urine

Urinalysis:               Large amount of ketones present

Organic acids:         Normal

Of the following, the MOST likely diagnosis is

A.     Fatty acid oxidation disorder

B.      Glycogen storage disease

C.      Hyperinsulinism

D.     Ketotic hypoglycemia

E.   Dawn phenomena 

Answer on 27/03/2019

PEDIATRIC MCQS: NEUROLOGY

PEDIATRIC NEUROLOGY

Question # 01:

A 12-year-old female is brought to the ED because she has altered consciousness, headache and fever of 102⁰F for 2 days. You examined her and you found no meningeal signs, but she requires repeated tactile stimulation to maintain arousal, she is not oriented except to person, makes frequent paraphasic errors, and can recall just one of three objects after a distracting task. The rest of the neurologic examination is unremarkable.

Lumbar puncture was done and CSF analysis shows a red blood cell count of 25 cells/hpf; white blood cell count of 120 cells/hpf with a lymphocytic predominance; glucose of 70 mg/dL; and protein of 95 mg/dL.

What is the best treatment to be initiated at this time?

A. Iduroxidine

B. Ceftriaxone

C. Vidarabine

D. Solumedrol

E. Acyclovir

Question # 02:

A 2 days old full-term infant has been evaluated for irritability and microcephaly. He is a product of uncomplicated pregnancy, labor and delivery for a healthy mother. Head ultrasonography and CT scan showed enlarged posterior horns of the lateral ventricles and punctuate calcifications which are both periventricular and diffuse. On neurological examination, he has increased tone and hyperreflexia. A dilated funduscopic exam shows a large area of absent retina with hyperpigmented margins.

What is the most likely diagnosis?

A. Congenital cytomegalovirus infection

B. Congenital toxoplasmosis

C. Congenital herpes simplex type 1

D. Congenital rubella

E. Congenital syphilis

Question # 03:

You have been asked to see a 5-dayold baby girl in the NICU for possible seizures. She was small for gestational age and born at 32 weeks gestation. On day 2 of life, she has had feeding difficulties and has received phototherapy for jaundice. On your exam, she is micro cephalic, has hepatosplenomegaly, and a purpuric rash.

You ordered head ultrasound which shows ventriculomegaly. Her blood labs elevated aminotransferases, direct and indirect hyperbilirubinemia, thrombocytopenia, and hemolytic anemia.

Which test is most likely to confirm this patient’s diagnosis?

A. Bone marrow biopsy

B. Head CT scan

C. Ophthalmologic examination

D. Rapid plasma reagin (RPR) test

E. Urine CMV culture

Question # 04:

A 13-year-old female came to the office with 2 days history of right facial weakness and she is unable to close her right eye. She has trouble sucking from a straw and also, she has drooling from her right side of the mouth. She denies paresthesias, ear pain or hyperacusis. On skin examination you note the following:

The most likely infectious agent causing the above symptoms is:

A. Herpes Simplex Virus Type 1

B. Borrelia burgdorferi

C. Rickettsia rickettsii

D. Mycoplasma pneumonia

E. Tuberculosis 

Question # 05:

The best treatment option for the patient in question # 04 is:

A. Acyclovir

B. Forscarnet

C. Doxycycline

D. Azithromycin

E. Fluconazole

CHECK ANSWERS TOMORROW AT 10 AM PLEASE

PEDIATRIC VACCINATIONS

QUESTION # 01

Vaccine that contains components of the organism is:

A. Tetanus

B. Diptheria

C. Acellular pertussis

D. Hepatitis B vaccine

E. Subunit influenza vaccine

QUESTION # 02

Vaccine that needs least number of booster doses is:

A. MMR

B. Hepatitis B

C. DTaP

D. Hib

E. IPV

QUESTION # 03

The contraindication to give DTaP vaccine is:

A. Family history seizures

B. Cerebral palsy

C. Family history SIDS

D. Family history of an adverse events after DTP or DTaP administration.

E. Encephalopathy within 7 days of administration of previous dose of DTP or DTaP.

QUESTION # 04

The following conditions are not contraindications to DTaP vaccinations except:

A. Temperature of less than 40.5°C, fussiness or mild drowsiness after a previous dose of DTP or DTaP.

B. Well-controlled convulsion

C. Developmental delay

D. Progressive encephalopathy

E. Family history of SIDS

QUESTION # 05

A child has meningococcal meningitis. 

Prophylaxis should not be given to the following category:

A. All medical personnel in that unit.

B. Household members.

C. Daycare and nursery school contacts.

D. Persons who had contact with patient’s oral secretions during the 7 days before the onset of illness.

E. Physician who intubated the child.

QUESTION # 06

A child has meningococcal meningitis. One of the sibling developed fever without UPPER RESPIRATORY TRACT INFECTION symptoms. 

The following statement is true about management of the sibling:

A. Sibling should receive acetaminophen therapy for fever.

B. Reassurance.

C. Sibling should be evaluated in the clinic next day.

D. Sibling should receive ibuprofen therapy for fever.

E. Meningococcal meningitis should be ruled out.

QUESTION # 07

The following statement is not true about the prophylaxis of meningococcal disease:

A. Penicillin therapy eradicates nasopharyngeal carrier.

B. Ciprofloxacin (500 mg orally as a single dose) can be used in person 18 years of age or older.

C. Ceftriaxone (125 mg in a single dose IM for children less than 12 years of age and 250 mg in a single dose IM for adolescents 12 years or older) can be used.

D. Rifampin (10 mg/kg orally every 12 hours for a total of four doses, maximum dose 600 mg and 5 mg/kg/dose for infants less than 1 month of age) is used.

E. Hospitalized patients should be isolated for droplet precautions for 24 hours after the onset of therapy.

QUESTION # 08

The following is a live virus vaccine:

A. Mumps

B. DTP

C. Pneumococcal

D. Hepatitis

E. Meningococcal

ANSWERS ON MONDAY DATED 31/12/2018

PEDIATRICS MCQS: TUBERCULOSIS

QUESTION # 01

If a mother is PPD positive and has a negative chest x-ray, the newborn infant should be:

A. Separated from the mother

B. Given INH prophylaxis

C. Evaluated with a chest radiography

D. Evaluated with a PPD testing

E. With the mother

QUESTION # 02

A mother has an active pulmonary tuberculosis. 

All of the following statements are true about management of the newborn except:

A. The newborn should receive INH therapy.

B. The newborn should be isolated from the mother regardless of her symptoms during INH therapy.

C. The newborn should be isolated from the mother if she has drug-resistant tuberculosis, she is noncompliance, and she is ill enough to require hospitalization.

D. The newborn should receive INH therapy until the mother’s sputum cultures are negative for at least 3 months.

E. The newborn should receive a Mantoux tuberculin skin test after 3 months of age. If positive, INH should be continued for a total duration of 9-12 months.

QUESTION # 03

The preferred therapy for a pregnant woman with an active pulmonary tuberculosis is a combination of:

A. INH, pyrazinamide, and rifampin

B. INH, ethionamide, and ethambutol

C. Rifampin, ethambutol, and ethionamide

D. INH, rifampin, and ethambutol

E. Streptomycin, INH, and rifampin

QUESTION # 04

The following CSF findings are appropriate for patients with a tuberculous meningitis:

a) Leukocyte counts are 2000, mostly polymorphs, glucose is 8 mg/dL, and protein is 350 mg/dL.

b) Leukocyte counts are 200, mostly lymphocytes, glucose is 50 mg/dL, and protein is 60 mg/dL.

c) Leukocyte counts are 350, mostly monocytes, glucose is 40 mg/dL, and protein is 70 mg/dL.

d) Leukocyte counts are 4,500, mostly lymphocytes, glucose is 30 mg/dL, and protein is 100 mg/dL.

e) Leukocyte counts are 400, mostly lymphocytes, glucose is 35 mg/dL, and protein is 3,900 mg/dL.

ANSWERS ON MONDAY 31/12/2018

PEDIATRIC MCQS: ABG INTERPRETATION

Study this arterial blood gas report of a 4-year-old child in the emergency unit with feeble peripheral pulses.

• pH :                 7.28
• PaCO2 :            32 mmHg
• PaO2 :              87 mmHg
• HCO3 :             12 mMol/L
• Base excess :    08 mMol/L

1. Give the complete ABG diagnosis and possible cause of the abnormality.

2. Name the most appropriate corrective measure for this child.

3. Calculate the predicted carbon dioxide level for this level of bicarbonate.

Answer on 18/12/2018

PEDIATRICS MCQS: PEDIATRIC NEUROLOGY

 PEDIATRIC NEUROLOGY



QUESTION # 01
Cyclic vomiting in children is a syndrome in which of the following groups?
A. Migraine with aura
B. Classic Migraine
C. Migraine without aura
D. Migraine equivalent
E. Epileptic syndrome

QUESTION # 02

A 10-year-old boy with a history of seizures and headache came to your office for an evaluation. You ordered a brain MRI, which is shown. The lesion shown in the MRI is resected and pathologic analysis is consistent with sub-ependymal giant cell astrocytoma.

What is the most likely diagnosis?

A. Tuberous sclerosis complex

B. Neurofibromatosis type 1 (NF1)

C. Neurofibromatosis type 2 (NF2)

D. Sturge-Weber syndrome

E. He probably does not have a neuro-cutaneous disorder; this tumor is most        often seen sporadically

Question # 03

The findings shown in the figures are consistent with which neuro-cutaneous syndrome?

Coronal T1-weighted pre-contrast MRI. 

What is your diagnosis?

A. Neuro-cutaneous melanosis

B. lncontinentia pigmenti

C. Hypomelanosis of Ito

D. Sturge-Weber syndrome

E. Epidermal nevus syndrome

QUESTION # 04

Physical complications of spina bifida include which of the following?

A. Allergy to latex

B. Leg weakness and paralysis

C. Club foot

D. (B) and (C)

E. (A) and (C)

QUESTION # 05

You are seeing a 3-year-old boy for his follow-up visit. He has a history of developmental delay and intellectual disability since early in life and has developed torticollis and spasticity in his limbs. He also has a history of seizures and aggressive behavior. He began biting himself to the point of bleeding and constantly self-inflicts injuries. He has had kidney stones and hyperuricemia.

Which of the following is not correct regarding this condition?

A. The genetic defect is in the gene HPRT1

B. It is autosomal recessive

C. Hypoxanthine guanine phosphoribosyltransferase is the deficient enzyme

D. It is caused by an enzymatic defect in the purine salvage pathway

E. Patients may have choreoathetotic movements

ANSWERS TOMORROW AT 10 AM.

PEDIATRIC MCQS: MISCELLANEOUS

QUESTION # 01

The preferred diagnostic study in patients with a sinus disease is:

A. AP x-ray of the sinuses
B. Lateral x-ray of the sinuses
C. PA x-ray of the sinuses
D. CT-scan of the sinuses
E. Ultrasonography of the sinuses

QUESTION # 02

A 5-month-old boy appears with respiratory distress for the last 24 hours. He had two episodes of pneumonia and four episodes of otitis media and six episodes of diarrhea. He is delayed for his growth and development. A physical examination reveals bilateral rales and right otitis media. 

All of the following findings are true for this patient except:

A. Total CBC lymphocyte counts are 1600/cmm.

B. Chest x-ray reveals bilateral infiltrates.

C. Blood culture is positive for Streptococcus pneumoniae.

D. Serum immunoglobulin levels are elevated.

E. Conductive hearing loss in the right ear.

QUESTION # 03

A full-term newborn appears with thick meconium stain. Her Apgar scores are 2 and 8 at 1 and 5 minutes respectively. The meconium is removed from the oropharynx. However, no meconium is visualized below the vocal cord. 

Most likely, this child will develop:

A. Meconium aspiration

B. Transient tachypnea of the newborn

C. Pneumothorax

D. Pneumomediastinum

E. No meconium aspiration

QUESTION # 04

The orchiopexy is performed for an undescended testis between:

A. 1-3 months of age

B. 3-6 months of age

C. 6-9 months of age

D. 9-15 months of age

E. 15-24 months of age

QUESTION # 05

A 9-year-old girls appears with fatigue, irritability, low-grade fever, arthralgia, abdominal pain, weight loss, and rash. A physical examination reveals erythema over the malar areas and bridge of the nose. She also has violaceous (heliotropic) discoloration of the upper eyelids and mild facial edema. 

All of the following laboratory findings are present in this patient except:

A. Coombs-positive anemia

B. Presence of antinuclear antibody

C. Absence of antibodies to DNA

D. Negative rheumatoid factor

E. Elevated serum creatine kinase

PEDIATRICS TOP-UPS

MANAGEMENT OF X-LINKED HYPOPHOSPHATEMIC RICKETS

A. Life-long therapy

1.        Phosphate (P04) solution 75 mg/kg/day 4-5 times a day (maximum 2 g) 15 ml = 10 mmol =300 mg

2.       Calcitriol 40 ng/kg OM X 3 months then 20 ng/kg (maximum 1 mcg) or Alfacalcidol 100 ng/kg OM X 3 months then 50 ng/kg (maximum 2 mcg)

B. Follow-up and Monitoring

1.        Six-weekly till biochemical parameters are stable (approximately 3 months) then 3-            monthly subsequently.

2.       Every visit:

a.       Parameters to be monitored:

·         Height (stadiometer)

·         Head circumference

·         Inter-condylar and inter-malleolar distances

·         Flaring of wrists, ankles, costochondral junction

·  Blood urea, creatinine, sodium, potassium, chloride, bicarbonate, calcium, phosphate, uric acid

         b.    Aim for:

·         Serum calcium >2.35 mmol/L

·         Serum phosphate >1 mmol/L

·         Serum alkaline phosphatase <500 U/L

·         Urinary calclum : creatinine <0.6

3.    Six-monthly:

•     iPTH (Aim for <10 pmol/L)

•   Urine oxalate : creatinine ratio (Aim for <0.268 mmol/mmol) If high refer to dietician

•     Renal ultrasound (for nephrocalcinosis)

0-     Normal

1 -    Faint hyperechogenic rim around medullary pyramids

2 -    More Intense rim with echoes faintly filling entire pyramid

3 -    Uniformly intense echoes throughout pyramids

4 -    Stone formation: solitary focus of echoes at the tip of the pyramid with              acoustic shadowing)

4.    Yearly:

•     Knee X-rays

•     Orthopedic review

•     Dental review

•  24 hour urinary protein excretion and creatinine clearance if there is nephrocalcinosis

•     Bone densitometry (also at diagnosis)

C. Screening of siblings: Initially at 3 months of age, then at 6 months of age

PEDIATRIC MCQS: PEDIATRIC MISCELLANEOUS

QUESTION # 01
A 3-year-old girl is seen in the emergency department 30 minutes after ingesting a large amount of her father's propranolol that was prescribed for the treatment of hypertension. You are working with a group of medical students who ask you what symptoms the patient may exhibit.

Of the following, the MOST likely symptom that would be seen is

A. Hyperglycemia

B. Hypertension

C. Seizures

D. Tachycardia

E. Tachypnea

ANSWER: TUESDAY 10 AM



QUESTION # 02
An 18-month-old boy is brought to your office because his parents are concerned about bowed legs. History reveals he was late in learning to walk independently (just starting at 15 months). Laboratory testing reveals the following:
• Alkaline phosphatase, 862 U/L; normal range, 150-420 U/L
• 25-hydroxyvitamin D, 31 ng/mL (77 nmol/L); normal range, 20-50 ng/mL (50 to 125 nmol/L)
• Parathyroid hormone, 15 pg/mL (15 ng/L); normal range, 10-65 pg/mL (10 to 65 ng/L)
• Serum calcium, 8.7 mg/dL (2.17 mmol/L)
• Serum phosphorus, 1.8 mg/dL (0.58 mmol/L).
A radiograph of the legs is shown below 

Of the following, the MOST likely diagnosis is
A. Fibrous dysplasia
B. Hypophosphatasia
C. Hypophosphatemic rickets
D. Osteogenesis imperfecta type I

E. Vitamin D deficiency rickets



ANSWER: TUESDAY 10 AM




QUESTION # 03
The newborn infant of a 28-year-old primigravida has severe respiratory distress and is noted to have pseudoepicanthus, flattened ears and nose, and bilateral club feet. There is no family history of renal failure. Abdominal ultrasonography reveals bilaterally enlarged echogenic kidneys with poor corticomedullary differentiation; there are no cysts or hydronephrosis. The liver, spleen, pancreas, and gall bladder are reported to be normal.

Of the following, the MOST likely cause of this neonate's condition is
A. Autosomal recessive polycystic kidney disease
B. Bilateral Wilms tumor
C. Multicystic dysplastic kidney
D. Nephronophthisis

E. Renal vein thrombosis


ANSWER: TUESDAY 10 AM


QUESTION # 04
A 5-year-old girl with classic 21-hydroxylase deficiency (congenital adrenal hyperplasia) develops gastroenteritis with fever (up to 38.9°C), vomiting, and diarrhea. Her regular medications include hydrocortisone and fludrocortisone. Upon presentation to the emergency department, she is tired-appearing and remains febrile. Her pulse rate is 162 beats/min, blood pressure is 62/40 mm Hg, and capillary refill is poor. Laboratory tests drawn in the emergency department are still pending, but fingerstick glucose level is 42 mg/dL (2.3 mmol/L). The patient is treated with a bolus of normal saline to restore circulatory support.
Of the following, the MOST important therapy to administer to this patient next is
A. Cortisone acetate intramuscularly and aldosterone intravenously
B. Cortisone acetate intramuscularly and dextrose intravenously
C. Dopamine and dextrose intravenously
D. Hydrocortisone hemisuccinate and aldosterone intravenously

E. Hydrocortisone hemisuccinate and dextrose intravenously


ANSWER: TUESDAY 10 AM


QUESTION # 05
A 13-year-old girl with systemic lupus erythematosus and lupus nephritis presents to your office for her REGULAR CHECK-UP. She has been doing very well and has not had any symptoms suggestive of active disease. Her current medications include prednisone, hydroxy-chloroquine, ranitidine, simvastatin, mycophenolate mofetil, enalapril, depot medroxyprogesterone, vitamin D with calcium, and a multivitamin. She asks you which of these medications are keeping her lupus disease under control.

Of the following, the response you are MOST likely to give is
A. Enalapril, hydroxychloroquine, mycophenolate mofetil, prednisone, and              simvastatin
B. Enalapril, hydroxychloroquine, mycophenolate mofetil, and prednisone
C. Hydroxychloroquine, mycophenolate mofetil, and prednisone
D. Hydroxychloroquine, mycophenolate mofetil, prednisone, and simvastatin

E. Mycophenolate mofetil, ranitidine, and prednisone


ANSWER: TUESDAY 10 AM