Theme:Paediatric Syndromes
AAngelman's syndrome
BCornelia de Lange syndrome
CCri du chat syndrome
DDown's syndrome
EPrader-Willi syndrome
FRett's syndrome
Select the most likely diagnosis in the following children:
A 4-year-old girl with hand wringing and some loss of developmental skills.
The correct answer is Rett's syndrome
Rett's Disorder, also known as Rett Syndrome, is diagnosed primarily in females. In children with Rett's Disorder, development proceeds in an apparently normal fashion over the first 6 to 18 months at which point parents notice a change in their child's behavior and some regression or loss of abilities, especially in gross motor skills such as walking and moving. This is followed by an obvious loss in abilities such as speech, reasoning, and hand use. The repetition of certain meaningless gestures or movements is an important clue to diagnosing Rett's Disorder; these gestures typically consist of constant hand-wringing or hand-washing
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A 2-year-old boy with gross obesity associated with compulsive eating and has some developmental delay.
Answer- prader willi syndrome .
Prader Willi syndrome is associated with compulsive eating and gross childhood obesity and is associated with some developmental delay. It is related to abnormalities on Chromosome 15.
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Arson is likely to be repeated if associated with epilepsy. - FALSE
Arson, in general, is the crime of setting a fire for an unlawful or improper purpose.Arson is not considered as part of automatisms seen in people with epilepsy. There is no reliable evidence to suggest that such behaviour increases people with epilepsy.
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the threshold temperatures below which malignant cardiac arrythmias spontaneously occur in hypothermic patients is
30 degree celsius .
Patients are at risk for malignant dysrhythmias at a body temperatures below 30 degree Celsius, with the risk increasing the lower the temperature. Although various rhythms can occur at any time the typical progression is from sinus bradycardia to slow atrial fibrillation to ventricular fibrillation and finally asystole. The hypothermic myocardium is extremely irritable and ventricular fibrillation may be induced by a variety of manipulations and interventions that stimulate the heart, including rough handling of the patient.
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from the corner of the mouth the correct depth for insertion of an endotracheal tube in an average sized female adult is ?
21 cms.
Correct ETT tube placement is about 2 cm above the carina. From the corner of the mouth this is approximately 23 cm and for women 21 cm. For newborn 10 cm, one year old 11 cm then age/2 + 12.
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the appropriate ratio of cardiac compression and ventilation during two person CPR for adult is ?
5 : 1
Basic Life Support
neonate infant child(1-8 years) adult(>8 years)
EAR 30-60/min 20/min 20/min 12/min
CPR 2 fingers 2 fingers heel of 1 hand 2 hands
depth <----------------------------------- 1/3 of chest ------------------------------------------>
rate 120/min 100/min 100/min 100/min
ratio 3: 1 5:1 5:1 5:1
(15:2 for one rescuer)
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Question 1. Regarding normal pregnancy
a. Pregnancy lasts on average 40 weeks from conception (False)
b. If a woman has a long menstrual cycle, the expected date of delivery should be extended to reflect this (True)
Explanation: Pregnancy lasts 40 weeks from the first day of the last menstrual period in a 28-day cycle, or 38 weeks from conception.
c.Most women will deliver on the expected date of delivery (False)
d. Fetuses may be considered independently viable from 20 weeks' gestation (False)
e. The puerperium lasts approximately 10 days (False)
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Question 2. Regarding physiological change in pregnancy
a. Cardiac output increases by 40% (True)
Explanation: Pregnancy is, however, associated with an increased coagulant activity. Any prolongation of the thrombin time suggesting a coagulation defect requires urgent attention.
b. Peripheral vascular resistance falls (True)
c. Blood pressure should be measured with the woman lying flat (False)
d. Oxygen consumption falls (False)
e. The ureters can appear dilated (True)
Explanation: Oxygen consumption increases by 15% to meet the increased needs of the mother and fetus.
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Question 3. In hyperemesis gravidarum
a. Antiemetics should be avoided if possible (True)
Explanation: though antiemetics are required in severe cases.
b. Thiamine deficiency can result in cerebral complications (True)
Explanation: Wernicke's encephalopathy has been described in severe cases.
c. There is an increased incidence of multiple pregnancy (True)
d. A pyrexia occurs (False)
e. Colicky abdominal pain is characteristic (False)
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Question 4. Regarding twins
a. Most are monozygotic (identical) (False)
Explanation: Two thirds are dizygotic.
b. Monozygotic twins face the greatest risks (True)
c. Pre-term labour is a major cause of mortality (True)
d. Infant mortality rates are higher for twins than for singletons (True)
Explanation: All categories of perinatal and infant mortality rates are higher for twins.
e. There is an increased incidence of pre-eclampsia (True).
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Question 5. Diabetes
a. Is associated with a decreased incidence of congenital abnormality (False)
b. Is associated with postmaturity rather than prematurity, resulting in increased birth weights (False)
c. Is associated with an increased incidence of respiratory distress in the neonate (True)
Explanation: This is more likely to arise due to prematurity, an increased incidence of hyaline membrane disease in infants of diabetic mothers, and occasionally a cardiomyopathy in the neonate.
d. Is associated with a reduced insulin requirement in pregnancy (False)
e. Maternal hypoglycaemia can occur in the puerperium (True)
Explanation: if insulin dosage is not reduced to levels approximating the requirement prior to pregnancy.
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Question 6. Congenital malformations
a. Can present with polyhydramnios (True)
Explanation: Abnormalities of liquor volume, either excess or reduction, may suggest congenital abnormalities.
b. Can present with oligohydramnios (True)
Explanation: Abnormalities of liquor volume, either excess or reduction, may suggest congenital abnormalities.
c. Can present with intra-uterine growth retardation (True)
Explanation: Abnormalities of liquor volume, either excess or reduction, may suggest congenital abnormalities.
d. Can present with a breech presentation at term (True)
Explanation: Assessment of the breech presentation at term includes ultrasound evaluation to exclude abnormalities such as neural tube defects/hydrocephalus.
e. Are normally detectable on ultrasound (True)
Explanation: Assessment of the breech presentation at term includes ultrasound evaluation to exclude abnormalities such as neural tube defects/hydrocephalus.
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Question 7. Oligohydramnios
a. Can be caused by amniocentesis (True)
Explanation: This could arise if the procedure precipitates pre-term premature rupture of the membranes.
b. Can be associated with 'postmaturity' (True)
Explanation: Liquor volume is reduced as the pregnancy is prolonged beyond the due date. This may indicate fetal compromise.
c. Can be caused by diabetes (False)
d. Can cause hypoxic damage to the fetal heart (False)
e. Makes the fetus more susceptible to trauma (False)
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Question 8. At 20 weeks' gestation
a. Serum screening for Down's syndrome should be discussed (False)
b. Assessing fetal growth is important (False)
c. An anomaly scan is often performed (True)
Explanation: The fetus can be assessed at this gestation and termination of pregnancy arranged if warranted.
d. Placenta praevia can be diagnosed on this scan (False)
e. Fetal movements can be felt (True)
Explanation: Fetal movements are normally felt by 20-22 weeks in a nulliparous woman and 16-18 weeks in a multiparous woman.
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Question 9. Regarding the use of oxytocin and amniotomy
a. Oxytocin can cause fetal hypoxia (True)
Explanation: if uterine hypertonicity is caused.
b. Abnormalities on a cardiotocograph after amniotomy should prompt vaginal examination (True)
Explanation: to exclude cord prolapse.
c. Once amniotomy is performed the process of induction should be regarded as irreversible (True)
Explanation: because there is a risk of ascending infection once the forewaters have been ruptured.
d. Oxytocin must be given in carefully titrated dose depending on the uterine activity (True)
Explanation: to minimise the complication of hypertonicity.
e. Oxytocin is used more commonly in nulliparous women as they are more sensitive to it (False)
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Question 10. Regarding occipito-posterior positions
a. They are detected by finding the anterior fontanelle in the posterior aspect of the pelvis (False)
b. A minority will rotate prior to full dilatation (False)
c. They are sometimes delivered by caesarean even though full dilatation has been reached (True)
Explanation: as the head is often high and this may be the least traumatic method of delivery.
d. The incidence is increased where the presenting part is very low in the pelvis (False)
e. They may be delivered by ventouse (True
Explanation: This is the optimal instrumental method of delivery.
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Question 11. Care on the postnatal wards includes:
a. Administration of anti-D to all rhesus-D negative women who have given birth to rhesus-D negative babies (False)
b. Arranging rubella immunisation where appropriate for 3 months post partum (False)
c. Discussion of contraceptive needs (True)
Explanation: This issue should always be discussed prior to discharge home, and every effort made to ensure the mother understands that she may conceive before having a period.
d. Regular checks to ensure bladder emptying for 24-48 hours following delivery (True)
Explanation: Problems with micturition often arise in the immediate postpartum period. This may be as a result of painful vulval tears, or operative delivery resulting in tissue oedema around the bladder base.
e. Women delivered by caesarean should be counselled regarding mode of delivery in future pregnancies (True)
Explanation: This is important as not all women attend for postnatal checks. Information should include the type of scar which is present on the uterus and the rationale for the caesarean delivery.
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Question 12. In relation to maternal mortality
a. Ectopic pregnancy accounts for more maternal deaths than thromboembolism (False)
b. Sepsis is no longer a concern since antibiotics became available (False)
c. Regional anaesthesia is safer than general anaesthesia (True)
Explanation: Regional anaesthesia should always be used when possible.
d. Deaths from amniotic fluid embolism are declining (False)
e. Caesarean delivery is now as safe as vaginal delivery provided a regional anaesthetic is used (False)
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Question 13. Precocious puberty
a. Results in tall stature (False)
b. Can be distinguished from pseudo-precocious puberty by measurement of LH levels (True)
Explanation: Precocious puberty results in short stature due to premature epiphyseal closure.
c. Breast buds are the first sign (True)
Explanation: Precocious puberty results in short stature due to premature epiphyseal closure.
d. Has no causative factor in 80% of girls (True)
Explanation: Most cases are constitutional.
e. Is difficult to define precisely because of the marked variation in the age of normal puberty (True)
Explanation: Most cases are constitutional.
f. Causes psychosocial difficulties (True)
Explanation: Precocious sexual activity and behavioural problems are common in affected girls.
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Question 14. Concerning normal puberty
a. The first event is thought to be nocturnal release of gonadotrophins (False)
b. Once initiated, occurs at the same speed in both sexes (False)
c. The average age of onset is falling (True)
Explanation: The average age of puberty has shown a gradual decrease over the last four decades. This is thought to be due to improved nutrition and less physical work.
d. Initial cycles are often anovulatory (True)
Explanation: Postmenarchal cycles are often anovulatory, and dysfunctional uterine bleeding is common. The frequency of ovulatory cycles gradually increases as the reproductive system continues to mature.
e. Growth stops after the menarche (False)
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Question 15. Regarding hirsutism
a. It is more common in Chinese than in Greek women (False)
b. SHBG is usually decreased (True)-SEX HORMONE BINDING GLOBULIN.
Explanation: Hirsutism shows great variation between different races, and is more common in Mediterranean women.
c. It may be successfully treated with the combined oral contraceptive pill (True)
Explanation: Hirsutism shows great variation between different races, and is more common in Mediterranean women.
Doh! d. It may be caused by diazepam (False)
e. In the presence of a regular menstrual cycle it is usually idiopathic (True)
Explanation: It may be caused by drugs such as diazoxide, danazol and phenytoin, but not diazepam.
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Question 16. Genital warts
a. Are caused by a virus which is usually sexually transmitted (True)
Explanation: Diabetes mellitus should be excluded by checking a random blood sugar in women with recurrent vaginal candidiasis.
b. May grow in pregnancy (True)
Explanation: Warts tend to grow rapidly in the third trimester of pregnancy.
c. Usually need to be treated under general anaesthetic (False)
d. Have a characteristic clinical appearance (True)
Explanation: The diagnosis is usually made on clinical appearance, but large or persistent warty lesions should always be biopsied to exclude a carcinoma.
e. In pregnancy can be treated with podophyllin (False).
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Question 17. Which of the following are true?
a. Urinary stone disease is one of the most common non-obstetric causes of abdominal pain in pregnancy (True)
Explanation: Urinary calculi typically cause pain in the flank. Early diagnosis is important in pregnancy because of the risk of septic obstruction of the kidney, and premature labour.
b. Abdominal X-irradiation in pregnancy is most harmful between 4 and 8 weeks' gestation (True)
Explanation: Abdominal X-irradiation during pregnancy is most harmful to the fetus when organogenesis is most rapid, and this is between the 4th and 8th week of gestation.
c. Ultrasound is useful in the investigation of a painful adnexal mass (True)
Explanation: Ultrasound is a useful non-invasive tool in the investigation of a pelvic mass, when a differentiation can be made between cystic and solid lesions. However, laparoscopy or laparotomy is usually needed for precise diagnosis.
d. Laparoscopy is useful in the investigation of pelvic pain accompanied by hypovolaemic shock (False)
e. Laparoscopy is normal in up to 30% of women with pelvic pain (True)
Explanation: In women with pelvic pain, laparoscopy will be normal in up to 30%, and no organic disease will be found.
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Question 1. Benzodiazepines:
(a) Are safer if taken in overdose than are other hypnotics. (True)
(b) Are potent inducers of hepatic enzyme systems. (False)
(c) Do not produce tolerance and physical dependence, unlike barbiturates. (False)
(d) Are sometimes used intravenously in status epilepticus. (True)
(e) Are GABA-receptor antagonists with neuroexcitatory effects. (False)
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Question 2. Treatments for depression may include:
(a) Muscarinic receptor antagonists. (False)
(b) Monoamine oxidase inhibitors. (True)
(c) Neuroleptics. (False)
(d) Dopamine-receptor antagonists. (False)
(e) Serotonin reuptake inhibitors. (True)
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prolonged therapy of which of the following drugs can lead to megaloblastic anaemia ?
a- phenytoin
b- primidone
c- phenobarbital
d- ethosuximide
e- methotrexate
only d is wrong . except ethosuximide all are the right options .
methotrexate inhibits DHF reductase . many anticonvulsants used for grand mal seizures and partial seizures can interfere with absorption and storage of folate in tissues . this does not occur with ethosuximide ,
an anticonvulsant used for petit mal seizures .
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transferrin is a beta globulin in the plasma which transports the iron in the FERRIC form .
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which of the follwing statements are true regarding the schilling test ?
a - concerns the excretion of an oral test dose of cobalt - 60 - labelled B12 and radioactivity in the urine is quantitated
b - is used to diagnose pernicious anemia
c - depends on the difference in B 12 absorption from the GIT between the normal and pernicious anemia subjects
d - depends on the renal absorption of the B 12
ONLY the first three are right .
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3 year old , iron toxicity , 20 FESO4 tablets swallowed . signs and symptoms ?
nausea
cyanosis
pallor
cardiovascular collapse
elevated blood sugar
the top 4 are present . except the blood sugar option . large amounts of iron salts are toxic although the fatalities are rare . most deaths - kids - 12 to 24 months . other symptoms include ,
abdominal pain , diarrhea , vomiting brown or bloody stomach contents containing pills . lassitude , drowsiness , and hyperventilation due to acidosis . if plasma iron concentrations exceed 3.5 mg/ml then IM or IV deferroxamine is given.
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microcytic hypochromic anemia usually responds to
a- folic acid
b- B 12
C- feso4
d- deferrioxamine
answer is c
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which of the following must be enzymatically reduced to be active as a coenzyme for the effective treatment of megaloblastic anemia in a 76 year old man ?
a - folic acid
b- fe so 4
c- B 12
d- vitamin k
e- vitamin D
answer is A
unlike the action of the folic acid derivative LEUCOVORIN , folic acid does not offset the action of the folate reductase inhibitors because it requires the enzyme DHF reductase for activation .
THF acid and methylTHF are required for normal erythropoesis and are also required cofactors for the purine and thymidilate synthesis .impaired thymidylate synthesis which leads to faulty DNA synthesis is responsible for the megaloblastic and macrocytic anemias.
folic acid required for methionine formation from homocysteine so FA def leads to hyperhomocysteinemia. this is an important risk factor for atherosclerosis and peripheral vascular disease . can lead to neural tube defects . high serum homocytstein levels associated with pathogenesis of colon cancer , diabetic retinopathy and other diseases .
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which of the following substances increase the absortion of inorganic iron ?
a - nicotinic acid
b- B6
C- PABA
d - vitamin k
e- VITAMIN C
the answer is e . the substances which decrease the absorption of the inorganic iron are phosphates . meat also facilitates iron absorption .
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which of the following substances wud be elevated in a 75 years old woman with pernicious anemia ?
a - 5 methyl folate
b- di hydrofolate
c- methionine
d- succinyl coA
e - ascorbic acid
answer is a .
in the absence of vitamin B12 the folate is trapped as 5 methyl folate . B 12 is necessary for the formation of the di hydrofolate , methionine from homocysteine , succinyl coA from the malonyl coA . ascorbic acid in high quantities enhances the absorption of the inorganic iron from the GIT .
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