Q: Carney complex includes which of the following components?
a.Large cell calcifying Sertoli cell tumors
b.Cardiac myxomas
c.Primary pigmented adrenocortical disease
d.Autosomal dominant transmission
e.All of the above
Explanation: This autosomal dominant disorder is mapped to chromosome 2p16 and may be due to a gain-of-function mutation. The adrenal disorder is a pigmented nodular adrenocortical disease with associated features such as lentigines, blue nevi, cardiac and skin myxomas, and sexual precocity in boys with large cell calcifying Sertoli tumors and melanotic schwannomas. (See Chapter 571 in Nelson
Textbook of Pediatrics, 17th ed.)
Sunday, October 5, 2008
Thursday, October 2, 2008
26 - Paediatrics Cases mcqs - 1
1q: Which one of the listed clinical scenarios is most consistent with a
diagnosis of SIDS?
a. A 2-year-old female dies suddenly and no autopsy is performed
b. A 3-month-old female dies during sleep and the cause of death is unknown
after autopsy
c. A 4-week-old female dies from respiratory complications after being born 10
weeks prematurely
d. A 9-month-old male dies and an autopsy finds evidence of repeated bone fractures
and bilateral retinal hemorrhages
e. A male is stillborn at 29 weeks of gestation to a mother with obstetrical complications
*Sudden infant death syndrome :
(SIDS) is a heterogeneous, multifactorial disorder, but by definition it refers to sudden death of infant under 1 year of age that is unexplained after thorough examination. Most cases of SIDS occur between 2 and 4
months of life, and the child usually dies during sleep (“crib death” or “cot death”). A risk factor for SIDS is sleeping in a prone position. Therefore healthy infants should sleep on their back or side. Maternal factors associated with SIDS include age less than 20, being unmarried, low socioeconomic group, smoking, and drug abuse. Infant factors associated with SIDS include prematurity, low birth weight, male sex, and a history of SIDS in a sibling. In contrast to SIDS, death from respiratory complications after being born 10 weeks prematurely is suggestive of hyaline membrane disease, while evidence of repeated bone fractures and bilateral retinal hemorrhages is suggestive of trauma, child abuse, or “shaken baby” syndrome.
diagnosis of SIDS?
a. A 2-year-old female dies suddenly and no autopsy is performed
b. A 3-month-old female dies during sleep and the cause of death is unknown
after autopsy
c. A 4-week-old female dies from respiratory complications after being born 10
weeks prematurely
d. A 9-month-old male dies and an autopsy finds evidence of repeated bone fractures
and bilateral retinal hemorrhages
e. A male is stillborn at 29 weeks of gestation to a mother with obstetrical complications
*Sudden infant death syndrome :
(SIDS) is a heterogeneous, multifactorial disorder, but by definition it refers to sudden death of infant under 1 year of age that is unexplained after thorough examination. Most cases of SIDS occur between 2 and 4
months of life, and the child usually dies during sleep (“crib death” or “cot death”). A risk factor for SIDS is sleeping in a prone position. Therefore healthy infants should sleep on their back or side. Maternal factors associated with SIDS include age less than 20, being unmarried, low socioeconomic group, smoking, and drug abuse. Infant factors associated with SIDS include prematurity, low birth weight, male sex, and a history of SIDS in a sibling. In contrast to SIDS, death from respiratory complications after being born 10 weeks prematurely is suggestive of hyaline membrane disease, while evidence of repeated bone fractures and bilateral retinal hemorrhages is suggestive of trauma, child abuse, or “shaken baby” syndrome.
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