LECTURE V Flashcards
CHECK ON LEARNING
in an adult this separates the thoracic and abdominal cavities? related to the formation of the diaphragm
musculotendinous partition
what are the four embryonic components the diaphragm is developed from?
septum transversarum
pleuroperitoneal membranes
dorsal mesentery of the esophagus
muscular ingrowth from the lateral body walls
this diaphragmatic disorder fails to close with pleuroperitoneal membranes with the other three parts of the diaphragm and so this results in herniation of abdominal contents into the thorax causing lung compression and affecting lung development and occurs on the left side?
congenital diaphragmatic hernia
this condition of the lungs in when half the diaphragm has defective musculature and balloons into the thoracic cavity as a membranous sheet
failure of the muscular tissue from the body wall to extend to the pleuroperitoneal membrane on the affected side
similar to CDH
eventration of diaphragm
this condition is when there is a congenital fissure in the anterior abdominal wall
protrusion of viscera
defect is to the right of the midline
bowel is uncovered and floating in amniotic fluid
result from failure of the lateral body folds to fuse completely
gastroschisis
Tuberous sclerosis complex
Sturge Weber Syndrome
Neurofibromatosis
these conditions are examples of?
Neurocutaneous syndromes
name this type of Neurocutaneous syndrome?
benign tumors that can occur anywhere but most commonly in the brain and skin
Tuberous sclerosis complex
name this type of Neurocutaneous syndrome?
rare neurocutaneous disorder with vascular malformations of the eye, skin and brain
Sturge Weber Syndrome
name this type of Neurocutaneous syndrome?
pathogenic cae-au-lait spots, gliomas, PNS, neurofibromas
Neurofibromatosis
this is a genetic skin disorder resulting in abnormal epidermal differentiation and excessive keratinization of skin?
characterized by dryness and scaling, may involve the entire body?
Ichthyosis
name this genetic skin disorder
this is a rare keratinizing disorder
autosomal recessive inheritance with a mutation in the ABCA12 gene
usually premature
despite intensive care, >50% die early
Harlquin Ichthyosis
name this genetic skin disorder
usually born premature
covered with thick, taut membrane
membranous skin cracks and falls off in large sheets
deficiency in TGM1 is likely cause
Collodion infant
name this genetic skin disorder
looks like colloidal baby at first, but scaling persists. Growth of hair and sweat glands impeded.
Autosomal recessive disorder
lamellar
name this genetic skin disorder
deletion or mutation in STS gene. Neonates have pink or red skin with large translucent scales that shed after birth
x-linked ichthyosis
name this genetic skin disorder
skin may have blisters and appear to be peeling at birth.
autosomal dominant inheritance
epidermolytic ichthyosis