Notes in IV. Hydrops to PKU

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Published	12/09/2024	a hemolytic disease caused by blood group antigen incompatibilitybetween mother and fetus.
Published	12/09/2024	2 types of Fetal Hydrops
Published	12/09/2024	● Maternal RH isoimmunization→ {{c1::RhIg}} (containing anti-D antibodies)→ At {{c2::28}} weeks of gestation and within {{c2::72}} hours of delivery t…
Published	12/09/2024	2 consequences of Fetal Hemolysis
Published	12/09/2024	What is the diagnosis for patients presenting like these?
Published	12/09/2024	Accumulation of edema fluid in the fetus during intrauterinegrowth. In the past, this is most commonly caused by RH blood groupincompatibility between…
Published	12/09/2024	In ABO incompatibility, fetal hydrops can cause hemolytic disease but there are factors why this does not proceed such as:1. Most anti-A and anti-B an…
Published	12/09/2024	One of the differences that can be seen with Rh-incompatibility andABO-incompatibility is the {{c1::affected child}}.
Published	12/09/2024	If Rh incompatibility is the cause of HDN, usually the affectedchild is from the {{c1::second}} and {{c1::subsequent}} pregnancies.
Published	12/09/2024	If ABO incompatibility is the cause of HDN, usually it is the {{c1::first-born}}.
Published	12/09/2024	In anemia, the combination of reduced {{c1::plasma oncotic pressure}}and increased h{{c1::ydrostatic pressure}} in the circulation secondary tocardiac…
Published	12/09/2024	In jaundice,  hemolysis produces {{c1::unconjugated}} bilirubin and the bilirubin passes through the infants poorly developed blood-brain barrier…
Published	12/09/2024	Which type of hydrops occue when a mother is RH positive but the father is RH negative?
Published	12/09/2024	When the fetus inherits red cell antigenic determinants from thefather that are foreign to the mother, a {{c1::maternal}} immunereaction may occur.
Published	12/09/2024	Type of fetal hydrops occuring due to chromosomal anomalies, cardiovascular defects, fetal anemia, etc.
Published	12/09/2024	Causes of Non-Immune Hydrops
Published	12/09/2024	Chromosomal Anomalies causing NON-IMMUNE HYDROPS
Published	12/09/2024	Cardiovascular Defects causing NON-IMMUNE HYDROPS
Published	12/09/2024	Most common cause of non-immune hydrops in some parts of the world
Published	12/09/2024	Caused by parvovirus B19 which is now a rapidly emerging cause ofhydrops
Published	12/09/2024	Post nuchal Fluid accumulation in Fetal Hydrops is also called as
Published	12/09/2024	→ Most severe and generalized manifestation of fetal hydrops→ Occur with minimal edema▪ Isolated pleural▪ Peritoneal▪ Postnuchal fluid→ Outcomes: stil…
Published	12/09/2024	Most serious threat of Hydrops Fetalis
Published	12/09/2024	Neural damage needs a blood bilirubin of {{c1::>20}} ml/dl in terminfants → threshold usually lower in preterm infants
Published	12/09/2024	Hydrops associated with {{c1::fetal anemia}}→ Both fetus and placenta are filled→ Organs enlarge due to congestion→ High hematopoietic activity for mo…
Published	12/09/2024	{{c1::Aplasia}} → occurs when parvovirus is implicated
Published	12/09/2024	increased number of immature RBCs (retics and erythroblasts) in the peripheral circulation
Published	12/09/2024	Give the Abnormalities Suggesting Inborn Errors of Metabolism for the following systems:General
Published	12/09/2024	Give the Abnormalities Suggesting Inborn Errors of Metabolism for the following systems:Neurologic
Published	12/09/2024	Give the Abnormalities Suggesting Inborn Errors of Metabolism for the following systems:Gastrointestinal
Published	12/09/2024	Give the Abnormalities Suggesting Inborn Errors of Metabolism for the following systems:Eyes
Published	12/09/2024	Give the Abnormalities Suggesting Inborn Errors of Metabolism for the following systems:Muscle, Joints
Published	12/09/2024	The clinical manifestations of these inborn errors of metabolism diseases are generally the result of either {{c1::abnormal metabolite accumulation}} …
Published	12/09/2024	Autosomal recessive disorder caused by severe deficiency of theenzyme phenylalanine hydroxylase (PAH) leading tohyperphenylalaninemia
Published	12/09/2024	PHENYLKETONURIAAutosomal {{c1::recessive}} disorder caused by severe deficiency of theenzyme {{c1::phenylalanine hydroxylase (PAH)}} leading to{{c1::h…
Published	12/09/2024	Symptoms of Phenylketonuria
Published	12/09/2024	Affected infants with PKU normal at birth → within a few weeks, plasma Phe increases → impairment of {{c1::brain development}} and {{c1::mentalretarda…
Published	12/09/2024	Characterized by:→ Seizures and other neurologic abnormalities→ Decreased pigmentation of the skin→ Characteristic musty or mousy odor→ Eczema
Published	12/09/2024	Management for PKU
Published	12/09/2024	{{c1::image-occlusion:rect:left=.2993:top=.5665:width=.1892:height=.0212:oi=1}}{{c2::image-occlusion:rect:left=.0811:top=.7534:width=.2297:height=.027…
Published	12/09/2024	{{c1::image-occlusion:rect:left=.0151:top=.3116:width=.2219:height=.3238:oi=1}}
Published	12/09/2024	● Normal children→ Less than 50% of dietary intake of Phe is necessary for {{c1::protein}}synthesis→ Remainder is converted to {{c1::Tyrosine}} by {{c…
Published	12/09/2024	● Phenylketonuria biochemical abnormality→ Inability to convert PKU to {{c1::Tyrosine}}
Published	12/09/2024	● Blockage of Phe metabolism activates minor shunt pathways thatyield several metabolites excreted in {{c1::sweat}} in large amounts→ Results in stron…
Published	12/09/2024	● PKU→ Requires mutation in both allele for PAH gene in {{c1::Ch12}}→ Autosomal {{c1::recessive}}▪ Both parents must be carriers for offspring to mani…
Published	12/09/2024	● The PAH System→ Deficiency of {{c1::PAH or DHPR}} can give rise to phenylketonuria→ NAD oxidized→ NADH reduced
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