congenital long QT syndromes

the two congenital LQTS are:
1. Romano Ward syndrome
2. Jervell Lange Neilsen syndrome

the major differences lie in inheritance, severity and associated deafness.

The romaNO warD syndrome is autosomal Dominant and has NO deafness
whereas the jervell lange neilsen is autosomal recessive with deafness

zero order kinetics

here are the seven drugs that show zero order kinetics:

1. Ethanol

2. Phenyoin

3. Tobutamide

4. Warfarin

5. Theophylline

6. Aspirin

7. Propranolol

fetal warfarin syndrome

the warfarin embryopathy has skeletal and neurological manifestations.

while the skeletal involvement can occur only in the first trimester, the neurological involvement can happen anytime during pregnancy.

the incidence of warfarin embryopathy is about 7 percent.

the skeletal involvement can be remembered as a triad consisting,

1.Intrauterine Growth retardation

2.Nasal hypoplasia

3.Chondrodysplasia punctata (stippled epiphyses).

there is a highly prevalent misconception that fetal warfarin syndrome is otherwise called as Contradi syndrome, probably due to some typographical error, at least among the medicos(in India) preparing for PG entrance examination.

In fact, this is confused with Conradi-Hunermann Syndrome, an XLD inherited syndrome, characterised by punctate calcification of cartilage, which is similar to the punctate calcification seen in warfarin embryopathy.

and wikipedia mentions that fetal warfarin syndrome is called as DiSala syndrome.

CONCEPT : appreciate the correlation that warfarin causes this embryopathy by inhibiting the carboxylation of osteocalcin in the developing fetal cartilage... and this is similar to the inhibition of carboxylation of clotting factors - 2, 7, 9, 10 - in the liver... and in both these situations, the carboxylation enables these proteins to "hold" calcium (calcium in bone and for clotting cascade).