Congenital

H2N (CH2]5 NH2. a syrupy fuming liquid, b.p. 178-180 - C. Soluble in water and alcohol. Cadaverine is one of the ptomaines and is found, associated with pulrescine, in putrefying tissues, being formed by bacterial action from the amino-acid lysine. It is found in the urine in some cases of the congenital disease cystinuria. The free base is poisonous, but its salts are not. 

It has been reported that Parylene N is deposited in a state of compressive stress (37). The inherent stress is 18 MPa (2300 psi) and is invariant with thickness. This congenital compressive stress can be removed and rendered tensile by a thermal cycle. 

Primary immunodeficiencies are uncommon, and may occur in 1 in 10,000 individuals (6). Many primary immunodeficiencies are hereditary and congenital, and first appear in infants and children. Primary immunodeficiencies are classified into four main groups (7) relating to the lymphocytes (B-ceUs, T-ceUs, or both), phagocytes, or the complement cascade (8). Primary deficiency diseases result from B-ceU defects in 50% of cases, from T-ceU defects in ca 10%, and from combined B- and T-ceU defects in ca 20%. Phagocytic disorders account for 18% and complement defects occur in 2% of all cases. 

In the case of hyperphenylalaninaemia, which occurs ia phenylketonuria because of a congenital absence of phenylalanine hydroxylase, the observed phenylalanine inhibition of proteia synthesis may result from competition between T.-phenylalanine and L-methionine for methionyl-/RNA. Patients sufferiag from maple symp urine disease, an inborn lack of branched chain oxo acid decarboxylase, are mentally retarded unless the condition is treated early enough. It is possible that the high level of branched-chain amino acids inhibits uptake of L-tryptophan and L-tyrosiae iato the brain. Brain iajury of mice within ten days after thek bkth was reported as a result of hypodermic kijections of monosodium glutamate (MSG) (0.5—4 g/kg). However, the FDA concluded that MSG is a safe kigredient, because mice are bom with underdeveloped brains regardless of MSG kijections (106). 

Arrhythmias. The first solution to cardiovascular problems arising from arrhythmias came about as a result of a complication caused by open-heart surgery. During procedures to correct congenital defects in children s hearts, the electrical conduction system often became impaired, and until it healed, the heart could not contract sufficiently without outside electrical stimulation. A system that plugged into a wall outlet was considered adequate until an electrical storm knocked out power, lea ding to the development of the first battery-powered external pacemaker. 

Congenital deficiency of prothrombin is inherited in an autosomal recessive fashion and is the rarest of all the hereditary coagulation disorders. Congenital dysprothrombinemia has also been recognized. 

Deficiency of Factor VII is relatively rare and inherited as an autosomal recessive disorder. Deficiency of Factor VII has been reported to be associated with bond abnormal bleeding and thrombotic tendencies. Deep vein thrombosis and pulmonary emboli have been reported in affected individuals. There is a very high frequency of Factor VII deficiency in people with the Dubin-Johnson syndrome, which is a congenital disorder of Hver function. 

Congenital deficiency of Factor IX results in hemophilia B, also known as Christmas disease, and is inherited in a sex-linked recessive manner. Instmctive is the fact that some female carriers are symptomatic. Combined deficiencies of Factors VIIFC and IX have been described. 

Congenital deficiency of Factor X is a rare autosomal recessive disorder. Several variants have been described. 

Congenital deficiency of Factor XI is a relatively rare coagulopathy that has been reported as both an autosomal dominant and autosomal recessive trait. This deficiency state occurs predominantly in the Jewish population. Most patients with this deficiency state remain asymptomatic until trauma or surgery is encountered. Spontaneous hemorrhage is rare in this population. 

Congenital deficiency of Factor XII is inherited as an autosomal recessive trait. Deficiency of this factor is rarely associated with any coagulopathy. It has been observed that people deficient in this factor may have an increased frequency of thromboembolic compHcations. 

Congenital deficiency of Factor Xlll is inherited as an autosomal recessive trait and is frequendy recognized at birth because of delayed persistent hemorrhage from the umbiUcus. In Factor Xlll-deficient people wound healing is defective and wound dehiscence is common. 

The incidence of congenital malformations such as cryptorchidism (undescended testes) and hypospadias (malformation of the penis) may have increased, but... 

World Health Organization, Congenital Malformations Worldwide A Reportfrom the International Clearinghouse for Birth Defects Monitoring Systems, Elsevier, Oxford, 1991, p. 113. 

MacDonald, H., and Tobin, J. O. H. (1978). Congenital cytomegalovitiis infection A collaborative study on epidemiological, clinical and laboratory findings. Dev. Med. Child Neurol. 20, 271-282. 

Coumarin is also widely used for long-term anticoagulation in chronic atrial fibrillation (particularly to avoid cardioembolic strokes), to prevent DVT or PE in patients with chronic hypercoagulability (e.g., congenital AT or protein C deficiency), or to prevent... 

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