Hyperammonemia type -1 and type -2 

A deficiency of the two enzymes of the urea cycle ,namely carbamoyl phosphate synthetase -1 and ornithine transcarboxylase are associated with an elevation of plasma ammonium concentration which is classified as type1 and type 11 hyperammonemia ,respectively.type-1 deficiency is the most severe UCD.it is a life and may be lethal while the delayed-onset type is less severe and shows up at mid to late age .type11 hyperammonemia is the most common inherited urea cycle disorder. the associated symptoms include lethargy ,vomiting ,coma and cerebral oedema. the symptoms are as severs as CPS-1 deficiency in case of males.

citrulline is useful in reducing ammonia levels.

citrullinemia 

the disease arises due to accumulation of ammonia and other toxic substances in blood. hyper ammonia can be quite severe. it is characterized by marked elevation of plasma citrulline and excretion of large quantities of citrulline(citrullinemia) in urine.

two type of citrullinemia is knows. type -1 or classic citrullinemia is usually associated with acute neonatal early onset . it is due to a deficiency of arginosuccinate synthase within a few days the infant suffers from energy loss ,poor feeding ,seizures ,convulsions and unconsciousness .these symptoms vary in severity. they can be managed on a low protein ,ammonia scavenger drugs and arginine supplementation. type11  citrullinemia is due to a defect in the mitochondria transporter ,citron. it is more common in people of Japanese descent. it mainly affects the nervous system which results in confusion restlessness, memory loss ,abnormal behaviors seizures ,and coma. the symptoms generally show up in adulthood. these patients respond well on a high protein and low carbohydrate diet.

Argininosuccinic aciduria 

it is also known as argininosuccinic acidemia. in this condition argininosuccinic acid elevated in the blood and urine of the patient due to a deficiency of arginosuccinate lyase. sometime elevated levels of ammonia  are also reported. this lethargic unwilling to eat, and experienced difficulty in breathing.

Argininemia

the affected individual develop progressive spasticity ,stiffness in the legs, developmental delays and difficulty with balance and coordination. the symptoms  become evident by age 3. the plasma concentration of arginine is markedly elevated (hyperargininemia)