The nutritional therapy of histidinemia

Snyderman, Selma E.; Sansaricq, Claude; Norton, Patricia M.; Manka, Maya

doi:10.1016/s0022-3476(79)80716-7

Cited by 8 publications

(2 citation statements)

References 17 publications

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“…Relatively few subjects have been followed past childhood and, as some patients fi rst develop signs in adolescence (108), a longer follow-up may be needed. Treatment with a low histidine diet is available and has been associated with clinical improvement in some patients with clinical problems (108,109).…”

Section: Histidinemiamentioning

confidence: 99%

Issues in Antenatal and Neonatal Screening and Surveillance for Hereditary and Congenital Disorders

Holtzman¹,

Leonard²,

Farfel³

1981

Annu. Rev. Public Health

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Section: Histidinemiamentioning

confidence: 99%

Issues in Antenatal and Neonatal Screening and Surveillance for Hereditary and Congenital Disorders

Holtzman¹,

Leonard²,

Farfel³

1981

Annu. Rev. Public Health

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“…(HEPATOLOGY 1995;21:1405-1412 therapy restricting histidine-rich foods has been recommended. 6 The Peruvian hishis mouse shows a similar autoso-ma1 recessive enzyme defi~iency,~ caused by a single amino acid substitution, reflecting a guanine to adenine substitution in the histidase locus8 on murine chromosome 10.' This results in the production of an unstable protein.…”

mentioning

confidence: 99%

Histidinemia in mice: A metabolic defect treated using a novel approach to hepatocellular transplantation

Selden¹,

Calnan²,

Morgan³

et al. 1995

Hepatology

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Histidinemia in mice and in humans is an autosomal recessive disorder of histidine metabolism that leads to high-histidine levels in both plasma and urine and is caused by a lack of hepatic histidine-alpha-deaminase (histidase). We have used a novel approach to hepatocellular transplantation to effect a complete phenotypic cure of histidinemia in a mouse model. Mice lacking histidase were treated with isolated liver cells (approximately 18 x 10(6) hepatocytes and 9 x 10(6) nonparenchymal cells) from histidase-competent donors transplanted into the peritoneum (active transplant group). Recipient mice showed a dramatic decrease, by more than 75%, in urinary histidine levels from day one throughout the course of the experiment, resulting in levels within the normal range for wild-type mice. In comparison, there was no change in urinary histidine levels in the control group of histidase-deficient mice treated with isolated liver cells from mice lacking histidase (statistical comparison between the two groups, P < .003, two-way ANOVA). Histologically, ectopic liver tissue was seen in the peritoneum in association with abdominal wall, pancreas, and peritoneal connective tissue; immunohistochemical evidence showed expression of histidase in the ectopic liver tissue in the active transplant group. This report is the first to show complete correction of a defective biochemical phenotype achieved by hepatocellular transplantation.

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Follow-up study of a nation-wide neonatal metabolic screening program in Japan

et al. 1984

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A nationwide neonatal screening program for phenylketonuria (PKU), maple syrup urine disease (MSUD), homocystinuria, histidinemia and galactosemia was started in Japan in 1977. The total number of infants screened had reached 6,311,754 by March, 1982. A follow-up study revealed the incidence of the disease in Japan: 1/108,823 for PKU; 1/450,840 for hyperphenylalaninemia (HPA); 1/1,577,939 for biopterin deficiency; 1/525,980 for MSUD; 1/1,051,959 for homocystinuria; 1/8,371 for histidinemia, and 1/788,969 for galactosemia type 1. The incidences of PKU, HPA, homocystinuria, and galactosemia (type 1) were found to be markedly low in Japan as compared with those in Caucasian countries. There was no great difference in the incidence of MSUD between both. On the other hand, the incidence of histidinemia was higher in Japan. It was found that most of the patients with PKU, HPA, MSUD, homocystinuria, or galactosemia are developing normally due to the early initiation of dietary treatment. These results clearly indicate that the neonatal mass screening program plays a great role in preventing the occurrence of handicapped children.

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The nutritional therapy of histidinemia

Cited by 8 publications

References 17 publications

Issues in Antenatal and Neonatal Screening and Surveillance for Hereditary and Congenital Disorders

Issues in Antenatal and Neonatal Screening and Surveillance for Hereditary and Congenital Disorders

Histidinemia in mice: A metabolic defect treated using a novel approach to hepatocellular transplantation

Follow-up study of a nation-wide neonatal metabolic screening program in Japan

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