hypoproteinemia/tyrosine

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Human milk protein supplementation for the prevention of hypoproteinemia without metabolic imbalance in breast milk-fed, very low-birth-weight infants.

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In a group of 18 infants with birth weights of 1,500 gm or less, either preterm transitional or mature human milk was given during the time of initial hospitalization. Half of the infants were given protein supplement isolated from mature human milk which increased the protein content of the

Liver transplantation in a case of hypoproteinemia and coagulopathy.

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A female infant with hypoproteinemia and coagulopathy associated with hypertyrosinemia was successfully treated with living-related liver transplantation (LRLT). On the 12th day of life plasma amino acid analysis revealed a marked elevation of tyrosine, so the patient was fed on a low-tyrosine and

A new hepato-pancreato-renal disorder resembling tyrosinemia involving neuropathy and abnormal metabolism of polyunsaturated acids.

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This report describes a new disorder resembling hereditary tyrosinemia (HT) but differing from it in several respects. Similarities include failure to thrive with hypoproteinemia, micronodular cirrhosis, alpha-fetoprotein positive hepatocellular carcinoma, renal Fanconi syndrome with renal tubular

Profiles of serum proteins and free amino acids associated with chloroquine retinopathy.

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OBJECTIVE The effects of chronic chloroquine administration on serum proteins and free amino acids of rabbits and the accompanying ultrastructural changes in the retina were investigated. METHODS Thirty pigmented rabbits were injected intramuscularly with chloroquine diphosphate (14 mg/Kg bw).

[A difficult and complicated case study: neonatal intrahepatic cholestasis caused by citrin deficiency].

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Neonatal intrahepatic cholestasis caused by citrin deficiency (NICCD) is a kind of inborn errors of metabolism, with the main clinic manifestations of jaundice, hepatomegaly, and abnormal liver function indices. As a mitochondrial solute carrier protein, citrin plays important roles in aerobic

BLOOD PLASMA PROTEIN PRODUCTION AS INFLUENCED BY AMINO ACIDS : CYSTINE EMERGES AS A KEY AMINO ACID UNDER FIXED CONDITIONS.

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When blood plasma proteins are depleted by bleeding with return of the washed red blood cells (plasmapheresis) it is possible to bring dogs to a steady state of hypoproteinemia and a uniform plasma protein production on a basal low protein diet. These dogs are clinically normal. By the introduction

[Studies on the clinical manifestation and SLC25A13 gene mutation of Chinese patients with neonatal intrahepatic cholestasis caused by citrin deficiency].

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OBJECTIVE To investigate the clinical and laboratory features of neonatal intrahepatic cholestasis caused by citrin deficiency (NICCD) and to characterize the molecular basis and prognosis of this disease. METHODS Twenty-six patients with NICCD were collected because of idiopathic intrahepatic

PLASMA PROTEIN PRODUCTION INFLUENCED BY AMINO ACID MIXTURES AND LACK OF ESSENTIAL AMINO ACIDS : A DEFICIENCY STATE RELATED TO UNKNOWN FACTORS.

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When blood plasma proteins are depleted by bleeding with return of red cells suspended in saline (plasmapheresis) it is possible to bring dogs to a steady state of hypoproteinemia and a constant level of plasma protein production if the diet nitrogen intake is controlled and limited. Such dogs are

BLOOD PLASMA PROTEIN PRODUCTION AND UTILIZATION : THE INFLUENCE OF AMINO ACIDS AND OF STERILE ABSCESSES.

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Effect of short-term essential amino acid-containing dialysate in young children on CAPD.

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In young children on CAPD, hypoproteinemia and malnutrition are often observed. We used essential amino acid-containing dialysate (EAAD) to assess short-term effectiveness on serum amino acid concentrations in young children undergoing CAPD. EAAD consisted of a 540 ml, 1.5% glucose-containing

Utilization of knockout mice to examine the potential role of gastric histamine H2-receptors in Menetrier's disease.

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Menetrier's disease is characterized by giant gastric folds with foveolar hyperplasia and cystic dilatation, hypoproteinemia, and enhanced mucus secretion. The etiology remains unresolved and an effective treatment has yet to be established. Here we show that histamine H(2)-receptor deficient mice

Metabolic derangements in deficiency of citrin, a liver-type mitochondrial aspartate-glutamate carrier.

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Citrin, encoded by SLC25A13, is a liver-type mitochondrial aspartate-glutamate carrier (AGC), of which deficiency, in autosomal recessive trait, causes neonatal intrahepatic cholestasis (NICCD) and adult-onset type II citrullinemia (CTLN2). NICCD patients have jaundice, hypoproteinemia,

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