Congenital Lactose Intolerance of Gastrogen Origin Associated With Cataracts

Russo, Giuseppe; Molcica, Florindo; Mazzone, D; Santonocito, B

doi:10.1111/j.1651-2227.1974.tb04830.x

Cited by 7 publications

(3 citation statements)

References 10 publications

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“…Ru sso et a\. (29) have confirmed these findings. At 8 months of age, two intraduodenal L TT gave no lactos uria in their patient , whereas two oral L TT were followed by lactosuria.…”

Section: Discussionmentioning

confidence: 70%

“…Holzel (18) reported normal lactase activity after recovery of the acu te phase of the di ease, but hi s patient was younger at biopsy. Ru sso et al (29) reported from Sicily that four adult male family members had cow's milk intolerance and that 250 ml of cow's milk orally gave each member abdominal pain and lactosuria. Two of eight controls got abdominal pain but no lactosuria.…”

Section: Discussionmentioning

confidence: 99%

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A Boy With Severe Infantile Gastrogen Lactose Intolerance and Acquired Lactase Deficiency

1979

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A 10-year-old boy with severe familial lactose intolerance in infancy (vomiting, failure to thrive, lactosuria (5.25 g/l), sucrosuria (12 g/l), and aminoaciduria. Intestinal disaccharidases (including lactase and sucrase) normal at age 6 and 20 weeks. Oral lactose tolerance test at this age resulted in lactosuria (4.6 g/l); sucrose tolerance test, in sucrosuria (18.5 g/l). In contrast, intraduodenal lactose tolerance test gave only low lactose excretion in urine (0.28 g/l). He improved rapidly and had no lactosuria on intraduodenal feeding with citric acid milk. The lactosuria diminished as age increased, but was still higher at age 6 years than that of controls. He tolerated normal disaccharide containing food after 1.5 years of age. At 5.5 to 6 years, he had symptoms of lactose malabsorption, and an isolated lactase deficiency was proved. At 10 years, he still tolerates only limited amounts of milk. The defect in severe familial infantile lactose intolerance seems to be localized in the gastric mucosa. Acquired lactase deficiency can appear later in childhood in this syndrome.

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“…Ru sso et a\. (29) have confirmed these findings. At 8 months of age, two intraduodenal L TT gave no lactos uria in their patient , whereas two oral L TT were followed by lactosuria.…”

Section: Discussionmentioning

confidence: 70%

Section: Discussionmentioning

confidence: 99%

A Boy With Severe Infantile Gastrogen Lactose Intolerance and Acquired Lactase Deficiency

1979

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“…In 1954, three patients with mental retardation, hiatus hernia, and sucrosuria were described (1) and later an additional twelve patients with a rather similar presentation were reported (2) although the possible causal relationship between the mellituria and the mental retardation was clearly and properly dismissed. Two groups of workers have now demonstrated that the lactosuria of familial lactose intolerance can be circumvented by intraduodenal feeding, and have suggested that the abnormality in the absorption of the disaccharide (2) was localized to the gastric mucosa (3,4).…”

Section: Benign Disacchariduriamentioning

confidence: 99%

Benign Disacchariduria

Potter

1981

Acta Paediatrica

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Pregnancy-exaggerated galactosemia and congenital cataracts

Ramakrishnan¹,

Sulochana²,

Punitham³

et al. 1998

Indian J Pediatr

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One child in a family and two children in another family had galactosemia and congenital cataract. Two of them had total soft cataracts while in one, cataract was less soft. In addition, they had mild lactosuria. The mothers of the affected children had significant lactosuria and mild galactosuria without cataracts. Fathers did not have galactosuria or lactosuria. Clinically unaffected siblings in one family had mild galactosuria and lactosuria. Pregnancy-exaggerated galactosemia was suspected in these two mothers who gave birth to children with congenital cataract. As an extension of this work, 5001 pregnant women were screened for galactose in urine just before the delivery of babies. Mild galactosuria was present in 54 (1.08%). Three children had congenital cataract and one had changes in posterior pole and cornea. Restriction of lactose by reducing intake of milk and milk products during pregnancy by mothers with galactosuria is recommended to avoid the birth of children with congenital cataract.

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Congenital Lactose Intolerance of Gastrogen Origin Associated With Cataracts

Cited by 7 publications

References 10 publications

A Boy With Severe Infantile Gastrogen Lactose Intolerance and Acquired Lactase Deficiency

A Boy With Severe Infantile Gastrogen Lactose Intolerance and Acquired Lactase Deficiency

Benign Disacchariduria

Pregnancy-exaggerated galactosemia and congenital cataracts

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