Familial enteropathy: A syndrome of protracted diarrhea from birth, failure to thrive, and hypoplastic villus atrophy

Davidson, Garry J.; Cutz, Ernest; Hamilton, J. R.; Gall, David

doi:10.1016/0016-5085(78)90458-4

Cited by 243 publications

(169 citation statements)

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“…Goblet cells have scarcely been studied and are described as cells with normal morphology and distribution [3,8]. However, in a recent study Kučinskiené et al (2004) [16] described a decreased number of goblet cells with low mucus secretion in a MID case.…”

Section: Discussionmentioning

confidence: 98%

“…Microvillous inclusion disease (MID), or congenital microvillous atrophy [7], is a serious disorder within the syndrome of intractable diarrhea of infancy [8]. It appears to be transmitted as an autosomal recessive trait [7], although the relevant molecular abnormality has not been identified.…”

Section: Introductionmentioning

confidence: 99%

“…The main histological features of the disease include diffuse villous atrophy without inflammatory changes and accumulation of periodic acid-Schiff (PAS)-positive material within the apical cytoplasm of enterocytes [7,8,22,23,31]. The definitive diagnosis of MID rests on distinctive ultrastructural findings: microvillous inclusions (more frequently in villous enterocytes), increased electrondense secretory granules (preferentially in crypt epithelial cells), and poorly developed brush-border microvilli on the intestinal surface epithelium [3,7,22,23,31,33].…”

Section: Introductionmentioning

confidence: 99%

“…We investigated both the enterocytes and the goblet cells, which have poorly been studied in MID [3,8]. The ultrastructural features of MID goblet cells were compared with those of controls.…”

Section: Introductionmentioning

confidence: 99%

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Unusual ultrastructural features in microvillous inclusion disease: a report of two cases

et al. 2006

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Microvillous Inclusion Disease (MID) is an inherited disorder characterized by intractable diarrhea in infancy. Ultrastructural detection of pathognomonic microvillous inclusions in the enterocytes is essential for diagnosis. The aim of this research is to contribute to the knowledge of MID studying enterocytes and goblet cells (gc). Samples of duodenal mucosa from two young infants with MID (aged 75 days and 3 months, respectively) were studied by light and electron microscopy. Detection in the intestinal villi of immature gc (with microvilli) in one of the cases led us to seek them in control samples. The total number of gc with microvilli (immature) and without microvilli (mature) were counted. In both MID specimens, light microscopy showed atrophy of villi and PAS-positive material in the enterocyte cytoplasm. The ultrastructure of villous enterocytes was characterized by brush-border abnormalities, microvillous inclusions, dense apical granules, and lysosomes. Intermediate structures between microvillous inclusions and lysosomes were also detected within a cell, as were rare microvilli on the lateral membrane of the enterocytes. In one MID specimen, immature gc were also identified in the absorptive compartment. Only mature gc were observed in the controls. The significance of the latter finding requires further studies.

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Section: Discussionmentioning

confidence: 98%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Unusual ultrastructural features in microvillous inclusion disease: a report of two cases

et al. 2006

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“…MID is a very rare disease. It was first described by Davidson et al (1978). The pathognomonic features of MID are hypoplastic villus atrophy with electron microscopic findings of intracellular brush border inclusions (Cutz et al, 1989).…”

Section: Microvillus Inclusion Disease Datamentioning

confidence: 99%

Is the Exact Test Better than the Asymptotic Test for Testing Marginal Homogeneity in 2 × 2 Tables?

Park

2002

Biom. J.

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McNemar test is commonly used to test for the marginal homogeneity in 2 × 2 contingency tables. McNemar test is an asymptotic test based either on standard normal distribution or on the chi‐square distribution. When the total sample size is small, an exact version of McNemar test is available based on the binomial probabilities. The example in the paper came from a clinical study to investigate the effect of epidermal growth factor for children who had microvillus inclusion diseases. There were only six observations available. The test results differ between the exact test and the asymptotic test. It is a common belief that with this small sample size the exact test be used. However, we claim that McNemar test performs better than the exact test even when the sample size is small. In order to investigate the performances of McNemar test and the exact test, we identify the parameters that affect the test results and then perform sensitivity analysis. In addition, through Monte Carlo simulation studies we compare the empirical sizes and powers of these tests as well as other asymptotic tests such as Wald test and the likelihood ratio test.

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The Fat Overload Syndrome

Heyman¹

1981

Am J Dis Child

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Familial enteropathy: A syndrome of protracted diarrhea from birth, failure to thrive, and hypoplastic villus atrophy

Cited by 243 publications

References 16 publications

Unusual ultrastructural features in microvillous inclusion disease: a report of two cases

Unusual ultrastructural features in microvillous inclusion disease: a report of two cases

Is the Exact Test Better than the Asymptotic Test for Testing Marginal Homogeneity in 2 × 2 Tables?

The Fat Overload Syndrome

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