Infantile pyloric stenosis associated with major anomalies of the alimentary tract

Ahmed, Saeed

doi:10.1016/s0022-3468(70)80013-6

Cited by 22 publications

(15 citation statements)

References 17 publications

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“…27 32 Nowadays the prognosis for infants with pyloric stenosis depends on the associated anomalies, which are present in 6 to 20% of patients. 1 3 14 16 33 The association with oesophageal atresia is well known4 6 14 33 34 but sometimes difficult to establish without radiological help, especially in those with a gastrostomy. The association with rectal agenesis4 or Hirschsprung's disease'8 is less well known, as is that with cleft lip and palate,16 which makes feeding difficult.…”

Section: Resultsmentioning

confidence: 99%

Recent results of treatment of infantile hypertrophic pyloric stenosis.

Zeidan

Wyatt

Mackersie

et al. 1988

Archives of Disease in Childhood

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Section: Resultsmentioning

confidence: 99%

Recent results of treatment of infantile hypertrophic pyloric stenosis.

Zeidan

Wyatt

Mackersie

et al. 1988

Archives of Disease in Childhood

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“…Case reports and reviews of gastrointestinal anomalies associated with HSCR suggest that pyloric stenosis has an association with both nonsyndromic HSCR and with intestinal neuronal dysplasia, but the mechanism by which this occurs is as yet unclear [Ahmed, 1970;Whalen and Asch, 1985;Sayed and al-Alaiyan, 1996;Martucciello et al, 2002].…”

Section: Discussionmentioning

confidence: 99%

Further delineation of the phenotype associated with heterozygous mutations in ZFHX1B

Wilson

Mowat

Moal

et al. 2003

American J of Med Genetics Pt A

101

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Mutations or deletions involving ZFHX1B (previously SIP1) have recently been found to cause one form of syndromic Hirschsprung disease (HSCR), associated with microcephaly, mental retardation, and distinctive facial features. Patients with the characteristic facial phenotype and severe mental retardation, but without HSCR, have now also been shown to have mutations in this gene. Mutations of ZFHX1B are frequently associated with other congenital anomalies, including congenital heart disease, hypospadias, renal tract anomalies, and agenesis of the corpus callosum (ACC). We present the clinical data and mutation analysis results from a series of 23 patients with this clinical syndrome, of whom 21 have proven ZFHX1B mutations or deletions (15 previously unpublished). Two patients with the typical features (one with and one without HSCR) did not have detectable abnormalities of ZFHX1B. We emphasize that this syndrome can be recognized by the facial phenotype in the absence of either HSCR or other congenital anomalies, and needs to be considered in the differential diagnosis of dysmorphism with severe mental retardation +/- epilepsy.

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“…Other complications that tended to cause confusion and were associated with delay in diagnosis included Meckel's diverticulitis, 'enterocolitis' of the stomach or intestine, malrotation and volvulus, duodenal stenosis, pyloric stenosis, and peritonitis due to leakage from a gastrostomy. Several of these complications have been recorded (Schwartz and Dale, 1955;Berg, 1956;Ahmed, 1970;Glasson et al, 1973), and in particular those consequent upon gastrostomy (Connor and Sealey, 1956;Holder et al, 1964;Cozzi and Wilkinson, 1967;Glasson et al, 1971;Koop et al, 1974). 3 babies in our series died during the initial admission because of complications arising from the gastrostomy.…”

Section: Discussionmentioning

confidence: 98%

Preventable death in oesophageal atresia.

Brereton¹,

Zachary²,

Spitz³

1978

Archives of Disease in Childhood

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SUMMARY We report on experience gained in the treatment of 158 cases of oesophageal atresia presenting during a period of 10 years. The factors influencing mortality were analysed. During the period studied there was a slight improvement in survival, and this was probably due mainly to improved preoperative preparation of those babies undergoing primary repair. At best, 'staging' was thought to have had little influence on the survival of poor risk cases. Midwives, obstetricians, paediatricians, surgeons, and general practitioners did not do all that they could have done to prevent morbidity and mortality in these babies. At least one-third of the 79 deaths could have been prevented, and several deaths were caused solely by lack of awareness of the possible complications and associated abnormalities.Although much progress has been made in the treatment of infants with oesophageal atresia since 1943 (Haight and Towsley), before which time practically all cases died, there is still an unacceptably high mortality rate for 'poor risk' cases. Formerly, technical errors were responsible for many deaths, but advances in operative techniques, preoperative management, and paediatric anaesthesia have succeeded in substantially reducing the overall mortality rate. Since it is unlikely that any new operation of major importance will be developed in the foreseeable future, it is necessary to look to other factors in order to improve survival in these infants, particularly the early nursing and paediatric care, the preoperative management and choice of operation by the surgeon, and the subsequent community and environmental care.

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Infantile pyloric stenosis associated with major anomalies of the alimentary tract

Cited by 22 publications

References 17 publications

Recent results of treatment of infantile hypertrophic pyloric stenosis.

Recent results of treatment of infantile hypertrophic pyloric stenosis.

Further delineation of the phenotype associated with heterozygous mutations in ZFHX1B

Preventable death in oesophageal atresia.

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