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  1. Walkingshaw R
    Matched MeSH terms: Hirschsprung Disease
  2. Thambi Dorai CR, Hamzaini H, Rohana R
    Clin Anat, 2010 May;23(4):455-9.
    PMID: 20196129 DOI: 10.1002/ca.20949
    A baby girl with prenatal diagnosis of complex cardiac anomalies and diaphragmatic hernia was born at 36 weeks of gestation. At 4 hr of life, the baby developed respiratory distress and was intubated. She was found to have right hetetrotaxy with total anomalous pulmonary venous drainage into the portal vein, five hepatic veins draining the liver and intrathoracic herniation of the stomach. The child also developed abdominal distension on the second day of life with passage of scanty meconium. The diagnosis of Hirschsprung's disease (HD) was confirmed by histology. HD in association with right heterotaxy has not been reported earlier. The association of heterotaxy with HD in our patient raises a possible genetic link between the two anomalies that needs further research.
    Matched MeSH terms: Hirschsprung Disease/pathology*
  3. Gunadi, Juwitasari T, Damayanti NNR, Kaniashari DS, Kencana SMS, Hastuti J
    Med J Malaysia, 2020 05;75(Suppl 1):28-31.
    PMID: 32483104
    INTRODUCTION: Despite various definitive methods that are used for treating Hirschsprung's disease (HSCR), there are few studies comparing the effect of different pull-through methods on the growth outcomes of patients. We aimed to compare the effect of three different pull-through methods, namely Duhamel, Soave and transanal endorectal pullthrough (TEPT), on HSCR growth outcomes of patients.

    METHODS: Medical records of all HSCR patients who underwent pull-through at the Dr. Sardjito Hospital, Indonesia between January 2010 and August 2016 were reviewed for their growth outcomes before and after the surgery.

    RESULTS: We included 64 HSCR patients, 45 males and 19 females, of which 14, 17, and 33 patients underwent Duhamel, Soave, and TEPT respectively. There were no nutritional status differences in HSCR patients after Duhamel, Soave, and TEPT surgery (p=0.07, 0.17, and 0.79, respectively). Z-score average of weight-for-age did not differ between three surgical methods (p=0.77 and 0.15 for preoperative and postoperative, respectively). In addition, the improvement of nutritional status was achieved in 21.2% HSCR patients after TEPT, 14.3% post Duhamel and 5.9% following Soave procedure, but these differences did not reach a significant level (p=0.34).

    DISCUSSION: Our study shows no difference in effect on the growth outcomes in HSCR patients following Duhamel, Soave and TEPT procedure. Further study with a larger sample size is important to give valuable long-term growth outcomes for HSCR patients after pull-through.

    Matched MeSH terms: Hirschsprung Disease/surgery*
  4. Ziad F, Katchy KC, Al Ramadan S, Alexander S, Kumar S
    Ann Saudi Med, 2006;26(3):200-4.
    PMID: 16861859
    BACKGROUND: Hirschsprung disease [HD] is a predominantly childhood disorder of intestinal motility with a multifactorial and polygenic etiology. The objective of this study was to document the clinical and pathological features of HD in Kuwait, which has an estimated consanguinity rate of 54%.

    METHODS: We analyzed all rectal and colonic biopsies (n=268) for suspected HD identified from the records in the Pathology Department of Al-Sabah Hospital for the period between 1994 and 2004.

    RESULTS: One hundred and two patients (87 males and 15 females) had histologically confirmed HD. Fifty-eight (57%) were neonates (<1 month of age), while 21% were more than 4 months old. The diagnosis was based on open biopsy in 11 cases and rectal biopsies in 91 cases. Nine patients with open biopsies presented as intestinal obstruction, necrotizing enterocolitis, or perforation. The extent of the disease was unknown in 13 patients. There were 67 males and 3 females with short segment HD. Nine had long segment, two ultra-short segment and eight total colonic aganglionosis (TCA). Five TCA cases involved the small intestine. A skip area was observed in two cases. Six patients had other anomalies. A positive family history for HD was established in three patients. Two of these were male siblings from a consanguineous marriage and had Waardenburg syndrome.

    CONCLUSION: This study has highlighted an exceptionally strong male predominance of short segment and a relatively high frequency (5.6%) of small intestinal involvement in HD in Kuwait. These data call for a more detailed epidemiological study with special emphasis on genetics.

    Matched MeSH terms: Hirschsprung Disease/pathology*
  5. Ramesh JC, Ramanujam TM, Yik YI, Goh DW
    J Pediatr Surg, 1999 Nov;34(11):1691-4.
    PMID: 10591572
    The authors evaluated the safety and benefits of 1-stage pull-through in comparison with staged repair of Hirschsprung's disease under circumstances prevailing in a developing country.
    Matched MeSH terms: Hirschsprung Disease/diagnosis; Hirschsprung Disease/mortality; Hirschsprung Disease/surgery*
  6. Chen CK, Teo SC, Phui VE, Saman MA
    Agri, 2015;27(4):210-4.
    PMID: 26860495 DOI: 10.5505/agri.2015.66487
    The application of ultrasound-guided transversus abdominis plane (TAP) block in paediatric population is gaining popularity among anaesthetists. We present a case series of ultrasound-guided TAP block in ten neonate and infants undergoing colostomy and reversal of stoma. Classical TAP as described by Hebbard was carried out and a maximum dosage of 1ml/kg of 0.25% levobupivacaine was injected. Pain score was assessed using Neonatal Infant Pain Scale for 24 hours. In all patients, the block was successful with minimal hemodynamic changes intraoperatively and no additional systemic analgesia was needed intraoperative and immediate postoperatively. Ultrasound-guided TAP block has an important role in providing safe and effective analgesia for colostomy creation and reversal of stoma surgeries in paediatric population.
    Matched MeSH terms: Hirschsprung Disease/surgery
  7. Balasubramaniam S, Keng WT, Ngu LH, Michel LG, Irina G
    Singapore Med J, 2010 Mar;51(3):e54-7.
    PMID: 20428734
    Mowat-Wilson syndrome (MWS) is a recently delineated mental retardation; a multiple congenital anomaly syndrome characterised by a typical facial gestalt, Hirschsprung disease or severe constipation, genitourinary anomaly, congenital heart defects, agenesis of corpus callosum and eye defects. Some cases also present with epilepsy, growth retardation with microcephaly and speech impairment. MWS was first described in 1998 by Mowat et al, and approximately 180 cases have been reported as of August 2008. The syndrome occurs as a result of heterozygous mutations or deletions in the zinc finger E-box-binding homeobox 2 gene, ZEB2, previously called ZFHX1B (SIP1). Most cases reported so far were sporadic occurrences; however, rare cases of sibling recurrence have been cited. The facial phenotype is particularly important for the initial clinical diagnosis and provides the hallmark, warranting ZEB2 mutational analysis even in the absence of Hirschsprung disease. We present the first two molecularly confirmed Malaysian MWS patients, one of whom has a novel mutation.
    Matched MeSH terms: Hirschsprung Disease/genetics
  8. Hutson JM, Hynes MC, Kearsey I, Yik YI, Veysey DM, Tudball CF, et al.
    Pediatr Surg Int, 2020 Jan;36(1):11-19.
    PMID: 31673760 DOI: 10.1007/s00383-019-04587-x
    Children with chronic idiopathic constipation (CIC) often end up at the surgeon when medical treatments have failed. This opinion piece discusses a recently described pattern of CIC called 'Rapid transit constipation (RTC)' first identified in 2011 as part of surgical workup. RTC was identified using a nuclear medicine gastrointestinal transit study (NMGIT or nuclear transit study) to determine the site of slowing within the bowel and to inform surgical treatment. Unexpectedly, we found that RTC occured in 29% of 1000 transit studies in a retrospective audit. Irritable bowel syndrome (IBS) occurs in 7-21% of the population, with a higher prevalence in young children and with constipation type dominating in the young. While 60% improve with time, 40% continue with symptoms. First-line therapy for IBS in adults is a diet low in fermentable oligosaccharides, disaccharides, monosaccharides and polyols which reduces symptoms in > 70% of patients. In children with functional gastrointestinal disorders, fructose intolerance occurs in 35-55%. Reducing fructose produced significant improvement in 77-82% of intolerant patients. In children with RTC and a positive breath test upon fructose challenge, we found that exclusion of fructose significantly improved constipation, abdominal pain, stool consistency and decreased laxative use. We hypothesise that positive breath tests and improvement of pain and bowel frequency with sugar exclusion diets in RTC suggest these children have IBS-C. These observations raise the possibility that many children with CIC could be treated by reducing fructose early in their diet and this might prevent the development of IBS in later life.
    Matched MeSH terms: Hirschsprung Disease/surgery
  9. Chong PP, Chieng DC, Low LY, Hafeez A, Shamsudin MN, Seow HF, et al.
    J Med Microbiol, 2006 Apr;55(Pt 4):423-428.
    PMID: 16533990 DOI: 10.1099/jmm.0.46045-0
    The incidence of candidaemia among immunocompromised patients in Malaysia is increasing at an alarming rate. Isolation of clinical strains that are resistant to fluconazole has also risen markedly. We report here the repeated isolation of Candida tropicalis from the blood of a neonatal patient with Hirschsprung's disease. In vitro fluconazole susceptibility tests of the eight isolates obtained at different time points showed that seven of the isolates were resistant and one isolate was scored as susceptible dose-dependent. Random amplification of polymorphic DNA fingerprinting of the isolates using three primers and subsequent phylogenetic analysis revealed that these isolates were highly similar strains having minor genetic divergence, with a mean pairwise similarity coefficient of 0.893+/-0.041. The source of the infectious agent was thought to be the central venous catheter, as culture of its tip produced fluconazole-resistant C. tropicalis. This study demonstrates the utility of applying molecular epidemiology techniques to complement traditional mycological culture and drug susceptibility tests for accurate and appropriate management of recurrent candidaemia and highlights the need for newer antifungals that can combat the emergence of fluconazole-resistant C. tropicalis strains.
    Matched MeSH terms: Hirschsprung Disease/complications*
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