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pyloric stenosis [keywords]
- Hypertrophic pyloric stenosis in twins; genetic or environmental factors. [JOURNAL ARTICLE]
- Clin Genet 2014 Apr 12.
The etiology of infantile hypertrophic pyloric stenosis (IHPS) remains obscure. Over 120 years after the condition has become a clinical entity the debate whether the cause of IHPS is genetic, environmental or both, has not yet reached a final conclusion. Herein, we present a pair of monozygotic male twins with IHPS together with a review of the literature. We aimed to support genetic predisposition in the epidemiology of IHPS, adding a twin data to the literature and to review the associated articles about the pathogenesis and inheritance patterns.
- Advances in infantile hypertrophic pyloric stenosis. [JOURNAL ARTICLE]
- Expert Rev Gastroenterol Hepatol 2014 Apr 10.
Infantile hypertrophic pyloric stenosis (IHPS) is a common condition in infancy, characterized by an acquired narrowing of the pylorus, which requires surgery. These infants usually present with projectile, nonbilious vomiting, with a palpable 'olive' in the abdomen and sometimes a 'peristaltic wave' after being fed with formula or breast milk. Although IHPS is a common disorder, its etiology is largely unknown. Surgical intervention is the standard treatment, preoperative preparation, however is essential to optimal outcome. In this review, the latest advances in IHPS regarding epidemiology, etiology, diagnostics and treatment will be discussed.
- [A case of early antral gastric cancer diagnosed during follow up of pyloric stenosis by the gastro-duodenal ulcer]. [English Abstract, Journal Article]
- Fukuoka Igaku Zasshi 2013 Dec; 104(12):589-94.
A 65-year-old man was admitted to our hospital with nausea, vomiting and appetite loss. First upper endoscopic examination and X-ray examination showed a peptic ulcer and a pyloric stenosis. Fiberscope could not go through the pyloric ring. Computed tomography examination and biopsy showed no evidence of malignancy. Though we considered surgical resection of the stenosis at first, he could eat a staple food with therapy of proton pump inhibitor. So we followed up with upper endoscopic examinations. Second, third and forth upper endoscopic examinations showed no evidence of malignancy. Fifth upper endoscopic examination showed an ulcer scar on the pyloric ring and a 0-IIc carcinoma in the antral greater curvature. Distal gastrectomy with D2 lymph node dissection and B-II reconstruction. Pathologically, a mucosal carcinoma with no lymph node metastasis and U1-III peptic ulcer were diagnosed.
- LIM homeodomain transcription factor Isl1 directs normal pyloric development via targeting Gata3. [JOURNAL ARTICLE]
- BMC Biol 2014 Mar 27; 12(1):25.
Abnormalities in pyloric development or in contractile function of the pylorus cause reflux of duodenal contents into the stomach and increase the risk of gastric metaplasia and cancer. Abnormalities of the pyloric region are also linked to congenital defects such as the relatively common neonatal hypertrophic pyloric stenosis, and primary duodenogastric reflux. Therefore, understanding pyloric development is of great clinical relevance. Here, we investigated the role of the LIM homeodomain (LIM-HD) transcription factor Isl1 in pyloric development.Examination of Isl1 expression in developing mouse stomach by immunohistochemistry (IHC), whole mount in situ hybridization (WISH) and real-time quantitative PCR (RT-qPCR) demonstrated that Isl1 is highly expressed in developing mouse stomach, principally in the smooth muscle layer of the pylorus. Isl1 expression was also examined by immunofluorescence in human hypertrophic pyloric stenosis where the majority of smooth muscle cells were found to express Isl1. Isl1 function in embryonic stomach development was investigated utilizing a tamoxifen inducible Isl1 knockout mouse model. Isl1 deficiency led to nearly complete absence of the pyloric outer longitudinal muscle (OLM) layer at E18.5 which is consistent with Gata3 null mouse phenotype. Chromatin immunoprecipitation (ChIP), luciferase assays, and electrophoretic mobility shift assays (EMSA) revealed that Isl1 ensures normal pyloric development by directly targeting Gata3.This study demonstrates that the Isl1-Gata3 transcription regulatory axis is essential for normal pyloric development. These findings are highly clinically relevant and may help to better understand pathways leading to pyloric disease.
- Brief report adult patient presenting an interstitial (9) (q21.32q31.1) direct duplication resulting from the malsegregation of a paternal balanced insertional translocation. [JOURNAL ARTICLE]
- Birth Defects Res A Clin Mol Teratol 2014 Mar 26.
The partial trisomy 9q syndrome is a well-defined chromosomal disorder with over 40 reported cases in the literature. However, 9q duplications derived from an insertional translocation have rarely been reported.Cytogenetic and molecular analyses using G-banding, fluorescence in situ hybridization, and single nucleotide polymorphism array were performed in a 25-year-old male patient with intellectual disability, behavioral abnormalities, speech delay, postnatal growth retardation, distinctive facial features, and pyloric stenosis.G-banding analysis showed an extra chromosome segment of unknown origin inserted into band 4q25. A 16,747,601 bp duplication of 9q21.32q31.1 inserted into band 4q25 and a balanced (4;9) insertional translocation were identified by single nucleotide polymorphism array and fluorescence in situ hybridization analysis respectively in the patient and his healthy father. A literature review was performed to refine genotype-phenotype correlation of the partial trisomy 9q syndrome.This is the first report on the molecular characterization of a partial trisomy 9q syndrome derived from an insertional translocation between nonhomologous chromosomes. Our findings provide important information for genetic counseling and prenatal diagnosis of future pregnancies in this family. Birth Defects Research (Part A), 2014. © 2014 Wiley Periodicals, Inc.
- The ins and outs of pyloromyotomy: what we have learned in 35 years. [JOURNAL ARTICLE]
- Pediatr Surg Int 2014 Mar 14.
The aim of the study is to evaluate a large series of infantile hypertrophic pyloric stenosis (IHPS) patients treated by one pediatric surgeon focusing on their diagnostic difficulties and complications.From July 1969 to December 2003 (inclusive), the charts of 791 infants with IHPS were retrospectively reviewed.There were 647 (82 %) males and 144 (18 %) females; mean age was 38 days, median 51 (range 7 days-10 months). When ultrasonography (US) was routinely used (1990), the age at diagnosis decreased to <40 days. The mean weight before and after routine US was 3.2 kg, median 3 (range 1.5-6). Twenty-five (3.1 %) were premature at diagnosis, mean age 49 days, median 56, (range 1-3 months) and mean weight 2.5 kg, median 2.3 (range 1.5-3.2). Eighty-one (10 %) had a positive family history. Forty-four (5 %) were non-Caucasians. Seventy-five (9 %) had other medical conditions, anomalies and/or associated findings. Sixty (7 %) patients had abnormal preoperative electrolytes. Ten (1.2 %) pylorics occurred after newborn operations. Of the entire total (791) who were treated, there were 13 (1.7 %) not operated on. All operations were done open initially through one of two right upper quadrant incisions, and then through an upper midline incision under general endotracheal anesthesia; 14 (1.7 %) had concomitant procedures. Prophylactic antibiotics (from 1982) decreased the wound infection rate to 3.9 %. There were a total of 87 (10 %) complications which included 9 (1.1 %) intraoperative, (including mistaken diagnoses) 78 (9 %) postoperative: 59 (2 %) early (<1 month) and 19 (2.4 %) late (>1 month). The 13 (1.6 %) postoperative transfers (12 from non-pediatric surgeons) had 16 (18 %) complications (including 1 death); five (33 %) requiring reoperation (4 incomplete, 1 perforation). There were two deaths.IHPS should be considered in any vomiting infant. US allows earlier diagnosis. Serious complications are uncommon and avoidable, but recognizable and easily corrected. Higher surgeon volume of pyloromyotomies (>14 per year) is associated with fewer complications.
- Use of macrolides in mother and child and risk of infantile hypertrophic pyloric stenosis: nationwide cohort study. [Journal Article, Research Support, Non-U.S. Gov't]
- BMJ 2014.:g1908.
To assess the association between use of macrolide antibiotics in mothers and infants from pregnancy onset until 120 days after birth and infantile hypertrophic pyloric stenosis (IHPS).Nationwide register based cohort study.Denmark, 1996-2011.999,378 liveborn singletons and linked individual level information on macrolide prescriptions (maternal use during pregnancy, n=30,091; maternal use after birth, n=21,557; use in infants, n=6591), surgery for IHPS, and potential confounders.Surgery for IHPS by three categories of macrolide use: in mothers during pregnancy, in mothers after birth, and in infants after birth.880 infants developed IHPS (0.9 cases per 1000 births). Compared with infants with no use of macrolides, the adjusted rate ratio for IHPS in infants with use of macrolides during days 0 to 13 after birth was 29.8 (95% confidence interval 16.4 to 54.1) and during days 14 to 120 was 3.24 (1.20 to 8.74); the corresponding absolute risk differences were 24.4 (95% confidence interval 13.0 to 44.1) and 0.65 (0.06 to 2.21) cases per 1000 infants exposed to macrolides, respectively. The rate ratio for maternal use of macrolides for days 0 to 13 after birth was 3.49 (1.92 to 6.34) and for days 14 to 120 was 0.70 (0.26 to 1.90); the corresponding absolute risk differences were 2.15 (0.82 to 4.64) and -0.11 (-0.26 to 0.31). The rate ratios for maternal use of macrolides during pregnancy were 1.02 (0.65 to 1.59) for weeks 0 to 27 and 1.77 (0.95 to 3.31) for weeks 28 to birth; the corresponding absolute risk differences were 0.01 (-0.31 to 0.50) and 0.67 (-0.06 to 2.02).Treatment of young infants with macrolide antibiotics was strongly associated with IHPS and should therefore only be administered if potential treatment benefits outweigh the risk. Maternal use of macrolides during the first two weeks after birth was also associated with an increased risk of IHPS. A possible association was also found with use during late pregnancy.
- The clinical features of infantile hypertrophic pyloric stenosis in Chinese Han population: analysis from 1998 to 2010. [Journal Article, Research Support, Non-U.S. Gov't]
- PLoS One 2014; 9(2):e88925.
To investigate clinical features of infantile hypertrophic pyloric stenosis (IHPS) in Chinese Han population.Three hundred and sixteen hospitalized patients with IHPS from January 1998 to February 2010 were retrospectively reviewed, and data including patient's sex, onset age, other coexisting congenital defects, pyloric circular muscle thickness evaluated by ultrasonograph, serum electrolytes concentration, and results of arterial blood gas analysis on admission were collected. The patients were divided into two groups: the duration between first onset and admission less than or equal to 10 days (early onset group), and more than 10 days (late onset group). The results of arterial blood gas and serum electrolyte concentration were compared between the two groups.There were 271 males and 45 females in 316 patients; the onset age ranged between 1 and 351 (26.5±26.6) days. The birth weight ranged between 1.6 and 4.5 (3.23±0.44) kilograms; coexisting congenital defects were found in 65 cases (20.6%). Pyloric circular muscle thickness was 4-8 (5.4±1.0) millimetres (mm). For the early onset group, the rates of hypokalemia, hypochloraemia and hypercapnia were significantly lower than those in the late onset group (18.67% VS 50%, P<0.0001; 46.03% VS 71.01%, P = 0.003; 56.58% VS 83.44%, P = 2.17×10(-5); respectively).The symptom duration in Chinese Han population was longer than that in other populations. And as the prolongation of symptom duration, the incidence of acid-base imbalance increased significantly. Infants with persistent vomiting at the age of 3∼5 weeks after birth should be considered IHPS, and go to hospital as soon as possible in order to reduce the incidence of hypokalemia, hypochloraemia and hypercapnia, and avoid deterioration.
- [Pyloric stenosis due to adenocarcinoma of the pylorus]. [English Abstract, Journal Article]
- Rev Col Bras Cir 2013 Sep-Oct; 40(5):430-2.
We present a case of primary malignant pylorus neoplasia, emphasizing its rarity, the difficulty of diagnosis and the importance of an adequate prepare before the endoscopic procedure. Literature shows that tumors compromising the first, third and fourth segments of duodenum are rare, especially those of the pyloric area, representing only 0.35% of all malignant tumors of the gastrointestinal tract. Adenocarcinoma is the most common histological type, representing 50% of all malignant neoplasias at this site. The most common symptoms are weight loss, nausea, vomiting, abdominal pain, abdominal mass, sudden changes in intestinal habits and iron deficiency anemia secondary to chronic intestinal hemorrhage. Survival after five years is only 18%. We report a case of a seventy-one year-old male referring early satiety, epigastric pain, retrosternal burning and dyspepsia, with unspecific results in complementary exams, since complete gastric emptying was not achieved. As symptoms worsened, the patient was submitted to laparotomy, with identification of gastric dilation and severe pyloric stenosis, macroscopically suggesting malignancy. The chosen procedure was a subtotal gastrectomy. Adjuvant radiotherapy and chemotherapy were not used. The patient is been followed-up without tumor recurrence so far.
- The gut or the brain?-gastrointestinal misdiagnoses of infantile brain tumors. [JOURNAL ARTICLE]
- Childs Nerv Syst 2014 Feb 18.
Central nervous system tumors account for the largest number of cancer deaths in childhood. Brain tumors in infants less than 3 years of age are rare; symptoms and signs are often non-specific. Patent anterior fontanelles/unfused cranial sutures in infants can accommodate rising intracranial pressure without acutely compromising the neurological status. We hypothesize that vomiting as the initial symptom, in infants with brain tumors, can possibly lead to extensive gastrointestinal evaluation, delaying the diagnosis of intracranial pathology.We conducted a retrospective chart review of infants less than 3 years of age diagnosed with brain tumors over the period of 4.7 years from February 2008 to October 2012 at Inova Children's Hospital, Virginia.We identified three of 21 patients (14.3 %) who presented with vomiting and underwent initial or extensive abdominal imaging investigations. All patients were relatively young (median age, 5.4 months). Working diagnoses were pyloric stenosis, viral gastritis, or gastroesophageal reflux. All patients eventually had computed tomography of the head to rule out increased intracranial pressure and were found to have large brain tumors with obstructive hydrocephalus. Tumor locations were cerebral hemispheres (2/3) and posterior fossa (1/3). All patients had biologically aggressive high-grade tumors (glioblastoma multiforme, atypical teratoid rhabdoid tumor, and anaplastic/large cell medulloblastoma) and died within weeks of diagnosis.Our study highlights a clinical challenge of persistent vomiting in infants, which in the absence of convincing gastrointestinal pathology after evaluation should raise the physician's suspicion of an underlying intracranial pathology even if neurological features are absent.