Does Maternal Omega 3 Supplementation Protect Against Infantile Hypertrophic Pyloric Stenosis?

OBJECTIVES: Infantile hypertrophic pyloric stenosis (IHPS) is potentially life threatening. The etiology of IHPS remains unknown and many risk factors have been reported. We aimed to assess the prevalence of known risk factors and investigate maternal nutrition and habits as possible additional risk factors for IHPS. METHODS: This case-control study includes mothers of infants diagnosed with IHPS and control mothers of infants, age 2 to 11 months, hospitalized in the pediatric department due to other conditions. Cases of IHPS were identified by review of all infants diagnosed with IHPS and operated upon in 2010 to 2016 at 2 major hospitals in central Israel. Data regarding potential risk factors were collected via questionnaires in both study groups. RESULTS: Sixty-six cases and 67 controls were included in the study. Maternal omega 3 supplement consumption during pregnancy was significantly less common among cases of IHPS as compared with controls (P = 0.031). Consumption of omega 3 supplement was defined as consumption of at least 1 to 2 per week during the pregnancy period. Following adjustment for known risk factors, including male sex and maternal smoking, maternal omega 3 supplement consumption remained associated with a significantly lower risk of developing IHPS (odds ratio = 0.303, 95% confidence interval 0.111-0.828, P = 0.02). CONCLUSIONS: Maternal omega 3 supplement consumption during pregnancy was associated with a significantly reduced risk of IHPS. Further studies are needed to support these results and investigate possible mechanisms of the effect of omega 3.

The incidence of infantile hypertrophic pyloric stenosis and its association with folic acid supplementation during pregnancy: A nested case-control study.

BACKGROUND AND RATIONALE: Several studies have suggested that the incidence of infantile hypertrophic pyloric stenosis (IHPS) has decreased in recent decades. This decrement is controversial and not fully explained. Concurrently, there has been a major increase in folic acid consumption by pregnant women to prevent neural tube defects. We aimed to describe IHPS incidence in Israel in recent years and to assess its potential association with folic acid consumption. METHODS: Using the electronic medical database of a 2.1 million member health organization in Israel, we identified all cases (n = 1899) of IHPS occurring between 1999 and 2015. Cases were individually matched with up to 5 controls (n = 7350) by birth date, sex, and region. Odds ratios and 95% confidence intervals by tertiles of cumulative dose of supplemented folic acid between three months prior to pregnancy and up to birth of index child were calculated using conditional logistic regression. RESULTS: During the study period IHPS incidence declined from 4.3 in 1999 to 2.1 per 1000 live births in 2015(p < 0.0001). No significant (p = 0.81) association was observed between folic acid intake during pregnancy and risk of IHPS incidence. Preterm birth and infant's use of macrolides during first 3 postnatal months were significantly (p < 0.01) associated with increased risk of IHPS. CONCLUSIONS: Similar to other countries, IHPS incidence in Israel has decreased in recent years. The decrement cannot be explained by increased use of folic acid. TYPE OF STUDY: Case Control Study. LEVEL OF EVIDENCE: Level III. SUMMARY: Using linkage to a large electronic patient database, this study investigated the association between the decrease in infantile hypertrophic pyloric stenosis and maternal exposure to folic acid during pregnancy.

A case of Menkes disease caused by novel mutation in the ATP7A gene with infantile hypertrophic pyloric stenosis / 대한소아신경학회지

Menkes disease is caused by mutations in the ATP7A gene that lead to intracellular copper transport defects and characterized by brownish twisted (kinky) hair accompanied by growth retardation and intellectual disability. Reduced nitric oxide (NO) production contributes to infantile hypertrophic pyloric stenosis (IHPS) because NO plays an important role in smooth muscle relaxation. Here we describe a case of Menkes disease and IHPS in a 72-day-old male patient with severe persistent vomiting and convulsions with a novel ATP7A mutation.

Open transumbilical pyloromyotomy: is it more painful than the laparoscopic approach?

BACKGROUND: Open transumbilical pyloromyotomy (UMBP) and laparoscopic pyloromyotomy (LAP) have been compared on different outcomes, but postoperative pain as a primary end point had never been assessed. The aim of this study was to compare the use of analgesia in UMBP and LAP patients. METHODS: Infants with hypertrophic pyloric stenosis treated by UMBP in 2008-2009 were matched with LAP-treated infants. Demographics, type and use of analgesia, and length of stay were recorded. Statistical analysis was performed using the Fisher exact test. RESULTS: Each group contained 19 patients (N = 38) with comparable demographics and no comorbid condition. Bupivacaine was injected intraoperatively in all UMBP and 89% of LAP infants. There was a trend toward increased acetaminophen use in LAP infants (79% vs 58%, P = .61) in the recovery room. There was no difference in opiates use (3 UMBP vs 1 LAP, P = .60). In the ward, more UMBP patients received acetaminophen (78% vs 53%, P = .03). This difference was significant. Mean postoperative length of stay was similar in both groups. CONCLUSION: Our study suggests that UMBP infants might experience more postoperative pain in the ward, without any impact on various outcomes. A prospective study with a larger sample size should be undertaken to verify these findings.

Epidemiological features of infantile hypertrophic pyloric stenosis in Taiwan: a national study 1996-2004.

BACKGROUND AND AIM: The incidence of infantile hypertrophic pyloric stenosis (IHPS) varies among different countries and is supposed to be lower in Asian countries than in Western countries. However, the incidence of IHPS in Taiwan has not been well investigated. METHODS: The National Health Insurance (NHI) program was implemented in Taiwan in 1995 and covers most of the population (>99%). We used the NHI database to investigate the epidemiological features of IHPS in Taiwan and to compare the data with that of other countries. RESULTS: We identified 962 new IHPS cases during the period from 1996 to 2004. The overall incidence of IHPS was 0.39 (0.34-0.50) cases per 1000 live births. The estimation was 0.39-0.59 per 1000 live births after adjustment for the misdiagnosis rate. The peak incidence (0.58 per 1000 live births) occurred in winter in 1999. Rates were consistently higher in male subjects. The 1-year survival rate was not significantly different in the patients receiving pyloromyotomy in medical centers, regional hospitals, and district hospitals (P=0.389). CONCLUSIONS: Taiwan had the second lowest incidence of IHPS reported in the medical literature. IHPS patients can be successfully treated in district and general hospitals with good prognosis.

Delayed presentation of hypertrophic pyloric stenosis: a rare case.

Hypertrophic pyloric stenosis (HPS) is the most common cause of gastric outlet obstruction in infants, with an incidence of 1.5 to 3 per 1000 live births, and classically presents at 3 to 4 weeks of age. Delayed presentation of HPS is an extremely rare occurrence after early infancy. With the exclusion of congenital HPS, gastric outlet obstruction in childhood is a rare condition (1:100,000). We report a case of delayed presentation of HPS in a 4 1/2-year-old child in whom the pylorus was hypertrophied and appeared like an "olive," and for which pyloromyotomy was curative.

Estenose hipertrófica do piloro: caracterização clínica, radiológica e ecográfica / Hypertrophic pyloric stenosis: clinical, radiographic and sonographic characterization

Estenose hipertrófica do piloro é uma condição comum em infantes com 2 - 12 semanas de idade e cuja causa permanece desconhecida. O diagnóstico clínico baseia-se na história de vômitos não-biliosos em jato, sinais de hiperperistalse gástrica e "tumor" pilórico palpável ao exame físico. Os autores ilustram os típicos achados desta alteração em seriografias do trato gastrointestinal superior e na ultra-sonografia abdominal. Critérios diagnósticos são descritos e as aplicações desses dois métodos são estabelecidos segundo a literatura vigente

Hypertrophic pyloric stenosis is a common condition in infants with 2 - 12 weeks of postnatal life. The cause of this disease remains obscure. Clinical diagnosis is based on the history of projectile, nonbilious vomiting, gastric hyperperistalsis and a palpable pyloric "tumor". The authors review the typical findings seen on upper gastrointestinal x-ray series and abdominal ultrasonography. The diagnostic criteria for hypertrophic pyloric stenosis are presented and the applications of these two methods are established on the basis of the current literature.

A Case of Pseudo-Bartter's Syndrome Due to Hypertrophic Pyloric Stenosis

We report a 3-month old boy admitted to our hospital with Bartter's syndrome like symptoms and laboratory findings, which were vomiting, failure to thrive, hypochloremic and hypokalemic metabolic alkalosis associated with hyperreninemia, hyperaldosteronism and normal blood pressure. However, the urine chloride level was low. Hypertrophic pyloric stenosis was diagnosed through abdominal ultrasonography. Fredet-Ramstedt operation was done after electrolyte correction. After surgery he made a good recovery and gained body weight. The electrolytes maintained within a normal limit without any potassium supplementations after surgery. Differential diagnosis from Bartter's syndrome was made on the basis of a decrease in urine chloride and the non-necessity for potassium supplementation after surgery. It is relatively rare for hypertrophic pyloric stenosis to induce pseudo-Bartter's syndrome. The importance of considering this diagnosis in such cases is discussed.

Reduced Messenger RNA Expression of the Neuronal Nitric Oxide Synthase Gene in Infantile Hypertrophic Pyloric Stenosis

PURPOSE: Nitric oxide synthesized by neuronal nitric oxide synthase (nNOS) has been described as a mediator of smooth muscle relaxation in the mammalian gastrointestinal tract. Impaired expression of the nNOS gene is suggested in the development of infantile hypertrophic pyloric stenosis (IHPS). We examined the expression of nNOS mRNA in pyloric muscle biopsy specimens obtained from 8 patients with IHPS and attempted to correlate the results with the clinical characteristics. METHODS: The expression of nNOS mRNA in pyloric muscle biopsy specimens for 8 patients with IHPS was examined using a reverse transcription- polymerase chain reaction (RT-PCR) technique. For the control, a smooth muscle layer specimen of a neonate with a normal pylorus was used. RESULTS: In the control specimen, the level of nNOS mRNA expression was 48.4% of beta-actin mRNA. In the two thinnest (each 3 mm) of pyloric muscle thicknesses as determined by ultra-sonography, the expressed nNOS mRNA were 16.7% and 30.3%. The two thickest (each 8.3 mm) expressed as 35.3% and 22.9% nNOS. The two samples from the earliest age of symptomatic onset (1 day, 7 days after birth) expressed as 25.6% and 4.8%. The two from the latest age of onset (each 30 days) expressed as 7.4% and 10.5%. The control specimen revealed a higher level of nNOS mRNA expression than those of the IHPS specimens. There was no significant correlation between the clinical characteristics and the levels of nNOS mRNA in the IHPS specimens. CONCLUSION: Since a low level of nNOS mRNA expression may lead to impaired production of NO, our observations indicate that the hypertrophic pyloric muscle of an IHPS patient may be the result of a reduced expression of the nNOS gene at the mRNA level. In IHPS patients, there was no correlation between the clinical characteristics and the levels of expressed nNOS mRNA.

Radiological evaluation congenital gastrointestinal tract anomalies

With the improvements, during recent years, in the control of the infections and nutritional diseases thesubject of congenital malformation becomes of increasing importance. The radiologic signs are crucial for promptdiagnosis of anomalies of alimentary tract and with early identification of resulting complication, surgicaltherapy is usually life-saving. 30 cases of congenital anomalies of alimentary tract in infants were reviewed inrespect of age, sex, incidence and radiological findings. 1. The most common lesion was hypertrophic pyloricstenosis, followed by congenital megacolon and anorectal anomaly, tracheoesophageal fistula, intestinal atresia.2. Male outnumbered female in most congenital anomalies of alimentary tract. 25 cases were under the age of 1month. 3. Common symptoms of upper gastrointestinal tract obstruction are vomiting and abdominal distention. Inthe obstruction of lower gastrointestinal tract, abdominal distention and failure of meconium passage were noted.4. Roentgenologic finding were as follows, a. Chest A-P and lateral view; In tracheoesophageal fistula, sacculardilatation of upper esphagus and displacement of trachea anterolaterally were the most common finding. b. Simpleabdomen: Obstructive pattern of proximal portion of duodenum shows in 11 cases, of distal bowel shows in 16 cases.Duodenal atresia showed “double bubble” sing, hypertrophic pyloric stenosis showed marked gastric distention,paucity of air in small bowel and increased gastric peristalsis were the most common finding. Hirschsprung'sdisease showed absence of rectal gas almostly. The variable length between blind hindgut to anus was seen inanorectal anomalies. c. Esophagogram: Blind sac of upper esophagus was seen at the 4th thoracic spinal level anddisplacement of trachea anteriolaterally. 1 case of tracheoesophageal fistula had an intact esophageal lumen. d.Upper G-I series; In hypertrophic pyloric stenosis, delayed gastric emptying, string or double tract sign were themost common finding and catapiller sign, tit sign, shoulder sing or open umbrella sign were noted. e. Bariumenema; In congenital megacolon, transitional zone and irregular bizzar contraction, “choppywave” were noted.Radiograph made 24 hours after barium enema showed retained barium in the colon. f. Selective retrogradefistulogram, distal loopgram & voiding cystogram: In anorectal anomalies, 3 cases showed rectourethral fistula and2 cases showed rectovaginal fistula and 1 case showed rectovesical fistula. And membranous imperforate anus is in1 case.

A Case of Congenital Hypertrophic Pylocric Stenosis Associated with Gastroesophageal Reflux

Congenital hypertrophic pyloric stenosis is characterized by projectile vomiting and pyloric thickening, and must be differentiated with gastroesophageal reflux. Gastroesophageal reflux may defined as relaxation of lower esophageal sphincter causing return of stomach content into eophagus, and frequently associated with hiatal hernia. The authors presented a case, having congenital hypertrophic pyloric stencsis and gastroesophageal reflux simultaneously which must be differentiated each other, in 28 days old newborn male with chief problem of severe projectile vomiting. A brief review of literatures was made on this subject.

Clinical Study of Congenital Megacolon

The clinical study was made on 38 cases of the congenital megacolon who had been admitted to Dept. of Pediatrics and General surgery, National Medical Center from Jan., 1869 to Dec., 1977 We obtained the following results. 1. The sex ratio was 4.4 : 1, of male preponderance. 2. The onset of symptoms was within 1 week after birth in majority of the cases. 3. All patients were delivered at fullterm and the familial occurrence was not observed. The associated anomalies were a case of mongolism with congenital heart disease and of congenital hypertrophic pyloric stenosis. 4. In about half of the cases, the definitive diagnosis was made before the age of 6months 5. The clinical symptomes were abdominal distension, constipation, vomiting, diarrhea, visible peristalsis and weight loss in order of frequency. 6. Meconium passage was spontaneous in 41.2% and after digital examination or enema in 58.8%. 7. The majority of the cases(79%) were so called short segment type. One male patient was proved to be total aganglionic colon. 8. The definitive operation was performed in 18 cases, in which 14 cases(77.7%) received Swenson's procedure. The early complications were wound infection, leakage, fistula, pelvic abscess and the late complications were stricture, intestinal obstruction, enterocolitis, fecal impaction, malnutrition in order of frequency. 9. The total mortality rate was 23.7% and the operation mortality was 16.7%. 10. The results of one stage operation was somewhat better than two stage operation. 11. The follow up results were good in 83.3% of operated cases.