Understanding infant pyloric stenosis (1)

PS book coverIn the previous two posts I have “translated” several parts of the larger share of the small book I have co-written with the emeritus Prof. Dr Ian Rogers, titled  The consequence and cause of pyloric stenosis of infancy: Two personal stories.  My co-author has addressed his section to the medical profession but gives much information that is of no small interest to many of us who have been touched by infant pyloric stenosis (“PS”).  It has certainly helped me to understand much more of the what, how and why of this rather enigmatic condition, something I have longed for ever since I became aware of the strange markings on my belly!

In this post I want to revisit (in more detail than my initial post) the first pages of Dr Rogers’ section, in which he writes about the function of the hormone gastrin in digestion.  He mentions that the Glasgow academics under whom he trained specialised in the physiology (working) of the stomach and intestines, clearly because duodenal ulcers were a major cause of illness at the time.

When Ian Rogers finished his surgical training in the early 1970s, it was known that –

  • Stomach01hyper- (or over-) acidity could be constitutional or acquired, and was the major cause of duodenal ulcers;
  • hydrochloric acid release is triggered by the vagus nerve and/or the hormone gastrin;
  • the upper (proximal or first) part of the stomach secretes the hydrochloric acid;
  • gastrin occurs in the mucosa (lining) of the distal (or end) part of the stomach;
  • food in the stomach causes chemical change which releases gastrin into the bloodstream and this as well as stomach activity triggers the release of acid into the stomach contents;
  • acid is essential to digestion as well as sterilising food;
  • normally, gastrin controls acidity, not allowing its level to become too high or low;
  • duodenal ulcers were thought to be caused by hyper-acidity, most common in anxious men; often this could be controlled by cutting the vagus nerve, and otherwise by a gastro-enterostomy (which involves more radical surgery).

It has only become known in more recent decades that Helicobacter pylori bacteria are also involved in hyper-acidity and cause some 80% of duodenal ulcers, and also that antibiotic treatment and/or suppression of vagus nerve activity with atropine could control acidity, almost eliminating the need for surgery to relieve gastric ulcers.

Although both men and women have rates of H. pylori infection that rise during their lives and are almost similar at age 70, the acid-secreting stomach cells are more numerous in adult men than women.

Thus there are some interesting parallels between patients with PS of infancy and those with gastric ulcers in adulthood –

  • a 5:1 male preponderance;
  • high acidity;
  • a good appetite;
  • other family members affected.

The above facts represent what was commonly known in the early 1970s when Ian Rogers completed his surgical training in London and returned to Glasgow.

In later pages Dr Rogers continues by describing the “clinical (observable) signs” of PS – as they give many clues to its cause.

  1. Peptic ulcer3it arises very early in a baby’s life;
  2. it affects many more boys than girls;
  3. it cures itself in time if the patient can be treated medically;
  4. it can have a strong family link;
  5. if the pyloric tumour is split surgically the swelling soon disappears;
  6. the swelling does not disappear if the blockage is surgically by-passed;
  7. the high acidity in the stomach cannot be explained as accumulation due to the pylorus being closed;
  8. survivors continue to secrete higher than usual acid;
  9. PS babies have a ravenous appetite;
  10. they are often first-born babies;
  11. some PS babies are found to have a superficial duodenal ulcer.

Despite these clear pointers, the medical community still finds the cause of PS a mystery more than 120 years after it was first fully described.

Dr Rogers then refers to the significant 1921 paper of the Scottish Dr John Thompson who observed that –

  • the “pyloric tumour” is in fact over-developed muscle;
  • PS is self-limiting (see #3 above);
  • PS can be managed with small feeds, daily stomach wash-outs, and if feeding has been unsuitable, with IV feeding;
  • there are 3 kinds of PS: the acute (with sudden and violent symptoms), ordinary, and very mild.

Thompson accepted the theory that PS is caused by a functional abnormality, referring to an 18th century anatomist who found that all muscles develop with use, but involuntary muscles (ones like the pylorus over which we have no control) more than others.  However, this theory was not further explored, even though PS occurs also in other mammals.

Dr Rogers has now laid the foundations for his conclusions about the cause of PS, and in the next post we’ll see how what he has been mentioned in these three posts applies to gastrin, hyper-acidity and PS in the newborn.

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6 thoughts on “Understanding infant pyloric stenosis (1)

  1. ian rogers

    Dear Fred,

    Many thanks for your post and for sending it to me. I have asked for a preliminary print of some of the colour pages at the printers before giving him the go ahead. Is that OK? Will keep in touch.
    Everything these days is genetic at its foundations. I do not know where the genes are that control parietal cell development and mass – perhaps someone does? However the control of acid secretion from the parietal cell is stimulated by gastrin. Cholecystokin 2(CCK2) is really the same as gastrin but acts locally on the cell and does not travel in the blood stream like gastrin. It is a local mediator and has the same effect. CCK1 on the other hand stimulates the D cells in the stomach lining. These cells secret Somatostatin which inhibits acid secretion. The location of the gene controlling CCK1 and CCK2 is known and you can knock out these genes in mice. Researchers have found that when CCK1/histamine is knocked out acid secretion falls and does not recover. When CCK1/histamine is knocked out although acid is temporaily impaired, it recovers. Hence the geneticists thus far frustrated should look at the CCK1 gene in babies with PS. I have attached a slightly revised PDF which includes this stuff at the very end under genetics and gives the reference.

    Best wishes,

    Ian and Hista

    Date: Sat, 29 Mar 2014 11:17:49 +0000 To: irogers2000@hotmail.com

    Reply
  2. Wendy

    “Despite these clear pointers, the medical community still finds the cause of PS a mystery more than 120 years after it was first fully described.” These words from your blog baffle me, anger me, and confuse me. Because it doesn’t make sense that the cause has not yet been discovered – certainly it is within our capability to be able to do so – I have to assume some type of politics is involved. Do the surgeons have such a tight grip over this area that research into the cause is impeded? Is funding for this type of condition disregarded since surgery can ‘cure’ PS? Do research institutes favor other areas of research? Is the pyloric stenosis problem seen as a done deal? In any case, I appreciate the information that you are sharing with us, Fred, and I thank you for your work to decipher/decode and present the medical info to us lay people. Can’t wait to read the next installment!

    Reply
    1. Fred Vanderbom Post author

      Your deep interest in the many issues around PS adds to my determination to keep writing for this blog, Wendy: thank you! I wish I knew the answers to your questions and concerns. But not only I, I believe the interested world knows much more about PS now than just a few years ago, let alone when this ugly condition was starving us to death. Yes, there are still several issues large and small that puzzle, frustrate or anger us, but we’ll continue to dig into the flow of information and rap on some doors.

      Reply
  3. survivingnursingtoday

    My son had pyloric stenosis and underwent a pyloromyotomy and subsequently had a duodenal perforation, sepsis, and peritonitis. We were told the duodenal perforation was caused by an inexperienced Surgeon. Now after reading this I’m wondering if he had a duodenal ulcer that coincidentally perforated a day after surgery. Also, my son has had severe bradycardia. Cardiology says it is not cardiac origin. They think it mediated by the vagus nerve. How close is the vagus nerve to the pyloric and could this be related?

    Reply
    1. Fred Vanderbom Post author

      I assume that your son had his pyloromyotomy as an infant? It would be very rare for a baby to have a gastric or duodenal ulcer and I have never come across this in the literature or online forums. Mucosal and duodenal perforations during pyloromyotomy by inexperienced or careless surgeons are fairly common (the stats vary). Standard procedure is to check for any perforation before wound closure, and from what you mention, this was almost certainly not done. How sad.
      The vagus nerve is of course not unrelated to pylorus: it is sometimes cut when the stomach is over acidic as this can cause ulcers, scarring, and thus adult PS. As a non-medico with a narrow interest in PS-related matters, I’m sorry not to know much about the relation between the vagus nerve and heart. However, you may be aware that a small percentage of infant PS patients have other “midline” developmental conditions (incl. of the heart). Best wishes!

      Reply

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