Wednesday, January 20, 2016

Pediatric auto-brewery syndrome

A 13-year-old girl had long-standing short bowel syndrome secondary to jejunal atresia and necrotizing enterocolitis, and underwent extensive small bowel resection in the neonatal period. During the 6 months before her visit to this pediatric gastrointestinal clinic, she was noticed to have recurrent episodes of bizarre behavior, somnolence, disorientation, and a fruity odor of her breath and was suspected to be abusing alcohol. She was diagnosed as having alcohol intoxication when ethanol blood levels were repeatedly elevated, in the range of 250 mg/dL to 350 mg/dL. However, the patient persistently denied any intake of alcohol or alcoholic beverages. Nevertheless, this was suspected to be an adolescent behavior disorder. After repeated episodes of apparent intoxication, a psychiatric evaluation was performed and recommendation was made for admission for alcohol detoxification in a rehabilitation facility where she would be continuously monitored. While in the rehabilitation center, with no access to alcoholic beverages, she continued to have intoxicated behavior and again showed elevated blood ethanol levels. d-Lactate levels were repeatedly undetectable. She was finally suspected to be producing ethanol endogenously by fermentation by a mechanism similar to d-lactic acidosis because of her short gut.
 
The family noted that these episodes tended to be observed frequently after ingestion of excess carbohydrates and juices. The father, who is a physician, had started to monitor her ethanol level using a commercial breath analyzer in relation to meals and continued to monitor the child for access to alcoholic beverages. A strong correlation was found between her elevated ethanol levels and intake of high carbohydrate meals or fructose-containing drinks. Her symptoms did not resolve with empiric courses of Bactrim™ (Roche Pharmaceuticals, Nutley, NJ), Flagyl™ (Pharmacia, Peapack, NJ), and Augmentin™ (SmithKline Beecham, Philadelphia, PA) for suspected bacterial overgrowth. She then underwent upper gastrointestinal endoscopy study to obtain aspirates from her small intestinal fluids, which were sent for bacterial and fungal cultures. The aspirate grew abundant amounts of two types of yeast:Candida glabrata and Sacchromyces cerevisiae. After appropriate antibiotics were given based on fungal sensitivity studies (fluconazole), the symptoms resolved and there was no recurrence of the elevated ethanol levels.
 
Dahshan A, Donovan K. Auto-brewery syndrome in a child with short gut
syndrome: case report and review of the literature. J Pediatr Gastroenterol Nutr.
2001 Aug;33(2):214-5.
 
This is a case of a 3-year-old female patient with SBS. She had a birthweight of 1800 g and gastroschisis with an apple-peel-type malformation of the small bowel that was operated at the age of 8 h with closure of the abdomen and a loop enterostomy (jejunum). The stoma was closed when the patient was 2 mo old. One year later, however, the patient was re-operated because of an obstruction of the small intestine. The operation was complicated, and the inferior mesenteric vessel was accidentally divided. Despite direct anastomosis, blood circulation was not restored, and 24 h later the patient was re-operated, where half of the proximal colon and a large portion of the small intestine were resected. Because of a stricture in the jejuno-colonic anastomosis, another resection of the intestine was performed 1 wk later, and the patient lost more than half of her colon and a large portion of the jejunum, leaving 17 cm of small bowel remaining.

Initially, the patient received total parenteral nutrition by a central venous catheter, but enteral feeding was slowly introduced and was increased to about 20–30% of her nutritional need. Because of several episodes of septicaemia, which was based on suspected bacterial overgrowth from the intestine, the patient was given a Lactobacillus-containing carbohydrate-rich fruit drink 2–4 times a day when she was 3 y of age. At this age, she received one-third of her nutrition by enteral feeding. A couple of weeks later, the parents noticed a distinct smell of alcohol in the girl's bedroom. Moreover, they had also seen her walking erratically and behaving in a generally bizarre manner. A breath analyser showed a value of more than 22 mmol/l of ethanol.

The carbohydrate-rich fruit drink was discontinued for 1 wk, whereby all symptoms immediately disappeared. When the fruit drink was reinstated, the same symptoms of alcohol intoxication reappeared. A blood test showed an ethanol concentration of 15 mmol/l.

Jansson-Nettelbladt E, Meurling S, Petrini B, Sjölin J. Endogenous ethanol
fermentation in a child with short bowel syndrome. Acta Paediatr. 2006
Apr;95(4):502-4.

When did you first realize that your gut creates its own alcohol?
Matthew Hogg:
I suffered from digestive upsets throughout my childhood. I was initially diagnosed with irritable bowel syndrome, but in my teens I experienced a severe worsening of symptoms, like bloating and gas after meals—so much so that I could feel the bubbling of fermentation occurring in my lower abdomen. More worryingly, I developed new, quite frightening symptoms. I would feel intoxicated, as well as exerpience a long list of whole-body symptoms, including chronic fatigue, muscular aches and pains, chronic headaches, mental impairment, mood disturbances, and so on.
Did you feel hungover afterwards?
Yeah, by my late teens I was experiencing severe alcoholic hangovers that would usually be at their worst the morning after eating a high carbohydrate meal. I'd get pounding headaches, severe nausea, occasional vomiting, dehydration, dry mouth, cold sweats, and shaky hands. It was as if I'd been out the previous night and drunk the bar dry, but I hadn't consumed any alcohol...
How has this illness affected your life? It's had a huge and devastating impact on my life. Up until the age of 16, I was a straight-A student, and I found academic work enjoyable and rewarding. I was also a keen athlete and sportsman, and I had a great social life. As the auto-brewery syndrome began to assert itself, all of this changed. I found myself struggling badly at school when, in my mind, I knew I shouldn't be having any problems. I also had to quit sports because I'd feel exhausted after a gentle run and found myself struggling to get up in the mornings. I felt frightened, not knowing what was happening to me, as well as frustrated and angry that I was unable to function at the high level I was used to. My social life suffered badly, and I felt alone and detached from my friends and lacked the energy and motivation to be a part of things...
I imagine this could be the last thing you'd want to do, but have you ever eaten a load of sugary foods to get drunk for recreational reasons? Honestly, there are times in social situations—or when nothing else is available, if I'm away from home—that I've either been forced to or chosen to eat sugary and starchy foods. But, as a rule, I prefer to stick to the low-carb diet because the negative consequences outweigh the momentary pleasure. It's always been the case that I feel more hungover than drunk as a result of auto-brewery syndrome, so, although people may assume this condition is a cheap way to get drunk for recreational purposes, that's unfortunately not the reality.
But you can get drunk by eating sugar and carbs, right?
Yes. There were many times, throughout my later high school years in particular, when I felt moments of drunkenness without having consumed any alcohol. I'd describe them as periods rather than moments, actually, as they lasted for a few hours at a time. These periods of intoxication always followed a meal, and after a few hours—which is a typical time period for digestion and absorption—the effects would wear off and I would feel normal consciousness return.
My overriding memory of this time is feeling frustrated that my brain wasn't functioning at the level I was used to. I looked at equations in my favorite science classes and knew I should have no problem understanding and solving them, but they now looked like gibberish. There were times when I also acted out of character. I was generally everyone's friend at school—a social butterfly. But there were instances when I upset people with uncharacteristic behavior akin to a drunk who stirs up trouble or lets things slip that they wouldn't have when sober.

http://www.vice.com/read/my-gut-turns-yeast-into-alcohol
 

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