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2.
Pediatr Neurol ; 64: 92-93, 2016 11.
Article in English | MEDLINE | ID: mdl-27720712

ABSTRACT

BACKGROUND: A severe neurological abnormality has not been previously described in individuals with hereditary fructose intolerance, which typically presents early in childhood with severe metabolic acidosis and hypoglycemia. PATIENT DESCRIPTION: We describe a boy who by age five years had required multiple admissions to the pediatric intensive care unit for an aggressive and atypical, relapsing and remitting neuropathy with features of acute motor axonal neuropathy (AMAN). It was later discovered that he also had undiagnosed hereditary fructose intolerance, and the severity and frequency of his neurological episodes diminished following an exclusion diet. His asymptomatic younger brother was diagnosed with hereditary fructose intolerance on screening. He is on a fructose-free diet and has not developed neurological symptoms. CONCLUSIONS: Ongoing low-level exposure to fructose prior to diagnosis may have contributed to our patient's neurological dysfunction. Early diagnosis and treatment may prevent neurological complications of hereditary fructose intolerance.


Subject(s)
Fructose Intolerance/diet therapy , Fructose Intolerance/physiopathology , Peripheral Nervous System Diseases/diet therapy , Peripheral Nervous System Diseases/physiopathology , Child, Preschool , Diagnosis, Differential , Fructose Intolerance/diagnosis , Humans , Male , Peripheral Nervous System Diseases/diagnosis
4.
Arab J Gastroenterol ; 12(1): 1-4, 2011 Mar.
Article in English | MEDLINE | ID: mdl-21429446

ABSTRACT

Malabsorption of carbohydrates such as fructose, lactose or sorbitol can often be detected among patients suffering from so-called non specific abdominal complaints. Sometimes the differential diagnosis may be difficult. So far successful treatment consists of dietary interventions only. Nevertheless, many questions are remaining still unanswered.


Subject(s)
Dietary Carbohydrates/metabolism , Malabsorption Syndromes , Diagnosis, Differential , Fructose Intolerance/diagnosis , Fructose Intolerance/diet therapy , Fructose Intolerance/epidemiology , Fructose Intolerance/physiopathology , Humans , Lactose Intolerance/diagnosis , Lactose Intolerance/diet therapy , Lactose Intolerance/epidemiology , Lactose Intolerance/physiopathology , Malabsorption Syndromes/diagnosis , Malabsorption Syndromes/diet therapy , Malabsorption Syndromes/epidemiology , Malabsorption Syndromes/physiopathology , Sorbitol/metabolism
6.
Best Pract Res Clin Gastroenterol ; 23(3): 395-406, 2009.
Article in English | MEDLINE | ID: mdl-19505667

ABSTRACT

The gastrointestinal tract possesses a huge epithelial surface area and performs many different tasks. Amongst them are the digestive and absorptive functions. Disorders of intestinal absorption and secretion comprise a variety of different diseases, e.g. coeliac disease, lactase deficiency or Whipple's disease. In principle, impaired small intestinal function can occur with or without morphological alterations of the intestinal mucosa. Therefore, in the work up of a malabsorptive syndrome an early small intestinal biopsy is encouraged in conjunction with breath tests and stool analysis to guide further management. In addition, there is an array of functional tests, the clinical availability of which becomes more and more limited. In any case, early diagnosis of the underlying pathophysiology is most important, in order to initiate proper therapy. In this chapter, diagnostic procedure of malabsorption is discussed with special attention to specific disease like coeliac disease, Whipple's disease, giardiasis and short bowel syndrome. Furthermore, bacterial overgrowth, carbohydrate malabsorption and specific nutrient malabsorption (e.g. for iron or vitamins) and protein-losing enteropathy are presented with obligatory and optional tests as used in the clinical setting.


Subject(s)
Gastrointestinal Diseases/diagnosis , Intestines/physiopathology , Breath Tests , Carbohydrate Metabolism/physiology , Celiac Disease/diagnosis , Celiac Disease/therapy , Fructose Intolerance/diagnosis , Fructose Intolerance/physiopathology , Gastrointestinal Diseases/physiopathology , Gastrointestinal Diseases/therapy , Giardiasis/diagnosis , Humans , Intestinal Absorption/physiology , Malabsorption Syndromes/diagnosis , Short Bowel Syndrome/diagnosis , Short Bowel Syndrome/microbiology , Short Bowel Syndrome/therapy
7.
J Am Diet Assoc ; 109(7): 1204-14, 2009 Jul.
Article in English | MEDLINE | ID: mdl-19559137

ABSTRACT

This review summarizes what is known about the effect of diet on irritable bowel syndrome (IBS) symptoms emphasizing data from randomized, controlled clinical trials. Studies suggest that IBS symptoms in one quarter of patients may be caused or exacerbated by one or more dietary components. Recent studies indicate that a diet restricted in fermentable, poorly absorbed carbohydrates, including fructose, fructans (present in wheat and onions), sorbitol, and other sugar alcohols is beneficial, but confirmatory studies are needed. Despite a long history of enthusiastic use, fiber is marginally beneficial. Insoluble fiber may worsen symptoms. Some patients with IBS, especially those with constipation, will improve with increased intake of soluble fiber. Prebiotic fibers have not been adequately tested. Daily use of peppermint oil is effective in relieving IBS symptoms. The usefulness of probiotics in the form of foods such as live-culture yogurt and buttermilk for IBS symptoms is not established. In clinical practice, it is very difficult to establish that a patient's symptoms result from an adverse reaction to food. A double blind placebo-controlled food challenge is the most reliable method, but it is not suitable for routine clinical use. A modified exclusion diet and stepwise reintroduction of foods or trials of eliminating classes of food may be useful.


Subject(s)
Diet , Irritable Bowel Syndrome/diet therapy , Irritable Bowel Syndrome/metabolism , Constipation/diet therapy , Constipation/etiology , Diet/adverse effects , Dietary Fiber/therapeutic use , Fermentation , Food Hypersensitivity/complications , Food Hypersensitivity/physiopathology , Fructose Intolerance/complications , Fructose Intolerance/physiopathology , Humans , Irritable Bowel Syndrome/etiology , Lactose Intolerance/complications , Lactose Intolerance/physiopathology , Mentha piperita , Plant Oils/therapeutic use , Probiotics/therapeutic use , Randomized Controlled Trials as Topic , Sorbitol/adverse effects , Sorbitol/metabolism
8.
Neurogastroenterol Motil ; 20(5): 505-11, 2008 May.
Article in English | MEDLINE | ID: mdl-18221251

ABSTRACT

Although incomplete fructose absorption has been implicated to cause gastrointestinal symptoms, foods containing high fructose corn syrup (HFCS) contain glucose. Glucose increases fructose absorption in healthy subjects. Our hypothesis was that fructose intolerance is less prevalent after HFCS consumption compared to fructose alone in healthy subjects and irritable bowel syndrome (IBS). Breath hydrogen levels and gastrointestinal symptoms were assessed after 40 g of fructose (12% solution) prepared either in water or as HFCS, administered in double-blind randomized order on 2 days in 20 healthy subjects and 30 patients with IBS. Gastrointestinal symptoms were recorded on 100-mm Visual Analogue Scales. Breath hydrogen excretion was more frequently abnormal (P < 0.01) after fructose (68%) than HFCS (26%) in controls and patients. Fructose intolerance (i.e. abnormal breath test and symptoms) was more prevalent after fructose than HFCS in healthy subjects (25% vs. 0%, P = 0.002) and patients (40% vs. 7%, P = 0.062). Scores for several symptoms (e.g. bloating r = 0.35) were correlated (P < or = 0.01) to peak breath hydrogen excretion after fructose but not HFCS; in the fructose group, this association did not differ between healthy subjects and patients. Symptoms were not significantly different after fructose compared to HFCS. Fructose intolerance is more prevalent with fructose alone than with HFCS in health and in IBS. The prevalence of fructose intolerance is not significantly different between health and IBS. Current methods for identifying fructose intolerance should be modified to more closely reproduce fructose ingestion in daily life.


Subject(s)
Fructose Intolerance/diagnosis , Fructose Intolerance/physiopathology , Fructose/administration & dosage , Health Status , Irritable Bowel Syndrome/diagnosis , Irritable Bowel Syndrome/physiopathology , Adult , Breath Tests/methods , Cross-Over Studies , Double-Blind Method , Female , Fructose/adverse effects , Humans , Male , Zea mays/adverse effects
9.
Ann Endocrinol (Paris) ; 68(6): 456-9, 2007 Dec.
Article in English | MEDLINE | ID: mdl-18035330

ABSTRACT

We present the case of a 17-year-old male who was diagnosed at birth with hereditary fructose intolerance (HFI). The patient complained of morning-time asthenia and post-prandial drowsiness despite a correct sleep pattern. The physical examination and biological check-up only showed severe vitamin C deficiency (<10 mol/l; normal range: 26-84). The patient's tiredness was attributed to this vitamin C deficiency, which is a frequent side-affect of the fructose-free diet. A change in diet associated with a supplementation in vitamin C was advised, with an increase in vegetable intake, principally avoiding carrots, onions, leaks and tinned sweet-corn. This case offers the opportunity for a review of this rare disease. Two kinds of fructose metabolism disorders (both autosomal recessive) are recognized: 1) essential fructosuria caused by a deficiency of fructokinase, which has no clinical consequence and requires no dietary treatment; 2) HFI, linked to three main mutations identified in aldolase B gene that may be confirmed by fructose breath test, intravenous fructose tolerance test, and genetic testing. In HFI, fructose ingestion generally induces gastro-intestinal (nausea and vomiting, abdominal pain, meteorism) and hypoglycemic symptoms. Fasting is well tolerated. If the condition remains undiagnosed, it leads to liver disease with hepatomegaly, proximal tubular dysfunction, and slow growth and weight gain. In conclusion, endocrinologists should be aware of this rare metabolic disease in order to provide careful follow-up, particularly important when the patient reaches adulthood. Moreover, hypoglycemia induced by fructose absorption, unexplained liver disease, irritable bowel syndrome or familial gout in an adult is suggestive of the diagnosis.


Subject(s)
Fructose Intolerance/diagnosis , Fructose Intolerance/genetics , Adolescent , Ascorbic Acid/therapeutic use , Asthenia/etiology , Diagnosis, Differential , Diet , Fructose/metabolism , Fructose Intolerance/diet therapy , Fructose Intolerance/physiopathology , Fructose-Bisphosphate Aldolase/deficiency , Glycogen/metabolism , Humans , Male
12.
Scand J Gastroenterol ; 35(10): 1048-52, 2000 Oct.
Article in English | MEDLINE | ID: mdl-11099057

ABSTRACT

BACKGROUND: Fructose malabsorption is characterized by the inability to absorb fructose efficiently. As a consequence fructose reaches the colon where it is broken down by bacteria to short fatty acids, CO2 and H2. Bloating, cramps, osmotic diarrhea and other symptoms of irritable bowel syndrome are the consequences and can be seen in about 50% of fructose malabsorbers. We have previously shown that fructose malabsorption is associated with early signs of mental depression and low serum tryptophan concentrations. It was therefore of interest whether a fructose-reduced diet could not only improve gastrointestinal complaints but also depressive signs seen in fructose malabsorbers. METHODS: Fifty-three adults (12 males, 41 females), who were identified as fructose malabsorbers according to their breath-H2 concentrations, filled out a Beck's depression inventory-questionnaire, and a questionnaire with arbitrary scales for measurement of meteorism, stool frequency and quality of life for a 4-week period before dietary intervention and 4 weeks after dietary change as for fructose- and sorbitol-reduced diet. RESULTS: Depression scores were reduced by 65.2% after 4 weeks of diet (P < 0.0001), and there was a significant reduction of meteorism (P < 0.0001) and stool frequency (P < 0.01). Improvement of signs of depression and of meteorism was more pronounced in females than in males. CONCLUSION: Fructose- and sorbitol-reduced diet in subjects with fructose malabsorption does not only reduce gastrointestinal symptoms but also improves mood and early signs of depression.


Subject(s)
Affect , Digestive System/physiopathology , Fructose Intolerance/diet therapy , Fructose , Sorbitol , Adolescent , Adult , Aged , Breath Tests , Colonic Diseases, Functional/etiology , Depression/etiology , Female , Fructose Intolerance/physiopathology , Fructose Intolerance/psychology , Humans , Hydrogen/analysis , Male , Middle Aged , Quality of Life , Tryptophan/blood
15.
J Inherit Metab Dis ; 16(1): 73-7, 1993.
Article in English | MEDLINE | ID: mdl-8487506

ABSTRACT

A patient is described who presented with the signs and symptoms of hereditary fructose intolerance a few hours after her first fructose challenge. The diagnosis was confirmed by the demonstration of reduced activity of hepatic aldolase B towards fructose-1-phosphate. A second liver biopsy 10 months later had normal aldolase B activity towards fructose-1-phosphate and a fructose tolerance test was also normal. A possible explanation for these findings is proposed.


Subject(s)
Fructose Intolerance/genetics , Female , Fructose Intolerance/metabolism , Fructose Intolerance/physiopathology , Fructose-Bisphosphate Aldolase/metabolism , Fructosephosphates/metabolism , Humans , Infant , Infant Food , Liver/enzymology , Liver/metabolism
17.
In. Restrepo G., Jorge Emilio; Guzman V., Jose Miguel; Botero A., Rafael Claudino; Velez A., Hernan; Ruiz P., Oscar. Gastroenterologia hematologia nutricion. Medellin, Corporacion para Investigaciones Biologicas, 1990. p.531-51, ilus, tab.
Monography in Spanish | LILACS | ID: lil-133898
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