Long-Term Dietary Changes in Subjects with Glucose Galactose Malabsorption Secondary to Biallelic Mutations of SLC5A1.

BACKGROUND: Glucose galactose malabsorption (GGM) is a congenital diarrheal disorder of intestinal Na+/glucose cotransport (SGLT1/SLC5A1). The required glucose and galactose-restricted diet has been well described in infancy, but long-term nutrition follow-up is limited. AIM: To perform a comprehensive nutritional assessment on a cohort of patients with GGM to gain insights into the consumption patterns within the population. METHODS: A cross-sectional study examining dietary intake of a GGM cohort using prospective food records. The calories and nutrients of all foods, beverages, and condiments were analyzed with descriptive statistics and compared to intake patterns of age- and sex-matched NHANES groups. RESULTS: The six patients were 0.7-26 years old. Whole foods and vegetable fats were major parts of the diet, while dairy and added sweeteners were restricted. Compared to typical US intakes, mean macronutrient distribution was 88th percentile from fat, 18th percentile from carbohydrates, and 78th percentile from protein. Fructose consumption, as a proportion of total sugar intake, decreased with age, from 86.1 to 50.4%. Meanwhile, glucose consumption increased with age, from 13.8 to 48.6% of sugar intake. However, the actual amount of glucose consumed remained low, equivalent to 4th percentile of US consumption level. Galactose intake was marginal throughout life. CONCLUSIONS: A GGM diet is a high-fat and high-protein/low-carbohydrate diet that is rich in fruits and vegetables but limited in dairy and added sugar. Relatively less fructose but more glucose is incorporated into the diet with age. Future studies should investigate the effects of the GGM diet on gut microbiome and long-term health.

The Oley Foundation and Consumer Support Groups.

Patients with intestinal failure (IF) often require home parenteral and/or enteral nutrition (HPEN). There are many complications associated with both IF and the use of HPEN, including infection and intolerance. Psychosocial effects, such as depression, isolation, fatigue, anxiety, financial stress, are also associated with IF and HPEN and can be difficult to address. Support groups offer patients and caregivers the opportunity to talk to and learn from others who have had similar experiences. The Oley Foundation, a nonprofit organization for HPEN consumers, caregivers, and clinicians, fulfills the role of a traditional support group while offering many other resources and programs.

Increased risk for major depressive disorder in severely obese patients after bariatric surgery - a 12-year nationwide cohort study.

BACKGROUND: Bariatric surgery is associated with a significant improvement in depressive mood in the initial postoperative years, but the maintenance of the improvement is under debate. AIM: To explore the association between bariatric surgery and major depressive disorder (MDD) in a 12-year nationwide cohort study. METHOD: Using the National Health Insurance Research Database of Taiwan, we identified 2302 patients who underwent bariatric surgery in 2001-2009. These patients were matched by propensity score to 6493 obese patients who did not receive bariatric surgery. We followed the surgical and control cohorts until death, any diagnosis of MDD or 31 December 2012. We used Cox proportional hazard regression models to calculate the relative risk of MDD in those who received bariatric surgery. RESULTS: Overall, there was a 1.70-fold (95% CI: 1.27-2.27) higher risk of MDD in the surgical group. Subjects receiving malabsorptive procedures showed a higher risk of MDD (3.01, 95% CI: 1.78-5.09) than those receiving restrictive procedures (1.51, 95% CI: 1.10-2.07). Stratified by follow-up period, there was a higher risk of MDD in the surgical group (2.92, 95% CI: 1.75-4.88) than in the restrictive group four years after bariatric surgery. CONCLUSIONS: Bariatric surgery was significantly associated with an elevated risk of MDD. KEY MESSAGES Bariatric surgery is associated with a significant improvement in depressive mood in the initial postoperative years, but the improvement is not maintained. Less is known about the relationship between bariatric surgery and risk of major depressive disorder. This was the first nationwide cohort study which found that bariatric surgery was significantly associated with an elevated risk of MDD (aHR: 1.70; CI: 1.27-2.27), mainly with malabsorptive procedures (aHR: 3.01; CI: 1.78-5.09) and at time points more than four years after surgery (aHR: 2.92; CI: 1.75-4.88) compared with the risk in matched controls. These findings imply an association between long-term malabsorption and the postoperative incidence of MDD. Long-term malabsorption might be related to the incidence of major depressive disorder after bariatric surgery. The possible causal relationship between nutritional deficiency after bariatric surgery and major depressive disorder warrants further investigation.

The active problem solving of patients dependent on home parenteral nutrition: A qualitative analysis.

BACKGROUND & AIMS: People with chronic, type 3, intestinal failure often require long-term home parenteral nutrition (HPN). People receiving HPN have frequent interactions with their healthcare, due to the need for close monitoring and due to recurrent hospital admissions. Individuals' responses to, and interactions with, their health care service provides are poorly described. We conducted a service evaluation to explore people's experiences of HPN-related healthcare interactions in order to identify how service providers can best meet the individualised needs of patients. METHODS: We interviewed ten people receiving HPN. The participants were asked about their healthcare interactions related to HPN. The data were analysed using a qualitative research method known as interpretive phenomenological analysis. This analytical approach is concerned with the meaning that people ascribe to particular events, in this case their HPN-related healthcare interactions. RESULTS: The participants in this study described a range of psychosocial problems related to their HPN healthcare interactions, including reliance, risk and restrictions. Participants' solutions to these problems included adaptation of their routine, self-guardianship and passivity. Sometimes, these solutions generated secondary problems of their own, including concerns with resource use, negative healthcare interactions and conflicts of responsibility. A range of contextual factors influenced how participants interpreted their healthcare interactions and the solutions available to them. These contextual factors included continuity of healthcare professionals, the attitude of staff, and information and knowledge about HPN. CONCLUSIONS: By attending to the individual meaning ascribed to healthcare events, and the contextual factors that surround these events, we have been able to better understand the decisions made by patients dependent on HPN. This suggests that healthcare professionals may also better understand their patients' decision making by attending to the individual meaning that patients ascribe to healthcare events and to contextual factors. We propose a model to describe the process of problem -> resolution -> problem in which participants are actively engaged.

Social circumstances and medical complications in children with intestinal failure.

UNLABELLED: Although most children discharged on home parenteral nutrition (HPN) will achieve enteral autonomy, some remain parenteral nutrition dependent; those who develop life-threatening complications may undergo small bowel transplantation (SBTx). The aim of this study was to investigate the relationship between social circumstances, compliance and complications. SUBJECTS AND METHODS: An observational study in 2008-2012 on 64 children (34 HPN, 30 SBTx) from three units (two regional gastroenterology; one transplant). Social circumstances were assessed routinely as part of discharge planning; adherence by families to home care management was scored, and episodes of catheter-related blood stream infection and graft rejection were recorded for 2 years and related to compliance and social circumstances. RESULTS: A quarter of families had a disadvantaged parent: non-English speaking (n=11), unable to read (n=5), physical disability (n=3), mental health problems disclosed (n=10); 20% children were cared for by a lone parent. Discharge home was delayed by social factors (n=9) and need for rehousing (n=17, 27%). 17/34 (50%) of HPN and 12/30 (40%) of transplant families were assessed as fully adherent. 10 families were assessed as non-adherent, eight were subject to child protection review and care was taken over by another family member (n=3) or foster parents (n=2). The risk of catheter-related blood stream infection was increased by parental disadvantage and age <3 years (p<0.05). Poor compliance was associated with complications in HPN and SBTx recipients. CONCLUSIONS: Children receiving complex home care may be socially isolated and measures to support improved compliance such as increased community support, social care involvement and respite care may improve outcomes.

[Pseudomalabsorption of levothyroxine: a case report]. / Pseudomalabsorção de levotiroxina: relato de um caso.

A 49-year-old woman who had been treated with sodium levothyroxine because of hypothyroidism after diagnosis of Hashimoto thyroiditis and total thyroidectomy for multinodular atoxic goiter was evaluated for persistent hypothyroidism despite the use of large doses of levothyroxine (600 microg/day). The patient showed signs and symptoms of hypothyroidism and her laboratory tests were: TSH of 351 microUI/mL, free thyroxine of 0.20 ng/dL, and total triiodothyronine of 27 ng/dL. She was submitted, under medical supervision, to a levothyroxine overload test with no evidence of malabsorption of the thyroid hormone. Diagnosis of factitious disorder and Munchausen syndrome leading to the pseudomalabsorption of levothyroxine was considered.

Self-reported milk intolerance in irritable bowel syndrome: what should we believe?

BACKGROUND & AIMS: The real importance of lactose malabsorption in irritable bowel syndrome (IBS) is still controversial. The aim is to define the relationship between patient perception of milk tolerance/intolerance, lactose malabsorption and abdominal symptoms in IBS. METHODS: A hydrogen breath test (HBT) after an oral load of lactose was carried out in 475 consecutive IBS patients, diagnosed according to the Rome criteria. Data were analyzed in 201 age- and sex-matched pairs of IBS patients, classified according to self-reported milk tolerance/intolerance. Hydrogen peak and excretion, predominant presenting symptom and the occurrence of symptoms during the test, were evaluated. RESULTS: The prevalence of positive HBT and the occurrence of symptoms during the test was similar in milk "tolerant" (68.6%, 40.7% respectively) and "intolerant" patients (75.6%, 42.7% respectively), as well as peak (76.4 vs 75.2 ppm) and amount of H2 excreted (57.8 vs. 53.2 ppm/h). The positive predictive value for self-reported milk intolerance was 0.75, and the negative predictive value in regular milk users was 0.31, reflecting the prevalence of lactose malabsorption in the general population more than the awareness of milk tolerance. CONCLUSIONS: In IBS patients, self-reported milk intolerance does not help in identifying lactose malabsorbers. The opposite does not rule out the occurrence of symptoms after a lactose load. Lactose is, indeed, responsible for symptoms in some IBS patients, however, these patients can only be identified by the occurrence of symptoms during the test, and not on the individual perception of milk intolerance.

[Malabsorption methods in bariatric surgery]. / Malabsorptionsmethoden in der bariatrischen Chirurgie.

In Europe, the incidence of morbid obesity amounts approximately 1% of a nation's population. At the time being, a surgical procedure represents the only effective long-term therapy of morbid obesity. In this regard, the gastric bypass is the most common surgical method in the USA, also gaining relevancy in Europe. The development, the basic principle and the actual operation technique are shown. The mode of functioning, complications, surgical alternatives, risks, results and outcome are discussed. Over the past years, bariatric procedures and techniques have been improved, which resulted in a reduction of the risk and in an improvement of the results. Long- term success can be achieved regarding weight reduction and decrease of comorbidities. The gastric bypass is a safe and effective surgical procedure in the treatment of morbid obesity.

Carbohydrate malabsorption syndromes and early signs of mental depression in females.

Fructose and lactose malabsorption are characterized by impaired duodenal fructose transport or by the deficiency of mucosal lactase, respectively. As a consequence, the nonabsorbed saccharides reach the colon, where they are broken down by bacteria to short fatty acids, CO2, and H2. Bloating, cramps, osmotic diarrhea, and other symptoms of irritable bowel syndrome are the consequence and can be seen in about 50% of carbohydrate malabsorbers. We have previously shown that fructose as well as lactose malabsorption were associated with signs of mental depression. It was therefore of interest to investigate possible interactions between fructose and lactose malabsorption and their influence on the development of signs of depression. In all, 111 otherwise healthy volunteers (81 females and 30 males) with gastrointestinal complaints were analyzed by measuring breath H2 concentrations after an oral dose of 50 g lactose and of 50 g fructose one week apart. They were classified as normals, isolated fructose malabsorbers, isolated lactose malabsorbers, and combined fructose/lactose malabsorbers. All patients filled out a Beck's depression inventory-questionnaire. Twenty-five individuals (22.5%) were neither fructose nor lactose malabsorbers (group 1), 69 (62.2%) were only fructose malabsorbers (group 2), 4 (3.6%) were only lactose malabsorbers (group 3), and 13 (11.7%) presented with fructose and lactose malabsorption together (group 4). Isolated fructose malabsorption and combined fructose/lactose malabsorption was significantly associated with a higher Beck's depression score. Further analysis of the data show that this association was strong in females (P < 0.01), but there was no such association between carbohydrate malabsorption and early signs of depression in males. In conclusion, the data confirm that fructose malabsorption may play a role in the development of mental depression in females and additional lactose malabsorption seems to further increase the risk for development of mental depression.

Fructose malabsorption is associated with decreased plasma tryptophan.

Fructose malabsorption is characterized by the inability to absorb fructose efficiently. Consequently fructose reaches the colon and is broken down by bacteria to short-fatty-acids, CO2 and H2. Recently we found that fructose malabsorption was associated with signs of depression. It was therefore of interest to find out whether fructose malabsorption is associated with abnormal tryptophan metabolism. Breath hydrogen concentrations were measured in 50 after an oral dose of 50 g fructose allowing to classify them as normals (n = 15) or fructose malabsorbers (n = 35). Blood samples were taken for tryptophan and kynurenine measurements. Fructose malabsorbers showed significantly lower plasma tryptophan concentrations and significantly higher depression scores compared to normals. Fructose malabsorption is associated with lower tryptophan levels which may play a role in the development of depressive disorders.

Fructose malabsorption is associated with early signs of mental depression.

Fructose malabsorption is characterized by the inability to absorb fructose efficiently. As a consequence fructose reaches the colon were 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 consequence and can be seen in about 50% of fructose malabsorbers. Having made the observation that persons with fructose malabsorption very often seem to present not only with signs of irritable bowel syndrome but also with signs of pre-menstrual syndrome and mental depression, it was of interest to establish whether such an association could be demonstrated in patients. Fifty-five adults with gastrointestinal complaints of unknown origin (12 males, 43 females) were analyzed by measuring breath hydrogen concentrations after an oral dose of 50 g fructose and were classified as normals or fructose malabsorbers according to their breath H2 concentrations. All patients filled out a Beck s depression inventory - questionnaire. Fructose malabsorption was detected in 36 of 55 individuals (65.5%). Subjects with fructose malabsorption (DeltaH2 concentrations >10 p.p.m. after fructose load) showed a significantly higher score in the Beck s depression inventory than normal fructose absorbers. This was true especially for females. Fructose malabsorption may play a role in the development of depressed mood. Fructose malabsorption should be considered in patients with symptoms of major depression or pre-menstrual syndrome. Further studies are needed to clarify the background of this association.

[The psychosomatic development of children who recovered from secondary malabsorption syndrome]. / Rozwój psychosomatyczny dzieci z przebytym wtórnym zespolem zlego wchlaniania.

The study encompassed 100 children who recovered from secondary malabsorption syndrome. The following were evaluated: physical development (body weight, height, skin-fold thickness on the arm, shoulder-blade and abdomen) and IQ in the Wechsler scale. Children who had suffered from secondary malabsorption syndrome in infancy showed lower parameters of physical development compared with healthy children. These differences pertained mainly to body weight and height and to the intelligence quotient.

An infant with cystic fibrosis and a mother with an eating disorder: a difficult combination.

The feeding of an infant with cystic fibrosis and associated malabsorption was made difficult by the mother's attitudes to body shape and food. She had a history of anorexia nervosa. Such parents may require special advice and support.

[Psychosocial aspects of the so-called short-bowel syndrome in children]. / Psychosociale aspecten van het zogenaamde korte-darmsyndroom bij kinderen.

In a retrospective study of 16 patients with (congenital) bowel pathology, aged 2 to 12, attention was paid to the psychosocial aspects of the short bowel syndrome. Parents were interviewed and parents' and teachers' reports of children's behavioural and emotional problems were obtained. Parents as well as children appeared to have reached a reasonable level of adjustment. Parents' emotions concerning the period of hospitalisation still appeared to be strikingly strong. Although most children did not show obvious psychopathology, problems were observed in the areas of behaviour and attention, interfering with optimal school functioning in the case of some children.

Dietary compliance in the short bowel syndrome.

Psychologic problems associated with the short bowel syndrome have received limited recognition. The literature has previously considered these problems as due to the underlying condition or its treatment. We present a case illustrating these as well as addressing the problem of compliance to treatment that has been previously neglected. The occurrence of metabolic acidosis in this patient whenever there was not strict dietary adherence to diet provide a ready marker to her noncompliance.

Home parenteral nutrition for the short bowel syndrome. Psychological issues.

Home parenteral nutrition (HPN) for the short bowel syndrome represents a treatment modality that raises issues about prolonged machine dependency for living. Psychological reactions are described for patients with inflammatory bowel disorders and anatomical loss of small bowel. Liaison psychiatry involvement with 50 patients in the HPN program has identified such problems as grief reactions, depression, organic brain syndromes, drug dependency, and body image changes as they influence the initial adjustment to the in-hospital phase of HPN learning for patient and family. The importance of family and mental status examination are emphasized in the evaluation of the patient before and during the institution of an PHN program. Outlined are psychological parameters that need to be considered when assessing what factors might impede or enhance the acquisition and use of HPN information.

The neurological manifestations of malabsorption.

The clinical and pathological findings in patients with neurological disorders in association with disordered function of the small intestine, in particular coeliac disease, are outlined. The possible significance of the abnormalities of pyridoxine, tyrosine and tryptophan metabolism are considered in relation to biopterin derivatives and their relevance to neurological dysfunction.