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1.
Pediatr Diabetes ; 21(7): 1249-1255, 2020 11.
Article in English | MEDLINE | ID: mdl-32662200

ABSTRACT

BACKGROUND: Hypoglycemia is the most common and severe complication of insulin treatment during the management of type 1 diabetes mellitus (T1DM). Despite its importance, there is a lack of data about the efficacy and superiority of the carbohydrate sources used in hypoglycemia management in children and adolescents. OBJECTIVE: We aimed to compare the effectiveness of honey, fruit juice, and sugar cubes as simple carbohydrates used in the primary treatment of hypoglycemia in children and adolescents with T1DM, who attended a diabetes summer camp. METHODS: A prospective randomized study was performed in a 5-days-long diabetes summer camp. Three different types of simple carbohydrates; sugar cubes, honey, or fruit juice were randomly given for the treatment of hypoglycemia and the recovery results in the three groups were compared. RESULTS: About 32 patients (53.1% male, mean age 12.9 ± 1.9 years) were included and 158 mild hypoglycemic episodes were observed. Sugar cubes, honey, and fruit juice were given in 46 (29.1%), 60 (37.9%), and 52 (33%) events, respectively. We found that honey and fruit juice had similar efficiency in recovering hypoglycemia in 15 minutes with a rate of 95% and 98%, respectively. However, sugar cubes had a significantly lower impact on treatment of hypoglycemia than the others, with a recovery rate of 84.7% at 15 minutes. CONCLUSIONS: This study showed, for the first time, that honey and fruit juice were more effective in treating hypoglycemia than sugar cubes, and can be preferred in treating hypoglycemic events in children and adolescents with T1DM.


Subject(s)
Diabetes Mellitus, Type 1/complications , Dietary Carbohydrates/therapeutic use , Fruit and Vegetable Juices , Honey , Hypoglycemia/diet therapy , Adolescent , Blood Glucose , Child , Diabetes Mellitus, Type 1/blood , Diabetes Mellitus, Type 1/therapy , Female , Humans , Hypoglycemia/blood , Hypoglycemia/etiology , Male , Prospective Studies , Time Factors
2.
Intern Med ; 59(15): 1803-1809, 2020 Aug 01.
Article in English | MEDLINE | ID: mdl-32461526

ABSTRACT

Objective As direct jejunal feeding often causes great fluctuation in glucose levels, continuous or slow infusion is recommended for jejunal tube-fed patients. However, continuous feeding results in prolonged immobility and the loss of activities of daily living. We investigated whether or not intermittent feeding of a low-carbohydrate high-monounsaturated fatty acid (LC/HM) nutrient formula reduces glucose fluctuation in patients who have undergone jejunotomy. Methods Ten bed-ridden non-diabetic patients receiving enteral feeding via a jejunostomy tube were enrolled in this study. LC/HM formula and standard control formula were infused in cross-over order for each patient at a speed of 160 kcal/h. Blood glucose levels were monitored by a continuous glucose monitoring system during the investigation period. Results The mean and standard deviation of the glucose concentrations and mean amplitude of glucose excursion (MAGE) were markedly lower while receiving LC/HM formula than while receiving control standard formula (104 vs. 136 mg/dL, 18.1 vs. 58.1 mg/dL, 50.8 vs. 160 mg/dL, respectively). The post-infusion hyperglycemia [area under the curve (AUC) >140 mg/dL] and peak value of the glucose level were also significantly lower in patients fed LC/HM than the control (25.7 vs. 880 mg・h/dL and 153 vs. 272 mg/dL, respectively). Reactive hypoglycemia (AUC <70 mg/dL) was also significantly lower (0.63 vs. 16.7 mg・h/dL) and the minimum value of the glucose level higher (78.4 vs. 61.8 mg/dL) in patients fed LC/HM than the control. Conclusion The LC/HM formula is considered to markedly inhibit glycemic spikes and prevent rebound hypoglycemia in patients who receive enteral feeding after jejunostomy.


Subject(s)
Dietary Carbohydrates/administration & dosage , Enteral Nutrition/methods , Hyperglycemia/diet therapy , Hypoglycemia/diet therapy , Activities of Daily Living , Aged , Aged, 80 and over , Blood Glucose , Cross-Over Studies , Female , Humans , Intubation, Gastrointestinal/methods , Jejunostomy/methods , Male , Monitoring, Physiologic , Nutritional Status
4.
BMJ Case Rep ; 12(9)2019 Sep 16.
Article in English | MEDLINE | ID: mdl-31527214

ABSTRACT

A 61-year-old man, without noteworthy medical history, presented with complaints of progressive fatigue and flushes. Diagnostic imaging revealed a large tumour in the stomach with liver metastases, and histopathological examination showed a well-differentiated gastric neuroendocrine tumour (NET). After chemotherapy, everolimus was administered, and upon progression, PD-1 inhibitor PDR001 was started. Two weeks after the first gift, he was admitted with loss of consciousness and a blood glucose level of 1.6 mmol/L. Plasma insulin was below 0.5 mU/L, C-peptide level was 250 pmol/L, insulin-like growth factor (IGF)-II was 804 ng/mL, and pro-IGF-IIE level was 80 µg/L. Based on the clinical findings, the patient was diagnosed with non-islet cell tumour hypoglycaemia (NICTH) with an overproduction of pro-IGF-IIE and eventually IGF-II due to progressive metastatic well-differentiated gastric NET. NICTH is a very rare condition. It has been reported in several tumour types but has never been described as a consequence of NET.


Subject(s)
Hypoglycemia/etiology , Intestinal Neoplasms/pathology , Liver Neoplasms/secondary , Neuroendocrine Tumors/pathology , Pancreatic Neoplasms/pathology , Stomach Neoplasms/pathology , Antineoplastic Agents/therapeutic use , Blood Glucose/analysis , Drug Therapy, Combination , Enteral Nutrition , Fatal Outcome , Humans , Hypoglycemia/diet therapy , Intestinal Neoplasms/drug therapy , Liver Neoplasms/drug therapy , Male , Middle Aged , Neuroendocrine Tumors/drug therapy , Pancreatic Neoplasms/drug therapy , Stomach Neoplasms/drug therapy
5.
Lakartidningen ; 1162019 Feb 19.
Article in Swedish | MEDLINE | ID: mdl-31192390

ABSTRACT

Bariatric surgery is a well-documented and good alternative for treatment of obesity with and without type 2-diabetes. One of the documented complications is postprandial hypoglycemia, with possibly serious consequences. We present such a case, what is known of underlying mechanisms, and treatment options.


Subject(s)
Gastric Bypass/adverse effects , Hypoglycemia/etiology , Female , Humans , Hypoglycemia/diet therapy , Hypoglycemia/physiopathology , Middle Aged , Obesity/surgery , Postoperative Complications/diet therapy , Postoperative Complications/physiopathology
6.
Obes Surg ; 29(7): 2092-2099, 2019 07.
Article in English | MEDLINE | ID: mdl-30976983

ABSTRACT

BACKGROUND: Hypoglycemia is an increasingly recognized complication of bariatric surgery. Mechanisms contributing to glucose lowering remain incompletely understood. We aimed to identify differentially abundant plasma proteins in patients with post-bariatric hypoglycemia (PBH) after Roux-en-Y gastric bypass (RYGB), compared to asymptomatic post-RYGB. METHODS: Proteomic analysis of blood samples collected after overnight fast and mixed meal challenge in individuals with PBH, asymptomatic RYGB, severe obesity, or overweight recruited from outpatient hypoglycemia or bariatric clinics. RESULTS: The top-ranking differentially abundant protein at 120 min after mixed meal was fibroblast growth factor 19 (FGF-19), an intestinally derived hormone regulated by bile acid-FXR signaling; levels were 2.4-fold higher in PBH vs. asymptomatic post-RYGB (mean + SEM, 1094 ± 141 vs. 428 ± 45, P < 0.001, FDR < 0.01). FGF-19 ELISA confirmed 3.5-fold higher concentrations in PBH versus asymptomatic (360 ± 70 vs. 103 ± 18, P = 0.025). To explore potential links between increased FGF-19 and GLP-1, residual samples from other human studies in which GLP-1 was modulated were assayed. FGF-19 levels did not change in response to infusion of GLP-1 and PYY in overweight/obese individuals. Infusion of the GLP-1 receptor antagonist exendin 9-39 in recently operated asymptomatic post-RYGB did not alter FGF-19 levels after mixed meal. By contrast, GLP-1 receptor antagonist infusion yielded a significant increase in FGF-19 levels after oral glucose in individuals with PBH. While plasma bile acids did not differ between PBH and asymptomatic post-RYGB, these data suggest unique interrelationships between GLP-1 and FGF-19 in PBH. CONCLUSIONS: Taken together, these data support FGF-19 as a potential contributor to insulin-independent pathways driving postprandial hypoglycemia in PBH.


Subject(s)
Bariatric Surgery/adverse effects , Fibroblast Growth Factors/blood , Hypoglycemia/blood , Hypoglycemia/etiology , Obesity, Morbid/surgery , Postoperative Complications/blood , Adult , Blood Glucose/metabolism , Blood Proteins/analysis , Blood Proteins/metabolism , Case-Control Studies , Female , Gastric Bypass/adverse effects , Gastrointestinal Hormones/blood , Glucagon-Like Peptide 1/blood , Glucagon-Like Peptide-1 Receptor/antagonists & inhibitors , Humans , Hypoglycemia/diet therapy , Hypoglycemia/drug therapy , Male , Meals , Middle Aged , Obesity, Morbid/blood , Peptide Fragments/therapeutic use , Postoperative Complications/diet therapy , Postoperative Complications/drug therapy , Proteome/analysis , Proteomics , Up-Regulation
7.
J Neonatal Perinatal Med ; 12(3): 285-293, 2019.
Article in English | MEDLINE | ID: mdl-30932901

ABSTRACT

OBJECTIVE: To ascertain the rate of in-hospital supplementation as it relates to early breastfeeding (BF) and early formula feeding (FF) and its effects on BF (exclusive and partial) at the time of discharge for infants born to women with pregestational diabetes mellitus (PGDM). METHODS: Retrospective cohort investigation of 282 women with PGDM who intended to BF and their asymptomatic infants admitted to the newborn nursery for blood glucose monitoring and routine care. Early feeding was defined by the initial feeding if given within four hours of birth. RESULTS: Of the 282 mother-infant dyads, for 134 (48%) early feeding was BF and for 148 (52%) early feeding was FF. Times from birth to BF and FF (median 1 hr, 0.3-6) were similar, while the time to first BF for those who FF and supplemented was longer (median 6 hr., 1-24). Ninety-seven infants (72%) who first BF also supplemented. Of these, 22 (23%) BF exclusively, 67 (69%) BF partially and 8 (8%) FF at discharge. One hundred seventeen (79%) who first FF also supplemented. Of these, 21 (18%) BF exclusively, 76 (65%) BF partially and 20 (17%) FF at discharge. CONCLUSION: Regardless of the type of first feeding, the majority of infants born to women with PGDM require supplementation. Even when medically indicated, in-hospital supplementation is an obstacle, albeit not absolute, to exclusive BF at discharge. Parents should be reminded that occasional supplementation should not deter resumption and continuation of BF.


Subject(s)
Breast Feeding/statistics & numerical data , Dietary Supplements/statistics & numerical data , Infant Formula/statistics & numerical data , Pregnancy in Diabetics , Adult , Delivery, Obstetric/statistics & numerical data , Female , Hospitalization/statistics & numerical data , Humans , Hypoglycemia/congenital , Hypoglycemia/diet therapy , Infant , Infant, Newborn , Maternal Age , Pregnancy , Retrospective Studies
8.
Mol Genet Metab ; 126(4): 355-361, 2019 04.
Article in English | MEDLINE | ID: mdl-30846352

ABSTRACT

BACKGROUND: Regular carbohydrate intake to avoid hypoglycemia is the mainstay of dietary treatment in glycogen storage disease type I (GSDI). The aim of this study was to evaluate the quality of dietary treatment and glycemic control in a cohort of GSDI patients, in relation to the presence of typical long-term complications. METHODS: Data of 25 patients (22 GSD subtype Ia and 3 GSDIb, median age 20y) from the Swiss hepatic glycogen storage disease registry was analyzed cross-sectionally. Frequency and type of hypoglycemia symptoms were assessed prospectively using a structured questionnaire. Diagnostic continuous glucose monitoring (CGM) was performed as part of usual clinical care to assess glycemic control in 14 patients, usually once per year with a mean duration of 6.2 ±â€¯1.1 consecutive days per patient per measurement. RESULTS: Although maintenance of euglycemia is the primary goal of dietary treatment, few patients (n = 3, 13%) performed capillary blood glucose measurements regularly. Symptoms possibly associated with hypoglycemia were present in 13 patients (57%), but CGM revealed periods of low glucose (<4 mmol/l) in all patients, irrespective of the presence of symptoms. GSDIa patients with liver adenomas (n = 9, 41%) showed a higher frequency and area under the curve (AUC) of low blood glucose than patients without adenomas (frequency 2.7 ±â€¯0.8 vs. 1.5 ±â€¯0.7 per day, AUC 0.11 ±â€¯0.08 vs. 0.03 ±â€¯0.02 mmol/l/d; p < 0.05). Similarly, the presence of microalbuminuria was also associated with the frequency of low blood glucose. Z-Scores of bone density correlated negatively with lactate levels. CONCLUSION: The quality of glucose control is related to the presence of typical long-term complications in GSDI. Many patients experience episodes of asymptomatic low blood glucose. Regular assessment of glucose control is an essential element to evaluate the quality of treatment, and increasing the frequency of glucose self-monitoring remains an important goal of patient education and motivation. CGM devices may support patients to optimize dietary therapy in everyday life.


Subject(s)
Blood Glucose/analysis , Glycogen Storage Disease Type I/complications , Glycogen Storage Disease Type I/epidemiology , Adenoma, Liver Cell/etiology , Adolescent , Adult , Bone Density , Cohort Studies , Cross-Sectional Studies , Female , Glucose/administration & dosage , Glycogen Storage Disease Type I/diet therapy , Humans , Hypoglycemia/complications , Hypoglycemia/diet therapy , Hypoglycemia/epidemiology , Male , Registries , Switzerland , Young Adult
9.
MCN Am J Matern Child Nurs ; 44(3): 157-163, 2019.
Article in English | MEDLINE | ID: mdl-30882766

ABSTRACT

PURPOSE: The purpose of this study was to explore maternal child nurses' knowledge and beliefs about using pasteurized donor human milk (PDHM) to treat newborns with hypoglycemia. Pasteurized donor human milk has been used for decades in neonatal intensive care units, but its use is relatively new in the well-baby population. STUDY DESIGN AND METHODS: Focus groups of maternal child nurses were conducted to explore this topic. RESULTS: Six focus groups that included a total 20 nurses were held. Four themes were identified: 1) nurses presumed safety of PDHM but lacked knowledge, 2) nurses' role as patient-family advocate, 3) nurses' logistical concerns about implementation of PDHM, and 4) nurses lacked clarity on formal milk sharing versus PDHM. CLINICAL IMPLICATIONS: As the use of PDHM increases for well babies, nurses will need more education about PDHM, its safety profile, its use in breastfeeding support and protection of the infant microbiome, and how PDHM differs from informal milk sharing. Nurses play an important role in helping parents weigh risks and benefits of using PDHM or formula when supplementation is needed during the hospital stay. It is important that nurses feel confident in their own knowledge and ability to address parental concerns so they can advocate for their patients and support parental decision-making.


Subject(s)
Hypoglycemia/diet therapy , Infant Formula/statistics & numerical data , Milk, Human/metabolism , Pasteurization , Breast Feeding/methods , Clinical Competence , Focus Groups/methods , Humans , Infant, Newborn , Intensive Care Units, Neonatal/organization & administration , Intensive Care Units, Neonatal/statistics & numerical data , Milk Banks/statistics & numerical data , Nurses/psychology , Nurses/standards , Qualitative Research
10.
BMJ Case Rep ; 20182018 Jun 19.
Article in English | MEDLINE | ID: mdl-29925555

ABSTRACT

Hypoglycaemia is a well-documented 'stroke mimic'. The literature documents numerous case reports of patients presenting with focal neurological deficits subsequently attributed to hypoglycaemia. The significant majority of these cases are found in patients with pre-existing diabetes. We present case histories of two patients with no history of diabetes presenting to secondary care with reactive hypoglycaemia causing transient symptoms that were responsible for referral as a possible transient ischaemic attack. Although uncommon, metabolic disturbances such as hypoglycaemia should be considered in all patients presenting as a suspected stroke, even if they are not known to have diabetes.


Subject(s)
Hypoglycemia/complications , Ischemic Attack, Transient/etiology , Aged , Aged, 80 and over , Blood Glucose/analysis , Brain/diagnostic imaging , Carotid Arteries/diagnostic imaging , Carotid Artery Diseases/diagnosis , Diagnosis, Differential , Glucose Tolerance Test , Humans , Hypoglycemia/diagnosis , Hypoglycemia/diet therapy , Magnetic Resonance Imaging , Male , Risk Factors , Ultrasonography, Doppler
11.
Nutrients ; 10(6)2018 May 25.
Article in English | MEDLINE | ID: mdl-29799438

ABSTRACT

Post-bariatric hypoglycemia (PBH) is an increasingly recognized long-term complication of bariatric surgery. The nutritional treatment of PBH includes a high-fiber diet and the restriction of soluble and high-glycemic index carbohydrates; however, these measures are not always enough to prevent hypoglycemia. We evaluated the efficacy of uncooked cornstarch, a low-glycemic index carbohydrate characterized by slow intestinal degradation and absorption, in addition to a high-fiber diet, for the treatment of PBH. We report the cases of two young women suffering from severe postprandial and fasting hypoglycemia following Roux-en-Y gastric bypass (RYGB). The patients underwent Continuous Glucose Monitoring (CGM) before and 12⁻16 weeks after the administration of uncooked cornstarch (respectively 1.25 g/kg b.w. and 1.8 g/kg b.w.) in addition to a high-fiber diet. In both patients, CGM showed more stable glucose levels throughout monitoring, a remarkable reduction of the time spent in hypoglycemia (.


Subject(s)
Blood Glucose/metabolism , Dietary Fiber/administration & dosage , Gastric Bypass/adverse effects , Glycemic Index , Hypoglycemia/diet therapy , Starch/administration & dosage , Adult , Biomarkers/blood , Dietary Fiber/metabolism , Female , Humans , Hypoglycemia/blood , Hypoglycemia/diagnosis , Hypoglycemia/etiology , Nutritional Status , Starch/metabolism , Time Factors , Treatment Outcome
12.
J Inherit Metab Dis ; 41(6): 917-927, 2018 11.
Article in English | MEDLINE | ID: mdl-29802555

ABSTRACT

Management of liver glycogen storage diseases (GSDs) primarily involves maintaining normoglycemia through dietary modifications and regular glucose monitoring. Self-monitoring of blood glucose is typically done 3-6 times per day, and may not sufficiently capture periods of asymptomatic hypoglycemia, particularly during sleep. Continuous glucose monitoring systems (CGMS) provide 24-h continuous glucose data and have been used effectively in diabetes mellitus to monitor metabolic control and optimize treatment. This is a relatively new approach in GSDs with only a handful of studies exploring this modality. In this study we used Dexcom CGMS to study the glycemic profile of 14 pediatric and six adult patients with GSD I, III, and IX. A total of 176 days of CGMS data were available. The CGMS was found to be a reliable tool in monitoring glucose levels and trends at all times of the day with good concordance with finger-stick glucose values. This study revealed that in addition to overnight hypoglycemia, CGMS can uncover previously undetected, subclinical, low glucose levels during daytime hours. Additionally, the CGMS detected daytime and overnight hyperglycemia, an often overlooked concern in liver GSDs. The CGMS with concurrent dietary adjustments made by a metabolic dietitian improved metabolic parameters and stabilized blood glucose levels. The CGMS was found to be a safe, effective, and reliable method for optimizing treatment in patients with GSD I, III, and IX.


Subject(s)
Blood Glucose/analysis , Glycogen Storage Disease/blood , Monitoring, Physiologic/instrumentation , Adolescent , Adult , Child , Child, Preschool , Female , Glycogen Storage Disease/diet therapy , Humans , Hyperglycemia/prevention & control , Hypoglycemia/blood , Hypoglycemia/diet therapy , Infant , Liver/metabolism , Male , Middle Aged , Young Adult
13.
Nutr Hosp ; 35(1): 237-244, 2018 Jan 10.
Article in Spanish | MEDLINE | ID: mdl-29565174

ABSTRACT

INTRODUCTION: 3-hydroxy-3-methylglutaryl-CoA (HMG-CoA) lyase deficiency is an autosomal recessive disorder that usually presents in the neonatal period with vomiting, metabolic acidosis, hypoglycemia and absent ketonuria. Few cases are reported in the literature, and optimal dietary management and long term outcome are not fully understood. CASE REPORT: We report a 2 year old girl with HMG-CoA-lyase deficiency who had limited fasting tolerance on a low protein diet, with several recurrent hospital admissions with severe hypoketotic hypoglycaemia and metabolic acidosis. We also review the dietary management and outcome of other reported cases in the literature. DISCUSSION: In order to define optimal dietary treatment, it is important to collect higher numbers of case studies with detailed dietary management, fasting times and outcome.


Subject(s)
Acetyl-CoA C-Acetyltransferase/deficiency , Amino Acid Metabolism, Inborn Errors/diet therapy , Acidosis/diet therapy , Acidosis/etiology , Child, Preschool , Diet, Protein-Restricted , Enteral Nutrition , Female , Gastrostomy , Humans , Hypoglycemia/diet therapy , Hypoglycemia/etiology
14.
Rev. neurol. (Ed. impr.) ; 66(6): 193-200, 16 mar., 2018. tab
Article in Spanish | IBECS | ID: ibc-172285

ABSTRACT

Introducción. La dieta cetogénica es una terapia no farmacológica que se ha usado como alternativa para el tratamiento de la epilepsia refractaria desde 1921. Es una dieta alta en grasas y baja en hidratos de carbono, que se utiliza en el tratamiento de la epilepsia refractaria pediátrica, efectiva en alrededor del 50% de los pacientes que la inician. Se trata de un plan alimentario desequilibrado tanto en macro como en micronutrientes, que puede causar déficits nutricionales en energía, proteínas, minerales y vitaminas, y exceso de lípidos, con riesgo de ocasionar efectos secundarios no deseados tanto en el inicio del tratamiento como de forma tardía. Desarrollo. Se describen los efectos adversos más frecuentes que se presentan en forma aguda o tardíamente, y se brindan herramientas para su manejo y prevención durante el tratamiento para pediatras y equipos de dieta cetogénica. Conclusiones. Algunos efectos adversos son de difícil interpretación, y pueden ser manifestación por afectación a diferentes niveles del organismo, poniendo en duda si son secundarios a la dieta, a la medicación antiepiléptica o a intercurrencias del propio paciente. Es importante seguir el protocolo de frecuencia de estudios y evaluaciones para detectar y prevenir estos efectos, y consultar a centros de referencia evaluando el coste-beneficio de continuar o no el tratamiento (AU)


Introduction. The ketogenic diet is a non-pharmacological treatment that has been used as a therapeutic alternative for the management of refractory epilepsy since 1921. It is a diet, high in fats and low in carbohydrates, which is used to treat paediatric refractory epilepsy, and is effective in around 50% of the patients that begin to follow it. It is a nutrition plan that is imbalanced in terms of both macro- and micro-nutrients, which can give rise to nutritional deficits in energy, proteins, minerals and vitamins, as well as an excess of lipids. There is therefore a risk of it causing undesirable side effects both at the outset and in later stages of the treatment. Development. The most frequent adverse effects that may occur either in acute form or in later stages are described, and tools that can be used in their management and prevention during treatment are proposed for paediatricians and ketogenic diet teams. Conclusions. Some adverse effects are difficult to interpret and may manifest due to involvement at different levels of the organism, thereby arousing doubts as to whether they are caused by the diet, the antiepileptic medication or by the patient's own intercurrent complications. It is important to follow the study and evaluation frequency protocol in order to detect and prevent these effects, as well as to consult reference centres in order to evaluate the cost-benefit of continuing the treatment or not (AU)


Subject(s)
Humans , Male , Female , Child , Epilepsy/complications , Epilepsy/diet therapy , Diet, Ketogenic , Hypoglycemia/complications , Hypoglycemia/diet therapy , Acidosis/complications , Diet, Ketogenic/adverse effects , Clinical Protocols , Hyperlipidemias/complications , Hyperlipidemias/diet therapy , Cardiovascular Diseases/complications , Cardiovascular Diseases/diet therapy
15.
Nutr. hosp ; 35(1): 237-244, ene.-feb. 2018. tab, graf
Article in English | IBECS | ID: ibc-172112

ABSTRACT

Introduction: 3-hydroxy-3-methylglutaryl-CoA (HMG-CoA) lyase deficiency is an autosomal recessive disorder that usually presents in the neonatal period with vomiting, metabolic acidosis, hypoglycemia and absent ketonuria. Few cases are reported in the literature, and optimal dietary management and long term outcome are not fully understood. Case report: We report a 2 year old girl with HMG-CoA-lyase deficiency who had limited fasting tolerance on a low protein diet, with several recurrent hospital admissions with severe hypoketotic hypoglycaemia and metabolic acidosis. We also review the dietary management and outcome of other reported cases in the literature. Discussion: In order to define optimal dietary treatment, it is important to collect higher numbers of case studies with detailed dietary management, fasting times and outcome (AU)


Introducción: la deficiencia de la 3-hidroxi-3-metilglutaril-CoA (HMG-CoA) liasa es un desorden autosómico recesivo que normalmente se presenta en la infancia con vómitos, acidosis metabólica, hipoglicemia y sin cetonuria. Se han publicado pocos casos en la literatura científica sobre el mejor tratamiento dietético para el adecuado desarrollo de los pacientes a largo plazo, por lo que esta deficiencia no es bien conocida. Caso clínico: presentamos una niña de 2 años con deficiencia de la 3-hidroxi-3-metilglutaril-CoA (HMG-CoA) liasa. Recibiendo una dieta baja en proteína con una tolerancia de ayuno limitada con episodios recurrentes de admisión hospitalaria con hipoglicemia hipoketotica y acidosis metabólica. También hemos revisado el tratamiento dietético y el desarrollo de otros casos publicados en la literatura científica. Discusión: es importante recoger más casos clínicos describiendo el tratamiento dietético seguido, el tiempo máximo de ayuno y el desarrollo de los pacientes con el objetivo de definir el mejor tratamiento (AU)


Subject(s)
Humans , Female , Infant , Hydroxymethylglutaryl-CoA Synthase/deficiency , Metabolism, Inborn Errors/diet therapy , Ketosis/diet therapy , Leucine/adverse effects , Dietary Fats/adverse effects , Hypoglycemia/diet therapy
16.
Biosci Biotechnol Biochem ; 82(1): 114-119, 2018 Jan.
Article in English | MEDLINE | ID: mdl-29207918

ABSTRACT

Green-plant membrane is a phytonutrient present in green leafy vegetables at high concentration. Postprandial increases in blood triglyceride levels result in insulin resistance and type 2 diabetes. Additionally, dietary life and eating order also affect postprandial hypertriglyceridemia. In this study, the effects of once-daily intake of green-plant membrane with dietary oil on postprandial hypertriglyceridemia were investigated in vitro and in vivo. In vitro, green-plant membrane bound hydrophobic bile acids but did not inhibit pancreatic lipase activity. Following the administration, green-plant membrane with dietary oil in rats, oral fat tolerance tests, increases in serum triglycerides levels were significantly reduced. Moreover, fecal total lipid and bile acid volumes were significantly increased in rats that administered 200 mg/mL green-plant membrane. These results suggest that green-plant membrane with dietary oil inhibits dietary fat absorption via promotion of bile acid excretion in feces and the effectiveness of eating green-plant membrane, such as green leafy vegetables, with meals.


Subject(s)
Bile Acids and Salts/biosynthesis , Dietary Fats , Hypoglycemia/diet therapy , Plants/chemistry , Spinacia oleracea/chemistry , Animals , Brassica/chemistry , Dietary Fats/administration & dosage , Dietary Fats/pharmacology , Rats , Triglycerides/blood , Vegetable Products
17.
Mol Genet Metab ; 123(3): 326-330, 2018 03.
Article in English | MEDLINE | ID: mdl-29223626

ABSTRACT

BACKGROUND: Glycemic control in hepatic glycogen storage diseases (GSDs) relies on specific nutritional recommendations, including strict avoidance of a fasting period. Uncooked cornstarch (UCCS) is an important therapeutic component. A new modified UCCS, Glycosade™, was created with the objective of prolonging euglycemia. We aimed to determine the length of euglycemia on Glycosade™ using a continuous glucose monitor (CGM) and to evaluate whether longer euglycemia and thus less nighttime interruptions would improve sleep and quality of life (QoL) after the introduction of the modified cornstarch. METHODS: We conducted a prospective cohort study to assess quality and quantity of sleep and quality of life (QoL) in patients with GSDs on standard UCCS and after the introduction of Glycosade™. Sleep and QoL evaluation was done for patients using validated questionnaires, a standardized sleep diary and actigraphy. Length of fast and glucose variability were determined with CGM. RESULTS: Nine adults with GSD Ia took part in the study. Glycosade™ introduction was done under close supervision during a hospital admission. Comparison of sleep in 9 patients showed sleep disturbances on standard UCCS that were improved with Glycosade™. QoL was normal both pre and post Glycosade™. The CGM confirmed maintenance of a longer fasting period with Glycosade™ at home. CONCLUSION: Glycosade™ represents an alternative option for GSD patients. We showed possible benefits in terms of sleep quality. We also confirmed the longer length of fast on Glycosade™. SYNOPSIS: A new modified form of uncooked starch for patients with glycogen storage disease represents an alternative option as it showed a longer length of fast and improvements in sleep quality.


Subject(s)
Fasting/physiology , Glycogen Storage Disease/physiopathology , Hypoglycemia/diet therapy , Quality of Life , Sleep/physiology , Starch , Actigraphy , Adult , Blood Glucose/physiology , Female , Glucose/administration & dosage , Glycogen Storage Disease/blood , Humans , Hypoglycemia/blood , Hypoglycemia/drug therapy , Hypoglycemia/physiopathology , Male , Middle Aged , Prospective Studies , Treatment Outcome , Young Adult
18.
Nutr. hosp ; 34(5): 1252-1259, sept.-oct. 2017. tab
Article in Spanish | IBECS | ID: ibc-167587

ABSTRACT

El paciente crítico presenta una respuesta metabólica a la agresión que afecta al metabolismo hidrocarbonado, lo cual causa hiperglucemia y aumento de la variabilidad glucémica y lo hace susceptible a la infección, con aumento de morbilidad y mortalidad. Asimismo, se han detectado hipoglucemias graves como consecuencia del tratamiento insulínico intensivo, que provocan un efecto deletéreo en su evolución clínica, por lo que un correcto control de la glucemia plasmática contribuiría a reducir morbilidad y mortalidad. En el paciente crítico, el metabolismo de la glucosa se encuentra en fase de allostasis como consecuencia del estrés metabólico, produciéndose un aumento de resistencia periférica a la insulina que provoca un desequilibrio con la función de las células beta pancreáticas, que aumentan la secreción de insulina para mantener la glucosa plasmática en rangos de normalidad. Son numerosos los estudios publicados sobre el tratamiento con insulina y la variabilidad glucémica, y escasos los que se refieren al tratamiento nutrometabólico de la hiperglucemia del paciente crítico. De todos ellos podemos deducir que se recomienda mantener la glucemia siempre inferior a 180 mg/dl y, a ser posible, no superior a 150 mg/dl, estableciéndose un rango inferior de 110-140 mg/dl. Además, el control estricto de la glucemia aumenta el riesgo de hipoglucemias graves (≤ 40 mg/dl) y de la consiguiente mortalidad, por lo que debe desaconsejarse. Asimismo, la variabilidad glucémica aumenta de forma independiente la mortalidad en el paciente crítico, por lo que los protocolos deben dirigirse a evitarla. El tratamiento nutricional con dietas específicas para diabetes no solo mejora el control de la hiperglucemia y disminuye las necesidades de insulina sino que disminuye la variabilidad glucémica y puede disminuir la incidencia de complicaciones infecciosas, por lo que se recomienda, al menos durante la primera semana de estancia. Por último, la diabetes parece que modula las consecuencias de la hiperglucemia en el paciente crítico, por lo que los pacientes con diabetes podrían beneficiarse de un objetivo glucémico más elevado que aquellos sin diabetes pero con hiperglucemia de estrés (AU)


Critically ill patients show a metabolic response to injury that affects carbohydrate metabolism, causing hyperglycemia and an increase in glycemic variability that makes the critically ill patient susceptible to infection, resulting in morbidity and mortality increase. Also, severe hypoglycemia was detected as a consequence of intensive insulin treatment that provokes deleterious effects in their clinical evolution, so a correct monitoring of plasma glucose would contribute to reduce morbidity and mortality. In critically ill patients, glucose metabolism is in allostasis stage as a consequence of metabolic stress, producing an increase in peripheral resistance to insulin that causes an imbalance with the pancreatic beta-cell function, increasing insulin secretion to maintain plasma glucose levels within normality ranges. Numerous studies have been published about treatments with insulin and glycemic variability, whereas there are very few about nutrometabolic treatment of hyperglycemia in critically ill patients. Of all of them we can conclude that it is always recommended to keep glucose levels under 180 mg/dl, and when possible, not over 150 mg/dl, establishing a lower range of 110-140 mg/dl. Moreover, tight glycemic control increases the risk of severe hypoglycemia (≤ 40 mg/dl) and its subsequent mortality, so we advise against it. Besides, glycemic variability has been independently associated with an increase of mortality in critically ill patients and, consequently, protocols should aim at avoiding it. Nutritional treatment with diabetes-specific diets not only improves hyperglycemic control and decreases insulin needs, but also decreases glycemic variability and could reduce the incidence of infectious complications. Therefore, they are recommended, at least during the first week of stay. Finally, diabetes seems to modulates the consequences of hyperglycemia in critically ill patients, so diabetic patients could benefit from a higher glycemic target than those without diabetes but with stress hyperglycemia (AU)


Subject(s)
Humans , Male , Middle Aged , Hyperglycemia/complications , Hyperglycemia/diet therapy , Critical Illness/epidemiology , Hypoglycemia/diet therapy , Enteral Nutrition/methods , Communicable Diseases/complications , Body Mass Index , Hypertension/complications , Tobacco Use Disorder/complications , Diuresis/physiology , Insulin/administration & dosage , Blood Glucose/analysis
19.
Neonatal Netw ; 36(4): 233-238, 2017 Jul 01.
Article in English | MEDLINE | ID: mdl-28764827

ABSTRACT

Hospitals are challenged to implement measures to improve health outcomes, decrease costly interventions, and increase patient satisfaction. By following a nurse-driven protocol, our institution has successfully met these three challenges in our treatment of newborns diagnosed with neonatal hypoglycemia (NH). Based on results of a randomized clinical trial, a multidisciplinary team trialed glucose gel as a standard treatment for NH. During the first year, admission rates to the NICU for NH decreased by 73 percent. Exclusive breastfeeding rates for this population increased to 49 percent and 40 additional families remained together on the mother baby unit. This practice change is improving health outcomes, decreasing expensive interventions, and increasing satisfaction among the population of infants at risk for NH.


Subject(s)
Gels/therapeutic use , Glucose/therapeutic use , Hypoglycemia/diet therapy , Infant, Newborn, Diseases/diet therapy , Intensive Care Units, Neonatal/standards , Neonatal Nursing/standards , Practice Guidelines as Topic , Female , Humans , Infant, Newborn
20.
Curr Diab Rep ; 17(9): 74, 2017 Sep.
Article in English | MEDLINE | ID: mdl-28755062

ABSTRACT

PURPOSE OF REVIEW: The purpose of this review is to discuss strategies to reduce rates of hypoglycemia in the non-critical care setting. RECENT FINDINGS: Strategies to reduce hypoglycemia rates should focus on the most common causes of iatrogenic hypoglycemia. Creating a standardized insulin order set with built-in clinical decision support can help reduce rates of hypoglycemia. Coordination of blood glucose monitoring, meal tray delivery, and insulin administration is an important and challenging task. Protocols and processes should be in place to deal with interruptions in nutrition to minimize risk of hypoglycemia. A glucose management page that has all the pertinent information summarized in one page allows for active surveillance and quick identification of patients who may be at risk of hypoglycemia. Finally, education of prescribers, nurses, food and nutrition services, and patients is important so that every member of the healthcare team can work together to prevent hypoglycemia. By implementing strategies to reduce hypoglycemia, we hope to lower rates of adverse events and improve quality of care while also reducing hospital costs. Future research should focus on the impact of an overall reduction in hypoglycemia to determine whether the expected benefits are achieved.


Subject(s)
Critical Care , Hospitals , Hypoglycemia/therapy , Blood Glucose/analysis , Humans , Hypoglycemia/blood , Hypoglycemia/diet therapy , Hypoglycemia/etiology , Hypoglycemic Agents/therapeutic use , Insulin/therapeutic use , Risk Factors
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