Prolonged Intravenous Feeding Research Articles

Wernicke Encephalopathy in a Pediatric Patient with Avoidant Restrictive Food Intake Disorder: A Rare Presentation of Thiamine Deficiency.

BACKGROUND Wernicke encephalopathy is traditionally associated with chronic alcoholism, nutritional imbalance, prolonged intravenous feeding, hyperemesis, anorexia nervosa, and malabsorption syndromes. We report a case of Wernicke's encephalopathy in a 12-year-old girl with avoidant restrictive food intake disorder. CASE REPORT The patient had lost 45.4 kg of body weight due to self-imposed changes to her diet, before presenting with decreased oral intake for 2-3 weeks, intermittent nausea, crampy epigastric pain, and post-prandial emesis. Her weight on admission was 78.2 kg. She received intravenous fluids of dextrose 5% with normal saline while she initially attempted to eat, but the post-prandial emesis persisted. She developed a fear of vomiting, which led to even more severe food intake restriction. After a week, she began to report double vision and blurred peripheral vision, with physical findings of nystagmus and an ataxic gait. She was empirically started on thiamine after negative neurology workup, with improvement of her gait, blurry vision, and nystagmus. Thiamine deficiency was later confirmed. CONCLUSIONS In patients with large amounts of weight loss presenting with neurological symptoms, Wernicke's encephalopathy must be considered in the differential diagnosis. Avoidant restrictive food intake disorder is rarely reported to cause Wernicke's encephalopathy. To the best of our knowledge, this is the first pediatric case demonstrating that Wernicke encephalopathy can occur in this type of eating disorder and not just in anorexia nervosa.

Anorexia nervosa and Wernicke-Korsakoff syndrome: case report an literature review

IntroductionWenicke-Korsakoff syndrome (WKS) is a neurological disorder caused by thiamine deficiency. Wernicke Encephalopathy (WE) is the acute phase and the chronic phase is called Korsakoff-syndrome (KS).ObjectivesTo review the current literature on the management of WKS in a patient with anorexia nervosa.MethodsWe report the case of a 63-year-old woman admitted to the Psychiatry Unit after weight loss in the last 3 months (from 39 kg to 33,500 kg). She only made one meal a day. By exploration and analysis, neoplastic disease is ruled out (thoraco-abdomino-pelvic CT without pathological findings). She has maintained restrictive intakes for more than 30 years. A long-term anorexia nervosa (AN) is suspected, with a worsening of restrictive behavior in recent months. Upon admission, she has a weight of 33,500 kg and a BMI of 14,10. She has a left palpebral ptosis and an alteration of the anterograde memory as well as affectation of executive functions. Progressive oral diet is started, and due to the suspicion of a WKS, thiamine ev is started for a week and then continued with oral thiamine. Thiamine levels are extracted once the ev treatment has begun, so we do not have previous levels to know if they were decreased. Brain MRI shows bilateral hyperintensities in white matter and at supratentorial level in T2 and FLAIR. After a month and a half of admission, the patient has progressively regained weight, has managed to make adequate intakes and has improvement in memory.ResultsAn adverse consequence of severe malnutrition in AN due to severe food restriction and purging behavior is thiamine deficiency, and also global cerebral atrophy and concomitant cognitive deficits can be found. Thiamine deficiency occurs in 38% of individuals with AN and is often unrecognized. WKS is caused by thiamine deficiency, and WE is the acute phase of this syndrome (presentation of triad can vary). The chronic phase is KS and consists in amnesia with confabulations. WKS typically develops after malnourishment in alcoholic patients but can be associated in nonalcoholic such as prolonged intravenous feeding, hyperemesis, anorexia nervosa, refeeding after starvation, thyrotoxicosis, malabsorption syndromes; hemodialysis; peritoneal dialysis; AIDS; malignancy. WKS is a clinical diagnosis, and no specific abnormalities have been found in cerebrospinal fluid, brain imaging or electroencephalograms. MRI has a sensitivity of 53%, but high specificity of 93%, and shows an increased signal in T2 and FLAIR sequences, bilaterally symmetrical in the paraventricular regions of the thalamus, the hypothalamus, mamillary bodies, the periaquedutal region, the floor of the fourth ventricle and midline cerebellum.ConclusionsIf the disorder is suspected, thiamine should be initiated immediately in order to prevent irreversible brain damage, with an estimated mortality rate of about 20%, or to the chronic form of the WE in up to 85% of survivorsDisclosure of InterestNone Declared

Wernicke′s encephalopathy following hyperemesis gravidarum

Wernicke's encephalopathy (WE) is a potentially reversible yet serious neurological manifestation caused by vitamin B1(thiamine) deficiency. It is commonly associated with heavy alcohol consumption. Other clinical associations are with hyperemesis gravidarum (HG), starvation, and prolonged intravenous feeding. Most patients present with the triad of ocular signs, ataxia, and confusion. It can be associated with life-threatening complication like central pontine myelinolysis (CPM). We report two cases of WE following HG, with two different outcomes.

Complications of treatment in the neonatal period

The lifesaving therapies and procedures used in the care of neonates may result in untoward effects and complications. Retinitis of prematurity (ROP) may present as a spectrum of disease ranging from spontaneously resolving vasculopathy to retinal detachment and blindness. Total parenteral nutrition (TPN) is found in 40–60% of infants requiring prolonged intravenous feeding for intestinal failure. Host factors combined with deleterious components of TPN play a role. There is cholestasis, steatosis, portal inflammation and oedema, ductopenia and fibrosis. Sudden unexpected death (SUD) is the typical presentation of cardiac tamponade due to TPN solution entering the pericardial sac. Arterial cannulation for frequent blood pressure monitoring and blood sampling may be complicated by vasospasm, thrombosis, haematoma, infection, breakage, migration, displacement, peripheral nerve damage, ischaemia and tissue necrosis. The immature central nervous system is particularly susceptible to cerebrovascular damage. Periventricular leukomalacia (PML) with destruction of white matter and severe germinal matrix haemorrhage with intraventricular rupture and infarction may occur. Bronchopulmonary dysplasia (BPD) in the post-surfactant era is confined to very low birth weight infants. There is disturbed alveogenesis with large and simplified airspaces, interstitial fibrosis, and disruption of microvascular development.

Non-alcoholic acute Wernicke's encephalopathy: Role of MRI in non typical cases

AimAcute Wernicke's encephalopathy (WE) is a severe neurological disorder caused by thiamine deficiency, most commonly found in chronic alcoholics. It is not so easy to suspect acute WE when the clinical picture does not include all the typical symptoms and alcohol abuse is not reported. Three rare cases of Wernicke's encephalopathy (WE) in non-alcoholic patients are reported. Cases presentationTwo patients developed the disease following prolonged intravenous feeding, the third was carrying a gastric lymphoma. None of them presented with the classic clinical triad of WE (ophtalmoplegia/nystagmus, ataxia and consciousness disturbance), showing just one or two of the typical symptoms. Brain Magnetic Resonance Imaging (MRI) represented the key tool to suspect and define WE diagnosis, showing a picture characterized by bilaterally altered signal of the thalamic pulvinar, mesencephalic cup, mammillary bodies, periaqueductal grey matter and floor of fourth ventricle. All patients dramatically improved within 48h after administration of thiamine. ConclusionWe emphasize that WE should be suspected in all patients showing typical MRI features presenting with at least one of the clinical triad of WE.

Loss of Vision: A Rare Presentation In Hyperemesis Gravidarum Induced Wernicke’s Encephalopathy

Wernicke’s encephalopathy (WE) is a potentially fatal but readily reversible medical emergency caused by thiamine deficiency. WE is most commonly associated with heavy alcohol consumption, but is also seen in other clinical settings such as Hyperemesis Gravidarum (HG), starvation, and prolonged intravenous feeding. After Sheehan’s first description of WE following HG there are few case reports of similar etiology from various parts of the world [1,2,3,4]. WE is popularly known and taught as the clinical triad of ophthalmoplegia, confusion and ataxia.We discuss a pregnant woman with HG presenting with visual deterioration and her optic fundi showing optic disc edema on right side and retinal hemorrhage on left side. Its a rare initial presentation of WE.

Promoting Major Pediatric Surgical Care in a Low‐Income Country: A 4‐Year Experience in Eritrea

Access to pediatric surgical care in many sub-Saharan African countries is strongly limited by lack of medical facilities, adequate transport system, and trained medical and nursing manpower. The mortality rate for major congenital abnormalities remains extremely elevated in this area of the world. Strong efforts have been spent during the past decades to elevate the level of pediatric surgery standards in these countries through cooperation programs acting through periodical medical missions or supporting local medical teaching institutions. This is a report of a partnership between an Italian Medical Institution and the Eritrean Ministry of Health with the goal to improve pediatric surgical standard of care in the country. During the past 4 years, teams composed of two pediatric surgeons, a pediatric anesthetist, and a pediatric nurse regularly visited the Orotta Medical and Surgical Referral Hospital of Asmara (Eritrea) to offer modern surgical treatment to children with major congenital abnormalities and to offer academic training to local medical and nursing staff. The team worked in local health structures. A total of 714 patients have been visited and 430 surgical procedures have been performed during 35 weeks of clinical work. Among them were 32 anorectal malformations, 11 Hirschsprung's disease cases, 8 bladder extrophies, and many other major surgical problems, such as congenital intestinal obstructions, obstructive uropathies, and solid tumors. The standard of care has been based on the principle of researching sustainable solutions. Surgical options and timing of each procedure have been decided to reduce hospitalization and the recourse to temporary stomas, indwelling catheterization, and prolonged intravenous feeding. Posterior sagittal anorectoplasties (PSARP) and posterior vagino-anorectoplasty (PSVARP) were performed for anorectal malformations, introducing minimal technical variants to reduce the need for postoperative nursing. Endorectal pull-through of functional stoma was the treatment of choice for patients with Hirschsprung's disease because frozen sections were not possible. Eight late-referred bladder extrophy cases were all managed by internal diversion (Mainz II pouch). Solid abdominal tumors always came to observation weeks or months after the first symptoms appeared. No CT scan was available and indications of surgery were based on clinical symptoms only. Only 11 of 18 cases were resectable, and only 5 of them with favorable histology survived, 2 after adjuvant therapy abroad. A large number of hypospadias were observed at the mean age of 4.5 years. Failures of previous attempts at correction were frequently found. The postoperative complications rate progressively decreased with the use of dripping stents to avoid the risk of accidental catheter removal or kinking. On the basis of our experience, major pediatric surgery in many under-resourced areas of sub-Saharan Africa can be developed, taking care to adapt surgical options to local conditions. Late referral of many congenital abnormalities, the impact of local culture, difficulties to establish regular follow-up, and shortage of facilities and medical devices must always be kept in mind before transferring modern protocols of management. Strong efforts have been devoted to train local medical and nursing staff to establish pediatric surgical manpower to cope with a still largely unanswered demand of care in this area of Africa.

Lesson of the Week: Wernicke's encephalopathy after vertical banded gastroplasty for morbid obesity

Thiamine deficiency is known to lead to certain neurological sequelae including Wernicke- Korsakoff encephalopathy. Signs attributable to this condition include ataxia, ophthalmoplegia, nystagmus, and mental confusion. Recognised predisposing conditions include alcoholism gastric carcinoma, pyloric obstruction, hyperemesis gravidarum, and prolonged intravenous feeding. We have recently encountered two cases of Wernicke's encephalopathy after vertical banded gastroplasty for morbid obesity . Other neurological sequelae are recognised after vertical banded gastroplasty, including Guillain-Barre syndrome, psychosis, and pseudoathetosis, but the causes are multifactorial.

CaxP and Ca/P in the parenteral feeding of preterm infants.

Preterm infants requiring prolonged intravenous feeding frequently develop pathologic fractures and rickets. Infants who receive large amounts of calcium have fewer fractures. This observation led us to determine the maximal amounts of calcium and phosphate that can be added to parenteral nutrition solutions without the precipitation of calcium phosphate and to determine the optimal ratio of calcium to phosphate in these solutions. Clinical observations and in vitro experiments indicate that the product of calcium x phosphate (CaxP) in the dextrose-amino acid solution should not exceed 75 square millimolar (square millimole per square liter) to prevent calcium phosphate precipitation in barium-impregnated silicone rubber catheters and should not exceed 100 square millimolar in solutions administered through peripheral veins. Seven intake and output studies were performed in preterm infants to determine the ratio of calcium to phosphate (Ca/P) in the total parenteral nutrition solutions that minimized urinary losses. A Ca/P ratio of 5.0 minimized the sum of the calcium plus phosphate losses in the urine. However, experience with long-term total parenteral nutrition in preterm infants, awareness of the acute and life-threatening effects of body phosphate depletion, and an unmeasured endogenous enteric calcium secretion all suggest that a Ca/P ratio of approximately 3.0 provides a safer compromise between the acute and serious complications of phosphate deficiency and the chronic problems of fractures and rickets due to calcium deficiency.

A patient with copper deficiency anemia while on prolonged intravenous feeding: G. Joffe, A. Etzioni, J. Levy, et al. Clin Pediatr 20:226–228, (March), 1981

A patient with copper deficiency anemia while on prolonged intravenous feeding: G. Joffe, A. Etzioni, J. Levy, et al. Clin Pediatr 20:226–228, (March), 1981

Lethal familial protracted diarrhoea.

24 children with severe protracted diarrhoea from 10 families, in which at least one sibling was affected, are reported. In two families the siblings were from 1st-cousin marriages, in one family both parents had unaffected children from previous marriages, and in another family the mother had a normal daughter from an earlier marriage. The onset of the diarrhoea was on the first day of life in 12 infants, some time during the first 17 days in 10, and at 13 weeks and 1 year 6 days in the remaining two. In each case the diarrhoea was `cholera-like'. Investigations failed to show any of the established causes of protracted diarrhoea and 21 (87·5%) infants died after an illness that had lasted between 12 days and 6 years 38 weeks, despite periods of prolonged intravenous feeding and the administration of a wide variety of pharmacological agents. The 2 patients who recovered appeared to do so spontaneously. 14 (58%) had associated extra-gastrointestinal or gastrointestinal-related anomalies. Steady-state perfusion studies were performed in the proximal jejunum of 2 patients, and in the colon of one. In both cases the jejunum was in a net secretory state with respect to water, glucose absorption was markedly reduced, and the transmural potential difference was also depressed; in one of these patients fructose absorption was also reduced, and in the other colonic function appeared to be normal. These studies suggest that the diarrhoea resulted from small intestinal secretion overwhelming the reabsorptive capacity of a normally-functioning colon. Although this series of lethal protracted diarrhoea does not represent a single disease entity, the familial pattern suggests an autosomal recessive mode of inheritance for at least one of the conditions.

Enhancement of fat elimination during intravenous feeding.

ABSTRACT. Forget, P. P. F. X., Fernandes, J. and Haverkamp Begemann, P. (Department of Paediatrics, Sophia Children's Hospital and Neonatal Unit, Erasmus University, Rotterdam, the Netherlands). Enhancement of fat utilization during prolonged intravenous feeding. AdaPaediatrScand, 63:750, 1974.‐An 8‐year‐old girl with severe underweight caused by anorexia nervosa was treated with total intravenous nutrition for 4 weeks. During this period the Intralipid dose was stepwise increased, the doses of Vamin and glucose were kept constant. The Intralipid dose was monitored by the determination of the serum Intralipid levels. The fat utilization was investigated by intravenous fat tolerance tests and the estimation of postheparin lipoprotein lipase activity of the plasma. The Intralipid elimination constant increased from 796 to 2296/min, the postheparin lipoprotein lipase activity increased from 50 to 317 μEq fatty acid/min/l. These data enabled us to increase the Intralipid dose from 3 g fat/kg/per day to 8 g fat/kg/per day, without an increase of the triglyceride blood levels. We may conclude that lipoprotein lipase is an inducible enzyme. It is not clear which component of the hyper‐caloric intravenous regime causes this induction.

Editorial: Prolonged intravenous feeding.

Editorial: Prolonged intravenous feeding.