- Is Parenteral Levothyroxine Therapy Safe in Intractable Hypothyroidism? [Journal Article]
- JNJ Natl Med Assoc 2018; 110(3):245-249
- CONCLUSIONS: There are a few case reports regarding isolated l-thyroxine. We report successful long term results of twice weekly administered intramuscular l-thyroxine therapy. We also draw attention to neurologic side effects of high dose l-thyroxine therapy.
- Metabolic acidosis in short bowel syndrome: think D-lactic acid acidosis. [Journal Article]
- BCBMJ Case Rep 2018 May 16; 2018
- Short bowel syndrome (SBS) is a condition when a person's gastrointestinal function is insufficient to supply the body with essential nutrients and hydration. Patients with SBS suffer from diarrhoea ...
Short bowel syndrome (SBS) is a condition when a person's gastrointestinal function is insufficient to supply the body with essential nutrients and hydration. Patients with SBS suffer from diarrhoea and symptoms of malabsorption such as weight loss, electrolyte disturbances and vitamin deficiencies. Long-term management of this condition can be complicated by the underlying disease, the abnormal bowel function and issues related to treatment like administration of parenteral nutrition and the use of a central venous catheter. Here, we describe a case of D-lactic acid acidosis, a rarer complication of SBS, presenting with generalised weakness and severe metabolic acidosis.
- StatPearls [BOOK]
- BOOKStatPearls Publishing: Treasure Island (FL)
- Immunodeficiency results from a failure or absence of elements of the immune system including lymphocytes, phagocytes and complement system. These immunodeficiencies can be either primary such as Bru...
Immunodeficiency results from a failure or absence of elements of the immune system including lymphocytes, phagocytes and complement system. These immunodeficiencies can be either primary such as Bruton’s disease or secondary as the one caused by HIV infection. Primary ImmunodeficiencyB-cell Deficiencies X- linked Agammaglobulinemia (Bruton’s disease): First described by Bruton. X-linked disorder. Found in male babies expressed around 5 to 6 months of age (maternal IgG disappears). In boys, pre-B cells did not differentiate into mature B lymphocytes. There is a mutation in the gene that encodes for a tyrosine kinase protein. Low level of all immunoglobulins (IgG, IgA, IgM, IgD and IgE) is present. Infants with X-linked agammaglobulinemia suffer from recurrent bacterial infections: otitis media, bronchitis, septicemia, pneumonia, and arthritis, and Giardia lamblia causes intestinal malabsorption. Intermittent injections of large amounts of IgG keep the patient alive, but a patient may die at a younger age if infection with antibiotic-resistant bacteria occurs. Selective Immunoglobulin IgA Deficiencies : IgA deficiency is more common than other deficiencies of immunoglobulins. These patients are more prone to recurrent sinus and lung infections. A malfunctioning in heavy-chain gene switching may cause this problem. Treatment should not include gammaglobulin preparations to prevent hypersensitivity reactions. T-cell Immunodeficiencies Congenital thymic Aplasia (DiGeorge Syndrome): Tetany is present. Fungal and viral infections are common. A transplant of the fetal thymus is needed to correct this deficiency. Chronic Mucocutaneous Candidasis: Selective defect in functioning of T-cells. Patient with this disorder usually have a normal T-cell mediated immunity to microorganisms other than Candida. B-cells function is normal. Disorders affect both genders, and it is inherited. Patient in addition to the above will have other disorders like parathyroid deficiencies. Antifungals are useful. Hyper-IgM syndrome: This disorder is characterized by bacterial infections including pneumonia, meningitis, otitis, among others that start in early childhood. High levels of IgM. Other immunoglobulins are defective. Lymphocytes are normal in numbers. The gene encoding the CD40 ligand on T lymphocytes is faulty. B and T lymphocyte cooperation in the immune response is compromised. The failure to interact with CD40 results in an inability of the B cell to switch from the production of IgM to the other classes of antibodies. Immunoglobulin therapy is recommended. Interleukin-12 receptor deficiency: Mycobacterial infections are frequent due to the lack of the interleukin-12 receptor. Treatment involves selective antimicrobials. T-cell and B-cell Deficiencies Severe combined immunodeficiency disease (SCID): There is a failure of early stem cells to differentiate into T and B lymphocytes. Deficiency of the interleukin-2 receptor is the most prevalent. Other problems are due to defective genes encoding ZAP-70, Janus kinase 3 and the genes involved in the DNA recombination of immune cells receptors: RAG1 and RAG2. Clinically characterized by a variety of infections, including those caused by opportunistic pathogens. Selective antibiotics, antivirals, and antifungals are available after the pathogen identification. Immunosuppressive therapy is not needed after allograft transplantation. Wiskott-Aldrich syndrome: This syndrome is associated with normal T-cell numbers with reduced functions, which get progressively worse. IgM concentrations are reduced, but IgG levels are normal. Both IgA and IgE levels are elevated. These patients have a defective WASP which is involved in actin filament assembly. Immunodeficiency with ataxia-telangiectasia: This is a deficiency of T-cells associated with a lack of coordination of movement (ataxia) and dilation of small blood vessels of the facial area (telangiectasis). T-cells and their functions are diminished to various degrees. B-cell numbers and IgM concentrations are normal to low. IgG is often reduced, and IgA is considerably reduced. There is a high incidence of malignancy, especially leukemias, in these patients. MHC deficiency (Bare leukocyte syndrome): This subjects have have fewer CD4+ or CD8+ T lymphocytes that predispose to these individuals to be prone to recurrent infections. Antibody production is affected and predispose to bacteremia. Complement Deficiencies Hereditary angioedema : This disease has an autosomal dominant genetic pattern. Caused by C1 inhibitor deficiency. Clinically characterized by generalized edema including the one leading to acute suffocation. Therapy with oxymetholone and danazol can be helpful in correcting the defect. Recurrent infections: Frequent infections by extracellular bacteria may be caused by C3 deficiency. C5 deficiency predisposes to viral infections. Patients with deficiency of the membrane attack complex (MAC) are particularly susceptible to bacteremia caused by Neisseria species. Autoimmune diseases This are caused by C2 and C4 deficiencies and mimic systemic lupus erythematosus. Phagocyte Deficiencies Chronic granulomatous disease (CGD): It is mostly an X-linked disorder. It is clinically characterized by a defective NADPH that interferes with the intracellular ability of neutrophils to kill engulfed bacteria species. NAPDH oxidase is required for the generation of peroxidase and superoxides that will kill the organisms. The intracellular survival of the organisms leads to the formation of a granuloma, an organized structure consisting of mononuclear cells. These granulomas can become large enough to obstruct the stomach, esophagus, or bladder. Patients with this disease are very susceptible to opportunistic infection by certain bacteria and fungi especially with Serratia and Burkholderia. Nitroblue tetrazolium (NBT) dye reduction test confirms the diagnosis of CGD and the dichlorofluorescein (DCF) test is also useful. Aggressive therapy with wide spectrum antibiotics and antifungal agents is required. Leukocyte adhesion deficiency syndrome: Characterized by pyogenic infections including pneumonia and otitis. It is an autosomal recessive disease, and the faulty gene encodes for an integrin. There is an impaired adhesion and defective phagocytosis of bacteria. Treatment involves the use of selective antibiotics. Secondary Immunodeficiency Use of Drugs (Steroids) : Administration of steroids has direct effects on immune cell traffic and functions. T cells are more affected than B cells. Cytokine synthesis is inhibited. Nutrient Deficiencies : They are associated with impaired immune system. Affects cell-mediated immunity, antibody production, phagocyte function, complement system and cytokine synthesis. Aggravated by infections. Multiple enzymes with important roles require zinc, iron and other micronutrients. Obesity : It may cause impaired immune responses. There is altered NK function. Cytotoxicity is compromised and the ability of phagocytes to kill microorganisms. Acquired Immune Deficiency Syndrome (AIDS) : Caused by human immunodeficiency virus (HIV), which is a retrovirus transmitted sexually, perinatally or blood products. Immune dysfunction results from the direct effects of HIV and impairment of CD4 T cells. HIV proteins may act as superantigens. There is decreased responses to antigens and mitogens. Interleukin-2 and other cytokines are decreased. Infected cells may be killed by HIV-1 specific CD8+ T cells. In HIV-1 infection neutralizing antibodies appear to be ineffective in controlling viral replication and infection.
- Starch Malabsorption in Infants. [Journal Article]
- JPJ Pediatr Gastroenterol Nutr 2018; 66 Suppl 3:S65-S67
- Based on the developmental physiology of pancreatic amylase production, starch digestion in young infants was anticipated to be compromised whenever compared with that in older infants and toddlers. ...
Based on the developmental physiology of pancreatic amylase production, starch digestion in young infants was anticipated to be compromised whenever compared with that in older infants and toddlers. This appears to be the case, but with great variability among infants to digest starch. Evidence points to the importance of maltase-glucoamylase in young infants and its effect on starch digestion. These observations have critical importance for recommendations regarding the feeding of starch-containing foods to young infants.
- History of Acquired Monosaccharide Intolerance in Houston, Texas. [Journal Article]
- JPJ Pediatr Gastroenterol Nutr 2018; 66 Suppl 3:S50-S51
- It is appropriate that we begin the clinical part of this conference with a discussion of acquired monosaccharide intolerance. This disease is the most severe form of carbohydrate malabsorption and m...
It is appropriate that we begin the clinical part of this conference with a discussion of acquired monosaccharide intolerance. This disease is the most severe form of carbohydrate malabsorption and maldigestion that can afflict an infant. The following article describes the pathophysiology of the disease, as well as its treatment, much of which was based on studies done by the Section of Pediatric Gastroenterology and Nutrition at Baylor College of Medicine. Acquired monosaccharide intolerance has almost disappeared from the United States but remains a problem in the developing world where it is included as part of the diagnosis of environmental enteropathy. Present research on this disease seems to sometimes duplicate what is already known.
- Applying a Low-FODMAP Dietary Intervention to a Female Ultra-Endurance Runner With Irritable Bowel Syndrome During a Multi-Stage Ultra-Marathon. [Journal Article]
- IJInt J Sport Nutr Exerc Metab 2018 May 14; :1-19
- Malabsorption of Fermentable Oligo- Di- and Mono-saccharides and Polyols (FODMAPs) in response to prolonged exercise may increase incidence of upper- and lower-gastrointestinal symptoms (GIS), which ...
Malabsorption of Fermentable Oligo- Di- and Mono-saccharides and Polyols (FODMAPs) in response to prolonged exercise may increase incidence of upper- and lower-gastrointestinal symptoms (GIS), which are known to impair exercise performance. The case-study aimed to explore the impact of a low-FODMAP diet on exercise-associated GIS in a female ultra-endurance runner diagnosed with irritable bowel syndrome (IBS), competing in a six-day 186.7 km mountainous multi-stage ultra-marathon (MSUM). IBS symptom severity score at diagnosis was 410 and following a low-FODMAP diet (3.9 g FODMAPs·day-1) reduced to 70. The diet was applied six-days before (i.e., lead-in diet), and maintained during (5.1 g FODMAPs·day-1) the MSUM. Nutrition intake was analysed through dietary analysis software. A validated 100 mm visual analogue scale quantified GIS incidence and severity. GIS were modest during the MSUM (overall mean ± SD: bloating 27 ± 5 mm and flatulence 23 ± 8 mm), except severe nausea (67 ± 14 mm) experienced throughout. Total daily energy (11.7 ± 1.6 MJ·day-1) intake did not meet estimated energy requirements (range: 13.9-17.9 MJ·day-1). Total daily protein (1.4 ± 0.3 g·kgbody weight (BW)-1·day-1), carbohydrate (9.1 ± 1.3 g·kgBW-1·day-1), fat (1.1 ± 0.2 g·kgBW-1·day-1), and water (78.7 ± 6.4 ml·kgBW-1·day-1) intakes satisfied current consensus guidelines, except for carbohydrates. Carbohydrate intake during running failed to meet recommendations (43 ± 9 g·h-1). The runner successfully implemented a low-FODMAP diet completing the MSUM with minimal GIS. However, suboptimal energy and carbohydrate intake occurred, potentially exacerbated by nausea associated with running at altitude.
- Unusual manifestation of vitamin A deficiency presenting with generalized xerosis without night blindness. [Journal Article]
- CCClin Case Rep 2018; 6(5):878-882
- Vitamin A deficiency from malabsorption syndromes, including bariatric surgery, has become an emerging problem in developed countries. Early detection and prompt treatment lead to rapid and complete ...
Vitamin A deficiency from malabsorption syndromes, including bariatric surgery, has become an emerging problem in developed countries. Early detection and prompt treatment lead to rapid and complete recovery. Nevertheless, it may result in irreversible blindness or death if left untreated. Health care personnel should be aware of this condition.
- A Simple Colorimetric Method to Quantify Total Fecal Oil Using Oil-soluble Dyes in Laboratory Animals and Its Correlation with Liquid Chromatography-Mass Spectrometry Analysis. [Journal Article]
- ASAnal Sci 2018; 34(5):623-626
- We developed a colorimetric method for measuring the amount of oil in mouse stool after co-administering an oil-soluble dye. When the amount of oil in the feces calculated from the amounts of Sudan I...
We developed a colorimetric method for measuring the amount of oil in mouse stool after co-administering an oil-soluble dye. When the amount of oil in the feces calculated from the amounts of Sudan III and Oil Red O was plotted against the amount of oil detected by liquid chromatography-mass spectrometry, the graph was linear, showing a one-to-one correlation between two analyses. This method may be utilized to determine the efficacy of lipase inhibitors, or to assess fat malabsorption in vivo.
- Pathogenesis, diagnosis and treatment of anaemia in immune-mediated gastrointestinal disorders. [Review]
- BJBr J Haematol 2018 May 06
- Immune-mediated disorders affecting the gastrointestinal (GI) tract may compromise GI integrity, interfere with the absorption of nutrients and cause bleeding and inflammation. All these features con...
Immune-mediated disorders affecting the gastrointestinal (GI) tract may compromise GI integrity, interfere with the absorption of nutrients and cause bleeding and inflammation. All these features contribute to the pathogenesis of anaemia, the most prevalent extra-intestinal manifestation of immune-mediated GI disorders. Anaemia is most commonly due to iron deficiency and/or inflammation, but vitamin deficiencies and, more infrequently, autoimmune haemolysis or drug-induced myelosuppression can be involved. Here we address several issues related to the differential diagnosis and treatment of anaemia in immune-mediated GI disorders, giving particular relevance to the problem of iron deficiency anaemia associated with inflammation. It is emphasized how, in most cases, anaemias due to iron or vitamin deficiencies are best treated by parenteral administration of the deficient factor(s), and how the available high dose intravenous (IV) iron formulations can reduce ambulatory and social costs of IV iron supplementation, while improving patient's compliance to treatment. Actual and future treatment possibilities for anaemia of inflammation, involving the use of erythropoiesis stimulating agents, biologicals and hepcidin inhibitors are discussed.
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- Case Report of Wernicke's Encephalopathy After Sleeve Gastrectomy. [Journal Article]
- NCNutr Clin Pract 2017 Dec 14
- CONCLUSIONS: Bariatric surgery is a less common cause of WE but can lead to acute WE due to malabsorption of thiamin. In patients undergoing bariatric surgery, clinicians should be vigilant about the potential for WE to occur. In addition, based on history, WE should be considered in the differential diagnosis for symptoms of acute ischemic stroke.