Healthy blue man: congenital methemoglobinemia

Congenital methemoglobinemia, though often discussed in medical teachings is rarely encountered in clinical practice as the condition is asymptomatic. Here we present such a case and discuss in detail the clinical presentation of both congenital and acquired methemoglobinemia. We also outlined the management of the conditions. One should suspect methemoglobinemia when cyanosis is not being corrected by supplementing oxygen and and when the oxygen saturation is low by pulse oximetry and normal by arterial blood gas analysis. Treatment modalities for congenital methemoglobinemia is of cosmetic purpose, but timely intervention in acquired methemoglobinemia could be lifesaving. Methylene blue, Ascorbic acid and Riboflavin are drugs of choice

A 48–year–old female presented with severe neck pain

This article has no abstract. The first 100 words appear below: A 48-year-old married female got admitted on 8th September 2017 into the Boarder Guard Hospital, Pilkhana, Dhaka, Bangladesh with the complaints of severe neck pain associated with low grade backache for the last four years. This patient is hailing from Soliabazar, Jashore, Southwestern region of Bangladesh where she was treated with local doctors by cervical collar and analgesics for her neck and back pain. As the pain did not subside, so she was brought by her brother-in-law who is a BGB soldier to Boarder Guard hospital for better management

A 48–year–old female presented with severe neck pain

This article has no abstract. The first 100 words appear below: A 48-year-old married female got admitted on 8th September 2017 into the Boarder Guard Hospital, Pilkhana, Dhaka, Bangladesh with the complaints of severe neck pain associated with low grade backache for the last four years. This patient is hailing from Soliabazar, Jashore, Southwestern region of Bangladesh where she was treated with local doctors by cervical collar and analgesics for her neck and back pain. As the pain did not subside, so she was brought by her brother-in-law who is a BGB soldier to Boarder Guard hospital for better management

Nitrate/nitrite toxicity

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Recommendations for diagnosis and treatment of methemoglobinemia

Methemoglobinemia is a rare disorder associated with oxidization of divalent ferro-iron of hemoglobin (Hb) to ferri-iron of methemoglobin (MetHb). Methemoglobinemia can result from either inherited or acquired processes. Acquired forms are the most common, mainly due to the exposure to substances that cause oxidation of the Hb both directly or indirectly. Inherited forms are due either to autosomal recessive variants in the CYB5R3 gene or to autosomal dominant variants in the globin genes, collectively known as HbM disease. Our recommendations are based on a systematic literature search. A series of questions regarding the key signs and symptoms, the methods for diagnosis, the clinical management in neonatal/childhood/adulthood period, and the therapeutic approach of methemoglobinemia were formulated and the relative recommendations were produced. An agreement was obtained using a Delphi-like approach and the experts panel reached a final consensus >75% of agreement for all the questions. © 2021 The Authors. American Journal of Hematology published by Wiley Periodicals LLC

Recommendations for diagnosis and treatment of methemoglobinemia

Methemoglobinemia is a rare disorder associated with oxidization of divalent ferro-iron of hemoglobin (Hb) to ferri-iron of methemoglobin (MetHb). Methemoglobinemia can result from either inherited or acquired processes. Acquired forms are the most common, mainly due to the exposure to substances that cause oxidation of the Hb both directly or indirectly. Inherited forms are due either to autosomal recessive variants in the CYB5R3 gene or to autosomal dominant variants in the globin genes, collectively known as HbM disease. Our recommendations are based on a systematic literature search. A series of questions regarding the key signs and symptoms, the methods for diagnosis, the clinical management in neonatal/childhood/adulthood period, and the therapeutic approach of methemoglobinemia were formulated and the relative recommendations were produced. An agreement was obtained using a Delphi-like approach and the experts panel reached a final consensus >75% of agreement for all the questions

Recommendations for diagnosis and treatment of methemoglobinemia

Methemoglobinemia is a rare disorder associated with oxidization of divalent ferro-iron of hemoglobin (Hb) to ferri-iron of methemoglobin (MetHb). Methemoglobinemia can result from either inherited or acquired processes. Acquired forms are the most common, mainly due to the exposure to substances that cause oxidation of the Hb both directly or indirectly. Inherited forms are due either to autosomal recessive variants in the CYB5R3 gene or to autosomal dominant variants in the globin genes, collectively known as HbM disease. Our recommendations are based on a systematic literature search. A series of questions regarding the key signs and symptoms, the methods for diagnosis, the clinical management in neonatal/childhood/adulthood period, and the therapeutic approach of methemoglobinemia were formulated and the relative recommendations were produced. An agreement was obtained using a Delphi-like approach and the experts panel reached a final consensus >75% of agreement for all the questions.Genetics of disease, diagnosis and treatmen

Altered erythropoiesis and decreased number of erythrocytes in children with neuroblastoma

Neuroblastoma (NB) is a pediatric tumor presenting at diagnosis either as localized or metastatic disease, which mainly involves the bone marrow (BM). The physical occupancy of BM space by metastatic NB cells has been held responsible for impairment of BM function. Here, we investigated whether localized or metastatic NB may alter hematopoietic lineages' maturation and release of mature cells in the periphery, through gene expression profiling, analysis of BM smears, cell blood count and flow cytometry analysis. Gene ontology and disease-associated analysis of the genes significantly under-expressed in BM resident cells from children with localized and metastatic NB, as compared to healthy children, indicated anemia, blood group antigens, and heme and porphyrin biosynthesis as major functional annotation clusters. Accordingly, in children with NB there was a selective impairment of erythrocyte maturation at the ortho-chromic stage that resulted in reduced erythrocyte count in the periphery, regardless of the presence of metastatic cells in the BM. By considering all NB patients, low erythrocyte count at diagnosis associated with worse survival. Moreover, in the subset of metastatic patients, low erythrocyte count, hemoglobin and hematocrit and high red cell distribution width at follow-up also associated with worse outcome. These observations provide an alternative model to the tenet that infiltrating cells inhibit BM functions due to physical occupancy of space and may open a new area of research in NB to understand the mechanism(s) responsible for such selective impairment

Drinking Water Quality and Human Health

The quality of drinking water is paramount for public health. Despite important improvements in the last decades, access to safe drinking water is not universal. The World Health Organization estimates that almost 10% of the population in the world do not have access to improved drinking water sources. Among other diseases, waterborne infections cause diarrhea, which kills nearly one million people every year, mostly children under 5 years of age. On the other hand, chemical pollution is a concern in high-income countries and an increasing problem in low- and middle-income countries. Exposure to chemicals in drinking water may lead to a range of chronic non-communicable diseases (e.g., cancer, cardiovascular disease), adverse reproductive outcomes, and effects on children’s health (e.g., neurodevelopment), among other health effects. Although drinking water quality is regulated and monitored in many countries, increasing knowledge leads to the need for reviewing standards and guidelines on a nearly permanent basis, both for regulated and newly identified contaminants. Drinking water standards are mostly based on animal toxicity data, and more robust epidemiologic studies with accurate exposure assessment are needed. The current risk assessment paradigm dealing mostly with one-by-one chemicals dismisses the potential synergisms or interactions from exposures to mixtures of contaminants, particularly at the low-exposure range. Thus, evidence is needed on exposure and health effects of mixtures of contaminants in drinking water. Finally, water stress and water quality problems are expected to increase in the coming years due to climate change and increasing water demand by population growth, and new evidence is needed to design appropriate adaptation policies.This Special Issue of International Journal of Environmental Research and Public Health (IJERPH) focuses on the current state of knowledge on the links between drinking water quality and human health

Isolation and characterization of membrane vesicles secreted by human renal cells

Most cells release membrane vesicles for various purposes including, but not limited to, intercellular communication and disposal of membrane and soluble proteins. These vesicles are secreted into urine coming from the cells lining the urinary tract and bladder epithelium. These vesicles are a promising source of biomarkers for various cardiovascular and renal diseases. This thesis pursues twofold objectives, one being the development and improvement of an isolation method for urinary membrane vesicles and the second being proteomic characterization of the content of these vesicles. These objectives are important to realise the clinical potential of these vesicles. An alternative method for removal of contaminant high-abundant proteins was developed which preserves the activity of vesicular proteins. Moreover, lipid-affinity and lectin-affinity-based novel methods to enrich membrane vesicles from minimally processed urine were evaluated and developed. More than 600 proteins were identified in urinary membrane vesicles using shotgun proteomic analysis. Post-translational modification (PTM) proteomics was carried out to identify the PTM status of vesicular proteins. Many different PTMs like glycosylation, ubiquitination and palmitoylation were assessed. Surface glycan profiles of these vesicles were elucidated using fluorophore-linked lectin assay (FLLA) employing 18 different lectins. Lectin blotting, lectin-affinity chromatography using multiple lectins and hydrazide chemistry based enrichment of glycoproteins were carried out. As a result, 108 glycoproteins were identified. Immuno-affinity chromatography was used to enrich and identify ubiquitin-conjugated proteins present in urinary membrane vesicles. A number of potential palmitoylated proteins were identified as well. Computational prediction and validation methods were applied to these protein lists. In conclusion, novel methods to isolate urinary membrane vesicles were developed. In addition, a thorough proteomic characterisation of contents of urinary membrane vesicles was achieved. This work will serve as platform for further characterization of urinary membrane vesicles