The main characteristics of sickle cell disease

Blood transfusions for people with sickle cell disease before they undergo surgery Review question We wanted to determine if blood transfusions given to people with sickle cell disease before routine or emergency surgery prevent complications due to sickle cell disease or surgery without causing any severe side effects.

The main characteristics of sickle cell disease

Replacing fluids to treat acute episodes of pain in people with sickle cell disease Review question To assess the best route, quantity and type of fluid replacement for people with sickle cell disease with acute painful crises.

Background Sickle cell disease is a common genetic disorder characterised by periodic episodes of pain which usually happen again and again throughout life. These episodes occur when sickled cells obstruct blood vessels.

The degree of pain may range from a mild discomfort to a The main characteristics of sickle cell disease disabling pain where the person needs treatment in hospital.

A drop in body fluid levels promotes and sustains the sickling process. Routine treatment includes the use of drugs to relieve pain and the maintenance of adequate fluid levels.

Fluid levels will fall if insufficient fluids are taken in compared to the amount of fluid lost. The kidneys of people with sickle cell disease do not concentrate urine properly, which results in poor control of bodily fluids. Additional fluids are given to try and slow or stop the sickling process which should reduce the amount and duration of pain.

Fluids may be given in many ways, but orally or intravenously are most common. Fluids should be given with care so as to prevent fluid overload which may in turn cause adverse events such as heart failure or fluid building up in the lungs. We looked for randomised controlled trials to show the best way to replace fluids, which type of fluids and how much fluid to replace to treat acute episodes of pain in people with sickle cell disease.

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This is an update of a previously published Cochrane Review. Search date The evidence is current to: Trial characteristics We did not find any randomized controlled trials trials where the people taking part are put into different treatment groups completely at random which we could include in the review.

Key results We conclude that there is a need for large multi-centre trials to assess fluid replacement therapy for people with sickle cell disease with acute painful crises. Treating vaso-occlusive crises is complex and requires multiple interventions.

Extra fluids, generally oral or intravenousare routinely administered during acute painful episodes to people with sickle cell disease regardless of the individual's state of hydration.

Reports of their use during these acute painful episodes do not state the efficacy of any single route, type or quantity of fluid compared to another. However, there are no randomised controlled trials that have assessed the safety and efficacy of different routes, types or quantities of fluid.

This systematic review identifies the need for a multicentre randomised controlled trial assessing the efficacy and possible adverse effects of different routes, types and quantities of fluid administered to people with sickle cell disease during acute painful episodes.

NIH Halts SWiTCH Study

Read the full abstract Treating vaso-occlusive painful crises in people with sickle cell disease is complex and requires multiple interventions. Extra fluids are routinely given as adjunct treatment, regardless of the individual's state of hydration with the aim of slowing or stopping the sickling process and thereby alleviating pain.

To determine the optimal route, quantity and type of fluid replacement for people with sickle cell disease with acute painful crises.

The main characteristics of sickle cell disease

We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register which comprises of references identified from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings.

Date of most recent search of the Group's Haemoglobinopathies Trials Register: Randomised and quasi-randomised controlled trials that compared the administration of supplemental fluids adjunctive to analgesics by any route in people with any type of sickle cell disease during an acute painful episode, under medical supervision inpatient, day care or community.

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Data collection and analysis: No relevant trials have yet been identified. Sixteen trials were identified by the searches, all of which were not eligible for inclusion in the review.

Sickle cell disease is one of the commonest severe monogenic disorders in the world, due to the inheritance of two abnormal haemoglobin (beta globin) genes. Sickle cell disease can cause severe pain, significant end-organ damage, pulmonary complications, and premature death. Surgical interventions are more common in people with sickle cell disease, and occur at much younger ages . Order online or call APS at Publication Price: $ (APS member price $) Discounts are available for quantity purchases of ten or more. Order online or call American Pain Society at to place your order.. This guideline is the first comprehensive evidence-based guideline to address treatment of the pain of sickle-cell disease. A abnormal result. A possible result of a screening test. An abnormal result does not determine a diagnosis, and means additional testing is needed to see if the individual has a condition. Also referred to as positive result. acquired mutations. A change within a sequence of DNA caused by environment factors (sun, radiation, or chemicals), aging, or chance.

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3. J Pediatr Hematol Oncol. Dec doi: /MPH [Epub ahead of print] A Standardized Clinical Pathway to Decrease Hospital Admissions Among Febrile Children With Sickle Cell Disease.

The main characteristics of sickle cell disease

An autosome is a chromosome that is not an allosome (a sex chromosome). The members of an autosome pair in a diploid cell have the same morphology, unlike those in allosome pairs which may have different structures.

The DNA in autosomes is collectively known as atDNA or auDNA.. For example, humans have a diploid genome that usually contains 22 pairs of autosomes and one . Sickle cell disease (SCD) is a group of blood disorders typically inherited from a person's parents. The most common type is known as sickle cell anaemia (SCA).

It results in an abnormality in the oxygen-carrying protein haemoglobin found in red blood cells. This leads to a rigid, sickle-like shape under certain circumstances. Problems in sickle cell disease typically begin around 5 to 6. A abnormal result.

A possible result of a screening test. An abnormal result does not determine a diagnosis, and means additional testing is needed to see if the individual has a condition.

Also referred to as positive result. acquired mutations. A change within a sequence of DNA caused by environment factors (sun, radiation, or chemicals), aging, or chance.

About 72, years ago, the effects of a major volcanic eruption (Toba) with global consequences killed off many humans. By some estimates, as few as 2, humans survived the disaster --in Africa.

Sickle cell disease - Wikipedia