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Ehlers-Danlos Syndrome

Surgery in patients with EDS appears to be associated with a high risk of complications, which is why options should be carefully considered. Furthermore, optimal therapy for these patients includes the awareness that EDS is a disease involving fragility and bleeding in many organs. A nonsurgical approach can be the best choice for these patients, depending on the condition. The correct answer is false.

Vascular EDS (vEDS) is the most serious form of EDS. Like the other rare types, in order to inherit it you need to inherit one faulty gene from EACH of your parents.

Dental, oral and voice problemsVarious types of Ehlers-Danlos syndromes can affect the teeth, gums and other oral structures. Hypermobility of the temporomandibular (jaw) joint can result in dislocations, facial and head pain.

Elevating the relative head of your bed 4 to 6 inches using blocks of wood may help. If symptoms continue, see your doctor or a Gastroenterologist for evaluation and an upper endoscopy exam. Your doctor might suggest you take over-the-counter antacids or may prescribe stronger medications. Call your doctor if you are bleeding, feel like you are choking, have trouble-swallowing, or experience sudden weight loss. The good news is most people who have GERD do not need surgery.

C, Skin abnormalities such as piezogenic pedal papules, herniations of fat through the dermis that are seen in patients with hereditary disorders of connective tissue commonly. D, Cardiac abnormalities such as a left ventricular diverticulum. All patients were examined for joint and skin abnormalities prior to genotyping. Minor and Major criteria used in the diagnosis of classical and hypermobile EDS were evaluated (9, 10).

Both MS and hEDS groups showed a higher prevalence of abdominal symptoms compared to the control group; however, the hEDS group not only showed a higher prevalence but more frequent and severe symptoms meeting Rome IV criteria for diagnosis of FGIDs. Nearly half of the criteria were met by the hEDS patients for more than one FGID. The hEDS group also scored lower on quality of life (QOL) scores in comparison to either of the other groups with a mean score of 48.6 as compared to 54.2 in the Marfan group and 78.6 in the control group. Conclusion.

Because the gene(s) and pathogenic variant(s) responsible for hEDS have not been identified, prenatal testing is not possible. The diagnosis of hEDS is based on clinical evaluation and family history entirely. The gene(s) in which mutation causes hEDS are unknown and unmapped.

The autonomic nervous system regulates all body processes that occur automatically, such as heart rate, blood pressure, breathing, and digestion. To compensate for stretchy blood vessels and increased venous pooling (too much blood collecting in over-stretched veins) most people with hypermobility appear to make extra adrenaline, which may account for the high-energy, always-on-the- go lifestyles of many hypermobile people. Unfortunately, if you get tired too, your body responds by making more adrenaline, so you keep going, not realizing how tired you are really.

Molecular Pathogenesis

Joint hypermobility is a very common condition, and many people with loose joints shall have no related medical problems at all. Most people have just a few, such as achy joints and cold hands and feet, and they should not be concerned that they will go on to develop other complications overly. On the other hand, I have described some of the more common conditions associated with hypermobility, because those who do have many related problems may be relieved to understand that there is a unifying explanation for so many of the unusual things they have noticed about their bodies. Beyond the psychological relief of understanding their symptoms, with proper treatment most people with JHS can get significant relief from their physical symptoms, too.

GERD is the most common gastrointestinal disease in the world affecting over 10-20% of the adult population. GERD is often perceived as a Western disease and very little literature available about this disease in the Asian population. With growing obesity and westernization in Asia, the prevalence of GERD is rapidly increasing. With growing morbidity and health care cost there is a significant need for understanding the disease from the Asian perspective. This book would fill the gap in the knowledge on GERD among Asians and will highlight the difference in terms of epidemiology, diagnosis and management of GERD from the Western perspective.

Sleep studies done in a sleep lab are very helpful in demonstrating the severity and nature of sleep problems, and in ruling out other sleep problems like sleep apnea and periodic limb movements of sleep, which can coexist with hypermobility-related sleep problems. Recently home sleep monitors that can measure sleep stages (shallow, deep, REM) and arousals have become available.

Affected individuals are often diagnosed with chronic fatigue syndrome, fibromyalgia, depression, hypochondriasis, and/or malingering prior to recognition of joint establishment and laxity of the correct underlying diagnosis. Tendinitis and bursitis may occur [Rombaut et al 2010b, Rombaut et al 2011a, Rombaut et al 2011b], especially greater trochanteric bursitis in those with iliotibial band syndrome. EDS does not directly cause inflammation, and these problems are likely secondary to joint instability. Temporomandibular dysfunction (“TMJ syndrome”) is relatively common [Hagberg et al 2004, De Coster et al 2005], and can be thought of as a specific example of joint osteoarthritis and degeneration. Iliotibial band syndrome or “snapping hip” is a common symptom, often perceived by the affected individual as hip joint instability [Branson et al 2011].

In many cases, the joints become stiffer with age, although joint hypermobility and its associated symptoms can continue into adult life. JHS is widely thought to be a feature of an underlying condition affecting connective tissue called Ehlers-Danlos syndrome (EDS). Joint hypermobility is often hereditary (runs in families). One of the main causes is thought to be genetically determined changes to a type of protein called collagen.

This raises the possibility that abnormalities of the extracellular matrix might contribute to the development of autoimmunity in the presence of other environmental or genetic influences. Evidence suggests a link between connective tissue disorders such as the Ehlers-Danlos syndromes (EDS) and digestive system (gastrointestinal, GI) symptoms.

eds and acid reflux

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