1/8. Aneurysms and hypermobility in a 45-year-old woman.EDS type IV presents a diagnostic and therapeutic challenge to the primary care physician, surgeon, and rheumatologist. In patients for whom the diagnosis is known, avoidance of trauma, contact sports, or strenuous activities, joint bracing and protection, and counseling on contraception are helpful preventive strategies. In patients presenting with vascular, gastrointestinal, or obstetric complications, a history of hypermobility and skin fragility (easy bruising, abnormal scarring, poor wound healing) should lead to a suspicion of this diagnosis, and to caution in the use of certain invasive diagnostic and operative techniques. Efforts should be made to examine family members. Most importantly, when caring for such patients, the acute onset of headaches, chest pain, shortness of breath, and abdominal pain should arouse suspicion of a potentially catastrophic vascular or visceral event.- - - - - - - - - - ranking = 1keywords = physician (Clic here for more details about this article) |
2/8. Clinical considerations in the chiropractic management of the patient with marfan syndrome.OBJECTIVE: To describe the chiropractic management of a patient with whiplash-associated disorder and a covert, concomitant dissecting aneurysm of the thoracic aorta caused by marfan syndrome or a related variant. CLINICAL FEATURES: A 25-year-old man was referred by his family physician for chiropractic assessment and treatment of neck injuries received in a motor vehicle accident. After history, physical examination, and plain film radiographic investigation, a diagnosis of whiplash-associated disorder grade I was generated. INTERVENTION AND OUTCOME: The whiplash-associated disorder grade I was treated conservatively. Therapeutic management involved soft-tissue therapy to the suspensory and paraspinal musculature of the upper back and neck. Rotary, manual-style manipulative therapy of the cervical and compressive manipulative therapy of the thoracic spinal column were implemented to maintain range of motion and decrease pain. The patient achieved full recovery within a 3-week treatment period and was discharged from care. One week after discharge, he underwent a routine evaluation by his family physician, where an aortic murmur was identified. Diagnostic ultrasound revealed a dissecting aneurysm measuring 78 mm at the aortic root. Immediate surgical correction was initiated with a polyethylene terephthalate fiber graft. The pathologic report indicated that aortic features were consistent with an old (healed) aortic dissection. There was no evidence of acute dissection. Six month follow-up revealed that surgical repair was successful in arresting further aortic dissection. CONCLUSION: The patient had an old aortic dissection that pre-dated the chiropractic treatment (which included manipulative therapy) for the whiplash-associated disorder. Manipulative therapy, long considered an absolute contraindication for abdominal and aortic aneurysms, did not provoke the progression of the aortic dissection or other negative sequelae. The cause, histology, clinical features, and management considerations in the treatment of this patient's condition(s) are discussed.- - - - - - - - - - ranking = 2keywords = physician (Clic here for more details about this article) |
3/8. Successful reversal of deleterious coagulopathy by recombinant factor viia.Effective treatment of severe or uncontrolled bleeding is a challenge for physicians in the operating room and intensive care unit. However, even aggressive conventional therapy may ultimately fail in some patients. Administration of recombinant activated factor VII (rFVIIa) may be the only remaining therapeutic option to stop life-threatening coagulopathic bleeding. We here describe the clinical course of 5 patients exhibiting severe continuous bleeding that could not be stopped by surgical intervention and appropriate hemostatic management but resolved after a mean dose of 90 microg/kg of rFVIIa (range, 90-120 microg/kg). Four of the five patients recovered completely, and one patient died after developing sepsis in multiorgan failure. In all patients, bleeding from wound surfaces stopped within minutes of the administration of rFVIIa. Coagulation measurements improved, and transfusion requirements declined considerably. No adverse effects associated with rFVIIa were observed.- - - - - - - - - - ranking = 1keywords = physician (Clic here for more details about this article) |
4/8. marfan syndrome affecting four generations of a family without ocular involvement.Thirty eight relatives of a patient with marfan syndrome were screened for the presence of this disorder. marfan syndrome was newly diagnosed in living members of 4 generations in this family without evidence of ocular abnormality in any. After screening, 10 relatives were newly diagnosed as having definite, and 5 relatives as having possible, marfan syndrome. family screening has drawbacks as well as benefits for the patients. The main benefit is the identification and treatment of previously undiagnosed patients at risk of cardiac complications which are the major cause of mortality. The drawbacks include employment problems created for patients with marfan syndrome as a direct consequence of our screening programme and the anxiety induced in previously asymptomatic family members who did not realize that they could be at risk. Also, the 4 adult patients with possible marfan syndrome found it difficult to accept that a definite diagnosis could not be reached after they had been invited to attend a screening programme for a serious genetic disorder. This report illustrates the importance of screening all the relatives of a patient with marfan syndrome to identify previously undiagnosed cases. However, before screening a family, the physician should be aware that a clear diagnosis may not be reached in all patients, and financial, psychological or social problems may arise as a result of the screening programme.- - - - - - - - - - ranking = 1keywords = physician (Clic here for more details about this article) |
5/8. marfan syndrome in the parturient.Early recognition of the marfan syndrome and knowledge of its potentially lethal complications facilitates successful treatment of these individuals. It is through a joint effort by many specialist physicians such as the obstetrician, cardiologist, and anesthesiologist that these patients can be managed safely through pregnancy, labor, and delivery.- - - - - - - - - - ranking = 1keywords = physician (Clic here for more details about this article) |
6/8. The marfan syndrome. An underdiagnosed killer.The marfan syndrome is an inherited connective-tissue disorder. The sequela of the syndrome is premature death that is most often due to cardiac complications and that can be prevented with early diagnosis and aggressive management. Although research has identified a gene responsible for the myriad of phenotypic presentations, the diagnosis of marfan syndrome remains clinical. The family physician is well positioned, with consultation from an experienced cardiac surgeon, to orchestrate the diagnosis and management of the marfan syndrome.- - - - - - - - - - ranking = 1keywords = physician (Clic here for more details about this article) |
7/8. Dolichonychia in a patient with the marfan syndrome.Dolichonychia is a descriptive term for elongated, slender nails. Although published observations of this finding are uncommon, dolichonychia has previously been seen in patients with connective tissue disorders, with hypohidrotic ectoderma dysplasia, or with abnormal hormonal states. Dolichonychia is a clinical feature which may be observed in patients with the marfan syndrome. A woman with the marfan syndrome and dolichonychia is described. Measurements of the patient's arm span, height, lower segment, hand length, and middle finger length were performed. In addition, the length-to-width ratio of each of her finger nails was determined. The mean of finger nail length-to-width quotients in the patient with the marfan syndrome was 1.54. In conclusion, the detection of dolichonychia should prompt the physician to rule out associated systemic conditions: after eunuchoidism, hypohidrotic ectodermal dysplasia, and hypopituitarism have been excluded, an evaluation for ehlers-danlos syndrome and the marfan syndrome should be considered.- - - - - - - - - - ranking = 1keywords = physician (Clic here for more details about this article) |
8/8. Pulmonary dysfunction secondary to mandibular retrognathia in Marfan's syndrome.Referrals to pulmonary physicians for polysomnography to evaluate snoring or the possibility of sleep apnea syndrome often evolve into a multidisciplinary clinical problem. We present a young woman with two congenital abnormalities (Marfan's syndrome and retrognathism) which both may affect her decreased exercise tolerance, daytime hypersomnolence, and fatigue. polysomnography and pulmonary exercise testing documented improvement in her upper airway diameter and exercise capacity. Her improved sleep pattern and her understanding of her exercise performance limitations afforded her a new outlook on life.- - - - - - - - - - ranking = 1keywords = physician (Clic here for more details about this article) |