close
TEACHING TOPIC
1. Case: Renal Failure, Stiff Joints, and Skin Fibrosis
A 46-year-old woman with end-stage renal disease developed stiffness of her joints and skin. She had been well until 7 years before when an episode of group A streptococcal pneumonia complicated by septic shock left her with chronic kidney disease and peripheral neuropathy. The patient also has respiratory distress and congestive heart failure. What is the differential diagnosis?
Clinical Pearls
Nephrogenic Systemic Fibrosis
Nephrogenic systemic fibrosis has been described only in patients with kidney disease, typically among those receiving dialysis treatments but also in some who have undergone successful renal transplantation or who have had an episode of acute renal failure. This condition is manifested by progressive, painful tightening of the skin, with tethering to underlying fascia, usually beginning on the hands and feet and extending proximally; it is associated with woody induration, brawny hyperpigmentation, and peau d'orange changes of the affected skin. Extracutaneous fibrosis involving the heart, lungs, diaphragm, skeletal muscle, liver, genitourinary tract, and central nervous system have been reported.
Fibrosing Disorders
There are many causes of fibrosis. Skin tightening and joint stiffness are features of various systemic fibrosing disorders. Localized idiopathic cutaneous fibrosing disorders include morphea (localized scleroderma), linear scleroderma, scleredema diabeticorum, and lipodermatosclerosis. Systemic idiopathic fibrosing disorders include diffuse systemic sclerosis, scleromyxedema, and eosinophilic fasciitis. See table below.
TEACHING TOPIC
2. Spinal Stenosis
A 72-year-old woman with hypertension presents with a 4-month history of lower back discomfort that radiates to both buttocks and lateral thighs. Previously, she had walked 2 miles a day; now she has difficulty walking 2 blocks and standing up for more than 15 minutes. Her physical examination is notable only for a slightly stooped posture and a reduction of vibratory sensation in both great toes. How should she be evaluated and treated?
Clinical Pearls
Spinal Stenosis: Symptoms
The most common symptom associated with lumbar spinal stenosis is neurogenic claudication — discomfort that radiates beyond the spinal area into the buttocks and frequently into the thigh and lower leg; it is exacerbated by lumbar extension and improves with lumbar flexion. Patients with symptomatic stenosis are generally comfortable when sitting and have worsening pain with prolonged walking.
Best Radiographic Tests for Spinal Stenosis
Either magnetic resonance imaging (MRI) or computed tomography (CT) may confirm the presence of spinal stenosis, since both modalities can detect the cardinal features of stenosis — reductions in the cross-sectional area of the central canal and neural foramina due to a combination of disk protrusion, redundancy and hypertrophy of facet joints, with accompanying osteophytes. Bony findings such as facet arthropathy can be seen more clearly with CT scans, whereas soft-tissue lesions involving ligaments and disks are better detected with MRI scans. Because CT myelography is invasive and requires intrathecal contrast material, MRI is generally preferred. CT myelography can be used for patients who are not candidates for MRI (e.g., because of clostrophobia or metallic implants) and in rare, specific clinical situations.
TEACHING TOPIC
3. Hepatitis E
Hepatitis E virus (HEV) is considered responsible for acute hepatitis but has not been thought to cause progression to chronic hepatitis. The authors of this paper identified 14 transplant patients (three who received liver transplants, nine who received kidney transplants, and two who received kidney and pancreas transplants) who all developed acute HEV infection following transplant. Chronic hepatitis developed in eight of these patients, confirmed by persistently elevated aminotransferase levels, serum HEV RNA, and histologic features of chronic hepatitis.
“We have demonstrated that HEV infection can evolve to chronic hepatitis, at least in organ-transplant recipients.”
Clinical Pearl
HEV Infection
Hepatitis E virus (HEV) infection is transmitted by the fecal–oral route and may be a zoonosis in industrialized countries. Acute hepatitis caused by HEV is endemic in developing countries and appears to be an emerging disease in industrialized countries. Seroprevalence studies have reported anti-HEV IgG antibodies in 6 to 16% of renal-transplant recipients. HEV infection can cause elevated liver enzyme levels and the virus can be detected in the serum or stool. In a recent study by Shrestha and colleagues (NEJM, 2007), an HEV recombinant protein vaccine was found to be safe and effective in preventing HEV infection.
1. Case: Renal Failure, Stiff Joints, and Skin Fibrosis
A 46-year-old woman with end-stage renal disease developed stiffness of her joints and skin. She had been well until 7 years before when an episode of group A streptococcal pneumonia complicated by septic shock left her with chronic kidney disease and peripheral neuropathy. The patient also has respiratory distress and congestive heart failure. What is the differential diagnosis?
Clinical Pearls
Nephrogenic Systemic FibrosisNephrogenic systemic fibrosis has been described only in patients with kidney disease, typically among those receiving dialysis treatments but also in some who have undergone successful renal transplantation or who have had an episode of acute renal failure. This condition is manifested by progressive, painful tightening of the skin, with tethering to underlying fascia, usually beginning on the hands and feet and extending proximally; it is associated with woody induration, brawny hyperpigmentation, and peau d'orange changes of the affected skin. Extracutaneous fibrosis involving the heart, lungs, diaphragm, skeletal muscle, liver, genitourinary tract, and central nervous system have been reported.
Fibrosing DisordersThere are many causes of fibrosis. Skin tightening and joint stiffness are features of various systemic fibrosing disorders. Localized idiopathic cutaneous fibrosing disorders include morphea (localized scleroderma), linear scleroderma, scleredema diabeticorum, and lipodermatosclerosis. Systemic idiopathic fibrosing disorders include diffuse systemic sclerosis, scleromyxedema, and eosinophilic fasciitis. See table below.
TEACHING TOPIC
2. Spinal Stenosis
A 72-year-old woman with hypertension presents with a 4-month history of lower back discomfort that radiates to both buttocks and lateral thighs. Previously, she had walked 2 miles a day; now she has difficulty walking 2 blocks and standing up for more than 15 minutes. Her physical examination is notable only for a slightly stooped posture and a reduction of vibratory sensation in both great toes. How should she be evaluated and treated?
Clinical Pearls
Spinal Stenosis: SymptomsThe most common symptom associated with lumbar spinal stenosis is neurogenic claudication — discomfort that radiates beyond the spinal area into the buttocks and frequently into the thigh and lower leg; it is exacerbated by lumbar extension and improves with lumbar flexion. Patients with symptomatic stenosis are generally comfortable when sitting and have worsening pain with prolonged walking.
Best Radiographic Tests for Spinal StenosisEither magnetic resonance imaging (MRI) or computed tomography (CT) may confirm the presence of spinal stenosis, since both modalities can detect the cardinal features of stenosis — reductions in the cross-sectional area of the central canal and neural foramina due to a combination of disk protrusion, redundancy and hypertrophy of facet joints, with accompanying osteophytes. Bony findings such as facet arthropathy can be seen more clearly with CT scans, whereas soft-tissue lesions involving ligaments and disks are better detected with MRI scans. Because CT myelography is invasive and requires intrathecal contrast material, MRI is generally preferred. CT myelography can be used for patients who are not candidates for MRI (e.g., because of clostrophobia or metallic implants) and in rare, specific clinical situations.
TEACHING TOPIC
3. Hepatitis E
Hepatitis E virus (HEV) is considered responsible for acute hepatitis but has not been thought to cause progression to chronic hepatitis. The authors of this paper identified 14 transplant patients (three who received liver transplants, nine who received kidney transplants, and two who received kidney and pancreas transplants) who all developed acute HEV infection following transplant. Chronic hepatitis developed in eight of these patients, confirmed by persistently elevated aminotransferase levels, serum HEV RNA, and histologic features of chronic hepatitis.
“We have demonstrated that HEV infection can evolve to chronic hepatitis, at least in organ-transplant recipients.”
Clinical Pearl
HEV InfectionHepatitis E virus (HEV) infection is transmitted by the fecal–oral route and may be a zoonosis in industrialized countries. Acute hepatitis caused by HEV is endemic in developing countries and appears to be an emerging disease in industrialized countries. Seroprevalence studies have reported anti-HEV IgG antibodies in 6 to 16% of renal-transplant recipients. HEV infection can cause elevated liver enzyme levels and the virus can be detected in the serum or stool. In a recent study by Shrestha and colleagues (NEJM, 2007), an HEV recombinant protein vaccine was found to be safe and effective in preventing HEV infection.
全站熱搜
留言列表
