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In additional, at least приведу ссылку doors have been Trends in age-adjusted incidence, primary surgery, sentinel lymph node biopsy SLNBand axillary lymph node dissection were examined. Coronary artery aneurysms CAAsespecially multiple, are a rare cardiac pathology and an unusual cause of cardiac chest pain.
 
 

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Federal government websites often end in. Before sharing sensitive information, make sure you’re on a federal government site. The site is secure. NCBI Bookshelf. Taimur Saleem ; Donald T. Authors Taimur Saleem 1 ; Donald T. Baril 2. Paget Schroetter syndrome PSS is effort-induced thrombosis of the axillary and subclavian veins associated with compression of the subclavian vein at the thoracic outlet.

It is the venous variant of thoracic outlet syndrome TOS , the syndrome of symptoms associated with compression of the subclavian vein, subclavian artery, or brachial plexus as they pass through the thoracic outlet. This activity reviews the evaluation and management of Paget Schroetter syndrome and highlights the role of the interprofessional team in the care of patients with this condition.

Objectives: Describe the pathophysiology of Paget Schroetter syndrome. Review the evaluation of a patient with Paget Schroetter syndrome. Summarize the treatment options for a patient with Paget Schroetter syndrome. Explain modalities to improve care coordination among interprofessional team members in order to improve outcomes for patients affected by Paget Schroetter syndrome.

Access free multiple choice questions on this topic. The description of this entity dates back to when Sir James Paget reported a case of spontaneous thrombosis of the subclavian vein in a patient. In , von Schroetter hypothesized that the condition was a consequence of injury to the vein from repetitive musculoskeletal motion. The subclavian vein travels in the proximity of the clavicle, first rib, anterior scalene, and subclavius muscles. PSS is related to the compression and subsequent thrombosis of the subclavian vein due to these structures.

PSS is more commonly seen in younger patients in their 20s and 30s with a male to female ratio of Often these patients will not have any other significant medical comorbidities. The incidence ranges from 1 to 2 per , individuals per year in various studies.

It is more commonly encountered on the right side. Often affected individuals with PSS will be athletes, specifically, those engaged in the repetitive over-the-head motion of their arms, including baseball or softball pitchers, swimmers, rowers, or weightlifters. Repetitive strain from vigorous physical activity and compression of the subclavian vein from adjacent anatomic structures leads to venous injury and subsequent thrombosis. This impedes the venous return from the affected extremity leading to edema.

Over time, the body forms collaterals to bypass the venous obstruction. In the chronic phase, the vein becomes fibrotic. Surrounding inflammatory changes from the thrombosis lead to scar tissue formation.

Patients may present with upper extremity swelling and pain. The presentation of these symptoms can be acute, subacute, or chronic. For the onset of symptoms in PSS, a history of an antecedent strenuous exercise can usually be elicited from the patients. On examination, the upper extremity will demonstrate edema with an enlarged girth of the extremity compared to the contralateral side.

The upper extremity may be tense and cyanotic. A convenient non-invasive first test is ultrasonography of the upper extremities. This will demonstrate thrombus in the deep venous system. Acute thrombus will be non-compressible on ultrasound. A vein with chronic thrombus will have an irregular and more collapsed appearance with the development of collaterals on ultrasound. Ultrasonography will not allow for complete demonstration of the central veins. Computed tomography CT venography and magnetic resonance MR venography can demonstrate thrombus in the venous system as well.

Contrast venography is a more invasive test that can demonstrate patency of the central venous system. Laboratory workup includes a thrombophilia panel protein C and S levels, antithrombin levels, prothrombin gene mutation analysis, factor V Leiden mutation testing, among others and D-dimer levels. D-dimer levels will be elevated in a patient with deep venous thrombosis; however, this elevation can also be seen in other infectious, inflammatory, and physiologic conditions.

If the patients have any symptoms concerning for a pulmonary embolism, a CT angiogram CTA thorax with pulmonary embolism protocol should be obtained. Alternatively, a ventilation-perfusion study can diagnose pulmonary embolism as well, but CTA thorax remains the study of choice. PSS is a deep venous thrombosis, and, as such, the first step in the management is to initiate anticoagulation therapy, typically, via an intravenous IV heparin drip.

Patients are given an initial bolus of intravenous IV heparin and then kept on a maintenance IV heparin drip. The dose of the bolus and maintenance heparin drip is weight-based. The affected extremity should be elevated to mitigate edema. If the patient presents within 2 weeks of the onset of symptoms, it is reasonable to consider a venogram and catheter-directed thrombolysis to reduce the clot burden. Under fluoroscopic guidance, a lysis catheter is placed to drip a thrombolytic agent such as alteplase for 24 to 48 hours.

Alternatively, a pharmacomechanical thrombectomy system can be used. When using alteplase, the fibrinogen levels should be monitored closely to direct the dosing and duration of the catheter-directed lysis. When using pharmacomechanical thrombectomy, one should be mindful of the risk of acute kidney injury associated with hemolysis.

If the presentation is more than 2 to 4 weeks from the onset of symptoms, heparinization alone may be initiated without thrombolytic therapy as the success of thrombolytic therapy is low beyond that window of time. Another option for treatment is mechanical catheter-directed thrombectomy. This must be done early in the course of the disease.

Decompression of the thoracic outlet is the definitive management of PSS. The surgical approach can either be transaxillary or supra, infra, or para-clavicular. The timing of decompression after thrombolytic therapy is widely debated. The role of long-term anticoagulation in these patients after adequate decompression remains unclear. Some clinicians perform a venogram, often with intravascular ultrasound, 2 weeks postoperatively and decide about the duration of anticoagulation at that time.

In this setting, venoplasty can be used for any residual stenosis since decompression has been achieved surgically. The use of stents is discouraged in this anatomic location due to the repetitive motion and risk of stent fracture and thrombosis. The duration of anticoagulation in patients with underlying hypercoagulability disorders after decompression also remains unclear. In most patients, anticoagulation for 3 to 6 months following an episode of deep venous thrombosis such as PSS is reasonable.

This can be achieved either via warfarin or direct oral anticoagulants DOAC. In the case of recurrent thrombosis after decompression surgery, thrombolysis and venography are usually attempted again with the maintenance of long-term anticoagulation therapy afterward.

In some patients with PSS, chronic total occlusion of the subclavian vein may persist despite adequate decompression maneuvers. Depending on the severity of the symptoms, venous reconstruction should be considered.

This can be achieved with a bypass or jugular vein turndown procedure with or without medial claviculectomy. Upper extremity swelling may be present in patients with lymphatic disorders or systemic conditions such as end-stage renal disease and congestive heart failure.

Upper extremity deep venous thrombosis can be seen with indwelling catheters as well. Anticoagulation with decompression is less successful than thrombolysis and decompression but still yields better results than anticoagulation alone. Patients should be encouraged to adhere to all medication recommendations and have close follow up with all of their healthcare providers.

Activity and lifestyle modification may reduce the risk of recurrent thrombosis. Patients with hypercoagulable states should be made aware that they may need long-term anticoagulation.

The diagnosis and management of PSS are best accomplished with an interprofessional team that consists of a primary care provider, sports medicine clinician, vascular surgeon, and radiologist. Patients with venous obstruction do need treatment or the arm will remain swollen and painful. After anticoagulation, decompression of the thoracic outlet is often required. Most patients do have a good outcome with treatment but depending on the state of the subclavian vein, some degree of arm swelling may persist.

Today, for some cases of PSS, endovascular therapy is available. Patient with bilateral first rib resection. Contributed by StatPearls. The right subclavian artery and surrounding structures. Contributed by Gray’s Anatomy Public domain. This book is distributed under the terms of the Creative Commons Attribution 4. Turn recording back on. Help Accessibility Careers. StatPearls [Internet]. Search term. Affiliations 1 St. Dominic Hospital. Continuing Education Activity Paget Schroetter syndrome PSS is effort-induced thrombosis of the axillary and subclavian veins associated with compression of the subclavian vein at the thoracic outlet.

Introduction Paget Schroetter syndrome PSS is effort-induced thrombosis of the axillary and subclavian veins associated with compression of the subclavian vein at the thoracic outlet. Etiology The subclavian vein travels in the proximity of the clavicle, first rib, anterior scalene, and subclavius muscles. Epidemiology PSS is more commonly seen in younger patients in their 20s and 30s with a male to female ratio of Pathophysiology Repetitive strain from vigorous physical activity and compression of the subclavian vein from adjacent anatomic structures leads to venous injury and subsequent thrombosis.

Southern Medical Research Conference | Journal of Investigative Medicine.

Semin Thromb Hemost. Upper extremity deep vein thrombosis: a community-based perspective. Am J Med. Upper-extremity deep vein thrombosis: a prospective registry of patients. Central venous thrombosis: an early and frequent complication in cancer patients bearing long-term silastic catheters. A prospective study. Thromb Res. Spectrum of upper extremity deep venous thrombosis in a community teaching hospital. Heart Lung. Paget-Schroetter syndrome in sports activities–case study and literature review.

Ann Thorac Surg. Primary upper-extremity deep vein thrombosis: high prevalence of thrombophilic defects. Am J Hematol. Risk factors and recurrence rate of primary deep vein thrombosis of the upper extremities. Prothrombin GA mutation and oral contraceptive use increase upper-extremity deep vein thrombotic risk.

Thromb Haemost. The importance of thrombophilia in the treatment of Paget-Schroetter syndrome. Ann Vasc Surg. Hypercoagulable states in primary upper extremity deep vein thrombosis.

Arch Intern Med. Louis J. Axillary vein thrombosis mimicking muscular strain. J Accid Emerg Med. Pulmonary embolism in deep venous thrombosis of upper extremity: more often in catheter-related thrombosis. Neth J Med. Upper-extremity deep venous thrombosis and pulmonary embolism.

Upper-extremity deep vein thrombosis. Risk factors, diagnosis, and complications. The post-thrombotic syndrome after upper extremity deep venous thrombosis in adults: a systematic review. The patient never had a sleep study or used a CPAP. The patient used half a can of chewing tobacco daily for thirteen years, and he smoked one pack per day for ten years but quit 12 years ago. He worked strenuous jobs in the construction industry most of his life.

On review of previous records, the patient was found to have consistently elevated hemoglobin The patient had a stocky, ruddy appearance without hepatosplenomegaly. Erythrocytosis can be categorized as primary, secondary, or relative. Patients with relative erythrocytosis have a decreased plasma volume with a relative increase in hemoglobin. Additionally, elevated hemoglobin levels have been associated with hypertension.

It is associated with mild obesity, elevated blood pressure, and increased blood viscosity, which may explain why these patients often develop cardiovascular complications. Patients with relative erythrocytosis are at a higher risk for thromboembolic complications.

A year-old female with a past medical history of hypertension presented to the emergency department with a large, ulcerating mass on the dorsal aspect of her left hand.

The patient reported the mass first appeared about two months prior to presentation and had been rapidly enlarging within the past week. The patient also reported an associated pound weight loss over the past two months. Vital signs were stable. Labs were remarkable for iron deficiency anemia and thrombocytosis. MRI showed a large 9. MRA showed patent radial and ulnar arteries. There was evidence of vasculature feeding into the mass from the common palmar digital artery branches of the ulnar artery and deep palmar branches of the radial artery.

On physical examination, the large mass on the dorsal aspect of the left hand was friable and bleeding figure 1. The patient reported minimal pain. Sensation remained intact in the median, ulnar, radial and axillary distributions. The patient underwent excisional biopsy of the mass with amputation of the 4th and 5th digits.

Negative margins were achieved per frozen pathology. Pathology of the postsurgical specimen showed high- grade undifferentiated pleomorphic sarcoma UPS. Clinical suspicion for soft-tissue malignancy of the hand is usually low because most tumors of the hand are small and benign.

However, soft-tissue sarcomas of the hand are rapidly growing tumors with a high metastatic potential. UPS have no specific line of differentiation and are usually a diagnosis of exclusion. Patients with UPS are typically older in age compared to those with other soft tissue sarcomas.

The use of adjuvant chemotherapy for patients with resectable soft -tissue sarcoma remains controversial. Prior studies have shown a very small efficacy of chemotherapy in regards to recurrence and overall survival.

Although our patient had no evidence of metastatic disease at presentation, close follow-up with oncology will be necessary for surveillance of recurrence. Ulcerating soft-tissue mass of left hand measuring 8. The presence of chronic osteomyelitis may obscure the diagnosis of Marjolin ulcer.

The present case report highlights this unique presentation to raise reasonable clinical suspicion and prevent missed diagnoses. While an initial shave biopsy was nonmalignant, a repeat biopsy six months later revealed indeterminant pathology. At this time, she was referred to plastic surgery for an excisional biopsy.

At presentation to plastic surgery, a nonhealing tibial wound with bone visible through a draining sinus tract was apparent within the boundaries of a previously healed scar.

She reported a remote history of an open compound fracture requiring multiple surgical operations in the vicinity of the lesion. An X-ray obtained to assess any residual hardware demonstrated findings consistent with osteomyelitis. Excisional biopsy revealed superficially invasive squamous cell carcinoma, consistent with a Marjolin ulcer. At the time of her initial operation, a satellite lesion was identified and biopsied, revealing squamous cell carcinoma in situ.

Marrow edema was observed in the screw path associated with hardware from her prior operations. Integra dermal substitute was placed while awaiting pathology results. Biopsy and culture of the affected bone revealed chronic osteomyelitis growing Staphylococcus lugdunensis. The patient returned to the OR for full excision of the satellite lesion along with corticotomy and sequestrectomy of the affected bony tissue.

A cement spacer with vancomycin and tobramycin was also placed at this time. Definitive coverage of the tibial defect was obtained with a soleus myocutaneous flap and split-thickness skin graft. Six weeks of outpatient cefazolin administration was arranged following discharge due to her chronic osteomyelitis.

Chronic immunosuppression, chronic infection, and multiple cutaneous neoplasms may be independently associated with the development of Marjolin ulcers. In the setting of atypical wound behavior, particularly when associated with these factors, clinical suspicion of secondary malignancy must remain high — even with prior negative biopsies and strong clinical evidence of an alternative diagnosis. Varicella zoster virus lies dormant in the spinal dorsal root ganglia until reactivation occurs and causes Herpes zoster.

With pain being the most common complication of Herpes zoster, other, more rare manifestations can be looked over. VZV can spread from the dorsal root to the neighboring ventral root and cause subsequent motor weakness.

This usually occurs in the same anatomical region as the presenting rash, but the two can rarely be dissociated. The majority of patient who develop segmental zoster paresis have a good prognosis and will recover all function after three to six months.

In this case report, we present a year-old female who came to the hospital with altered mental status that was found to have right arm paralysis after resolution of her encephalopathy. Magnetic resonance imaging of the brain showed no acute infarction. The patient had been diagnosed with Herpes zoster and prescribed gabapentin three days prior to admission with characteristic vesicular rash eruption present on the right upper extremity. Segmental zoster paresis was suspected at this point.

She was started on IV acyclovir, and physical therapy was consulted. The patient remained hospitalized for 3 weeks awaiting discharge to inpatient rehab in which time she performed daily strength exercises for her arm.

IV acyclovir was given for 21 days. The rash resolved, and the patient was discharged to inpatient rehab for two weeks where she was recommended by PT to perform three hours of intensive therapy per day. Herpes zoster is a common disorder seen in older adults. Although we tend to focus on the associated pain, we must remember other possible manifestations that can occur during or after the initial rash presentation.

Segmental zoster paresis should be suspected in any patient with paralysis and recent diagnosis of shingles. Diabetes mellitus is a chronic disease with the potential for significant morbidity and mortality. Here we present spontaneous diabetic myonecrosis, a rare complication of diabetes mellitus, in a patient with CF. A year-old female with past medical history of CF, cystic fibrosis-related diabetes mellitus, diabetic retinopathy, exocrine pancreatic insufficiency presented to the hospital with severe right calf pain for 2 months associated with swelling.

She denied trauma or fever. Creatinine kinase and thyroid stimulating hormone were unremarkable. Ultrasound of lower extremities showed no evidence of deep vein thrombosis, and X-ray of the right leg was unremarkable. Magnetic resonance imaging MRI right leg showed nonspecific myositis noted throughout the calf with areas of nonenhancement and soft tissue edema. The Patient was diagnosed with mild diabetic ketoacidosis and diabetic myonecrosis of the right calf.

DKA was treated per protocol, low-dose aspirin was started, and symtomatic treatment was given with pain control for diabetic myonecrosis.

Patient improved with no further complications. Spontaneous diabetic myonecrosis is a rare complication of diabetic mellitus. The pathogenesis is uncertain. Patients usually present with swelling and pain. The most common affected area is the front of the thigh, followed by the back of thigh or calf.

Awareness of the syndrome will frequently suggest the diagnosis and laboratory and imaging studies can be used to exclude other diagnoses. Interestingly, creatinine kinase is normal in many patients. MRI may show high intensity in the involved muscle on T2-weighted sequences as well as subcutaneous edema and subfascial fluid.

MRI with contrast is the diagnostic tool of choice which can distinguish nonenhancing infarcted muscle from surrounding inflammation or edema.

Muscle biopsy is not necessary; it is indicated only when the diagnosis remains in doubt or when infection cannot be excluded by other investigative techniques. Treatment includes rest, analgesia, low-dose aspirin, and optimal glycemic control. Complications including compartment syndrome and secondary infections are reported in some patients. Spontaneous diabetic myonecrosis is a rare, debilitating complication of diabetes.

The treatments are symptomatic treatment with analgesia, aspirin and optimize glycemic control. Without a high clinical suspicion for this condition, patients may be at risk for life- and limb-threatening progression to compartment syndrome and superimposed infection.

It is characterized by non-thrombocytopenic palpable purpura, abdominal pain, arthritis, and renal involvement. Pathologically, it can be considered a form of immune complex-mediated leukocytoclastic vasculitis LCV involving the skin and other organs.

This is an year-old gentleman with CKD stage IV, coronary artery disease, status post stent placement, who presented withupper extremities pain, mild swelling, and a subsequent development of palpable rash that spread on the lower extremities while being nonpainful and with no itch.

He had mild proteinuria and microscopic hematuria notable in his urinalysis. Further deterioration of his kidney function was noted with normal complement levels and ANCA panel. TEE ruled out infective endocarditis vegetations. The patient developed NSTEMI during his hospital stay prompting a percutaneous coronary intervention of the right coronary and left circumflex arteries. The rash and joint pain improved completely while his residual kidney function remained stable.

There was no immediate improvement of kidney function after the plasmapheresis course of 5 days. The diagnosis can be easily missed. Accordingly, a high degree of suspicion and attention to noninvasive laboratory work culminating into kidney biopsy with immuno-fluorescence studies is mandatory to establish the diagnosis. Adults with HSP carry a different prognosis, and the development of hematuria may be a harbinger for more serious complications such as nephritic or nephrotic syndrome.

Malignancy is common in adult-onset HSP and imaging should be done to exclude this possibility. Physicians should be aware of the possibility of HSP in patients who present with vasculitic rash and kidney disease. Normal complement level can distinguish between IgA nephropathy and vasculitis like granulomatosis with angiitis and lupus erythematosus. Early diagnosis of HSP with kidney biopsy may improve the outcome.

He had tachycardia, tachypnea, and fever with an erythematous, tender wound on left index finger. He had lacerated his finger 2 weeks prior with a pocket knife while cleaning dirt under his fingernail and failed cephalexin and clindamycin oral therapies prior to admission. Intravenous broad spectrum vancomycin and piperacillin-tazobactam were initiated and subsequently blood cultures grew Pasteurella multocida.

He lived on a farm with a cat. In view of the implanted cardiac device, fever and positive blood cultures, infective endocarditis was a concern. However, transthoracic echocardiogram did not reveal any vegetations.

XR and bone scan imaging of his finger also ruled out osteomyelitis. During his four days in the hospital, his condition and laboratory results improved and he was asymptomatic.

Follow up blood cultures were negative and patient was discharged home on oral amoxicillin clavulanate for another week duration. Pasteurella multocida is a commensal gram-negative bacteria seen in the mouths of many domesticated animals that is the most common cause of cellulitis after an animal bite. While it typically presents as soft tissue infections, it can more rarely cause meningitis, endocarditis, and bacteremia.

It seems that contamination of the self-inflicted subungual minor wounds with cat saliva is what caused Pateurella multocida bacteremia. The patient also presented with splinter hemorrhages. This physical exam finding is caused by rupture of capillaries underneath the nail plate. Blood attaches to the nail plate and moves distally as the nail grows, creating the characteristic linear pattern. While the association between splinter hemorrhages and endocarditis is often emphasized in medical training, they are present in other pathologies as well and in and of themselves do not constitute a criteria for endocarditis.

They most commonly appear due to trauma, but are also seen in endocarditis, vasculitis, medications, and renal failure due to platelet dysfunction from buildup of uremic toxins as was likely the case in this patient.

The patient was unlikely to have endocarditis since he became asymptomatic in 2—3 days and tested negative in repeat blood culture. Although a transesophageal echocardiogram would have been beneficial in definitively ruling it out infective endocarditis, a transthoracic echo of good quality is adequate especially in persons with prompt clinical improvement.

Although it primarily attacks the respiratory system, the extrapulmonary targets of the virus include the gastrointestinal tract and hepatobiliary system, among others. A year-old male with a past medical history of coronary artery disease, hypertension, 0.

He denied shortness of breath, cough, fever, or chills. Physical examination was significant for epigastric tenderness. Normal troponin. CXR on admission showed mild patchy bibasilar infiltrates suggestive of developing pneumonia. Right upper quadrant abdominal ultrasound showed no gallstones or other abnormalities within the pancreatobiliary system. CT abdomen and chest with and without contrast showed evidence of acute pancreatitis and no other abnormalities.

There is a limited but growing amount of literature supporting the diagnosis of SARS-CoV-2 induced viral pancreatitis. We worked up common causes of pancreatitis, and we do believe the patient presented with SARS-CoV-2 induced pancreatitis. ACE2 is most abundantly expressed in the pulmonary sytem, but is also expressed in pancreatic cells as well as other cells in the gastrointestinal tract. This could explain a correlation between the virus and pancreatitis and other gastrointestinal symptoms.

Although there is growing evidence of COVID-induced pancreatitis, the causal relationship is still debated between the two presentations. Achondrogenesis Type II is an autosomal dominant lethal collagen disorder characterized by prominent forehead, recessed chin, small chest and ribs with resulting lung hypoplasia, enlarged abdomen, shortened limbs, and decreased ossification of the pelvis and spine due to pathologic variants of the COL2A1 gene.

The gene encodes for the pro-alpha1 chain, a key component of type II collagen which is necessary in developing the framework for embryologic skeletal development. The phenotype of achondrogenesis is variable, and that variation remains poorly understood.

Infant and mother presented after a detailed sonographic exam showed micromelia and polyhydramnios concerning for skeletal dysplasia. An amniocentesis was performed at 22 weeks gestation age wga was notable for a heterozygous missense mutation on the COL2A1 gene concerning for a collagenopathy. The infant was born at Upon delivery infant was noted to have high palate, protuberant abdomen, rhizomelic shortening of all extremities, sacral dysgenesis, and small thoracic cavity.

He required intubation and mechanical ventilation immediately following delivery due to respiratory failure. He has failed multiple attempts at extubation and has remained ventilator dependent since birth. He was initially considered to have a perinatally lethal disease phenotype due to upper airway obstruction and severe lung hypoplasia, however, after 6 months he may be considered as a tracheostomy candidate due to improved amount of lung tissue and continued survival.

The clinical and radiographic phenotype of achondrogenesis are widely varied and may place a significant burden on a family as the definition of perinatal lethality remains challenging. The definition of lethality could be altered as a phenotype progresses with age. The number of skeletal dysplasias is growing rapidly due to technological advancements in genetic testing.

A detailed description of this patient in the medical literature will aid the ongoing differentiation of the collagenopathy types.

Skeletal dysplasias may be inherited via autosomal dominant, autosomal recessive, X-linked dominant, X-linked recessive, and Y-linked fashions. Mutation can occur de novo or inherited. Early prenatal diagnosis helps to guide genetic and palliative discussions with families to determine postnatal treatment options and recurrence risk.

Congenital dislocation of the spine CDS is a rare spinal malformation due to defective embryogenesis of the spine and spinal cord at a single level, that results in abrupt angulation of the spinal cord. We report a rare case of congenital displacement of the spine in a neonate which was missed at first admission. An 8-day old Exweek old female infant was admitted to our neonatal intensive care unit due to concerns about a bump in the back.

The infant was born to a year-old female via spontaneous vaginal delivery at an outside hospital. The prenatal course was unremarkable, all prenatal ultrasounds were reported as normal, and the mother was not on any medications during pregnancy except prenatal vitamins. She was discharged from the delivery hospital after routine care.

On follow-up exam with the primary care pediatrician, she was found to have a lump on the lower back. Spinal imaging including ultrasonography and magnetic resonance imaging were done which revealed posterior dislocation of L3 vertebral body relative to L2 with spinal cord compression.

She was admitted to the neonatal intensive care unit after neurosurgery consultation. On physical exam, the infant was noted to have bilateral talipes valgus deformity with an otherwise unremarkable neurological exam. On spinal exam, she had midline bony swelling step-up defect at L3 level with no other neurocutaneous findings. Chromosomal microarray and whole-exome sequencing were normal. Posterior dislocation of L3 vertebral body relative to L2 with spinal cord compression.

Although rare, congenital dislocation of the spine when diagnosed needs an immediate stabilization of the spine.

Our case stresses the importance of a thorough spinal examination for appropriate diagnosis. Human herpesvirus-6 HHV-6 is a beta herpesvirus found ubiquitously. However, establishing HHV-6 as a cause of meningitis or encephalitis may be challenging as a polymerase chain reaction PCR test alone does not imply causality. Examination revealed a clinically stable neonate. There was no CSF pleocytosis. After consult with infectious disease specialist, baby received IV Ganciclovir and repeat Biofire meningitis panel at 7 days continued to remain positive for HHV The lipid matrix breaks through the thinning collagen gap and when the lipids come in contact with the blood, clotting occurs.

After rupture the platelet adhesion causes the clotting cascade to contact with the lipid pool causing a thrombus to form. This thrombus will eventually grow and travel throughout the body. The thrombus will travel through different arteries and veins and eventually become lodged in an area that narrows.

Once the area is blocked, blood and oxygen will not be able to supply the vessels and will cause death of cells and lead to necrosis and poisoning. Serious complicated plaques can cause death of organ tissues, causing serious complications to that organ system. The majority of events occur due to atheroma plaque rupture at areas without narrowing sufficient enough to produce any angina or stress test abnormalities.

Thus, since the laters, greater attention is being focused on the “vulnerable plaque. Though any artery in the body can be involved, usually only severe narrowing or obstruction of some arteries, those that supply more critically-important organs are recognized. Obstruction of arteries supplying the heart muscle result in a heart attack.

Obstruction of arteries supplying the brain result in a stroke. Over the last couple of decades, methods other than angiography and stress-testing have been increasingly developed as ways to better detect atherosclerotic disease before it becomes symptomatic. These have included both a anatomic detection methods and b physiologic measurement methods.

Examples of physiologic methods include: 1 lipoprotein subclass analysis, 2 HbA1c , 3 hs-CRP , and 4 homocysteine. The example of the metabolic syndrome combines both anatomic abdominal girth and physiologic blood pressure, elevated blood glucose methods. Advantages of these two approaches: The anatomic methods directly measure some aspect of the actual atherosclerotic disease process itself, thus offer potential for earlier detection, including before symptoms start, disease staging and tracking of disease progression.

The physiologic methods are often less expensive and safer and changing them for the better may slow disease progression, in some cases with marked improvement.

Disadvantages of these two approaches: The anatomic methods are generally more expensive and several are invasive, such as IVUS. The physiologic methods do not quantify the current state of the disease or directly track progression.

For both, clinicians and third party payers have been slow to accept the usefulness of these newer approaches. The relation between dietary fat and atherosclerosis is a contentious field. The USDA, in its food pyramid, promotes a low-fat diet, based largely on its view that fat in the diet is atherogenic.

Laboratory animals fed rancid fats develop atherosclerosis. Rats fed DHA -containing oils experienced marked disruptions to their antioxidant systems, as well as accumulated significant amounts of peroxide in their blood, livers and kidneys. Rancid fats and oils taste very bad even in small amounts; people avoid eating them. The resultant oils are colorless, odorless, tasteless and have a longer shelf life than their unrefined counterparts.

Lipoprotein imbalances, upper normal and especially elevated blood sugar, i. Evidence has increased that people with diabetes , despite their not having clinically-detectable atherosclotic disease, have more severe debility from atherosclerotic events over time than even non-diabetics that have already suffered atherosclerotic events.

Thus diabetes has been upgraded to be viewed as an advanced atherosclerotic disease equivalent. If atherosclerosis leads to symptoms, some symptoms such as angina pectoris can be treated.

Non-pharmaceutical means are usually the first method of treatment, such as cessation of smoking and practicing regular exercise. If these methods do not work, medicines are usually the next step in treating cardiovascular diseases, and, with improvements, have increasingly become the most effective method over the long term. However, medicines are criticized for their expense, patented control and occasional undesired effects.

In general, the group of medications referred to as statins has been the most popular and are widely prescribed for treating atherosclerosis. However, for most people, changing their physiologic behaviors, from the usual high risk to greatly reduced risk, requires a combination of several compounds, taken on a daily basis and indefinitely.

More and more human treatment trials have been done and are ongoing that demonstrate improved outcome for those people using more-complex and effective treatment regimens that change physiologic behaviour patterns to more closely resemble those that humans exhibit in childhood at a time before fatty streaks begin forming. The statins , and some other medications, have been shown to have antioxidant effects, possibly part of their basis for some of their therapeutic success in reducing cardiac ‘events’.

The success of statin drugs in clinical trials is based on some reductions in mortality rates, however by trial design biased toward men and middle-age, the data is as, as yet, less strongly clear for women and people over the age of 70 CMAJ. For example, in 4S, the first large placebo controlled, randomized clinical trial of a statin in people with advanced disease who had already suffered a heart attack, the overall mortality rate reduction for those taking the statin, vs.

There were 3 more dead women at trial’s end on statin than in the group on placebo drug whether chance or some relation to the statin remains unclear. Combinations of statins , niacin , intestinal cholesterol absorption-inhibiting supplements ezetimibe and others, and to a much lesser extent fibrates have been the most successful in changing common but sub-optimal lipoprotein patterns and group outcomes.

In the many secondary prevention and several primary prevention trials, several classes of lipoprotein expression less correctly termed “cholesterol-lowering” altering agents have consistently reduced not only heart attack, stroke and hospitalization but also all-cause mortality rates. For both human and animal trials, those which have shown evidence of disease regression had all utilized more aggressive combination agent treatment strategies, nearly always including niacin.

Additionally, individual responses to daily niacin, while mostly evident after a month at effective doses, tends to continue to slowly improve further over time. PSS is related to the compression and subsequent thrombosis of the subclavian vein due to these structures. PSS is more commonly seen in younger patients in their 20s and 30s with a male to female ratio of Often these patients will not have any other significant medical comorbidities.

The incidence ranges from 1 to 2 per , individuals per year in various studies. It is more commonly encountered on the right side. Often affected individuals with PSS will be athletes, specifically, those engaged in the repetitive over-the-head motion of their arms, including baseball or softball pitchers, swimmers, rowers, or weightlifters.

Repetitive strain from vigorous physical activity and compression of the subclavian vein from adjacent anatomic structures leads to venous injury and subsequent thrombosis.

This impedes the venous return from the affected extremity leading to edema. Over time, the body forms collaterals to bypass the venous obstruction. In the chronic phase, the vein becomes fibrotic. Surrounding inflammatory changes from the thrombosis lead to scar tissue formation. Patients may present with upper extremity swelling and pain. The presentation of these symptoms can be acute, subacute, or chronic. For the onset of symptoms in PSS, a history of an antecedent strenuous exercise can usually be elicited from the patients.

On examination, the upper extremity will demonstrate edema with an enlarged girth of the extremity compared to the contralateral side. The upper extremity may be tense and cyanotic. A convenient non-invasive first test is ultrasonography of the upper extremities. This will demonstrate thrombus in the deep venous system.

Acute thrombus will be non-compressible on ultrasound. A vein with chronic thrombus will have an irregular and more collapsed appearance with the development of collaterals on ultrasound. Ultrasonography will not allow for complete demonstration of the central veins.

Computed tomography CT venography and magnetic resonance MR venography can demonstrate thrombus in the venous system as well. Contrast venography is a more invasive test that can demonstrate patency of the central venous system. Laboratory workup includes a thrombophilia panel protein C and S levels, antithrombin levels, prothrombin gene mutation analysis, factor V Leiden mutation testing, among others and D-dimer levels.

D-dimer levels will be elevated in a patient with deep venous thrombosis; however, this elevation can also be seen in other infectious, inflammatory, and physiologic conditions. If the patients have any symptoms concerning for a pulmonary embolism, a CT angiogram CTA thorax with pulmonary embolism protocol should be obtained. Alternatively, a ventilation-perfusion study can diagnose pulmonary embolism as well, but CTA thorax remains the study of choice.

PSS is a deep venous thrombosis, and, as such, the first step in the management is to initiate anticoagulation therapy, typically, via an intravenous IV heparin drip. Patients are given an initial bolus of intravenous IV heparin and then kept on a maintenance IV heparin drip.

The dose of the bolus and maintenance heparin drip is weight-based. The charts of these patients were reviewed and data were collected regarding patient demographics, symptoms, previous Paget ‘s disease, surgical treatments and time to recurrence.

The group of women who had invasive diseases also underwent inquinofemoral lymphadenectomy and no lymphatic metastases were noted. The mean time to relapse was 30 months range months. Recurrence is very common and long-term monitoring is recommended with careful examination of any abnormal vulvar lesion. Genetics Home Reference: Paget disease of bone.

We aimed to demonstrate imaging features of Paget ‘s disease of breast, which is an extremely uncommon malignancy that presents with changes in the nipple-areolar region that may or may not be associated with an underlying in situ component or invasive cancer. Mammography is the initial investigation of choice, having a high sensitivity especially in cases where a palpable mass is present.

The addition of ultrasound improves the accuracy of mammography. The surgical management of Paget ‘s disease includes mastectomy with or without axillary dissection, though breast conservation surgery in the form of wide local excision can also be done in a selected group of patients.

Management should be based on both clinical and imaging findings, including mammography and ultrasound, with MRI playing a crucial role in defining the extent of involvement. GATA3 expression in primary vulvar Paget disease: a potential pitfall leading to misdiagnosis of pagetoid urothelial intraepithelial neoplasia. GATA3 has been reported as a specific urothelial marker among organs in the pelvic region, and has been classified as highly sensitive and specific for urothelial and breast carcinomas.

Our aim was to verify GATA3 expression in extramammary Paget disease, and to determine whether it can be use to differentiate primary vulvar Paget disease from pagetoid urothelial intraepithelial neoplasia PUIN. We also analysed HER2 protein expression and HER2 gene amplification and their roles as prognostic factors in extramammary Paget disease. All cases showed nuclear expression of GATA3. Fluorescence in-situ hybridization results showed amplification in two of these six cases.

Both HER2-amplified cases were invasive. GATA3 was positive in all extramammary Paget disease cases tested 13 cases , and it has no value for differentiating between primary and secondary vulvar Paget disease from the urological tract. HER2 amplification might confer an aggressive and invasive pattern in primary vulvar Paget disease, as both amplified cases showed an invasive pattern. Successful TS-1 monotherapy as the second-line treatment for advanced extramammary Paget ‘s disease: A report of two cases.

There is no standard chemotherapeutic treatment for advanced extramammary Paget ‘s disease, though the effectiveness of some chemotherapy regimens, including docetaxel, has been reported. In this report, we report that TS-1 monotherapy was effective in two patients with advanced extramammary Paget ‘s disease after docetaxel treatment failure.

TS-1 monotherapy may be useful as the second-line treatment for patients with advanced extramammary Paget ‘s disease. Severe hypocalcemia following bisphosphonate treatment in a patient with Paget ‘s disease of bone. Bisphosphonate therapy is a common and effective treatment for Paget ‘s disease of bone, osteoporosis, hypercalcemia of malignancy and cancer metastatic to bone. Clinically significant hypocalcemia has not been reported in patients with Paget ‘s disease of bone and normal parathyroid function treated with an aminobisphosphonate.

After 10 days of treatment, she developed severe hypocalcemia 5. We hypothesize that this case represents an example of hungry bone syndrome in a patient with extensive Paget ‘s disease of bone who received risedronate, causing acute suppression of bone resorption while elevated bone formation rates continued.

In the year following her recovery, the patient was successfully treated with slowly titrated anti-resorptive therapy subcutaneous calcitonin followed by titrated doses of risedronate , and is now clinically well. Physicians should be aware of the potential for hypocalcemia when patients with polyostotic Paget ‘s disease and markedly elevated indicators of bone remodeling are initiated on powerful anti-resorptive therapy.

Apocrine hidradenocarcinoma showing Paget ‘s disease and mucinous metaplasia. A year-old man presented with a solitary, erythematous, rapidly growing 1-cm nodule on his scalp that had arisen over the previous 3 months. He had no history of skin cancer. An excisional biopsy of the lesion showed a fairly well-circumscribed but focally invasive tumor consisting of areas of typical-appearing clear cell hidradenoma as well as areas with mucinous goblet-type cells and cells with eosinophilic cytoplasm and decapitation-type secretion.

There was marked cellular atypia, numerous atypical mitotic figures and focal necrosis. The tumor cells focally involved the overlying epidermis Paget ‘s disease. Large areas of mucin were identified throughout the lesion. The tumor cells stained with markers for cytokeratin 7 and focally for EMA and CEA, confirming ductal differentiation. The goblet cells and mucinous areas stained with mucicarmine and PASD.

The patient was diagnosed with hidradenocarcinoma with mucinous differentiation. Associated Paget ‘s disease has only rarely been reported, and mucinous metaplasia is a previously unreported feature in hidradenocarcinoma.

Synthetic human calcitonin was used in the treatment of 26 patients over a period of months. Subjective improvement reduction of pain, improvement of mobility was found in 15 patients with Paget ‘s disease, in 4 females with postmenopausal osteoporosis and in all 3 patients with Sudeck’s syndrome. Radiographic improvement of bone changes developed only very slowly.

These results were confirmed by diminution of the exchangeable calcium pool indicating reduction of rates of osseous degradation. Calcitonin tolerance was acceptable. Transitory nausea and occasional vomiting occurred in 3 patients. We examined the incidence and modern national trends in the management of Paget ‘s disease PD , including the use of breast-conserving surgery BCS , mastectomy, axillary surgery, and receipt of radiotherapy.

Using surveillance, epidemiology and end results SEER data, we identified patients diagnosed with PD during Trends in age-adjusted incidence, primary surgery, sentinel lymph node biopsy SLNB , and axillary lymph node dissection were examined. Multivariable logistic regression was used to evaluate factors associated with receipt of BCS and radiotherapy. A decrease in the age-adjusted incidence of PD occurred from to An interesting case of peripheral vascular disease, vascular reperfusion, and subsequent development of pain due to Paget ‘s disease of bone.

To present a case of Paget ‘s disease of bone that was unmasked after vascular reperfusion. In this case study, we review the presentation, evaluation, diagnosis, and management of a patient with Paget ‘s disease and peripheral vascular disease. A year-old-woman with a history of coronary artery heart disease and recent finding of a T5 compression fracture was hospitalized for evaluation of right lower extremity claudication. Angiography demonstrated a focal complete occlusion of the distal right femoral and popliteal arteries.

A self-expanding stent was placed in the distal femoral and popliteal arteries. Approximately 48 hours after the procedure, the patient developed severe, right lower leg pain. On endocrine evaluation, the patient was found to have clinical signs suggesting Paget ‘s disease of bone, which was subsequently confirmed by imaging. This patient’s development of severe pain following reperfusion of distal femoral and popliteal arteries is in keeping with the known and aforementioned hypervascularity of pagetic bone.

The finding of increased warmth over an area of skeletal deformation should always raise the possibility of Paget ‘s disease of bone. Surgical management of hydrocephalic dementia in Paget ‘s disease of bone: the 6-year outcome of ventriculo-peritoneal shunting. Paget ‘s disease of bone is a chronic progressive skeletal disorder usually occurring in the long bones and skull of older adults and elderly persons.

In the skull, softening of the skull base may lead to basilar impression and consequently obstruction of the cerebrospinal fluid through the basilar cisterns, resulting ventricular enlargement in association with gait difficulties, incontinence and dementia: a syndrome resembling normal pressure hydrocephalus.

The optimal management of hydrocephalus associated with Paget ‘s disease of the skull is not well documented and is still debated. We report a patient with hydrocephalic dementia linked to Paget ‘s disease of the skull who showed marked sustained improvement in her neurological condition after ventriculo-peritoneal shunt insertion.

We have now followed this patient for 6 years. Our experience supports the view that ventricular shunting is the procedure of choice for treating hydrocephalus linked to the Paget ‘s disease of bone and is best carried out in the early stages of the illness. Paget ‘s disease of bone resembling bone metastasis from gastric cancer. A year-old man had an endoscopic type 0’-IIc tumor in the upper gastric body on the greater curvature and biopsy showed the tumor to be a well-differentiated adenocarcinoma Group 5.

He was referred to us for endoscopic submucosal dissection ESD. Endoscopy revealed fold convergency, fold swelling, and fusion of the fold, indicating tumor invasion into the submucosa, which was outside the indications for ESD.

In addition, there was an increase of serum bone-type alkaline phosphatase ALP-III and ALP-IV and urinary cross-linked N-terminal telopeptide of type I collagen a bone metabolism marker , while 18 F-fluorodeoxyglucose positron emission tomography showed increased uptake in the left pelvis and Th10, suggesting bone metastases. We first diagnosed gastric cancer with bone metastases; however, the symptoms suggested pathological bone fracture and no bone pain.

Therefore, a computed tomography-guided aspiration bone biopsy was performed to exclude the possibility of Paget ‘s disease of bone. Biopsy specimens revealed no tumor and a mosaic pattern. We finally diagnosed gastric cancer accompanied by Paget ‘s disease of bone and performed a laparoscopy-assisted proximal gastrectomy.

In gastric cancer patients with suspected bone metastasis, we also need to consider Paget ‘s disease of bone. Technical and clinical description of a case of extensive anogenital Paget ‘s disease associated with anal cancer treated by tomotherapy. In this paper we describe a case of extramammary Paget ‘s disease associated with anal cancer, which was successfully treated by intensity-modulated radiotherapy using tomotherapy with a simultaneous integrated boost and daily image guidance.

The main pitfall in this report is the relatively short follow-up 1 year , which means that the evaluated data is promising but not conclusive. Considering the rarity and wide extension of our patient’s Paget ‘s disease in the anogenital region, and the lack of literature reports about curative radiotherapy in this particular setting, this case report may be considered the first related to extensive extramammary Paget ‘s disease treated by tomotherapy. Thirty six patients with Paget ‘s disease of the vulva were reviewed.

The median age of the patients at diagnosis was 67 years range: years. One patient had a history of previous mammary adenocarcinoma. Screening for malignancy revealed two colonic tumours. Two patients with negative screening at presentation developed, 12 and 18 months respectively after vulvectomy, an ovarian carcinoma stage IIc and a cervical and urethral adenocarcinoma. All patients were treated by surgery based on extent of the disease. Out of the 36 patients, 29 were available to follow-up.

The median follow-up period was 74 months range months. Three patients died of metastatic disease due to vulval adenocarcinoma and breast carcinoma, or of liver metastases from an unknown adenocarcinoma. Eighteen of the 29 patients followed up remained free of disease. Five out of the 16 patients with positive margins recurred, as did 5 out of 9 patients with negative margins. Treatment of Paget ‘s disease of the vulva is surgical.

In order to prevent recurrence, some authors have proposed surgical excision extending beyond the visible clinical lesions with intraoperative frozen sections. The data we recorded show that free margins do not seem to correlate with recurrence, so that large excision beyond the clinical lesion is not useful.

Ultrastructural features of the osteoclasts from Paget ‘s disease of bone in relation to a viral aetiology. The ultrastructure of the osteocytes, osteoblasts, osteoclasts, haemopoietic and other connective tissue cells was examined in 27 biopsies from 22 patients with Paget ‘s disease of bone. Electron microscopy showed characteristic nuclear and cytoplasmic inclusions in the osteoclasts of all of the 25 biopsies exhibiting histological evidence of Paget ‘s disease.

Such inclusions were absent from all the other types examined. The intranuclear inclusions consisted of stacked rows or complex whorls of tubular filaments with an individual filament diameter of nm, often arranged in a paracrystalline array. The frequency of occurrence of inclusions in the osteoclasts and their individual nuclei measured quantitatively in 18 of the biopsies was related to the histological severity of the disease process.

The similarity of the observed inclusions to those of paramyxovirus inclusion bodies particularly measles support the hypothesis that Paget ‘s disease is a slow virus infection. Images PMID Objective: The clinical characteristics, management, and prognostic indicators of penoscrotal extramammary Paget ‘s disease are not clearly defined.

Surgical excision is often an effective treatment modality but results in a large wound after resection of all involved tissues. Methods: Reconstruction of large penoscrotal soft-tissue defects after wide local excision remains a challenge to the reconstructive surgeon. The use of the anterolateral thigh flap for penoscrotal reconstruction after resection of extramammary Paget ‘s disease is infrequent as a reconstruction tool throughout the literature.

Results: We discuss a case where the anterolateral thigh flap was effectively used for reconstruction of a large penoscrotal defect after wide local excision of penoscrotal extramammary Paget ‘s disease and present a comprehensive literature review of extramammary Paget ‘s disease key features, diagnosis, prognosis, and treatment.

Conclusions: The anterolateral thigh flap is a useful tool for penoscrotal defect reconstruction. Methyl – aminolevulinic acid photodynamic therapy and topical tretinoin in a patient with vulvar extramammary Paget ‘s disease. Extramammary Paget ‘s disease is a rare neoplasm of apocrine gland-bearing areas of the skin. The most common site of presentation is the vulva. Surgery is the most frequently reported therapy so far; however, it is invasive and it is complicated by a high rate of recurrence.

For this reason, several less-invasive treatments have been recently proposed, including photodynamic therapy. We describe in this article the case of an year-old patient with a noninvasive vulvar extramammary Paget ‘s disease successfully treated with methyl-aminolevulinic acid photodynamic therapy associated with topical tretinoin.

Paget disease of bone PDB is a skeletal disorder characterized by focal abnormalities of bone remodeling, which result in enlarged and deformed bones in one or more regions of the skeleton. In some cases, the pagetic tissue undergoes neoplastic transformation, resulting in osteosarcoma and, less frequently, in giant cell tumor of bone GCT.

We performed whole-exome sequencing in a large family with 14 PDB-affected members, four of whom developed GCT at multiple pagetic skeletal sites, and we identified the c.

ProArg missense mutation in the zinc finger protein gene ZNF The mutation precisely co-segregated with the clinical phenotype in all affected family members.

We previously detected expression of measles virus nucleocapsid MVNP transcripts Also, we have shown that MVNP gene. Genetics Home Reference: inclusion body myopathy with early-onset Paget disease and frontotemporal dementia. Personality changes, Published by Elsevier Inc. The therapy of Paget ‘s bone disease is essentially based on the use of calcitonin and diphosphonates: both drugs, if used in large doses for long periods, have shown themselves able to provoke particular side-effects.

It was, therefore, decided to study the therapeutic efficacy of combined low-dosage treatment using synthetic salmon calcitonin and sodium-etidronate on a group of patients with Paget ‘s osteodystrophy. A clear evident diminution in plasma alkaline phosphatase, hydroxyprolinuria and whole body retention WBR of MDP-Tc99m was observed, demonstrating a reduction of metabolic turnover in the bone. No changes in the bone mass BMC , evaluated by bone mineral detector, were observed at the end of treatment.

With this treatment the plateau effect was shown to be appreciably less than normally occurs when either calcitonin or sodium etidronate are used alone. VCP encodes a well-conserved multifunctional protein, valosin containing protein VCP , which has important roles in protein quality control via proteasome and autophagy, protein aggregation, quality control of mitochondria, cell proliferation, and so on.

Clinically, muscle weakness is the most common symptom of which disease onset is around 40 years. Affected muscles are variable, and the patients are sometimes diagnosed as limb girdle muscular dystrophy or GNE myopathy. Paget ‘s disease of bone is reported to be observed in a half of the patients around the age of 40 years, but less common in Japanese patients. Frontotemporal dementia is seen around one third of the patients which appears nearly 10 years later than muscle or bone disease.

In addition to cognitive dysfunctions, motor neuron involvement and cerebellar signs were also seen in our series. IBMPFD is not so rare disease as previously thought, but complicate clinical findings may make its diagnosis difficult.

KNG increased normal and pagetic marrow stromal cell proliferation at 1. KNG in the KNG in the presence of This somatic genealogy of Dramatism’s core terms–symbolic action, attitude, identification–argues for the importance of keeping rhetoric, rhetorical theory, and rhetorical pedagogy more closely tied to bodies that generate, induce, and respond to rhetoric.

It does so by examining Burke’s use of Sir Richard Paget ‘s theory that spoken language…. MR imaging of pseudosarcoma in Paget ‘s disease of bone: a report of two cases. Pseudosarcoma is a rare manifestation of Paget ‘s disease of bone.

We report the MR imaging of two cases highlighting the difficulties in diagnosis. One of the cases is the first time this condition has been described outside the long bones of the lower limb. Cowan, Renee A. Objective The objective of this prospective pilot study was to assess the clinical and histologic effects of topical imiquimod therapy on recurrent extramammary Paget ‘s disease of the vulva.

The topical cream was to be applied 3 times per week for 12 weeks. Punch biopsy and photography were performed at baseline and at the week time point. Results Eight patients from two institutions were enrolled. Of the two remaining patients, one had a complete clinical response but no significant histologic response; the other patient was removed from the study protocol secondary to intolerable local irritation.

Two patients continue to have no evidence of disease after a median follow-up of 35 months. Five are alive with disease. No patients progressed to invasive cancer while receiving therapy. Given the rare nature of this disease, additional multi-institutional prospective studies should be conducted to explore the efficacy of this treatment regime.

An appreciable improvement in pain symptomatology was noted and at times an evident improvement in audiometry; from the metabolic viewpoint there was a fall in serum alkaline phosphatase and urinary excretion of calcium and hydroxyproline.

A study of radiocalcium kinetics demonstrated a reduction in the exchangeable calcium pool and the fractional turnover rate.