1/11. Successful preoperative diagnosis of massive ovarian edema aided by comparative imaging study using magnetic resonance and ultrasound.Massive ovarian edema (MOE) is a rare disease. Therefore, preoperative diagnostic method of massive ovarian edema (MOE) has not been established. We have succeeded in making a preoperative diagnosis of MOE aided by ultrasonogram and magnetic resonance imaging (MRI) and the patient's ovaries were preserved. Characteristics and proposed diagnostic imaging criteria for MOE are discussed.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
2/11. Sonographic characteristics of massive ovarian edema.Massive ovarian edema (MOE) is a very rare disease characterized by a tumor-like enlargement of the ovary. The ultrasound findings have been reported as a solid tumor-like mass or as a solid mass containing a cystic component. Recent reports using magnetic resonance imaging of MOE have demonstrated that multiple ovarian follicles situated around the periphery of the cortex of the enlarged ovary is the most important diagnostic indicator of MOE. This finding was first demonstrated by ultrasound in the case we present here. Thus we suggest that the ultrasound detection of multiple peripheral ovarian follicles in a solid ovarian tumor-like mass may make the preoperative diagnosis of MOE possible by ultrasound alone.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
3/11. An autopsy case of pulmonary capillary hemangiomatosis without evidence of pulmonary hypertension.We report a case of pulmonary capillary hemangiomatosis (PCH) affecting a 37-year-old woman. PCH is a rare disease accompanying intrapulmonary capillary growth and pulmonary hypertension. In the present case, capillaries infiltrated the alveolar and bronchial walls but not the vascular walls. No signs or symptoms of pulmonary hypertension or right heart hypertrophy/dilatation were present. These findings suggest that vascular involvement in PCH is responsible for the induction of pulmonary hypertension.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
4/11. Prostatic adenocarcinoma masquerading as lymphoma and presentation with axillary-subclavian vein thrombosis.Axillary and/or subclavian vein thrombosis is a relatively rare disease, constituting about 2% of all cases of deep venous thrombosis (DVT). We report on a patient who presented with axillary-subclavian vein thrombosis and had metastatic prostatic adenocarcinoma.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
5/11. Lipoprotein glomerulopathy associated with psoriasis vulgaris: report of 2 cases with apolipoprotein e3/3.Lipoprotein glomerulopathy (LPG) is a rare disease, characterized by a special histology, including dilated glomerular capillaries filled with pale-stained and meshlike lipoprotein thrombi. It always presents with proteinuria or nephrotic syndrome. Although hyperlipidemia is not always seen, most patients have type III hyperlipoproteinemia with apolipoprotein (apo) E2/3 phenotyping. Although the clinical feature of LPG is rarely described, LPG associated with other glomerulopathy, including IgA nephropathy, membranous nephropathy, and lupus nephritis, has been documented. Until now, there have been no reports of psoriasis vulgaris associated with LPG. The authors present 2 cases of LPG with apo E3/3 genotyping associated with psoriasis vulgaris. The first patient was a 65-year-old woman who presented with nephrotic syndrome with daily urinary protein loss of 9.05 g and itchy erythematous scaly plaques on her trunk and lower limbs for 1 year. The renal biopsy results showed LPG, and the skin biopsy results showed psoriasis. The second patient was a 50-year-old man with history of psoriasis over his trunk and 4 limbs for 30 years. He also presented with nephrotic syndrome with daily urinary protein loss of 7.55 g. The renal biopsy results also showed LPG. The genotype of apo E showed E3/3, and lipoprotein electrophoresis showed a type III hyperlipoproteinemia-like pattern in both cases. The authors suggest that presence of apo E3/3 genotype cannot rule out the diagnosis of type III hyperlipoproteinemia and LPG. Besides, LPG should be included in the differential diagnosis of psoriatic patients with nephrotic syndrome, especially in Asian patients who show poor response to traditional therapy. Renal biopsy should be performed to make the definitive diagnosis.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
6/11. Acute hemorrhagic edema of infancy: a case report.BACKGROUND: Acute hemorrhagic edema of infancy (AHEI), or Seidlmayer's disease, is a type of leukocytoclastic vasculitis proper of infants and children. It is characterized by a local increase in temperature, erythematous edema and purpuric lesions involving mainly the face and extremities. There usually is no visceral involvement. The disease is self-limited, bearing a benign clinical course. infection, drugs and immunization have been considered as precipitating factors. The main differential diagnosis is Henoch-Schonlein purpura (HSP). methods: We describe a classic example of acute hemorrhagic edema of infancy, and comment on the clinical features, pathology, immunopathology and proposed therapy. We characterize the differences between AHEI and HSP. RESULTS: A course of corticosteroids was given to avoid quick progression of disease. CONCLUSION: Acute hemorrhagic edema of infancy is a rare disease, and the most striking classic feature of the disease is the contrast between the acuteness of the cutaneous lesions and the good general condition of the patient. Considering its clinical features, pathology and immunopathology, AHEI can be justifiably characterized as a unique disorder, distinct from HSP.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
7/11. Transient bone marrow edema syndrome progressing to avascular necrosis of the hip - a case report and review of the literature.Transient bone marrow edema syndrome (TMES) is a rare disease of unknown etiology. diagnosis is made by exclusion. There is still controversy as to whether TMES is considered to be a reversible form of avascular necrosis (AVN), a disease entity of its own or a form of non-traumatic algodystrophy. We here describe the extremely rare occurrence of three cases of TMES that progressed to AVN.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
8/11. Fatal acute pulmonary embolism in a patient with pelvic lipomatosis after surgery performed after transatlantic airplane travel.We describe a case of a 37-yr-old patient who traveled from europe to the United States and succumbed to a massive pulmonary embolism 6 days after elective pelvic surgery despite routine postoperative thrombotic prophylaxis. In retrospect, he was likely to have developed a deep venous thrombosis during the transatlantic trip to our hospital. Anesthesiologists and other physicians involved in perioperative management need to be aware of the prevalence of venous thromboembolism in patients with a history of recent prolonged air travel. This is particularly true in tertiary referral centers, where patients with rare diseases may have a major surgical intervention within days of prolonged air travel.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
9/11. Tenderness over the hyoid bone can indicate epiglottitis in adults.adult acute epiglottitis is a rare but life-threatening disease caused by obstruction of the airway. The symptoms and signs of this disease may be nonspecific without apparent airway compromise. We encountered 3 consecutive cases of adult patients with this disease in a single 5-month period in one physician's office. In all cases, physical examination revealed tenderness of the anterior neck over the hyoid bone. These observations assisted us in identifying this rare disease quickly. We suggest that tenderness over the hyoid bone should raise suspicion of adult acute epiglottitis.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
10/11. Juvenile dermatomyositis presenting with periorbital edema.Juvenile dermatomyositis is a rare disease that affects the skin and muscles. It often presents with a classic heliotrope eyelid rash. We present a case of juvenile dermatomyositis presenting with significant bilateral periorbital edema, with its complete resolution after systemic antiinflammatory therapy.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
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