Epstein's syndrome is a rare disease whish is characterized by the association of thrombocytopenia, macrothrombocytopathia, nephritis and deafness. We experienced a case of Epstein's syndrome in a 12 years old male patient who was presented with a life long history of bleeding, usually as epistaxis, bilateral sensorineural deafness and hematuria with proteinuria starting in late childhood. Hematologic studies showed thrombocytopenia with giant platelets and anemia. A bone marrow aspirate revealed the megakaryocytes to be adequate in number and many giant size platelets. Platelet do not respond to addition of A and epinephrine; collagen and ristocetin induced agglutination response is decreased. It is difficult to be certain the association of thrombocytopenia with giant platelets, nephritis and deafness constitutes a new hereditary disease with a distinct pathogenesis or if it is an expansion of the well recognized Alport's syndrome of hereditary nephritis deafness. We report a case of Epstein's syndrome syndrome with brief review of related literatures.
Agglutination ; Anemia ; Blood Platelets ; Bone Marrow ; Child ; Collagen ; Deafness ; Epinephrine ; Epistaxis ; Genetic Diseases, Inborn ; Hematuria ; Hemorrhage ; Humans ; Male ; Megakaryocytes ; Nephritis ; Nephritis, Hereditary ; Proteinuria ; Rare Diseases ; Ristocetin ; Thrombocytopenia

No abstract available.
Mucocutaneous Lymph Node Syndrome

No abstract available.
Humans ; Infant, Newborn* ; Membranes* ; Rupture* ; Sepsis

No abstract available.
Animals ; Cerebrospinal Fluid Pressure* ; Cerebrospinal Fluid* ; Guinea Pigs* ; Guinea*

Objective: This study aimed to develop and verify an endometriosis self-assessment tool (ESAT). Methods: A non-experimental, descriptive, correlational study design was used. Candidate items were developed based on a conceptual framework constructed using the results of in-depth interviews and an integrative literature review. The construct validity of the developed tool was also examined. One-hundred and forty-two participants (117 patients with endometriosis and 25 patients without endometriosis) were included in the validity and reliability tests. The data were collected between August and December 2018. Nomological validity was verified based on significant correlations between the ESAT and the quality-of-life scores. Results: A 21-item ESAT was developed, and its construct validity was supported. Exploratory factor analysis indicated that the tool consisted of four components (gastrointestinal symptoms, dysmenorrhea, usual symptoms, and the amount and characteristics of menstrual bleeding) with a variance of 61.6%. The variance in quality-of-life scores, as explained by the ESAT scores, was relatively high. Receiver operator characteristics curve analysis indicated that ESAT scores significantly differentiated endometriosis from non-endometriosis with fair discriminatory power at a cut-off score of 50 (sensitivity, 0.76; specificity, 0.72; area under the curve, >0.75; P<0.001). This means that patients with ESAT scores >50 points were more likely to have endometriosis. Thus, the reliability of the ESAT was confirmed. Conclusion The devised tool appears valid and reliable. This tool may allow women to determine their risk of endometriosis by distinguishing between normal and pathological menstruation-related symptoms.

Endovascular aneurysm repair (EVAR) for the treatment of abdominal aortic aneurysm (AAA) is a widely used method, and its decreased invasiveness compared to traditional surgical repair has brought about reduced rates of morbidity and mortality. Several vascular complications related to the procedure have been reported, but non-vascular complications have rarely occurred. We report herein the case of a 78-year-old man who underwent EVAR for AAA and presented with active duodenal ulcer bleeding and acute acalculous cholecystitis as complications after the procedure. We must consider that a wide spectrum of complications may occur following EVAR, and therefore it is important to evaluate the risks of complication and to take the necessary measures to minimize them.
Acalculous Cholecystitis ; Aged ; Aneurysm ; Aortic Aneurysm, Abdominal ; Duodenal Ulcer ; Hemorrhage ; Humans

PURPOSE: Complete resection of the tumor nodule(mural nodule or solid portion of the tumor) is the essential goal of surgical treatment for heman-gioblastoma. The purpose of this study was to classify the morphologic types of intracranial hemangioblastoma on MRI and to compare the location and contour of tumor nodule on MRI with those on angiography. MATERIALS AND METHODS: The MRI findings of 34 lesions(38 lesions if 4 spinal cord lesions were included) in 26 patients(17 males and 9 females, range of age, 18-67 years, mean, 39 years) with surgically and histopathologically proved intracranial hemangioblastomas were reviewed. Seventeen patients underwent CT scanning in a short interval. Contrast-enahnced T1 -weighted imaging pa- tterns of hemangioblastoma were classified according to Ho's morphologic types. The location and contour of tumor nodule were compared between MRI and angiography in 15 patients(24 lesions). RESULTS: By location, cerebellar hemisphere was predominated(55%), followed by cerebellar vermis(26%), supratentorial region(5%), and medulla oblongata (3%). Spinal cord lesions(11%) were seen in 3 patients of 5 von HippeI-Lindau diseases. The frequency of morphologic types was as follows; Type 1 (purely cystic), 3%, Type 2(mural nodule), 50%, Type 3(cyst with wall enhancement), 3%, Type 4 (cystic nodule), 15%, Type 5(solid with internal cyst), 9%, and Type 6(solid), 20%. All tumor nodules(33 lesions) enhanced intensely with intravenous contrast material on MRI, of which 24 lesions(in 15 patients) revealed hypervascular masses fed by pial arteries on angiography. They were superficial and abutted pia mater partially or in large portion on both MR I and angiography. CONCLUSION: Over 70% of intracranial hemangioblastomas had a surrounding cyst, and superficial, pial-based location and number of the tumor nodules on MRI was correlated well with those on angiography. MRI is the examination of choice for preoperative evaluation of intracranial hemangioblastoma.
Angiography ; Arteries ; Female ; Hemangioblastoma* ; Humans ; Magnetic Resonance Imaging* ; Male ; Medulla Oblongata ; Pia Mater ; Spinal Cord ; Tomography, X-Ray Computed

A 29-year-old male patient was admitted due to subacute onset of right chest pain. He had no history of trauma, chest surgery or other medical disease. Chest roentgenogram showed an expansile, radiolucent lesion on the posterior segment of right seventh rib. Computed tomogram of the chest also showed lytic expansile mass with septation. He took an en-bloc resection of the involved rib. Pathologically there were multiple cystic spaces, filled with blood and collagenofibrous tissue proliferation and locally areas of new bone formation and giant cells of osteoclastic type, which was compatible with aneurysmal bone cyst. We report a case of aneurysmal bone cyst of the rib with a brief review of literature.
Adult ; Aneurysm ; Bone Cysts ; Chest Pain ; Giant Cells ; Humans ; Male ; Osteoclasts ; Osteogenesis ; Ribs* ; Thorax

Although crystalloid solutions such as Ringer's lactate have become the standard for initial volume replacement after hemorrhage, it is controversy whether this fluid alone restores and maintains the depressed physiologic function after severe hemorrhage. Six anesthetized dogs were bled(35ml/kg) and resuscitated with Ringers lactate solution (100 ml/kg). Physiologic variables are measured initialy, after hemorrhage, after resucitation. Compared with initial value, after resuscitation data show marked reduction in hemoglobin, oxygen carrying capacity, vascular resistance, and electrolyte. But cardiac output and blood gas analysis data was not changed. Prothrombin time and partial thromboplastin time was prolongated. From this study crystalloid resuscitation in severe hemorrhagic shock restore cardiac output and perfusion, but fail to maintain adequate oxygen carrying capacity.
Animals ; Blood Gas Analysis ; Cardiac Output ; Natural Resources ; Dogs* ; Hemorrhage ; Lactic Acid ; Oxygen ; Partial Thromboplastin Time ; Perfusion ; Prothrombin Time ; Resuscitation* ; Shock, Hemorrhagic* ; Vascular Resistance