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

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

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

OBJECTIVE: The LiquoGuard® system is a new ventricular-type monitoring device that facilitates intracranial pressure (ICP)-controlled or volume-controlled drainage of cerebrospinal fluid (CSF). The purpose of this study is to report the authors' experience with the LiquoGuard® ICP monitoring system, as well as the clinical safety, usefulness, and limitations of this device in the management of patients with traumatic brain injury (TBI). METHODS: Intraventricular ICP monitoring was performed on 10 patients with TBI using the LiquoGuard® monitoring system. ICP measurements, volume of drained CSF, and clinical outcomes were analyzed and discussed. RESULTS: ICP monitoring was performed on 10 patients for a mean duration of 6.9 days. With a mean 82,718 records per patient, the mean initial ICP was 16.4 mm Hg and the average ICP across the total duration of monitoring was 15.5 mm Hg. The mean volume of drained CSF was 29.2 cc/day, with no CSF drained in 4 patients. Seven of 10 patients showed 1 or 2 episodes of abnormal ICP measurements. No patient exhibited complications associated with ICP monitoring. CONCLUSION: The LiquoGuard® system is a versatile tool in the management of TBI patients. Its use is both reliable and feasible for ICP monitoring and therapeutic drainage of CSF. However, episodes of abnormal ICP measurements were frequently observed in patients with slit ventricles, and further study may be needed to overcome this issue.
Brain Injuries* ; Cerebrospinal Fluid ; Drainage ; Humans ; Injections, Intraventricular ; Intracranial Pressure* ; Monitoring, Physiologic