1/12. enzyme therapy in gaucher disease type 2: an autopsy case.A Japanese patient with gaucher disease type 2 was treated with enzyme therapy, alglucerase, from 7 to 22 months of age. Whereas hematologic parameters were normalized and hepatosplenomegaly was alleviated, no improvement in neurologic symptoms occurred, and the patient died of respiratory failure at age 22 months. Postmortem examination revealed massive intra-alveolar infiltration of Gaucher cells in lungs and in the central nervous system, i.e., the presence of Gaucher cells in the perivascular Virchow-Robins spaces in the cortex and deep white matter and extensive lamilar necrosis with reactive proliferation of blood vessels and macrophage infiltration of the cerebral cortex. It is suggested that enzyme therapy, with thus far recommended dose, does not prevent long-term respiratory and central nervous system involvement in severe varients of Gaucher disease.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
2/12. Esophageal erosion as a possible bacterial entry site in an acute lymphoblastic leukemia patient with sepsis.A 69-year-old man with relapsed acute lymphoid leukemia was treated with adriamycin, vincristine, and prednisolone. During this chemotherapy, the patient developed sepsis and meningitis. Although many kinds of antimicrobial drugs, including imipenem, meropenem, amphotericin-B, and gamma-globulin were administered, the patient died of respiratory failure. A positive result for enterococcus faecalis was obtained in both blood and cerebrospinal fluid culture. autopsy revealed multiple small erosions in the lower esophagus. Histopathological examination showed multiple nuclear inclusion bodies of herpes simplex virus in the squamous epithelial cells at the edge of the erosions. Moreover, proliferation of micrococci was observed at the base of the erosions and in the lumina of the submucosal small vessels. These findings suggested that E faecalis entered the blood circulation from this lesion. In many patients with febrile neutropenia, the pathogenesis of infection remains unclear. Our case seems significant for clarifying the focus and pathogenesis of febrile neutropenia.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
3/12. How can we approach a left-sided stab wound in the neck, with isolated tracheal laceration?Any penetrating trauma to the mediastinum may cause great vessel damage resulting in massive bleeding or even shock. Associated tracheal injury increases morbidity and mortality. We report a case of a penetrating mediastinal injury with isolated tracheal laceration. The stab entered in the left supraclavicular fossa. The patient presented with right-sided pneumothorax, pneumomediastinum and respiratory collapse. bronchoscopy revealed two injuries in the trachea that were repaired by right thoracotomy and left cervicotomy. The patient was discharged on postoperative day 6. Isolated thoracic tracheal injury is very rare in mediastinal stab wounds and operative strategy may change according to bronchoscopic findings.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
4/12. Myelopathy due to spinal epidural abscess without cord compression: a diagnostic pitfall.Spinal epidural abscess (SEA) is a neurological emergency that requires urgent diagnosis and treatment. We report 2 patients with SEA, in whom, on neuropathological examination, the neurological signs were found to be caused by spinal cord ischemia due to thrombosis of leptomeningeal vessels and compression of spinal arteries, respectively, while evidence of spinal cord compression was absent. Clinicians and neuropathologists should be aware of the variable mechanisms underlying the neurological involvement in SEA. Absence of spinal cord compression by the abscess may hamper early diagnosis and treatment.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
5/12. Fulminant pulmonary calciphylaxis and metastatic calcification causing acute respiratory failure in a uremic patient.calciphylaxis is a rare and life-threatening disorder characterized by small-vessel mural calcification with intimal proliferation, fibrosis, and thrombosis, resulting in tissue ischemic necrosis. Although it has been viewed as a systemic disease involving mainly the dermis, subcutaneous fat, or muscle, calciphylaxis of other organs rarely is reported. We describe the case of a 25-year-old uremic woman who rapidly developed massive pulmonary calcification that led to acute respiratory failure after the initiation of hemodialysis therapy. Chest radiography and high-resolution computed tomography showed typical pulmonary calcification. Pulmonary calciphylaxis and metastatic calcification were confirmed further by lung tissue biopsy. No skin or muscle calciphylaxis was discovered. Despite multiple factors precipitating pulmonary calciphylaxis in this patient, we speculate that hemodialysis was the main culprit in accelerating the development of fulminant pulmonary calciphylaxis and metastatic calcification. Alteration in the local environment from an acid to an alkaline condition and a relatively high dialysate calcium level in the presence of systemic hyperphosphatemia are believed to have facilitated the deposition of calcification. This case highlights the importance of "visceral calciphylaxis" and early identification of its causes.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
6/12. Bronchopulmonary foregut malformation in association with diaphragmatic eventration.Bronchopulmonary foregut malformations are uncommon anomalies, and their association with diaphragmatic eventration is rare. This report presents 2 cases with this association. Embryologically, a simultaneous disturbance in the development of pulmonary vessels and developing esophagotracheal septum may explain the etiopathogenesis of this association. Abnormal budding along the developing foregut tube may lead to foregut cysts. In both cases, the bronchopulmonary foregut malformations were excised, and the diaphragm was repaired. The results of surgery were gratifying in both cases.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
7/12. Acute respiratory failure caused by pulmonary vasculitis after L-tryptophan ingestion.This report describes two women who presented with severe respiratory failure and diffuse pulmonary infiltrates in the fall of 1989. Both required prolonged assisted ventilation because of severe shunt physiology. Open lung biopsies on admission revealed a small vessel vasculitis as the sole morphologic abnormality in both patients. Both responded to high dose corticosteroids. Neither patient exhibited evidence of systemic vasculitis, and neither had serologic evidence of an immune disorder. Common to both patients was ingestion of L-tryptophan. One patient exhibited several features of the eosinophilia-myalgia syndrome. The other patient did not appear to have the syndrome, but the temporal relationship between the onset of symptoms and initiation of L-tryptophan treatment was striking. The presentation of these patients alters our notions concerning the spectrum of clinical manifestations caused by this agent, and the response to methylprednisolone supports its efficacy in the treatment of this disorder.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
8/12. Noninvasive assessment of cardiopulmonary function in critically ill infants and children.echocardiography and Doppler echocardiography allow for the accurate noninvasive assessment of cardiac structure and function. Two-dimensional echocardiography accurately demonstrates both normal and abnormal intracardiac and great vessel anatomy and assists in differentiating cardiac from noncardiac causes of respiratory distress. M-mode echocardiography allows for documentation of cardiac chamber size and left ventricular function. Doppler echocardiography is used to measure cardiac output, assess AV and semilunar valve function, detect abnormal flow patterns within the heart and great vessels, and assist in the assessment of pulmonary artery pressures. This article discusses the uses of all these modalities as they apply to the critically ill infant and child.- - - - - - - - - - ranking = 2keywords = vessel (Clic here for more details about this article) |
9/12. Acute respiratory failure and pulmonary arteritis without parenchymal involvement: demonstration in a patient with rheumatoid arthritis.A 28-year-old woman with a eight-year history of rheumatoid arthritis presented with a three-day history of dyspnea. Physical and electrocardiographic findings were consistent with pulmonary hypertension. Arterial blood gases revealed a ventilation-perfusion mismatch. Chest roentgenogram was normal. After transient improvement, she suddenly deteriorated and died. At autopsy, a necrotizing pulmonary panarteritis was found without parenchymal involvement by rheumatoid disease. The pulmonary arteries were the only vessels affected. Immunofluorescent staining revealed immunoproteins scattered throughout the vessel walls without localization to the basement membrane. The unique features of the case are discussed in relation to pulmonary hypertension and rheumatoid lung disease in which vascular lesions are usually associated with honeycomb lung. The association between the rheumatoid arthritis and pulmonary vasculitis was probably coincidental.- - - - - - - - - - ranking = 2keywords = vessel (Clic here for more details about this article) |
10/12. Clinicopathological conference: a newborn monozygotic twin with abnormal facial appearance and respiratory insufficiency.The second of twins males expired of respiratory insufficiency shortly after birth. Unusual facial appearance included prominent forehead, flat nasal bridge, widely separated inner canthi, downward slanting eyes with narrow palpebral fissures, epicanthic folds, small mouth and micrognathia, and apparently low-set ears; there was also cryptorchidism bilaterally and a simian crease bilaterally. The pregnancy of the 19-year-old black woman was not unusual, but polyhydramnios accompanied the delivery of this twin. The twin placentae were diamniotic and monochorionic, one being slightly immature with a two-vessel cord. We interpreted the karyotype as 46,XY,del(10) (p11-15): The brother and parents were normal. The dysmorphic features of this and three previously reported cases of 10p- do not permit definition of a syndrome. These are apparently the first monozygous twins presenting discordance of chromosomal structure; previously reported chromosomal discordance in monozygous twins involved numerical abnormality. Considering that the mechanism monozygous twinning is not understood, we note that the occurrence of both twinning and a structural aberration in one of the twins suggests the possibility of a common cause.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
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