Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0476273 (respiratory distress)
 19,632 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report a 64-year-old Japanese woman who died one year after the onset of progressive gait disturbance and dementia. She noted a difficulty in holding a glass and hand tremor in June of 1996 when she was 63 years old. In July of 1996, she tended to lean toward left when she walked. She also noted truncal titubation. In November of 1996, she started to have visual hallucination and delusion in which she said "I see something is flying on the wall.", "Somebody has come into my room", and things like that. She was admitted to our service on November 22, 1996. On admission, she was alert and general physical examination was unremarkable. Neurologic examination revealed disturbance in recent memory. Hasegawa's dementia rating scale was 22/30. She showed vivid visual hallucination with colors in which she saw faces of dwarfs and angels, a space ship, and others. Higher cerebral functions were normal. She showed left oculomotor palsy which was a sequel of an aneurysm and subarachnoid hemorrhage nine years before. Otherwise cranial nerves were unremarkable. She showed ataxic gait, limb ataxia, truncal titubation, and postural hand tremor. She had no weakness and no muscle atrophy. Deep tendon reflexes were within normal limits. Plantar response was flexor. Sensation was intact. Laboratory examination was also unremarkable. Complete survey for occult malignancy was negative. CSF was under a normal pressure and cell count was 1/microliter, total protein 27 mg/dl, and sugar 68 mg/dl. Cranial CT scan was unremarkable. MRI was not obtained because of the presence of an aneurysm clip in the left internal carotid-posterior communication artery junction. She showed progressive deterioration in her mental function. By January 1997, she became unable to stand or walk with marked dementia. Repeated CSF exams and cranial CT scans were unremarkable. She suffered from several episodes of aspiration pneumonia. A trial of three days methylprednisolone pulse therapy was given starting on March 7, 1997, which was of no effect on her neurologic status. On March 28, 1997, she was intubated because of acute respiratory distress syndrome. In April 2, her body temperature rose to 38 degrees C. On April 9, 1997, her blood pressure dropped and resuscitation was unsuccessful. She was pronounced dead on the same day. The patient was discussed in a neurologic CPC and the chief discussant arrived at the conclusion that the patient had primary leptomeningeal lymphoma. Other possibilities entertained among the audience included brain stem encephalitis of unknown type, carcinomatous cerebellar degeneration plus limbic encephalitis, Creutzfeldt-Jakob disease, thalamic degeneration, and progressive multifocal leukoencephalopathy. Post-mortem examination revealed thickening and clouding of the leptomeninges; Gram-positive diplococci were found in the leptomeninges. This meningitis appeared to have been an complication in the terminal stage of her illness. Microscopic examination revealed astrocytosis in the midbrain tegmentum. Cerebral cortices showed only mild astrtocytosis. No cerebellar atrophy was seen and Purkinje cells were retained which excluded paraneoplastic cerebellar degeneration. Neuropathologic diagnosis was bacterial meningitis, however, the presence of brain stem encephalitis prior to the onset of bacterial meningitis could not be excluded. It is interesting to note that the diagnosis of the primary neurologic disease of this patient was not easy even after autopsy. As autopsy permission was obtained only for the brain, it was not clear whether or not this patient had an occult malignancy somewhere in her body, however, there was no evidence to indicate paraneoplastic degeneration of the central nervous system. As the patient did not have meningeal signs until one month before her death, it is difficult to ascribe her entire neurologic problems to her meningitis. Finally, her visual hallucination was vivid and colorful; we thought this might have been
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PMID:[A 64-year-old woman with progressive gait disturbance and dementia for one year]. 978 11

A 3-month-old infant treated for 3 weeks for suspected bronchiolitis, developed episodes of profound desaturation. A lateral X-ray showed displacement and compression of the trachea. Respiratory arrest, from which she was successfully resuscitated, occurred just before MRI scan. The mass was removed at thoracotomy and a histological diagnosis of a bronchogenic cyst was made. Mediastinal masses in babies are relatively rare, and the situation in which they present with acute respiratory distress may prove extremely challenging to the anaesthetist. Bronchogenic cysts are difficult to diagnose pre-operatively and awareness may assist in the peri-operative management of these infants.
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PMID:A bronchogenic cyst in an infant causing tracheal occlusion and cardiac arrest. 1036 63

As many as 10% to 20% of patients with disseminated hemangiomas involving vital organs fail to respond to conventional treatment with steroids, radiotherapy, laser or cyclophosphamide. For the last years, interferon-alpha-2 has been successfully used to treat complicated giant hemangiomas, because of its ability to inhibit endothelial cell proliferation. We report the case of a 3-months-old infant presenting with a giant cervicofacial hemangioma with extension to the chest wall and larynx, causing episodes of severe respiratory distress. CT scan and MRI revealed a second vascular lesion measuring 24 x 16 mm in the liver. Initial treatment with corticosteroids (prednisolone at a dose of 1-3 mg/kg/daily) and endoscopic laser barely improved the symptoms and the patient presented more episodes of shortness of breath requiring admission to the ICU. Interferon therapy was started at a dose of 3 million units/m2/every other day and was continued for 9 months. At the completion of the therapy, there was complete regression of the hemangioma. Four months later, the patient is asymptomatic. No side-effects were presented for hyperthermia when the treatment was started resolved with antithermics. Blood tests were always within the normal range.
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PMID:[Interferon treatment of giant hemangioma]. 1057 Aug 61

Although 3D MRA has been shown to provide excellent depiction of the pulmonary arterial tree, its clinical use has been limited due to lengthy breath-holding requirements. Employing the newest gradient generation (1.5 T MR system, amplitude of 40 mT/m and a slew rate of 200 mT/m/msec), we evaluated a technique permitting the dynamic acquisition of 3D data sets of the entire pulmonary tree in under 4 seconds. Coronal image sets were collected using a repetition time of 1.64 msec and an echo time of 0.6 msec, resulting in an acquisition time of 3.74 seconds. Three volunteers and eight dyspneic patients with known or suspected pulmonary embolism underwent MRI of the pulmonary arteries. The pulmonary arterial tree was visible to a subsegmental level in all examined subjects. Regarding the presence of pulmonary emboli in four patients, there was complete concordance between MR angiographic findings and those of corroborative studies. We conclude that diagnostic MRA of the pulmonary vasculature can be obtained even in patients with severe respiratory distress.
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PMID:Dynamic 3D MR angiography of the pulmonary arteries in under four seconds. 1124 9

Congenital midline nasal masses are rare entities occurring once in 20-40,000 births. They are often misdiagnosed or even missed. These tumors are most commonly present in newborn infants and children, but rarely they can be discovered in adults as well. The differential diagnostic possibilities are dermoids, gliomas, encephaloceles and epidermoid cysts. Although they are benign, they have the potential for disfigurement, destruction and causing a meningitis, therefore they require prompt diagnosis and management. Because of a possible connection to intracranial structures a complete radiologic evaluation is essential. The treatment for these nasal masses is surgical resection, endoscopically controlled procedures are preferred. We report the case of a newborn with respiratory distress shortly after birth. The infant was discharged from the hospital, because the unexplained symptoms were not progressive. After a few days the child was send to an otolaryngologist, who found a suspicious mass near the top of the nose and performed a biopsy. At 20 days of age the infant was brought to our children's hospital for additional diagnostic procedures and therapy. The MRI showed a soft tissue mass (1.2 x 0.7 x 1.1 cm) in the nasal cavity. There was no clear-cut evidence of an intracranial extension, for further evaluation a radionuclide scan was performed. This confirmed the integrity of the base of the skull. 6 weeks after the birth a smooth tumor was successfully removed by an endoscopically controlled operation. Histopathologic studies confirmed neuroglial tissue. Because of ist rare incidence, many physicians are not familiar with the diagnosis and management of these tumors. Respiratory abnormalities in newborn should always lead to a careful physical examination and complete radiologic evaluation, i.e. MRI, CT and radionuclide scan.
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PMID:[Nasal glioma as a rare cause of obstructed nasal breathing in a newborn infant]. 1141 67

An MRI method is described for demonstrating improved oxygenation of human tumors and normal tissues during carbogen inhalation (95% O2, 5% CO2). T2*-weighted gradient-echo imaging was performed before, during, and after carbogen breathing in 47 tumor patients and 13 male volunteers. Analysis of artifacts and signal intensity was performed. Thirty-six successful tumor examinations were obtained. Twenty showed significant whole-tumor signal increases (mean 21.0%, range 6.5-82.4%), and one decreased (-26.5 +/- 8.0%). Patterns of signal change were heterogeneous in responding tumors. Five of 13 normal prostate glands (four volunteers and nine patients with nonprostatic tumors) showed significant enhancement (mean 11.4%, range 8.4-14.0%). An increase in brain signal was seen in 11 of 13 assessable patients (mean 8.0 +/- 3.7%, range 5.0-11.7%). T2*-weighted tumor MRI during carbogen breathing is possible in humans. High failure rates occurred due to respiratory distress. Significant enhancement was seen in 56%, suggesting improved tissue oxygenation and blood flow, which could identify these patients as more likely to benefit from carbogen radiosensitization.
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PMID:BOLD MRI of human tumor oxygenation during carbogen breathing. 1147 74

We propose a haphazard branching theory to support the concept of bronchopulmonary malinosculations, and we apply this theory to classify congenital bronchopulmonary vascular malformation (BPVM) based on the anatomical results we have found. Between January 1990 and December 1997, a total of 22 pediatric patients (10 male and 12 female), aged 2 days to 14 years (median, 19.6 months), with congenital BPVM were enrolled in this retrospective study. Study modalities include the clinical features and plain chest films (n = 22) plus at least two of the following: echocardiography (n = 13), barium esophagraphy (n = 2), bronchoscopy (n = 4), contrast bronchography (n = 8), high-resolution direct coronal CT (n = 1) and electron beam or ultrafast CT (n = 1) of the chest, MRI (n = 10), MRA (n = 1), contrast cineangiocardiography (n = 9), surgery (n = 11), or autopsy (n = 2). The salient clinical features were recurrent lung infections in 14 patients, acute respiratory distress in 13, associated cardiovascular malformations in 8, dextroversion in 7, congestive heart failure in 7, dextrocardia in 4, and complex congenital heart diseases in 4. There were abnormal openings (malinosculations) of the pulmonary airway in 20 patients: to an artery in 12, to a vein in 8, and to the lung parenchyma in 9. These 22 patients with congenital BPVM can be classified into bronchial malinosculation (10 cases), arterial malinosculation (2 cases), and bronchoarterial malinosculation (10 cases). Congenital BPVM can be classified in terms of bronchopulmonary malinosculation based upon a haphazard branching theory, in which abnormal communications between two independent systems (primitive foregut system and aortic-pulmonary arch system) occurred coincidentally rather than causally.
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PMID:Revisit on congenital bronchopulmonary vascular malformations: a haphazard branching theory of malinosculations and its clinical classification and implication. 1174 54

We report an infant with intermittent urinary excretion of D-2-hydroxyglutaric (D-2-OHG) acid who died at the age of 10 months from cardiogenic shock due to cardiomyopathy. High urinary concentrations of D-2-OHG and succinic acid, as well as increased levels of lactic acid were detected on three different occasions, whereas a normal urinary profile of organic acids was found on one occasion. The clinical findings of our patient consisted of generalized hypotonia, irritability, developmental delay, generalized tonic seizures, lethargy, cardiomyopathy, and respiratory distress. Cerebral MRI revealed bilateral lesions in the substantia nigra, the periaqueductal area, the medial part of the thalamus, the hypothalamus, the caudate nucleus, putamen and globus pallidus. This pattern is suggestive of a mitochondriopathy. However, respiratory chain enzyme activities were normal in fibroblasts. Exogenous supplementation of D-2-OHG acid strongly inhibited cytochrome-c oxidase activity in fibroblasts from the patient and from normal controls in vitro. The results suggest that our patient has an unusual form of D-2-hydroxyglutaric aciduria (D-2-OHGA), different from the patients published so far, and that the increase of lactic acid and some citric acid cycle intermediates encountered in some patients with D-2-OHGA may be due to a functional defect of the respiratory chain caused by D-2-OHG acid.
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PMID:D-2-Hydroxyglutaric aciduria in a patient with a severe clinical phenotype and unusual MRI findings. 1199 77

We report a 71-year-old man with intravascular malignant lymphomatosis who showed high serum LDH and urinary disturbance for one year before manifesting dementia. High serum LDH was found at a health check at age 70. Two months later, he had an onset of backache and urinary retention. MRI of the spinal cord was unremarkable. One year later, he showed decline of mental activities and was admitted to our hospital. He was agitated and confused. However cranial nerve palsy or limb weakness was not noted. The MRI of the brain showed T2-high signal in bilateral occipital, right temporal lobe and the left insular cortices. The abdominal CT scan showed swelling of the adrenals on both sides. Adrenal biopsy revealed diffuse large B cell lymphoma. He developed respiratory distress and he died two months after the admission. Post mortem examination revealed intravascular and extravascular proliferation of lymphoma cells in most of the internal organs including adrenals, spleen, liver and the kidneys. In the brain, the laminar necrosis was seen in the left occipital cortex and hemorrhagic infarctions were noted in the insular and temporal cortices and the medial temporal cortex. Sacral spinal cord showed necrosis of the gray matters and loss of myelinated fibers in the white matter. Intravascular proliferation of the lymphoma cells were also seen in the vessels of the brain and the spinal cord. This patient suggests the importance of survey for intravascular malignant lymphomatosis, when high serum LDH and myelopathy of lumbosacral area are seen.
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PMID:[A patient with intravascular malignant lymphomatosis presenting subacute dementia one year after sustained urinary retention and high serum LDH activity from the onset]. 1208 Jun 10

Airway invasion is a life-threatening complication of thyroid cancer. An important issue that deserves better attention is the differentiation between the clinical features of tracheal wall invasion versus those of an obstructive endotracheal lesion. We present information on the clinical course, diagnostic modalities utilized, management instituted, along with the prognosis, and follow-up data on a group of patients presenting with obstructive endotracheal lesions of thyroid cancer. Two thousand four hundred and eighty-nine thyroid cancer patients were seen at our institution from December 1975 to May 2000. Thirteen patients presented with symptoms of respiratory distress related to obstructive endotracheal lesions. At presentation, 11 patients underwent endoscopic examination. Imaging studies consisting of I123 whole body scan (WBS), computed tomography/magnetic resonance imaging (CT/MRI) of neck and chest, whole body positron emission tomography using 18-fluoro-2-deoxy-D-glucose ((FDG)PET) were done, as also was determination of the tumour markers, serum thyroglobulin (TG) and calcitonin. Patients were followed for one to 108 months after the initial presentation. Intraluminal tracheal obstruction was severe in eight patients; five had near-total-occlusion. Paralysis of the vocal folds was present in five. Evidence of metastatic disease was present in most patients. Dissociation between iodine uptake and TG synthesis was evident in five patients during follow-up. Four patients died of cancer. Of the nine living patients; cancer persisted in six, recurred in two patients, and remitted in one. This study has identified obstructive endotracheal lesion of thyroid cancer as a distinct entity apart from tracheal wall disease. These data provide evidence that intraluminal tracheal invasion of thyroid cancer is an ominous sign and a frequent cause of morbidity.
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PMID:Obstructive endotracheal lesions of thyroid cancer. 1238 90


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