Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0032285 (pneumonia)
 54,520 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The first Scandinavian cases of Zellweger syndrome (ZS) are described. A brother and sister, children of first cousins, had the typical clinical symptoms and pathological findings. Extensive metabolic studies in the boy were negative. Pipecolic acid in the urine was not elevated. Both children died at 14 weeks of age. Two months earlier the girl had suffered severe intestinal bleeding. Both had pneumocystic carinii pneumonia at autopsy although no evidence of immune deficiency had been found in the boy. The girl had used up her visible iron depots while the boy still had abundant but probably physiologic amounts of hemosiderin in the RES. Most of the cerebral abnormalities are unspecfic and possibly related to anoxia or other causes of delayed maturation. The white matter abnormalities in ZS patients may only be quantitatively different from the common "fatty metamorphosis" in infants. Previously reported ultrastructural abnormalities (absence of peroxisomes and very sparse smooth endoplasmic reticulum, as well as mitochondrial abnormalities) which are possibly unique for ZS, are confirmed. It is stressed that these were seen despite phenobarbital treatment which normally stimulates the formation of smooth endoplasmic reticulum.
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PMID:The Zellweger syndrome: subcellular pathology, neuropathology, and the demonstration of pneumocystis carinii pneumonitis in two siblings. 30 90

Fourteen colorectal cancer patients with metastatic liver metastases who received prophylactic intra-arterial infusion chemotherapy (IAIC) after curative resection for liver metastases at the National Cancer Center Hospital from May 1987 to December 1989 were reviewed. 5-fluorouracil (5-FU) (15 mg/kg) and mitomycin C (MMC) (0.1 mg/kg) were infused through implantable ports weekly or every two weeks. Five patients (35.7%) developed recurrent tumors in the residual liver during 3-12 months after hepatic resection. The se five patients had more than two metastatic lesions in the liver at hepatectomy. Four patients with solitary metastatic tumor had no recurrent liver diseases. The most serious complication was sclerosing cholangitis which occurred in two patients (14%) in this series. Both cases needed drainage PTCD, but one of them died from gastro-intestinal bleeding due to hepatic failure and the other from pneumonia. These two cases showed no recurrent liver cancers.
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PMID:[Results of prophylactic intra-arterial infusion chemotherapy after hepatic resection in colorectal metastases]. 211 8

We have studied 97 patients with dementia who have been discharged from our hospital and 106 inpatients with dementia who have been admitted during last two years in our hospital. The diagnosis of dementia was done according to the criteria of DSM-III. Based on their clinical course, neurological signs, Hachinski's ischemic score and neuroradiological findings, we divided patients into 4 groups, [senile dementia of the Alzheimer type (SDAT), vascular dementia (VD), unclassified dementia and other dementias which includes dementia with Parkinson's disease or motor neuron disease, etc.]. Concerning 70 demented patients who died during hospitalization, the average age of onset and the duration of illness of SDAT were 80.5 years old and 4.6 years respectively and those of VD were 77.6 years old and 2.7 years respectively. The common causes of death were pneumonia (50%) and cardiac failure (24%). Recurrence of cerebral vascular accident (CVA) was also another frequent cause of death in VD. The most common behavioral problems causing admission in patients of SDAT were aimless wandering, nocturnal delirium, illusion and hallucination. In VD, nocturnal delirium, aimless wandering, violence and abnormal monologue were most common causes of admission. The important causes degrading ADL of inpatients were fracture, especially fracture of the hip joint, pneumonia, intestinal bleeding and CVA. Concerning the increase of the population of over 75 years old, it will be suggested that the care and treatment of demented patients in this age group will become a major social problem.
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PMID:[Clinical and epidemiological studies on inpatients with dementia]. 238 92

We have reviewed all the records of 23 patients hospitalized in our institution from January 1981 till December 1991 and presenting a confirmed diagnosis of esophageal perforation. We have studied the aetiology, the localization, morbidity and mortality and we discuss the therapeutic management. 9 patients presented a cervical perforation, 13 patients a thoracic perforation and 1 patient an abdominal perforation. Among the patients with cervical perforation 2 patients had a local revision with drainage, 3 patients a primary suture and 4 patients were treated conservatively. No complication was found in this group. The patients with thoracic perforation have been treated as follow: 3 conservatively, 8 with thoracotomy, primary suture +/- patch, drainage, 2 patients with thoracotomy and drainage alone. All complications happened in this group: 2 gastro-intestinal bleeding, 2 ARDS, 3 mediastinitis, 1 pneumonia. 2 patients in a very poor general condition died, one with a metastatic breast carcinoma, the other after a CVI with a massive gastro-intestinal bleeding. The cervical perforations have an excellent prognosis and can be treated conservatively if they are asymptomatic and do not display a pleural lesion. The thoracic perforations can be treated surgically if they are diagnosed early before septic complications. If not, they will be better treated conservatively with drainage. The intraabdominal perforations have to be treated as every intraabdominal perforation. In this case, we perform a primary suture completed with fundoplication.
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PMID:[The value of conservative therapy, surgical treatment and interventional radiology in perforations and ruptures of the esophagus]. 822 36

Following the detection of cytomegalovirus antigen in mesangial cells of some patients with IgA nephropathy, an important role of human cytomegalovirus in the pathogenesis of IgA nephropathy has been discussed. We studied a case of IgA nephropathy with rapid deterioration of renal function associated with cytomegalovirus infection. Following an infection of the upper respiratory tract, a 57-year-old woman developed with hematuria and acute renal failure. The histological diagnosis of IgA nephropathy was established and renal function transiently improved during immunosuppressive therapy. However, the ensuing clinical course was complicated by severe bleeding from intestinal ulcera, thrombocytopenia, pneumonia and relapse of renal failure. The histological investigation of colonic mucosa showed characteristic "owl's eye" cells leading to the diagnosis of cytomegalovirus disease as the cause of intestinal bleeding. Immunosuppression was stopped and treatment with ganciclovir started. Pneumonia as well as intestinal bleeding disappeared and, of particular note, renal function improved considerably. Following discontinuation of antiviral therapy CMV-disease reoccurred and renal function deteriorated again. The patient was restarted on ganciclovir therapy and, again, serum creatinine fell quickly. This impressive and reproducible clinical improvement of renal insufficiency under antiviral therapy with ganciclovir provides some evidence for an important role of cytomegalovirus in the pathogenesis of this case of IgA nephropathy.
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PMID:Remission of IgA nephropathy following treatment of cytomegalovirus infection with ganciclovir. 969 35

In order to verify the safety of an ideal length of hospital stay (5-6 days) after open colectomy, we reviewed complications after 371 consecutive, elective colorectal resections for cancer at our institution between April 1991 and December 1998. Specifically, age of the patient, length of hospital stay and when the complication was diagnosed were registered. The median postoperative hospital stay was 9 days (range, 4-34 days). No difference in length of hospital stay was detected in patients < or = 65 years old versus > 65 years old (P = NS). All major complications (anastomotic leak, intestinal bleeding, intestinal occlusion, pneumonia, pulmonary embolism, pulmonary edema, stroke, angina pectoris, and fascial dehiscence) were diagnosed before the fifth postoperative day (P < 0.05). Among the minor complications (vomiting, packed red blood cells transfusion, diarrhea, wound infection, urinary tract infection, and pleural effusion), none requiring hospitalization was detected later then 5 days after the operation. We conclude that postoperative length of stay after colorectal resection for cancer can be reduced safely to five to six days after the operation.
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PMID:[The ideal length of hospital stay in the surgical treatment of colorectal cancer]. 1214 16

Rheumatological conditions can sometimes present as emergencies. These can occur due to the disease process or may be iatrogenic. Some of the important articular emergencies are septic arthritis, acute polyarthritis and atlanto-axial dislocation. Classical polyarteritis nodosa may present with massive gastro-intestinal bleeding, intestinal perforation or acute pancreatitis. Adult respiratory distress syndrome, bilateral pneumonitis and diffuse alveolar haemorrhage due to systemic lupus erythematosus or systemic necrotising vasculitis and ventilatory failure due to polymyositis are some of the respiratory emergencies. Scleroderma is well known to cause renal crisis which can be fatal if not diagnosed and managed promptly. Microscopic polyangiitis and Wegener's granulomatosis may cause rapidly progressive renal failure. Cerebrovascular accident, cortical vein thrombosis, seizures and acute psychosis are important neurological complications of rheumatic disease. Cardiac emergencies include tamponade, acute myocarditis and acute myocardial infarction. Vision can be threatened in Behcet's disease, temporal arteritis and seronegative spondylarthritis. Catastrophic antiphospholipid syndrome is a devastating emergency. The management of above emergencies includes critical care, immunosuppression when indicated and withdrawal of the offending drug. Anticoagulants have to be used in the management of antiphospholipid syndrome. A good understanding of these conditions is of paramount importance for proper management.
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PMID:Emergencies in rheumatology. 1516 86

A 12 y old girl was admitted 24 days after start a WHO multidrug therapy scheme for multibacillary leprosy (dapsone, clofazimine and rifampicin) with intense jaundice, generalized lymphadenopathy, hepatoesplenomegaly, oral erosions, conjunctivitis, morbiliform rash and edema of face, ankles and hands. The main laboratory data on admission included: hemoglobin, 8.4 g/dL; WBC, 15,710 cells/mm3; platelet count, 100,000 cells/mm3; INR = 1.49; increased serum levels of aspartate and alanine aminotransferases, gamma-glutamyl transpeptidase, alkaline phosphatase, direct and indirect bilirubin. Following, the clinical conditions had deteriorated, developing exfoliative dermatitis, shock, generalized edema, acute renal and hepatic failure, pancytopenia, intestinal bleeding, pneumonia, urinary tract infection and bacteremia, needing adrenergic drugs, replacement of fluids and blood product components, and antibiotics. Ten days after admission she started to improve, and was discharged to home at day 39th, after start new supervised treatment for leprosy with clofazimine and rifampicin, without adverse effects. This presentation fulfils the criteria for the diagnosis of dapsone hypersensitivity syndrome (fever, generalized lymphadenopathy, exfoliative rash, anemia and liver involvement with mixed hepatocellular and cholestatic features). Physicians, mainly in geographical areas with high prevalence rates of leprosy, should be aware to this severe, and probably not so rare, hypersensitivity reaction to dapsone.
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PMID:Dapsone hypersensitivity syndrome in an adolescent during treatment during of leprosy. 1565 79

We report a 49-year-old man who was a human T-cell leukemia virus type 1 (HTLV-1) carrier, born in Okinawa prefecture where both strongyloidiasis and HTLV-1 are endemic. He presented with fever, headache and urinary retention. On the basis of CSF examination and MRI findings, his condition was diagnosed as myelitis. He received methylprednisolone pulse therapy. He was transferred to our hospital due to severe paralytic ileus. Strongyloides stercoralis (S. stercoralis) was found in the duodenal stained tissue of a biopsy specimen. Ivermectin applied both orally and through enema were ineffective because of severe ileus and intestinal bleeding. Nine mg (200 microg/kg) of ivermectin solution was administered subcutaneously every other day for five days (total amount 45 mg). The S. stercoralis burden in the stool decreased and paralytic ileus gradually resolved. Three weeks after the resolution of S. stercoralis infection, purulent meningitis developed and acute obstructive hydrocephalus appeared. The hydrocephalus improved by ventricular drainage. Approximately three months after drainage, he died of incidental aspiratory pneumonia. Autopsy showed neither eggs nor larvae of S. stercoralis in the organs. In this case, the fourth reported case in the world, subcutaneous ivermectin injection was dramatically effective. We should consider a diagnosis of strongyloidiasis for any patient from Okinawa prefecture who was an HTLV-1 carrier presenting with unknown origin ileus after treatment of steroid therapy.
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PMID:[Fulminant strongyloidiasis successfully treated by subcutaneous ivermectin: an autopsy case]. 1838 29

We report multi-organ histopathological changes in a patient with protein-losing enteropathy (PLE) over 12 years after Fontan operation. A 14-year-old boy with right isomerism heart and single ventricle had undergone Fontan procedure at 19 months of age, and PLE was diagnosed at 28 months. He had several episodes of intestinal bleeding and pre-renal failure with elevated creatine, and eventually died of pneumonia. The intrapulmonary small arteries showed medial and intimal thickening resembling pulmonary hypertension. No major ulcerative lesions were found in the small or large intestines. Dilated lymph ducts, one of the characteristic features of PLE, were not seen in mucosal and submucosal areas. Liver cirrhosis was obvious despite little increase in liver enzymes. Histological changes in bilateral kidneys were subtle despite repeated episodes of renal failure. Thus, there may be significant discrepancies between clinical manifestations and multi-organ histological changes in failed Fontan patients.
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PMID:Diverse multi-organ histopathologic changes in a failed Fontan patient. 2761 84


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