Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027947 (neutropenia)
 17,527 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Twenty-eight patients with defective neutrophil mobility were classified, largely on results of parent values, into primary (17)--mainly Shwachman's syndrome--and probable secondary (11). They presented with frequent infections and/or allergy and these symptoms were essentially similar in both groups. Neutropenia was virtually confined. to the patients with Shwachman's syndrome. Diagnoses associated with secondary neutrophil mobility defects included hydrocephalus with shunt, cystic fibrosis, and immunoglobulin deficiency. The neutrophil mobility of nearly nearly all the patients studied increased when levamisole was added in vitro, but there was no clear evidence of clinical benefit when patients were given the drug.
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PMID:Clinicopathological findings in patients with primary and secondary defects of neutrophil mobility. 70 97

The clinical activity of piperacillin was evaluated in 34 children (mean age: 8 years) presenting with severe infection (septicaemia, meningitis, bronchopneumonia, pyelonephritis). A bacteriological diagnosis was established in 24 cases. The mean duration of treatment was 11 days, and the mean dose 220 mg/kg/day administered in three injections. In 25 cases piperacillin was combined with another antibiotic, usually an aminoglycoside (20 cases). Clinical cure or improvement was obtained in 29 children (85%). Treatment was well tolerated, with only 2 cases of moderate blood eosinophilia. In view of these results the authors suggest that piperacillin could be used in children in two circumstances: severe infections caused by Gram-negative cocci or bacilli in children with cystic fibrosis or neutropenia, and against infections contracted in intensive care units, or in children with febrile leucopenia, combined with an aminoglycoside in the absence of, or pending bacteriological results.
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PMID:[Indications for piperacillin in pediatrics]. 294 80

A 3 1/2 year old girl presented with failure to thrive and a five month history of diarrhoea and recurrent cough. The results of sweat sodium tests suggested a diagnosis of cystic fibrosis; but atypical organisms were found (Haemophilus influenzae, Candida albicans, but no Staphylococcus aureus), she failed to respond to treatment, and her sweat sodium concentrations fell in response to fludrocortisone. She also had hyperglobulinaemia, neutropenia, and reduced numbers of T4 lymphocytes, which prompted the performance of a test for antibody to human immunodeficiency virus (HIV). This proved positive, and she was treated with co-trimoxazole, zidovudine, and human immunoglobulin. Both parents and two siblings were also positive for HIV, though all had normal sweat sodium concentrations. Children with symptoms suggestive of cystic fibrosis but who also show atypical features, as in this case, should have their HIV state checked.
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PMID:Abnormal sweat electrolytes in symptomatic human immunodeficiency virus infection in a child. 312 Oct 56

Shwachman's syndrome is, after cystic fibrosis, the most common cause of exocrine pancreatic insufficiency in childhood. The cause of the disorder is not known but we were struck by the fact that the histological appearances of pancreatic atrophy in this condition resemble those seen in experimental copper deficiency, in which the pancreatic acinar damage persists long after the copper deficiency is relieved. Other features of Shwachman's syndrome include neutropenia, anaemia and abnormalities of the ribs and of the metaphyses of long bones. All these findings have also been reported in children with copper deficiency during the 1st year of life. We suggest that some or all cases of Shwachman's syndrome are caused by a period of copper deficiency in early infancy.
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PMID:Hypothesis: Shwachman's syndrome of exocrine pancreatic insufficiency may be caused by neonatal copper deficiency. 319 Jan 62

The efficacy of ceftazidime in the treatment of infections in compromised children was evaluated in 80 such episodes occurring in 64 patients with various underlying diseases. Among the patients treated, 9 were newborns with severe neonatal distress, 21 were children with cancer and neutropenia, 8 were surgical patients, 22 had cystic fibrosis and 4 were suffering from meningitis. The following types of infections were treated: 19 bacteriologically documented and 8 possible septicemias (the latter only in newborns and neutropenic cancer patients); 2 severe upper respiratory tract infections in cancer patients; 8 soft tissue or skin infections; 1 cholangitis; 1 pneumonia; 1 osteomyelitis; 1 mediastinitis; 35 infectious exacerbations of underlying pulmonary disease in cystic fibrosis patients; and 4 meningitides. In almost all cases ceftazidime was administered intravenously in combination with an aminoglycoside. In 2 cases it was also given intrathecally or intraventricularly. Bacteriological documentation was achieved in 70 out of 80 episodes. A successful outcome was obtained in 79% of the cases with slight and statistically nonsignificant differences between groups of patients with different etiological patterns in terms of prevalence of gram-positive microorganisms. Tolerance of the treatment was uniformly good, only one patient showing a mild, transient transaminase elevation.
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PMID:Evaluation of ceftazidime in the treatment of 80 infectious episodes in compromised children. 390 33

Six common clinical situations in infants and children are discussed from the point of view of standard therapeutic regimens: neonatal sepsis and meningitis; febrile episodes in neutropenia; bacterial meningitis; acute pulmonary exacerbations of cystic fibrosis; pneumonia, bone and joint infections, and cellulitis in patients less than four years of age; and intra-abdominal sepsis. Potential or actual problems with these therapeutic regimens and newer therapeutic options are outlined.
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PMID:Current needs for new beta-lactam antibiotics in pediatrics. 407 87

Pseudomonas aeruginosa infection is unusual in individuals with human immunodeficiency virus infection, and it most often occurs in the setting of other risk factors, such as neutropenia or cytotoxic drug use. We noted an increasing number of pulmonary isolates of this organism in our clinic population and sought to describe the clinical correlates of this finding. Our study consisted of a retrospective review of the microbiology, radiology, and clinical records of 1,852 HIV-seropositive adults seen at a university-based outpatient AIDS clinic. We identified 16 individuals with Pseudomonas bronchopulmonary infection. All subjects had advanced HIV disease with prior AIDS diagnoses, and mean CD4 counts of 25/mm3 (0.025 x 10(9)/L). Pseudomonas was the sole pulmonary pathogen in 14 of 16 patients and was associated with new chest X-ray abnormalities in 14 cases. Four individuals had acute pseudomonal pneumonia with sepsis; this presentation was associated with hospitalization and other known risk factors for Pseudomonas infection. In contrast, 12 patients had more indolent, community-acquired infection, which had a low mortality rate and occurred in the absence of other risk factors. Survivors of the initial bout of Pseudomonas infection had an 86% relapse rate despite a median survival of only 4.5 months. This pattern of pseudomonal disease is reminiscent of cystic fibrosis and suggests a role for maintenance therapy.
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PMID:Pseudomonas aeruginosa bronchopulmonary infection in late human immunodeficiency virus disease. 821 56

Ciprofloxacin is a broad spectrum fluoroquinolone antibacterial agent. Since its introduction in the 1980s, most Gram-negative bacteria have remained highly susceptible to this agent in vitro; Gram-positive bacteria are generally susceptible or moderately susceptible. Ciprofloxacin attains therapeutic concentrations in most tissues and body fluids. The results of clinical trials with ciprofloxacin have confirmed its clinical efficacy and low potential for adverse effects. Ciprofloxacin is effective in the treatment of a wide variety of infections, particularly those caused by Gram-negative pathogens. These include complicated urinary tract infections, sexually transmitted diseases (gonorrhoea and chancroid), skin and bone infections, gastrointestinal infections caused by multiresistant organisms, lower respiratory tract infections (including those in patients with cystic fibrosis), febrile neutropenia (combined with an agent which possesses good activity against Gram-positive bacteria), intra-abdominal infections (combined with an antianaerobic agent) and malignant external otitis. Ciprofloxacin should not be considered a first-line empirical therapy for respiratory tract infections if penicillin-susceptible Streptococcus pneumoniae is the primary pathogen; however, it is an appropriate treatment option in patients with mixed infections (where S. pneumoniae may or may not be present) or in patients with predisposing factors for Gram-negative infections. Clinically important drug interactions involving ciprofloxacin are well documented and avoidable with conscientious prescribing. Recommended dosage adjustments in patients with impaired renal function vary between countries; major adjustments are not required until the estimated creatinine clearance is < 30 ml/min/1.73m2 (or when the serum creatinine level is > or = 2 mg/dl). Ciprofloxacin is one of the few broad spectrum antibacterials available in both intravenous and oral formulations. In this respect, it offers the potential for cost savings with sequential intravenous and oral therapy in appropriately selected patients and may allow early discharge from hospital in some instances. In conclusion, ciprofloxacin has retained its excellent activity against most Gram-negative bacteria, and fulfilled its potential as an important antibacterial drug in the treatment of a wide range of infections. Rational prescribing will help to ensure the continued clinical usefulness of this valuable antimicrobial drug.
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PMID:Ciprofloxacin. An updated review of its pharmacology, therapeutic efficacy and tolerability. 873 21

Shwachman's syndrome is a rare disease characterized by the association of chronic diarrhea due to exocrine pancreatic insufficiency, metaphyseal dysostosis, and neutropenia. The diagnosis requires demonstration of lipomatosis, or fatty replacement of the pancreas, which is the typical pathological feature of the disease. Magnetic resonance imaging (MRI) was performed in 13 patients with exocrine pancreatic insufficiency, 7 with Shwachman's syndrome, 2 with Pearson's syndrome, 1 with normal sweat test later diagnosed as cystic fibrosis, and 3 without identified syndrome, and in 7 control children. Ultrasonography in the patients did not differentiate between atrophy and lipomatosis and could not be performed in 3. MRI visualized the pancreas in all. The same image was noted in all patients with Shwachman's syndrome with a normal-sized or enlarged pancreas, a hyperintense signal on T1- and T2-weighted image, and a null signal on short time inversion recovery (STIR)-weighted image, characteristic of fat. In all other patients, the findings were very different: The pancreas was a small structure surrounded with fat. In 1 patient without identified syndrome, the pancreas appeared to be partially replaced with fat. MRI is an excellent imaging technique to correlate the nature of a tissue and its radiological representation, especially fat, which gives a very typical signal. In our brief series of patients with Shwachman's syndrome, MRI had 100% positive predictive value in demonstrating lipomatosis. In atypical cases of pancreatic insufficiency in which some of the clinical features of Shwachman's syndrome are absent. MRI is an invaluable aid in the diagnostic procedure.
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PMID:Magnetic resonance imaging for diagnosis of Shwachman's syndrome. 898 52

Pseudomonas aeruginosa is an ubiquitous environmental bacterium. It can be recovered, often in high numbers, in common food, especially vegetables. Moreover, it can be recovered in low numbers in drinking water. A small percentage of clones of P. aeruginosa possesses the required number of virulence factors to cause infection. However, P. aeruginosa will not proliferate on normal tissue but requires previously organs. Further narrowing the risk to human health is that only certain specific hosts are at risk, including patients with profound neutropenia, cystic fibrosis, severe burns, and those subject to foreign device installation. Other than these very well-defined groups, the general population is refractory to infection with P. aeruginosa. Because of its ubiquitous nature, it is not only not practical to eliminate P. aeruginosa from our food and drinking water, but attempts to do so would produce disinfection byproducts more hazardous than the species itself. Moreover, because there is no readily available sensitive and specific means to detect and identify P. aeruginosa available in the field, any potential regulation governing its control would not have a defined laboratory test measure of outcome. Accordingly, attempts to regulate P. aeruginosa in drinking water would not yield public health protection benefits and could, in fact, be counterproductive in this regard.
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PMID:Pseudomonas aeruginosa: assessment of risk from drinking water. 909 14


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