Pathologic quiz case: deep venous thrombosis in a 21-year-old woman with a retroperitoneal mass. Pathologic diagnosis: malignant peripheral nerve sheath tumor.
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Adult↗
Biomedical subjects
S Islam
Publications and source records attributed to S Islam.
Pathologic quiz case. Systemic mastocytosis.
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Adult↗
Tracheal ligation increases mitogen-activated protein kinase activity and attenuates surfactant protein B mRNA in fetal sheep lungs.
BACKGROUND: Tracheal ligation has been shown to accelerate fetal pulmonary growth in normal and hypoplastic lungs. Our aim was to study the effects of tracheal ligation on established molecular markers of growth and differentiation [mitogen-activated protein (MAP) kinase] and maturity [surfactant protein B (SPB) and fatty acid synthase (FAS)]. MATERIALS AND METHODS: Tracheal ligation was performed on four 100-day-gestation fetal sheep, with four age-matched fetuses undergoing maternal laparotomy and hysterotomy as control. Lungs from surviving fetuses (n = 2 in each group) were harvested after 4 days and frozen in liquid nitrogen. Protein lysates were prepared, and MAP kinase enzymatic assays [extracellular signal regulated protein kinase (ERK)-1 and -2] and Western blots were performed. Total RNA was isolated, and a fetal sheep lung cDNA library was created. The sheep SPB and FAS genes were cloned and sequenced. Northern blots were performed with the new clones, normalizing to beta-actin. RESULTS: Tracheal ligation lungs contained a larger volume of fluid (40 ml) compared with age-matched controls (8 ml). MAP kinase enzymatic ERK-1 activity was increased and SPB mRNA expression was reduced in fetal lungs after tracheal ligation. Neither ERK-2 enzymatic activities and FAS mRNA nor ERK protein levels were affected by tracheal ligation, by Western blot analysis. CONCLUSION: Tracheal ligation-induced fetal lung growth may be mediated in part via the MAP kinase pathway. Expression of SPB mRNA is attenuated by tracheal ligation, whereas FAS, one of the key enzymes that synthesizes the lipid portion of surfactant, is not affected.
Animals↗
Evolution of long-range and short-range order in La0.67Ca0.33MnO3-delta during solid-state transformation using high-energy ball milling.
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Journal Article↗
Association of apolipoprotein A phenotypes and oxidized low-density lipoprotein immune complexes in children.
BACKGROUND: Small apolipoprotein A (apo [A]) phenotypes and oxidized low-density lipoprotein immune complexes (oxLDL-ICs) are known to be associated with the development of atherosclerosis in adults. Presence of these factors in children and their relationships with other known cardiovascular risk factors have not been well documented. OBJECTIVE: To examine the relationship of oxLDL-ICs with apo(A) phenotypes and other known cardiovascular risk factors in children. DESIGN: A survey of asymptomatic 9- to 11-year-old children, randomly selected from a cohort of children stratified based on family history of premature coronary artery disease. SETTING: A preventive medicine research institute. PARTICIPANTS: Thirty-five children with or without a family history of premature coronary artery disease who are participating in a longitudinal cardiovascular health study. MAIN OUTCOME MEASURES: The influence of apo(A) phenotypes on plasma levels of oxLDL-ICs after controlling for lipid/lipoprotein levels, percentage of body fat, and physical fitness. RESULTS: Oxidized low-density lipoprotein immune complexes were significantly correlated with the levels of total cholesterol (r = 0.56, P< or =.05), low-density lipoprotein cholesterol (r = 0.64, P< or =.01), and low-density lipoprotein cholesterol/high-density lipoprotein cholesterol (r = 0.54, P<.05). Oxidized low-density lipoprotein immune complexes were also correlated with total cholesterol high-density lipoprotein cholesterol (r = 0.49, P< or =.06) and percentage of body fat (r = 0.48, P< or =.06). However, they achieved only a borderline level of statistical significance after adjustment for multiple comparisons. Multiple regression analysis demonstrated that small apo(A) phenotypes, levels of low-density lipoprotein cholesterol, and family history of premature coronary artery disease explained 54% of the variation of oxLDL-ICs using a parsimonious model (P = .001). CONCLUSIONS: Significant correlations exist between oxLDL-ICs and known cardiovascular risk factors in children. The association of oxLDL-ICs with the genetically controlled small apo(A) phenotype suggests that the genetic predisposition to immune complex formation may be an important determinant of future coronary artery disease.
Adult↗
Extracorporeal membrane oxygenation for nonneonatal acute respiratory failure.
HYPOTHESIS: Extracorporeal membrane oxygenation (ECMO) is effective in nonneonatal acute respiratory failure under certain circumstances. DESIGN: Retrospective medical record review. SETTING: The intensive care unit of a tertiary care hospital. PATIENTS: Thirty-four nonneonatal patients (mean age, 22 years; range, 8 days to 56 years), with ratios of the PaO2 to the fraction of inspired oxygen persistently below 70, who were treated with ECMO after maximal ventilator therapy had failed (mean time of ventilator therapy, 6.9 days; range, 1-41 days). The mean ECMO duration was 304 hours (range, 56-934 hours). Patients were grouped into 7 categories based on their diagnosis: sepsis or sepsis syndrome (n = 3), bacterial or fungal pneumonia (n = 10), viral pneumonia (n = 5), trauma or burn (n = 2), inhalation injury without burn (n = 1), immunocompromised state (due to transplantation or chemotherapy) (n = 8), and acute respiratory failure of unknown origin (n = 5). MAIN OUTCOME MEASURE: Survival to hospital discharge following ECMO therapy. RESULTS: Overall survival was 53% (18 patients). All 6 patients (100%) with viral pneumonias or isolated inhalation injuries survived. Of 13 patients with bacterial pneumonia, sepsis, or sepsis syndrome not complicated by multiorgan failure, 10 (77%) survived. In contrast, all but 1 of the immunocompromised patients died. Survival in patients who were intubated for less than 9 days before ECMO was 64%, whereas survival fell precipitously to 22% for patients who experienced mechanical ventilation for 9 or more days before the implementation of ECMO. Finally, the proportion of patients who died while receiving ECMO therapy was greater when the ECMO duration exceeded 300 hours (62% vs. 38%; P<.05). CONCLUSIONS: Nonneonatal survival with ECMO therapy is strongly dependent on the diagnosis. Pre-ECMO intubation for less than 9 days had little effect on survival. Survival rates decreased when the length of time of receiving ECMO exceeded 300 hours.
Acute Disease↗
Prenatal vitamin E treatment improves lung growth in fetal rats with congenital diaphragmatic hernia.
PURPOSE: Congenital diaphragmatic hernia (CDH) is associated with pulmonary hypoplasia. To discover factors that would accelerate fetal lung growth, the authors developed models of hypoplasia, found that antioxidants improved lung growth in vitro, and then proceeded to in vivo studies. METHODS: Timed-pregnant rats were fed nitrofen (100 mg) on gestational day 9.5 (term, 22), and fetal lungs were harvested at day 13.5 and placed in organ culture in serum-free media with (n = 10) or without (n = 9) additional vitamin E (0.134 IU/mL). Camera lucida tracings were made daily on live, unstained lungs for 4 days, scanned, digitized, and analyzed for multiple growth parameters. Similar nitrofen-exposed rats were fed an optimized total dose of 150 IU vitamin E (n = 19) or olive oil (n = 13) from days 16.5 to 20.5, and fetal lungs were harvested at day 21.5, weighed and fixed for histology, or homogenized and biochemically analyzed. RESULTS: Vitamin E accelerated hypoplastic fetal lung growth in vitro as measured by area, perimeter, lung bud count, perimeter over square root area, and fractal dimension. In vivo vitamin E significantly increased lung weights, total DNA, and protein contents. CONCLUSIONS: Vitamin E accelerates hypoplastic fetal rat lung growth and complexity in vitro, and prenatal vitamin E treatment in vivo improves pulmonary hypoplasia in fetal rats with CDH.
Animals↗
Diltiazem reduces pulmonary arterial pressures in recurrent pulmonary hypertension associated with pulmonary hypoplasia.
BACKGROUND/PURPOSE: Recurrent pulmonary hypertension in the neonatal population is an unusual event with dire consequences. Pulmonary hypertension seen in association with pulmonary hypoplasia may be refractory to conventional medical management. The effect of the calcium channel antagonist diltiazem was studied in five patients with severe pulmonary hypertension. METHODS: A retrospective review of the hospital records was performed to determine the efficacy of diltiazem for refractory pulmonary hypertension. All five patients experienced and did not respond to maximal conventional therapy, which included inhaled nitric oxide, intravenous nitrates, and extracorporeal membrane oxygenation (ECMO). Right ventricular pressures were determined by transthoracic echocardiograms and were used to document improvement in the pressure gradients. Statistical analyses were performed using a paired Student's ttest. A P value of less than .05 was considered significant. RESULTS: Diltiazem significantly reduced the right ventricular systolic pressure (RVSP) from 82 +/- 8.4 mm Hg to 58.4 +/- 7 mm Hg (P = .008). Two patients died; one had a large ventricular septal defect, and the other suffered multisystem organ failure secondary to sepsis. The surviving patients were weaned off diltiazem and did not experience recurrent pulmonary hypertension. CONCLUSIONS: In cases of pulmonary hypoplasia with recurrent pulmonary hypertension, diltiazem may be considered as a therapy. A multicenter prospective trial is advocated.
Blood Pressure↗
Experience with living donor kidney transplantation in Bangladesh.
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Adult↗
Immunocytochemical and in situ hybridization studies of the expression and distribution of three subunits of a complex with N-methyl-D-aspartate receptor-like properties.
A group of four proteins with recognition sites for L-glutamate, N-methyl-D-aspartate, glycine, and competitive and non-competitive inhibitors of N-methyl-D-aspartate receptors was previously purified from rat brain synaptic membranes. The biochemical and immunochemical characteristics of this complex, as well as the sequences of the complementary DNAs of three subunits, are distinct from those of other glutamate receptors, transporters, or enzymes. The function of this complex has not yet been defined, but it appears to be involved in glutamate-induced neuronal excitation and toxicity. It is not known whether all protein components of the complex are expressed in the same populations of brain cells. In the present study, immunohistochemical and in situ hybridization were used to map the distribution of the glutamate-binding, glycine/thienylcyclohexylpiperidine-binding, and carboxypiperazinyl-propylphosphonate-binding protein subunits of the complex. These proteins were abundantly expressed in pyramidal neurons of the hippocampus and cerebral cortex, and in granule cells of the dentate gyrus, cerebellum, and olfactory tubercle. Based on these results, it was concluded that the three subunits of the complex have similar patterns of expression in rat brain. The distribution of one subunit of the complex, glutamate-binding protein, was traced throughout the rat brain, thus providing a potential map of the expression of the complex in rodent brain. In addition, probes were developed in the present study that should be useful in future explorations of the role of these proteins in brain function and of the possible co-localization of the protein subunits in single cells or cell processes.
Animals↗
Case report: a rare association of primary biliary cirrhosis and systemic lupus erythematosus and review of the literature.
We report a rare occurrence of systemic lupus erythematosus in a patient known to have had well-documented primary biliary cirrhosis for 10 years. The presentation was dramatic with pericardial tamponade, but responded well to high dose corticosteroid. There are only five such definite associations reported in the literature. In the present case, other possible causes were considered, such as drug-induced cholestasis, drug-induced lupus, autoimmune chronic active hepatitis and the overlap syndrome.
Biopsy↗
Family functioning, neurocognitive functioning, and behavior problems in children with sickle cell disease.
OBJECTIVE: To investigate the independent and combined contributions of neurocognitive and family functioning to mother-reported behavior problems in children with sickle cell disease (SCD) and evaluate the factor structure of the Family Environment Scale (FES) with African American families. METHOD: The study sample included 289 children enrolled in the multisite Cooperative Study of Sickle Cell Disease. The study protocol included neuropsychological evaluation and brain magnetic resonance imaging (MRI) of the children, and mothers completed the Child Behavior Checklist and Family Environment Scale. RESULTS: With child and maternal demographic parameters controlled, conflicted family functioning, but not neurocognitive functioning, accounted for a significant portion of the variance in mother-reported behavior problems. The factor structure of the FES for families of children with SCD was found to be similar to that for other families. CONCLUSIONS: Family functioning may be a salient target for fostering adaptation to chronic childhood illness.
Adaptation, Psychological↗
Division of responsibility for asthma management tasks between caregivers and children in the inner city.
This investigation examined caregiver and child perceptions of the division of responsibility for asthma management tasks in families. The study sample included 789 children with asthma, aged 6 to 9 years, who lived in the inner city. These children and their primary caregivers completed the Asthma Responsibility Interview. The correlation between the caregiver's and child's ratings of the child's responsibility was low (.19), with children rating themselves as more responsible than their caregivers rated them. Caregiver and child ratings of the child's responsibility increased with the child's age; however, caregivers' ratings of their own responsibility remained constant over the age range studied. Kappa statistics ranged from -.03 to .12, with up to 16% of children reporting less responsibility for self-care than was indicated by the caregiver. More than one third of families reported four or more asthma caregivers. The discrepancy between the caregiver's and child's perceptions and the involvement of multiple caregivers raise the possibility of unintentional nonadherence.
Asthma↗
Right bundle branch block as a cause of false-negative ECG classification of inferior myocardial infarction.
It is generally accepted in clinical electrocardiography that a right bundle branch block (RBBB) does not interfere with the electrocardiographic (ECG) diagnosis of myocardial infarction (MI). The basic assumption is that the initial excitation wavefronts are relatively unchanged in RBBB. This study compared serial changes in Q wave duration in inferior leads II, III, and aVF in 9 patients who developed RBBB within 3 weeks after myocardial revascularization procedure (RBBB group) and in 41 revascularized patients without RBBB in the same observation period (control group). Q wave durations in the electrocardiograms obtained before the patients' procedures were not significantly different between the study and control groups. However, Q wave durations shortened significantly more in the RBBB group than in the control group. The most pronounced Q wave duration shortening took place in lead aVF, -18.2 ms in the RBBB group versus -3.8 ms in the control group (P = .0001). The shortening was less pronounced, although significant, in leads II and III: II, -7.6 +/- -10.9 ms in the RBBB group vs -2.3 +/- -3.5 ms in the control group (P = .01); III, -11.3 +/- -10.5 ms vs -2.6 +/- -6.5 ms (P = .002); aVF, -18.2 +/- -13.5 ms vs -3.8 +/- -5.3 ms (P < .0001). It is concluded that incident RBBB complicating revascularization procedures may cause significant alterations in spatial orientation of the initial excitation wavefronts. This may be a potential source of false-negative ECG diagnosis of inferior MI, particularly in clinical trials where serial ECG analysis is an important part in MI classification.
Bundle-Branch Block↗
Crystallization of an intact GST-estrogen receptor hormone binding domain fusion protein.
Crystals of an intact GST-estrogen receptor hormone binding domain fusion protein have been grown from solutions of MPD. The crystals grew as clusters of thin plates and needles of maximum dimensions 100 x 20 x 1 micrometer but were unsuitable for X-ray diffraction analysis. However, examination by electron microscopy shows an ordered lattice in which the protein molecules are clearly visible. Image analysis of electron micrographs of the protein crystals revealed electron stain-excluding density which showed a two-domain trimeric structure in projection, with each molecule of dimensions 12.0 x 5.0 nm diameter. The use of GST-fusion proteins in crystallisation are discussed.
Crystallization↗
Intra-abdominal hypertension after life-threatening penetrating abdominal trauma: prophylaxis, incidence, and clinical relevance to gastric mucosal pH and abdominal compartment syndrome.
OBJECTIVE: To define the incidence, prophylaxis, and treatment of intra-abdominal hypertension (IAH) and its relevance to gut mucosal pH (pHi), multiorgan dysfunction syndrome, and the abdominal compartment syndrome (ACS). METHODS: Seventy patients in the SICU at a Level I trauma center (1992-1996) with life threatening penetrating abdominal trauma had intra-abdominal pressure estimated by bladder pressure. pHi was measured by gastric tonometry every 4 to 6 hours. IAH (intra-abdominal pressure> 25 cm of H2O) was treated by bedside or operating room laparotomy. RESULTS: Injury severity was comparable between patients who had mesh closure as prophylaxis for IAH (n = 45) and those who had fascial suture (n = 25). IAH was seen in 10 (22.2%) in the mesh group versus 13 (52%) in the fascial suture group (p = 0.012) for an overall incidence of 32.9%. Forty-two patients had pHi monitoring, and 11 of them had IAH. Of the 11 patients, eight patients (72.7%) had acidotic pHi (7.10 +/- 0.2) with IAH without exhibiting the classic signs of ACS. The pHi improved after abdominal decompression in six and none developed ACS. Only two patients with IAH and low pHi went on to develop ACS, despite abdominal decompression. Multiorgan dysfunction syndrome points and death were less in patients without IAH than those with IAH and in patients who had mesh closure. CONCLUSIONS: IAH is frequent after major abdominal trauma. It may cause gut mucosal acidosis at lower bladder pressures, long before the onset of clinical ACS. Uncorrected, it may lead to splanchnic hypoperfusion, ACS, distant organ failure, and death. Prophylactic mesh closure of the abdomen may facilitate the prevention and bedside treatment of IAH and reduce these complications.
Abdomen↗
Weight-independent cardiovascular fitness and coronary risk factors.
BACKGROUND: It is unclear whether cardiovascular fitness influences risk factors for coronary artery disease and non-insulin-dependent diabetes mellitus independently of level of body fat because fitness is often measured in tasks that involve moving body weight (eg, running) and because body weight and level of body fat are correlated. OBJECTIVE: To measure fitness during a task in which body weight was controlled experimentally (ie, supine cycling). DESIGN: Cross-sectional. PARTICIPANTS: Seventy-four children, 7 to 13 years of age, recruited through school flyers and newspaper advertisements, varying in level of body fat from 7% to 61%. There were 31 boys and 43 girls; 35 were white and 39 were black. MAIN INDEPENDENT VARIABLES: Cardiovascular fitness was expressed as submaximal heart rate while cycling at a power output of 49 W, and level of body fat was measured with dual x-ray absorptiometry. MAIN OUTCOME MEASURES: Blood pressure and levels of triglycerides, total cholesterol, high-density lipoprotein cholesterol, low-density lipoprotein cholesterol, insulin, glucose, and glycohemoglobin. RESULTS: Controlling for age and sex, level of body fat was significantly (P < .01) related to unfavorable levels of systolic blood pressure (r = 0.32), triglycerides (r = 0.42), high-density lipoprotein cholesterol (r = -0.31), insulin (r = 0.50), and total cholesterol to high-density lipoprotein cholesterol ratio (r = 0.37); submaximal heart rate was not significantly correlated with any of the other variables. CONCLUSIONS: When cardiovascular fitness was measured in a weight-independent task, it was not significantly related to level of body fat or the major coronary artery disease and non-insulin-dependent diabetes mellitus risk factors. In addition, we confirmed previous findings that higher levels of body fat are associated with a very unfavorable risk profile.
Adipose Tissue↗
Glutamine is superior to glucose in stimulating water and electrolyte absorption across rabbit ileum.
L-Glutamine is the primary metabolic fuel of the intestinal mucosa. This in vivo study compares the effect of L-glutamine 50 mM with that of D-glucose 50 mM on water and electrolyte absorption in jejunal and ileal loops of healthy rabbits. Using polyethylene glycol (PEG) as a nonabsorbable marker and an incubation at 37 degrees C, we found that absorption of water (P = 0.000), sodium (P = 0.002), potassium (P = 0.001), and chloride (P = 0.003) from the glutamine electrolyte solution was greater than from the glucose electrolyte solution in the ileum. A similar trend was shown in the jejunum. We conclude that L-glutamine may be a useful component to be tested in oral rehydration solutions for treating diarrheal dehydration.
Animals↗