Seasonal variations, acute rhinosinusitis and orbital infections in children.

BACKGROUND: Orbital infections in children are commonly secondary to acute bacterial rhinosinusitis (ABRS). It is unclear whether seasonal variations can predispose to these complications mirroring acute rhinosinusitis incidence. OBJECTIVE: To determine the incidence of ABRS as a cause of orbital infections and whether seasonality is a risk factor. METHODS: A retrospective review of all children who presented to West Virginia University children's hospital between 2012 and 2022 were reviewed. All children with CT evidence of orbital infection were included. Date of occurrence, age, gender, and presence of sinusitis were reviewed. Children with orbital infection secondary to tumors, trauma, or surgery were excluded. RESULTS: 118 patients were identified with mean age of 7.3 years with 65 (55.1 %) males. 66 (55.9 %) children had concomitant sinusitis on CT scan, and the distribution of orbital complications per season showed 37 (31.4 %) cases occurred in the winter season, followed by 42 (35.6 %) cases in spring, 24 (20.3 %) cases in summer, and 15 (12.7 %) in fall. Children with orbital infections during winter & spring had sinusitis in 62 % of children vs. 33 % in other seasons (P = 0.02). Preseptal cellulitis was present in 79 (67 %) children, 39 (33 %) children with orbital cellulitis, and 40 (33.9 %) children with abscesses. 77.6 % children were treated with IV antibiotics and 94 % with oral antibiotics, and 14 (11.9 %) with systemic steroids. Only 18 (15.3 %) children required surgery. CONCLUSIONS: There seems to be a seasonal predisposition for orbital complications mainly in the winter and spring seasons. Rhinosinusitis was present in 55.6 % of children presenting with orbital infections.

Association between adaptive behaviour and age in adults with Down syndrome without dementia: examining the range and severity of adaptive behaviour problems.

BACKGROUND: Studies on adaptive behaviour and ageing in adults with Down syndrome (DS) (without dementia) have typically analysed age-related change in terms of the total item scores on questionnaires. This research extends the literature by investigating whether the age-related changes in adaptive abilities could be differentially attributed to changes in the number or severity (intensity) of behavioural questionnaire items endorsed. METHODS: The Adaptive Behaviour Assessment System-II Adult (ABAS-II Adult) was completed by parents and caregivers of 53 adults with DS aged between 16 and 56 years. Twenty adults with DS and their parents/caregivers were a part of a longitudinal study, which provided two time points of data. In addition 33 adults with DS and their parents/caregivers from a cross-sectional study were included. Random effects regression analyses were used to examine the patterns in item scores associated with ageing. RESULTS: Increasing age was found to be significantly associated with lower adaptive behaviour abilities for all the adaptive behaviour composite scores, expect for the practical composite. These associations were entirely related to fewer ABAS-II Adult items being selected as present for the older participants, as opposed to the scores being attributable to lower item severity. CONCLUSIONS: This study provides evidence for a differential pattern of age-related change for various adaptive behaviour skills in terms of range, but not severity. Possible reasons for this pattern will be discussed. Overall, these findings suggest that adults with DS may benefit from additional support in terms of their social and conceptual abilities as they age.

A phase II study of menogaril (7R-O-methylnogarol) in patients with relapsed/refractory acute myeloid leukemia: a study of the Eastern Cooperative Oncology Group.

Fifty-one patients (47 evaluable) with AML, 27 in first relapse and 20 either in second relapse or refractory were treated with menogaril, 100 mg/m2/day as a 90-min infusion daily for 5 days. The complete response (CR) rate was 17% (8/47), and there was one partial response. Seven of eight responders were in first relapse with a 26% response rate among first relapse patients (7/27). The median duration of survival was 3 months for all first relapse patients and 4.3 months for all other patients. Toxicity included grades 3-4 pancytopenia and fever (100% of patients) and grades 3-4 stomatitis and hepatic enzyme elevation (25% of patients). Grades 3-4 cardiac toxicity occurred in three patients (two grade 3 arrhythmias and one heart block). All had previously received anthracyclines. Remission duration was 1.6-48+ months; two patients underwent bone marrow transplantation and continue in CR at 36+ and 48+ months. The nontransplanted patients remained in CR 1.6, 2.0, 3, 7, 14 and 27 months. Activity and toxicity of menogaril in this study were comparable to that of other clinically useful anthracyclines in AML. Further investigation of this agent in AML is warranted.

Plasmapheresis. A therapeutic option in the management of heparin-associated thrombocytopenia with thrombosis.

Heparin-associated thrombocytopenia with thrombosis (HATT) is an uncommon syndrome that is estimated to occur in 1-5% of patients with heparin-induced thrombocytopenia. Early diagnosis requires careful clinical surveillance, and the management of these patients can be complex. Cessation of heparin therapy and substitution or addition of oral anticoagulants, antiplatelet agents, dextrans, and prostacyclin analogues have been advocated. The authors are aware of only two case reports in the literature that examine the use of plasmapheresis as a therapeutic alternative. The authors report a case of a 53-year-old white man who developed HATT after a single protamine-reversed exposure to heparin. Controlled platelet aggregation studies performed before and after apheresis sessions documented a dramatic response and rapid normalization of platelet number and function in the patient. The authors conclude that plasmapheresis could be a valuable adjunct in the successful management of patients with HATT. When done in conjunction with platelet aggregation studies, an objective measurement of therapeutic efficacy can be achieved.

Potential markers of the atherosclerotic process in high-risk children.

Children whose parents had early coronary heart disease were investigated. In order to assess high-risk parameters serum total cholesterol (TC), total triglyceride (TT), high-density lipoprotein cholesterol (HDLC), low-density lipoprotein cholesterol (LDLC), lipid peroxide (LP), prostacyclin (PGI2), thromboxane (TX) levels, and the distribution of the complement 3 (C3), protein phenotypes were measured. Compared to a group of control children, the offspring of high-risk subjects had increased LDLC, LP, TC, and TX levels, a higher incidence of fast-slow heterozygotes, and decreased HDLC and PGI2 levels. The measurement of serum PGI2, TX levels and the distribution of C3 protein phenotypes may give further information about the true risk of atherosclerosis.

Low level of prostacyclin as a risk of ischaemic heart disease.

We have studied the relationship between levels of lipids and prostacyclin in 3- to 14-year-old children with a family history of ischaemic heart disease. The total levels of cholesterol in the serum of these children increased, while levels of the HDL-fraction and prostacyclin (measured as 6-keto-prostaglandin F1a) decreased significantly. Levels of thromboxane B2 in the plasma were unchanged. It is suggested that, in childhood, abnormalities of lipid metabolism co-exist with decreased levels of prostacyclin. The latter may be a new important indicator of early atherosclerotic disease.

Balance of prostacyclin and thromboxane in offspring of parents with premature coronary arterial disease.

6-keto-prostaglandin F1a and thromboxane B2 were determined in order to obtain more information about the prostacyclin synthesis and thromboxane A2 release in 3- to 18-year-old healthy children and in offspring of parents who have had an acute myocardial infarction before the age of 45. The authors demonstrated a reduction of plasma prostacyclin synthesis in children with a positive family history of premature coronary arterial disease. Thromboxane levels in the affected adolescent boys were significantly lower compared with the controls. The ratio of thromboxane:prostacyclin in endangered children did not show a significant difference from that of healthy controls. These data indicate that prostaglandins are a definitive marker for identifying cardiovascular risk children. It must be supposed that in adolescence, only in boys, with a positive family history of premature coronary arterial disease, a compensatory mechanism exists to protect them from developing an imbalance in the regulation of prostaglandins.

Inhibition of ACAT activity after 7-oxo-PGI2 treatment in cholesterol-fed rabbits.

It has been supposed that prostacyclin (PGI2) and its analogues have important antiatherogenic effects. The aim of this work was to test the effect of PGI2 and 7-oxo-PGI2- (a stable analogue of PGI2) (6) treatment on the acyl CoA: cholesterol-acyltransferase (ACAT) activity in the aortic wall of rabbits. The rabbits had been previously fed with cholesterol and treated with PGI2 and 7-oxo-PGI2 intravenously. Cholesterol feeding increased ACAT activity compared to the control group which was not fed with cholesterol: 16.84 nmol/mg prot./h and 10.03 nmol/mg prot./h, respectively. PGI2 treatment of the cholesterol fed group did not cause a significant decrease, while 7-oxo-PGI2 treatment significantly decreased aortic ACAT activity compared to the cholesterol-fed control group; 14.31 nmol/mg prot./h; 11.53 nmol/mg prot./h and 16.84 nmol/mg prot./h, respectively. The decrease found in the ACAT activity after PGI2 and 7-oxo-PGI2 treatment are new data for the protective effect of these agents against atherosclerosis.

[Plasma prostacyclin and thromboxane concentration in healthy children and in offspring of parents suffering from myocardial infarct]. / Plazma prosztaciklin és tromboxán koncentráció egészséges gyermekeken és fiatalkori myocardialis infarctusban megbetegedett szülök utódaiban.

6-keto-prostaglandin F1a and thromboxane B2 were determined by radioimmunoassay in 135 healthy children, as a control group, and in 125 offsprings of parents suffering from premature coronary artery disease. Plasma prostacyclin concentration had decreased in children with a positive family history of coronary artery disease. It was demonstrated a strong reduction of thromboxane level in boys from age 11 in endangered group while the thromboxane/prostacyclin ratio did not differ from the control. It was supposed that in adolescent sons of parents who have had an acute myocardial infarction before the age of 45, a compensatory mechanism exists to protect them from disturbances in regulation of the balance between prostacyclin and thromboxane.

Chronic lymphocytic leukemia (CLL) terminating in multiple myeloma: report of two cases.

Two cases of multiple myeloma (MM) developed late in the course of chronic lymphocytic leukemia (CLL). An 81-yr-old white female developed, after 6 yr of CLL, IgAk MM with sheets of plasma cells abutting sheets of lymphocytes in the bone marrow, multiple pathologic fractures, and 0.26 g/24 free k light chains in the urine. A 74-yr-old white male developed, after 16 yr of CLL, k light chain MM with 20% plasma cells in the bone marrow, multiple panthologic fractures, and 3.7 g/24 hr free k light chains in the urine. In both cases the CLL had responded well to intermittent low-dose chlorambucil therapy, but the MM failed to respond to cyclic melphalanprednisone therapy. A review of 105 cases of CLL seen at the Geisinger Medical Center failed to turn up any other cases of MM developing during the course of CLL. The suggestion that there is an increased prevalence of MM in CLL is an attractive one because both diseases are B cell neoplasms and because of the increased frequency of asymptomatic monoclonal gammopathies in CLL found by others.

The activated partial thromboplastin time as a monitor of heparin therapy: a warning.

The degree of correlation between the whole blood clotting time (WBCT) and the activated partial thromboplastin time (APTT) was evaluated in 42 patients receiving intermittent intravenous heparin therapy. Correlation between the two tests was found to be poor (correlation coefficient 0.52), primarily for reasons related to characteristics of the APTT system employed. The relationship in vitro between the APTT and the level of heparin was evaluated and found to vary widely over a range of heparin concentrations. This study emphasizes the marked variability between various APTT systems and the dangers involved with substituting the APTT for the WBCT to monitor heparin without appropriate initial evaluation.

Decreased platelet aggregation during pregnancy correlates with gestational age.

In vivo platelet aggregation was determined in pregnant women at different gestational ages and nonpregnant women by a modification of a method. A higher platelet count ratio (PCR) was found in pregnant women after a gestational age of 13 weeks. At 16-30 and 31-41 weeks of gestation, in vivo platelet aggregation was significantly decreased. The PCR, meaning the ratio of non-aggregated platelets to all circulating platelets, correlated significantly with gestational age. It is suggested that the decrease in in vivo aggregation measured during pregnancy reflects a summarized effect of different factors during pregnancy connected to placenta and uteroplacental vessels.

Association between total cholesterol and thromboxane B2 levels in offspring of parents suffering from premature coronary artery disease.

Relationship between plasma thromboxane B2 concentration and serum total cholesterol level was studied in 129 healthy 3 to 18 years old children, 77 girls and 52 boys, without any family history of premature coronary artery disease and in 181 offspring, 105 girls and 76 boys, of parents suffering from acute myocardial infarction before the age of 45. It was identified an enhancement in serum total cholesterol level of endangered children, and an elevated release of thromboxane A2 in affected girls. A significant negative correlation was found between serum total cholesterol concentration and plasma thromboxane B2 level in healthy girls. However, there was no correlation between serum total cholesterol level and plasma thromboxane B2 concentration in the children whose parents had premature coronary artery disease. It appears, from our results, that this in an alteration of thromboxane A2 release of platelets in children of families with high risk of cardiovascular diseases.

Association between total cholesterol and plasma thromboxane B2 in human adults.

Apparently healthy adults were examined for their lipid and thromboxane parameters. The correlation between the levels of total cholesterol in serum and thromboxane B2/TXB2/ in plasma was strong mainly in men (p less than 0.01). However, the association between atherogenic index /AI/ and TXB2 levels tended to be higher in women. The authors suggest that, in healthy adults, elevated cholesterol levels coexist with abnormalities in thromboxane metabolism.

Prostacyclin and thromboxane levels of children of parents suffering from early ischemic heart disease.

Offsprings of parents who had acute myocardial infarction before age of 45 years were investigated. The aim of this examination was to obtain information whether the variation in the balance of prostacyclin/thromboxane ratio is a common cardiovascular risk factor in children. In children whose parents have had early myocardial infarction, a significant decrease was shown in 6-keto-prostaglandin F1 alpha level while the thromboxane B2/6-keto-prostaglandin F1 alpha ratio increased in these children. Plasma tromboxane B2 levels hardly differed from those of the control in that group of children whose one parent and at least one of the grandparents or uncles or aunts suffered from coronary heart disease. Plasma thromboxane concentration was lower in another group of children whose "only" one parent had myocardial infarction. It may be supposed that this is a compensatory mechanism in the offspring of parents suffering from early coronary heart disease.

Chronic neutrophilic leukemia evolving from a myelodysplastic syndrome.

Chronic neutrophilic leukemia (CNL) is a rare hematologic disorder usually presenting with a persistent neutrophilia in the leukemoid range (WBC > 40-50 x 10(9)/1) and consisting largely of mature neutrophils. Patients have no obvious cause for an elevated white count and typically have an elevated leukocyte alkaline phosphatase score, hepatosplenomegaly, elevated vitamin B12 and are Philadelphia chromosome-negative. CNL has occasionally been associated with paraproteinemia or outright myeloma. Dysplastic features within the neutrophils in CNL have rarely been reported. We report the clinical, pathological and cytogenetic features of a case of CNL in an elderly white female initially diagnosed with refractory anemia with excess blasts, which subsequently progressed to CNL.