Clinical and biochemical spectrum of metabolic cardiomyopathy in Egyptian children

BackgroundInborn errors of metabolism (IEMs) commonly present with pediatric cardiomyopathy. Identification of the underlying cause is necessary as it may lead to improved outcomes.ObjectivesWe aimed to investigate the diagnostic rate, the clinical, and biochemical spectra of IEMs among Egyptian pediatric patients presenting with cardiomyopathy, and their outcome measures.MethodsWe retrospectively analyzed the clinical, biochemical, and radiological data of 1512 children diagnosed with cardiomyopathy at Cairo University Children''s Hospital over a 5-year duration.ResultsTwo hundred twenty-nine children were clinically suspected as IEMs and underwent metabolic workup. Nineteen different IEMs were confirmed in 57 (24.4%) of the suspected children. Their median age at presentation was 2.6 years and the majority had extra-cardiac manifestations. Hypertrophic cardiomyopathy represented 43/57 (75.4%) of confirmed cases, while dilated cardiomyopathy represented 13/57 (22.8%), and one patient presented with a mixed phenotype. Twenty- six patients (45.6%) survived, while 31 patients (54%) either died or were lost to follow up and assumed deceased.ConclusionsWe developed for the first time a database and a diagnostic scheme for metabolic cardiomyopathies in Egyptian children. With the recent introduction of enzyme replacement therapy, many metabolic disorders became treatable, thus establishing an early and accurate diagnosis is extremely important.     

Bleeding, Obstruction, and Perforation in a Series of Patients With Aggressive Gastric Lymphoma Treated With Primary Chemotherapy

Background The management of patients with gastric lymphoma has evolved, with a shift toward nonsurgical treatment. The rates of surgical complications in patients receiving chemotherapy have been insufficiently studied. The objective of this study was to assess the frequency of bleeding, perforation, and gastric outlet obstruction in patients who received chemotherapy as primary treatment for gastric diffuse large B cell lymphoma (DLBCL).Methods We reviewed files of all patients with gastric DLBCL who were diagnosed and treated primarily with chemotherapy in our hospital between 1990 and 2005.Results Eighteen (25%) of 73 patients experienced surgical complications, of whom 6 (8%) underwent surgery. Eight patients (11%), six with active lymphoma, experienced gastric bleeding; one required gastrectomy. Eight patients (11%) developed gastric outlet obstruction, of whom three were treated conservatively, three required surgery, one stopped treatment, and one received further chemotherapy. Six of the eight patients had no evidence of active lymphoma at the time of obstruction. Two additional patients underwent gastrectomy due to resistant or relapsed disease. Gastric perforation was not observed. Median survival was 90 months for the entire series, 94 months for patients with gastric outlet obstruction, and 11.5 months for patients with gastric bleeding.Conclusions Given the rate of surgical complications, especially gastric bleeding and gastric outlet obstruction, there is still an important role for the surgical consultant in the treatment of patients with gastric DLBCL receiving chemotherapy. Gastric perforation, although frequently cited as a complication, is in fact rarely observed.     

Preinvasive and invasive cervical cancer: an ex vivo proton magic angle spinning magnetic resonance spectroscopy study

The aim of this study was to obtain (1)H MR spectra using magic angle spinning (MAS) techniques from punch biopsies (<20 mg) of preinvasive and invasive cervical disease and to correlate the spectral profiles with sample classification on the basis of histopathology. Tissue samples were obtained at colposcopic examination, during local treatment of cervical intraepithelial neoplasia (CIN) or at hysterectomy. (1)H MAS MRS was performed at 25 degrees C while spinning the sample at 4.5 kHz. After measurement, the tissue was immersed in formalin and the pathology determined. Histological examination after (1)H MAS MRS defined 27 samples with squamous cell carcinoma (SCC), 12 with CIN and 39 with only normal tissue. The standardized integrals of the lipid, choline and creatine regions of the spectra were significantly higher in SCC than in normal or CIN tissue. There was no obvious difference in the standardized integral of the region 4.15-3.5 ppm. The acyl fatty acid side-chain length was longer or less unsaturated in SCC than in normal tissue. Normal tissue from patients with SCC showed significantly higher triglycerides than normal tissue from patients with benign uterine disease but significantly lower triglycerides than SCC tissue. (1)H MAS MRS of the uterine cervix ex vivo may be used to differentiate non-invasive from invasive cervical lesions, increase interpretation of in vivo MRS and provide insights into tumor biology.     

Thrombopoietin receptor agonist switch in children with persistent and chronic severe immune thrombocytopenia: A retrospective analysis in a large tertiary center

We retrospectively analyzed sequential therapy with romiplostim and eltrombopag in 23 children with immune thrombocytopenia: switching from romiplostim to eltrombopag (10 patients) or vice versa (13 patients). The median age of patients at enrollment in the study was 5.6 years (2‐15 years). Switching from romiplostim to eltrombopag was effective in eight (80%) patients, whereas switching from eltrombopag to romiplostim was effective in eight (62%) patients. The response rate was similar in patients failing the first thrombopoietin receptor agonist and those who had previous response. To date, all responders continue to maintain platelets over 50 × 10⁹/L at 13‐39 months after switching.     

Effect of oral ketoprofen on pain perception during copper IUD insertion among parous women: A randomized double-blind controlled trial

Objective

The study aims to evaluate the analgesic effect of oral ketoprofen prior to copper intrauterine device (IUD) insertion on pain perception during the insertion procedure.Study design: A randomized double-blind controlled trial (Clinical Trials. Gov: NCT02905058).Setting: Assiut Women's Health Hospital, Assiut, Egypt.

Prognostic determinants in patients with uterine and ovarian clear carcinoma

Objective

The purpose of this study is to analyze and compare the demographics, treatment, recurrence, and survival rates in patients with uterine clear cell carcinoma (UCCC) and ovarian clear cell carcinoma (OCCC).

Methods

A retrospective review of the Cancer Registry database was performed. All patients with UCCC and OCCC who underwent surgical staging at the two participating institutions, between January, 1995 and December, 2007, were identified. Categorical variables were evaluated by Chi square test. Survival estimates were plotted utilizing the Kaplan-Meier method.

Results

Analysis of 41 women with UCCC and 121 with OCCC was performed. In patients with OCCC, 48.4% had localized disease, 18.9% had regional spread, 31.1% had distant metastasis, and in 1.6% spread is unknown; compared to UCCC, 41.5% had localized disease, 12.2% regional spread, and 46.3% distant metastasis (p = 0.2). The median progression free survival was 31.4 months in women with UCCC, compared to 145 months in patients with OCCC (p = 0.04). UCCC women had a median overall survival of 39.5 months, compared to 155.8 months in patients with OCCC (p = 0.002). In the multivariate Cox regression model, age > 55 years old, tumor extension, optimal cytoreduction, and platinum-based chemotherapy were identified as independent predictors of overall survival. UCCC vs. OCCC was not associated with decreased overall survival in multivariate analysis.

Conclusion

OCCC and UCCC have the same rate of localized disease, regional spread and distant metastasis. After controlling for age, tumor extension, optimal cytoreduction, and platinum based chemotherapy, UCCC was not associated with decreased overall survival compared to OCCC.     

Temperamental correlates of disruptive behavior disorders in young children: preliminary findings.

BACKGROUND: Our objective was to test the hypothesis that temperamental behavioral disinhibition measured in early childhood would be associated with disruptive behavior disorders. METHODS: We used variables from laboratory-based behavioral observations originally devised to assess behavioral inhibition to construct a theory-based a priori definition of "behavioral disinhibition" in 200 young children at-risk for panic disorder, depression, or both and 84 children of parents without anxiety or major depressive disorder. We then compared behaviorally disinhibited and nonbehaviorally disinhibited children on rates of DSM-III-R disorders and measures of academic and social dysfunction. RESULTS: Behavioral disinhibition was significantly associated with higher rates of disruptive behavior disorders and mood disorders. Children with behavioral disinhibition were significantly more likely than nondisinhibited, noninhibited children to have attention-deficit/hyperactivity disorder (ADHD) and to have comorbid mood and disruptive behavior disorders. Moreover, disinhibited children had lower Global Assessment of Functioning Scale scores and were more likely to have been in special classes and to have problems with school behavior and leisure activities. CONCLUSIONS: These results suggest that behavioral disinhibition may represent a temperamental precursor to disruptive behavior problems, particularly ADHD. Longitudinal studies using behavioral assessments of behavioral disinhibition are needed to confirm these findings.     

Diagnosis and management of orbital pseudotumor

Orbital pseudotumor, also known as idiopathic orbital inflammatory syndrome (IOIS), may have protean clinical manifestations. Some presentations of IOIS may mimic common conditions such as orbital cellulitis and optic neuritis. IOIS should be considered a diagnosis of exclusion, with evaluation directed toward eliminating other causes of orbital disease. Orbital magnetic resonance imaging is the single most important diagnostic test, but serologic studies are necessary to exclude a systemic cause. Biopsy is usually not performed at presentation, as the risk of producing damage to vital structures within the orbit outweighs the benefits. Patients with multiple recurrences, or those unresponsive to therapy, should have biopsy samples taken. Corticosteroids are the mainstay of therapy and are administered for several months to ensure remission. Radiotherapy may be used in patients who fail to respond to steroids or who have a rapidly progressive course. For those patients who are refractory to both corticosteroids and radiotherapy, anecdotal reports have suggested the use of chemotherapeutic agents such as cyclophosphamide, methotrexate, and cyclosporine.     

Physical activity and risk of revision total knee arthroplasty in individuals with knee osteoarthritis: a matched case-control study

OBJECTIVE: To determine if physical activity was a risk factor for revision arthroplasty after primary total knee arthroplasty (TKA) due to osteoarthritis (OA) within the previous 15 years. METHODS: This was a matched case-control study. The cases had primary TKA followed by revision arthroplasty. Controls had primary TKA and no revision arthroplasty. Cases and controls were matched for age, sex, number of knees replaced, and date of primary TKA. Standardized telephone interviews were conducted to assess historical leisure activity, occupational activity, and instrumental activities of daily living after primary TKA in metabolic equivalent (MET)-hours per week. Conditional logistic regression was performed to identify the variables that predicted the need for revision arthroplasty. RESULTS: Seventeen female and 9 male pairs, aged 47 to 85 years, participated. Most of the reported activity was of low impact and low or moderate intensity. Cases reported a median of 44.5 (range 0 to 137) MET-hours of total historical physical activity per week compared with controls' 55.1 (range 0 to 278) MET-hours. Total historical physical activity was not associated with the risk of revision arthroplasty (OR 0.99, 95% CI 0.99-1.01). Participants with primary TKA (controls) consistently reported more MET-hours of leisure and occupational activity than those with revision arthroplasty (cases) regardless of the number of knees replaced or whether or not walking was accounted for. CONCLUSION: This study quantified and described patterns of physical activity in a population with TKA. Physical activity did not appear to be a risk factor for revision arthroplasty. Our results suggest that individuals undergoing primary TKA should be encouraged to remain active after surgery.