Association of simple hematological parameters with disease manifestations,activity, and severity in patients with systemic sclerosis

Clinical Rheumatology - Neutrophil-to-lymphocyte ratio (NLR), monocyte-to-lymphocyte ratio (MLR), mean platelet volume (MPV), and red cell distribution width (RDW) may potentially reflect...     

Views of Mothers Having Children with Intellectual Disability Regarding Sexual Education: A Qualitative Study

This study was designed to evaluate the views of mothers having children with intellectual disability on sexual education for their children. Focused group methodology and in-depth interviews (face-to-face) were utilized for qualitative investigation. Interviews were performed with nine mothers having children with disability, aged 7–18 years registered at the “Education Practice Centre and Job Training Centre” from 2012 to 2013. Data was collected using a semi-structured questionnaire prepared by the researchers through screening. The questionnaire focused on the selection of sexual education. A voice-recording device was used during the interviews; face-to-face interviews were performed in a suitable physical environment without any voice recording. After they were given information about the study and voice recording the mothers’ verbal consents were obtained. In this study, it was found that all mothers having children with intellectual disability ignored the sexual education for their children with intellectual disability. All mothers, especially having a boy with intellectual disability, said that a sexual education was needed for their children, and they thought this education could be given by an institution like a rehabilitation centre. People generally thought that children with intellectual disability would not have a sexual life, family planning was thought to be unnecessary to be involved in sexual education, they were scared that their children would be sexually abused and therefore kept them away even from their healthy siblings. These findings are thought to be a guide for nurses, rehabilitation centres and schools working with children with intellectual disability to prioritize the sexual education of these children, raise awareness in families and children on this subject and enable them to sustain their healthy sexual developments and protect them from possible danger.     

Lumbar puncture requirement in acute hemiparesis: Diagnosis of tuberculous meningitis after hemiparesis in a child

Background

Infections are an important acquired cause of cerebral arteriopathy. Tuberculous (TB) meningitis leading to infectious cerebral vasculopathy is a rare cause of acute hemiparesis.

Case report

A 14-year-old male patient was examined after acute hemiparesis developing within 1 day. Neurological examination revealed total hemiplegia on the left side. Brain MRI findings showed bilateral focal T2-weighted signal hyperintensity in the subcortical and deep white matter regions. There were also areas of restricted diffusion in the right basal ganglia. Although the father had a history of pulmonary TB, the patient had not been given TB prophylaxis because of PPD negativity. At lumbar puncture, opening cerebrospinal fluid (CSF) pressure was 50 cm/H20, CSF protein 66.9 mg/dL, and glucose 54 mg/dL (concurrent blood glucose 93 mg/dL); 170 polymorphonuclear leukocytes per cubic millimeter were present in CSF. Following tests for TB, treatment was started immediately with four anti-TB drugs. TB PCR of CSF and acid-fast bacteria (AFB) staining in gastric aspirate were positive. At clinical follow-up, the patient was able to walk with support at the end of the first month.

Conclusion

Various infectious agents have been reported as causes of cerebral vasculopathy. TB, which affects a significant number of patients worldwide, should be kept in mind in terms of cerebral vascular complications. Lumbar puncture is essential in order to diagnose TB meningitis.     

Novel adenosine deaminase 2 mutations in a child with a fatal vasculopathy

Adenosine deaminase 2 (ADA2) deficiency due to CECR1 mutations is a recently defined disorder that involves systemic inflammation and vasculopathy often associated with polyarteritis nodosa. We report on a 5-year-old girl with a severe vasculopathy who carried two novel mutations in CECR1. Conclusion: Identification of CECR1 mutations in patients with vasculopathy may lead to earlier diagnosis of ADA2 deficiency.     

Assessment of Neuropsychological Late Effects in Survivors of Childhood Leukemia

The neurologic dysfunctions caused by treatment may affect health and quality of life in survivors of childhood leukemia. The objective of this study was to identify the neuropsychological late effects of leukemia treatment to provide an assessment about the degree and incidence of these late effects. Neurological and ophtalmological examination, cranial magnetic resonance imaging (MRI), auditory and neurocognitive tests, and questionnaires of quality of life were performed to 44 acute leukemia survivors at least 5 years after diagnosis. Median time since completion of chemotherapy was 7.5 years (2–18) and median age at the time of the study was 16.4 years (8–31). At least one or more late effects detected by physical examination (PE), neurological tests, or neurocognitive tests encountered in 80% of the patients, and 64% of the patients specified at least one complaint in the quality of life questionnaire. MRI revealed pathological findings in 18% and electroencephalogram (EEG) abnormalities were present in 9% of the patients. Evaluation of total intelligence scores revealed that 30% of patients’ IQ scores were <80 and 70% of the patients’ scores demonstrated neurocognitive dysfunctions. The patients >6 years at the time of diagnosis were found to have more psychological problems and higher rates of smoking and alcohol consumption. The most frequent complaint was headache and the most common problem in school was denoted as difficulty in concentration. Our study demonstrated that most of the survivors of childhood leukemia are at risk of developing neuropsycological late effects.     

Relationship between exercise-induced heart rate increase and the formation of microbubbles and high-intensity transient signals in mechanical heart valve implanted patients

Introduction

The formation and collapse of vapor-filled bubbles near a mechanical heart valve is called cavitation. Microbubbles can be detected in vivo by doppler ultrasonography (USG) as HITS (high intensity transient signals) in cranial circulation. We investigated the relationship between exercise induced heart rate increase and HITS formation in cranial circulation.

Material and methods

Thirty-nine mechanical heart valve implanted (8 aortic valve replacement (AVR) + mitral valve replacement (MVR), 9 AVR, 22 MVR) patients aged 18-80 years old were included in our study. Microbubbles were counted in the left ventricular cavity via transthoracic echocardiography at rest per cardiac cycle. Afterwards transcranial Doppler USG was performed and HITS were counted in each patient''s middle cerebral artery at 5 min duration. Subsequently an exercise test according to the Bruce protocol was performed. After achieving maximal heart rate, microbubbles in the left ventricle and HITS were counted again.

Results

Microbubbles in the left ventricle and transcranial HITS increased after exercise significantly compared to resting values (15.79 ±10.91 microbubbles/beat vs. 26.51 ±18.00 microbubbles/beat, p < 0.001; 6.13 ±8.07 HITS/5 min vs. 13.15 ±15.87 HITS/5 min, p = 0.001). There was a significant correlation between microbubbles and HITS counts after peak exercise (r = 0.55, p < 0.001).

Conclusions

In our study, we found that the microbubbles were increasing as the heart rate increased and more HITS were propelled to the cerebral circulation. As previously shown, HITS can alter cognitive functions. Therefore heart rate control is essential in mechanical heart valve patients to protect neurocognitive functions.     

Effect of postpartum counseling on postpartum contraceptive use

Objective The aim of this study was to evaluate the effect of postpartum counseling on postpartum contraceptive use. Methods One hundred and forty-three women who delivered between 1 January 2004 and 31 September 2004 and counseled about postpartum contraception were included in the study. The participants were interviewed by telephone. Age, gravidity, parity, and mode of delivery of the participants were recorded. Their method of contraception before pregnancy, their decision on the contraceptive method after counseling and the method actually used were asked. Results Just after postpartum counseling, 47 women (32.9%) decided to use the intrauterine device (IUD), 23 (16.1%) condoms, 16 (11.2%) progestin injections, 7 (4.9%) oral contraceptives, and 7 (4.9%) coitus interruptus for contraception. Thirty-six women (25.2%) did not decide on any method of use. At the time of the telephone interview the actual method used was learned. Fifty-one women (35.7%) were using coitus interruptus, 45 women (31.5%) condoms, and 14 (9.8%) the IUD. Sixteen women (11.2%) were reported as not using any methods. Conclusion In spite of postpartum counseling, a high majority of the women appeared to use traditional and less effective contraceptive methods.     

Activity of adenosine deaminase in mothers who have conceived a fetus with central nervous system malformations

OBJECTIVE: To establish the adenosine deaminase (ADA) activity in women who had been pregnant with a child suffering from central nervous system (CNS) anomaly. METHODS: The study group comprised 68 women who had been pregnant with an affected child, and 68 controls matched for age, gestational age and body mass index. Maternal venous blood was collected for measurement of ADA levels. We defined the diagnostic sensitivity, specificity and area under receiver-operating characteristic curve for ADA. RESULTS: Plasma ADA activity was significantly higher in the study group (12.3 U/L, range 1.7-33.3) compared to the median value of 3.3 U/L (range 1.1-34.4) in normal pregnancies (P<0.05). The cut-off point of ADA >5.9 U/L was associated with the highest combination of specificity (58.8%) and sensitivity (86.8%). CONCLUSION: ADA activity in women who have conceived a fetus with CNS malformations was significantly higher than that in normal pregnant women.