The effect of erythropoietin therapy and hemoglobin levels on the immune response to Engerix-B vaccination in chronic kidney disease

BACKGROUND: Patients undergoing chronic hemodialysis have an increased risk of acquiring hepatitis B infection. Only 43-66% of dialysis patients develop effective anti-HBs titers after vaccination. AIM: To evaluate the effect of recombinant erythropoietin (rEPO) therapy and basal hemoglobin levels on the outcome of the immune response to four doses of IM 40 microg Engerix-B vaccination in hemodialysis and chronic kidney disease (CKD) patients before starting replacement therapy. SUBJECTS AND METHODS: One hundred and three patients were included in the study: 34 hemodialysis patients treated with rEPO (Group A), 36 predialytic patients who did not treated with rEPO (Group B) and 33 predialytic patients treated with rEPO (Group C). Plasma creatinine in predialytic patients was 2-7 mg/dL. All patients' HBsAg and anti-HBs antibodies were negative. Patients were immunized with IM 40 microg Engerix-B at 0, 1, 3, and 6 months. Anti-HBs titers were measured at 7th month. RESULTS: Eighty seven point one percent of patients from group C developed protective anti-HBs titers compared with 69.4% from group B and 44.1% from group A (p = 0.001). Patients from all groups with baseline hemoglobin levels above 11 gr/dL developed protective anti-HBs titers significantly more than patients with baseline hemoglobin levels below 11 gr/dL (p < 0.05). CONCLUSION: Predialytic patients treated with rEPO and with hemoglobin levels higher than 11 gr/dL had significantly better immune response outcomes to Engerix-B vaccination. Immunization against hepatitis B infection should be considered at early stages of CKD prior to the deterioration in kidney functions and the development of renal anemia.     

Progressive multifocal leukoencephalopathy in a lung transplant recipient.

Progressive multifocal leukoencephalopathy (PML) is a sub-acute, demyelinating disease of the brain caused by a human polyomavirus. We describe a patient with the onset of PML 7 months after lung transplantation. The patient was treated with immunosuppressive modulation and cidofovir, a new anti-viral therapy for PML, with stabilization of the symptoms. We also review the 4 additional reports in the literature of PML after heart and lung transplantation. Progressive multifocal leukoencephalopathy may become more prevalent as the population of heart and lung transplantation recipients increases.     

Development of an Arabic version of the National Eye Institute Visual Function Questionnaire as a tool to study eye diseases patients in Egypt

AIM:To develop and test an Arabic version of the National Eye Institute Visual Function Questionnaire-25 (NEI-VFQ-25).METHODS:NEI-VFQ-25 was translated into Arabic according to WHO translation guidelines. We enrolled adult consenting patients with bilateral chronic eye diseases who presented to 14 hospitals across Egypt from October to December 2012, and documented their clinical findings. Psychometric properties were then tested using STATA.RESULTS:We recruited 379 patients, whose mean age was (54.5±15)y. Of 46.2% were males, 227 had cataract, 31 had glaucoma, 23 had retinal detachment, 37 had diabetic retinopathy, and 61 had miscellaneous visual defects. Non-response rate and the floor and ceiling numbers of the Arabic version (ARB-VFQ-25) were calculated. Internal consistency was high in all subscales (except general health), with Cronbach-α ranging from 0.702-0.911. Test-retest reliability was high (intraclass correlation coefficient 0.79).CONCLUSION:ARB-VFQ-25 is a reliable and valid tool for assessing visual functions of Arabic speaking patients. However, some questions had high non-response rates and should be substituted by available alternatives. Our results support the importance of including self-reported visual functions as part of routine ophthalmologic examination.     

Nodular mixed lymphoma: results of a randomized trial failing to confirm prolonged disease-free survival with COPP chemotherapy

Fifty-two patients with stage III or IV nodular mixed lymphocytic- histiocytic lymphoma (NM) were entered on a prospective randomized trial comparing cyclophosphamide-prednisone (CP) to either COPP (cyclophosphamide, vincristine, procarbazine, prednisone) or BCVP (BCNU, cyclophosphamide, vincristine, prednisone). The COPP regimen utilized in this Eastern Cooperative Oncology Group (ECOG) trial was similar to the four-drug regimen C-MOPP reported by the National Cancer Institute to achieve prolonged relapse-free survival in this histology. No significant differences in complete response rates, response duration, or overall survival were noted among the three regimens. A pattern of continuous late relapse was observed for all three chemotherapy programs. Although 11 of the 18 (61%) COPP patients achieved a complete response, only 3/11 (27%) remain disease-free with a median follow-up of over 3 yr. However, two of these three long-term complete responders have died with no clinical evidence of recurrent disease. The COPP patients received 84% of the calculated ideal doses of cyclophosphamide and 78% of the ideal dosage of procarbazine. Grade 3-4 hematologic toxicity was noted in 22% of the COPP group, 36% with BCVP, and 0% for the CP patients. We were unable to confirm the ability of COPP to achieve durable complete remissions in NM lymphoma. The cyclophosphamide-prednisone combination was equally effective when compared with COPP and BCVP, but produced minimal toxicity.     

Surface antigens on malignant Sezary and T-CLL cells correspond to those of mature T cells

Tumor cells from eight adult patients with T-cell chronic malignancies were investigated with a series of monoclonal antibodies recognizing T- cell differentiation antigens. This series allowed definition of discrete subpopulations of mature T cells with functional specialization. All six patients with Sezary syndrome and one patient with T-chronic lymphocytic leukemia had cells with the same phenotype as normal helper/inducer T cells, whereas the other patient with T- chronic lymphocytic leukemia had cell with the same phenotype as normal cytotoxic/suppressor T cells. Some clinical manifestations observed in these patients may reflect retention of functional activities by their malignant cells.     

Anatomic substrate for preexcitation in idiopathic myocardial hypertrophy with fibroelastosis of the left ventricle

Serial section of the conduction system and both atrioventricular (A-V) rims was performed in three patients who died with known preexcitatlon and idiopathic myocardial hypertrophy with fibroelastosis proved at autopsy. All three patients had type B preexcitation and a leftward and inferior 20 ms vector, suggesting a right free wall anomalous pathway. Patient 1 had no arrhythmia, Patient 2 died suddenly and Patient 3 had recurrent paroxysmal supraventricular tachycardia. Electrophysiologic study in Patients 1 and 3 revealed a bidirectionally conducting anomalous pathway with short refractoriness. Patient 1 had no, and Patient 3 had easily inducible A-V reentrant paroxysmal supraventricular tachycardia. Electrophysiologic study in Patient 3 revealed an anterior right free wall or anteroseptal anomalous pathway, manifested by the shortest stimulus-delta interval with pacing of the right anterior atrium. No electrophysiologic studies were performed in Patient 2.In Patient 1 serial section revealed a large right free wall anomalous pathway with myocardial disarray on the ventricular side of the anomalous pathway. In Patient 2, there were two small anomalous pathways in the right free wall. In Patient 3, no anomalous pathway was found in the right free wall; however, the right atrium was connected to the infundibular septum anterior to the membranous septum. This anomalous pathway had continuity with an anterior A-V nodal-like structure.In summary, (1) all three patients anatomically had a right-sided anomalous pathway (as predicted on electrocardlography in all three and electrophyBiologically in Patients 1 and 3). (2) In Patient 2, myocardial disarray in the Kent bundle may have prevented paroxysmal supraventricular tachycardia. (3) In Patient 3, histologic study revealed an anterior septal anomalous pathway on the right side with an anterior A-V nodal-like structure. Because the anomalous pathway did not show any A-V nodal properties, the significance of this structure is not clear. (4) The relation of the right-sided anomalous pathway to the left-sided fibroelastosis is not known.     

Israeli children with autism spectrum disorder are not macrocephalic

The prevalence of macrocephaly in autism spectrum disorder is reported to be much higher than in the general population, 12% to 37%. Progressive macrocephaly is even considered a warning sign for the development of autism. We evaluated the prevalence of an abnormal head circumference in children with autism in Israel and compared it with the head circumferences of children with developmental language disorder and children with normal development. We did not find a higher prevalence of macrocephaly among Israeli children with autism spectrum disorder (4.4%). Although children with autism spectrum disorder had a significantly higher rate of a head circumference above the 75th percentile compared with children with developmental language disorder, it was not significantly different compared with normal controls. We conclude that there is no increased prevalence of macrocephaly in Israeli children with autism; this can be attributed to a different genetic background.     

Hanging leg weight--a rapid technique for estimating total body weight in pediatric resuscitation

The accuracy of two methods of rapidly estimating total body weight in children was assessed. The first method correlated patient length to known total body weight, and the second correlated the weight of both legs weighted together to known total body weight. One hundred children undergoing general anesthesia in the operating rooms of Childrens Hospital of Los Angeles were entered into the study. Each child had a known preoperative body weight. After induction of anesthesia, each child's supine length was measured and hanging (inert) legs were weighted. There was excellent linear correlation between hanging leg weight and total body weight (correlation coefficient [r2], 0.95) for all patients, and good linear correlation between supine length and total body weight, r2, 0.86). Range restriction analysis for both techniques showed poor correlation for total body weights of less than 10 kg and poor correlation for the supine length technique when total body weight was more than 25 kg. The hanging leg weight of an inert child has excellent correlation to total body weight for children weighing more than 10 kg.     

Responses of the human auditory cortex to changes in one versus two stimulus features

Neuromagnetic responses were recorded with a 24 SQUID magnetometer in two oddball experiments to determine whether mismatch responses to changes in single stimulus features are additive. In experiment 1, the one feature deviants differed from standards in interstimulus interval (ISI) or frequency, and the two feature deviants in both ISI and frequence. In experiment 2, deviants differed in duration, frequency, or both. All deviants evoked a mismatch field (MMF) with sources close to each other in the supratemporal auditory cortex. Except for the ISI deviants, the MMF sources were about 1 cm anterior to the source of the 100ms response, N100m, to the standards. In the two experiments, MMFs obtained in response to the two feature deviants resembled closely the sum of MMFs in response to one feature deviants. The results suggest that the standards leave a multiple neuronal representation in the human auditory cortex. The particular neuronal traces of the representation react independently to changes in different features of sound stimuli.