ABSTRACT
There is little published data on benznidazole dosing, or levels in cerebrospinal fluid. In this report, we describe the clinical course of an immunosuppressed patient with Chagas central nervous system involvement. He was treated successfully with larger benznidazole doses than are recommended, in order to reach therapeutically effective concentrations in the brain.
Subject(s)
Brain/metabolism , Chagas Disease/immunology , Immunosuppressive Agents/administration & dosage , Kidney Transplantation , Nitroimidazoles/administration & dosage , Humans , Immunocompromised Host , Male , Middle Aged , Nitroimidazoles/pharmacokineticsABSTRACT
Myotonic dystrophy type 1 (DM1) is a multisystem genetic disorder caused by a triplet nucleotide repeat expansion in the 3' untranslated region of the Dystrophia Myotonica-Protein Kinase (DMPK) gene. DMPK gene transcripts containing CUG expanded repeats accumulate in nuclear foci and ultimately cause altered splicing/gene expression of numerous secondary genes. The study of primary cell cultures derived from patients with DM1 has allowed the identification and further characterization of molecular mechanisms underlying the pathology in the natural context of the disease. In this study we show for the first time impaired nuclear structure in fibroblasts of DM1 patients. DM1-derived fibroblasts exhibited altered localization of the nuclear envelope (NE) proteins emerin and lamins A/C and B1 with concomitant increased size and altered shape of nuclei. Abnormal NE organization is more common in DM1 fibroblasts containing abundant nuclear foci, implying expression of the expanded RNA as determinant of nuclear defects. That transient expression of the DMPK 3' UTR containing 960 CTG but not with the 3' UTR lacking CTG repeats is sufficient to generate NE disruption in normal fibroblasts confirms the direct impact of mutant RNA on NE architecture. We also evidence nucleoli distortion in DM1 fibroblasts by immunostaining of the nucleolar protein fibrillarin, implying a broader effect of the mutant RNA on nuclear structure. In summary, these findings reveal that NE disruption, a hallmark of laminopathy disorders, is a novel characteristic of DM1.
Subject(s)
Cell Nucleus/pathology , Fibroblasts/metabolism , Fibroblasts/pathology , Myotonic Dystrophy/genetics , Myotonic Dystrophy/pathology , Cell Nucleolus/pathology , Cells, Cultured , Humans , Trinucleotide Repeat ExpansionSubject(s)
Collagen/genetics , Mutation , Nephritis, Hereditary/genetics , Adolescent , Female , Humans , Male , Mexico , Nephritis, Hereditary/ethnologyABSTRACT
Uremic polyneuropathy (UPNP) is a serious complication of chronic renal failure (CRF) in adults; however, its prevalence is unknown in the pediatric population. An easy-to-perform maneuver for its detection in children is the evaluation of "H" reflex. The objective of this study was to validate the usefulness of the "H" reflex maneuver for the diagnosis of UPNP in pediatric dialysis patients for CRF. Thirty-seven CRF patients were paired with healthy controls by age and sex. The patients were being treated with dialysis or one of its variants. Information was obtained regarding diagnosis, duration, and control of dialysis. Neurological examination was performed, conduction velocities in sensory and motor nerves were measured, and "H" reflex elicited bilaterally. Peripheral polyneuropathy was determined by the presence of at least two nerves with alterations in latency and/or conduction velocities. It was found that 59.4% (22/37) of the children with CRF treated with dialysis developed UPNP, 17 with ambulatory peritoneal dialysis, and 5 with hemodialysis. There was no difference in diagnosis, duration of dialysis, or control of the same in these patients from other CRF patients who did not have UPNP. All patients were clinically asymptomatic. "H" reflex showed a sensibility of 44%, a specificity of 87%, a predictive value positive of 66%, and a predictive value negative of 76%, when measured to 28 msec. With a 30 msec duration specificity rises to 95%. UPNP presents asymptomatically in pediatric patients. "H" reflex is an adequate screening test for the selection of pediatric patients to be tested further.
Subject(s)
H-Reflex , Kidney Failure, Chronic/complications , Peripheral Nervous System Diseases/diagnosis , Peripheral Nervous System Diseases/etiology , Adolescent , Child , Female , Humans , Kidney Failure, Chronic/therapy , Male , Neural Conduction , Peritoneal Dialysis , Prospective Studies , Reaction Time , Renal Dialysis , Sensitivity and SpecificityABSTRACT
In patients with chronic renal failure (CRF), parenteral transmission of the hepatitis B virus (HBV) is common. The response to the recombinant vaccine is 50%-80% of seroprotection. Therefore, to improve seroprotection, different strategies such as dose augmentation, vaccination at the predialysis stage, subcutaneous application, and using interleukin were tried, with unsatisfactory results. In children, there are no studies demonstrating the efficacy of the vaccine. The aim of this study was to evaluate the efficacy of the recombinant vaccine in children with CRF, in late as well as early phases, through the quantification of antibodies against the surface antigen in response to different doses of the vaccine against HBV. There were 103 patients who were assigned to three groups: (1) 25 patients with CRF in the early phase (undergoing pharmacological treatment only); (2) 67 patients with CRF in the late phase (treatment with peritoneal dialysis or hemodialysis); (3) 11 patients with CRF in the early phase (undergoing low-dose pharmacological treatment only). The antibodies against the serum antigen (HBsAg) were measured by the aEIA method. Urea, creatinine, and creatinine clearance were measured at 0, 2, and 12 months. In our seroprotection results we observed that group 1 and 3 developed earlier seroconversion (50% first month). In patients undergoing dialysis the seroconversion happened in 91% at month 13, but with lower concentration than group 1 and/or group 3 (p < 0.05). In conclusion, there is a better response in predialysis patients. The levels of antibodies are similar in groups 1 and 3 (with small doses), which are similar to the complete doses for an efficient immunity in children with chronic renal failure.
Subject(s)
Hepatitis B Antibodies/analysis , Hepatitis B Vaccines/immunology , Kidney Failure, Chronic/immunology , Peritoneal Dialysis , Renal Dialysis , Vaccines, Synthetic/immunology , Adolescent , Child , Child, Preschool , Female , Hepatitis B Surface Antigens/immunology , Hepatitis B Vaccines/administration & dosage , Humans , Immunization Schedule , Infant , Injections, Intramuscular , Injections, Subcutaneous , Kidney Failure, Chronic/therapy , Male , Vaccines, Synthetic/administration & dosageABSTRACT
The aim of this study is to quantify the intelligence output in our pediatric population on continuous ambulatory peritoneal dialysis (CAPD) treatment. A total of 30 children were studied, with an age range of eight to 18 years. For evaluating a global intelligence quotient (IQ), the Wechsler test was applied according to their age. And, as a complement, a Bender test was also requested to deny or confirm brain damage. The Wechsler test showed an average intelligence quotient in most of the children (76.7%); a small group (16.7%) was classified as dull normal, 1 child had mild retardation, and 1 was borderline. All of them had a Bender test that did not correlate with brain damage. Most of them maintained a very high verbal IQ, but, when the performance IQ was qualified, 34% got a low score, and a certain difficulty in solving this part of the test was observed. Maybe this was influenced by chronicity of the sickness and/or the blood urea nitrogen level. In conclusion, the child's plasticity is his best quality to cope with uremia and the alterations caused by it. Therefore the idea is to consider the intelligence quotient as the capacity that the subjects have to use their own resources to cope with their environment. And, even though the neurological alterations exist, the child's plasticity helps him/her maintain a good global intelligence quotient, even though he/she is not having immediate transplantation.
Subject(s)
Brain Damage, Chronic/psychology , Intelligence , Kidney Failure, Chronic/psychology , Peritoneal Dialysis, Continuous Ambulatory/psychology , Adolescent , Bender-Gestalt Test , Brain Damage, Chronic/diagnosis , Brain Damage, Chronic/etiology , Child , Female , Humans , Intellectual Disability/diagnosis , Intellectual Disability/etiology , Intellectual Disability/psychology , Kidney Failure, Chronic/etiology , Kidney Failure, Chronic/therapy , Male , Reference Values , Risk Factors , Wechsler ScalesABSTRACT
The hospital records of all patients on continuous ambulatory peritoneal dialysis (CAPD) who were followed from January 1992 to June 1993 in a third level pediatric hospital were retrospectively reviewed to compare the peritonitis rates of groups of patients according to the dialysis system employed. All patients had Tenckhoff catheters installed by open technique. The groups were A) traditional spike connection system (37 patients), and B) disconnect systems (16 patients) that comprised the O-set system (10 patients) and the basic Y-set system (6 patients). Age was 11.9 +/- 3.7 years (mean +/- SD, range 3 months to 17 years). Follow-up time was 8.5 +/- 4.6 months for the traditional system group (304 cumulated months) and 7.2 +/- 3.6 months for the disconnect systems group (116 cumulated months). Peritonitis rates were 1 episode every 10 months for group A, versus 1 episode every 58 months for group B (p < 0.05). Our results strongly suggest that disconnect systems for peritoneal dialysis can significantly decrease peritonitis rates, although longer follow-up periods are needed.
Subject(s)
Peritoneal Dialysis, Continuous Ambulatory/adverse effects , Peritonitis/etiology , Adolescent , Child , Child, Preschool , Female , Humans , Infant , Male , Peritoneal Dialysis, Continuous Ambulatory/instrumentation , Peritonitis/drug therapy , Peritonitis/microbiology , Retrospective StudiesABSTRACT
PIP: The objective of this study was to analyze the acceptability, effectiveness, and continuation of long-acting progestagens in certain sectors of the population where its advantages are more obvious by virtue of certain socioeconomic, cultural, and geographic characteristics, and by lack of availability of family planning services, as in rural areas and urban marginal zones. The study was performed using females of reproductive age residing in rural areas of the Mexican States of Hidalgo, Puebla, and Yucatan between July 1981 and September 1982. Originally the investigation included a total of 462 women, of whom 94 were lost to follow-up, leaving a total of 368 patients (79.6%) with effective follow-up. The contraceptive used was 19-Nor progestagen, norethisterone enanthate (NET), in 200 mg doses administered intramuscularly. The 1st dose was applied between the 1st to the 5th day of the menstrual cycle and each 60 calendar days thereafter. Of a total of 94 discontinuations, 2/3 occurred during the 1st 6 months of the study. The final discontinuation rate was 14.46%. The studied population showed a continuation rate of 85.54% after 12 months of use. Observing rates by reasons for discontinuation, the principal causes in descending order were nonmedical reasons, amenorrhea, pregnancy, bleeding and other secondary effects. The greatest number of discontinuations, considering each particular reason, occurred predominantly during the 1st 6 months of the study. Distribution of users by age group show that more than 2/3 (71.8%) were between 20 and 34 years old. Distribution by number of previous pregnancies show 56.5% with 5 or more gestations. To analyze behavior of menstrual cycles, "cycles" were defined as period of 30 calendar days. Under this concept, more than 2/3 (70.9%) of the users had from 1 to 7 days of bleeding per cycle. On the basis of the results of the study, it is possible to conclude the following: 1) Continuation of NET use is greater than that observed with other long-lasting progestagens. This seems to be especially so when employed in areas where limitations exist for the utilization of other contraceptive means. 2) Contraceptive effectiveness is greater when administration is scheduled at 60 day intervals rather than longer intervals. 3) Unlike other long-acting progestagens, NET conserves the normal menstrual pattern in the majority of users, which favorably influences its continuation.^ieng
Subject(s)
Contraceptive Agents, Female/administration & dosage , Norethindrone/analogs & derivatives , Adolescent , Adult , Delayed-Action Preparations , Drug Evaluation , Female , Health Knowledge, Attitudes, Practice , Humans , Injections, Intramuscular , Mexico , Norethindrone/administration & dosage , Pregnancy , Rural PopulationABSTRACT
Se presentan los resultados de un estudio efectuado en 368 mujeres residentes en areas rurales de Mexico, que utilizaron enantato de noretisterona como anticonceptivo inyectable de intervalo. El esquema de administracion fue de 200mg por via intramuscular cada 60 dias. Se encontro una descontinuacion de 14.46 a un ano de uso, siendo las razones principales las no medicas y en segundo lugar, los trastornos del ciclo. La tasa de embarazos resulto de 2.09 por ciento. Tambien se observo que el 70.9 por ciento de las usuarias presentaron 1 a 7 dias de sangrado por mes, es decir, que mostraron una regularidad considerable en sus ciclos. Se comentan asimismo las posibles razones de la alta continuidad observada con el metodo y la baja incidencia de trastornos menstruales
Subject(s)
Humans , Female , Family Development Planning , Norethindrone , Rural Population , MexicoABSTRACT
PIP: 361 retrospective surveys were carried out among users of medroxyprogesterone acetate (MPA) given in a trimestral regimen of 150 mg/dose in rural areas of 3 Mexican states. Gynecology-obstetric antecedents, previous experience with oral contraceptives (OCs), effects of injections on the menstrual cycle, causes of method suspension, and opinion concerning administration of medication were analyzed. 78.6% of the patients were multigravidae with 4 or more pregnancies; 39.1% were former users of OCs, and 23.4% had stopped taking them because of side effects. The side effects of MPA on the menstrual cycle were: amenorrhea (19.8%); hemorrhage/cycle of 10-30 days in 14.7%; and hemorrhage/cycle of 30 or more days in 11.6%. Only 14.7% of users stopped the injections and of these, 80.3% did so due to menstrual cycle disorders. 99.7% of the users thought the method was comfortable as a family planning procedure. (author's)^ieng