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1.
Pediatr Int ; 2021 Nov 22.
Artigo em Inglês | MEDLINE | ID: mdl-34807498

RESUMO

We performed a retrospective survey and verification of the medical records of death cases of children (and adolescents; aged <18 years) between 2014 and 2016 in pediatric specialty training facilities in Japan. Of the 2827 registered cases at 163 facilities, 2348 cases were included. The rate of identified deaths compared with the demographic survey was 18.2%-21.0% by age group. The breakdown of deaths was determined as follows: 638 cases (27.2%) were due to external factors or unknown causes, 118 (5.0%) were suspected to involve child maltreatment, 932 (39.7%) were of moderate or high preventability or were indeterminable cases. Further detailed verification was required for 1,333 cases (56.8%). Comparison of the three prefectures with high rates of identified deaths in Japan revealed no significant differences, such as in the distribution of diseases, suggesting that there was little selection bias. The autopsy rate of deaths of unknown cause was 43.4%, indicating a high ratio of forensic autopsies. However, sufficient clinical information was not collected; therefore, thorough evaluations were difficult to perform. Cases with a moderate or high possibility of involvement of child maltreatment accounted for 5%, similar to previous studies. However, more objective evaluation is necessary. Preventable death cases including potentially preventable accounted for 25%, indicating that proposals need to be made for specific preventive measures. Individual primary verification followed by secondary verification by multiple organizations is effective. It is anticipated that a Child Death Review (CDR) system with such a multi-layered structure will be established; however, the following challenges were revealed: 1. The subjects of CDR are all child deaths. Even if natural death cases are entrusted to medical organizations, and complicated cases to other special panels, the numbers are very high. Procedures need to be established to sufficiently verify these cases. 2. Although demographic statistics are useful for identifying all deaths, care must be taken when interpreting such data. 3. Detailed verification of the cause-of-death will affect the determination of subsequent preventability. Because verification based only on clinical information is difficult, a procedure that collates non-medical information source should be established. 4. It is necessary to organize the procedures to objectively evaluate the involvement of child maltreatment and raise awareness among practitioners. 5. To propose specific preventive measures, a mechanism to ensure multi-professional diverse perspectives is crucial, in addition to fostering the foundation of individual practitioners. In addition, to implement the proposed measures, it is necessary to discuss the responsibilities and authority of each organization. 6. Once the CDR system is implemented, verification of the system should be repeated. Efforts to learn from child deaths and prevent deaths that are preventable as much as possible are essential duties of pediatricians. Pediatricians are expected to undertake the identified challenges and promote and lead the implementation of the CDR system.

2.
J Acoust Soc Am ; 150(3): 2189, 2021 09.
Artigo em Inglês | MEDLINE | ID: mdl-34598649

RESUMO

Relatively little is known about spinner dolphins in Malaysian waters and the wider Southeast Asian region. This note represents the first known acoustic recording of the species sighted opportunistically in the northern Straits of Malacca. Over a brief 20 min sighting, 46 whistles were recorded and four tonal types were detected, with 54.4% being upsweep whistles. The whistle duration ranged from 36 to 977 ms and the frequency ranged from 6.6 to 23.8 kHz. Fifty-seven click trains with a mean interclick interval of 41.5 ± 19.3 ms were detected. These findings provide a baseline for future regional acoustic research on this species.


Assuntos
Golfinhos , Stenella , Acústica , Animais , Malásia , Espectrografia do Som , Vocalização Animal
3.
PLoS One ; 16(8): e0255586, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34388156

RESUMO

Dugongs (Dugong dugon) are seagrass specialists distributed in shallow coastal waters in tropical and subtropical seas. The area and distribution of the dugongs' feeding trails, which are unvegetated winding tracks left after feeding, have been used as an indicator of their feeding ground utilization. However, current ground-based measurements of these trails require a large amount of time and effort. Here, we developed effective methods to observe the dugongs' feeding trails using unmanned aerial vehicle (UAV) images (1) by extracting the dugong feeding trails using deep neural networks. Furthermore, we demonstrated two applications as follows; (2) extraction of the daily new feeding trails with deep neural networks and (3) estimation the direction of the feeding trails. We obtained aerial photographs from the intertidal seagrass bed at Talibong Island, Trang Province, Thailand. The F1 scores, which are a measure of binary classification model's accuracy taking false positives and false negatives into account, for the method (1) were 89.5% and 87.7% for the images with ground sampling resolutions of 1 cm/pixel and 0.5 cm/pixel, respectively, while the F1 score for the method (2) was 61.9%. The F1 score for the method (1) was high enough to perform scientific studies on the dugong. However, the method (2) should be improved, and there remains a need for manual correction. The mean area of the extracted daily new feeding trails from September 12-27, 2019, was 187.8 m2 per day (n = 9). Total 63.9% of the feeding trails was estimated to have direction within a range of 112.5° and 157.5°. These proposed new methods will reduce the time and efforts required for future feeding trail observations and contribute to future assessments of the dugongs' seagrass habitat use.


Assuntos
Conservação dos Recursos Naturais , Dugong/fisiologia , Ecossistema , Comportamento Alimentar , Processamento de Imagem Assistida por Computador/métodos , Redes Neurais de Computação , Tecnologia de Sensoriamento Remoto/métodos , Animais , Tecnologia de Sensoriamento Remoto/instrumentação
4.
Clin Case Rep ; 6(11): 2089-2091, 2018 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-30455897

RESUMO

We present a case of severe Raynaud phenomenon (RP) in an infant. The current strategy of RP treatment is incomplete; excluding secondary Raynaud phenomenon is vital as well. This case aims to help those with similar symptoms in the future by gathering data on cases.

5.
PLoS One ; 13(7): e0201029, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-30024958

RESUMO

Recent advances in biotelemetry techniques, especially positioning methods, have revealed the detailed behaviour and movement of aquatic organisms. Behavioural intermittence in animal locomotion, such as the Lévy walk, is a popular topic in the field of movement ecology. Previous attempts to describe intermittent locomotion quantitatively have been constrained by the spatial and temporal resolution possible with conventional biotelemetry systems. This study developed a fine-scale spatiotemporal three-dimensional positioning method using a new biotelemetry system with a positional precision of <10 cm and positioning interval of <10 s. Using this proposed positioning method, the intermittent stop-and-go locomotion of Siebold's wrasses (Pseudolabrus sieboldi) was observed during travel from an unsuitable to a suitable location following displacement. The fish displayed behavioural intermittence in relocating to a suitable location. Initially, their movement halted for reorientation, after which they moved intermittently yet in a straight line to the suitable location. To test the positioning ability of the proposed method, data sets were resampled at intervals of 5, 10, 30, 60, and 300 s. Longer sampling intervals failed to identify reorientations and underestimated the number of stops, distance travelled, and speed. Overall, the results highlighted the adequacy and ability of the proposed positioning method to observe the intermittent locomotion of fish, such as stop-and-go behaviour, in a natural environment.


Assuntos
Migração Animal/fisiologia , Ecologia , Peixes/fisiologia , Locomoção/fisiologia , Modelos Estatísticos , Animais , Análise Espaço-Temporal
6.
Medicine (Baltimore) ; 97(25): e11135, 2018 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-29924014

RESUMO

To determine whether a peripheral capillary oxygen saturation (SpO2) of 95% to 96% should be considered "nonurgent" in school-aged children, as suggested by the Canadian Emergency Department Triage and Acuity Scale.School-aged children (6-12 years old) with a normal body temperature (36.5-37.4°C) who visited our department between September, 2014 and August, 2015 (n = 4556) were divided into 4 groups based on SpO2: group A: 99% to 100%; group B: 97% to 98%; group C: 95% to 96%; and group D: ≤94%. The heart rate (HR), respiratory rate (RR), and hospitalization rate were compared among the groups, and also between children with SpO2 95% to 96% and matched controls with SpO2 ≥97% (n = 280 each).Among 4556 eligible patients, groups A, B, C, and D comprised 2700 patients (59.3%), 1534 patients (33.6%), 280 patients (6.2%), and 42 patients (0.9%), respectively. The median (interquartile range [IQR]) RR significantly increased with decreasing SpO2 (23 [20-25], 24 [20-26], 24 [23-30], and 30 [24-40] breaths/min in groups A-D, respectively; P < .001). Similarly, the median (IQR) HR significantly increased with decreasing SpO2 (93 [83-104], 98 [87-110], 107 [93-119], and 121 [109-137] bpm, groups A-D, respectively; P < .001). Group D had the highest annual hospital admission rate (18 cases/42 patients, 42.9%). Further, the HR and RR differed significantly between the cases (107 [93-119] bpm; 24 [23-30] breaths/min) and controls (96 [86-106] bpm; 24 [20-28] breaths/min, respectively) (P < .001 and P = .02, respectively).An SpO2 of 95% to 96% among school-aged children should not be considered "nonurgent," but rather a significant clinical situation that requires early review of HR and RR. Prompt interventions among this group of children will help prevent further destabilization of vital signs, which will, in turn, contribute to decreased healthcare costs.


Assuntos
Serviço Hospitalar de Emergência , Frequência Cardíaca , Oximetria/métodos , Oxigênio/análise , Taxa Respiratória , Triagem , Canadá , Criança , Serviço Hospitalar de Emergência/normas , Serviço Hospitalar de Emergência/estatística & dados numéricos , Feminino , Humanos , Masculino , População , Troca Gasosa Pulmonar/fisiologia , Melhoria de Qualidade , Estudos Retrospectivos , Triagem/métodos , Triagem/normas
7.
Child Abuse Negl ; 79: 11-21, 2018 05.
Artigo em Inglês | MEDLINE | ID: mdl-29407852

RESUMO

This study aimed to investigate the penetration rate of child protection teams (CPTs) in medical institutions and associations between CPT functions and hospital services. We collected data in October of 2015 from 377 hospitals in Japan offering pediatric organ transplantation. The questionnaire included questions regarding the existence of a CPT, the number of child maltreatment cases discussed and reported per year, CPT functions including 21 items about staffing, manuals, meeting, prevention, education, and collaboration, and the services provided by the hospital. Of the 377 institutions, 122 (32.4%) answered the survey. There were significant associations between CPT functions and the number of pediatric beds (r = .27), number of pediatricians (r = .27), number of outpatients (r = .39), number of emergency outpatients (r = .28), and emergency medical care (p = .009). In a multiple regression analysis, CPT functions were significantly associated with the number of CPT members, pediatric outpatient numbers, and pediatric emergency outpatient numbers. Japan has no CPT guidelines that outline what CPTs should offer in terms of structure, staffing, functions, and systems. Hospitals with many pediatric and emergency outpatients are expected to play major roles in providing services such as specialty care, intensive care, and education. They are also expected to play a role in detecting and managing child maltreatment, and have, by their own initiative, improved their capacities to achieve these goals.


Assuntos
Maus-Tratos Infantis/prevenção & controle , Serviços de Proteção Infantil/estatística & dados numéricos , Equipe de Assistência ao Paciente/organização & administração , Criança , Maus-Tratos Infantis/estatística & dados numéricos , Proteção da Criança/estatística & dados numéricos , Estudos Transversais , Hospitais/estatística & dados numéricos , Humanos , Japão , Corpo Clínico Hospitalar/estatística & dados numéricos , Pacientes Ambulatoriais/estatística & dados numéricos , Equipe de Assistência ao Paciente/estatística & dados numéricos , Papel Profissional , Inquéritos e Questionários
8.
Sci Rep ; 7(1): 3895, 2017 06 20.
Artigo em Inglês | MEDLINE | ID: mdl-28634330

RESUMO

The majority of surveys on food webs of aquatic ecosystems have been conducted during the day owning to difficulties in sampling animals at night. In this study, to examine diurnal changes in predator-prey interactions in a temperate seagrass Zostera marina bed, a quantitative day/night survey of fish, the dominant animal community, coupled with acoustic telemetry of their predators, was conducted. The number of species, abundance, and biomass of piscivorous predators and mean trophic level during the night were significantly higher than those in the day in all seasons. Analysis of the stomach contents of 182 piscivorous predators showed that no fish predation occurred during the day whereas predation occurred during the night in winter, spring, and summer. Acoustic telemetry demonstrated nocturnal visits by dominant piscivorous fish species (rockfishes and conger eel) to the seagrass bed. We conclude that the nocturnal visits by piscivorous fishes increased the predation risk and trophic level in the fish nursery. The ecological functions of seagrass beds should be reevaluated accounting for day/night changes in food webs; these areas serve as nurseries for juvenile and small-sized fishes during the day and as foraging grounds for predators during the night.


Assuntos
Peixes , Comportamento Predatório , Animais , Ecossistema , Meio Ambiente , Cadeia Alimentar
9.
Pediatr Int ; 59(7): 764-768, 2017 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-28295898

RESUMO

BACKGROUND: In 2014, we created a training program for personnel in medical institutions in Japan to combat child maltreatment. The aim of the present study was to document the effectiveness of this program. METHODS: Participants completed a questionnaire before and after the training lecture. The questionnaire designed for the training program included demographic questions such years of practice and area of specialty (i.e. physician, nurse, social worker, public health nurse, technician, and others), as well as experience of suspected child maltreatment cases and training in dealing with such cases. The questionnaire included 15 statements designed to ascertain practical knowledge and attitudes relevant to addressing child maltreatment. Baseline score measured before the lecture was compared with that obtained after the lecture. RESULTS: A total of 760 participants completed the survey, including 227 physicians, 223 nurses, 38 technologists, 27 social workers, 11 public health nurses, and 174 with other occupations, and 60 participants who left their occupation as blank. There was a significant difference between the baseline score of participants with versus without experience in suspected child maltreatment or training to deal with child maltreatment (F = 16.3; P < 0.001). After the lecture, the average score rose above the baseline (11.18 vs 10.57). The rate of correct answers for nine questionnaire items increased significantly. CONCLUSIONS: Professionals from a range of fields need clinical skills and judgement to decide if a child's injuries are due to maltreatment. The combination of increased clinical experience along with a high-quality didactic lecture, appears to be the most effective method of raising awareness and enhancing skills.


Assuntos
Maus-Tratos Infantis/diagnóstico , Maus-Tratos Infantis/prevenção & controle , Educação Médica Continuada/métodos , Educação Continuada em Enfermagem/métodos , Pessoal de Saúde/educação , Atitude do Pessoal de Saúde , Criança , Competência Clínica , Humanos , Japão , Avaliação de Programas e Projetos de Saúde
10.
Proc Biol Sci ; 283(1832)2016 06 15.
Artigo em Inglês | MEDLINE | ID: mdl-27252017

RESUMO

There is a potential trade-off between grouping and the optimizing of the energetic efficiency of individual locomotion. Although intermittent locomotion, e.g. glide and upward swimming (GAU), can reduce the cost of locomotion at the individual level, the link between the optimization of individual intermittent locomotion and the behavioural synchronization in a group, especially among members with different sizes, is unknown. Here, we continuously monitored the schooling behaviour of a negatively buoyant fish, Pacific bluefin tuna (N = 10; 21.0 ∼ 24.5 cm), for 24 h in an open-sea net cage using accelerometry. All the fish repeated GAU during the recording periods. Although the GAU synchrony was maintained at high levels (overall mean = 0.62 for the cross-correlation coefficient of the GAU timings), larger fish glided for a longer duration per glide and more frequently than smaller fish. Similar-sized pairs showed significantly higher GAU synchrony than differently sized pairs. Our accelerometry results and the simulation based on hydrodynamic theory indicated that the advantage of intermittent locomotion in energy savings may not be fully optimized for smaller animals in a group when faced with the maintenance of group cohesion, suggesting that size assortative shoaling would be advantageous.


Assuntos
Tamanho Corporal , Natação , Atum/fisiologia , Animais , Oceano Pacífico
11.
Springerplus ; 4: 412, 2015.
Artigo em Inglês | MEDLINE | ID: mdl-26266083

RESUMO

BACKGROUND: Low competency for determination of brain death (BD) and unfamiliarity with Japanese BD (JBD) criteria among pediatricians were highlighted in previous nationwide studies. Because the JBD criteria were amended in 2010 to allow organ donation from pediatric brain-dead donors, we created a 2-day training course to assess knowledge and improve skill in the determination and diagnosis of pediatric BD. METHODS: The course consisted of two modules: a multistation round session and a group discussion session, and was bookended by a before and after 20-question test. In the multistation round session, participants rotated between stations staffed by expert faculty members. For hands-on skill development, we used the Sim Junior 3G™ simulation mannequin (Laerdal Medical, Wappingers Falls, NY, USA) for structured simulations. In the group discussion session, we implemented simulation-based role playing to practice decision making in prepared scenarios of complicated clinical situations. We investigated the participants' impressions of the course by self-scoring and questionnaires. RESULTS: Of 147 pediatric healthcare providers from multiple specialties who participated in this course, 145 completed the entire process. The course was evaluated in three aspects with self-scoring and questionnaires: (1) value (4.58 ± 0.64; range 1-5); (2) time schedule (2.40 ± 0.61; range 1-3); and (3) difficulty (2.89 ± 0.43; range 1-5). Finally, participants scored the entire course program (9.64 ± 1.69; range 1-11). Various positive feedbacks were obtained from a total of 93 participants. Post-test scores (83.6 %) were significantly higher than pre-test scores (62.9 %). CONCLUSION: This simulation-based course represents an effective method to train pediatric healthcare providers in determining BD in Japan and may improve baseline knowledge of BD among participants.

12.
J Exp Biol ; 215(Pt 16): 2751-9, 2012 Aug 15.
Artigo em Inglês | MEDLINE | ID: mdl-22837447

RESUMO

Sedentary and territorial rockfish of the genus Sebastes exhibit distinctive homing ability and can travel back to an original location after displacements of metres or even kilometres. However, little is known about the behavioural and sensory mechanisms involved in homing. Although our previous study demonstrated that nocturnal black rockfish Sebastes cheni predominantly use their olfactory sense for homing from an unfamiliar area, the possibility of using landmarks in a familiar area cannot be discounted; i.e. site-specific fish are likely to use three-dimensional spatial memory for navigation and orientation. Using high-resolution acoustic telemetry, we investigated whether S. cheni exhibit distinctive homing paths. Results show that all of the eight rockfish increased their effort within a small area of an unfamiliar region around the release site just after displacement, suggesting that the rockfish probably searched for the homeward direction. The rockfish showed the search movement in the upstream and/or downstream direction, which did not lead home. Finally, after returning to their familiar area, the rockfish exhibited more directed movements with faster speeds at a shallower depth, which was similar to the depth utilised in daily life as well as that of the fish capture.


Assuntos
Ecossistema , Peixes/fisiologia , Comportamento de Retorno ao Território Vital/fisiologia , Movimento/fisiologia , Orientação/fisiologia , Animais , Baías , Japão , Natação/fisiologia , Fatores de Tempo , Movimentos da Água
13.
J Acoust Soc Am ; 129(6): 3623-9, 2011 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-21682387

RESUMO

Dugongs (Dugong dugon) produce bird-like calls such as chirps and trills. The vocal responses of dugongs to playbacks of several acoustic stimuli were investigated. Animals were exposed to four different playback stimuli: a recorded chirp from a wild dugong, a synthesized down-sweep sound, a synthesized constant-frequency sound, and silence. Wild dugongs vocalized more frequently after playback of broadcast chirps than that after constant-frequency sounds or silence. The down-sweep sound also elicited more vocal responses than did silence. No significant difference was found between the broadcast chirps and the down-sweep sound. The ratio of wild dugong chirps to all calls and the dominant frequencies of the wild dugong calls were significantly higher during playbacks of broadcast chirps, down-sweep sounds, and constant-frequency sounds than during those of silence. The source level and duration of dugong chirps increased significantly as signaling distance increased. No significant correlation was found between signaling distance and the source level of trills. These results show that dugongs vocalize to playbacks of frequency-modulated signals and suggest that the source level of dugong chirps may be manipulated to compensate for transmission loss between the source and receiver. This study provides the first behavioral observations revealing the function of dugong chirps.


Assuntos
Dugong/fisiologia , Vocalização Animal , Estimulação Acústica , Acústica/instrumentação , Animais , Limiar Auditivo , Análise de Fourier , Processamento de Sinais Assistido por Computador , Espectrografia do Som , Fatores de Tempo , Transdutores
14.
J Acoust Soc Am ; 126(4): 1954-9, 2009 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-19813807

RESUMO

Dugongs (Dugong dugon) were monitored using simultaneous passive acoustic methods and visual observations in Thai waters during January 2008. Chirp and trill calls were detected by a towed stereo hydrophone array system. Two teams of experienced observers conducted standard visual observations on the same boat. Comparisons of detection probabilities of acoustic and visual monitoring between two independent observers were calculated. Acoustic and visual detection probabilities were 15.1% and 15.7%, respectively, employing a 300 s matching time interval. When conspecific chirp calls were broadcast from an underwater speaker deployed on the side of the observation boat, the detection probability of acoustic monitoring rose to 19.2%. The visual detection probability was 12.5%. Vocal hot spots characterized by frequent acoustic detection of calls were suggested by dispersion analysis, while dugongs were visually observed constantly throughout the focal area (p<0.001). Passive acoustic monitoring assisted the survey since detection performance similar to that of experienced visual observers was shown. Playback of conspecific chirps appeared to increase the detection probability, which could be beneficial for future field surveys using passive acoustics in order to ensure the attendance of dugongs in the focal area.


Assuntos
Dugong , Vocalização Animal , Estimulação Acústica , Acústica/instrumentação , Animais , Animais Selvagens , Modelos Estatísticos , Oceanos e Mares , Probabilidade , Espectrografia do Som , Fatores de Tempo
15.
J Acoust Soc Am ; 120(3): 1356-60, 2006 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-17004458

RESUMO

Little is known about feeding behavior of wild dugongs (Dugong dugon) because direct measurements of feeding events in the water were scarcely feasible. In this study, the authors achieved the first successful feeding sound monitoring in a seagrass area using a full-band underwater recording system (called automatic underwater sound monitoring system for dugong: AUSOMS-D). In total, 175 feeding sounds were identified in 205 h of recording. Feeding sounds were only detected at night, implying diurnal differences in the feeding behavior of the studied dugong population. Differences in periodicity of feeding sounds suggested that two or more individuals were in the acoustically observable area. Furthermore, a feeding position monitored by two AUSOMS-Ds was used to calculate source levels of dugong feeding sounds. Assuming spherical_propagation, source levels were measured between 70.6 and 79.0 dB rms re 1 microPa/square root of Hz.


Assuntos
Acústica/instrumentação , Dugong , Meio Ambiente , Comportamento Alimentar , Vocalização Animal , Animais , Ritmo Circadiano , Tailândia
16.
J Acoust Soc Am ; 119(6): 3726-33, 2006 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-16838515

RESUMO

To quantitatively examine the diurnal, or tidal, effects on dugong behavior, we employed passive acoustic observation techniques to monitor the animals. Automatic underwater sound monitoring systems for dugongs (AUSOMS-D) were deployed on the sea floor at depths of about 5 m south of Talibong Island, Thailand. The AUSOMS-D recorded underwater sound in stereo at a sampling frequency of 44.1 kHz for more than 116 consecutive hours. Dugong calls were automatically detected by newly developed software with a detection rate of 36.1% and a false alarm rate of 2.9%. In total, 3453 calls were detected during the 164 h of recording. The autocorrelation of the call rate indicated an attendance cycle of about 24 or 25 h, and the most frequent vocalizations were observed from 0300 to 0500 h. The calculated bearings of the sound sources, i.e., dugongs, were used as an indicator to track the relative numbers of dugongs during the monitoring periods.


Assuntos
Ritmo Circadiano/fisiologia , Dugong/fisiologia , Espectrografia do Som/veterinária , Vocalização Animal/fisiologia , Animais , Comportamento Animal/fisiologia , Espectrografia do Som/instrumentação , Espectrografia do Som/métodos , Tailândia , Vocalização Animal/classificação , Movimentos da Água
17.
Pediatr Int ; 48(2): 105-11, 2006 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-16635166

RESUMO

BACKGROUND: The occurrence of male patients with ornithine transcarbamylase (OTC) deficiency during adolescence or in adulthood has now been recognized. The aim of this study was to determine the prognostic factors that affect the prognosis of life, to explore a basis for therapeutic strategy. METHODS: In 10 patients, nine of whom carried the R40H mutation and the other one carrying the Y55D mutation in the OTC gene, 32 demographic and laboratory data were first compared between survivors and non-survivors, using the unpaired t-test. The factors with significant difference were then subjected to multiple regression analysis. RESULTS: The factors that exhibited significant difference were: age at onset, concentration of plasma ammonium, blood pH, and concentrations of six amino acids in plasma. The multiple regression analysis then revealed concentrations of ammonium, leucine, lysine, isoleucine, phenylalanine, glutamine and proline to be significant prognostic factors. The amino acid profile in the 10 patients showed increases in glutamine, proline, lysine, valine and methionine, and decreases in serine, ornithine and arginine. There was an inverse correlation between the age at onset and the level of the residual hepatic OTC activity. CONCLUSION: The results implied that: (i) the plasma amino acid profile was unique, in comparison to other liver diseases; (ii) the plasma concentration of each of the (mentioned above) six amino acids was a significant predictor of prognosis; and (iii) suppression of protein catabolism, as suggested by the higher concentrations in isoleucine and leucine in the non-survivors, prevention of glutamine-induced brain edema, correction of alkalosis, and supplementation with ornithine or arginine may improve the prognosis of life.


Assuntos
Aminoácidos/sangue , Doença da Deficiência de Ornitina Carbomoiltransferase/sangue , Doença da Deficiência de Ornitina Carbomoiltransferase/epidemiologia , Adolescente , Adulto , Idade de Início , Amônia/sangue , Criança , Glutamina/sangue , Humanos , Fígado/enzimologia , Lisina/sangue , Masculino , Pessoa de Meia-Idade , Doença da Deficiência de Ornitina Carbomoiltransferase/diagnóstico , Prognóstico , Análise de Regressão
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