The carnitine level of the umbilical cord blood in relation to the course of labor

Total and free carnitine were analyzed in cord blood of 24 newborn babies, with uncomplicated delivery, and 12 neonates with protacted labour. At birth, the mean neonatal carnitine value (total 33.7 +/- 10.1; free 23.5 +/- 7.5 mumol/l) was lower than the mean adult value (60.0 +/- 10.0 and 50.5 +/- 7.3, resp.). The carnitine values in the group with protracted labour increase slowly with duration of delivery. In our opinion carnitine values of neonates are not suitable for diagnosis of systemic carnitine deficiency syndrome.     

对我国儿童肾移植发展的思考与建议

肾移植是儿童终末期肾衰最重要的替代治疗手段。国内数家大的移植中心儿童肾移植长期存活率已接近国外先进水平,但我国肾移植患儿总数较少,主要原因是经费不足和供体缺乏。为推动儿童肾移植的发展,建议建立国家医疗救助制度,鼓励亲属活体肾脏捐献,严格掌握适应证,合理使用免疫抑制剂,加强多中心协作,建立移植随访网络。     

难治性肾病综合征典型病例的临床决策

难治性肾病综合征在肾内科临床实践中并不少见,其主要治疗是联合应用激素和细胞毒药物（如环磷酰胺、环孢素等）。少数患者,经以上联合治疗后仍不能达到临床缓解。而对于这些患者,长期使用激素及细胞毒药物不仅不能取得满意的效果,而且可能出现严重感染等并发症,甚至危及患者的生命,故在临床中应权衡利弊进行治疗决策。     

移植排斥反应中若干问题的思考

随着器官移植术和免疫抑制药物的发展,器官移植已成为挽救各类终末期器官功能衰竭患者的最有效手段。然而,移植排斥反应依然是困扰移植学界的难题。移植排斥反应的发生机制和治疗过程中体现了矛盾的普遍性和特殊性,用辩证思考的方法可以有效地指导移植排斥反应机制研究和临床治疗。     

Psychotherapeutic Interventions and Processes

Cognitive behavioral therapy (CBT) has been one of the most influential developments in psychiatry ever. Since it was developed and introduced, a vast number of treatment protocols focused on specific psychiatric syndromes have been developed. Although this has benefitted many patients and advanced the field, CBT as a treatment with its focus on alleviating psychiatric syndromes seemed to have reached a plateau and a process-focus approach is now emerging within the family of CBT models. This represents a new form of idiographic functional analysis guided by models that integrate a coherent set of change processes. Here, I describe the foundations of this new approach to mental health, called process-based therapy (PBT) and discuss its scientific and clinical implications.     

Comparison between two opposite homicidal syndromes (Syndrome e vs. limbic psychotic trigger reaction)

Two syndromes with opposite kinds of homicidal acts also implicate opposite activation of frontal lobe and limbic systems. I. Fried's “Syndrome E” refers to planned, ideologically-motivated mass murders, committed in groups and accompanied by “elation.” Implicated are heightened functioning of certain frontal lobe regions with certain reduced limbic involvement . In contrast, A. A. Pontius' “Limbic Psychotic Trigger Reaction” delineates unplanned, motiveless homicidal acts (so far 17 cases) committed by a single loner with flat affect, implicating a partial limbic seizure hyperactivation with fleeting psychosis and brief secondary dysfunctioning of certain frontal regions.     

Antidepressants and cognitive-behavioral therapy for symptom syndromes

Somatic symptoms are common in primary care and clinicians often prescribe antidepressants as adjunctive therapy. There are many possible reasons why this may work, including treating comorbid depression or anxiety, inhibition of ascending pain pathways, inhibition of prefrontal cortical areas that are responsible for "attention" to noxious stimuli, and the direct effects of the medications on the syndrome. There are good theoretical reasons why antidepressants with balanced norepinephrine and serotonin effects may be more effective than those that act predominantly on one pathway, though head-to-head comparisons are lacking. For the 11 painful syndromes review in this article, cognitive-behavioral therapy is most consistently demonstrated to be effective, with various antidepressants having more or less randomized controlled data supporting or refuting effectiveness. This article reviews the randomized controlled trial data for the use of antidepressant and cognitive-behavior therapy for 11 somatic syndromes: irritable bowel syndrome, chronic back pain, headache, fibromyalgia, chronic fatigue syndrome, tinnitus, menopausal symptoms, chronic facial pain, noncardiac chest pain, interstitial cystitis, and chronic pelvic pain. For some syndromes, the data for or against treatment effectiveness is relatively robust, for many, however, the data, one way or the other is scanty.     

胃癌辅助化疗的选择和应用

局部进展期胃癌手术后是否给予全身辅助化疗以及关于标准方案的临床研究,一直是临床关注的热点问题,对ⅡB期以上的进展期胃癌术后给予辅助治疗已经成为共识,但对于采用何种方案作为标准方案尚缺乏循证学依据,而且东西方医学界的意见存在较大的争议,我们一方面期待具有更高级别证据的临床研究结果,另一方面在现有证据下对胃癌术后进行最大程...     

A Case of Partial Biotinidase Deficiency Associated With Autism

We report the case of a child with partial biotinidase deficiency and autistic developmental disorder. We arrived at the diagnosis of biotinidase deficiency when the child was almost 4 years of age. Consequently, he began cofactor biotin treatment (10 mg daily) which did not resolve his autistic behavior. His younger brother was affected by partial biotinidase deficiency diagnosed at birth through our neonatal screening program. He was precociously treated with cofactor biotin therapy (10 mg daily) and did not show any behavioral abnormality or developmental delay. Since the brain is quite vulnerable to biotin deficiency, delayed biotin therapy could result in neurological damage. Our patient is the first case of partial biotinidase deficiency associated with autism. We hypothesize that the low biotinidase activity could have caused biotin deficiency in his brain and cerebrospinal fluids and consequently serious neurological problems, such as stereotyped and autistic behaviors, which were irreversible in spite of biotin supplementation.     

A case of partial biotinidase deficiency associated with autism.

We report the case of a child with partial biotinidase deficiency and autistic developmental disorder. We arrived at the diagnosis of biotinidase deficiency when the child was almost 4 years of age. Consequently, he began cofactor biotin treatment (10 mg daily) which did not resolve his autistic behavior. His younger brother was affected by partial biotinidase deficiency diagnosed at birth through our neonatal screening program. He was precociously treated with cofactor biotin therapy (10 mg daily) and did not show any behavioral abnormality or developmental delay. Since the brain is quite vulnerable to biotin deficiency, delayed biotin therapy could result in neurological damage. Our patient is the first case of partial biotinidase deficiency associated with autism. We hypothesize that the low biotinidase activity could have caused biotin deficiency in his brain and cerebrospinal fluids and consequently serious neurological problems, such as stereotyped and autistic behaviors, which were irreversible in spite of biotin supplementation.     

Risk and resiliency processes in ethnically diverse families in poverty.

Families living in poverty face numerous stressors that threaten the health and well-being of family members. This study examined the relationships among family-level poverty-related stress (PRS), individual-level coping with PRS, and a wide range of psychological symptoms in an ethnically diverse sample of 98 families (300 family members) living at or below 150% of the federal poverty line. Hierarchical linear model (HLM) analyses revealed that family PRS is robustly related to a wide range of psychological syndromes for family members of both genders, all ages, and all ethnic backgrounds. In addition, primary and secondary control coping were both found to serve as buffers of PRS for many syndromes. For several psychological syndromes, parents showed significantly higher levels of symptoms, but the link between PRS and symptoms was significantly stronger for children than for adults. Ethnicity was not a significant predictor in overall HLM models or follow-up analyses, suggesting that the broad construct of PRS and the theoretical model tested here apply across the 3 major ethnic groups included in this study. The findings suggest that family-based, coping-focused interventions have the potential to promote resiliency and break linkages in the pernicious cycle of family economic stress.     

Fluency disorders in genetic syndromes

The characteristics of various genetic syndromes have included “stuttering” as a primary symptom associated with that syndrome. Specifically, Down syndrome, fragile X syndrome, Prader-Willi syndrome, Tourette syndrome, Neurofibromatosis type I, and Turner syndrome all list “stuttering” as a characteristic of that syndrome. An extensive review of these syndromes indicated clients diagnosed with these syndromes do show evidence of nonfluency patterns, but not all would be considered stuttering. Many of the syndromes are marked by degrees of mental retardation that probably contribute to a higher than average prevalence of stuttering, as well as a higher than average prevalence of other fluency disorders (when compared to the population at large).

An in-depth analysis of the available data indicates that some of these genetic syndromes show patterns of stuttering that may be indicative of only that syndrome (or similar syndromes) that can be differentially diagnosed from developmental stuttering. Among these patterns are the word-final nonfluencies noted in Prader-Willi syndrome; the presence of stuttering in the absence of secondary behaviors noted in Prader-Willi syndrome and; the presence of palilalia, word-final and word-medial nonfluencies, and word-medial and word-final nonfluencies in Tourette syndrome. Implications for future research are discussed in light of these findings.

Educational objectives: The reader will be able to: (1) describe the various different genetic syndromes that are associated with fluency disorders; (2) describe the types of nonfluencies that are associated with the major types of genetic syndromes that have fluency disorders; (3) describe the behaviors that may assist in differentially diagnosing different types of speech characteristics associated with various genetic syndromes.     

Dilemmas in diagnosis and treatment of Gilles de la Tourette syndrome.

Gilles de la Tourette syndrome (TS) is a neurological disorder which has an inordinate risk of being diagnosed as psychogenic in nature because of commonly shared behavioral symptomes with syndromes of psychological origin. An overview of TS is presented including its history, symptomatology, and treatment of choice. The problems and pitfalls inherent in the diagnostic process which lead to psychogenic misconceptions are discussed. Treatment considerations include the secondary emotional problems and the negative consequences of the medication for TS. The implications for training professionals are discussed, but the essential point is that without an adequate history of the onset of symptoms, the potential for misdiagnosis is dramatically increased.     

Initial experiences with the Reiber 1979 determination of autochthonous IgG production within the scope of clinical and immunologic follow-up studies in multiple sclerosis treated with immunosuppressive therapy

We carried out clinical and immunological follows-up to 21 patients suffering from multiple sclerosis, 16 of whom had been immunosuppressively treated. A remission in clinical way was possible to point out with 8 of 12 patients (66%) -average remission by 1 score acc. to the Bronx-Scale- and with these patients a local formation of immunoglobulin acc. to Reiber (1979) could be proven. With 40 to 60% of the patients pathological changes were found out by determining of routine parameters by means of liquor examinations (total number of cells, sediment, total protein, electrophoresis) in which the humoral values responded not so sensitively to immunosuppressive therapy than the cellular values. For the present, liquor- and immunodiagnostic is being considered as a solid part of diagnostic programm and therapy control with regard to patients suffering from multiple sclerosis.     

Experimental allergic encephalomyelitis as a model for the study of therapeutic concepts for encephalomyelitis disseminata]

The induction of immunological tolerance with, and for, the caused organotypical antigen is a conception for a specific therapy for neuroimmunological diseases with at least a partial autoallergic pathogenesis. Appropriate to a set step-by-step programme for the development of antigen-specific therapy preventive tolerance experiments were carried out at the model of experimental allergic encephalomyelitis (EAEM) with allogenic myelin basic protein (BP) prepared from rabbits. The result is: 100 ug BP given intravenously simultaneously with 100 ug BP in incomplete Freud's adjuvant given intracutanously twice a week and 40 mg Cyclophosphamid given daily during the minor clinical incidence rate and no signs of EAEM pathomorphologically. A longlasting tolerance for the BP could be obtained as a test proved after 100 days. Hints are given for further potential therapeutic treatments, such as the use of antigen bound chemically to the immunosuppressive drug or the use of chemically modified BP for the induction of a specific tolerance.     

Intrinsic and extrinsic religiosity in preadolescence and adolescence: Effect on psychological adjustment

The aim of this study was to determine the prevalence of expectation of secondary gain among first-generation immigrant Turkish patients referred to a psychiatric hospital and whether this prevalence differs from that of Dutch outpatients. The study was carried out in the outpatient department of a Dutch psychiatric hospital. Expectation of secondary gain was assessed by directly asking the patients, via questionnaires whether they hoped to gain specific “benefits” from being in therapy. The study found that 31.7% of the Turkish patients reported holding expectations of secondary gain while being in therapy versus 42.2% of the Dutch patients. Our finding of a large proportion of Turkish patients with expectations of secondary gain is consistent with the results of an earlier study of Dutch patients. However, in contrast to the findings of empirical research, Turkish immigrants were not found more than Dutch patients to have expectations of secondary gain. In fact, such expectations were more common among Dutch patients.     

Neuropsychological syndromes in right hemisphere stroke patients

A specific set of syndromes, based on different intrahemispheric lesion locations, has not yet been described in patients with right hemisphere lesions. To explore whether statistically derived clusters could give clues to a syndrome structure, neuropsychological data from a sample of 106 patients with right hemisphere stroke were studied. CT data were available for 58 of the patients. Based on factor analysis of eight test and four ratings variables, six variables were chosen for a cluster analysis. A structure of 13 clusters was considered statistically valid. Combining clusters with parallel test profiles into main cluster classes, five right hemisphere syndromes proved clinically valid: an above average syndrome ( n =46), denial perseverance (frontal) syndrome ( n =14). a depressed mood syndrome ( n = 14), a focal RH syndrome ( n =18) and a global RH syndrome ( n =9). It is suggested that syndromes are related to intrahemispheric location of the lesion, such as the extent of anterior and posterior damage.     

The psychoanalytic view of phobias. Part I: Freud's theories of phobias and anxiety.

This paper initiates a series of communications on psychoanalytic and current psychiatric approaches to the understanding and treatment of phobic syndromes with a review and discussion of Freud's evolving ideas on phobias and anxiety. A list of issues that any theory of phobic syndromes must address is compiled on the basis of Freud's work.