Attempt to restore lymphocytes with defective T-cell function by mixing with normal cells in vitro

Blood leukocytes from a patient with T-cell immunodeficiency failed to respond to stimulation with phytohemagglutinin (PHA) in vitro although ample lymphocytes were present. Leukocytes from this patient were mixed in various proportions with normal leukocytes, and the mixtures were stimulated with PHA and cultured for three days. There was no restoration of PHA responsiveness of the patient''s cells in the presence of normal cells. This would indicate that the defect in the patient''s cell population was not due to the absence of any facilitating factor or cooperative function that could be provided by normal T cells or other blood leukocytes. Since in thymic aplasia the patient''s own cells are rapidly restored to normal PHA responsiveness by an unknown facilitating factor after thymic transplantation, we suggest that this mixed-cell culture method might differentiate between patients who need a bone marrow transplant and those who will respond to a thymus transplant.     

Lytic,nontransforming Epstein-Barr virus (EBV) from a patient with chronic active EBV infection.

A new wild-type isolate of Epstein-Barr virus (EBV) was identified in follow-up studies of a case of chronic active EBV infection in an 8-year-old girl who had high titres of antibody to viral capsid antigen and early antigen (EA) (greater than 20 480 and 2560 respectively), persistent splenomegaly and abnormal immunologic features. More than 10 throat washings from this patient failed to transform cord blood lymphocytes (CBL), but at least 7 were able to induce EA in Raji cells. Supernatants from cultures of the lymphoblastoid cell line obtained by in-vitro infection of this patient''s leukocytes with the B95-8 strain of EBV revealed a herpesvirus particle when examined by electron microscopy. The same supernatants were unable to transform CBL but could induce EA in Raji cells upon superinfection. In 30 or more trials the patient''s lymphocytes never transformed spontaneously but did become positive for EBV nuclear antigen and EA in the first week of culture at least twice. Parallel studies performed on the father of the patient yielded similar results. This, then, is the first report documenting lytic activity associated with a wild-type EBV isolate.     

Radiologic assessment in the pediatric intensive care unit.

The severely ill infant or child who requires admission to a pediatric intensive care unit (PICU) often presents with a complex set of problems necessitating multiple and frequent management decisions. Diagnostic imaging plays an important role, not only in the initial assessment of the patient''s condition and establishing a diagnosis, but also in monitoring the patient''s progress and the effects of interventional therapeutic measures. Bedside studies obtained using portable equipment are often limited but can provide much useful information when a careful and detailed approach is utilized in producing the radiograph and interpreting the examination. This article reviews some of the basic principles of radiographic interpretation and details some of the diagnostic points which, when promptly recognized, can lead to a better understanding of the patient''s condition and thus to improved patient care and management. While chest radiography is stressed, studies of other regions including the upper airway, abdomen, skull, and extremities are discussed. A brief consideration of the expanding role of new modality imaging (i.e., ultrasound, CT) is also included. Multiple illustrative examples of common and uncommon problems are shown.     

Reticulum cell sarcoma with unusual presenting features: fever and low cerebrospinal fluid glucose levels.

Reticulum cell sarcoma of the central nervous system usually presents as a focal mass lesion. A patient is described who presented with declining intellectual function, fever, and low glucose levels and pleocytosis in the cerebrospinal fluid, which initially suggested an infectious process. This clinical presentation has not been documented previously. The patient''s condition improved following treatment with dexamethasone and radiation.     

Fistules pulmonaires artério-veineuses

A case of multiple pulmonary arteriovenous fistulas is reported. Hereditary hemorrhagic telangiectasia is a characteristic associated finding, and in this instance affected 10 members of the patient''s family over four generations. This association suggests that the pulmonary condition in its congenital form is part of a generalized vascular dysplasia. Clinically, the patient experienced increased dyspnea and fatigue but cyanosis and polycythemia were not noted. After surgical excision of the fistula with conservation of as much pulmonary tissue as possible, prompt relief of symptoms was obtained. Furthermore, angiographic studies revealed that the small fistulas in the other lung did not enlarge. The presence of multiple fistulas is not a contraindication to surgery, and such fistulas should be excised to improve the patient''s condition and prevent further complications.     

Ethical conflicts in long-term care of the aged: nutritional problems and the patient-care worker relationship.

The patient-care worker relationship was analysed by observation and unstructured group discussion in four long-stay somatogeriatric wards at Saint Lars Hospital. Investigation centred on patients entering the terminal phase who could no longer be spoon-fed. The relationship was complicated and reciprocal, and failure by the care worker to interpret her role and the dying patient''s behaviour correctly led to emotional conflict and double-binding, with resultant anxiety for both herself and the patient. Infusions and tube-feeding prescribed in such cases were given not for the patient''s benefit but to relieve anxiety in care workers and relatives. Permitting the patient a natural, painless death from water deficiency may be preferable to prolonging pain and discomfort by intervening with infusion and tube-feeding.     

Effects of living with and looking after survivors of a stroke.

Information from a two year, longitudinal study on a community sample of patients with acute stroke was analysed to determine the effects of the stroke on the mood of the chief carer (the person living with the patient). Increased anxiety was the most commonly reported change six months after stroke. Significant depression was seen in 11-13% of carers over the first two years after stroke. The patient''s functional disability was associated with depression in the carer over the first year but not at two years. A perceived poor recovery by the patient, a low level of general activities by the patient, and depression in the patient were also associated with depression in the carer within the first year. At two years after stroke none of the measured factors were related to a carer''s level of depression. Carers of patients who have suffered stroke showed anxiety and emotional distress unrelated to the patient''s physical disability after two years. More help from stroke support groups for carers is perhaps needed.     

Ectopia cilia with pedigree analysis: Second case report in the world

We present a case of ectopia cilia in a 28-year-old male patient. Ectopia cilia was were seen in the outer third of left upper eyelid. The patient''s maternal grandfather also had ectopia cilia of the left upper eyelid as reported by the patient''s mother. Ectopia cilia is a rare condition seen in humans. Only 12 cases of ectopic cilia in humans have been reported so far in the world. The present case of ectopia cilia is the second case report in the world with pedigree analysis.     

The importance of patient selection for photochemotherapy in psoriasis.

Twenty-four patients with psoriasis were treated with orally administered 8-methoxypsoralen followed by exposure to high-intensity long-wavelength ultraviolet radiation (PUVA) at a psoriasis day care centre. Among the 20 with plaque type psoriasis the condition cleared in 13 (65%), after a mean of 20.7 treatment sessions, and improved but failed to clear in 4 (20%); the treatment failed in the other 3 (15%). The other four patients had erythrodermic, pustular or inflammatory psoriasis, and all failed to respond to PUVA therapy. Factors to be considered in patient selection for this form of therapy are the type of psoriasis, the patient''s skin type and th proportion of the body surface area involved.     

External irradiation of the hypophysis for Cushing's disease

During the past 12 years 17 patients with Cushing''s disease (bilateral adrenal hyperplasia secondary to excessive pituitary adrenocorticotrophic hormone) have been treated initially with external pituitary irradiation. Of the 15 patients who have had adequate follow-up, nine showed complete biochemical remission, and one showed biochemical improvement. There were no complications. It is therefore recommended that the first mode of therapy for all patients with Cushing''s disease should be pituitary irradiation if the patient''s clinical condition permits.     

Pseudohypertension secondary to a noncompressible brachial artery

The interesting problem is considered of a comatose alcoholic diabetic with an extremely high systolic blood pressure, as determined by the usual means, who was subsequently found to have severe medial calcinosis and normal intraarterial blood pressure. The syndrome of the noncompressible brachial artery surely accounts for this patient''s falsely elevated blood pressure reading. Though infrequently reported, this condition can be one cause of “difficult to control” hypertension in the elderly and in the diabetic patient.     

中耳术后并发耳廓软骨膜炎致耳廓畸形——1例病例报告及文献回顾

目的:通过报道1例中耳术后并发耳廓软骨膜炎致耳廓畸形患者的病历资料,以加强临床医师对该疾病的认识与防范。方法:对本例患者的病历资料进行回顾性阐述,结合该患者的临床特点揭示中耳术后并发耳廓软骨膜炎的发生发展过程,并通过文献回顾阐述该疾病的发病原因、病原学特点及治疗方法。结果:耳廓软骨膜炎是耳科手术术后较为棘手但相对少见的并发症,最为常见铜绿假单胞杆菌感染所致。治疗方法视患者病情而定,包括抗菌药物应用、脓肿切开引流、病变软骨切除术。对于合并糖尿病的老年患者中耳术后更易引发耳廓软骨膜炎,且疗程更长、预后更差。结论:中耳术后并发耳廓软骨膜炎临床需警惕,对合并糖尿病的老年患者应多加防范;早诊断,早治疗,避免增加患者痛苦、加重其经济负担,并与患者做好充分沟通,避免医疗纠纷的发生。     

Informed Consent in California: Latent Liability Without `Negligence'

Informed consent is a legal obligation due from a physician to his patient, an obligation which may not be met by the physician''s skillful treatment of his patient. It may only be met by the treating physician obtaining from his patient knowing authorization for carrying out the intended medical procedure. The physician is required to disclose whatever would be material to his patient''s decision, including the nature and purpose of the procedure, and the risks and alternatives. The disclosures should be made by the physician to his patient, and not through use of consent forms which are not particular to individual patients. To minimize any subsequent claim by the patient that there was a lack of adequate disclosures, the physician should record in the patient''s chart the circumstances of the patient''s consent, and should not rely on the patient''s unreliable ability to recall those circumstances.     

Legal obligation of physicians to disclose information to patients.

Obtaining a patient''s consent is a routine daily process for physicians, although many are unaware of the scope of this legal obligation. In 1980 the Supreme Court of Canada changed the law relating to informed consent; promotion of patient autonomy shifted the focus from a standard of professional disclosure to one of a "reasonable patient." Physicians have a legal obligation to disclose to patients specific information, the scope of which is determined by a court on the basis of a reasonable patient''s expectation and the circumstances of the case. This gives rise to many controversies in the practice of clinical medicine. It is difficult for physicians to know which treatment risks require disclosure, since this is decided by a court in a retrospective analysis of the evidence. Will the court recognize exceptions to the duty of disclosing information? If several health care professionals are involved in a patient''s care who has the duty to disclose information? Can this duty be delegated? This paper provides physicians with guidelines that are consistent with the promotion of patient autonomy and comply with the doctrine of informed consent. In addition, it suggests ways of improving awareness of the doctrine and procedures to ease its application.     

Cadaver nephrectomy: an operation on the donor's family.

Thirty-two relatives of cadaver kidney donors were interviewed six months or longer after the donor''s death. Most had positive attitudes to kidney transplantation that had been strengthened by experience, especially when they knew that they were fulfilling the donor''s wishes. Twenty-three of the relatives had gained some solace from knowing that others might benefit from their misfortune, but three claimed adverse effects. In identifying the factors that influenced them to grant permission or hesitate, relatives revealed defects in the way their permission had been sought. Twelve did not clearly understand the donor''s hopeless prognosis until then, and seven reacted adversely to the interviewers, finding them blunt and callous. Nevertheless, most were pleased that they had been asked. Doctors who care for unconscious, dying patients should try to give relatives explicit information on the patient''s condition, whether or not the patient is a potential kidney donor, and permission for organ recovery should not be sought until they understand that death is inevitable.     

When families cannot "let go": ethical decision-making at the bedside.

Shared decision-making at the bedside is now a regular feature of medical practice. When disagreements arise between a patient and family members caregivers sometimes find themselves caught in a complex tangle of human relationships that strains monochrome ethical thinking. The patient''s expressed wishes are often compromised for the sake of the family''s needs. Conversely, a unilateral appeal for patient autonomy may prove insensitive to the hurt and the needs of the family. We describe a relatively unsuccessful attempt by a patient''s caregivers to buy time to maximize the interests of the patient and her family and discuss the way in which the family dynamics militated against the rather obvious solution of promoting the patient''s right to refuse treatment. The purpose of this article is not to evoke sympathy for health care professionals in dealing with such conflicts but rather to heighten awareness of the issues at stake and to stimulate thinking about ways and means to bring about a more favourable outcome than the one described here.     

Withholding the artificial administration of fluids and food from elderly patients with dementia: ethnographic study

ObjectiveTo clarify the practice of withholding the artificial administration of fluids and food from elderly patients with dementia in nursing homes.DesignQualitative, ethnographic study in two phases.Setting10 wards in two nursing homes in the Netherlands.Participants35 patients with dementia, eight doctors, 43 nurses, and 32 families.ResultsThe clinical course of dementia was considered normal and was rarely reason to begin the artificial administration of fluids and food in advanced disease. Fluids and food seemed to be given mainly when there was an acute illness or a condition that needed medical treatment and which required hydration to be effective. The medical condition of the patient, the wishes of the family, and the interpretations of the patients'' quality of life by their care providers were considered more important than living wills and policy agreements.ConclusionsDoctors'' decisions about withholding the artificial administration of fluids and food from elderly patients with dementia are influenced more by the clinical course of the illness, the presumed quality of life of the patient, and the patient''s medical condition than they are by advanced planning of care. In an attempt to understand the wishes of the patient doctors try to create the broadest possible basis for the decision making process and its outcome, mainly by involving the family.

What is already known on this topic

Debate has focused on whether it is beneficial to withhold the artificial administration of fluids and food from patients with advanced dementia

What this study adds

The course of dementia, the patient''s quality of life, and the patient''s current medical condition influence doctors'' decision making more than advanced planning of careDoctors try to create the broadest possible basis for the decision making process and its outcome, mainly by involving the family     

Calciphylaxis in Man

A patient with renal failure developed widespread calcification in the thighs after the injection of iron-dextran (Imferon). This is considered as an example of calciphylaxis, a process in which calcium is laid down in parts of the body following the administration of a “challenger.” Iron-dextran is a known challenger, and should be used with caution in uraemic subjects, who may be sensitized by high serum parathyroid hormone levels. Contributing factors may have been a high calcium/phosphate product, steroids, and the patient''s immobility.     

青少年GATA2缺陷继发骨髓增生异常综合征一例并文献复习

摘要 目的：提高对青少年GATA2缺陷继发骨髓增生异常综合征（MDS）疾病的认识。方法：回顾性分析我院收治的1例青少年GATA2缺陷继发MDS患者的诊疗过程,并结合相关文献进行复习总结。结果：患者男,17岁,2018年6月于我科诊断为MDS(MDS-EB-I,IPSS中危-1;WPSS高危;IPSS-R高危),继发骨髓纤维化。完善血液遗传全外显子基因检查提示患者GATA2基因突变。修正诊断为GATA2缺陷综合征、继发MDS( MDS-EB-I,IPSS中危-1;WPSS高危;IPSS-R高危) 、继发骨髓纤维化。完善患者姐姐血常规检查提示白细胞轻度减少,检查患者姐姐GATA2基因检测到GATA2基因错义突变。患者治疗期间反复出现多部位感染。进一步检查患者父母GATA2基因提示患者父亲GATA2基因存在错义突变。患者GATA2基因突变系父系遗传。结论：对于青少年MDS患者,应对其进行血液遗传学全外显子基因检查以确认其有无先天性疾病;对于存在先天性基因突变的患者,建议行家系筛查,并尽早行造血干细胞移植治疗。     

GENERAL ANESTHESIA FOR EYE OPERATIONS—A Consideration of Some Pertinent Factors Involved in Administration

In the administration of general anesthesia for surgical operations on the eye, care must be taken to consider the patient''s total physiological condition. A patient with eye problems may have generalized changes of more than moderate extent. Most patients are in the age group in which the incidence of cardiovascular and pulmonary problems is relatively high. If the patient is in a younger age group, perhaps diabetes or the collagen diseases must be suspected. Care must be taken to prevent undue strains to the eye during and immediately after the operation.Constant care and an awareness of possible complication is necessary for successful management in these cases.