Asthmatic patients with psychosis need special care.
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Biomedical subjects
Publications and source records attributed to M L Levy.
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Controversy surrounds the benefits of routine surveillance magnetic resonance or computed tomographic imaging for monitoring children after resection of primitive neuroectodermal tumors. A recent study suggested that serial imaging studies detect only a small minority of tumor recurrences in patients with symptoms. The authors concluded that even in patients with recurrence documented by imaging, no patient with a recurrence survived long (average, 5 mo) and that surveillance scanning is of little clinical value in children with primitive neuroectodermal tumors. We reviewed our experience with 25 patients (28% of our total series) who presented to the Children's Hospital Los Angeles, Los Angeles, CA, from 1985 to 1993 with recurrent tumors after surgery and adjuvant therapy. Recurrent tumors were detected on routine imaging in 19 asymptomatic patients (76%) and in 6 symptomatic patients (24%). Recurrences were documented 15 months (mean) after the initial diagnosis in asymptomatic children and 5 months (mean) after the initial diagnosis in children with symptoms of recurrent tumor (P < or = 0.01). Asymptomatic patients with recurrence documented on serial imaging had prolonged survival when compared with those who were symptomatic (P < or = 0.05). The surviving patients with recurrence remained alive for more than 24 months after documentation of recurrence. Early detection of local tumor recurrence by surveillance scanning may provide a critical therapeutic window for successful treatment with aggressive or novel therapies.
With the continued use of bone marrow transplantation in the treatment of malignancies, immunodeficiencies, and some metabolic diseases in children, it remains important for the dermatologist to be aware of the clinical and diagnostic features, and pathophysiology of Graft vs. Host disease. Specifically, the dermatologist should keep in mind that the skin is a primary site for involvement in this process. Recognition of the spectrum of cutaneous disease, its methods of laboratory diagnosis, and treatment should be known to those physicians caring for patients undergoing this type of therapy. A brief review of these and other aspects of Graft vs. Host disease is presented in this report.
Incontinentia pigmenti (IP) is a genodermatosis that segregates as an X linked dominant trait with male lethality. The disease has been linked to Xq28 in a number of studies. A few affected males have been documented, most of whom have a 47,XXY karyotype. We report a family with two paternally related half sisters, each affected with IP. The father is healthy, clinically normal, and has a 46,XY normal male karyotype. Linkage analysis of 12 polymorphic markers (two X linked and 10 autosomal) confirms paternity. X inactivation studies with the human androgen receptor (HUMARA) indicate that the paternal X chromosome is inactivated preferentially in each girl, implying that this chromosome carries the IP mutation, and that the father is a gonadal mosaic for the IP mutation.
A 1-month-old infant with a Dandy-Walker malformation underwent the placement of a ventriculoperitoneal shunt to treat progressive hydrocephalus. On the initial computed tomographic scan, a subependymal cystic lesion larger than 1 cm in diameter was noted in the head of the right caudate nucleus, which enlarged to 3 cm in diameter on follow-up imaging studies over the course of a year. Biopsies taken at the time of fenestration failed to show the presence of neoplasia. This appears to be the first reported case of a cyst of this nature to show progressive enlargement.
Continued elevations in Intracranial Pressure (ICP) following traumatic or ischemic compromise are known to cause markedly increased morbidity and mortality. Because of the side effects of barbiturates including hypotension and prolonged recovery time, the use of shorter-acting anesthetic agents to control ICP has been considered. Etomidate, when administered by continuous infusion, has been shown to decrease cerebral metabolism resulting in a secondary decrease in cerebral blood flow with minimal changes in cerebral perfusion pressure. We initially intended to randomize 20 patients prospectively into a study protocol that would assess the effects of either pentobarbital or the cardioprotective agent etomidate on ICP and cardiac performance. Given the sequelae of the therapy, we were only able to randomize seven patients with cerebral edema refractory to medical management to receive either etomidate or pentobarbital in a blinded fashion. Three patients who received etomidate developed renal compromise (mean low creatinine clearance 41 ml/min, range 37-44 ml/min) which was initially noted at 24 hours. We believed that this represented an adverse effect that was probably related to the study drug and the study was stopped. Each patient received a 0.30 mg/kg IV induction of etomidate and then 0.02 mg/kg/min continuous infusion for 24-72 hours titrated burst suppression. All patients also received dexamethasone 2 mg IV every six hours to prevent the adrenocortical insufficiency that might occur as a consequence of etomidate-induced suppression of cortisol synthesis. Intracranial pressure decreased (mean = 12mmHg) following the initiation of etomidate. Cardiac parameters remained unchanged (cardiac output 4.8 +/- .6 liters/min).(ABSTRACT TRUNCATED AT 250 WORDS)
OBJECTIVE: In 1991, we noted the emergence amongst our extremely low birth weight neonates of a new clinical entity, invasive fungal dermatitis, characterized by erosive, crusting lesions and a high rate of subsequent systemic fungal infection. We sought to define this condition and examine potential risk factors. METHODS: Sixteen neonates with invasive fungal dermatitis were seen during a 2-year period in three Baylor College of Medicine affiliated intensive care nurseries. Seven were confirmed cases, with skin biopsy evidence of invasion beyond the stratum corneum. Nine had a consistent clinical course and a positive potassium hydroxide examination of skin scrapings or isolation of fungi from skin or systemic cultures. Three controls were matched to each case by hospital, date of admission, and birth weight. Data was collected by retrospective chart review. RESULTS: Invasive fungal dermatitis occurred in 5.9% of at-risk infants. Case patients had a mean birth weight of 635 g and developed skin lesions at a mean age of 9 days (range, 6 to 14). Candida albicans was the most commonly implicated pathogen, but other Candida species, Aspergillus, Trichosporon beigelii, and Curvularia were also seen. Disseminated infection occurred in 69%, all due to Candida sp. Case patients were significantly more premature than controls (mean gestation, 24.4 vs 25.9 weeks) and were more likely to be delivered vaginally (81% vs 50%). Postnatal steroids were administered to cases (81%) more often than controls (46%). Case patients had more prolonged hyperglycemia (as assessed by insulin administration) than controls (mean 4.3 vs 2.0 days). CONCLUSIONS: Invasive fungal dermatitis is a disease of the smallest, most immature neonates and is associated with vaginal birth, steroid administration, and hyperglycemia. We speculate that the skin serves as a portal of entry for colonizing fungal species and may thus lead to disseminated infection. Methods to improve skin barrier function may be useful in preventing this disorder.
The literature reveals several ominous trends in firearm injuries in the United States. The crack epidemic of the mid-1980s, the increasing availability of handguns and more lethal weapons, and, particularly in Southern California, the rise of gang violence have all contributed significantly to this trend. Although the crack epidemic seems to have abated, the weapons it produced and the criminal elements it encouraged are still around. Many public health experts have advocated gun control as a means to stop this violence, and they have several compelling examples to justify their efforts. Gun control, however, is beyond the scope of this article. Neurosurgeons can have some effect at the local level on gang violence, however. The University of Southern California Department of Neurological Surgery has been working with the Think First Organization and Community Youth Gang Service Organization to reach out to children before they become involved with the gangs. These organizations are also working with the justice system to reform young people already involved with gangs. The hope is that these patterns of violence can be arrested on the community level.
Even this information is only partial. To study fully the effects of treatment would require optimal care at all points from time of injury, including rapid prehospital resuscitation, rapid transport to an optimally equipped and staffed hospital, immediate evaluation and treatment of the initial injury and all complications, rapid and comprehensive rehabilitation, and supportive and flexible home and work settings for the patient on discharge. Patients would need to be stratified for premorbid characteristics, including intelligence, personal traits, and training. Prolonged follow-up, possibly for several years, would be required to determine true outcome. No current study contains sufficient numbers of patients treated optimally and studied for prolonged periods, but this should be done. One way of looking at such patients is to decide that many should be treated to salvage a few. The other way of looking at them is that so many must receive care, at great emotional and economic cost to themselves and others, that such treatment is inappropriate for any of them. Treating all such patients would be a major undertaking. If most of these patients were treated vigorously, a great proportion of them would still die but probably not for a number of days. During this period, their families would be under extreme stress. Once stabilized and receiving ongoing care, some patients would enter a permanent vegetative state and survive for prolonged periods until their prognosis was clear and care was withdrawn, again causing family stress as well as high cost. Some would likely survive although impaired. The charges and real costs of care for all these patients would be tremendous. The question therefore arises as to how to decide what to do about caring for a large group of patients whose maximal care would be costly in emotional and financial terms, particularly at a time when it is recognized that resources for medical care are going to be limited. When discussing such patients as a group with a view toward developing practice guidelines, many considerations must be brought to bear. One consideration is the certainty of the prognosis in both a quantitative and a qualitative sense in an individual case. It is not clear that one can be certain in patients except when there are overwhelmingly unfavorable features. As has been noted, even patients who have been shot through the geographic center of the brain and are posturing can make excellent recoveries. This would push toward aggressive treatment for many patients. Decision making must therefore be considered in terms of bioethics. The major principle-based systems of bioethics are deontologic, arising from accepted principles, and utilitarian, arising from effect on outcome. A virtue-based ethic for physicians arising from "the caring bond and the public trust" is being revived as a balance to analytical ethics. A similar orientation from the point of view of patients is communitarian ethics, that is asking for only what is reasonable and not so much as might harm others. Some of the issues to be considered include the sanctity of life while taking into account the criteria for life--vegetative function versus some level of mental function. One must also review each decision from the viewpoints of all the parties involved--patients, family and friends, physicians, and society--in the context of a heterogeneous society in which individual rights and tolerance enforced by law are primary features. In the patients' terms, there is a desire and right to medical care to maintain a healthy productive life. Even if impaired to some extent, patients may still have an interest in living. Balancing benefits and burdens of life is a complex problem. There is also the right, based on patients' values, to refuse care if there is the wish not to take a chance of having a significantly compromised existence. Such declaration before injury should be honored...
Vascular disruption secondary to penetrating head trauma is most commonly in the form of surgically treatable intracranial and intracerebral hemorrhage. This article discusses the management of major cerbrobascular lesions associated with penetrating injuries to the neck and cranial vault, excluding traumatic aneurysms.
D-2-Hydroxyglutaric aciduria was documented in a newborn who presented with seizures, hypotonia, cortical blindness, a movement disorder, and developmental delay. Her clinical presentation differs from that of patients with L-2-hydroxyglutaric aciduria and a single previously reported patient with D-2-hydroxyglutaric aciduria. Cerebrospinal fluid levels of gamma-aminobutyric acid were elevated, while biogenic amine metabolites were normal. The movement disorder in our patient and in those with L-2-hydroxyglutaric aciduria suggests involvement of the basal ganglia in the disease process. Prenatal diagnosis of an affected fetus was accomplished during a subsequent pregnancy.
A 17-year-old youth had a solitary basal cell carcinoma of the nose. This malignancy is rare in children and usually occurs with another disease or syndrome. The de novo form in this patient is especially rare.
Epidermolysis bullosa (EB) represents a group of rare hereditary mechanobullous disorders marked by blister formation following relatively minor trauma. There are three categories depending on the site of disruption within the skin: simplex (above the basement membrane), dystrophic (below the basement membrane), and junctional (at the lamina lucida). Laryngeal involvement is rare, but has been reported primarily in association with junctional EB. Presented is our experience with 5 children with EB who demonstrated laryngeal involvement. Three children required tracheostomies acutely and 1 died of airway obstruction. One patient, with the Dowling-Meara type of EB simplex, was treated conservatively with good results. Tracheostomy should be an early consideration in the management of these patients to protect the airway in the acute setting and to prevent any further laryngeal injury associated with endotracheal intubation. The rate of complications of tracheostomy appears to be low; however, the prognosis for decannulation is poor.
In an attempt to evaluate the response of patients who have low admission Glasgow Coma Scale scores (GCS) after a penetrating craniocerebral injury to aggressive management, we evaluated a series of 190 patients with penetrating injuries who presented with a GCS score of 3, 4, or 5 during a 6-year period. Entrance criteria required replicable neurological examinations that were not altered by the presence of hypotension, drugs/toxins, or systemic injury. The surgical patients included 21 patients with an admission GCS score of 3, 24 with an admission GCS score of 4, and 15 with an admission GCS score of 5. All patients underwent surgical intervention and aggressive perioperative management in the neurosurgical intensive care, including resuscitative protocols. Five of the patients with a GCS score of 3 survived, all with poor outcomes. Seven of the patients with a GCS score of 4 survived, although only one had a good outcome. Eleven of the patients with a GCS score of 5 survived. Five had a Glasgow Outcome Score of 2, five had a Glasgow Outcome Score of 3, and one had a Glasgow Outcome Score of 4. We have devised a prospective model of outcome based on our series in an attempt to predict nonsurvivors at admission (while overpredicting for survivors). The variables most predictive of mortality include admission GCS score and subarachnoid hemorrhage in one model and admission GCS score and pupillary changes in a second, when pupillary response was definitive at admission (P < or = 0.00005).(ABSTRACT TRUNCATED AT 250 WORDS)
The use of a small-diameter, rigid ventriculoscope as a stylet to place a catheter optimally within cerebrospinal fluid-containing spaces, is described.
Three cases of fat graft prolapse into the cerebellopontine angle with clinical deterioration are presented. These patients had undergone translabyrinthine craniotomy for cerebellopontine angle tumors and had autologous abdominal fat strips placed to obliterate the dural defect and the mastoid cavity. Two patients suffered significant morbidity, and one patient died. Clinical presentation, computed tomographic scan findings, methods of treatment, and avoidance of this complication are discussed.
This article discusses guidelines the authors have established in managing patients with ruptured intracranial aneurysms and associated vasospasm. These guidelines represent methods of improving cardiac output or the ideal cardiac indices, to improve or maximize cerebral blood flow and potentially improve outcome. The guidelines for hypervolemic therapy for the volume and timing of intravenous fluid administration and the target cardiac performance parameters also are discussed.
A wide variety of unusual genodermatoses may be encountered by physicians during training or later in their practice. We have briefly described some of these and have outlined laboratory studies that may aid in their diagnosis.