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PubMed1976

[Freshly frozen preserved plasma for the treatment of intravascular coagulation in polytraumatized patients].

Coagulation disorders in hemorrhagic shock need not represent an isolated intravascular coagulation. They may also occur as a complex of local disseminated intravascular consumption, extravascular consumption, dilution, and reduced synthesis of coagulation factors. In the severely bleeding patient with hemorrhagic diathesis heparin is contraindicated because it does not normalize coagulability. Therefore, it fails to stop hemorrhage and shock remains untreatable. Fresh frozen plasma, however, has proved to be suitable as simultaneous substitution therapy of coagulopathy and of hypovolemic shock. 11 patients suffering from traumatic-hemorrhagic shock associated with intravascular coagulation and hemorrhagic diathesis were successfully treated with fresh frozen plasma, after conventional shock therapy had failed over a period of hours.

Blood Preservation↗
PubMed1999

Dermatologic manifestations of Hermansky-Pudlak syndrome in patients with and without a 16-base pair duplication in the HPS1 gene.

BACKGROUND: Hermansky-Pudlak syndrome (HPS) consists of oculocutaneous albinism, a platelet storage pool deficiency, and lysosomal accumulation of ceroid lipofuscin. Patients with HPS from northwest Puerto Rico are homozygous for a 16-base pair (bp) duplication in exon 15 of HPS1, a gene on chromosome 10q23 known to cause the disorder. OBJECTIVE: To determine the dermatologic findings of patients with HPS. DESIGN: Survey of inpatients with HPS by physical examination. SETTING: National Institutes of Health Clinical Center, Bethesda, Md (a tertiary referral hospital). PATIENTS: Sixty-five patients aged 3 to 54 years were diagnosed on the basis of the absence of platelet dense bodies in individuals with albinism and a bleeding diathesis. The presence of a 16-bp duplication in HPS1 was determined by polymerase chain reaction amplification; 40 patients were homozygous for the duplication and 25 lacked the duplication. All patients with the duplication were from northwest Puerto Rico; all patients without the duplication were non-Puerto Rican except 4 from central Puerto Rico. RESULTS: Both patients homozygous for the 16-bp duplication and patients without the duplication displayed skin color ranging from white to light brown. Patients with the duplication, as well as those lacking the duplication, had hair color ranging from white to brown and eye color ranging from blue to brown. New findings in both groups of patients with HPS were melanocytic nevi with dysplastic features, acanthosis nigricans-like lesions in the axilla and neck, and trichomegaly. Eighty percent of patients with the duplication exhibited features of solar damage, including multiple freckles, stellate lentigines, actinic keratoses, and, occasionally, basal cell or squamous cell carcinomas. Only 8% of patients lacking the 16-bp duplication displayed these findings. As a group, the patients with the duplication lived closer to the equator than those without the duplication. CONCLUSION: Patients with HPS exhibit wide variation in pigmentation and dermatologic findings.

Adolescent↗
PubMed1984

Spontaneous factor XI inhibitors. Seven additional cases and a review of the literature.

Circulating anticoagulants are endogenous blood components that inhibit the action of clotting factors. In some inhibitor conditions this inactivation in the function of the hemostatic system may lead to life-threatening hemorrhagic diathesis. Inhibitors directed against factor XI are generally associated with little or no impairment of the hemostatic system. We analyzed all reported cases of spontaneous factor XI inhibitor in the international literature, as well as cases identified at the Yale--New Haven (Conn) Hospital between 1970 and 1980, considering clinical spectrum, diagnosis, and therapy.

Adolescent↗
PubMed1986

Multiple myeloma with polyneuropathy and coagulopathy. A case report of the polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin change (POEMS) syndrome.

A patient with the syndrome of polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes (POEMS) had multiple myeloma and IgA-kappa monoclonal gammopathy. To our knowledge, of over 50 cases in the literature, this is the only instance of kappa light chains noted in a patient with the complete syndrome. Sural nerve biopsy revealed increased immunofluorescence for IgA and kappa chains. Furthermore, this patient had a hemorrhagic diathesis characterized by a prolonged bleeding time and a prolonged thrombin time due to a qualitative platelet dysfunction and an apparent inhibition of fibrin monomer polymerization. In none of the other cases of POEMS syndrome was a coagulopathy reported. A short course of plasmapheresis improved the bleeding time but did not alter the polyneuropathy.

Blood Coagulation Disorders↗
PubMed2003

Anticoagulant-related bleeding in older persons with atrial fibrillation: physicians' fears often unfounded.

BACKGROUND: Many studies have documented the underuse of anticoagulant (ie, warfarin sodium) therapy as stroke prophylaxis in older persons with atrial fibrillation. Failure to prescribe anticoagulant agents to these patients is often due to physicians' perceiving the risk of major bleeding as unacceptably high because of the presence of such clinical risk factors as hypertension, falls, a history of gastrointestinal tract bleeding, and lack of assurance about compliance. OBJECTIVES: To critically appraise whether the presence of additional clinical factors that increase the risk of bleeding affects the chance of anticoagulant-related hemorrhage, and to develop an approach to the use of anticoagulant agents in older patients with atrial fibrillation who have any of these factors. METHODS: Systematic MEDLINE literature search from January 1966 to March 2002. RESULTS: Many of the factors that are purported to be barriers to anticoagulant therapy in older persons with atrial fibrillation probably should not influence the choice of stroke prophylaxis in these patients. These include previous episodes of upper gastrointestinal tract bleeding, predisposition to falling, and old age in itself. For some other factors, such as alcoholism, participation in activities that predispose to trauma, the presence of a bleeding diathesis or thrombocytopenia, and noncompliance with monitoring, there is little or conflicting evidence about their effect on anticoagulant-related bleeding. However, they should be considered in the clinical decision-making process. CONCLUSIONS: For many older patients with atrial fibrillation, physicians' fears of the risk of bleeding in association with anticoagulant therapy are often exaggerated and unfounded. Therefore, the salient issue in selecting older patients with atrial fibrillation for anticoagulation is accurately estimating their stroke risk, with bleeding risk during anticoagulation being a lesser issue, relevant to only a few patients.

Aged↗
PubMed1982

Complications of plasma exchange in neurological patients.

Complications occurred during 154 plasma exchanges performed in 17 patients for a variety of neurological disorders. Mild, self-limited, toxic reaction to citrate was the most frequent complication (7.8%), followed by transient hypotension (2.6%) and visual scotomata (1.3%). More serious complications, occurring on one occasion each, were cardiac arrhythmia, myocardial infarction, and gross hemolysis. There were no infections. The spectrum and frequency of complications in these plasma exchanges were compared with those in other series dealing with both neurological and nonneurological patients. Several risk factors for development of complications were identified, one of the most important being the nature of the illness being treated. The incidence of two of the most serious complications, severe infection and bleeding diathesis, appeared to be higher in patients with renal insufficiency or hematologic disorders, respectively, than in neurological patients.

Adolescent↗
PubMed1984

Cerebral ischemia in the presence of lupus anticoagulant.

Two patients with manifestations of cerebral ischemia were found to have a circulating coagulation inhibitor. This immunoglobulin, termed lupus anticoagulant, results in a prolonged partial thromboplastin time. Paradoxically, it is usually associated with a thrombotic tendency rather than a bleeding diathesis. It is most commonly found in systemic lupus erythematosus, which our patients did not have. These two patients represent the interesting phenomenon of cerebral ischemia in the presence of an endogenous inhibitor of coagulation.

Blood Coagulation Factors↗
PubMed1981

Verrucous carcinoma of the middle ear.

A case of a highly destructive, cytologically nondysplastic squamous epithelial lesion of the middle ear is presented. The cranial nerve involvement and bone destruction are more extensive than has been seen in cholesteatoma. Cultures are negative for Pseudomonas, and the patient does not have the reported diathesis for malignant otitis externa. The gross and microscopic features are those of verrucous carcinoma. To our knowledge, the middle ear has not been previously reported as a site of involvement by verrucous carcinoma.

Aged↗
PubMed1985

Intranasal dermal analogue tumor (membranous basal cell adenoma). Ultrastructure and immunohistochemistry.

To our knowledge, this is the first case of an intranasal monomorphic salivary gland adenoma (dermal analogue type) arising in a man exhibiting the salivary gland-skin adnexal tumor diathesis. Ultrastructurally, this tumor is composed of cells displaying squamous, secretory, luminal, and, very rarely, myoepithelial differentiation. Additionally, this is the first ultrastructural demonstration of intralobular Langerhans' cells in a dermal analogue tumor from any site. These Langerhans' cells can be demonstrated histologically by S-100 protein immunostaining. Epithelial tumor cells per se display no glial fibrillary acidic protein immunoreactivity, but rare epithelial cells stain faintly for S-100 protein. These findings seem to be distinct from pleomorphic adenoma and adenoid cystic carcinoma, the principal histologic differential diagnosis.

Adenoma↗
PubMed1991

Acquired von Willebrand's syndrome. Therapeutic and diagnostic implications.

Acquired von Willebrand's syndrome is a newly recognized bleeding diathesis thought to be caused by autoantibodies to the von Willebrand factor. Acquired von Willebrand's syndrome has been reported in association with lymphoproliferative disorders and benign monoclonal gammopathies. Clinical features and laboratory abnormalities of this disease are similar to congenital von Willebrand's disease, but the optimal treatment may differ. We describe a 75-year-old man with chronic lymphocytic leukemia and recurrent epistaxis and also discuss the pathogenesis, diagnosis, and treatment of both the congenital and acquired disorders.

Aged↗
PubMed1988

Continuous intracranial pressure monitoring and serial electroencephalographic recordings in severely asphyxiated term neonates.

We report our observations from intensive intracranial pressure (ICP) monitoring and serial clinical neurologic and electroencephalographic examinations in ten asphyxiated full-term neonates, of whom five died and at least two survivors had multiple severe neurologic handicaps. Direct measurements of ICP were obtained by a newly developed infant subarachnoid bolt and/or a transfontanelle pressure transducer. Simultaneous ICPs were recorded and correlated when possible. We noted a dependence of transfontanelle ICP values on application technique and force. In infants with no bleeding diathesis, the subarachnoid bolt was safe and no complications were encountered. Only six infants experienced pathologic elevations of ICP following birth asphyxia, and of these infants only two had sustained, marked increases of ICP. We also noted abundant fluctuations of cerebral perfusion pressure (mean arterial blood pressure minus ICP), but the majority of fluctuations were accounted for by mean arterial pressure changes rather than ICP changes. We found no deterioration of clinical neurologic function as measured by serial mental status examinations and electroencephalogram samples at the time the maximum ICP was measured. We also noted very little change in ICP during most electrographic seizures. In these infants ICP did increase after birth but major ICP elevations were uncommon and did not appear to introduce any acute functional neurologic disturbances. Most changes in cerebral perfusion pressure were attributed to blood pressure rather than ICP changes. It appears unlikely that cerebral edema and elevated ICP play a major role in determining neurologic outcome in some asphyxiated term infants.

Asphyxia Neonatorum↗
PubMed1993

A 2- to 3-year outcome after bronchiolitis.

OBJECTIVE: To determine the risk factors and short-term outcome until 3 years of age for subsequent wheezing in children with early childhood bronchiolitis or pneumonia. DESIGN: Prospective follow-up of a patient group. SETTING: University hospital providing primary care for all pediatric patients of a defined area. PATIENTS: One hundred twenty-seven children under 2 years of age hospitalized owing to wheezing (n = 83) or pneumonia (n = 44) during 12 months in 1981 to 1982. One hundred eight children completed the prospective follow-up until 3 years of age. INTERVENTIONS: None. MAIN RESULTS: The wheezing and pneumonia groups had equal viral and bacterial etiologic findings. History of wheezing, atopic eczema, and elevated serum IgE levels were more common in patients with wheezing than with pneumonia. Subsequent wheezing was seen after bronchiolitis in 76% (61 of 80) of the children at 1 to 2 years of age and in 58% (44 of 76) at 2 to 3 years of age. The respective figures were significantly lower, 9% (three of 33) and 16% (five of 32), in patients with pneumonia. An analysis of risk factors did not reveal any with a significant effect on subsequent wheezing. CONCLUSIONS: Subsequent wheezing is common after bronchiolitis, but rare after early childhood pneumonia, although caused by the same viruses or bacteria. Atopic diathesis is the host factor associated with initial wheezing. No genetic or environmental risk factor had significant association with later wheezing.

Asthma↗
PubMed1978

Genetic strategies in biological psychiatry.

Studies of the etiology of schizophrenia and other psychiatric illnesses may be executed by using a genetic framework in the experimental design. This article describes research strategies for identifying the genetic factors that produce a vulnerability to a psychiatric illness. The proposed strategies evaluate the role of a given genetic factor by comparing the transmission of this factor within pedigrees to the transmission of that illness. In a biologically heterogeneous disorder, these strategies can be used to identify homogeneous subgroups. This report also describes a strategy for identification of the environmental events that promote the development of a psychiatric illness, either independently or in conjunction with the genetic diathesis.

Chromosome Mapping↗
PubMed1984

The Chestnut Lodge follow-up study. II. Long-term outcome of schizophrenia and the affective disorders.

This report details the long-term course for systematically rediagnosed (and largely chronically ill) patients with schizophrenia (n = 163) and with bipolar (n = 19) and unipolar (n = 44) affective disorders from the Chestnut Lodge, Rockville, Md, follow-up study. Their conditions were assessed and they are described rigorously from multiple outcome perspectives. Except in the realm of symptomatic diathesis, striking differences emerged between these major axis I disorders consonant with Kraepelin's original observations. Roughly two thirds of the schizophrenic patients were functioning marginally or worse at follow-up, compared with one third of the unipolar cohort. The reverse held for better outcomes. Outcome varied little as a function of follow-up interval (time) across all diagnostic categories. Representative case examples serve to place the ratings in meaningful clinical contexts.

Adolescent↗
PubMed1985

Institutional rearing and diagnostic outcome in children of schizophrenic mothers. A prospective high-risk study.

Within a prospective, longitudinal study of offspring of schizophrenic mothers (so-called high-risk children), diagnostic outcome (schizophrenia, "schizotypal" personality disorder, other diagnoses, and no mental illness) was predicted by the mother's age at first hospitalization and by institutionalization during the first five years of life. Institutionalization was unrelated to adult psychopathology in a low-risk control group. These results are interpreted as supporting a diathesis-stress model of schizophrenic origin.

Adolescent↗
PubMed1985

Thought disorder in children at risk for psychosis.

The Thought Disorder Index, which has been shown to be a reliable and valid measure of the degree of thought disorder in adult psychotic patients, was used to assess thought disorder in children. Normal children aged 5 to 16 years, hospitalized psychotic children, children hospitalized for nonpsychotic behavioral problems, and children born to a psychotic mother (high-risk) were tested. In the normal sample, thought disorder decreases with age. The level of thought disorder in psychotic children and high-risk children was about three times higher than that of the normal children, whereas the level of thought disorder of the nonpsychotic hospitalized children was no different from that of the normal children. The study also detected differences in the kinds of thought disorder that characterize the psychotic and high-risk groups vs the other children. The presence of thought disorder may be thought of as a useful indicator of the diathesis for functional psychosis. The Thought Disorder Index merits selection as a tool for investigating thought disorder as a potential precursor variable in studies of children at risk for schizophrenia and manic-depressive illness.

Adolescent↗
PubMed1989

Confirming unexpressed genotypes for schizophrenia. Risks in the offspring of Fischer's Danish identical and fraternal discordant twins.

Margit Fischer reported in 1971 that the risk of schizophrenia in the offspring of her Danish schizophrenic monozygotic twins and their normal cotwins was equal and not different from the risks in the children of schizophrenics in the literature. All of her identical and fraternal twins who had children and all of their offspring have been followed up through the Danish National Psychiatric Register as of 1985, some 18 years after study by Fischer. The morbid risk (age-corrected) for schizophrenia and schizophrenia-related disorders in the offspring of schizophrenic identical twins is 16.8%; it is 17.4% in their normal cotwins' offspring. The risks in the offspring of schizophrenic fraternal twins and their normal cotwins are 17.4% and 2.1%, respectively. The results suggest that discordance in identical twins may primarily be explained by the capacity of a schizophrenic genotype or diathesis to be unexpressed unless it is released by some kinds of environmental, including nonfamilial, stressors. Sporadic cases and phenocopies caused by cerebral abnormalities, diseases, or viruses would thus be deemphasized as necessary or sufficient explanatory causes for schizophrenia in our study but could account for some of the remaining discordance, infrequent phenocopies should encourage linkage researchers, but unexpression of genotypes will frustrate them.

Adolescent↗
PubMed1992

A family study of obsessive-compulsive disorder.

First-degree relatives of probands with obsessive-compulsive disorder (OCD) (n = 32) and psychiatrically normal controls (n = 33) were blindly interviewed with the use of the Diagnostic Interview Schedule. The morbidity risk for anxiety disorders was increased among the relatives of obsessional subjects compared with that for the relatives of controls, but the risk for OCD was not. Risk for a more broadly defined OCD (including relatives with obsessions and compulsions not meeting criteria for OCD) was increased among the parents of obsessional subjects but not among the parents of controls (16% vs 3%). The findings suggest that an anxiety disorder diathesis is transmitted in families with OCD, but that its expression within these families is variable. The findings also support the current practice of classifying OCD as an anxiety disorder.

Adolescent↗