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PubMedDate not supplied

The "pinch and slide" blepharoplasty: safe and predictable aesthetic results.

Blepharoplasty is one of the most common facial cosmetic surgical procedures. When done properly, this relatively simple operation can result in a dramatic improvement for the patient with relatively little downtime. However, when it is performed improperly, the results can be crippling for the patient and often difficult for the surgeon to correct. Standard treatments for upper eyelid dermatochalasis include surgical excision of skin, muscle, and fat. Several techniques have been described for removing some or all of these components, depending on the patients' anatomic requirements. In particular, the "pinch" technique can be used to remove either the upper eyelid skin alone or a combination of skin, muscle, and fat. While this technique is not new, its appearance in the literature is sparse. We demonstrate herein how a modified version of the pinch technique can be used to remove the central orbital fat pad by "sliding" the pad through the medial fat pad incision. This procedure maintains the integrity of the central orbital septum and the delicate structures that lie beneath.

Blepharoplasty↗
PubMed1976

The Jewish Hospital of St Louis Therapeutic Grand Rounds No. 14. Hypersensitivity pneumonitis.

Two examples of immunologically mediated diseases of the lung have been discussed. The first, extrinsic allergic alveolitis, is caused by sensitivity to inhalation of an organic dust. It is largely a restrictive type of lung disease and is probably caused by a combination of type 3 and type 4 allergic reactions. The second disease, allergic bronchopulmonary aspergillosis, is caused by a type 1 and type 3 allergic response to Aspergillus antigen in a basically atopic, generally asthmatic individual. It is vital to keep the diagnostic index of suspicion high so as to recognize these diseases early enough to prevent irreparable tissue damage. Extrinsic allergic alveolitis may masquerade as idopathic pulmonary fibrosis or Hamman-Rich syndrome. Allergic aspergillosis may be called Loeffler pneumonia or PIE syndrome (pulmonary infiltrates with eosiniphilia). Failure to diagnose properly may result in extensive pulmonary fibrosis or bronchiectasis and condemn the patient to a lifetime as a pulmonary cripple.

Antigens↗
PubMed2000

Myoclonus from selective dentate nucleus degeneration in type 3 Gaucher disease.

OBJECTIVE: To describe a case with a new genetic variant of type 3 Gaucher disease presenting with stimulus-sensitive and action myoclonus in the presence of selective dentate abnormalities. DESIGN: Clinical, pathologic, and molecular genetic studies. SETTING: Medical school departments. PATIENT: A 6-year-old girl with type 3 Gaucher disease experienced progressively crippling generalized stimulus-sensitive and action myoclonus. Repeated electroencephalographic examination did not show cortical activity associated with the myoclonus, suggesting its subcortical origin. Neuropathological examination revealed selective degeneration of the cerebellar dentate nucleus and dentatorubrothalamic pathway in the face of essentially complete lack of storage in the brain. Mutation analysis identified the following 2 mutant alleles: one with a V394L mutation and the other with the lesion RecTL (D409H + L444P + A456P + V460V), which resulted from a recombination event, with the pseudogene located 16 kilobases downstream from the structural gene. CONCLUSION: Given the restricted abnormalities, this genetically unique case provides insight into the pathogenesis of myoclonus and suggests a prominent role for the cerebellar dentate nucleus in its genesis.

Cerebellar Nuclei↗
PubMed1976

Reared in adversity: institutional care of children in the 18th century.

The earliest public pediatric care of the 18th century in this country took the form of "outdoor relief." Institutional care followed, first almshouses were built; then orphanages, hospitals, and dispensaries. Almshouses not only included workhouses but provided comprehensive medical services. Throughout the 18th century, people often referred to the almshouses as hospitals. As general hospitals, they rendered a variety of pediatric services to sick children, including the idiotic and hopelessly crippled, and the newborns delivered in the maternity wards; and they tendered services for well children, such as foundlings, abandoned children, and the children of destitute parents, placing infants in foster homes and indenturing older children for training in various trades and crafts. The voluntary hospitals, on the other hand, were for the "worthy" poor and limited their services to the insane and the curable sick. There were only two opened during the 18th century-the Pennsylvania Hospital in 1752 and the New York Hospital in 1791. The former excluded young children during the 18th century. Orphanages preceded the voluntary hospitals in point of time, offering many pediatric services to children, well and sick. Finally, at the end of the century, the independent dispensaries appeared, the first in Philadelphia in 1786. By the middle of this 20th century, practically all of them had been absorbed by hospitals. In these institutions, pediatric knowledge advanced and medical manpower developed even during the 18th century. By the end of that century, social movements began from which evolved the 19th-century concern for the welfare of children.

Adult↗
PubMed1977

General revenue sharing funds for the health care of mothers and children.

In a study of the use of General Revenue Sharing (GRS) funds by the states and by cities of 100,000 and over in the United States for fiscal years 1973 and 1974, we found that few states and large cities allocated or requested GRS funds for Maternal and Child Health (MCH), Crippled Children (CC), and related services. One third of the states and cities reported suggestions for use of GRS funds for MCH, CC, and related services.

Child↗
PubMed1988

Reflex sympathetic dystrophy syndrome in children and adolescents. Report of 18 cases and review of the literature.

Reflex sympathetic dystrophy syndrome is a well-recognized disorder in adults, but it is rarely diagnosed in the pediatric age group. This report summarizes our experience with this condition from 1975 to 1985. We diagnosed, treated, and followed up this condition in 18 children and adolescents. The condition usually followed trauma. The most prominent feature in all patients was a constant limb pain with episodes of paroxysmal exacerbation. The pain was associated with two or more of the following: edema, hyperhidrosis or anhidrosis, cyanosis or erythema, and, in severe cases, dystrophic skin changes and muscle atrophy. Roentgenograms were normal. Bone scans were helpful to exclude other possible causes of bone and joint pain. Reflex sympathetic dystrophy syndrome in children probably often goes unrecognized, sometimes being confused with psychiatric conditions such as conversion reaction and malingering. Reflex sympathetic dystrophy syndrome should always be considered in the differential diagnosis of unexplained persistent limb pain in children: early recognition and proper management may result in the prevention of potentially crippling sequelae.

Adolescent↗
PubMed1976

Early debridement in pit viper bites.

From my observations of snakebite over the last 22 years and the studies I have done, several things are important: (1) Pit viper envenomation is a surgical emergency as is any disease in which gangrene of human tissue occurs. (2) Severe pit viper envenomation causes complex problems similar to those seen in Gram-negative septicemia, and they require complex methods of treatment. (3) Early surgical inspection of the snakebite wound is as essential as early appendectomy in appendicitis. Its results are as gratifying. (4) Present knowledge of anesthesia, coagulation problems, infections and antibiotics, blood gas changes, electrolytes and fluid therapy, and other advances in the surgical field allow the physician to treat severe pit viper envenomation by scientific means rather than by hocus-pocus. (5) Crippling from pit viper envenomation is caused by too little treatment, too much first aid, or both.

Adolescent↗
PubMed2003

The consequences of premature abandonment of affirmative action in medical school admissions.

The US Supreme Court recently accepted on appeal 2 cases from the University of Michigan regarding the constitutionality of race-conscious decision making in higher education admissions. The consequences of the Court's decision will directly affect the future of medicine in the United States. Medical schools have a societal obligation to select and educate the physician workforce of the future. To outlaw the use of affirmative action in the admissions process would cripple the profession's ability to achieve racial and ethnic diversity. Preserving this diversity in medical school admissions programs is important for 4 major reasons (1) adequate representation among students and faculty of the diversity in US society is indispensable for quality medical education; (2) increasing the diversity of the physician workforce will improve access to health care for underserved populations; (3) increasing the diversity of the research workforce can accelerate advances in medical and public health research; and (4) diversity among managers of health care organizations makes good business sense. This article explores these reasons in detail, reviews the history and effectiveness of affirmative action in medical school admissions programs, and explains why alternatives to affirmative action are unworkable.

College Admission Test↗
PubMed1999

From Horus the child to Hephaestus who limps: a romp through history.

The question of why Hephaestus, the Greek god of smiths, limped has been the subject of much debate, mainly on mythological grounds. This debate extended also into the field of medical diagnosis, with attempts at defining the nature of the deformity that made the crippled Hephaestus the buffoon of the other Olympic gods. One problem encountered in these debates was the changes to which the ugly young Hephaestus was subjected with the passing of time-from a limping deformed youth to the later dignified and normal man. While some authors, largely influenced by poetic Greek texts and vase paintings, attributed the limp to talipes (club-feet), others pointed to certain features suggestive of achondroplasia. Since the image of the early Hephaestus is based mainly on the much earlier concept of the Egyptian god Ptah, who as the triune god of the resurrection sometimes is depicted as an achondroplastic dwarf (Ptah-Pataikos), the suggestion of the possible achondroplastic dwarf-like nature of the early Hephaestus is not implausible. It is supported by similarities in the image of Hephaestus to some features in other Egyptian gods, such as the domestic god Bes, the guardian of the new-born, and the Horus the Child or Harpocrates (Greek), yet another protector of youth and "the symbol of everything that is young and vigorous" [Budge, 1969: The Gods of the Egyptians, or Studies in Egyptian Mythology. Volume I.]. The characteristic feature of this child-god is the "lock of Harpocrates" on the right side of his head. That this lock can sometimes also be seen not only on the head of Ptah-Pataikos and of Bes but also on the young Hephaestus is highly suggestive of the Egyptian influence on his image. Recently, however, another interesting explanation of Hephaestus's limp has been suggested that may explain why the Egyptian influenced image of the early achondroplastic Hephaestus changed to the later, more Grecian view of the smith-god who hobbled because of club-feet. Improvements in composition-analysis of samples from antique statues and various utensils have led to the suggestion that the introduction of new smelting techniques in antique times may have exposed ancient metal workers to the effects of various toxic metals causing, for instance, chronic lead poisoning or, more relevant here, chronic arsenic poisoning causing peripheral neuritis with weakness and lameness of one or both lower extremities. Later changes in smelting technique, and recognition or guess-work of a possible connection between these techniques and toxic effects, may explain the change from the buffoon-like achondroplastic walk to the club-footed limp and eventual normal behaviour of Hephaestus, the Smith. In other words: Did Hephaestus limp because of his arsen-neuritis?

Achondroplasia↗
PubMed2000

Social and occupational differences in chronic obstructive lung disease in Denmark 1981-1993.

BACKGROUND: Chronic obstructive pulmonary disease (COPD) is a common crippling disorder related to exposure to noxious dust and fumes. The purpose of this study was to estimate relative rates of COPD in socioeconomic groups and in 'classic' high-risk industries. METHODS: Cohorts of all gainfully employed 20-59 year old Danes in the years 1981, 1986, and 1991 were formed, to compare standardized hospitalization ratios (SHR) and time trends (1981-93). RESULTS: The risk ratio (RR) between unskilled workers and senior salaried staff was 2.31, (95% CI 2.13-2.51) for men and 1.62 (1.38-1.92) for women. Among classic high-risk occupations we found a decreasing SHR in farmers and an increasing SHR in the hotel and restaurant industry and for taxi and bus drivers. The study confirmed earlier reports on high risks in the metal, rubber, and bakery industries. CONCLUSIONS: Apart from the reduced SHR among farmers there were no signs of decreasing differences in COPD risk.

Adult↗
PubMed1997

Tay-Sachs disease-causing mutations and neutral polymorphisms in the Hex A gene.

Tay-Sachs disease is an autosomal recessive disorder affecting the central nervous system. The disorder results from mutations in the gene encoding the alpha-subunit of beta-hexosaminidase A, a lysosomal enzyme composed of alpha and beta polypeptides. Seventy-eight mutations in the Hex A gene have been described and include 65 single base substitutions, one large and 10 small deletions, and two small insertions. Because these mutations cripple the catalytic activity of beta-hexosaminidase to varying degrees, Tay-Sachs disease displays clinical heterogeneity. Forty-five of the single base substitutions cause missense mutations; 39 of these are disease causing, three are benign but cause a change in phenotype, and three are neutral polymorphisms. Six nonsense mutations and 14 splice site lesions result from single base substitutions, and all but one of the splice site lesions cause a severe form of Tay-Sachs disease. Eight frameshift mutations arise from six deletion- and two insertion-type lesions. One of these insertions, consisting of four bases within exon 11, is found in 80% of the carriers of Tay-Sachs disease from the Ashkenazi Jewish population, an ethnic group that has a 10-fold higher gene frequency for a severe form of the disorder than the general population. A very large deletion, 7.5 kilobases, including all of exon 1 and portions of DNA upstream and downstream from that exon, is the major mutation found in Tay-Sachs disease carriers from the French Canadian population, a geographic isolate displaying an elevated carrier frequency. Most of the other mutations are confined to single pedigrees. Identification of these mutations has permitted more accurate carrier information, prenatal diagnosis, and disease prognosis. In conjunction with a precise tertiary structure of the enzyme, these mutations could be used to gain insight into the structure-function relationships of the lysosomal enzyme.

Age of Onset↗
PubMed1997

Alzheimer disease hyperphosphorylated tau aggregates hydrophobically.

The chemical interaction that condenses the hyperphosphorylated protein tau in Alzheimer's disease (AD P-tau) into neurofibrillary tangles and cripples synaptic transmission remains unknown. Only beta-sheet, positive ion salt bridges between phosphates, and hydrophobic association can create tangles of just AD P-tau. We have correlated transmission electron microscope (TEM) images of tau aggregation with different percentages of beta-sheet in aqueous suspensions of tau while using buffers that block dispositive or tripositive ionic bridges between intermolecular phosphates. Circular dichroism (CD) studies were performed at different temperatures from 5-85 degrees C using AD P-tau, AD P-tau dephosphorylated with hydrofluoric acid (HF AD P-tau) or alkaline phosphatase (AP AD P-tau), and recombinant human tau with 3-repeats and two amino terminal inserts (R-39) and using bovine tau (B tau) isolated without heat or acid treatment. Secondary structure was estimated from CD spectra at 5 degrees C using the Lincomb algorithm. Each preparation except one demonstrated an inverse temperature transition, Ti, in the CD at 197 nm. No correlation was found between beta-sheet content and aggregation, leaving only hydrophobic interaction as the remaining possibility. Thirteen of 21 possible phosphorylation sites in AD P-tau lie adjacent to positive residues in tau's primary structure. Occupation of five to nine phosphate sites on AD P-tau appears sufficient to reduce or neutralize tau's basic character. AD P-tau's hydrophobic character is indicated by its low inverse temperature transition, Ti. The Ti for AD P-tau was 24.5 degrees C or 28 degrees C, whereas for B tau with three phosphates it was 32 degrees C, for unphosphorylated tau R-39 it was 38 degrees C, and for dephosphorylated HF AD P-tau it was 37.5 degrees C. The hydrophobic protein elastin and its analogs coalesce and precipitate at their Ti of 24-29 degrees C, well below body temperature. We hypothesize that AD P-tau causes tangle accumulation by this mechanism.

Alkaline Phosphatase↗
PubMed2000

Dysregulation of lymphocyte proliferation by chromosomal translocations and sequential genetic changes.

Enzymatically mediated rearrangement of Ig and T-cell receptor genes is essential for generating the huge molecular repertoire of the mammalian immune system, but it also carries a danger for the organism in the form of high risk zones for illegitimate juxtaposition of DNA from other areas of the genome. Translocation-dependent activation of oncogenes, transcription factors or developmental genes can trigger the development of neoplasia in a lineage-specific fashion. These events are not sufficient for tumorigenesis, however, since some of the most prominent tumor-associated translocations, such as Ig/myc and Ig/bcl-2, have been detected in normal individuals who did not develop tumors. Tumor development must, therefore, require subsequent genetic changes. Among them, the increased expression of genes that protect against apoptosis or, alternatively, mutations that cripple apoptosis-activating genes play a prominent role. Some of the translocations associated with T-cell leukemia, myeloid leukemia, and a variety of sarcomas act by generating fusion proteins. The participating genes encode transcription factors and/or developmental regulators. Fusion protein-expressing cells may serve as targets for specific interference with abnormal signaling pathways or for targeted immune attack. Using PCR to detect cells carrying such translocations is useful for tumor diagnosis, prognosis, and choice of therapy.

Abelson murine leukemia virus↗
PubMed1996

Economic costs of anxiety disorders.

Anxiety disorders are estimated to affect 26.9 million individuals in the United States at some point during their lives. This study used the human capital approach to estimate the direct and indirect costs of these highly prevalent disorders. In 1990, costs associated with anxiety disorders were $46.6 billion, 31.5% of total expenditures for mental illness. Less than one-quarter of costs associated with anxiety disorders were for direct medical treatment; over three-quarters were attributable to lost or reduced productivity. Most of these indirect costs were associated with morbidity, as mortality accounted for just 2.7% of the total. Greater availability of effective, relatively low-cost outpatient treatment could substantially reduce the economic and social burden of these common and often crippling disorders.

Absenteeism↗
PubMed2005

Clotting factor concentrates given to prevent bleeding and bleeding-related complications in people with hemophilia A or B.

BACKGROUND: People with severe hemophilia A or B, X-linked bleeding disorders due to decreased blood levels of coagulants, suffer recurrent bleeding into joints and soft tissues. Before clotting factor concentrates were available, most people with severe hemophilia developed crippling musculoskeletal deformities. Clotting factor concentrate prophylaxis aims to preserve joint function by converting severe hemophilia (factor VIII or IX less than 1%) into a clinically milder form of the disease. Prophylaxis has long been used in Sweden, but not universally adopted because of medical, psychosocial, and cost controversies. Use of clotting factor concentrates is the single largest predictor of cost in treating hemophilia. OBJECTIVES: To determine the effectiveness of clotting factor concentrate prophylaxis in the management of people with hemophilia A or B. SEARCH STRATEGY: We searched the Cystic Fibrosis and Genetic Disorders Group's Trials Register comprising references from comprehensive electronic database searches and handsearches of journals and abstract books. Reference lists of relevant articles were reviewed. Most recent search: January 2002. SELECTION CRITERIA: Randomized controlled trials (RCTs) evaluating people with severe hemophilia A or B, receiving prophylactic clotting factor concentrates. DATA COLLECTION AND ANALYSIS: Two reviewers independently reviewed studies for eligibility, assessed methodological quality and extracted data. MAIN RESULTS: Twenty-nine studies were identified, of which four (including 37 participants) were eligible for inclusion. Three studies evaluated hemophilia A; one showed a decrease in frequency of joint bleeds with prophylaxis compared to placebo (non-physiological dose), with a rate difference (RD) -10.80 (95% confidence interval (CI) -16.33 to -5.27) bleeds per year. The remaining two studies evaluating hemophilia A compared two prophylaxis regimens, one study showed no difference in joint bleed frequency, RD -5.04 (95%CI -17.02 to 6.94) bleeds per year and another failed to demonstrate an advantage of factor VIII dosing based on individual pharmacokinetic data over the standard prophylaxis regimen with RD -0.14 (95% CI -1.34 to 1.05) bleeds per year. The fourth study evaluated hemophilia B and showed fewer joint bleeds with weekly (15 IU/kg) versus bi-weekly (7.5 IU/kg) prophylaxis, RD -3.30 (95% CI -5.50 to - 1.10) bleeds per year. AUTHORS' CONCLUSIONS: There is insufficient evidence to determine whether prophylactic clotting factor concentrates decrease bleeding and bleeding-related complications in hemophilia A or B, compared to placebo, on-demand treatment, or prophylaxis based on pharmacokinetic data from individuals. Well-designed RCTs are needed to assess the effectiveness of prophylactic clotting factor concentrates. Two clinical trials are ongoing.

Blood Coagulation Factors↗
PubMed2006

Clotting factor concentrates given to prevent bleeding and bleeding-related complications in people with hemophilia A or B.

BACKGROUND: People with severe hemophilia A or B, X-linked bleeding disorders due to decreased blood levels of coagulants, suffer recurrent bleeding into joints and soft tissues. Before clotting factor concentrates were available, most people with severe hemophilia developed crippling musculoskeletal deformities. Clotting factor concentrate prophylaxis aims to preserve joint function by converting severe hemophilia (factor VIII or IX less than 1%) into a clinically milder form of the disease. Prophylaxis has long been used in Sweden, but not universally adopted because of medical, psychosocial, and cost controversies. Use of clotting factor concentrates is the single largest predictor of cost in treating hemophilia. OBJECTIVES: To determine the effectiveness of clotting factor concentrate prophylaxis in the management of people with hemophilia A or B. SEARCH STRATEGY: We searched the Cystic Fibrosis and Genetic Disorders Group's Trials Register comprising references from comprehensive electronic database searches and handsearches of journals and abstract books. Reference lists of relevant articles were reviewed. Most recent search: November 2005. SELECTION CRITERIA: Randomized controlled trials (RCTs) evaluating people with severe hemophilia A or B, receiving prophylactic clotting factor concentrates. DATA COLLECTION AND ANALYSIS: Two authors independently reviewed studies for eligibility, assessed methodological quality and extracted data. MAIN RESULTS: Twenty-nine studies were identified; four studies (including 37 participants) were eligible for inclusion. Three studies evaluated hemophilia A; one showed a decrease in frequency of joint bleeds with prophylaxis compared to placebo (non-physiological dose), with a rate difference (RD) -10.80 (95% confidence interval (CI) -16.33 to -5.27) bleeds per year. The remaining two studies evaluating hemophilia A compared two prophylaxis regimens, one study showed no difference in joint bleed frequency, RD -5.04 (95%CI -17.02 to 6.94) bleeds per year and another failed to demonstrate an advantage of factor VIII dosing based on individual pharmacokinetic data over the standard prophylaxis regimen with RD -0.14 (95% CI -1.34 to 1.05) bleeds per year. The fourth study evaluated hemophilia B and showed fewer joint bleeds with weekly (15 IU/kg) versus bi-weekly (7.5 IU/kg) prophylaxis, RD -3.30 (95% CI -5.50 to - 1.10) bleeds per year. AUTHORS' CONCLUSIONS: There is insufficient evidence from randomised controlled trials to determine whether prophylactic clotting factor concentrates decrease bleeding and bleeding-related complications in hemophilia A or B, compared to placebo, on-demand treatment, or prophylaxis based on pharmacokinetic data from individuals. Well-designed RCTs are needed to assess the effectiveness of prophylactic clotting factor concentrates. Two clinical trials are ongoing.

Blood Coagulation Factors↗
PubMed1979

A new treatment strategy for hemophilia B: incorporation of factor IX into red cell ghosts.

Although patients with severe hemophilia B have lifelong spontaneous hemorrhage and crippling hemarthroses, patients with 0.01--0.03 U/ml Factor IX activity have a milder disease state. The clinical condition of severely affected individuals could potentially be improved by prolonging the half-life of transfused Factor IX. The feasibility of incorporating Factor IX into red cell ghosts was suggested by resealing experiments with similar sized molecules such as albumin. We have prepared resealed red cell ghosts containing human Factor IX and X. Human red cells were subjected to hypotonic lysis at 0 degrees C, pH 6.0. Commercial prothrombin complex concentrate was dissolved in the lysing medium immediately prior to the addition of the red cells. After being returned to isotonicity, the red cell ghosts were annealed at 37 degrees C for 30 minutes and then washed extensively. When intact red cell ghosts were tested, no Factor IX or X activity could be demonstrated. After disruption of the red cell ghost membranes with 3M urea or 2% Triton X-100, the procoagulants could be quantitatively recovered. Similar recovery of the clotting factors could be demonstrated from the lysate and the early wash samples. Red cells from Factor IX and X deficient patients served equally well as those from normal subjects. Red cell ghosts prepared in similar fashion but not exposed to the procoagulants had negligible clotting activity. We have demonstrated that human clotting factors can be incorporated into red cell ghosts. The ability of this system to prolong the biological half-life of Factor IX is under investigation.

Erythrocyte Membrane↗
PubMed1987

Osteogenesis imperfecta type III: an ancient mutation in Africa?

In a survey of institutions for crippled persons in Zimbabwe, 58 patients with osteogenesis imperfecta (OI) were identified; 42 had the rare OI Type III. The Shona and Ndebele people, who comprise the major tribal groups in Zimbabwe, both had a similar and relatively high gene frequency for this disorder. Both tribes were derived from common progenitors, but until 150 years ago had been geographically separated for 2 millenia. Subsequently, they have remained culturally and socially distinct. The implications are that the mutation for OI III in Africa occurred at least 2,000 years ago.

Female↗