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PubMed2000

[Intracranial aneurysms and autosomal dominant polycystic kidney disease].

Three women aged 55, 47 and 40 years with polycystic kidney disease had several relatives with cystic kidneys, some of whom had died or been crippled after (presumably) a subarachnoid haemorrhage. Two of these patients had a haemorrhage from an aneurysm of a cerebral artery; after clipping of the vessel they recovered without sequelae. The third patient had magnetic resonance (MR) angiography performed, which revealed no aneurysm. The prevalence of intracranial, saccular aneurysms in patients with autosomal dominant polycystic kidney disease (ADPKD) is about 10%. ADPKD patients with questions about the risk of a subarachnoid haemorrhage should be informed about the need of blood pressure control and the possibility of screening by MR angiography. Diagnosed aneurysms can be treated neurosurgically or endovascularly. Since aneurysms develop in the course of life, screening as a rule is only necessary from the age of 20 years, and its repetition every 5 years should be considered.

Adult↗
PubMed2000

Simple cystectomy in patients requiring urinary diversion.

PURPOSE: Urinary diversion is an alternative treatment for conditions such as radiation and interstitial cystitis, neurogenic bladder and severe incontinence. The subsequent complication rate may reach 60% due to the retained nonfunctional bladder. The subsequent cystectomy rate may be as high as 20% because of pyocystis, hematuria, pelvic pain and rarely bladder cancer. We describe simple cystectomy involving bladder removal without the adjacent structures, avoiding deep pelvic dissection. MATERIALS AND METHODS: Ileal conduit or continent diversion and simple cystectomy were performed for crippling bladder symptoms secondary to neurogenic bladder, pelvic radiation, hematuria and/or severe incontinence in 12 women and 7 men with a mean age of 63 years. We performed urinary diversion and simple cystectomy only when all conservative means of treatment had failed. The majority of patients had multiple co-morbidities, previous surgeries and pelvic radiation. An average of 5 conservative procedures (range 2 to 10) had been done before simple cystectomy. None of these patients had urothelial malignancy. Simple cystectomy was done concomitantly with urinary diversion in 13 cases and later as a separate procedure in 6 due to complications of a retained nonfunctional bladder. RESULTS: Mean followup was 15 months. No mortality was associated with surgery. For simple cystectomy only mean operative time was 30 minutes and mean estimated blood loss was 300 cc. Median time from symptom presentation to cystectomy was 35 months and mean hospital stay was 7 days. In all cases symptoms were dramatically alleviated. No morbidity was directly attributable to simple cystectomy. CONCLUSIONS: Simple cystectomy is well tolerated and should be performed at urinary diversion to avoid later complications of the retained bladder. Technically this procedure is not demanding and is relatively easy to perform even after previous pelvic radiation.

Comorbidity↗
PubMed2001

Eosinophilic cystitis in adults.

PURPOSE: We describe the largest clinical experience with the diagnosis and management of largely anecdotally reported eosinophilic cystitis. MATERIALS AND METHODS: Five women and 12 men 18 to 84 years with proved eosinophilic cystitis were treated in a 23-year period. Some combination of hematuria, irritative voiding, dysuria and suprapubic pain was present in 14 cases (82%). The remaining 3 patients (18%) were asymptomatic and the diagnosis was made by cystoscopy done because of a history of bladder carcinoma. Available data included no peripheral eosinophilia in 10 of 10 patients studied, pyuria in 12 (92%), microhematuria in 11 of 13 (84%), sterile urine in all 17, abnormal urine cytology in 2 of 17 (12%), bilateral hydronephrosis in 1 and a bladder mass or thickening in 2. Cystoscopy showed erythema in all cases and tumor-like lesions or edema in 3 (17.6%). Histological studies revealed eosinophilic cystitis in all 17 patients, while in 1 with no history of bladder carcinoma eosinophilic cystitis coexisted with carcinoma. RESULTS: Two patients were lost to followup and the remaining 15 were followed 1 to 37 months. After biopsy and fulguration of the lesions 10 patients received no further treatment, including 6 with complete symptom resolution and 1 with improvement. The 3 asymptomatic patients with a history of bladder carcinoma remained asymptomatic and disease-free. Another 4 patients underwent medical therapy and improved, of whom 1 had recurrence that was successfully re-treated medically. The remaining patient, who was symptomatic, underwent cystoprostatectomy for end stage bladder disease. CONCLUSIONS: Manifestations of eosinophilic cystitis indistinguishably mimic those of other inflammatory and malignant bladder disorders that may precede or coexist with it. The condition usually follows a benign course in most cases but occasionally its relentless progression causes crippling disease.

Adolescent↗
PubMed2000

Factor VIII inhibitor with catalytic activity towards factor VIII.

Hemophilia A is an X chromosome-linked recessive disorder resulting in defective or deficient factor VIII (FVIII) molecules, which, in its severe form, is a life-threatening, crippling hemorrhagic disease. Infusion of purified FVIII to patients with severe hemophilia A results in approximately 25% of the cases in the emergence of anti-FVIII antibodies (inhibitors) that are known to neutralize the procoagulant activity of FVIII by steric hindrance. We recently reported on the proteolysis of FVIII by alloantibodies in the plasma of two high responder patients with severe hemophilia A, demonstrating a new mechanism by which FVIII inhibitors may prevent the pro-coagulant function of FVIII. Hemophilia is the first model in which a direct link between the hydrolysis of the target molecule and the occurrence of clinical manifestations has been established. It also represents the first example in humans, of the induction of catalytic antibodies following the exogenous administration of an antigen. The characterization of FVIII inhibitors as site-specific proteases may provide new approaches to the treatment of inhibitors.

Amino Acid Sequence↗
PubMedDate not supplied

Multidrug therapy--the pathway for global leprosy elimination.

Introduction of dapsone therapy paved the way for better and more humanitarian way of dealing with the leprosy victims who, prior to 1943, were simply rejected and segregated from society. Emergence of dapsone-resistant M. leprae and mycobacterial persistence provoked our quest for another solution. More drugs were discovered for treatment of leprosy. But the real breakthrough was the recommendation of regimens of multidrug therapy (MDT) for the treatment of leprosy by the WHO Study Group on Chemotherapy of Leprosy in October 1981. Subsequent world wide development of leprosy control activities was phenomenal. The impact of MDT has led to the cure of over eight million leprosy sufferers and the saving of one million patients from becoming crippled. Leprosy prevalence has decreased by 80% in ten years. By the end of May 1999 the leprosy burden remained concentrated in only 12 countries of the world. These achievements are mainly attributed to the development and worldwide adoption of the MDT programme.

Drug Therapy, Combination↗
PubMed2000

Ischaemic optic neuropathy.

Ischaemic optic neuropathy is of two types: anterior (AION) and posterior (PION), the first involving the optic nerve head (ONH) and the second, the rest of the optic nerve. Pathogenetically AION and PION are very different diseases. AION represents an acute ischaemic disorder of the ONH supplied by the posterior ciliary artery (PCA), while PION has no specific location in the posterior part of the optic nerve and does not represent an ischaemic disorder of any definite artery. The most important step towards a logical understanding of the underlying causes, clinical features, pathogenesis and rational management of AION, is to understand the basic scientific issues involved; these are discussed in some detail. AION clinically is of two types: (1) that due to giant cell arteritis (arteritic AION: A-AION) and (2) non-arteritic AION (NA-AION). NA-AION, the more common of the two, is one of the most prevalent and visually crippling diseases in the middle-aged and elderly, and is potentially bilateral. NA-AION is a multifactorial disease, with many risk factors collectively contributing to its development. Although there is no known treatment for NA-AION, reduction of risk factors is important in decreasing chances of involvement of the second eye and of further episodes. Our studies have suggested that nocturnal arterial hypotension is an important risk factor for the development and progression of NA-AION. The role of nocturnal arterial hypotension in the pathogenesis of NA-AION and management of nocturnal hypotension is discussed. Potent antihypertensive drugs, when used aggressively and/or given at bedtime, are emerging as an important risk factor for nocturnal hypotension, and there is some evidence that NA-AION may be occurring as an iatrogenic disease in some individuals. A-AION, by contrast, is an ocular emergency and requires immediate treatment with systemic corticosteroids to prevent further visual loss. The clinical parameters which help to differentiate the two types of AION, and their respective management are discussed.

Blood Pressure↗
PubMed2001

Serum interleukin-10 levels are an independent prognostic factor for patients with Hodgkin's lymphoma.

BACKGROUND AND OBJECTIVES: Interleukin-10 (IL-10) is a pleiotropic cytokine which increases bcl-2 levels and protects cells from steroid or doxorubicin-induced apoptosis. Hodgkin and Reed-Sternberg (HRS) cells bear functional IL-10 receptors. Thus serum IL-10 (sIL-10) might inhibit apoptosis in HRS cells, which could occur as a result of either chemotherapy or the crippled immunoglobulin genes. DESIGN AND METHODS: We determined sIL-10 levels in 122 patients with Hodgkin's lymphoma (HL), treated with ABVD or equivalent regimens with or without radiotherapy, and correlated them with presenting clinical and laboratory features, as well as failure-free survival (FFS) and overall survival. RESULTS: Elevated sIL-10 levels ( > or = 10 pg/mL) were detected in 55 patients (45%), and were correlated with advanced stage and elevated serum b2-microglobulin levels. At 7 years FFS was 85% vs. 63% for patients with normal vs. elevated sIL-10 levels, respectively (p=0.01); overall survival was 97% vs. 73% (p=0.005). Multivariate analysis with Cox's proportional hazards model demonstrated that elevated sIL-10 levels were the strongest independent predictor of FFS, and were also associated with inferior overall survival. INTERPRETATION AND CONCLUSIONS: We conclude that sIL-10 levels are elevated in 45% of patients with HL, and are associated with inferior FFS and overall survival, independently of other established prognostic factors.

Adolescent↗
PubMed2001

Oct-2 and Bob-1 deficiency in Hodgkin and Reed Sternberg cells.

Hodgkin and Reed Sternberg (H-RS) cells represent the malignant cells in classical Hodgkin's disease. Although derived from germinal center B cells, they do not express surface immunoglobulin. This has been explained by the presence of crippling mutations within the immunoglobulin genes in numerous cases of Hodgkin's disease. As immunoglobulin gene expression in B cells requires an interaction between octamer sites and the transactivating factors Oct-2 and Bob-1, this study addresses the expression of the transcription factors Oct-2 and Bob-1 in H-RS cells. In Hodgkin's disease-derived cell lines, low levels of Oct-2 transcripts but no Oct-2 protein were detected. Transcripts of Bob-1, a B-cell-specific co-factor of Oct-2, could not be observed in these cell lines. Absence of Oct-2 and Bob-1 protein expression in primary H-RS cells was demonstrated by performing immunohistochemistry in 20 cases of classical Hodgkin's disease. H-RS cells stained negative for both proteins in all of the cases analyzed. In conclusion, absence of functional Oct-2 and Bob-1 cells represents a novel mechanism for immunoglobulin gene deregulation in H-RS cells. Lack of Oct-2 and Bob-1 points to a defect in transcription machinery in H-RS cells and is associated with lack of immunoglobulin gene expression in these cells.

3T3 Cells↗
PubMed1975

A survey of newborn intensive care centers in California.

Newborn intensive care has come of age in California. Twenty-one newborn intensive care centers and 11 community level units are now approved by Crippled Children Services in California. In 1973 there were more than 6,863 patients admitted to the 20 centers surveyed, over half requiring transport from referring hospitals. This paper provides information on the distribution, admission and occupancy rates, length of stay, costs and admission diagnoses for these high risk infants.

California↗
PubMed2001

IgG subclass responses in experimental silicosis.

Silicosis is a crippling fibrotic lung disease induced by inhaling crystalline silica. In addition to fibrosis, silica inhalation by humans is associated with a number of immunological effects including increased levels of serum immunoglobulins (in particular IgG), increased prevalence of autoantibodies, and autoimmune disease. Recent studies using rodent models have shown that experimental silicosis is associated with a T-helper (TH)1 pattern of T-cell activation in the lungs and lung-associated lymph nodes after silica inhalation, which are also the sites of increased IgG production. We therefore hypothesized that the subclass distribution of IgG production occurring in experimental silicosis would suggest TH1 activation as the primary stimulus for IgG production. Using an ELISPOT assay, we found increased IgG-secreting spot-forming cells of all IgG subclasses in lung-associated lymph nodes taken from silica-exposed rats 3 to 4 months after aerosol exposure to silica. Neither TH1- nor TH2-dependent IgG subclass-secreting cells were selectively enhanced. Our findings suggest that TH1 activation alone does not account for increased production of IgG in experimental silicosis.

Administration, Inhalation↗
PubMed1997

From crippledom to orthopaedic nursing: Pyrford 1908-1945.

This study is based on the records of the institution which finally became known as the Rowley Bristow Orthopaedic Hospital, Pyrford, Surrey. The hospital began as a home for crippled children run by a Church of England charity called the Waifs and Strays' Society. The arrival of a leading practitioner of the new discipline of orthopaedic surgery resulted in the transition from a social model of care to a medical one. One part of this process was the redefining of the role of most of the nurses as the recipients of nursing education, with a view to making orthopaedic nursing more attractive as an occupation. However, this educational strategy resulted in a progressive lowering of the entry age and the recruitment of large numbers of probationers who failed to complete the course.

History, 20th Century↗
PubMed1993

[A short look on the protection of the disabled in Turkish history until the Republic of Turkey I].

This study deals with the care taken through Turkish history to protect and shelter the disabled orphans, widows, crippled and the aged, etc. In the Seljukian and Ottoman periods there were different foundations which shouldered the duty and functioned as almhouses. Some of these foundations had houses for sheltering the disabled and the homeless, according to those quoted in the trust of deeds. These institiutions were "Dār-al rahā" (almhouse); Tāb-khāne" and "Imāret" (almshouse and soup kitchen for the poor), "Caravanserai" (inns), "Musāfir-khāne" (public guest house for travelers); "Dār-al ziyāfe" (temporary boarding house and kitchen); "Dār-al shifā" (hospital) and "Tekye" or "Zāviye" (dervish lodges), etc. ...

Persons with Disabilities↗
PubMed1999

[Professor Ireneusz Wierzejewski - his work in Konrad Biesalski's Orthopedic Institute, in the years 1910-1911 and its influence on the professional opinions of Poland's first professor in orthopedics].

Professor Ireneusz Wierzejewski was born in 1881. He completed his medical university training in 1908 in Munich. He obtained his doctor degree in 1909 in Lipsk, based on the work "Ueber den kongenitalen ulnadefekt" On the 01.07.1909, thanks to professor Lange's support, he got a permanent post in Berlin - Brandenburgische Kruppel - Heil und Erziehunganstalt, run by Konrad Biesalski. Here he performed the duties of the first assistant in the Institute and director of the orthopedic outpatient department until 15.09.1911. During this period I. Wierzejewski's professional personality formed: he had the chance to become acquainted with the specifics of his supervisors orthopedic thinking and he got to know basic surgical techniques in orthopedics. He was the first Polish professor in orthopedics to set up the first Polish Orthopedic Institute (in Poznan) modeled after Berlin Brandenburgische Kruppel - Heil und Erziehunganstalt. The organization and equipment was similar to K-H-E. This institution was later transformed into the first Polish orthopedic clinic. Just as in the German model, the Orthopedic Institute in Poznan had orthopedic workshops, which at the time was very unique in Poland. Indoctrinated with K. Biesalski idea of caring for the cripples in Germany, he strived to make it a reality in Poland. In his speeches during scientific congresses fought for an act analogous to the Prussian one from 06.05.1920. The time spent working in K. Biesalski's Institute formed the professional and scientific personality of Poland's first professor in orthopedics and indirectly but definitely influenced the creation of Poland's first Orthopedic Clinic.

History, 20th Century↗
PubMedDate not supplied

Disease: a sign of piety?: some moral associations of disease in medieval society.

Al-Jāhiz, an Arab writer and philosopher of the ninth century C.E., one of the most brilliant intellectuals of the Abbasid period, suggested in his work "Kitāb al-Bursān wa'l-'urjān wa'l-umyān wa'l-hulan" ("The Book of the lepers, lame, blind, and cross-eyed") that disease and physical imperfections should not be considered to be social stigma, but rather signs of special divine blessings. Cripples and sick persons, in his opinion, received spiritual compensation from G-d. This idea may not seem at first glance to belong to medieval Islamic tradition. This paper tries to analyze its place in that culture and its relation to other views on disease accepted there. I will especially try to analyze the correlation between piety and disease: Was it specific to this case or characteristic of the society. The problem of the association of disease and piety and divine favor, the problems of the moral implications of disease in general is certainly a vast one. This study is restricted to some of the ethical conceptions connected with illness in medieval Moslem society, including the religious minorities which were in a sense an integral part of that society.

Asia, Western↗
PubMed2000

Open wide.

Dentists who work with children can appreciate the difficulties encountered when attempting to provide high quality restorative care within a confined space. In this presentation, I will address the issue of how to maximize space available in the mouth whilst minimizing patient discomfort and maintaining a working position that does not cripple the operator! Topics covered include: Instruments which take up less space in the mouth Behavioural techniques and aids to maximize mouth opening Adaptation of dental equipment for use in the child patient Techniques to obtain radiographs with minimal discomfort Methods for administering local anaesthetic with less fuss Novel methods of rubber dam placement. Space within the dental surgery is also limited. A further issue to be addressed is the management of the whanau/family to optimize their presence in supporting the patient, yet minimize distractions within the confines of the surgery.

Anesthesia, Dental↗
PubMed1999

Interleukin-1 receptor antagonist gene therapy for arthritis.

Rheumtatoid arthritis (RA) is a crippling, autoimmune disease, and is characterized by inflammation and destruction of joint tissue. Interleukin-1 (IL-1) has been identified as a key pro-inflammatory cytokine responsible for inflammation. One of the mechanisms of regulation of activity of IL-1 is IL-1 receptor antagonist (IL-1ra)-mediated: IL-1RA competes with IL-1 for binding to the IL-1 receptor. Significant progress has been made in the potential application of IL-1ra gene therapyfor the treatment of arthritis. Various vectors have been tested for the delivery of the IL-1ra gene to the intra-articular region. Recent studies in humans have provided encouraging prospects for IL-1ra-mediated arthritis gene therapy.

Animals↗
PubMed2001

Gynecologic aspects of Crohn's disease.

Because Crohn's disease has a tendency to be transmural and to form fistulas, it may involve contiguous organs. Gynecologic involvement is frequent, diverse and often difficult to diagnose. Inflammation involving adjacent structures has been reported in as many as one third of patients. Enteric fistulas to the vagina, uterus, ovaries, perineum and vulva also have been reported. Diagnosis may not be obvious if pelvic involvement precedes active bowel disease, or if drainage is clear or mucoid. Abscesses, draining sinuses, edema and ulceration of the perineum or vulva are common, and are caused by direct extension from the involved bowel or by granulomas separated from the bowel by normal tissue. Lesions presenting with vulvar hypertrophy, a fluctuant mass or ulceration are easily misdiagnosed. Menstrual abnormalities are reported in more than one half of patients. Pelvic manifestations of Crohn's disease can be psychologically crippling. Patients or physicians may be hesitant to address serious psychosocial morbidity. To optimize management, physicians must be aware of the diverse manifestations, confusing presentations and psychologic morbidity of Crohn's disease.

Crohn Disease↗
PubMed2000

Image-guided sinus surgery: navigation without road maps.

Many otorhinolaryngologists have recognized the necessity of developing new methods to increase the safety of endoscopic sinus surgery and improve patient outcomes. Despite the rapid advances made in the field, serious iatrogenic complications still occur. To counteract these complications, one recent advancement has been the development of a computed tomographic image-guided surgery (IGS) system, which can enhance the ability to treat the crippling effects of sinus disease. These systems have gained a degree of accuracy, making them suitable for difficult surgical procedures, and they may soon become standard of care. The perioperative nurse must understand and be able to work with IGS.

Humans↗