Long-term FVII substitution in a preterm infant with severe gastrointestinal bleeding and FVII deficiency due to a homozygous donor splice mutation IVS4+1G-->A.

Congenital FVII deficiency is a rare bleeding disorder. Clinical complications are similar to those seen in hemophilia A, and an increased incidence of intracerebral hemorrhage related to birth trauma has been reported. The authors report on an infant who presented at the second day of life with melaena and hematemesis caused by congenital FVII deficiency with minimal activity of 4%. A homozygous mutation IVS4+G-->A, formerly described in 2 siblings, who died of brain hemorrhage within the first month of life, was identified. Severe bleeding events were prevented with prophylactic treatment. Early identification of the underlying mutation helps to assess the risk of hemorrhage and prevent severe bleeding by prophylactic FVII therapy.

Clinical and economic impact of various strategies for varicella immunity screening and vaccination of health care personnel.

BACKGROUND: Exposure to patients with varicella or herpes zoster causes considerable disruption to a health care facility's operations and has a significant health and economic impact. However, practices related to screening for immunity and immunization of health care personnel (HCP) for varicella vary widely. METHODS: A decision tree model was built to evaluate the cost-effectiveness of 8 different strategies of screening and vaccinating HCP for varicella. The outcomes are presented as probability of acquiring varicella, economic impact of varicella per employee per year, and cost to prevent additional cases of varicella. Monte Carlo simulations and 1-way sensitivity analyses were performed to address the uncertainties inherent to the model. Alternative epidemiologic and technologic scenarios were also analyzed. RESULTS: Performing a clinical screening followed by serologic testing of HCP with negative history diminished the cost impact of varicella by >99% compared with not having a program. Vaccinating HCP with negative screen cost approximately $50,000 per case of varicella prevented at the current level of U.S. population immunity, but was projected to be cost-saving at 92% or lower immunity prevalence. Improving vaccine acceptance rates and using highly sensitive assays also optimize cost-effectiveness. CONCLUSION: Strategies relying on screening and vaccinating HCP for varicella on employment were shown to be cost-effective for health care facilities and are consistent with current national guidelines for varicella prevention.

Preoperative screening for coagulation disorders in children undergoing adenoidectomy (AT) and tonsillectomy (TE): does it prevent bleeding complications?

BACKGROUND: Bleeding remains the most important complication of adenotonsillectomy in children. Preoperative coagulation tests are widely used to detect unknown bleeding disorders. To determine the efficacy of preoperative coagulation screening in preventing bleeding complications. PATIENTS: Study group 1: 148 healthy children referred by the otorhinolaryngology department for preoperative pediatric examination. Study group 2: 124 healthy children sent to the hemostaseologic clinic for preoperative investigation of a prolonged PTT. METHOD: The incidence of relevant coagulation disorders detected by a standardized bleeding history and coagulation screening tests was studied prospectively in 2 study groups planned for AT and/or TE. The frequency of abnormal bleeding was investigated retrospectively in those children who underwent surgery. RESULTS: Bleeding disorders were detected in 7/148 and 15/124 children in study group 1 and 2 respectively. 141/148 and 79/124 children actually underwent surgery, 62 TE +/- AT + 79 AT alone and 26 TE +/- AT + 53 AT alone respectively. Major bleeding occurred in 1/141 patients (1 TE) in study group 1. Preoperatively, this child had shown normal coagulation screening tests. In 4/79 patients (3 TE, 1 AT) in study group 2, surgery was complicated by major bleeding. Despite extensive testing, no relevant bleeding disorder had been diagnosed in these children preoperatively. Sensitivity of coagulation screening tests for major bleeding was 0 in study group 1. CONCLUSIONS: In our study, coagulation screening failed to effectively identify patients at risk of bleeding.

Bleeding and surgery in children with Glanzmann thrombasthenia with and without the use of recombinant factor VII a.

BACKGROUND: An inherited deficiency of platelet glycoprotein II b/III a (GP II b/III a), Glanzmann thrombasthenia, can lead to excessive bleeding and require platelet transfusion to secure hemostasis. Antibodies to GP II b/III a or HLA may platelet transfusion render ineffective to stop bleeding or to cover surgery. Recombinant factor VII a has been introduced as therapeutic alternative and has been suggested to be effective. PATIENTS AND AIMS OF THE STUDY: In a retrospective evaluation, bleeding episodes and surgery in six patients treated with antifibrinolytics and with and without the additional use of rFVII a were analysed to achieve informations for treatment indication and efficacy. RESULTS: Nineteen mucosal and subcutaneous bleeding episodes, two dental surgeries and seven joint bleeds occurred. In 11 mild to moderate mucocutaneous bleeds treated without rFVII a, seven stopped within 48 hours, three stopped until the fourth day; one showed recurrence. Three bleeds were treated with rFVII a and responded within 24 hours. One severe bleed treated without rFVII a did not stop until the 8 (th) day after cautery. In 4 severe bleeds treated with rFVII a, one stopped within 24 hours, one showed recurrence, one was treated with platelet transfusion concurrently and one did not respond to rFVII a. Clinical signs persisted in one conservatively treated elbow joint bleed, whereas in two episodes treated with rFVII a, the bleeding responded within 5 and 7 days and in four episodes in at least 4 days. Two dental surgeries showed no recurrence after rFVII a over 18 or 36 hours. CONCLUSIONS: In severe mucocutaneous bleeding episodes or joint bleeding rFVII a is of some benefit whereas in surgeries like teeth extraction, prophylactically administered rFVII a seems effective to avoid bleeding. In mild to moderate mucocutaneous bleeding events, antifibrinolytics and local measures were sufficient in most cases and the additional use of rFVII a does not seem to be necessary. Further information is needed to elaborate clear indications for the rational use of rFVII a in bleeding episodes in patients with Glanzmann thrombasthenia compared to standardized baseline treatment. This information may generate a prospective multicenter study to provide clear advice with respect to bleeding site, severity and duration.

Improvements in billing processes and information systems increase cash collections in a public health care system.

Management information systems are an essential tool for health care managers. Denver Health, an integrated safety net institution, upgraded its financial management information system during 1997 and experienced almost an 18 percent or $23 million increase in cash collections from 1997 to 1998 and a 17 percent or over $25 million increase from 1998 to 1999. Cash collections continued to increase in 2000 with a $12.0 million (6.8%) ncrease over 1999. This article explains the information system factors that have contributed to this increase and describes the continued management efforts that are expected to continue to improve cash collections.

[Controlled hypotension in difficult intraocular anterior segment surgery (author's transl)]. / Kontrollierte Hypotension bei schwierigen intraokularen Eingriffen.

During anaesthesia controlled hypotension -- induced by sodium nitroprusside -- was used in 60 patients undergoing intraocular anterior segment surgery. The advantages of this procedure are that the iris-lens diaphragma and the vitreous body have a lesser tendency to protrude than with other techniques despite even more superficial anaesthesia.

[The clinical picture of metastatic myotic endophthalmitis (author's transl)]. / Zur Klinik der metastatischen mykotischen Endophthalmitis

Mycotic Septicaemia (especially with the Candida species) is not an uncommon hazard of hospitalized patients, especially those on intravenous hyperalimentation. Two such patients with endogenous mycotic bilateral endophthalmitis are presented. In spite of typical ocular symptoms diagnosis was delayed. Two further unilateral cases of a more atypical form of endogenous mycotic endophthalmitis in otherwise seemingly healthy patients are also described. Correlating histopathological findings in three of these 4 cases to the clinical histories, conclusions are drawn to aid an early diagnosis which is of paramount importance if the necessary antimyotic treatment is to preserve visual function.

[Controlled hypotension by sodium-nitroprusside in general anaesthesia for difficult intraocular surgery (preliminary report) (author's transl)]. / Kontrollierte Hypotension durch Natrium-Nitroprussid bei der Allgemeinnarkose für schwierige intraokulare Eingriffe (Vorläufige Mitteilung).

General anaesthesia was performed in 75 patients with difficult intraocular procedures in which the standard relaxation and hyperventilation was supplemented by controlled short-term hypotension by sodium-nitroprusside. This resulted in a significant decrease of the "vis a tergo"--e.g. the threatening prolaps of intraocular tissue. Preliminary experience revealed that intraoperative complications in these difficult situations are reduced. As this facilitates also the task of the anaesthesist this broadens our indications for complex intraocular surgery particularly in younger patients.