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The method of the blood loss severity estimation, basing on electrochemically registered globular volume deficiency (ECRGVD) was proposed. Classification of an acute blood loss severity accordingly to ECRGVD value was elaborated, general principles of complex treatment of hemorrhage were proposed.
One hundred and fifty patients with severe falciparum malaria were administered sequential combination of dihydroartemisinin suppository followed by an oral mefloquine tablet. Dihydroartemisinin suppositories (80 mg/capsule) were given rectally once daily for 3 days with a total dose of 8-10 mg/kg. Two doses of mefloquine, 15 mg/kg/dose and 10 mg/kg/dose, were given at 72 hr and 84 hr, respectively. The mean [SD] parasite clearance time and fever clearance time were 46.1 [15.7] hr and 82.5 [59.6] hr, respectively. No death or major adverse drug effects occurred. The cure rate at 28 days of inpatient follow-up was 95% (113 of 119 patients). In severe malaria, dihydroartemisinin suppository followed by oral mefloquine is a suitable alternative treatment to parenteral drugs.
The authors report a case of lethal neonatal dwarfism characterized by striking micromelia, fused rudimentary and supernumerary digits, large, soft head, pronounced hypertelorism, protruding eyes set laterally, enormous omphalocele and severe deficiency of tubular bone and spine ossification. Histologic examination showed lack of ossification of the cartilaginous anlage of many tubular bones. The cartilage had irregularly distributed chondrocytes. The matrix contained hypocellular and degenerated areas with scattered large chondrocytes. In a few bones a very disorganized growth cartilage was present. The case is similar to that described by Piepkorn et al. (1977) and may represent a severe form of "boomerang dysplasia" (Kozlowski et al., 1981; Tenconi et al., 1983; Kozlowski et al., 1985; Winship et al., 1990).
OBJECTIVE: To evaluate the acute physiology and chronic health evaluation III (APACHE III) and acute lung injury (ALI) scale in the severity and prognosis of severe acute respiratory syndrome (SARS). METHODS: The clinical data of 38 SARS patients, including survivors (24 cases) and no survivors (14 cases) were collected and evaluated with APACHE III and ALI scoring systems. The correlation of scores and prognosis was evaluated. RESULTS: The scores of APACHE III in the non survivors were higher remarkably than those in the survivor group (P < 0.001). The scores of APACHE III had positive correlation with the overall fatality rate. When the scores of APACHE III was higher than 60, the mortality increased obviously (chi(2) = 3.886, P < 0.05). Elderly patients with SARS who were over 60 years old had a high mortality (chi(2) = 8.660, P < 0.05). The scores of ALI in the non survivors had not statistical significance than those in the survivor group (P = 0.127). CONCLUSIONS: The score of APACHE III in the SARS are correlated with the patient's condition and prognosis. Elderly patients with SARS have a high mortality.
A retrospective analysis was made of clinical and laboratory findings from 96 patients admitted to a general resuscitation unit for severe mechanical injury and shock. The degree of experienced shock, score rate of shock production in an injury, parameters of central hemodynamics, biochemistry and coagulogram of mixed venous blood, acid-base balance of arterialized capillary blood were analyzed in all the affected patients. The data were processed by a computer by widely using the multifactor regression analysis and special conversion of homeostatic values. With the derived decision rule, the standardized severity was retrospectively calculated for the affected patients in the time course during the posttraumatic period and this assessment was demonstrated to be comparable with clinical findings.
Since the 14kb human Duchenne muscular dystrophy (DMD) cDNA was cloned and its protein product "dystrophin" was discovered, immunochemical, biochemical and genetic analyses of dystrophin (dystrophin testing) have provided an accurate diagnosis of DMD/Becker muscular dystrophy in the clinical field. We performed dystrophin testing for a 5-year-old boy and confirmed he had severe BMD. Multiplex PCR of the DMD gene showed in-frame type deletion from exon 45 to 48. Immunohistochemical analysis of the muscle specimen obtained from the patient showed a discontinuous and patchy staining pattern, using monoclonal antibody that recognizes the C-terminus domain of dystrophin. On immunoblot analysis, we detected a faint band of 390 kDa. Dystrophin quantity was less than 10% of that compared to normal controls. The correlation between clinical severity and dystrophin testing was discussed.
Cellular and mediator profiles in bronchoalveolar lavage have not been compared systematically between patients with asthma of different severities, mainly because the patients with more severe asthma have an increased need for antiinflammatory medication. Information is limited to comparisons of allergic and intrinsic asthma, which can be distinguished clinically. When patients from these two groups with similar degrees of bronchial hyperresponsiveness were compared, both groups showed increased numbers of activated T-helper lymphocytes; those in the allergic group expressed the IL-2 receptor (CD25+), whereas in patients with intrinsic asthma there was also an increased number of T-suppressor cells with the activation markers CD25, class II histocompatibility antigen, and very late activation antigen-I, as well as T-helper cells class II histocompatibility antigen and very late activation antigen-I. This pattern is compatible with a more chronic T-cell activation in patients with intrinsic asthma. In patients with allergic asthma the cytokine pattern is compatible with a pure TH2 response (elevated IL-4 and IL-5); however, intrinsic asthma is characterized by elevated IL-5 and IL-2 but not IL-4. Our own findings show similar concentrations of IL-1, IL-8, and granulocyte-macrophage colony-stimulating factor in bronchoalveolar lavage fluid of patients with allergic and intrinsic asthma, whereas IL-6 and interferon-gamma tended to be higher in patients with intrinsic asthma. There are probably fundamental differences in the pathogenesis of allergic and intrinsic asthma. These findings suggest that asthma does not depend on the presence of IgE or IL-4, although both may contribute to the pathogenesis of atopic asthma. The only common pathway in the different presentations of asthma that has been related to clinical symptoms appears to be IL-5-mediated activation of eosinophils; therapies aimed at this mechanism may be promising.
BACKGROUND: Hospital mortality and length of stay, both adjusted for severity of illness, have been used as indicators of effectiveness and efficiency of health care in critical patients. PATIENTS AND METHODS: 1,270 adult critical patients, consecutively admitted in 17 intensive care units (ICU) from Catalonia and the Balearic Islands, Spain, have been included. For each hospital, effectiveness has been assessed with a quality performance index (QOI) obtained by dividing the number of observed deaths by the number of deaths expected according to the MPM system (MPM II0). Efficiency has been assessed with a resource utilization index (RUI) obtained by dividing the number of observed weighted hospital days (WHD) by the number of expected WHD. WHD is a measure of resource use which weights ICU days more heavily than non-ICU days. Expected WHD have been obtained by a regression model including severity of illness and the presence/absence of surgery. RESULTS: Ten of the 17 hospitals life within one standard deviation of the mean on both clinical and economical indices. There are 3 hospitals with optimal values on both indices. There is no evidence of association between effectiveness and resource utilization. CONCLUSIONS: Clinical and economical performance of hospitals can be quantified with simple indicators which allow to compare centers. Hospitals can be effective and efficient at the same time.
We report on a child with severe midline facial cleft, bilateral cleft lip and palate, telecanthus, S-shaped palpebral fissures, limbic dermoid, midface hypoplasia, hypoplastic corpus callosum, and multiple skin appendages. This case may be an example of severe frontofacionasal "dysplasia" or a newly recognised syndrome.
Severe chronic neutropenia (SCN) is defined as an absolute neutrophil (ANC) of less than 0.5 x 10(9)/L, lasting for months or years. Congenital, cyclic, and idiopathic neutropenia are principal categories of SCN. Since 1994, the Severe Chronic Neutropenia International Registry (SCNIR) has collected data to monitor the clinical course, treatments, and disease outcomes for SCN patients. This report summarizes data for 853 patients, almost all treated with daily or alternate-day recombinant human granulocyte colony-stimulating factor (G-CSF or Filgrastim). G-CSF treatment increased the ANC overall from 0.34 x 10(9)/L +/- 0.018 pre-treatment to 3.70 x 10(9)/L +/- 0.18 during the first year of treatment. For most patients, the responses were durable with patients remaining on the same dose of G-CSF for many years. Long-term hematological observations showed stable mean leukocyte and neutrophil counts and gradually increasing hemoglobin levels. Thrombocytopenia developed in 4% of patients. As of January 1, 2000, myelodysplasia (MDS) or acute myelogenous leukemia (AML) has occurred in 35 of 387 patients with congenital neutropenia with a cumulative risk of 13% after 8 years of G-CSF treatment. This event occurred without a predictable relationship to the duration or dose of G-CSF treatment. No patients with cyclic or idiopathic neutropenia developed MDS or AML. Other important adverse events included hepatomegaly, osteoporosis, vasculitis, glomerulonephritis, and deaths in 4 of 14 cases requiring splenectomy. Growth and development and the outcome of pregnancy appeared to be unaffected by G-CSF treatment. These data indicate that congenital, cyclic, and idiopathic neutropenia can be effectively treated with long-term G-CSF. The risk of leukemia, osteoporosis, other potentially adverse events, and pregnancy outcome need to be further evaluated with continuing long-term observations.
We report on an Italian patient affected by severe lymphedema of lower limbs, genitalia and face, intestinal lymphangiectasia, seizures, and moderate mental retardation. He has a flat face, flat nasal bridge, and hypertelorism. We propose that he presents with a severe form of Hennekam syndrome.
We report on two brothers, born to double first cousin Jordanian Arab parents, with a syndrome comprising severe hypertelorism with upslanted palpebral fissures, brachycephaly, abnormal ears, sloping shoulders, enamel hypoplasia, and osteopenia with repeated fractures. Both have severe myopia, mild to moderate sensori-neural hearing loss and borderline intelligence. Results of chromosome analysis were normal as was a FISH assay for subtelomeric rearrangements. The father has mild hypertelorism but the family history is otherwise unremarkable. We think that this represents a previously unrecognized autosomal or X-linked recessive syndrome.
UNLABELLED: An infant with severe deficiency of complex III combined with less severe deficiencies of complexes I, II and IV of the mitochondrial respiratory chain in skeletal muscle tissue presented with intra-uterine growth retardation, generalized hypotonia and delayed motor development. In the following 3.5 years muscle tone and motor development gradually normalized whereas the lactic acidosis and enzyme activities did not improve. CONCLUSION: This report documents a favourable clinical course in a child with combined respiratory chain deficiency despite persistent biochemical abnormalities.
Lipoprotein lipase (LPL, triacylglycerol acylhydrolase; EC 3.1.1.3) deficiency (OMIM 238600) is an autosomal recessive inherited condition caused by mutations in the LPL gene, either in a homozygous or in a compound heterozygous state, leading to loss of lipolytic activity and resulting in severe hypertriglyceridaemia and subsequent risk for developing pancreatitis. Numerous LPL gene mutations leading to loss of catalytic function have been described. In this present study, we describe full clinical, biochemical and molecular analyses of severe hypertriglyceridaemic individuals in one Turkish and three Chinese families. We established one novel mutation (delCT1312-1313), a new combination of mutations (S193R and I194T) and four previously reported mutations (L252R, L252V, S193R and I194T) of the LPL gene and report phenotypes for these and four previously described mutations. Finally, we show that two patients homozygous for the LPL gene delCT1312-1313 mutations are characterized by absence of LPL activity that coincides with absence of LPL protein.
Transient ischemic dilatation (TID) of the left ventricle observed during single photon myocardial perfusion emission computed tomography (SPECT) is an important non-perfusion finding that may not only suggest underlying significant (usually multi-vessel) coronary artery disease (CAD) but also an independent prognostic factor of adverse outcomes regardless of abnormal or normal perfusion finding. We present a patient with no significant epicardial coronary disease who had significant TID and considerable decrease in the left ventricular ejection fraction with left ventricular dilatation after a rest-stress Tc-99 tetrofosmin SPECT study in the setting of severe aortic stenosis. With the advent of gated SPECT imaging the additive value of determining rest and post stress EF, as demonstrated in this case, aided in the recognition of TID and transient decrease in the left ventricular ejection fraction. These are not necessarily related to obstructive epicardial coronary disease, but are a result of severe aortic valve disease causing subendocardial ischemia in the setting of multilple other non-ischemic etiologies of TID such as left ventricular hypertrophy and diabetes mellitus.
As the influence of sepsis on plasma cholinesterase activity is not clearly established, plasma cholinesterase activity was measured in 30 healthy surgical orthopaedic patients, 11 patients with severe postoperative infections and 18 patients with patent cirrhosis (Child-Pugh C). Plasma cholinesterase activities were significantly decreased (P < 0.001) in patients with postoperative infections (1,706 +/- 535 Ul.L-1) and in those with patent cirrhosis (1,318 +/- 538 Ul.L-1) in comparaison to healthy surgical patients (4,716 +/- 1,232 Ul.L-1). The decrease in patients with postoperative infections and in those with patent cirrhosis was similar. It remains to be assessed whether the activity of anaesthetic agents biotransformed by plasma cholinesterase is modified during severe infections.
A systematic neuropsychological assessment technique is described for use with severely physically disabled individuals, possibly with combined motor and cognitive disorders. Target neurological conditions may be, e.g., an incomplete locked-in state, a minimally conscious state, or severe combinations of paralysis, agnosia, and apraxia. Neuropsychological assessment in these patients is difficult, because standard neuropsychological tests require fast motor responses, which can be manual, verbal, or both. To assess the cognitive status of patients with residual motor function, tests have been applied that can be answered by a binary (yes/no) signal and whose outcomes were not based on reaction times. Further, a battery of neurophysiological examination procedures based on event-related brain potentials has been developed. These procedures can be performed directly at a patient's bedside (at home or in a hospital) and applied for assessment of cognitive functions even in patients without residual motor function.
Sixty-five consecutive eligible adult patients, who were treated as outpatients for stable severe-to-very severe COPD, were enrolled in the study. All of them received 23-valent pneumococcal capsular polysaccharide vaccine intramuscularly. Patients were seen monthly, as well as whenever they had symptoms suggestive of an exacerbation, at our outpatient clinic. Eighteen out of 65 patients suffered from acute exacerbation (AECOPD). Three of these patients presented two episodes of AECOPD. Patients with an acute exacerbation of COPD received azithromycin 500 mg/day once daily for 3 days and a short course of oral prednisolone 25 mg/die. In 16 cases, a single species was isolated, while in the remaining 5 cases at least two species were recovered. Clinical cure or improvement at the end of therapy (3-5 days post-therapy) was reported in 17 episodes of AECOPD with no relapse at the late post-therapy (10-14 days after the completion of treatment). Bacteriologic eradication or presumptive eradication rates at the end of therapy were 86% (24 out of 28 isolates). Azithromycin eradicated all isolates of Haemophilus influenzae, Moraxella catarrhalis, H. parainfluenzae, Klebsiella pneumoniae, and Klebsiella spp. isolated at baseline. Eradication of Sta aureus occurred in 1 of 3 isolates whereas azithromycin was unable to eradicate Pseudomonas aeruginosa isolates. Our data seem to indicate that pneumococcal vaccination reduces the possibility that an AECOPD is caused by Streptococcus pneumoniae. This finding allows the use of antibiotics such as azithromycin, which, otherwise, should be avoided because of resistances.