Showing papers on "Fulminant published in 2004"
TL;DR: In this article, a spectrum of disease ranging from a mild, self-limited course requiring only brief hospitalization to a rapidly progressive, fulminant illness resulting in the multiple organ dysfunction syndrome (MODS), with or without accompanying sepsis.
Abstract: Objective:Acute pancreatitis represents a spectrum of disease ranging from a mild, self-limited course requiring only brief hospitalization to a rapidly progressive, fulminant illness resulting in the multiple organ dysfunction syndrome (MODS), with or without accompanying sepsis. The goal of this c
295 citations
TL;DR: Intense CD8+ T cell responses to SNV may be induced by the encounter of the unnatural human host to this zoonotic virus without coevolution, which may also be the immunopathologic basis of other life-threatening human virus infections.
Abstract: We report on the role of specific CD8 + T cells in the pathogenesis of a highly lethal human viral disease, hantavirus pulmonary syndrome (HPS). HPS is a zoonotic disease caused by transmission of Sin Nombre virus (SNV) from chronically infected deer mice. In humans, this fulminant infection is characterized by lung capillary leakage, respiratory failure, and cardiogenic shock. Individuals with HLA-B*3501 have an increased risk of developing severe HPS, suggesting that CD8 + T cell responses to SNV contribute to pathogenesis. We identified three CD8 + T cell epitopes in SNV presented by HLA-B*3501 and quantitated circulating SNV-specific CD8 + T cells in 11 acute HPS patients using HLA/peptide tetramers. We found significantly higher frequencies of SNV-specific T cells in patients with severe HPS requiring mechanical ventilation (up to 44.2% of CD8 + T cells) than in moderately ill HPS patients hospitalized but not requiring mechanical ventilation (up to 9.8% of CD8 + T cells). These results imply that virus-specific CD8 + T cells contribute to HPS disease outcome. Intense CD8 + T cell responses to SNV may be induced by the encounter of the unnatural human host to this zoonotic virus without coevolution. This may also be the immunopathologic basis of other life-threatening human virus infections.
166 citations
TL;DR: It is concluded that influenza-associated acute encephalopathy may be an underestimated syndrome and is another reason to promote vaccination against influenza in infants and younger children.
159 citations
TL;DR: The transjugular shunt provides an excellent outcome in patients with severe fulminant/ACute, subacute, and chronic Budd-Chiari syndrome and may be regarded as a treatment for the acute and long-term management of these patients.
138 citations
TL;DR: The use of lamivudine in the treatment of immunocompetent patients with acute hepatitis B is limited and its use in clinical practice is still under investigation.
Abstract: Background: Experience with lamivudine treatment of immunocompetent patients with acute hepatitis B is limited.
Aim of study: To evaluate the safety and efficacy of lamivudine for the treatment of acute severe hepatitis B virus (HBV) infection in immunocompetent adults.
Patients and Methods: Fifteen patients (10 men, 5 women, mean age 34.3±7.3 years) with severe acute HBV infection were treated with lamivudine 100 mg daily for 3–6 months, starting 3–12 weeks after onset of infection. Prior to treatment, 5 patients had grade 1–4 encephalopathy; all patients had severe coagulopathy (mean INR was 4.5±6.4), and all patients had evidence of severe hepatocyte lysis (mean alanine aminotransferase 3738±1659 U/L, and mean total serum bilirubin 18±6.8 mg/dl). All patients had evidence of highly replicative HBV (mean HBV DNA 13.5 × 106±11 × 106 copies/ml).
Results: Thirteen patients (86.6%) responded to treatment. Encephalopathy disappeared within 3 days of treatment and coagulopathy improved within 1 week. Serum HBV DNA was undetectable (by polymerase chain reaction) within 4 weeks, and serum liver enzyme levels normalized within 8 weeks. Two patients in whom lamivudine therapy was delayed developed fulminant hepatitis and underwent urgent liver transplantation. (One died of vascular complications 1 month later). The 11 patients who were serum HBeAg-positive before treatment seroconverted, and HBeAb developed within 12 weeks in 9 of them; HBsAg was undetectable in all 11 tested patients, and protective titer of HBsAb developed within 12–16 weeks in 9 of them. Therapy was well tolerated in all cases.
Conclusions: These data indicate that lamivudine induces a prompt clinical, biochemical, serological and virological response in immunocompetent patients with de novo HBV infection. Lamivudine may prevent the progression of severe acute disease to fulminant or chronic hepatitis and should be considered for use in selected patients. A large randomized controlled, double-blind prospective study is needed.
89 citations
TL;DR: A high prevalence of exposure to factors potentiating hepatic damage with acute hepatitis B contributed to the outbreak's high mortality rate; mutations present in the outbreak strain might also have been a factor.
70 citations
TL;DR: The high incidence of intracerebral hemorrhage in the study raises concerns about the safety of t-PA in children with fulminant meningococcemia, and highlights the need to avoid strategies that use experimental drugs in an uncontrolled fashion and to participate in multiple-center trials, which are inevitably required to study rare diseases.
Abstract: Objective: Meningococcal disease causes septic shock with associated disseminated intravascular coagulation and hemorrhagic skin necrosis. In severe cases, widespread vascular thrombosis leads to gangrene of limbs and digits and contributes to morbidity and mortality. Uncontrolled case reports have suggested that thrombolytic therapy may prevent some complications, and the use of tissue plasminogen activator (t-PA) has been widespread. Our aim was to summarize the clinical outcome and adverse effects where systemic t-PA has been used to treat children with fulminant meningococcemia. Design: International, multiple-center, retrospective, observational case note study between January 1992 and June 2000. Setting: Twenty-four different hospitals in seven European countries and Australia. Patients: A total of 62 consecutive infants and children with severe meningococcal sepsis in whom t-PA was used for the treatment of predicted amputations and/or refractory shock (40 to treat severe ischemia, 12 to treat shock, and ten to treat both). Interventions: t-PA was administered with a median dose of 0.3 mg[middle dot]kg-1[middle dot]hr-1 (range, 0.008-1.13) and a median duration of 9 hrs (range, 1.2-83). Main Results: Twenty-nine of 62 patients died (47%; 95% confidence interval, 28-65). Seventeen of 33 survivors had amputations (11 below knee/elbow or greater loss; six less severe). In 12 of 50 patients to whom t-PA was given for imminent amputation, no amputations were observed. Five developed intracerebral hemorrhages (five of 62, 8%; 95% confidence interval, 0.5-16). Of these five, three died, one developed a persistent hemiparesis, and one recovered completely. Conclusions: The high incidence of intracerebral hemorrhage in our study raises concerns about the safety of t-PA in children with fulminant meningococcemia. However, due to the absence of a control group in such a severe subset of patients, whether t-PA is beneficial or harmful cannot be answered from the unrestricted use of the drug that is described in this report. Our experience highlights the need to avoid strategies that use experimental drugs in an uncontrolled fashion and to participate in multiple-center trials, which are inevitably required to study rare diseases.
58 citations
TL;DR: The pathologic findings of the lung in 3 cases of SARS were reported, and the presence of the SARS virus was determined by nested reverse transcription–polymerase chain reaction (RT-PCR), and the infected cells were identified by in situ hybridization in open-lung biopsy and postmortem necropsy specimens.
Abstract: Previous reports have indicated that patients with severe acute respiratory syndrome (SARS)-associated coronavirus infection could develop atypical pneumonia with fulminant pulmonary edema. However, the target cells of SARS viral infection have not been characterized in detail. We report the pathologic findings of the lung in 3 cases of SARS. Chest radiographs at 2 to 3 weeks of infection revealed an atypical pneumonia with pulmonary consolidation, a clinical characteristic of SARS infection. The presence of the SARS virus was determined by nested reverse transcription-polymerase chain reaction (RT-PCR), and the infected cells were identified by in situ hybridization in open-lung biopsy and postmortem necropsy specimens. Expression of SARS virus-encoded RNA was detected in all 3 cases by RT-PCR, and the SARS viral signal was localized in pneumocytes by using in situ hybridization.
52 citations
TL;DR: Patients with AIH type 1 are more likely to be older and have cirrhosis at the time of diagnosis, whereas those withAIH type 2 may have a more fulminant course and require life-long immunosuppressant medications.
Abstract: Autoimmune hepatitis (AIH) remains an enigmatic condition that affects children of all ages. Although much knowledge has emerged over the past four decades regarding autoimmune diseases, the lack of a spontaneous animal model has hindered a more complete understanding of the pathophysiology of AIH. Serum autoimmune markers, combined with biochemical and histologic evidence of hepatocellular injury and absent other diagnostic considerations, are necessary to diagnose AIH. The clinical spectrum ranges from asymptomatic elevation of aminotransferase levels to acute liver failure. Patients with AIH type 1 are more likely to be older and have cirrhosis at the time of diagnosis, whereas those with AIH type 2 may have a more fulminant course and require life-long immunosuppressant medications. Overlap syndromes occur in children but are rare. Treatment with prednisone and azathioprine, alone or in combination, reverses the clinical course and improves biochemical abnormalities. Should liver transplantation be necessary, autoimmune injury may recur in the allograft.
50 citations
TL;DR: Peripheral immune reaction was observed in 69.2% of fulminant Type 1 diabetic patients, indicating that autoreactive T cells might contribute, at least in part, to the development of fulmineant Type 2 diabetes.
Abstract: Aims/hypothesis
Fulminant Type 1 diabetes is a novel subtype of Type 1 diabetes that involves the abrupt onset of insulin-deficient hyperglycaemia. This subtype appears to be non-autoimmune because of the absence of diabetes-related autoantibodies in the serum, and of insulitis in pancreatic biopsy specimens. The pathogenesis of the disease is still unknown. In this study, we investigated whether T cell autoimmune responses are involved in fulminant Type 1 diabetes.
50 citations
TL;DR: The risk of a fulminant course of acute myocarditis was high in patients with elevated C-reactive protein, and creatine kinase concentrations, decreased left ventricular ejection fraction, and intraventricular conduction disturbances at the time of admission.
Abstract: Background A fulminant course can be difficult to predict at the onset of acute myocarditis, so the aim of the present study was to identify the predictive clinical symptoms/signs or laboratory findings. Methods and Results Thirty-nine patients with acute lymphocytic myocarditis, excluding 8 who manifested shock at admission, were studied. The fulminant group was defined as 12 patients who developed shock after admission, requiring intraaortic balloon pumping or percutaneous cardiopulmonary support, and the non-fulminant group comprised the 27 patients without shock. Various parameters at admission were compared between the 2 groups, together with multiple logistic regression analysis, excluding 6 patients with partially missing values. In the fulminant group, C-reactive protein (7.0±7.0 vs 2.3±2.2 mg/dl, p<0.01) and creatine kinase (1,147±876 vs 594 ±568 IU/L, p<0.05) concentrations were higher, intraventricular conduction disturbances were more frequent (9/12 vs 7/27 patients, p<0.01) and the left ventricular ejection fraction was lower (40.7±13.9 vs 50.1±10.6%, p<0.05) than in the non-fulminant group. In the multiple logistic regression analysis model with the presence/absence of a fulminant course considered as the independent variable, and C-reactive protein, creatine kinase, intraventricular conduction disturbances, and left ventricular ejection fraction as dependent variables, a high-risk group (expected proportion of fulminant course ≥0.5) and a low-risk group (<0.5) could be differentiated. A fulminant course occurred in 9/13 (69%) patients in the high-risk group, but in only 2/20 (10%) patients in the low risk group (p<0.001). Conclusions The risk of a fulminant course of acute myocarditis was high in patients with elevated C-reactive protein, and creatine kinase concentrations, decreased left ventricular ejection fraction, and intraventricular conduction disturbances at the time of admission. (Circ J 2004; 68: 734 - 739)
01 Feb 2004
TL;DR: 4 cases of fulminant gram-negative bullous cellulitis and septic shock in patients with cirrhosis are reported.
Abstract: Gram-negative infections are common in patients with cirrhosis, but skin infections are usually caused by gram-positive cocci. Gram-negative bacteria should be considered as a potential etiologic agent in patients with cirrhosis and severe bullous cellulitis. Culture of the bullous fluid may facilitate diagnosis and management. Early recognition is important because the course of the disease is usually rapid and fatal. We report 4 cases of fulminant gram-negative bullous cellulitis and septic shock in patients with cirrhosis.
TL;DR: Patients who survive the acute phase crisis of acute myocarditis have a favorable long-term survival rate, whether or not mechanical support is used.
Abstract: Background The long-term prognosis and cardiac function of fulminant myocarditis treated with percutaneous cardiopulmonary support (PCPS) was compared with the outcome of those not treated with PCPS. Methods and Results From 1991 to 2000, 14 patients with fulminant myocarditis (left ventricle ejection fraction (LVEF) ≤40%) were admitted to hospital. PCPS was necessary for treatment of shock in 8 (PCPS group), but not for the remaining 6 patients (non-PCPS group). In the PCPS group, 6 patients (75%) survived the critical phase and did not have any cardiac problems after discharge (range of follow-up period, 1.4-6.0 years). All patients in the non-PCPS group survived the acute phase; 1 patient had congestive heart failure 1.5 years after discharge, and another died from malignancy (follow-up period range, 2.2-9.4 years). Although the left ventricular ejection fraction (LVEF) of the PCPS group was significantly lower than that of the non-PCPS group in the acute phase, there was no significant difference in LVEF between the 2 groups in the chronic phase. Conclusion Patients who survive the acute phase crisis of acute myocarditis have a favorable long-term survival rate, whether or not mechanical support is used. (Circ J 2004; 68: 829 - 833)
TL;DR: SAA, CRP and WBC counts can be used as prognostic markers in LOS in preterm infants, with SAA being the earliest prognostic marker.
Abstract: Aim Late-onset sepsis (occurring after the first three days of life) is a serious complication in preterm infants. In order to assess the possible prognostic virtues of the acute phase inflammatory response in the disease, we compared the inflammatory response of preterm infants who died within 72 hours (h) (fulminant sepsis) to infants who recovered from the disease (non-fulminant sepsis). Methods Of 42 preterm infants that were evaluated: 10 had fulminant sepsis and 32 non-fulminant sepsis. Acute phase inflammatory response markers-C-reactive protein (CRP), serum amyloid A (SAA), interleukin (IL)-6 levels and white blood cell (WBC) counts were measured at the first suspicion of LOS and after 8, 24 and 48 h. Results Small for gestational age (SGA) infants who were treated with fewer days of antibiotics characterized the fulminant sepsis group. The initial high levels of inflammatory markers were similar in both groups, but as early as 8 h after onset significantly lower levels of SAA, CRP and WBC counts were documented in the fulminant sepsis group. The inflammatory response remained low at 24 and 48 h in the fulminant sepsis group, while in the survivors, significantly increased inflammatory markers were measured. Decreases in the levels of the inflammatory markers preceded episodes of metabolic acidosis and arterial hypotension that were more common in the fulminant sepsis group. Infant mortality correlated inversely with SAA levels at 8 h and with CRP and WBC counts at 24 h after onset. Conclusion SAA, CRP and WBC counts can be used as prognostic markers in LOS in preterm infants, with SAA being the earliest prognostic marker.
TL;DR: A fulminant case of streptococcal toxic shock syndrome and high dose intravenous immunoglobulin (IVIG) has shown promising effects in recent publications.
Abstract: A fulminant case of streptococcal toxic shock syndrome is described. Early surgery was life saving, and the antibiotic regimen should include clindamycin. The value of secondary measures is discussed. High dose intravenous immunoglobulin (IVIG) has shown promising effects in recent publications. Hyperbaric oxygen (HBO) treatment is under evaluation. Piercing of mucosal surfaces might be associated with severe infections.
TL;DR: A 25-year-old previously healthy pregnant woman who presented with fatigue, fever, and anicteric hepatitis is reported, with a miliary pattern of multiple, hypovascular, subcentimeter lesions scattered throughout the liver.
Abstract: Fulminant hepatitis due to herpes simplex virus (HSV) is rare in immunocompetent adults. Most reported cases have clearly established pregnancy as a condition that can predispose to disseminated HSV infection. We report a case of a 25-year-old previously healthy pregnant woman who presented with fatigue, fever, and anicteric hepatitis. Triphasic contrast-enhanced computed tomography demonstrated a miliary pattern of multiple, hypovascular, subcentimeter lesions scattered throughout the liver. Familiarity with the clinical findings and computed tomographic appearance may prompt early recognition of fulminant HSV hepatitis and allow differentiation from other hepatic disease during pregnancy.
TL;DR: A case of varicella infection in a previously healthy young adult complicated with acute respiratory distress syndrome (ARDS), rhabdomyolysis, acute hepatitis and disseminated intravascular coagulation (DIC) is described.
Abstract: Primary varicella infection in immunocompetent adults is very rare, but it has a high mortality rate because of serious complications. We describe a case of varicella infection in a previously healthy young adult complicated with acute respiratory distress syndrome (ARDS), rhabdomyolysis, acute hepatitis and disseminated intravascular coagulation (DIC). Acyclovir was administered for varicella infection and the ARDS was successfully treated with steroidpulse therapy, hemofiltration and a mechanical respiratory support with a positive end-expiratory pressure. Early administration of antiviral agents and extensive management were thought to be necessary for such patients with severe complications.
TL;DR: This report describes an unusual case in which spontaneous tumor lysis occurred in anaplastic large T-cell lymphoma associated with acute uric acid nephropathy, persistent oliguria, and shock, and chronicles the successful experience with continuous renal replacement therapy in the presence of compromised hemodynamic status.
Abstract: Acute spontaneous tumor lysis (ASTL) syndrome, an extremely rare disease, requires prompt recognition and aggressive management because it is fulminant at its outset, associated with severe metabolic derangement, and potentially reversible. We describe an unusual case in which spontaneous tumor lysis occurred in anaplastic large T-cell lymphoma associated with acute uric acid nephropathy, persistent oliguria, and shock. This case contrasts markedly with previously reported cases of ASTL syndrome, which developed mainly in the pathologic type of Burkitt lymphoma. To our knowledge, this is the first reported occurrence of ASTL syndrome associated with anaplastic large T-cell type lymphoma. This report also chronicles our successful experience with continuous renal replacement therapy in the presence of compromised hemodynamic status.
TL;DR: The first case of disseminated cryptococcosis caused by C. albidus is reported in a renal transplant recipient who had been successfully treated with fluconazole monotherapy, and this organism was isolated by skin biopsy and tissue culture.
Abstract: Cryptococcus albidus, a non-neoformans species of the genus Cryptococcus, is generally regarded as a rare cause of disease. There have been only 14 previously reported cases in which this organism has been isolated as a pathogen, none of which occurred in a renal transplant recipient. A 23-year-old renal transplant recipient taking medication consisting of cyclosporine and prednisolone was admitted with a 10-day history of dry cough, fever and progressive dyspnea. The next day, his respiratory status deteriorated dramatically, and he developed acute respiratory distress syndrome (ARDS) and fulminant septic shock. On the eighth hospital day, tender macules on both his shins coalesced to form erythematous patches. Cryptococcus albidus was isolated by skin biopsy and tissue culture. We report here the first case of disseminated cryptococcosis caused by C. albidus in a renal transplant recipient who had been successfully treated with fluconazole monotherapy.
TL;DR: Post-mortem analysis revealed large CD30- and CD45-positive lymphoma cells in an intravascular distribution in most of the organs studied and Histopathology of the spleen and bone marrow was significant for fulminant hemophagocytosis.
Abstract: We report a 55-year-old male with a diagnosis of intravascular lymphoma and hemophagocytosis. He initially presented with hemolytic anemia and splenomegaly and was successfully treated with oral steroids. His clinical course was later complicated by fever, cytopenias, hypoalbuminemia, disseminated intravascular coagulation, gastrointestinal bleeding and acute tubular necrosis. Results of an extensive investigation for fever of unknown origin were negative. Although the patient was treated aggressively with antimicrobials, transfusion support and dialysis, he expired 3 weeks after hospitalization. Post-mortem analysis revealed large CD30- and CD45-positive lymphoma cells in an intravascular distribution in most of the organs studied. Histopathology of the spleen and bone marrow was significant for fulminant hemophagocytosis.
Journal Article•
TL;DR: Early empirical antibiotic therapy should be given for severe pneumonia after cases of pulmonary melioidosis from Srinagarind Hospital, Khon Kaen, northeast Thailand, 90 acute vs 72 subacute/chronic forms.
Abstract: Between 1996 and 2002, 162 cases of pulmonary melioidosis were reported from Srinagarind Hospital, Khon Kaen, northeast Thailand, 90 acute vs 72 subacute/chronic. Patients averaged 50 years of age and half worked as farmers. The male to female ratio was between 2 and 3 to 1 depending on the subgrouping. Burkholderia pseudomallei was confirmed by a culture or a four-fold rise in titer in the majority of cases, while the others were presumptive diagnoses based on response to treatment. Pulmonary melioidosis presented as either acute fulminant pneumonia or as an indolent disease. The common concurrent medical illness was diabetes mellitus. Mean incubation of the acute vs the sub-acute/chronic form was 8.7 vs 54.4 days, respectively. Leukocytosis was detected in 70% of cases. Sputum Gram's stain was not sensitive for diagnosis. Sputum culture and blood culture were diagnostic for 31.1 vs 22.2 and 40 vs 37.5% of the acute vs subacute/chronic forms, respectively. The common radiographic patterns for acute pneumonia were localized patchy alveolar infiltrate or hematogenous pattern. A bilateral diffuse patchy alveolar infiltration or multiple nodular lesions characterized the latter. Upper-lobe involvement with early cavitation and rapid progression were common. In the subacute/chronic forms, the radiographic pattern sometimes mimicked tuberculosis, with upper lobe involvement, patchy alveolar infiltrate with cavities or fibroreticular lesions. In approximately 30% of cases, liver and/or splenic abscess were common sites of extrapulmonary infection. Respiratory failure and septic shock from acute pulmonary melioidosis was 20% fatal. Early empirical antibiotic therapy should be given for severe pneumonia.
TL;DR: There is some evidence supporting the immunogenetic predisposition mechanism in the development of this subtype of fulminant autoantibody-negative type 1 diabetes.
Abstract: Since 11 novel cases of fulminant autoantibody-negative type 1 diabetes were introduced by Imagawa and colleagues (1,2), about 150 additional cases have been reported. The development of typical fulminant autoantibody-negative type 1 diabetes is strictly confined to Japan. Although the pathogenetic mechanism still remains unclear, there is some evidence supporting the immunogenetic predisposition mechanism in the development of this subtype. A fulminant autoantibody-negative type 1 diabetic patient with insulitis and exocrine pancreatitis showed infiltration of a large number of CD8+ T-cell and peripheral GAD-reactive interferon-γ-producing CD4+ T-cell (3). According to previous reports, some HLA haplotypes, DRB1\*0405-DQB1\*0401, DQA1\*0303-DQB1\*0401, DQA1\*0302-DQB1\*0303, and DRB*0901 are closely associated with fulminant autoantibody-negative …
01 Oct 2004
TL;DR: In this article, the authors report the case of a Lemierre's syndrome secondary to a bout of intense cough, a cause not yet described in the literature, highlighting the importance of a quick diagnosis and the institution of an appropriate therapy.
Abstract: Lemierre's syndrome (LS) is an uncommon but potentially life-threatening complication of an anaerobic oropharyngeal infection, affecting young adults and adolescents. The disease is characterised by a septic thrombophlebitis of the internal jugular vein and "metastatic" infections, which can be followed by fulminant sepsis and rapid death. More recently, it has been reported a recrudescence of this condition, which could be attributable to alterations in antibiotic usage patterns. The authors report the case of a LS secondary to a bout of intense cough, a cause not yet described in the literature, highlighting the importance of a quick diagnosis and the institution of an appropriate therapy.
TL;DR: Sensitive and specific diagnostic criteria were established for detection of fulminant type 1 diabetes based on serum C-peptide and HbA(1c) levels at onset that discriminate fulminants type 2 diabetes from acute-onset type 1 Diabetes with high sensitivity and specificity.
Abstract: OBJECTIVE —Diagnostic criteria in fulminant type 1 diabetes, a novel subtype of type 1 diabetes, remain unclear. RESEARCH DESIGN AND METHODS —We analyzed basal and longitudinal changes of serum C-peptide levels during a 75-g oral glucose tolerance test (OGTT) in 125 consecutively recruited patients with type 1 diabetes including fulminant type 1 diabetes ( n = 25) and acute-onset type 1 diabetes ( n = 100). Discriminating criteria of fulminant type 1 diabetes were examined using receiver-operating characteristic curve analysis and multiple logistic regression analysis. RESULTS —The integrated values of serum C-peptide response during OGTT (ΣC-peptide) in fulminant type 1 diabetes at onset, 1 year, and 2 years after onset were markedly lower than those in acute-onset type 1 diabetes. None of the patients with fulminant type 1 diabetes had improvement of C-peptide response to OGTT. Fasting C-peptide values at onset in fulminant type 1 diabetes were significantly lower than those in acute-onset type 1 diabetes. We established diagnostic criteria of serum C-peptide and HbA 1c levels at onset that discriminate fulminant type 1 diabetes from acute-onset type 1 diabetes with high sensitivity and specificity: a criterion in which the levels of both the fasting C-peptide is ≤0.033 nmol/l and HbA 1c is ≤8.0% or a criterion in which the levels of both the ΣC-peptide is ≤0.540 nmol/l and HbA 1c is ≤8.0%. CONCLUSIONS —Fulminant type 1 diabetes has extremely low β-cell function at onset that rarely recovers after onset. Sensitive and specific diagnostic criteria were established for detection of fulminant type 1 diabetes based on serum C-peptide and HbA 1c levels at onset.
TL;DR: In this patient increased inflammatory cytokines ascribable to activation of macrophages and/or helper T cells were considered to play an important role in the pathogenesis of the pulmonary hemorrhage.
Abstract: We report a patient with systemic lupus erythematosus (SLE) who developed fulminant pulmonary hemorrhage. This patient also showed liver dysfunction, bicytopenia and hyperferritinemia, with an increase in serum levels of interleukin (IL)-1β, IL-6 and tumor necrosis factor-α (TNF-α) at the onset of pulmonary symptoms, probably indicating an associated hemophagocytic syndrome. Despite an acute progressive course temporarily requiring mechanical ventilation the patient was successfully treated with continuous drip infusion of tacrolimus, plasmapheresis and intravenous high-dose immunoglobulin and corticosteroid. In this patient increased inflammatory cytokines ascribable to activation of macrophages and/or helper T cells were considered to play an important role in the pathogenesis of the pulmonary hemorrhage. Because this complication is frequently fatal in SLE, intensive therapy, including immunosuppressants and plasmapheresis, should be actively considered as early as possible after onset.
TL;DR: A 35-year-old, Japanese male with fulminant ulcerative colitis is diagnosed with virus-associated hemophagocytic syndrome and successfully treated with antiviral therapy consisting of intravenous ganciclovir, gamma globulin, and granulocyte-colony stimulating factor.
Abstract: We report a case of hemophagocytic syndrome that developed in a 35-year-old, Japanese male with fulminant ulcerative colitis. The patient underwent an emergency operation, consisting of subtotal colectomy, ileostomy with rectal preservation (suprapubic mucous fistula). After the operation, peripheral blood counts showed progressive pancytopenia and bone marrow aspirate smears revealed hypocellular bone marrow with an increase in histiocytes, indicating hemophagocytic syndrome. Viral studies (serum antibody titer and antigenemia of cytomegalovirus) revealed systemic cytomegalovirus infection. The patient was diagnosed with virus-associated hemophagocytic syndrome and was successfully treated with antiviral therapy consisting of intravenous ganciclovir, gamma globulin, and granulocyte-colony stimulating factor.
TL;DR: Two children with fulminant myocarditis successfully treated with a 10-h infusion of high-dose IVIG are reported to have achieved a dramatic clinical improvement, with both cardiac enzymes and myocardial function normalized within 1-2 weeks after treatment.
TL;DR: A 3‐month‐old female who developed fulminant hepatic failure after ingesting less than 8 mL of clove oil was treated with intravenous N‐acetylcysteine (N‐AC) according to a protocol used for acetaminophen poisoning.
Abstract: We present a 3‐month‐old female who developed fulminant hepatic failure after ingesting less than 8 mL of clove oil. Initial treatment involved gastrointestinal decontamination, supportive measures...