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1.
Pan Afr Med J ; 42: 260, 2022.
Article in English | MEDLINE | ID: mdl-36338555

ABSTRACT

The novelty described in this case report is the simultaneous development of arterial and venous thrombosis in a previously healthy Caucasian 37-year-old male with newly diagnosed warm autoimmune haemolytic anaemia (WA-AIHA). Clinical presentation included sensorimotor dysphasia, right arm paresis, abdominal pain, and swelling of the left leg. Computed tomography angiography showed partial occlusion of the left middle cerebral artery and multiple infarcts of the kidneys and spleen, while Doppler ultrasound revealed thrombosis of the left popliteal vein. A therapeutic dose of low-molecular-weight-heparin was instituted together with rituximab, leading to the complete serological and haematological remission. The exact thrombotic risk factors in WA-AIHA are still not completely identified and no generally accepted guidelines on thromboprophylaxis exist. The severe onset of the WA-AIHA might point towards a close association between haemolysis itself and thrombosis, raising the question of the necessity of prophylactic anticoagulation.


Subject(s)
Anemia, Hemolytic, Autoimmune , Thrombosis , Venous Thromboembolism , Venous Thrombosis , Male , Humans , Adult , Anemia, Hemolytic, Autoimmune/complications , Anemia, Hemolytic, Autoimmune/diagnosis , Anticoagulants/therapeutic use , Venous Thrombosis/etiology , Venous Thrombosis/complications
2.
BMC Med Ethics ; 23(1): 12, 2022 02 16.
Article in English | MEDLINE | ID: mdl-35172834

ABSTRACT

BACKGROUND: Decisions about limitations of life sustaining treatments (LST) are made for end-of-life patients in intensive care units (ICUs). The aim of this research was to explore the professional and ethical attitudes and experiences of medical professionals on treatment of end-of-life patients in ICUs in the Republic of Croatia. METHODS: A cross-sectional study was conducted among physicians and nurses working in surgical, medical, neurological, and multidisciplinary ICUs in the total of 9 hospitals throughout Croatia using a questionnaire with closed and open type questions. Exploratory factor analysis was conducted to reduce data to a smaller set of summary variables. Mann-Whitney U test was used to analyse the differences between two groups and Kruskal-Wallis tests were used to analyse the differences between more than two groups. RESULTS: Less than third of participants (29.2%) stated they were included in the decision-making process, and physicians are much more included than nurses (p < 0.001). Sixty two percent of participants stated that the decision-making process took place between physicians. Eighteen percent of participants stated that 'do-not-attempt cardiopulmonary resuscitations' orders were frequently made in their ICUs. A decision to withdraw inotropes and antibiotics was frequently made as stated by 22.4% and 19.9% of participants, respectively. Withholding/withdrawing of LST were ethically acceptable to 64.2% of participants. Thirty seven percent of participants thought there was a significant difference between withholding and withdrawing LST from an ethical standpoint. Seventy-nine percent of participants stated that a verbal or written decision made by a capable patient should be respected. Physicians were more inclined to respect patient's wishes then nurses with high school education (p = 0.038). Nurses were more included in the decision-making process in neurological than in surgical, medical, or multidisciplinary ICUs (p < 0.001, p = 0.005, p = 0.023 respectively). Male participants in comparison to female (p = 0.002), and physicians in comparison to nurses with high school and college education (p < 0.001) displayed more liberal attitudes about LST limitation. CONCLUSIONS: DNACPR orders are not commonly made in Croatian ICUs, even though limitations of LST were found ethically acceptable by most of the participants. Attitudes of paternalistic and conservative nature were expected considering Croatia's geographical location in Southern Europe.


Subject(s)
Decision Making , Intensive Care Units , Attitude of Health Personnel , Croatia , Cross-Sectional Studies , Death , Female , Humans , Male
3.
Heart Lung ; 51: 17-21, 2022.
Article in English | MEDLINE | ID: mdl-34731692

ABSTRACT

BACKGROUND: Self-poisoning accounts for between 3 and 17% of intensive care unit (ICU) admissions. OBJECTIVES: To display the misused substances, intention, and outcome of patients admitted to the ICU due to self-poisoning. METHODS: A retrospective review of ICU admissions using an electronic patient database was performed. RESULTS: A total of 149 patients were admitted, accounting for 4.6% of ICU admissions. Self-poisoning with self-harm was more frequent than without self-harm intent (62.4% vs. 37.6%). 64.4% of ingested substances were misused prescribed drugs. Females used antipsychotics (X2 = 15, p< 0.05) and benzodiazepines (X2 =11.52, p< 0.05), and males ingested antipsychotics (X2 = 12, p< 0.05) with a self-harm intent. Antipsychotics were always used with a self-harm intent. Illicit drugs (X2 =9.14, p< 0.05) and ethanol (X2 =7.34, p< 0.05) were mostly used without self-harm intent. 102 patients (68.5%) continued treatment in a psychiatric clinic, more often women (X2 (1,N = 145)=7.94, p< 0.005). The readmission rate was low. Mortality was 2.6%. CONCLUSION: Self-poisoning is an infrequent cause of ICU admission. Prescribed psychotropic medications, including benzodiazepines, antipsychotics, and antidepressants were the most frequently misused substances. Psychiatric consultation before the ICU discharge and subsequent psychiatric treatment might lower the repetition and readmission rate.


Subject(s)
Antipsychotic Agents , Antipsychotic Agents/therapeutic use , Benzodiazepines , Female , Hospitalization , Humans , Intensive Care Units , Male , Prescriptions , Retrospective Studies
5.
Wien Klin Wochenschr ; 133(15-16): 832-839, 2021 Aug.
Article in English | MEDLINE | ID: mdl-33025258

ABSTRACT

AIMS: To investigate the clinical benefit of routine procalcitonin (PCT) measurement in the medical intensive care unit (ICU) of a tertiary referral hospital. METHODS: Adult patients with suspected infections were included. White blood cells, C­reactive protein (CRP), and PCT were measured. RESULTS: In this study 129 patients of median age 64 years (interquartile range 39-89 years) were prospectively included. The Acute Physiology And Chronic Health Evaluation II (APACHE II) and Sequential Organ Failure Assessment (SOFA) scores were 21 ± 14 and 7 ± 6, respectively. Intensive care unit (ICU) mortality was 22.5%. Immunocompromised patients constituted 39.5%. A significant correlation was observed between PCT and APACHE II (Spearman's rho 0.461, p < 0.01), PCT and SOFA (Spearman's rho 0.494, p < 0.01) and PCT and CRP (Spearman's rho 0.403, p < 0.01). Most patients (n = 83, 64.3%) received antibiotics before admission. No difference in PCT (1.56 ± 8 µg/L vs. 1.44 ± 13 µg/L, p = 0.6) was observed with respect to previous antibiotic therapy. Levels of PCT and CRP were significantly increased in patients with positive blood cultures, the infection caused by Gram-negative microorganism regardless of disease severity and pneumonia with complications. PCT did not differ among patients with positive vs negative urine culture (4.6 ± 16 µg/L vs. 1.76 ± 11.9 µg/L) or positive vs. negative endotracheal aspirate (1.93 ± 11.4 µg/L vs. 1.76 ± 1.11 µg/L). PCT-guided stewardship was applied in 36 patients (28%). CONCLUSION: Increased initial PCT levels might point to the development of more severe disease caused by Gram-negative bacteria, regardless of previous antibiotic treatment. The results pertain to immunocompetent and immunocompromised patients. Implementation of PCT-guided stewardship in those patients is possible and relies on experience as well as knowledge of reference change value for a marker within the specific setting.


Subject(s)
Procalcitonin , Sepsis , Adult , Aged , Aged, 80 and over , Croatia/epidemiology , Humans , Intensive Care Units , Middle Aged , Prognosis , ROC Curve
6.
Acta Clin Belg ; 75(5): 357-361, 2020 Oct.
Article in English | MEDLINE | ID: mdl-31007140

ABSTRACT

OBJECTIVES: Acquired thrombotic thrombocytopenic purpura (aTTP) is a rare autoimmune disorder characterized by auto-antibodies to Willebrand factor (vWF) cleaving enzyme (ADAMTS13), resulting in unusually large vWF multimers that lead to platelet aggregation, microthrombi formation and microangiopathic hemolytic anemia. Hemolysis in aTTP is mechanical; thus, direct antiglobulin test (Coombs test) is usually negative. Multiple autoimmune conditions and various auto-antibodies have been described in the context of chronic myelomonocytic leukemia (CMML). In this paper, we describe the first case of CMML with auto-antibodies to ADAMTS13, presenting initially as plasmapheresis-refractory Coombs-positive aTTP. Results: Although our patient was not treated for CMML, a complete remission of aTTP was eventually achieved with rituximab. Conclusion; We propose that aTTP should be in the differential diagnosis of CMML patients with thrombocytopenia and anemia (Coombs positive or not) who develop signs of thrombotic microangiopathy. Further studies are much needed to decipher the immune-mediated processes in CMML.


Subject(s)
Immunologic Factors/therapeutic use , Leukemia, Myelomonocytic, Chronic/complications , Plasmapheresis , Purpura, Thrombotic Thrombocytopenic/therapy , Rituximab/therapeutic use , ADAMTS13 Protein/immunology , Autoantibodies/immunology , Coombs Test , Female , Glucocorticoids/therapeutic use , Humans , Immunoglobulins, Intravenous/therapeutic use , Methylprednisolone/therapeutic use , Middle Aged , Purpura, Thrombotic Thrombocytopenic/complications , Purpura, Thrombotic Thrombocytopenic/immunology
7.
Crit Ultrasound J ; 8(Suppl 1): 12, 2016 Sep.
Article in English | MEDLINE | ID: mdl-27604617

ABSTRACT

TABLE OF CONTENTS: A1 Point-of-care ultrasound examination of cervical spine in emergency departmentYahya Acar, Onur Tezel, Necati SalmanA2 A new technique in verifying the placement of a nasogastric tube: obtaining the longitudinal view of nasogastric tube in addition to transverse view with ultrasoundYahya Acar, Necati Salman, Onur Tezel, Erdem CevikA3 Pseudoaneurysm of the femoral artery after cannulation of a central venous line. Should we always use ultrasound in these procedures?Margarita Algaba-Montes, Alberto Oviedo-García, Mayra Patricio-BordomásA4 Ultrasound-guided supraclavicular subclavian vein catheterization. A novel approach in emergency departmentMargarita Algaba-Montes, Alberto Oviedo-García, Mayra Patricio-BordomásA5 Clinical ultrasound in a septic and jaundice patient in the emergency departmentMargarita Algaba-Montes, Alberto Oviedo-García, Mayra Patricio-BordomásA6 Characterization of the eyes in preoperative cataract Saudi patients by using medical diagnostic ultrasoundMustafa Z. Mahmoud, Abdelmoneim SuliemanA7 High-frequency ultrasound in determining the causes of acute shoulder joint painMustafa Z. MahmoudA8 Teaching WINFOCUS Ultrasound Life Support Basic Level 1 for Providers in resource-limited countriesAbbas Ali, Alrayah Mustafa, Ihab Abdelrahman, Mustafa Bahar, Osama Ali, H. Lester Kirchner, Gregor ProsenA9 Changes of arterial stiffness and endothelial function during uncomplicated pregnancyAjda Anzic, Paul LeesonA10 Cardiovascular haemodynamic properties before, during and after pregnancyAjda Anzic, Paul LeesonA11 An old man with generalized weaknessMaryam Bahreini, Fatemeh RasooliA12 Ultrasonography for non-specific presentations of abdominal painMaryam Bahreini, Houman HosseinnejadA13 Introduction of a new imaging guideline for suspected renal colic in the emergency department: effect on CT Urogram utilisationGabriel Blecher, Robert Meek, Diana Egerton-WarburtonA14 Transabdominal ultrasound screening for pancreatic cancer in Croatian military veterans: a retrospective analysis from the first Croatian veteran's hospitalEdina Catic Cuti, Stanko Belina, Tihomir Vancina, Idriz KovacevicA15 The challenge of AAA: unusual case of obstructive jaundiceEdina Catic Cuti, Nadan RustemovicA16 Educational effectiveness of easy-made new simulator model for ultrasound-guided procedures in pediatric patients: vascular access and foreign body managementIkwan Chang, Jin Hee Lee, Young Ho Kwak, Do Kyun KimA17 Detection of uterine rupture by point-of-care ultrasound at emergency department: a case reportChi-Yung Cheng, Hsiu-Yung Pan, Chia-Te KungA18 Abdominal probe in the hands of interns as a relevant diagnostic tool in revealing the cause of heart failureEla Curcic, Ena Pritisanac, Ivo Planinc, Marijana Grgic Medic, Radovan RadonicA19 Needs assessment of the potential utility of point-of-care ultrasound within the Zanzibar health systemAbiola Fasina, Anthony J. Dean, Nova L. Panebianco, Patricia S. HenwoodA20 Ultrasonographic diagnosis of tracheal compressionOliviero Fochi, Moreno Favarato, Ezio BonanomiA21 The role of ultrasound in the detection of lung infiltrates in critically ill patients: a pilot studyMarijana Grgic Medic, Ivan Tomic, Radovan RadonicA22 The SAFER Lasso; a novel approach using point-of-care ultrasound to evaluate patients with abdominal complaints in the emergency departmentYoungrock Ha, Hongchuen TohA23 Awareness and use of clinician-performed ultrasound among clinical clerkship facultyElizabeth Harmon, Wilma Chan, Cameron Baston, Gail Morrison, Frances Shofer, Nova Panebianco, Anthony J. DeanA24 Clinical outcomes in the use of lung ultrasound for the diagnosis of pediatric pneumoniasAngela Hua, Sharon Kim, James TsungA25 Effectiveness of ultrasound in hypotensive patientsIsa Gunaydin, Zeynep Kekec, Mehmet Oguzhan AyA26 Moderate-to-severe left ventricular ejection fraction related to short-term mortality of patients with post-cardiac arrest syndrome after out-of-hospital cardiac arrestJinjoo Kim, Jinhyun Kim, Gyoosung Choi, Dowon ShimA27 Usefulness of abdominal ultrasound for acute pyelonephritis diagnosis after kidney transplantationJi-Han LeeA28 Lung ultrasound for assessing fluid tolerance in severe preeclampsiaJana Ambrozic, Katja Prokselj, Miha LucovnikA29 Optic nerve sheath ultrasound in severe preeclampsiaGabrijela Brzan Simenc, Jana Ambrozic, Miha LucovnikA30 Focused echocardiography monitoring in the postoperative period for non-cardiac patientsAsta Maciuliene, Almantas Maleckas, Algimantas Krisciukaitis, Vytautas Maciulis, Andrius MacasA31 POCUS-guided paediatric upper limb fracture reduction: algorithm, tricks, and tipsSharad MohiteA32 Point-of-care lung ultrasound: a good diagnostic tool for pneumonia in a septic patientZoltan Narancsik, Hugon MozinaA33 A case of undergraduate POCUS (r)evolutionSara Nikolic, Jan Hansel, Rok Petrovcic, Una Mrsic, Gregor ProsenA34 The Graz Summer School for ultrasound: from first contact to bedside application: three-and-a-half-day undergraduate ultrasound training: résumé after two years of continuous developmentSimon Orlob, Markus Lerchbaumer, Niklas Schönegger, Reinhard KaufmannA35 Usefulness of point-of-care ultrasound in the emergency room in a patient with acute abdominal painAlberto Oviedo-García, Margarita Algaba-Montes, Mayra Patricio-BordomásA36 Use of bedside ultrasound in a critically ill patient. A case reportAlberto Oviedo-García, Margarita Algaba-Montes, Mayra Patricio-BordomásA37 Diagnostic yield of clinical echocardiography for the emergency physicianAlberto Oviedo-García, Margarita Algaba-Montes, Mayra Patricio-BordomásA38 Focused cardiac ultrasound in early diagnosis of type A aortic dissection with atypical presentationChun-I Pan, Hsiu-Yung Pan, Chien-Hung WuA39 Detection of imperforated hymen by point-of-care ultrasoundHsiu-yung Pan, Chia-Te KungA40 Developing a point-of-care ultrasound curriculum for pediatric nurse practitioners practicing in the pediatric emergency departmentSarah Pasquale, Stephanie J. Doniger, Sharon Yellin, Gerardo ChiricoloA41 Use of transthoracic echocardiography in emergency setting: patient with mitral valve abscessMaja Potisek, Borut Drnovsek, Bostjan LeskovarA42 A young man with syncopeFatemeh Rasooli, Maryam BahreiniA43 Work-related repetitive use injuries in ultrasound fellowsKristine Robinson, Clara Kraft, Benjamin Moser, Stephen Davis, Shelley Layman, Yusef Sayeed, Joseph MinardiA44 Lung ultrasonography in the evaluation of pneumonia in childrenIrmina Sefic Pasic, Amra Dzananovic, Anes Pasic, Sandra Vegar ZubovicA45 Central venous catheter placement with the ultrasound aid: two years' experience of the Interventional unit, Division of Intensive Care Medicine, KBC ZagrebAna Godan Hauptman, Marijana Grgic Medic, Ivan Tomic, Ana Vujaklija Brajkovic, Jaksa Babel, Marina Peklic, Radovan RadonicA46 Duplicitas casui: two patients admitted due to acute liver failureVedran Radonic, Ivan Tomic, Luka Bielen, Marijana Grgic MedicA47 A pilot survey on an understanding of Bedside Point-of-Care Ultrasound (POCUS) among medical doctors in internal medicine: exposure, perceptions, interest, and barriers to trainingPeh Wee MingA48 Unusual case of defecation syncopeNur hafiza Yezid, Fatahul Laham MohammedA49 A case report of massive pulmonary embolism; a multidisciplinary approachZainal Abidin Huda, Wan Nasarudin Wan Ismail, W.Yus Haniff W.Isa, Hashairi Fauzi, Praveena Seeva, Mohd Zulfakar Mazlan.

8.
Coll Antropol ; 38(2): 665-9, 2014 Jun.
Article in English | MEDLINE | ID: mdl-25145004

ABSTRACT

Intravascular device infections could be serious complications with significant contributable morbidity and mortality. The aim of this prospective clinical study is to demonstrate the infection rate related to peripheral arterial catheters and their clinical significance in neurosurgical intensive care unit (ICU) patients. After removal, all arterial catheter tips were cultivated by semiquantitative method and clinical data were collected. During a period of two years, 186 arterial catheters were placed in 105 neurosurgical ICU patients. In 6 cases (3.2%) infection was presumably related to the arterial catheter. The rate of such probable catheter related infections was found to be 5/1000 catheter days. The isolated microorganisms were: Methicillin resistant Staphylococcus epidermidis (MRSE) in 4 cases, Corynebacterium species and Candida albicans each in one case respectively. Thirteen cases (7.0%) were interpreted as contamination and one as colonization. An association was found between the presence of infection from different sources and significant bacterial growth on the catheter. Patients with positive catheter culture had a significantly longer ICU stay, more cumulative catheter days, and a higher mortality rate than those with sterile catheters. We can conclude that the rate of probable peripheral arterial catheter related infection is low. A higher mortality rate in patients who experienced probable catheter related infection does not seem to be a consequence of the aforementioned infection. A more suitable explanation would be that patients with nosocomial infections and higher mortality risk have prolonged ICU stays. There is an increased chance of developing a catheter related infection in those patients who have more cumulative catheter days.


Subject(s)
Catheter-Related Infections/epidemiology , Cross Infection/epidemiology , Intensive Care Units , Adolescent , Adult , Aged , Aged, 80 and over , Child , Female , Humans , Male , Middle Aged , Young Adult
9.
Coll Antropol ; 38(1): 125-8, 2014 Mar.
Article in English | MEDLINE | ID: mdl-24851605

ABSTRACT

Acute pancreatitis is a disease with various degrees of clinical manifestations. Mild and moderate severe acute pancreatitis is an illness characterized with chemical inflammation which, in general, passes without major complications. Clinical picture of severe acute pancreatitis other side is commonly complicated with functional deterioration of other organs, and frequently has characteristics of multiple organ dysfunction or failure syndrome with or without bacterial super infection. We studied 82 patients admitted to the intensive care unit with severe acute pancreatitis, 14 died. The mortality was in statistically significant correlation with the severity of clinical condition at admission assessed by APACHE II score, and higher Ranson's and Glasgow criteria by admission. Adequate volume supplementation, on time, as well as percutaneous drainage of infected pancreas collection reduces a risk of pure outcome.


Subject(s)
APACHE , Bacterial Infections/complications , Multiple Organ Failure/complications , Pancreatitis/complications , Acute Disease , Adult , Aged , Bacterial Infections/mortality , Female , Humans , Male , Middle Aged , Multiple Organ Failure/mortality , Pancreatitis/mortality , Severity of Illness Index
11.
Croat Med J ; 52(5): 644-7, 2011 Oct 15.
Article in English | MEDLINE | ID: mdl-21990083

ABSTRACT

Adaptive rowing is rowing or sculling for rowers with a physical disability. It debuted at the Paralympic Games in 2008. In order to ensure an equitable playing field, rowers with similar levels of physical function and disability are classified into different sport classes for competition. Rowers with an inability to use a sliding seat and impaired trunk function resulting in an inability to perform trunk forward and backward lean via hip flexion/extension are assigned to the Arms and Shoulders (AS) class. AS rowers have to use a chest strap set immediately below the chest in order to localize any trunk movement in AS class. Conditions created by adaptations of rowing equipment and technique within the AS class create unique stresses on the upper thoracic region. The following case report demonstrates how etiology and management of a rib stress fracture in an AS rower differs in comparison to able-body rowers. Of significant importance were the limitations imposed on the rower's ability to maintain rowing-specific fitness, due to the nature of the rib stress fracture and requirement to decrease force transmission through the ribs for several weeks. The rower's gradual return to full training was further impacted by obligatory use of the chest strap, which directly applied pressure over the injured area. Protective orthosis for the chest was designed and applied in order to dissipate pressure of the chest strap over the thorax during rowing (most importantly at the catch position) both on the ergometer and in the boat.


Subject(s)
Fractures, Stress/etiology , Ribs/injuries , Ships , Shoulder/physiology , Sports/classification , Arm/physiology , Equipment Design/adverse effects , Fractures, Stress/physiopathology , Humans , Male , Protective Devices , Young Adult
12.
Coll Antropol ; 34(3): 1087-91, 2010 Sep.
Article in English | MEDLINE | ID: mdl-20977108

ABSTRACT

Thrombotic thrombocytopenic purpura (TTP) is a disorder characterized by disseminated thrombotic occlusions of the microcirculation. Identification of ADAMTS13 protease and its place in the pathophysiology of TTP led to better understanding of the disease and better survival for the diseased. Here we show a case report of a patient that had a normal ADAMTS13protease activity and an unusual clinical presentation and utilize that case to highlight how the absence of a severe ADAMTS13 protease deficiency does not preclude a diagnosis of TTP and how early initiation and continuation of plasma exchange therapy can lead to a positive outcome, even in a severely ill patient. Even though ADAMTS13 protease determination has no immediate influence on the decision whether or not to start the plasma exchange therapy, it has great impact on future management of the patient and should be determined whenever possible.


Subject(s)
ADAM Proteins/blood , Purpura, Thrombotic Thrombocytopenic/blood , ADAM Proteins/deficiency , ADAMTS13 Protein , Female , Humans , Middle Aged , Plasma Exchange , Purpura, Thrombotic Thrombocytopenic/diagnosis , Purpura, Thrombotic Thrombocytopenic/therapy
14.
Intern Med ; 48(9): 711-5, 2009.
Article in English | MEDLINE | ID: mdl-19420819

ABSTRACT

Chromic acid is a strong metal acid and acute poisoning is very rare, but very serious with severe skin injury, renal and liver failure. The majority of published cases were suicide attempts with lethal outcomes. We describe the case of a 55-year-old man who had accidentally taken a sip of 20% chromic acid (estimated chromium intake: 2.3 g). Renal and liver failure were not present at presentation, but appeared later in the course of disease. He was treated with hemodialysis, no chelating agents or other methods for enhancing elimination were used. Liver and renal function improved over the next 30 days and the patient was discharged after 45 days of hospitalization with no need for dialysis. In the follow-up period of eight months his renal function remained depressed, but stable.


Subject(s)
Acute Kidney Injury/chemically induced , Acute Kidney Injury/diagnosis , Chromates/poisoning , Liver Failure/chemically induced , Liver Failure/diagnosis , Acute Disease , Acute Kidney Injury/therapy , Follow-Up Studies , Humans , Liver Failure/therapy , Male , Middle Aged , Renal Dialysis/methods , Survival
15.
Blood ; 103(11): 4195-7, 2004 Jun 01.
Article in English | MEDLINE | ID: mdl-14982879

ABSTRACT

Thrombotic thrombocytopenic purpura (TTP) either occurs in a congenital form caused by ADAMTS13 gene mutations or it is acquired and most often due to ADAMTS13 inhibitory autoantibodies. In congenital TTP siblings are often affected, while acquired TTP occurs sporadically and familial clustering has not been described so far. We report identical twin sisters suffering from acquired TTP due to immunoglobulin G (IgG) autoantibodies inactivating ADAMTS13, suggesting an important role of hitherto unidentified genetic determinants of ADAMTS13 inhibitor formation. These cases also demonstrate that familial clustering is not sufficient for unambiguously diagnosing hereditary ADAMTS13 deficiency and congenital TTP.


Subject(s)
Metalloendopeptidases/genetics , Metalloendopeptidases/immunology , Purpura, Thrombotic Thrombocytopenic/genetics , Purpura, Thrombotic Thrombocytopenic/immunology , ADAM Proteins , ADAMTS13 Protein , Adult , Autoantibodies/blood , Female , Histocompatibility Testing , Humans , Metalloendopeptidases/deficiency , Twins, Monozygotic/genetics
16.
Acta Med Croatica ; 57(5): 337-46, 2003.
Article in Croatian | MEDLINE | ID: mdl-15011458

ABSTRACT

INTRODUCTION: The largest outbreak of hemorrhagic fever with renal syndrome (HFRS) to date occurred in Croatia in the year 2002. The epidemic started in winter, lasted throughout spring to summer months, ending not earlier than November. AIM: The aim of this study was to investigate and analyze the basic epidemiologic and clinical features of HFRS in Croatia by uniform and standardized prospective-retrospective analysis of all patients affected by the epidemic. PATIENTS AND METHODS: When the epidemic started, a patient questionnaire with questions on the basic demographic data, site of infection and other epidemiologic characteristics, clinical symptoms, disease severity and laboratory results was designed. Data on 401 patient with a clinical diagnosis of HFRS were collected. The etiologic diagnosis of the disease was confirmed by ELISA, and in some patients by indirect immunofluorescence test (IFT). The results were analyzed using a descriptive statistical method. RESULTS: HFRS was clinically diagnosed in 401 patients from all over Croatia. A total of 320 (79.8%) cases were reported to the Epidemiology Service of the National Institute of Public Health. The majority of patients (n = 128) were registered in June. Males were three times more affected than females. Apart from its long duration, this epidemic was characteristic for the involvement of general population, with only a small number of the affected from the potential risk groups (forestry workers 28, soldiers 14, farmers 18). The epidemic spread almost throughout inland Coratia. At least 44 patients were infected in the Plitvice Lakes area, 32 in Slunj, 27 on Sljeme, 24 in Velika, and at least 19 in the area of Kutjevo. The youngest patient was aged 4 and the oldest 80 years. The majority of patients were treated in Zagreb (University Hospital for Infectious Diseases--110, Zagreb University Hospital Center--3), followed by Karlovac (71 inpatients and 39 outpatients), Pozega (n = 79), and Rijeka (n = 37). Serologic analysis (ELISA method) detected Puumala virus in 161 and Dobrava virus in only 17 patients. The disease was confirmed by immunofluorescence method in 53 patients (mostly in Kariovac). During the 2002 outbreak, HFRS clinically manifested mostly in a milder form with general symptoms and transitory renal insufficiency, while hemorrhages were rarely recorded. According to our disease severity score, a mild form of the disease was recorded in 65%, moderately severe in 28%, severe in 5% and extremely severe form in 2% of the patients. One patient died. Two thirds of the patients were hospitalized during the febrile stage of the disease. All patients had fever, whereas headache and pain in the lumbar region were recorded in more than 90% of cases, polyuria in 75%, oliguira and vomiting in approximately 50%, respiratory symptoms in 35%, and hemorrhages (mostly on the skin and mucous membranes), vision disturbances, conjunctivitis and diarrhea in approximately 25% of patients. ESR was elevated in 64% and CRP in 93% of patients. Leukocytosis was recorded in 25% and thrombocytopenia in 70% of patients. Increased values of urea and creatinine and signs of liver damage were recorded in approximately 50% of the patients. CONCLUSION: The largest outbreak of HFRS occurred in Croatia in 2002, with more than 400 diseased throughout Croatia. This epidemic confirmed our previous assumption that the whole Croatia, apart from its narrow coastline area and islands, is a natural focus of HFRS with different causative types of hantaviruses. Efforts should be made to conduct a comprehensive ecologic and mammologic study on hantaviruses and their biologic characteristics in these areas.


Subject(s)
Disease Outbreaks , Hemorrhagic Fever with Renal Syndrome/epidemiology , Adult , Aged , Aged, 80 and over , Child , Child, Preschool , Croatia/epidemiology , Disease Outbreaks/statistics & numerical data , Female , Hemorrhagic Fever with Renal Syndrome/diagnosis , Humans , Male , Middle Aged
17.
Acta Med Croatica ; 57(5): 433-6, 2003.
Article in Croatian | MEDLINE | ID: mdl-15011473

ABSTRACT

UNLABELLED: Thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS) are classical diseases characterized by thrombocytopenia and microangiopathic hemolytic anemia. Microangiopathic hemolytic anemia is also a part of clinical picture in patients with hemorrhagic fever with renal syndrome (HFRS). Some overlap in other elements of clinical picture between TTP and HFRS is possible, which could pose difficulties in differential diagnosis. Early treatment of patients with TTP is essential and significantly improves the outcome, whereas the treatment of HFRS is mainly supportive. In the last ten years, we treated 13 patients with TTP and 17 patients with HFRS. Two patients with HFRS were initially treated as TTP because it was not possible to exclude TTP on the basis of clinical picture. Further clinical course and serologic tests excluded TTP and suggested HFRS. CONCLUSION: Sometimes it is difficult to distinguish HFRS from TTP because thrombocytopenia and microangiopathic hemolytic anemia are present in both diseases and overlaps in other parts of clinical picture are possible. The serious consequences of delay in the efficacious treatment of patients with TTP could also influence the physicians' decisions.


Subject(s)
Hemorrhagic Fever with Renal Syndrome/diagnosis , Purpura, Thrombotic Thrombocytopenic/diagnosis , Adult , Diagnosis, Differential , Female , Hemorrhagic Fever with Renal Syndrome/therapy , Humans , Male , Purpura, Thrombotic Thrombocytopenic/therapy
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