In this study, we report the first clinically identified case of severe fever with thrombocytopenia syndrome (SFTS) in a 73-year old man from Jeju Island, South Korea. Although his initial manifestation suggested tsutsugamushi disease with cutaneous lesion, later the patient presented with symptoms characteristic of SFTS. Despite intensive medical therapies upon the clinical diagnosis of SFTS, patient's condition rapidly deteriorated. SFTS is a fatal disease that requires early diagnosis and appropriate supportive treatment.
Diagnosis ; Early Diagnosis ; Fever* ; Humans ; Korea ; Scrub Typhus ; Thrombocytopenia*

Immune thrombocytopenia (ITP) is the most common cause of thrombocytopenia in children and can be defined as an autoimmune disorder of isolated thrombocytopenia without other causes of thrombocytopenia. This review will focus on the diagnostic approach of ITP, especially regarding the differential diagnosis. The practice of differential diagnosis has the goal of distinguishing primary ITP from secondary ITP and nonimmune thrombocytopenia requiring different treatments and showing different prognoses.
Child ; Diagnosis ; Diagnosis, Differential ; Humans ; Prognosis ; Purpura, Thrombocytopenic, Idiopathic* ; Thrombocytopenia

Fever ; Humans ; Plasma Cells ; pathology ; Thrombocytopenia ; diagnosis ; pathology

China ; Clinical Protocols ; Hospital Units ; Humans ; Physicians ; Thrombocytopenia ; diagnosis ; therapy

Neonatal thrombocytopenia, defined as platelet counts of less than 150,000/µL, is a frequent hematologic abnormality in neonatal period. Differential diagnosis of neonatal thrombocytopenia may be challenging to pediatric hematologists and neonatologists because neonatal thrombocytopenia is associated with diverse maternal or neonatal clinical conditions. An accurate diagnosis for neonatal thrombocytopenia will lead to appropriate evaluation and management. Platelet transfusion is the primary management of neonatal thrombocytopenia. Most thrombocytopenic newborns received platelet concentrates to prevent major hemorrhage or treat ongoing bleeding according to institutional policy. However, scientific evidences for benefit and consistent guideline for platelet transfusion in neonates are lacking, further investigation to establish the standard recommendation for platelet transfusion is needed. This article reviewed the diagnostic approach and current guideline for platelet transfusion management for neonatal thrombocytopenia.
Blood Platelets ; Diagnosis ; Diagnosis, Differential ; Hemorrhage ; Humans ; Infant ; Infant, Newborn ; Organizational Policy ; Platelet Count ; Platelet Transfusion ; Thrombocytopenia ; Thrombocytopenia, Neonatal Alloimmune

A clinical and laboratory study was conducted on 58 children who had been admitted to out pediatric department from February to September 1979, under the clinical diagnosis of septicemia. Following results were obtained: 1. Boys were affected more frequently than girls(1.3:1), and highest incidence was noticed in newborn period, comprising 50% of the total cases. 2. The common clinical manifestations, in order of frequency, were fever, lethargy, jandice, poor sucking and abdominal distension, and hyperbilirubionemia was the most common assocaited disease, followed by anemia and leukemia. 3. Clinical course revealed death in 6 patients(10.3%), discharge against advice in 7cases(21.1%) and complete recovery in the remaining 45 cases(77.6%). 4. Peripheral blood count showed leukocytosie in 40%, leukopenia in 12.1% and thrombocytopenia was seen in 45% of cases. 5. Gram staining of buffy coat smear showed bacteria in 34.5% and especially high ratio was noticed in newborn infants(48.3%). 6. Positive blood culture was seen in 39.7% with highest ratio of 45% in newborn infants. In positive blood culture group, toxic granules were noted in 32.8%, shift to left in 25.9%, hrombocytopenia in 18.9% and positive buffy coat smear in 15.5%. In summary, thrombocytopenia, toxic granules, shift to left were quite helpful in early diagnosis of sepsis. In addition to diagnostic value, examination of buffy coat smear could aid physician to select appropriate antibiotic regimen especially in sepsis of newborn period.
Anemia ; Bacteria ; Child* ; Diagnosis ; Early Diagnosis ; Fever ; Humans ; Incidence ; Infant, Newborn ; Lethargy ; Leukemia ; Leukopenia ; Sepsis* ; Thrombocytopenia

One 22-month-old boy who was admitted for a fever lasting 6 days as well as a cough and wheezing lasting 2 days was reported. He was diagnosed with influenza A (H1N1, severe type), severe pneumonia, acute respiratory distress syndrome (ARDS), Evans syndrome and multiple organ failure. This is the first case of novel influenza A (H1N1) and Evans syndrome. The pathogenesis is still unknown.
Anemia, Hemolytic, Autoimmune ; diagnosis ; Humans ; Infant ; Influenza A Virus, H1N1 Subtype ; pathogenicity ; Influenza, Human ; diagnosis ; virology ; Male ; Thrombocytopenia ; diagnosis

Severe fever with thrombocytopenia syndrome (SFTS) is an emerging infectious disease in South Korea with clinical features similar to those of scrub typhus. Infected patients with these two diseases usually visited the emergency department (ED) complaining of fever that was not responsive to treatment in a local clinic. Aggressive management of SFTS is required in order to prevent rapid progression and human-to-human transmission, thus rapid and accurate differential diagnosis of the two diseases in the ED is important. We reported three laboratory confirmed cases of SFTS during 2013-2014 in a single center, with fever, skin lesions, and history of outdoor activities in order to help in differential diagnosis between SFTS and scrub typhus in the ED.
Communicable Diseases, Emerging ; Diagnosis, Differential ; Emergency Service, Hospital ; Fever* ; Humans ; Korea ; Scrub Typhus* ; Skin ; Thrombocytopenia*

Giant platelet syndrome is a group of unique disorders characterized by the presence of abnormally large platelets, and usually accompanied by thrombocytopenia. Most cases of giant platelets are encountered in idiopathic thrombocytopenic purpura(ITP). In contrast, inherited giant platelet disorders, a group of heterogeneous diseases, are rare. Bernard-Soulier syndrome and its variants, and MYH9 related diseases have been defined at the molecular level. Abnormalities in transcription factors are implicated in a couple of macrothrombocytopenia syndromes. However, the molecular defects are unknown in gray platelet syndrome. It is important to make a proper diagnosis of congenital macrothrombocytopenia to avoid unnecessary medications and potentially dangerous treatment for presumed ITP.
Bernard-Soulier Syndrome* ; Blood Platelets ; Diagnosis ; Gray Platelet Syndrome ; Thrombocytopenia ; Transcription Factors

Hepatoblastoma is a rare pediatric malignancy which frequently presents at an advanced unresectable stage, but improved resectability and survival is being reported with the help of neoadjuvant chemotherapy. Twenty biopsy proven hepatoblastoma patients were diagnosed and managed during the period between January, 1987 and June, 1995. Median age at diagnosis was 13 months (2mo~7yr 10mo) with M : F = 13 : 7. The histologic profile included 13 epithelial (5 fetal, 4 mixed, 1 embryonal, 3 undetermined), and 5 mixed mesenchymal and epithelial types. Chemotherapy effectively reduced the tumor volume with statistical significance (p=0.008) and was able to convert 7 out of 9 initially unresectable cases (78%) to resectable ones. Fourteen operations were done, 12 radical and 2 palliative with or without adjuvant chemotherapy. The whole population was followed with a median duration of 33 months and the median survival in the whole group was 26 months. The curative resection group displayed a 5 year survival rate of 61.1% and none in the non-curative group survived for more than 13 months (p=0.0001). In univariate analysis for prognostic factors, large tumor size at diagnosis and absence of thrombocytopenia was associated with poor survival, but these differences were not statistically significant. Pure fetal histology was not associated with better prognosis and this may be due to a different histologic classification. In this new era of neoadjuvant chemotherapy, the optimal management strategy for hepatoblastoma is still debated with radical surgical resection at earliest possible time being the final goal. For now, individualized approach appears to be the choice.
Biopsy ; Chemotherapy, Adjuvant ; Classification ; Diagnosis ; Drug Therapy ; Hepatoblastoma* ; Humans ; Prognosis* ; Survival Rate ; Thrombocytopenia ; Tumor Burden