• Title/Summary/Keyword: Spherocytosis

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Pediatric Laparoscopic Splenectomy (소아의 복강경하 비장절제술)

  • Kim, Won-Woo;Kim, Eung-Kook;Song, Young-Tack
    • Advances in pediatric surgery
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    • v.5 no.1
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    • pp.53-57
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    • 1999
  • Pediatric laparoscopic splenectomy has been gradually accepted as the surgical management of a various splenic disorders, particularly in hematologic diseases. We report our experience with 16 patients who underwent this procedure because of hematologic disorders during the past 3 and a half years at the Department of Surgery, St. Mary's Hospital, the Catholic University Medical College. The mean age was 10 years(range 6-16 years) and the mean spleen weight was 210 gm(range 85-500 g). The indication for splenectomy were hereditary spherocytosis(6 cases), idiopathic thrombocytopenic purpura(8 cases), autoimmune hemolytic anemia(1 case), and idiopathic splenomegaly(1 case). All splenectomies were performed safely with mean estimated blood loss of 233 ml. Mean operative time and mean postoperative hospital stay were 157 min and 4.5 days, respectively. Postoperative pain, medication was needed in 3 cases, just one injection in immediate postoperative period. Diet was started on the second or third postoperative day. In conclusion, laparoscopic splenectomy in pediatric patients is a safe procedure, offering a small of abdominal scar, much less pain, a shorter hospital stay and car the lower postoperative morbidity.

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Effectiveness of Danazol as an adjunctive therapy in dogs with immune-mediated hemolytic anemia

  • Yo, Sechul;Park, Hyung-Jin;Song, Kun-Ho
    • Korean Journal of Veterinary Service
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    • v.45 no.3
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    • pp.165-169
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    • 2022
  • Immune-mediated hemolytic anemia (IMHA) is autoimmune disease which is anemia caused by own immune system destroying the red blood cells (RBC). It can be diagnosed with spherocytosis, positive auto-agglutination of RBCs and direct antiglobulin test (DAT, Coomb's test). The treatment for IMHA are blood transfusion, immunosuppressive agents including glucocorticoids and other supportive therapies. Danazol is synthetic androgen that has effect of interfering the autoimmune reaction to RBCs. It can be used as an adjunctive agent in addition to glucocorticoids. To investigate its effectiveness, the medical records of 10 IMHA-diagnosed dogs were evaluated. All subjects were treated with blood transfusion, prednisolone, mycophenolate mofetil, and intravenous human immunoglobulin G. Additionally, 6 subjects were administered with danazol and 4 subjects were not. The results of initial blood examination and responses to the treatment for IMHA were compared between the groups. There were significant differences in the number of blood transfusions; once in group with danazol, twice in group without danazol, duration of recovery to normal hematocrit; 7.67±3.08 days in group with danazol, 22.00±5.66 days in group without danazol, and hospitalization; 5.17±0.75 days in group with danazol, 12.75±2.22 days in group without danazol. Therefore, danazol has potential effective on treating IMHA for rapid improvement.

Laparoscopic Splenectomy in Children (소아에서의 복강경 비장 절제술)

  • Jung, Eun-Young;Kim, Dae-Yeon;Kim, Seong-Chul;Kim, In-Koo
    • Advances in pediatric surgery
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    • v.10 no.1
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    • pp.31-34
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    • 2004
  • The laparoscopic splenectomy (LS) became popular over the last 10 years. The advantage of LS over open splenectomy (OS) includes short hospital stay, improved cosmesis, less development of postoperative intestinal ileus, and less analgesics required. The purpose of this study is to evaluate the outcome of LS at Asan Medical Center from January 1999 to January 2003. The records of 14 consequent children who underwent splenectomy were reviewed retrospectively. Patients characteristics, morbidity, mortality, operative time, blood loss, and hospital stay were analyzed., Seven patients age 5 to 15 years underwent LS under the indications: idiopathic thrombocytic purpura (ITP, n=3), hereditary spherocytosis (n=3), and myelodysplastic syndrome (n=l). Seven patients, age 7 to 16 years, underwent OS during the same period for ITP (n=7). Median operative time was 120 mInutes (80 to 170 mins.) in OS, and 270 minutes (110 to 480 mins,) in LS (p<0.05). Median length of hospital stay was 6 days (3 to 8) in OS, and 4 days (3 to 6) in LS (p>0.05). Median splenic length was 12.0cm (9.2 to 18.0) in OS, 14.0 cm (10.0 to 19.5) (p>0.05). Accessory spleens were identified in 3 of 7 LS and 1 of 7 OS cases. In the LS group, there was no conversion to open surgery. Two patients in LS required blood transfusion postoperatively. LS in children can be performed as effectively and safely as OS.

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Clinical Characteristics of Human Parvovirus B19 Infection in Children

  • Jo, Kyo Jin;Lee, Yeoun Joo;Park, Kyung Mi;Yang, Eu Jeen;Yoo, Sukdong;Lim, Taek Jin;Park, Su Eun
    • Pediatric Infection and Vaccine
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    • v.27 no.2
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    • pp.111-116
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    • 2020
  • Purpose: Human parvovirus B19 infection is widespread and has a heterogeneous clinical spectrum, ranging from asymptomatic infection to potentially life-threatening complications. We investigated the various clinical features of human parvovirus B19 infection during an outbreak of the virus in our community. Methods: A retrospective chart review study was conducted at the Pusan National University Children's Hospital from December 2017 to April 2019. We investigated the clinical features of children with parvovirus B19 immunoglobulin M or parvovirus B19 DNA detected using polymerase chain reaction. Results: A total of 24 children were diagnosed with parvovirus B19 infection. Twelve (50%) had lace form rashes, and four (16.7%) had petechial rashes. Two (8.3%) were diagnosed with fever without a focus. Six (25%) developed aplastic crisis as a complication of infection, of whom three were previously diagnosed with hereditary spherocytosis and three with acute lymphoblastic leukemia. Conclusions: In addition to erythema infectiosum, the parvovirus B19 infection can present clinically with various types of rashes and fever without a focus. Furthermore, hematologic manifestations such as neutropenia and aplastic crisis can occur during infection.

A Clinical Study on Childhood Hemolytic Anemia According to Etiological Classification (소아 용혈성 빈혈 환자에서 원인에 따른 임상적 분석)

  • Kwon, Hae-Sik;Kang, Jung-Chul;Won, Sung-Chul;Oh, Seung-Hwan;Lyu, Chuhl-Joo
    • Clinical and Experimental Pediatrics
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    • v.46 no.9
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    • pp.883-888
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    • 2003
  • Purpose : The etiology of hemolytic anemia can be classified as either cellular or extracellular defects of red blood cells. The aim of this study was to investigate the clinical and laboratory findings of hemolytic anemia concerning its etiological classification. Methods : Clinical and laboratory findings of the patients with hemolytic anemia treated from January 1987 to May 2002 at Severance Hospital were analyzed retrospectively. They were divided into two groups based on the types of red cell defects(group I : erythrocytic defect, group II : extraerythrocytic defect). Results : Twenty one cases were included in group I, thirty four cases in group II, and three cases were unclassified. In group I, nineteen cases(90.5%) were diagnosed as hereditary spherocytosis and were proved to have red cell membrane disorders while two cases(9.5%) were shown to have red cell enzyme deficiencies. In group II, thirteen cases(38.2%) were noted as autoimmune hemolytic anemia, eleven cases(32.4%) as traumatic or microangiopathic hemolytic anemia, four cases(11.8%) as drug induced hemolytic anemia, two cases(5.9%) were related with systemic lupus erythematosus and one case(2.9%) with malignancy. Hemoglobin at the time of diagnosis(7.5 g/dL vs. 6.2 g/dL, P<0.05) and the incidence of splenomegaly(85.7% vs. 18.2%, P<0.05) were higher in group I though blood urea nitrogen(9.0/0.4 mg/dL vs. 27.8/1.6 mg/dL, P<0.05) was higher in group II. Conclusion : Comparing the clinical features of pediatric hemolytic anemia, we concluded as following : In cases associated with extraerythrocytic defect, blood tests revealed significant initial lower hematocrit with higher level of BUN and Cr while cases with erythrocytic defect, splenomegaly were more common noted.

A Clinical Study for Gall Bladder Stones in Children (소아 담석증에 대한 임상적 연구)

  • Eun, Ho Seon;Baek, Seoung Yon;Chung, Ki Sup
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • v.8 no.2
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    • pp.194-201
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    • 2005
  • Purpose: Gall bladder (GB) stones in children are rarely observed, and so reports of them have been quite limited in Korea. Therefore, we tried to assess the epidemiology and clinical features of children with GB stone diagnosed at Severance Hospital. Methods: A clinical study was performed on 18 patients below 15 years of age diagnosed with GB stone by abdominal ultrasonography or CT scan at Severance hospital from January 1999 to May 2005. Results: Concerning patients' profile, their sex ratio of male to female in total 18 cases was 2 : 1 with the mean age of $6{\pm}4.3years$. 15 patients (83.3%) were asymptomatic. Inducing factors were found in 14 children (77.8%) including ceftriaxone therapy in 6 cases (33.3%), infection in 4 cases (22.2%), spherocytosis in 3 cases (16.7%), Down syndrome and abdominal operation in 1 case (5.6%) respectively. Single stone was found in 13 cases (76.5%) and multiple in 4 cases (23.5%). 15 cases (88.2%) had the stones less than 5 mm in size, and 2 cases (11.8%) between 5 and 10 mm. 17 patients received conservative treatment, and one patient had cholecystectomy due to Meckel diverticulum. Conclusion: In this report, Pediatric GB stones are predominant in male children, without typical symptoms, having inducing factors, single and small. Recently the diagnosis of them has been increased because of the development of imaging study. But the reports for them were still rare in Korea. Therefore the study for them is more necessary to find it's clinical characteristics.

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A Retrospective Study of Invasive Bacterial Infections in Children with Asplenia (18세 이하 무비증 환자에서 발생한 침습성 세균 감염증에 대한 후향적 연구)

  • Choe, Yong-Joon;Seo, Euri;Lee, Jina
    • Pediatric Infection and Vaccine
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    • v.24 no.3
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    • pp.160-167
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    • 2017
  • Purpose: Because children with asplenia have an increased risk of fulminant infection associated with a high fatality, chemoprophylaxis, and vaccinations against encapsulated bacteria are recommended. However, there have been few reports of the burden of severe bacterial infection and the current status of chemoprophylaxis and immunization among children with asplenia in Korea. Methods: We conducted a retrospective study including children with asplenia who were treated at our institute between January 1997 and December 2016. Results: From a total of 213 children with asplenia, 114 (53.5%) had congenital asplenia and 58 (27.2%) had functional asplenia. The remaining 41 (19.3%) had acquired asplenia with the median age at splenectomy being 12.2 years (range, 5.0 to 16.9 years); the most common cause of splenectomy was hereditary spherocytosis (39.0%). The chemoprophylaxis rate was 16.4%. The immunization rates were 44.1% for pneumococcus, 53.0% for Haemophilus influenzae type B, and 10.7% for meningococcus. The incidence of invasive bacterial infection among children with asplenia was 0.28/100 person-year; a total of six episodes (2.8%) were observed in five patients with congenital asplenia and one patient with functional asplenia. The median age for these infections was 15 months (range, 4 to 68 months). Five of the six episodes were bacteremia, and the other was meningitis. The most common pathogen was Streptococcus pneumoniae (n=3), followed by H. influenzae (n=1). Three of the six patients (50.0%) died, all of whom had pneumococcal bacteremia. None of the six had chemoprophylaxis or proper vaccinations. Conclusions: Although there is an increased risk of a severe infection proper vaccinations and chemoprophylaxis are still lacking. Physicians should be encouraged to implement appropriate chemoprophylaxis and immunizations for patients with asplenia.