Te-Kau Chang

Pediatric liver tumors: initial presentation, image finding and outcome.

Background: Few reports have been carried out on the characteristics of pediatric liver tumors.

Stromal‐derived factor‐1 gene variations in pediatric patients with primary immune thrombocytopenia

Primary immune thrombocytopenia (ITP) of childhood is an autoimmune disease characterized by abnormally increased destruction of platelets and decreased megakaryopoiesis. Stromal‐derived factor‐1 (SDF‐1) plays a role in megakaryopoiesis and may be involved in the pathogenesis of ITP. Five single nucleotide polymorphisms (SNPs) of the SDF‐1 gene, including rs1801157, rs2839693, rs2297630, rs1065297, and rs266085, were assessed in 100 children with ITP and 126 healthy controls. The genotypes were analyzed by tetra ARMS polymerase chain reaction and confirmed by direct sequencing. Compared with controls, the rs2839693 A/A and rs266085 C/T genotypes were decreased in ITP patients (P = 0.004 and 0.007, respectively). The odds ratios of the latter genotypes were 0.48, 95% CI 0.28–0.82. Further analysis of the relationship between SDF‐1 polymorphisms and clinical features showed that rs2297630 A/G was associated with protection from chronicity (P = 0.002; OR, 0.07; 95% CI, 0.01–0.61) and steroid dependence (P = 0.007; OR, 0.10; 95% CI, 0.01–0.84) in ITP patients. However, rs266085 genotype C/C was associated with risk of steroid dependence (P = 0.012, OR 3.87, 95% CI 1.27–11.77). The findings of this study suggest that SDF‐1 gene variations may be associated with the occurrence and prognosis of childhood ITP.

Treatment With Propranolol for Infantile Hemangioma in 13 Taiwanese Newborns and Young Infants

BACKGROUND Hemangioma in infants has a benign self-limited course, but the 10% of cases with complications need further treatment. Successful treatment with propranolol in western countries has been reported over the past few years. We evaluated the efficacy of propranolol for treating infantile hemangioma in Taiwanese newborns and young infants. METHODS Patients below 1 year of age treated with propanolol between November 2009 and March 2011 were enrolled. Demographic data, clinical features, imaging findings, treatment regimens of propranolol, and outcome were investigated. RESULTS Thirteen patients were treated with propranolol at a dose of 2-3 mg/kg/day. Seven (53.8%) patients had solitary hemangioma and six had multiple ones. The indications for treatment were risk of local event in nine patients, functional risk in four, local complication in one, and life-threatening complication in one. The median age for starting propranolol was 4 months (range: 1-11 months). Responses to propranolol, such as decolorization, regression in tumor size, or improvement of hemangioma-associated complications were observed in all patients within 1-2 weeks after treatment. Propranolol-associated adverse effects occurred in two patients. One infant had occasional tachypnea, and the other had occasional pale-looking appearance. The symptoms resolved after dosage tapering. CONCLUSION Propranolol may be a promising therapeutic modality for infantile hemangioma. Therapeutic strategies are needed to evaluate the optimal treatment protocol and long-term adverse effects.

Acute immune thrombocytopenic purpura in infants: associated factors, clinical features, treatment and long-term outcome.

Abstract:  The natural course of acute immune thrombocytopenic purpura (ITP) in infants is poorly described in the literature. A retrospective study of 17 consecutive patients <1 yr of age admitted and treated for acute ITP between 1996 and 2005 was conducted. We investigated their demographics, vaccination history, clinical features, laboratory examinations, response to treatment and long‐term outcome. There were 11 male and six female infants. Their ages ranged from 24 d to 12 months with a median of 3 months. All infants presented with petechiae and/or ecchymoses. Fourteen cases had platelet counts below 20 × 109/L at the time of admission. They all had good response to a single course of treatment (14/17) or multiple courses of treatment (3/17). None had progressed into chronic ITP. Seven infants had a causal relationship with immunization, five associated with hepatitis B, one diphtheria–pertussis–tetanus, one diphtheria–tetanus–acellular pertussis‐inactivated poliovirus vaccine‐conjugated Haemophilus influenza vaccines. These seven infants responded to treatment within 3–9 d after therapy with intravenous immunoglobulin, high‐dose methylprednisolone or oral steroids. Re‐boosters with vaccines revealed no recurrence of the disease in all of these seven patients. The study suggests that further immunization is not contraindicated in infants experiencing acute ITP associated with vaccines.

Dislodgment of Port-A-Cath Catheters in Children

BACKGROUND Port-a-cath catheters are frequently used in children with malignancies. Their dislodgment is rare, but carries potentially serious risks. This study analyzed our 11-year experience of this important issue. METHODS Between June 1997 and January 2008, 290 ports of different brands were implanted in children by pediatric surgeons. Among the patients, 12 children with catheter dislodgement were retrospectively studied. Their ages ranged from 2-16 years, with a median of 6.4 years. Their body weights ranged from 12-39 kg, with a median of 20 kg. Ten patients presented with a port-a-cath dysfunction, while the other two patients were identified incidentally during surgery for removal of their ports. RESULTS The downstream ends of dislodged catheters were located in the right ventricle (five patients), right atrium (four), main pulmonary artery (one), left pulmonary artery (one) and right pulmonary artery (one). Eleven catheters were broken, and one catheter was disconnected from the port. Most (10/11) catheters were broken at the site of anastomosis to the port. All dislodged catheters were successfully retrieved without complications by transcatheter retrieval using a gooseneck snare. CONCLUSION The dislodgment rate of port-a-cath catheters in children in our series was 4.1%. Most (83%) catheters were broken at the site of anastomosis to the port. All dislodged catheters could be successfully retrieved by transcatheter retrieval using a gooseneck snare.

Congestive heart failure as presentation of acute lymphoblastic leukaemia with eosinophilia

A previously healthy 17-year-old girl presented with the sudden onset of chest tightness accompanied by dyspnoea on exertion. Physical examination showed splinter haemorrhages and a grade III/VI systolic murmur at the left sternal border with no radiation. Laboratory data showed a white cell count of 49AE8 · 10/l with 63% eosinophils (absolute eosinophil count of 31AE4 · 10/l). The eosinophils showed no vacuolation or degranulation. Biochemical studies showed normal levels of creatine kinase and creatine kinase MB, but an elevated troponin-I of 2AE19 (normal <0AE034 ng/ml). Transthoracic echocardiography in the parasternal long-axis view disclosed an intracardiac thrombus (arrows), seen attached to the mitral valve at the left ventricular posterior wall, associated with mild mitral regurgitation (left panel: AO, aorta; LA, left atrium; LV, left ventricle, RV, right ventricle). The multidetector-row computed tomography image disclosed diffuse mural thrombus that occurred more in association with the posterior wall (arrows, right panel). A bone marrow biopsy demonstrated precursor B lymphoblastic leukaemia with 40% lymphoblasts and an increase of eosinophils and their precursors. Cytogenetic analysis of the bone marrow aspirate showed t(3;6)(p22;p24). Further studies relevant to a hypereosinophilic disorder disclosed normal levels of interleukin 5, no FIP1L1-PDGFRA fusion gene and negative stool examination for parasites and ova. The patient received chemotherapy according to a high-risk protocol for acute lymphoblastic leukaemia, which resulted in bone marrow remission and a return of the peripheral eosinophil count to normal by 8 weeks. Subsequent echocardiography showed resolution of the left ventricular endocardial thrombosis.

Co-morbidity of Kawasaki Disease

Despite more than four decades of investigation, the etiology of Kawasaki disease remains obscure, and none of the proposed etiologic theories for the disease have achieved independent confirmation. Clinical and epidemiologic features support an infectious cause, but the etiology remains unclear. The authors present a case of Kawasaki disease associated with Epstein-Barr virus and Mycoplasma pneumoniae infection in a 3.5-y-old boy. He received two doses of intravenous immunoglobulin due to prolonged course of Kawasaki disease but later had complicated autoimmune haemolytic anaemia. His prolonged fever subsided after azithromycin administration. Epstein-Barr virus infection was confirmed by molecular microbiological pathology of cervical lymph node and serological tests. The serological tests for Mycoplasma pneumoniae also revealed a positive result. Thus, it is concluded that Mycoplasma pneumoniae and Epstein-Barr virus infections may occur simultaneously in a child with Kawasaki disease. In addition, autoimmune hemolytic anaemia may be noted in Kawasaki disease patients after high-dose IVIG administration. To the authors’ knowledge, this is the first report of Kawasaki disease with Epstein-Barr virus and Mycoplasma pneumoniae in the English-language literature.

C for colored urine: Acute hemolysis induced by high-dose ascorbic acid

Severe enterovirus-71 infections are unusual but potentially result in high morbidity and mortality. Cardiovascular and neurologic damages in life-threatening enterovirus-71 infections might be caused by several factors, including brainstem encephalitis, as well as infl ammatory process in the systemic and central nervous systems. 2 Although it is controversial as to whether high-dose ascorbic acid has anti-viral effects, 3 a few clinical practitioners in Taiwan are using it to treat children with enterovirus infection and the accompanying post-infectious systemic infl ammation. We report a 9-month-old male infant, weighing 12 kg, who was transferred to our hospital for suspicion of severe enterovirus infection. He had no signifi cant medical history except for glucose-6-phosphate dehydrogenase (G6PD) defi ciency. Three days before admission to our hospital, the patient presented with fever and multiple oral ulcers, and was admitted to a regional hospital. Tachycardia and decreased oxygen saturation detected by pulse oximeter were found in the morning before transferring to our hospital. Upon physical examinations, the patient voided some very deeply colored urine. Complete blood count revealed that his hemoglobin dropped to 5.4 g/dl, which was 11.1 g/dl two days prior. Both the direct and indirect Coombs tests were negative. When we reviewed his medical records, we found that he had been treated with a very high dose of ascorbic acid (2000 mg Q4H intravenously) for 2 days after being admitted to the previous hospital. After complete studies, we concluded that the patient ’ s clinical presentations and the normocytic anemia were caused by G6PD defi ciency-related acute hemolytic anemia (WHO class II for classifi cation of G6PD variants) rather than severe enterovirus infection or alternative causes. He was discharged after supportive treatments. G6PD defi ciency is the most common human enzyme defect. Certain drugs, foods, and infections can cause acute hemolysis in patients with G6PD defi ciency. This genetic disorder predisposes red blood cells to oxidative stress and hence, depending on the balance of oxidant species and antioxidants, may lead to red cell hemolysis. Vitamin C is a water-soluble antioxidant which has been found to suppress erythrocyte hemolysis induced by water-soluble radical initiator, and is thought to be one of the defense systems against reactive oxygen species. A few case studies on hemolytic anemia and complications occurring in patients with G6PD defi ciency after using high-dose ascorbic acid have been reported in the literature despite the mechanism remained unknown. 4,5 The doses that caused hemolysis described in previous case reports ranged from 80 to 160 gm of intravenous ascorbic acid in adults and 3 – 4 gm in children with G6PD defi ciency. Because tachycardia, poor activity and poor peripheral circulation are presentations common to both severe enterovirus infection and acute hemolytic anemia, the patient ’ s urine color may be helpful in the diagnosis. Although ascorbic acid is a useful antioxidant, caution should be exercised when using ascorbic acid to treat young children with G6PD defi ciency.

Intraabdominal mass as presentation of colonic mucormycosis in a child with acute lymphoblastic leukemia.

Gastrointestinal mucormycosis with clinical presentation as an abdominal mass has been rarely reported in the current literature. In this obseravation, We report an unusual case of a child with acute lymphoblastic leukemia and neutropenic fever. During hospitalization, increasing abdominal pain and distension occurred, and initialy treatments with broad-spectrum antibiotics were administered. However, the symptoms/signs progressed with the development of peritonitis and a palpated mass over the right lower quarter of the abdomen. Imaging studies showed ascending colon perforation with abscess formation. Right hemicolectomy was performed and colonic mucormycosis was confirmed on histologic examination and ascitic fluid culture. Immediately, intravenous liposomal amphotericin B was administered but the clinical condition rapidly deteriorated and the patient expired despite treatment.

A Magic Port-A-Cath

A 7-year-old boy with Burkitts lymphoma was referred by pediatric oncologists for severe chest pain when flushing a port-a-cath. Chest x-ray showed a dislodged port-a-cath and cardiomegaly ([Fig. 1][1]A) compared with the last film taken 6 months earlier ([Fig. 1][1]B). Echocardiography revealed