Pediatrics

A Maternal Participation Program in the context of Thai culture was developed based on the Neonatal Integrative Developmental Care model. A multiple case study design was used to explore the feasibility and acceptability and potential outcomes of implementing the program in a Thai NICU. The potential outcomes were growth and neurobehavioral development of very and moderately preterm infants. This program consisted of education and monitoring strategies including 4 teaching and practice sessions of 6 care practices, which included optimizing nutrition, positioning and handling, safeguarding sleep, promoting a healing environment, minimizing stress, and protecting the skin. The maternal participation program with education strategies was implemented one week after the infant’s admission to an NICU. Strategies to monitor the maternal participation level and to boost maternal participation were used. The maternal participation program was beneficial for 3 Thai mothers. They gave positiv...

Thalassemia disease is a common inherited hemolytic anemia frequently found in several parts of the world, especially in the Mediterranean and some Asian countries. Besides the complications of secondary hemochromatosis from regular red blood cell (RBC) transfusion and increased gastrointestinal absorption of iron, thromboembolism (TE) is one of the common long-term complications of beta-thalassemia disease, particularly in patients with non-transfusion-dependent thalassemia (NTDT), which is commonly seen after the second decade of life. The risk factors of TE in beta-thalassemia disease including exposure of phosphatidylserine of abnormal RBCs, increase of platelet activation and aggregation, elevation of endothelial microparticles and increased endothelial activation, decreased nitric oxide (NO) secondary to hemolysis, rise of platelet count and nucleated RBCs after splenectomy, organ dysfunction caused by hemochromatosis, and thrombophilia such as natural anticoagulant deficiencies leading to hypercoagulable state. The understanding of the pathophysiology would result in effective prevention of this complication of beta-thalassemia disease.

Osteosarcoma is the most common primary bone tumor in childhood and adolescence. Carboplatin, a platinum-derived agent, is used as neoadjuvant chemotherapy for pediatric osteosarcoma because of its anti-tumor activity and had low toxicity as compared to cisplatin. To determine demographic data, prognostic factors and outcome of childhood osteosarcoma treated with a carboplatin-based chemotherapeutic protocol at Chiang Mai University. A retrospective analysis was conducted on 34 osteosarcoma patients aged less than 18 years and treated between 2003 and 2011. Overall limb-salvage and amputation rates were 23.5% and 70.6%, respectively. With the mean follow-up time of 29.5 months (1.5-108.9), the Kaplan-Meier analysis for 3-year disease-free survival (DFS) and 3-year overall survival (OS) were 20.2±7.7% and 47.1±9.5% respectively. Patients who had initial pulmonary metastasis were at significantly greater risk for developing recurrence (p=0.02, OR=7; 1.2-40.1) and had a tendency to hav...

We report on 2 patients who developed widespread cerebral vasospasm and arterial ischemic strokes (AIS) after application of intrathecal (IT) cytarabine. In a 3-year-old child with acute lymphoblastic leukemia (ALL), left leg weakness, hyperreflexia, and clonus were noted 4 days after her first dose of IT cytarabine during the induction phase of her chemotherapy. Cerebral MRI revealed multiple acute cerebral ischemic infarcts and widespread cerebral vasospasm. A 5-year-old girl complained of right arm and leg pain and began limping 11 days after IT cytarabine. Symptoms progressed to right dense hemiplegia, left gaze deviation, headache, and speech arrest. MRI revealed 2 large cortical areas of diffusion restriction in the right frontal and left parietal lobes. Cerebral magnetic resonance angiography (MRA) showed irregular narrowing affecting much of the intracranial arterial circulation. Although the first child fully recovered from her neurologic symptoms, the second patient had pe...

A term male newborn born to a mother who had hereditary spherocytosis presented with neonatal jaundice at 20 hours of life. Complete blood count showed hemoglobin 17.1 g/dL, MCV 104.2 fL, MCH 32.9 pg, and MCHC 31.6 g/dL. The patient had indirect hyperbilirubinemia requiring phototherapy. The maximum total bilirubin level was 12.15 mg/dL at 20 hours of life. Peripheral blood smear revealed spherocytes, crenated red cells, and polychromasia. A flow cytometric test with eosin-5-maleimide- (EMA-) labeled RBC was performed in the patient and parents. The fluorescence histograms of EMA-labeled RBC from the patient and mother were shifted to the left, and the fluorescence ratio when compared with normal was 0.69 and 0.84, respectively. The flow cytometric test with EMA is useful in supporting the diagnosis of hereditary spherocytosis during newborn period.

Background: Febrile neutropenia (FN) is the most common complication in pediatric oncology patients. Appropriate empirical antibiotics treatment is essential for treatment outcome. Methods: This study was a randomized prospective controlled study to demonstrate the efficacy of piperacillin/tazobactam (PIP/TZO) monotherapy compared with ceftazidime/amikacin in children with FN. Pediatric oncology patients at Chiang Mai University Hospital, diagnosed with FN, were randomized to receive either PIP/TZO 320 mg/kg/day divided every 8 hours or ceftazidime 100 mg/kg/ day divided every 8 hours plus amikacin 15 mg/kg/day once daily. Treatment responses were compared between the two groups. Results: One-hundred and eighteen febrile neutropenic episodes in 70 patients (42 males and 28 females) were enrolled. The median age was 7 (3-10) years. The early response and complete response to initial treatment were achieved in 48/59 (81.4%) episodes and 41/59 (69.5%) episodes in PIP/TZO group compared with 40/59 (67.8%) episodes and 33/59 (55.9%) episodes in ceftazidime/amikacin group (p-value 0.091 and 0.128, respectively). Treatment modification in PIP/TZO group was required in 18/59 (30.5%) compared with 26/59 (44.1%) patients in ceftazidime/amikacin group (p-value 0.128). Similarly, the duration of fever, duration of neutropenia and duration of antibiotics treatment were not significantly different between two groups. No serious adverse events were observed. Conclusion: The treatment responses of PIP/TZO monotherapy and ceftazidime/amikacin therapy were not significantly different. Both therapies were effective for FN in pediatric oncology patients.

Orbital involvement is one of the extramedullary manifestations in acute leukemia. It is common in acute myeloid leukemia, but rare in acute lymphoblastic leukemia (ALL). We described a 3-year-old girl who presented with progressive proptosis of the right eye and was later diagnosed with precursor B-cell ALL. Initial blood count showed Hb 6.9 g/dL, WBC 42,000/mm3, lymphoblast 50%, and platelet count 185,000/mm3. Bone marrow aspiration revealed 90% lymphoblasts with positivity for CD10, CD19, CD20, CD22, and HLA-DR markers. Computed tomography (CT) scan of the brain and orbit revealed a homogeneous enhancing mass involving the right orbit with intracranial extension. The cytogenetic study showed 46,XX chromosomes. After 4 weeks of induction chemotherapy for very high-risk ALL, although the bone marrow was in remission, the proptosis was partially resolved. CT scan confirmed a decrease in size of the right orbital mass and degree of intracranial extension. Unfortunately, the patient a...

Background Clinical outcomes of patients with osteosarcoma remain unsatisfactory, with little improvement in a 5-year overall survival over the past three decades. There is a substantial need for further research and development to identify and develop more efficacious agents/regimens in order to improve clinical outcomes of patients for whom the prognosis is unfavorable. Recently, mycophenolate mofetil, a prodrug of mycophenolic acid, has been found to have anticancer activity against osteosarcoma in both in vitro and animal experiments, so that further investigation in humans is warranted. Methods A total of 27 patients with high-grade locally advanced or metastatic osteosarcoma will be enrolled into this phase II, multi-center, open-label, single-arm, two-stage clinical trial. The main objectives of this study are to determine the efficacy and safety of mycophenolate mofetil in the patients. The primary endpoint is progression-free survival at 16 weeks; the secondary endpoints in...

Objectives: To study the clinical manifestations and outcomes of treatment in thalassemia patients with dengue infection. Methods: A total of 95 acute febrile illness episodes among 88 thalassemia patients (male, 48; female, 40) from three university hospitals between 1997 and 2013, were retrospectively studied. Their median age was 11.6 years (range 2.0 to 19.9). The types of thalassemia included β-thal major (n = 8), β-thal/HbE (n = 32), AE Bart’s disease (n = 6), Hb H disease (n = 38), β-thal/HbS (n = 1), unspecified thalassemia disease (n = 1) and homozygous Hb Constant Spring (n = 2). Twenty-four patients were transfusion-dependent while the remaining patients were non-transfusion-dependent. Splenectomy was performed in 9 patients. Results: Eighty-two episodes were diagnosed with dengue infection (DF 22, DHF 60: grade I 23, II 20, III 13 and IV 4 cases) while 13 episodes were diagnosed with other self-limiting viral infections. The majority of patients presented anemia due to a...

Neonatal alloimmune thrombocytopenia (NAIT) is a result of platelet destruction by maternal antibodies directed against paternally-inherited antigens on neonatal platelets. NAIT is mostly caused by antibodies to human platelet antigens (HPA). This is a report of a term newborn with NAIT associated with an antibody to the human leukocyte antigens (HLA). The newborn presented with cutaneous bleeding at 5 days of age. The lowest platelet count was 36.8x109/L. An antibody to HLA-A11 was present in maternal serum while HPA antibodies were not detected. The HLA-A11 antigen was identified in the patient, sister, and father, but not the mother. The newborn received intravenous immunoglobulin and had a favorable outcome.

MYH9 disorder is characterized by macrothrombocytopenia with or without granulocyte DÖhle body-like inclusion bodies. Diagnosis is made by immunofluorescence analysis and genetic study of the MYH9 gene. Our collaborative study between Thailand and Japan began with 67 Thai patients with macrothrombocytopenia. Of these, 11 patients(16.4%), aged 4 months-22 years with platelet counts ranging from 2,000-99,000/uL were diagnosed with MYH9 disorder. MYH9 gene mutations occurred in exons 1,16,30,38,40. One novel mutation was identified (c.4338T>C, p.F1446A). The results indicate that patients with macrothrombocytopenia should be tested for MYH9 disorder in order to avoid misdiagnosis to the other diseases, such as chronic immune thrombocytopenia.

Abstract: Neonatal alloimmune thrombocytopenia (NAIT) is a result of platelet destruction by maternal antibodies directed against paternally-inherited antigens on neonatal platelets. NAIT is mostly caused by antibodies to human platelet antigens (HPA). This is a report of a term newborn with NAIT associated with an antibody to the human leukocyte antigens (HLA). The newborn presented with cutaneous bleeding at 5 days of age. The lowest platelet count was 36.8x109/L. An antibody to HLA-A11 was present in maternal serum while HPA antibodies were not detected. The HLA-A11 antigen was identified in the patient, sister, and father, but not the mother. The newborn received intravenous immunoglobulin and had a favorable outcome.

Kasabach-Merritt phenomenon (KMP) is relatively rare in childhood and adolescents with high mortality rate because of its hemorrhagic complications and unresponsiveness to treatments such as corticosteroids, vincristine, intravascular embolization, and/or surgery. Propranolol, aβ-adrenergic receptor blocker, has a promising efficacy against vascular tumors such as infantile hemangiomas. But limited and variable data has been reported regarding the role of propranolol in treatment of KMP. We herein reported the successful treatment of mild pediatric KMP with propranolol monotherapy in a case of a five-week-old child with kaposiform hemangioendothelioma with successful treatment of both clinical and hematologic responses. After eight months of follow-up, patient still had stable cutaneous lesion while receiving propranolol monotherapy. Regular hematologic monitoring was done in order to detect any late relapse of the disease. Six months after discontinuation of propranolol, patient ha...

Venomous snakes with hematotoxin-Russell's viper (Daboia spp), Malayan pit viper (Calloselasma rhodostoma), and green pit viper (Cryptelytrops albolabris and C macrops, previously named Trimeresurus spp) are commonly found in Thailand. Coagulation factor activation, thrombocytopenia, hyperfibrinolysis, and disseminated intravascular coagulation are the main mechanisms of hemorrhaging from these snake bites. The neurological involvement and hepatocellular injury after Russell's viper bites were reported in Sri Lanka, but there is no report from Southeast Asia. This case was a 12-year-old hill tribe boy who had ptosis and exotropia of the left eye, respiratory distress, and prolonged venous clotting time, prothrombin time, and activated partial thromboplastin time; low fibrinogen and platelet count; and transaminitis after being bitten by a darkish-colored snake. He did not respond to antivenom for cobra, Malayan pit viper, or Russell's viper. However, his neurological abn...

Hemophilia is an inherited bleeding disorder caused by deficiency of a specific coagulation factor. Factor VIII deficiency is responsible for hemophilia A while factor IX deficiency is responsible for hemophilia B. As per the 2020 annual global survey by the World Federation of Hemophilia, only 1828 Thai hemophiliacs have been registered to the national healthcare system. The reason for the low number is the underdiagnosis which is a major concern in the real-world practice among Asian countries. In Thailand, most hemophiliacs are diagnosed by general practitioners, pediatricians or internists at rural hospitals and are referred to hemophilia specialists at the Hemophilia Treatment Centers (HTCs). Despite the challenges pertaining to infrastructure and cost of treatment, Thailand has progressed substantially in providing the required hemophilia care, as evidenced by an evolution in acquiring and sharing knowledge as well as collaborative efforts among multiple stakeholders over the ...

Abdominal compartment syndrome (ACS) is a life-threatening condition which can occur in patients with hemophilia although they have trivial traumas. Hemostatic control for bleeding episodes in hemophilia patients with inhibitors is difficult particularly when the availability of bypassing agents, recombinant activated factor VII (rFVIIa) and activated prothrombin complex concentrate (APCC), is constrained. Plasma exchange with continuous infusion of factor concentrate has been reported as a life-saving intervention in these patients. We reported a teenager with severe hemophilia A and a high-titer inhibitor who underwent two surgeries for ACS which developed after a minor trauma. Computerized tomography angiogram (CTA) of abdomen revealed a large pelvic hematoma and a bleeding from the sigmoidal artery. He underwent an abdominal angiography followed by the first surgery to relieve the ACS, and the second surgery for abdominal closure. The patients received plasma exchange with cryo-removed plasma peri-operatively. High-dose factor VIII (FVIII) concentrate (100 U/kg) was started after plasma exchange followed by continuous infusion at the rate 14 units/kg/hour for 7 days. rFVIIa and APCC concomitant with tranexamic acid were used for breakthrough bleeding. He received six times of plasma exchange, three doses of rFVIIa and five doses of APCC. Bleeding was successfully stopped and the titers of inhibitor decreased from the maximum of 4,400 BU to 3,680 BU. Plasma exchange and continuous FVIII infusion can be considered as an option for life-threatening hemorrhage in hemophilia patients with high-titer inhibitors in the countries where an access to bypassing agents is limited.

Neonatal alloimmune thrombocytopenia (NAIT) is a result of platelet destruction by maternal antibodies directed against paternally-inherited antigens on neonatal platelets. NAIT is mostly caused by antibodies to human platelet antigens (HPA). This is a report of a term newborn with NAIT associated with an antibody to the human leukocyte antigens (HLA). The newborn presented with cutaneous bleeding at 5 days of age. The lowest platelet count was 36.8x10 9 /L. An antibody to HLA-A11 was present in maternal serum while HPA antibodies were not detected. The HLA-A11 antigen was identified in the patient, sister, and father, but not the mother. The newborn received intravenous immunoglobulin and had a favorable outcome.