Congenital deficiency of fibrin-stabilizing factor (factor XIII).
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Child, Preschool↗
Biomedical subjects
I J Hung
Publications and source records attributed to I J Hung.
Study of the acoustic reflex in human beings. I. Dynamic characteristics.
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Acoustics↗
Subcutaneous panniculitic T-cell lymphoma developing in a child with idiopathic myelofibrosis.
PURPOSE: Subcutaneous panniculitic T-cell lymphoma is reported in a child with idiopathic myelofibrosis. Both disease entities are rarely seen in children. PATIENT AND METHODS: A girl aged 5 years and 9 months had pancytopenia and severe constitutional symptoms. Idiopathic myelofibrosis was subsequently diagnosed. RESULTS: A transient response was achieved after treatment with a course of high-dose methylprednisolone therapy. However, proptosis and skin nodules developed during tapering of steroid therapy. A computed tomography scan of the orbit also revealed a mass lesion in the right lacrimal gland region. A skin biopsy specimen showed a subcutaneous panniculitic T-cell lymphoma. The clinical course was marked by high fever, profound pancytopenia, massive gastrointestinal bleeding, and severe, recurrent infections. Her condition rapidly deteriorated, and she died from polymicrobial sepsis 4 months after her initial examination. CONCLUSIONS: Subcutaneous panniculitic T-cell lymphoma is a distinctive clinicopathologic entity that is rarely seen in children. The association of myelofibrosis and peripheral T-cell lymphoma as seen in this has been rarely reported.
Child↗
The therapeutic outcome of medulloblastoma in 49 children.
The medical records of 63 patients with histologically-confirmed medulloblastoma were reviewed. From these, the clinical features and treatment outcome of 49 patients were available for analysis. Following tumor resections, 44 patients were given craniospinal radiation therapy with local boosts to the posterior fossa and other macroscopically involved areas. Twenty patients received chemotherapy: 17 as postoperative adjuvant or salvage therapies, and 3 as preradiation chemotherapy. The postoperative early death occurred in two patients. The projected 5-year survival and recurrence-free survival rate of the overall group and the 41 patients who had completed radiotherapy were 61%, 70% and 50%, 57%, respectively. Two significant favorable factors were identified: complete tumor resection and combination radiotherapy. A better five-year survival rate was found in patients who had complete tumor resection (78% vs 19%); with combination radiotherapy the rate was 70% vs 0%.
Adolescent↗
Candida tropicalis fungemia in children with leukemia and lymphoma.
Five episodes of fungemias are described; all had occurred in children with leukemia or lymphoma between January 1, 1978 and December 31, 1990. These fungemias comprised 3.4% of the total septicemias encountered during that period. Three episodes occurred during the induction phase and two during relapse. All patients had fever of varying degree and duration. In addition to steroids, all were receiving combination antibiotics before the fungemia had occurred. All patients had severe neutropenia lasting more than one week. Bacteremia preceded fungemia in four patients. Two episodes were diagnosed antemortem. The same species were isolated from other sites in three cases. Fever, chills and gastrointestinal symptoms were the most common clinical features; other symptoms included cough, dyspnea, oliguria and azotemia. One patient experienced skin lesion, dysphagia, hoarseness and hemiparesis. Only one patient survived. The prognosis from fungemia in leukemia and lymphoma patients is very poor. Empiric antifungal therapy is indicated in neutropenic patients who have recurrent or persistent fever despite one week of broad spectrum antibiotics. Early diagnosis and treatment will aid in improving the overall poor outcome of this disease.
Adolescent↗
Unusual clinical features of neonatal anemia due to fetomaternal transfusion.
Fetomaternal transfusion occurs in about 50 percent pregnancies. However, it can cause severe neonatal anemia at birth. A one-day-old baby girl, small for gestational age, was born to a G1P1 woman without perinatal blood loss or obstetric complication. Pallor and mild tachypnea were noted after birth. Laboratory examination revealed severe anemia, (Hb: 4.8 gm/dl, Hct: 14.7%), reticulocytosis and normoblastemia. Direct coombs' test was negative. Brain echo examination was normal. Maternal peripheral blood smear stained with Kleihauer-Betke technique demonstrated presence of fetal cells. Following packed red blood cells transfusion, the baby's symptoms improved. The unusual clinical features were described and discussed. Kleihauer-Betke test should be performed in unexpected neonatal anemia after birth.
Anemia, Neonatal↗
Interferon therapy in an infant with Kasabach-Merritt syndrome.
An infant with Kasabach-Merritt syndrome was treated with interferon-alfa 2b. Clinical and hematological responses were followed. Intron-A (Schering-Plough) was administered to a two and half-month-old male infant after failure of most other conventional therapies. A daily dose of 100,000 u/Kg was given subcutaneously for a total of 122 days. Clinical and laboratory aggravation was observed in the first eight days. The fibrinogen level increased to > 100 mg/dl after two weeks of Intron-A treatment. Local inflammatory signs began to improve after 21 days. The platelet count became normal after 80 days of treatment. During follow-up, there was continuous regression of the tumor.
Hemangioma↗
Poststreptococcal reactive arthritis: report of one case.
Poststreptococcal reactive arthritis (PSRA) is a clinical syndrome of reactive arthritis. It is associated with recent streptococcal infections, but could not fulfill the revised Jones criteria for acute rheumatic fever (ARF). The incidence of PSRA cardiac complications to develop was as high as the ARF's developing into rheumatic heart disease. A 9-year-old boy presented with limping gait. He had pain in his left knee for 4 days but no fever. His right knee was swelling with a limitation of movement. A throat culture showed positive growth for group A streptococcus, and consequently antistreptolysin-O serum titer and C-reactive protein were elevated. A synovial fluid examination was turbid but sterile. After 3 days the arthralgia subsided rapidly. A cardiac color Doppler and electrocardiogram showed no evidence of valvular disease. Under the threat of high incidence of rheumatic heart disease in PSRA, we treated this patient with prophylactic antibiotics as acute rheumatic fever. A clinic follow up one year later showed neither sequels nor heart murmur on physical examination.
Anti-Inflammatory Agents, Non-Steroidal↗