摘要:

Mesenchymal hamartoma (MH) is a rare but distinctive tumor-like malformation of the liver with predilection for infants in the first 2 years of life. MH accounted for only 5% of all primary hepatic tumors seen at Children's Hospital. Boston, over a 57-year-period from 1924 through 1981. There were nine children (five females, four males) with a median age of 11 months at the time of diagnosis (range: 5 months to 7 years). The most common presenting manifestation was abdominal enlargement with a palpable nontender mass. Exploratory laparotomy was performed in each case. The MHs were located in the right lobe and varied in size from 3 to 21 cm (average diameter. 16 cm). One MH also involved a portion of the left lobe. Four of seven children who underwent hepatic lobectomy were alive and symptom-free for 1 to 18 years later. There were three intraoperative deaths due to hemorrhage and cardiac arrest. One child underwent biopsy only with drainage of cyst fluid but died in the postoperative period because of pneumonia. The last child who had wedge resection of an incidental MH died of extrahepatic biliary atresia. Although complete surgical resection is curative, past experience indicates a substantial risk for operative mortality. In some cases, a more conservative approach to therapy is suggested with partial excision or drainage of cysts and possible resection at a future date.

ISSN:0192-8562    

出版商:OVID    

年代:1986

数据来源: OVID

摘要:

By using a modification of the microtiter solid-phase radioimmunoassay, we have measuredEscherichia coliL-Asparaginase (L-ASP) specific IgG, IgG4, and IgE antibodies in children who received L-ASP as part of their chemotherapy for leukemia and lymphoma. In 13 children with acute lymphoblastic leukemia induced with vincristine, prednisone, and L-ASP (10,000 IU/M2i.v. each week for 3 weeks), seven developed high titer specific IgG antibodies. Four of the seven relapsed at the time of their peaking IgG response (6–10 months). None of the six with low or absent L-ASP antibody response have relapsed (followed for 20–35 months). In six children with allergic reactions to L-ASP reinduction, all had high titers of L-ASP specific IgG4(≥20 U/ml) at the time of their reaction. In 16 other children with low L-ASP IgG4(<13 U/ml), none demonstrated allergic reactions to rechallenge. Specific IgE was not consistently detectable in either group. In 21 patients with leukemia or lymphoma on L-ASP with cyclophosphamide-containing regimens, none developed significant IgG antibody response, compared with seven of 13 not receiving cyclophosphamide (p < 0.001). We conclude: (a) development of L-ASP antibodies may have prognostic significance; (b) the detection of specific IgG4can predict L-ASP allergy; and (c) cyclophosphamide-containing regimens reduce antibody formation to L-ASP and may allow repetitive (without anaphylaxis) and more effective (avoiding neutralizing antibodies) use of L-ASP.

ISSN:0192-8562    

出版商:OVID    

年代:1986

数据来源: OVID

摘要:

We reviewed the clinical features of 100 cases of the sickle cell chest syndrome in 57 pediatric patients hospitalized with radiographic findings of pulmonary or pleural disease. Pulmonary infiltration was more common in the lower lobes (86%) than in the upper (25%) or middle lobes (22%). Pleural effusions were present in 38% of cases. Chest syndrome was recognized on presentation in 79% of cases, but was recognized only later in 21% of patients admitted for other indications. Patients recognized initially were more often febrile on admission (68%) than were subsequently recognized patients (33%) (p < 0.01). but fever eventually occurred in 99 of 100 cases. Pain was an antecedent or coincident problem in 67% of cases. Median hospital stay was 7 days in those 58 cases in which narcotics were given, but only 4 days in those 42 cases in which narcotics were not administered (p < 0.001). Polyvalent pneumococcal vaccine had been administered to 44 of our 57 patients at some time before their hospitalizations and 18 patients had been on oral penicillin prophylaxis. Blood was cultured in 93 cases and in only two instances grewStreptococcus pneumoniae.Serologic evidence ofHemoplulus influenzaetype b infection was found in two additional patients. We conclude that the sickle cell chest syndrome is an acute febrile pulmonary disease frequently associated with pain and/or narcotic analgesic therapy but infrequently associated with proven bacterial infection.

ISSN:0192-8562    

出版商:OVID    

年代:1986

数据来源: OVID

摘要:

Nine newly diagnosed, previously untreated children (mean age: 4.2 years, range: 1–9 years) with severe acute idiopathic thrombocytopenia purpura (mean platelet count: 5.8 × 109/L, range: 1–12 × 109/L) were treated with high-dose steroids (prednisone 4–8 mg/kg/day). Steroid dose was based on platelet count at presentation: Group I (platelets less than 5 × 109/L) was started on 8 mg/kg/day; Group II (platelets 5–15 × 109/L) received 6 mg/kg/day. All patients had serologic and histologic evidence of acute idiopathic thrombocytopenia purpura. On this protocol, it took a mean number of 1.9 days (1–3 days) to reach a platelet count of at least 20 109/L and 9.2 days (3–26 days) to reach a normal platelet count. No significant toxicity was observed except for weight gain ranging from 3–10% and mild behavioral problems. Both groups were on high-dose steroids (4–8 mg/kg/day) for 7.3 ± 2.1 days. Only one patient had a brief relapse to a platelet count of 18 × 109/L while on therapy (day 14), but responded promptly to an increase in prednisone dose. Presently, all nine patients are in remission and have not required maintenance medication.

ISSN:0192-8562    

出版商:OVID    

年代:1986

数据来源: OVID

摘要:

Hemostatic changes in 20 children with acute lymphoblastic leukemia (ALL) who were induced with L-asparaginase (L-asp). vincristine (VCR), and prednisone (PDN) (Group A) were prospectively evaluated. These data were compared with those of a previous group of ALL patients who received L-asp as a single agent during consolidation (Group B). In Group A patients, mean plasma antithrombin activity decreased in the first 2 weeks, though not significantly. Relative to pretreatment values, mean fibrinogen concentration diminished particularly by week 3 (p < 0.001). Activated partial thromboplastin time (APTT) decreased in the last week as well as after cessation of therapy with L-asp (p < 0.05). Mean platelet count increased significantly by week 3 (p < 0.05). Thromboelastograms performed in seven patients confirmed the tendency for thrombosis evidenced by a decreased APTT. Patients in Group B (L-asp alone during consolidation) had decreased concentrations of fibrinogen, AT. and Factors IX and × after L-asp therapy. APTT was prolonged. Our data demonstrate that the tendency for thrombosis is the predominant manifestation of L-asp induced coagulopathy, when the drug is associated with VCR and PDN. Thus the risk/benefit ratio for the use of L-asp early in induction in children with low risk ALL needs to be further evaluated.

ISSN:0192-8562    

出版商:OVID    

年代:1986

数据来源: OVID

摘要:

The virus-associated hemophagocytic syndrome (VAHS) is a histiocytic proliferative disorder with bone marrow and liver failure for which the connection with a specific virus is often tenuous. Epstein-Barr virus (EBV) is one candidate for the association, but serologic or culture confirmation may be lacking in a particular case. As a means of directly identifying the presence of EBV in patients' cells, molecular hybridization studies were carried out using a radioactively labeled viral DNA segment. DNA from mononuclear cells of two children with VAHS had specific hybridization to the EBV DNA probe. One of the patients had serologic evidence for EBV infection. Several immunologic deficiencies were found. VAHS may represent one of several hematologic and/or immunologic dysfunctions caused by EBV.

ISSN:0192-8562    

出版商:OVID    

年代:1986

数据来源: OVID

摘要:

Studies on proliferation and differentiation of granulocyte-monocyte progenitor cells in Chediak-Higashi syndrome (CHS) were done on a 1-month-old patient, using the soft-agar bone marrow culture technique. The number of granulocyte-macrophage colony-forming cells (GM-CFC) was markedly increased, but with a normal distribution into granulocyte, macrophage, or mixed colonies. Morphologic, cytochemical, and ultrastructural studies showed that 70% of the colonies consisted of cells with giant lysosomes typical of CHS, and in the remaining 30% abnormal cells were not detected. The supply of granulocyte-macrophage colony-stimulating factor (GM-CSF) by the patient's peripheral blood leukocytes was markedly decreased. Inhibition of normal in vitro granulopoiesis by the patient's lymphocytes or serum was not demonstrated. It appears that granulocyte progenitors in CHS proliferate normally, or even in excess, probably in response to intramedullary destruction of granulocytes. The majority of the progenitors are intrinsically defective and give rise to colonies that contain the abnormality. In others the defects are unidentifiable, probably due to the immaturity of the specific fusion process of the cytoplasmic granules. The abnormal leukocytes in CHS are also defective in their capacity to provide GM-CSF, and this may account in part to the overt neutropenia. These studies demonstrate that the basic cytoplasmic abnormalities of the granulocytes and monocytes in CHS are embedded in the granulocytic-monocytic committed stem cell.

ISSN:0192-8562    

出版商:OVID    

年代:1986

数据来源: OVID

摘要:

A small, round cell tumor of the left scapula was found in a 3-month-old female Caucasian child. The histology was consistent with Ewing's sarcoma. Subsequently, a solitary pulmonary metastasis was excised. Thirty-one months after diagnosis, the child presented with leukokoria and a solitary pleural metastasis. The histology of both was identical to that of the original scapular tumor. G-banded karyotypes of the pulmonary metastasis and the ocular tumor revealed the presence of a balanced translocation, 46,XX, t(11;22)(q24;q12), which supported the original diagnosis of Ewing's sarcoma.

ISSN:0192-8562    

出版商:OVID    

年代:1986

数据来源: OVID

摘要:

Three children presenting with severe symptomatic bleeding episodes (one child developing a life-threatening subdural hematoma) due to acquired coagulation inhibitors are reported. In two patients, there was a history of an antecedent viral syndrome. None had evidence of drug exposure or an underlying immune disorder. All responded to the administration of corticosteroids, although one patient was steroid-dependent and required immunosuppressive therapy to achieve a complete response. Laboratory characteristics did not clearly distinguish the type of circulating inhibitor present in these children. Since bleeding was a prominent feature, we suggest that the inhibitors noted represent multiple specific coagulation factor inhibitors, rather than a “lupus-like” inhibitor which represents a common antibody to phospholipid-dependent coagulation tests. Such inhibitors may not be as benign as the common lupus anticoagulant seen in adult patients and should be considered in the child with symptomatic bleeding and a prolonged partial thromboplastin time. The true incidence and exact mechanism of action of acquired inhibitors in children has not been established.

ISSN:0192-8562    

出版商:OVID    

年代:1986

数据来源: OVID

摘要:

A case of mesoblastic nephroma, hypercalcemia, and raised levels of prostaglandins in a 2-month-old female infant is reported. Plasma parathyroid hormone (PTH) was normal and urinary prostaglandins were raised. During surgery a prostaglandin arteriovenous gradient was demonstrated. A large quantity of PGE was extracted from the tumor by radioimmunoassay after incubation. Both blood calcium and urinary prostaglandins returned to normal after nephrectomy. These results show that this tumor produced prostaglandins, the mediators of hypercalcemia in this patient.

ISSN:0192-8562    

出版商:OVID    

年代:1986

数据来源: OVID