Treatment of esophageal caustic injuries: experience with high-dose dexamethasone

Caustic ingestion is one of the most life-threatening events in the pediatric age group. Several types of non-surgical management have been proposed for the esophageal strictures that inevitably follow ingestion. These include esophageal stenting and steroid therapy, which has received much attention in recent years. In this study, high-dose dexamethasone (1 mg/kg daily for 4–6 weeks) was administered to 9 children with endoscopic evidence of lesions equal to or greater than extended second degree who were at high risk of developing esophageal stenosis. Results were compared with those of other groups of children treated with the current protocol of 2 mg/kg daily oral prednisone. Clinical findings demonstrated that dexamethasone exerts a more effective anti-inflammatory action along with reduced cushingoid side effects; this enables children to better tolerate repeated dilatations, which often are necessary to maintain esophageal patency. However, since these treatment protocols were applied mainly to ammonia ingestions, which are known to induce less severe sequelae, further investigations are required in the presence of more serious initial lesions. Correspondence to: S. Cadranel     

Successful treatment of retinoic acid syndrome with high-dose dexamethasone pulse therapy in a child with acute promyelocytic leukemia treated with ATRA

A 5 year old female developed femoral pain, fever, and hemorrhagic tendency. She was diagnosed as having acute promyelocytic leukemia (APL). Approximately 2 weeks after the administration of all-trans retinoic acid (ATRA), she developed a high fever, edema, and respiratory distress which met the criteria for retinoic acid syndrome. At first, we tried to treat the patient with oral corticosteroid, however, this approach was unsuccessful. Considering the worsening of her condition, we then chose to administer a large dose of intravenous dexamethasone therapy for 3 days. Immediately after this therapy, she became afebrile, respiratory distress and edema disappeared, and there was a general improvement of the symptoms. All-trans retinoic acid at the reduced dose of 25 mg/m², was continued for an additional 6 weeks and then discontinued. Since the cessation of dexamethasone and ATRA, there has been no relapse of APL in this patient. Although based on only one case, we recommend the intravenous high-dose dexamethasone pulse therapy (13 mg/m² per day, for 3 days) for treating retinoic acid syndrome which develops in pediatric APL patients treated with ATRA.     

Neuroblastoma-Associated Paraneoplastic Syndrome with anti-Hu Antineuronal Antibodies Presenting at the Time of Recurrence

A 27-month-old girl presented with chest pain. Further evaluation confirmed posterior mediastinal neuroblastoma with bone marrow infiltration, which was treated with a combination of chemotherapy and surgery. Four months after completing treatment, she presented with myoclonus and weakness of her right arm. The myoclonus eventually subsided but her right arm weakness progressed to a right hemiplegia. High titers of antineuronal nuclear antibodies identified as anti-Hu were found in both serum and cerebrospinal fluid. One month later she presented with a relapse of her original tumor, from which she died. Identification of anti-Hu antineuronal nuclear antibodies in this neuroblastoma-associated paraneoplastic syndrome supports the hypothesis that the syndrome is due to autoimmune disease.     

Effect of steroid and high-dose immunoglobulin therapy on opsoclonus-myoclonus syndrome occurring in neuroblastoma

The authors describe a case of an 8-month-old boy with opsoclonus-myoclonus syndrome (OMS) and coincident unresectable neuroblastoma (NB). He achieved a complete remission for NB after 6 courses of standard-dose chemotherapy without significant neurological improvement despite the use of steroids and high-dose immunoglobulin (HIG), administered separately. Only the combined treatment withthese two drugs induced a complete disappearance of neurological symptoms. On the basis of this experience, the authors suggest the association of steroids plus HIG for the treatment of OMS in patients not responsive to conventional first line therapy with steroids. Med. Pediatr. Oncol. 30:15–17, 1998. © 1998 Wiley-Liss, Inc.     

Paraneoplastic gastro-intestinal anti-Hu syndrome in neuroblastoma

The anti-Hu syndrome is a well-known paraneoplastic syndrome and may be rarely seen in patients with neuroblastoma. However, it is relatively unknown that anti-Hu antibodies can cause gastro-intestinal signs and symptoms. We report on a child with neuroblastoma who presented with gastro-intestinal disturbances as a result of the anti-Hu syndrome and summaries two similar case reports reported in literature. Neuroblastoma patients with gastro-intestinal disturbances, ranging from constipation to a paralytic ileus, might suffer from the gastro-intestinal anti-Hu syndrome. The causative antibodies can be determined to diagnose or exclude this syndrome, and successful treatment is possible.     

Opsoclonus-myoclonus and anti-Hu positive limbic encephalitis in a patient with neuroblastoma

Opsoclonus-myoclonus syndrome (OMS) is seen in 2-3% of children with neuroblastoma and is believed to be caused by an autoimmune process elicited by the tumor. Although long-term neurologic sequelae are common in children with OMS, limbic encephalitis has not previously been reported. We report a child who developed limbic encephalitis associated with anti-Hu antibodies, 6 years after her initial diagnosis of neuroblastoma and OMS. This case demonstrates that patients with neuroblastoma and OMS are at risk for developing new paraneoplastic symptoms years after their original diagnosis and emphasizes the need for careful long-term follow-up.     

Multifactorial analysis of opsoclonus‐myoclonus syndrome etiology (“Tumor” vs. “No tumor”) in a cohort of 356 US children

1 Background

Pediatric opsoclonus‐myoclonus syndrome (OMS) presents a paradox of etiopathogenesis: A neuroblastic tumor (NB) is found in only one half of the cases, the others are ascribed to infections or designated as idiopathic.

2 Method

From an IRB‐approved observational study of 356 US children with OMS, secondary analysis of “etiology” and related factors was performed on a well‐characterized cohort. The “Tumor” (n = 173) and “No Tumor” groups (n = 183), as defined radiologically, were compared according to multiple factors considered potentially differentiating. Data were analyzed retrospectively using parametric and nonparametric tests as indicated.

3 Results

Patients with NB were not distinguishable by prodromal symptoms, OMS onset age, gender, race/ethnicity, OMS severity, rank order of neurological sign appearance, or geographic distribution. Various CSF immunologic biomarker abnormalities of OMS did not vary in the presence or absence of a detectable tumor: frequency of six lymphocyte subsets, or concentrations of 18 cytokines/chemokines, cytokine antagonists, chemokine receptors, cell adhesion molecules, or neuronal/glial markers. Prior responsiveness to conventional immunotherapy was not contingent on tumor/no tumor designation.

4 Conclusions

Multiple convergent factors provide compelling empirical evidence and rationalize the concept that OMS is one neurological disorder, regardless of apparent etiology. Limitations to the current clinical etiologic classifications as paraneoplastic, parainfectious/post‐infectious, and idiopathic etiology require antigen‐based biological solutions to tease out the molecular pathophysiology of viral/tumoral mechanisms. Systematic studies, regardless of presumed etiology, will be necessary to find the highest‐yield combination of imaging approaches, screening for infectious agents, and new biomarkers. Two testable hypotheses for future research are presented.     

The effects of early postnatal dexamethasone therapy on pulmonary outcome in premature infants with respiratory distress syndrome: a two-year follow-up study

AIM: To evaluate the pulmonary outcome at corrected age of 2 y on preterm infants who participated in a double-blind trial of early postnatal dexamethasone therapy (< 12 h after birth) for the prevention of chronic lung disease. METHODS: Clinical respiratory status, blood gases, acid-base balance and pulmonary function were evaluated at corrected age of 2 y in 116 preterm infants (59 infants in the control group; 57 in the dexamethasone-treated group). In the dexamethasone-treated group, dexamethasone was administered intravenously every 12 h in tapering doses: 0.25 mg/kg on days 1 through 7, 0.12 mg/kg on days 8 through 14, 0.05 mg/kg on days 15 through 21, and 0.02 mg/kg on days 21 through 28. RESULTS: The clinical and laboratory characteristics in the perinatal period were comparable between the groups. At the time of follow-up (mean +/- SD corrected age was 25.1 +/- 4.8 mo for the control group and 24.6 +/- 5.1 mo for the dexamethasone-treated group), there was a slightly lower mean body weight and body length, and a lower psychomotor developmental index in the dexamethasone-treated group than in the control group (10.9 +/- 2.1 vs 11.5 +/- 1.9 kg, 84.4 +/- 6.1 vs 85.9 +/- 5.8 cm, and 82 +/- 24 vs 89 +/- 26, respectively); however, these differences were not statistically significant. There were no significant differences between the control and dexamethasone-treated groups in clinical respiratory status, blood gases, acid-base balance or in lung mechanics (V(T): 9.5 +/- 2.0 vs 9.4 +/- 1.9 ml/kg; V(min): 0.23 +/- 0.04 vs 0.23 +/- 0.03 l/min/kg; C(RS): 13.1 +/- 3.9 vs 12.6 +/- 3.6 ml/kPa/kg; R(RS): 1.56 +/- 0.64 vs 1.62 +/- 0.58 kPa/l/s, respectively). CONCLUSION: There was no apparent adverse respiratory outcome associated with early postnatal dexamethasone therapy.     

原发性肾病综合征患儿血淋巴细胞凋亡、增殖及地塞米松对其作用

目的　探讨原发性肾病综合征 (PNS)患儿外周血淋巴细胞 (PBLs)凋亡、增殖及地塞米松 (Dex)对其作用。方法　将PNS分为微小病变组 (MCNS)和非微小病变组 (NMCNS)。碘化丙啶染色法测定PBLs凋亡率 ,观察空白组、Dex处理组和经PHA刺激后Dex处理组细胞凋亡情况 ;3 H 胸腺嘧啶 (3 H TdR)掺入法观察不同浓度Dex对PBLs增殖影响。结果　MCNS空白组PBLs凋亡率降低 ,Dex组凋亡率高于空白组 ;MCNS患儿PBLs对体外PHA刺激的增殖率降低 ,而空白组3 H TdR掺入量却增高。NMCNS与正常儿童均无明显差异。不同浓度Dex(10 -9～ 10 -6mol/L)呈剂量依赖性抑制PNS及正常儿童PHA刺激PBLs增殖。结论　MCNS组PBLs部分活化增殖 ,凋亡下调 ,可能参与MCNS的发病机制 ,而Dex可诱导其凋亡上调并抑制增殖 ,可能是糖皮质激素(GC)对PNS作用机制之一     

Paraneoplastic syndrome and intrathoracic Castleman disease

We report two cases of intrathoracic Castleman disease presenting with paraneoplastic syndrome. Patient 1 was a 10-year-old girl with short stature. She was found to have delayed bone age, slow growth velocity, and iron-deficiency anemia, which was refractory to treatment. Thrombocytosis and hypergammaglobulinemia were later detected. Chest X-ray revealed a hilar mass. Patient 2 was a 14-year-old boy who had severe cough, progressive mucocutaneous erosion, and dermatitis. Chest X-ray showed a mediastinal mass. Sections of skin biopsy showed findings consistent with pemphigus disease. In each case, the histological diagnosis of Castleman disease was made.     

儿童神经副肿瘤综合征临床特点

目的探讨神经系统副肿瘤综合征(PNS)的临床特点。方法回顾分析3例PNS患儿的临床表现、脑脊液检查、生化指标、脑电图、头颅MRI及腹部影像学检查资料。结果 3例患儿中男性2例、女性1例,发病年龄2～6岁。均出现神经系统症状,乳酸脱氢酶高,脑电图慢波增多,腹部影像学提示腹腔肿瘤;1例脑脊液白细胞增高、寡克隆蛋白阳性;2例头颅MRI出现相应改变。结论 PNS起病隐匿,行脑脊液、乳酸脱氢酶、头颅MRI、腹部影像学等检查有助于早期诊治。     

Sinus histiocytosis with massive lymphadenopathy (SHML) prednisone resistant but dexamethasone sensitive

Sinus histiocytosis with massive lymphadenopathy (SHML) is a histiocytic disorder affecting children and adults. It usually presents as markedly enlarged lymph nodes that require surgical biopsy for confirmation. This lesion is usually self-limited but can present in areas that can cause significant morbidity or disfigurement. We report a case that required therapy due to the severe disfigurement but was resistant till treated with dexamethasone. This case illustrates that SHML may be resistant to prednisone but still be sensitive to dexamethasone.     

小儿肾病综合征的冲击疗法

研究目的探讨小儿肾病综合征的治疗方法。研究方法小儿肾病综合征患儿共６６例，随机分为两组：冲击组（３４例）和对照组（３２例）。冲击组用地塞米松（１．５－３）ｍｇ／（ｋｇ．ｄ）加入１０％ＧＳ溶液（１００－１５０）ｍｌ静滴，每日１次，连用３ｄ，第４日停用，第５日开始隔日１次，共用６次。次日开始口服强的松（１．５－２）ｍｇ／（ｋｇ．ｄ），共４周，后渐减量。对照组仅用强的松，用法用量同冲击组。结果冲击完全缓