Cerebral edema and priapism in an adolescent with acute lymphoblastic leukemia

Priapism and increased intracranial pressure are both rare, but recognized, manifestations of leukemia. However, they have never been reported in the same patient. We report a 15-year-old male with acute lymphoblastic leukemia who presented with hyperleukocytosis, priapism, and increased intracranial pressure. Central nervous system leukostasis and cerebral edema may have been detected earlier, had his history of priapism been known. Management of hyperleukocytosis complicated by priapism and increased intracranial pressure is discussed.     

精神分裂症患儿利司培酮治疗前后血清瘦素和白细胞介素-1β变化的意义

目的探讨精神分裂症患儿利司培酮治疗前后血清瘦素和白细胞介素-1β(IL-1β)水平的变化及意义。方法观察组31例精神分裂症患儿,利司培酮治疗前和治疗8周后分别测量身高、体质量以计算体质量指数(BMI),用放射免疫法检测其空腹血清瘦素和IL-1β。选取31例健康儿童作为对照组。结果观察组治疗后BMI、血清瘦素水平均明显上升,与治疗前相比差异均有显著性(P均<0.05);观察组血清IL-1β水平在治疗前与对照组相比明显增高,治疗后明显下降,与治疗前相比差异有显著性(P<0.05)。结论首发精神分裂症患儿血清IL-1β水平明显升高,利司培酮治疗后易出现药源性肥胖,低IL-1β水平可能是瘦素抵抗的原因之一。     

Hydatid disease in children may have an atypical presentation

 Although there is voluminous literature describing various aspects of hydatid disease in children, little attention has been paid to the small group of patients whose symptoms result in atypical presentation. This article addresses this problem, describing the features in ten children aged from 2 to 12 years. The sites of involvement were within a choledochal cyst (1). the pelvic cavity (1), the spleen (1), and transverse mesocolon. Albendazole was efficacious in the treatment of one recurrent case, as well as in preventing recurrence. Accepted: 19 April 1999     

Solitary bone plasmocytoma of the spine in an adolescent

Solitary plasmocytoma (SP) represent only about 5% of plasma cell neoplasia. Most patients have generalized disease, that is, multiple myeloma (MM). Solitary bone plasmocytoma (SBP) is a localized plasma cell tumor and is a very rare disease in young patients. We reported here, a case of SPB in a 14-year-old girl with a 10-year disease-free survival after an aggressive treatment. The relationship of SBP to MM continues to be controversial. Recommendations on the diagnosis and management of SBP in adults, based on a literature search and consensus of expert opinion, were recently published on behalf of the Guidelines Working Group of the United Kingdom Myeloma Forum 1. MRI of the spine is necessary to assess local disease. Radiotherapy with doses of 45-50 Gy is the recommended treatment and gives a high rate of local control (83-96%). Chemotherapy remains controversial in contrast to MM, in which intensive chemotherapy with autologous bone marrow transplantation (ABMT) is widely accepted. At the present time, considering the good prognosis of patients with a normal MRI at diagnosis and a complete disappearance of the M protein after radiotherapy, we believe that ABMT should be reserved for relapse or primary therapeutic failure.     

Massive splenic infarction in an adolescent with hemoglobin S‐HPFH

Hemoglobin sickle‐hereditary persistence of fetal hemoglobin (S‐HPFH) is a condition in which there is compound heterozygosity for the Hb S mutation and the HPFH deletion. These patients have no anemia, little evidence of hemolysis and generally have a benign clinical course compared to other types of sickle cell anemia. We describe a 19‐year‐old male with HbS‐HPFH who had no history of anemia or vaso‐occlusive crisis, who presented with a massive splenic infarct. We conclude that patients with HbS‐HPFH can occasionally present with severe complications and require a high level of clinical suspicion for complications when presenting to the hospital. Pediatr Blood Cancer 2013; 60: E49–E51. © 2012 Wiley Periodicals, Inc.     

Osteosarcoma in patients younger than 12 years old without metastases have similar prognosis as adolescent and young adults

Background

Childhood cancer is relatively rare and tends to present specific age distribution, as a prognostic factor for some of these diseases. Information on how young age affects prognosis, response to chemotherapy, and local control options in children versus AYA with osteosarcoma (OST) is minimal.

Methods

In order to identify the main differences in clinicalpathologic features, surgical approaches and survival rates of primary high grade OST of the extremity between children (n = 156; <12 years old) and AYA (n = 397; 12–30 years old), the institutional database with 553 patients treated by BOTG studies over 15 years were reviewed.

Results

There were no differences in metastasess at diagnosis, tumor size, and grade of necrosis between the two age groups. The rate of amputation was 30% higher in the children group (P = 0.018). The rate of limb salvage surgery using reconstruction with allograft or autograft was 70% higher in the children group (P = 0.018) while endoprosthesis rate was 40% higher in the AYA group (P = 0.018). The log rank test revealed that survival is similar between the two age groups for non‐metastatic patients (P = 0.424 for OS and P = 0.393 for EFS). Metastatic patients of both ages group had higher risk of dying compared to non‐metastatic (HR 3.283 95% CI 2.581–4.177; P < 0.001). Children with metastases at diagnosis had less OS time (P = 0.049) and EFS time (P = 0.032) than adolescents.

Conclusion

Non‐metastatic OST in preadolescent patients does not appear to be significantly differentfrom those seen in AYA patients, but has local control challenges. Children presenting with metastases should be considered an ultra‐high‐risk group. Pediatr Blood Cancer 2015;62:1209–1213. © 2015 Wiley Periodicals, Inc.     

Febrile neutropenia as the presenting sign of appendicitis in an adolescent with acute myelogenous leukemia

The diagnosis and management of a surgical abdomen in patients with acute leukemia is quite difficult because of the complications and treatment of disease itself. A 13-year-old boy with acute myelogenous leukemia developed 2 episodes of febrile neutropenia during induction therapy. The second one was treated with a 5-day course of parenteral antimicrobial therapy, but the patient then presented with right lower quadrant abdominal tenderness, guarding, and rebound tenderness. Abdominal ultrasonography and computed tomography revealed appendicitis. Conservative medical management was unsuccessful, and appendectomy was performed 5 days after appendicitis was diagnosed. The patient's clinical manifestations resolved 5 days later. The case illustrates that fever may be the first manifestation of appendicitis in a child with acute myelogenous leukaemia who is neutropenic. Surgery is acceptable as first-line treatment in such cases.     

青少年儿童体质指数与血压关系探讨

目的探讨青少年儿童体质指数（BMI）与血压的关系。方法测量7963例贵阳市两城区8～17岁青少年儿童的血压、身高、体重,按WGOC标准将受检对象分为超重、肥胖及正常体重组,分析比较各组血压值差异及血压值与BMI的关系。结果①不同BMI组间收缩压（SBP）、舒张压（DBP）均值都是肥胖组〉超重组〉正常体重组（P〈0.01）;②BMI与SBP、DBP呈显著正相关（r=0.520、0.365,P均〈0.01）;控制年龄、性别、身高影响的偏相关分析显示BMI与SBP、DBP呈独立正相关（r=0.390、0.210,P均〈0.01）;③不同BMI组间高血压患病率分别为24.49%、10.76%、2.65%,肥胖组〉超重组〉非超重组（P〈0.01）;④超重、肥胖青少年儿童的高血压发病危险是体重正常青少年儿童的4倍和9倍。结论青少年儿童的BMI与血压密切相关,应重视对超重、肥胖青少年的行为干预。     

Quality of life in chronic illness: children, parents and paediatricians have different, but stable perceptions

AIM: Quality of life assessments can be helpful to estimate the well-being of chronically ill children. The aim of this study was to investigate the differences in perception of health-related quality of life (HRQoL) among children, parents and paediatricians at the time of diagnosis and after initial treatment in four chronic diseases. METHODS: HRQoL was assessed with the Health Utilities Index mark 3 (HUI3). The HUI3 consists of eight attributes (vision, hearing, speech, ambulation, dexterity, emotion, cognition and pain). RESULTS: Nineteen paediatricians and 60 patients (aged 10-17 years) and their parents with newly diagnosed acute lymphoblastic leukaemia, juvenile idiopathic arthritis, asthma or with cystic fibrosis admitted for pneumonia participated in the study. Health and well-being perceptions were clearly different among paediatricians, parents and patients, both at diagnosis and after initial treatment. Perception differences were more prominent in the subjective attributes, emotion and pain. The agreement for these attributes was 23% and 5%, respectively. Paediatricians assessed the patients to have less pain than the patients and parents did. The reverse was true for the attribute emotion. At follow-up, the agreement was higher for the attributes ambulation and pain. CONCLUSION: At the onset of a chronic disease and after initial treatment, paediatricians, parents and children have different perceptions of the child's quality of life, particularly as to the subjective attributes pain and emotion. In view of these differences in perception among patients, their caregivers and paediatricians, this study suggests that whenever possible, multi-respondent assessment of HRQoL should be considered.     

Recurrent intrathecal methotrexate induced neurotoxicity in an adolescent with acute lymphoblastic leukemia: Serial clinical and radiologic findings

Systemic and intrathecal methotrexate (MTX) are integral components of acute lymphoblastic leukemia (ALL) therapy, but can be associated with neurotoxicity. We describe here the case of an adolescent male with T-cell ALL who developed recurrent episodes of subacute neurotoxicity characterized by slurred speech, emotional lability, and hemiparesis after intrathecal MTX administration. Serial magnetic resonance imaging with diffusion-weighted imaging showed recurrent areas of restricted diffusion within cerebral hemispheric white matter, which correlated chronologically with the administration of intrathecal therapy and severity of clinical symptoms. Resolution of diffusion abnormalities did not preclude further toxicity and a large lesion could cause persisting symptoms.     

Inflammatory esophagogastric polyp and fold in an adolescent

The case of a 13 year old boy with an inflammatory esophagogastric polyp and ulcerative colitis is described. Endoscopy revealed a typical polyp and gastric fold complex at the esophagogastric junction and a hiatal hernia. Histology of a biopsy specimen confirmed an inflammatory polyp covered by hyperplastic squamous and gastric foveolar epithelium. Continuous 24 hour esophageal manometry suggested gastroesophageal reflux, which may be related to the pathogenesis of the lesion. Follow-up endoscopy showed marked regression of the polyp with medication for reflux eosphagitis. This clinical entity is rare in childhood and adolescence, and the manifestations may not be readily recognized. Therefore, endoscopic biopsy is important in children with esophageal polyps. However, polypectomy is unnecessary except when malignancy is suspected or when symptoms persist.