Thymic and adenotonsillar enlargement after successful treatment of malignancies

Anterior mediastinal and adenoid masses in children after cessation of chemotherapy for malignant disease often cause a diagnostic problem. Differential diagnosis of thymic enlargement and adenoid hyperplasia from recurrence frequently poses a challenge both for the radiologist and the physician. In this study the authors evaluated 491 patients with different malignant tumors for thymic and adenoid hyperplasia. Thymic hyperplasia was seen in 18 patients (5 Hodgkin disease (HD), 5 non-Hodgkin lymphoma (NHL), 4 Wilms tumor, 2 germ cell tumor, 1 Ewing sarcoma, and 1 neuroblastoma), only adenotonsillar hyperplasia was seen in 6 patients, all with NHL, and both thymic and adenotonsillar hyperplasia were seen in 3 patients (1 HD, 2 NHL). In 5 patients, adenoid hyperplasia was proven by biopsy; 1 patient underwent to adenoidectomy. Their histopathologic investigation showed polyclonal follicular hyperplasia. The authors recommend that patients with thymic and/or adenotonsillar enlargement after successful treatment of their primary malignancy should be evaluated cautiously before an invasive procedure is planned.     

Thymic carcinoma in a child with HIV infection

HIV infection predisposes to cancer during childhood. In addition to the AIDS-defining non-Hodgkin lymphoma (NHL) and Kaposi sarcoma, a range of other lymphoid malignancies and solid tumors have been described. We report the first case of an HIV-positive child with thymic carcinoma in the setting of regressing thymic cysts. The tumor expressed CKIT but failed to respond to imatinab mesylate after a transient response to multiagent chemotherapy. This case extends the spectrum of pediatric malignancy in the setting of HIV and suggests that patients with presumed benign thymic cysts require ongoing surveillance.     

Thymic enlargement in childhood

Thymic masses constitute one of the least common mediastinal masses in childhood. While producing symptoms of airway compromise, they also raise the suspicion of malignancy when detected. Radiological, operative and pathological findings of patients that have been operated for thymic masses in our institution is presented in this paper. Nine patients were operated in our institution during a 12-year-period between 1985-1997 for thymic masses. Ages of the patients ranged from four months to 13 years. With the exception of one, who was diagnosed with a routine chest x-ray, all the patients had respiratory complaints. All the patients had been evaluated with computed tomography preoperatively. In total, seven sternotomies and four thoracotomies were performed to reach the anterior mediastinum. The distribution of masses was as follows two malignant thymomas, three thymic hyperplasia, one lymphocyte-rich thymoma, one epithelial thymoma, one cystic thymoma and one lymphoblastic lymphoma. Although rare, thymic enlargement may be a cause of intractable respiratory complaints in childhood. Because of the high incidence of primary malignancy of the mediastinal neoplasms in childhood, thymic enlargement requires accurate pathological diagnosis and treatment. Median sternotomy with intensive anesthetical care allows proper tumoral exposure.     

Combined True Thymic Hyperplasia and Lymphoid Hyperplasia in Grave's Disease

True thymic hyperplasia (enlarged gland composed of histologically unremarkable cortical and medullary parenchyma) and lymphoid hyperplasia (medullary lymphoid follicles in the clinical setting of autoimmunity) usually develop as independent pathologic processes. We reviewed the clinical features and gross and microscopic pathology of 2 hyperthyroid patients with features of both thymic hyperplasia and lymphoid hyperplasia. The diagnosis of thymic hyperplasia was supported by thymic weights greater than two standard deviations above the mean weight for age and histologic evidence of expanded cortical and medullary parenchyma. The diagnosis of lymphoid hyperplasia was supported by the increased number and size of medullary lymphoid follicles and the association with Grave's disease. This unusual combination results from two separate pathogenic mechanisms operating simultaneously in hyperthyroid patients. Elevated thyroid hormones directly stimulate the proliferation of thymic epithelium, producing thymic hyperplasia. The immune abnormalities underlying Grave's disease can also result in lymphoid hyperplasia of the thymus.     

Cardiac abnormalities in children with hyperthyroidism

Summary The cardiac status of 18 hyperthyroid (HT) children (9 black and 9 white) was evaluated by echocardiography. Mitral regurgitation (MR) was diagnosed clinically in 33% (6 of the 9 blacks). None of the 9 white children had MR. Left ventricular end-diastolic diameter (LVEDD) and volume (LVEDV) did not differ from the predicted normal (PN) based on body surface area and heart rate, except in those with MR where increased LVEDD and LVEDV were noted (p<0.02). LV mass was +1.75 standard deviations (σ) of the PN (p<0.01), due to increased wall thickness or LVEDV. Left ventricular output (LVO) was +0.35σ PN (p=ns); however, when compared to that of normal children, LVO of HT was higher (p<0.001) due to the increased heart rate. Enhanced left ventricular contractility was suggested by increased rate of dimensional change during ejection (peak dD/dt-syst), with a mean value of −11.39 cm/sec as compared to the normal of −9.54 cm/sec (p<0.01). A linear multivariate regression equation differentiated the cardiac status of HT from that of normal children. Following treatment to euthyroid state, MR disappeared in 2 and became less in 4 patients. LVO, LV mass, and peak dD/dt-syst also became less. Significant cardiac changes occur in children with hyperthyroidism, which may be reversible in part after euthyroidism is restored. Supported in part by a grant (RR 305) from the General Clinical Research Centers Program of the Division of Research Resources, National Institutes of Health.     

甲巯咪唑治疗儿童甲状腺功能亢进症不良反应的观察

目的 研究甲巯咪唑治疗儿童甲状腺功能亢进症(简称“甲亢”)不良反应发生情况。方法 回顾性分析中国医科大学附属盛京医院2015年1月—2021年5月住院的304例甲亢患儿的临床资料。统计甲巯咪唑治疗相关不良反应的发生情况,探讨常见不良反应的危险因素。结果 304例患儿中,出现甲巯咪唑治疗相关不良反应87例(28.6%),其中男性20例(23%),女性67例(77%)。较常见的不良反应有粒细胞减少(12.8%)、皮疹(11.8%)、丙氨酸氨基转移酶升高(9.5%)及关节疼痛(3.0%),部分患儿可同时或间断出现多种不良反应。粒细胞减少主要出现在用药后3个月内(25/39,64%);丙氨酸氨基转移酶升高主要发生在用药后1个月内(17/29,59%);皮疹主要发生在用药后3个月内(30/36,83%)。对症治疗后上述不良反应大部分可恢复正常。多因素logistic回归分析显示,年龄小、治疗前中性粒细胞绝对计数低是甲巯咪唑治疗后粒细胞减少的危险因素(P<0.05)。结论 甲巯咪唑治疗儿童甲亢不良反应较常见,多发生在用药后3个月内,多数经过对症治疗可缓解。年龄越小,中性粒细胞绝对计数基线值越...     

Prolactin response to arginine in children with hyperthyroidism and primary hypothyroidism

Plasma prolactin (PRL) response to arginine was examined in 16 prepubertal and 18 pubertal children with constitutional short stature, 5 patients with hyperthyroidism and 4 patients with primary hypothyroidism. The mean basal concentration of plasma PRL was significantly higher (P<0.01) in primary hypothyroidism than in other groups. Arginine infusion elicited significant (P<0.05) rises in plasma PRL in all groups. The maximal increment of plasma PRL above the baseline level after arginine stimulation was significantly larger (P<0.05) in pubertal than in prepubertal females and was significantly smaller (P<0.05) in patients with hyperthyroidism than in age- and sex-matched controls. There was no sex difference in arginine-stimulated PRL secretion. These data suggest that arginine produces a significant increase in plasma PRL and the PRL response to arginine was greater in pubertal than in prepubertal children. Plasma PRL response to arginine is suppressed in children with hyperthyroidism and the basal plasma PRL is markedly elevated in primary hypothyroidism.     

心率变异性监测对小儿甲状腺功能亢进症自主神经功能的评价

目的　监测小儿甲状腺功能亢进症 (简称甲亢 ) 2 4h心率变异性 (HRV)的昼夜规律 ,探讨其自主神经功能异常的意义。方法　 2 4h动态心电图记录后 ,分别对 13例甲亢和 2 6例正常小儿 (对照组 )的HRV进行分析。结果　① 2 4h总HRV时域和频域指标提示甲亢组与对照组比较均有显著差异 (P <0 0 1)。②清醒期和睡眠期频域指标比较 :对照组低频标准单位 (LFNU)、低频 /高频比率 (LF/HF)在清醒期明显增高 (P <0 0 1) ,高频带 (HF)、高频标准单位 (HFNU)在睡眠期明显增高 (P <0 0 1) ;甲亢组总功率谱密度 (TP)、极低频带 (VLF)、低频带 (LF)、HF、LFNU、HFNU、LF/HF在清醒期和睡眠期比较无显著差异。③各组 2 4h的HRV时域动态变化比较 :对照组 2 4h的HRV时域变化有明显昼夜节律 ,以相邻正常RR间期差值均方根 (RMSSD)和相邻正常RR间期差值 >5 0ms占RR间期总数的百分比 (PNN50 )指标最敏感 ;甲亢组 2 4h的HRV时域各指标明显低于对照组 (P <0 0 1) ,失去昼夜规律。结论　甲亢患儿心脏自主神经受损 ,以迷走神经更严重。由于持续性的甲状腺素刺激 ,而表现为持续性交感神经占优势 ,失去昼夜规律 ,及时控制甲亢 ,并重新调整自主神经平衡对甲亢性心脏病发生有不可忽视的价值     

Thymic carcinoma, systemic lupus erythematosus, and hypertrophic pulmonary osteoarthropathy in an 11-year-old boy: a novel association

Thymic carcinoma is exceptionally rare in children and it has never previously been associated with autoimmune disorders. The authors report the case of an 11-year-old boy with thymic carcinoma, hypertrophic pulmonary osteoarthropathy, and an autoimmune disease that resembled systemic lupus erythematosus. To their knowledge, this is the first case of such complex clinical findings. The tumor was of high grade histologically and the boy died after 1 year, in spite of chemotherapy and radiotherapy. A review is presented of the available medical literature on thymic malignancy in childhood.     

Prolactin response to thyrotropin-releasing hormone in children with Turner's syndrome and hyperthyroidism

Plasma prolactin (PRL) response to synthetic thyrotropin-releasing hormone (TRH) was studied in 26 prepubertal and 19 pubertal children with constitutional short stature, 7 patients with Turner's syndrome and 10 patients with hyperthyroidism. The mean basal concentrations of plasma PRL did not differ among groups. In prepubertal children PRL responses to TRH were comparable in both sexes, while pubertal children plasma PRL levels after TRH in females were significantly higher (P<0.05) than those in age-matched males. Plasma PRL levels after TRH in patients with Turner's syndrome were significantly higher (P<0.05) than those in age-matched males, but were not significantly different from those in age-matched females. Plasma PRL response to TRH was markedly suppressed in patients with hyperthyroidism before treatment, but it returned to normal after treatment when patients became euthyroid. A significant correlation (P<0.05) between peak concentrations of plasma PRL after TRH stimulation and plasma T₃ but not T₄ levels was observed.These data suggest that a sex difference in TRH-stimulated PRL secretion appears around puberty and that plasma PRL response to TRH is suppressed in children with hyperthyroidism. The magnitude of plasma PRL response to TRH is closely correlated with the severity of hyperthyroidism when judged by plasma T₃ but not T₄ concentrations.     

DiGeorge综合征伴甲状腺功能亢进1例并文献复习

目的探讨DiGeorge综合征(DGS)合并甲状腺功能亢进(甲亢)患儿的临床特点,提高对该病的认识。方法报告1例DGS合并甲亢患儿的症状、辅助检查结果、外周血基因组DNA染色体芯片结果、治疗和随访情况;在中国期刊全文数据库(CNKI)和PubMed中检索DGS合并甲亢患儿的文献,检索时间为建库至2017年5月31日,总结DGS合并甲亢患儿的临床特征及其与遗传学异常的关联。结果患儿女,12岁,因"1年内抽搐发作2次,发现甲状腺功能异常4月"于2016年12月就诊于浙江大学医学院附属第一医院儿科。患儿有低钙抽搐、甲亢、身材矮小、智力异常、贫血和慢性中耳炎等多系统异常。染色体芯片检测结果显示22q11.21微缺失,缺失2 512 kb,基因组中位置18919095-21431174(hg19),诊断为DGS伴甲亢,予甲巯咪唑片(bid,起始为10 mg,2 d后改为5 mg)和补钙等对症治疗,血钙至正常范围后出院并继续口服甲巯咪唑片(5 mg,qd),出院3、6个月电话随访无抽搐发作。在PubMed中共检索到10篇英文文献报告了17例DGS合并甲亢患儿,病情不一,累及系统较多。结论 DGS临床表现多样,累及系统广泛,易误诊、漏诊和迟诊。对原发性甲状旁腺功能减退患儿,建议行染色体芯片分析并评估甲状腺功能。     

Thymic Hyperplasia Associated with Graves’ Disease in a 10-year-old Boy

Thymic hyperplasia associated with Graves’ disease is rarely reported in children, although it is not uncommon in adults. Occasionally, an enlarged thymus presents as an anterior mediastinal mass on a radiographic examination. Such patients often undergo invasive procedures such as a thymus biopsy or thymectomy because of suspected malignancy. However, an enlarged thymus with Graves’ disease is known to shrink after treatment with antithyroid drugs. Therefore, recognition of this benign course would avoid unnecessary surgical resection. This report presents the case of a 10-yr-old boy with Graves’ disease complicated with an anterior mediastinal mass. Computed tomography showed a homogenous mass with no invasion into the surrounding tissue. A gallium-67 scintigraphy showed no abnormal uptake. Shrinkage of the mass after treatment with an antithyroid drug (methyl-mercaptoimidazole) supported the diagnosis of thymic hyperplasia with Graves’ disease. This case report illustrates two important points. First, pediatricians should be aware that thymic hyperplasia can coexist with Graves’ disease, even in children. Second, close radiographic assessment would support a diagnosis of thymic hyperplasia and eliminate invasive diagnostic procedures.     

CHILDHOOD HYPERTHYROIDISM: Results of Treatment

Abstract. Mäenpää, J. and Kunsi, A. (Children's Hospital, University of Helsinki and Aurora Hospital Helsinki, Finland.) Childhood hyperthyroidism. Results of treatment. Acts Paediatr Scand, 69: 137, 1980.—40 hyperthyroid children were followed for 0.2–12 (mean 4.5) years. The treatment was antithyroid drugs in 20, subtotal thyroidectomy after a drug trial in 18 and primary thyroidectomy in 2 patients. 4 patients who relapsed (3 after surgery and 1 after a drug trial) were given radioiodide. 11 of the surgically treated glands were nodular. At the follow-up study 24 patients were euthyroid, 7 were on thyroxine therapy and in 5 others hypothyroidism was discovered. 2 subjects were still on antithyroid drugs and 2 relapsed. In 5 euthyroid patients the TRH test revealed a low thyroid reserve. In 28 of 34 subjects examined circulating antibodies to thyroid microsomes were present in high titres. Evidently, regular follow-up is needed because of the high risk of hypothyroidism.     

Thymic cyst haemorrhages and transient cholestasis in a 4-week-old infant

We report a 4-week-old boy with acute respiratory distress, due to massive haemorrhages in multiple thymic cysts. A right hemithymectomy was performed because of mechanical obstruction of the trachea by the cysts. The origin of the multilocular thymic cysts remained unclear. Most likely, these haemorrhages were caused by vitamin K deficiency, although the infant received vitamin K prophylaxis. In addition, he developed transient cholestasis, but the aetiology remained unclear. It is postulated that massive haemorrhages in thymic cysts produce large amounts of bilirubin, causing sludging of bile excretions in the liver. Four weeks after the operation, all laboratory findings were normal and 6 months after the operation the boy is still healthy. Conclusion This case report shows that respiratory distress in an infant can be caused by multiple haemorrhages in multilocular thymic cysts. Received: 12 June 1997 / Accepted in revised form: 4 September 1997     

Thymic Involution with Loss of Hassall's Corpuscles Mimicking Thymic Dysplasia in a Child with Transfusion-Associated Graft-Versus-Host Disease

A 7-year-old leukemic girl developed pancytopenia following chemotherapy and was given several transfusions of nonirradiated blood. Within 2 weeks she developed a maculopapular rash, fever, abnormal liver function, diarrhea, and wasting. She became septic and died 6 weeks later. Transfusion-associated graft-versus-host disease (GVHD) was suspected clinically. At autopsy, changes diagnostic of GVHD were present in the skin and liver. The remarkable feature of the case was the histopathology of the thymus, which was morphologically “dysplastic,” i.e., minute, lymphoid depleted, devoid of a corticomedullary demarcation, and completely lacking in Hassall's corpuscles. These changes were virtually identical to those seen in the thymus of children with severe combined immunodeficiency disease (SCID). There was no evidence of preexisting immune deficiency. There is compelling experimental evidence that GVHD can produce changes in the thymus that are identical to those of “thymic dysplasia.” These observations have led to the hypothesis that immunodeficiency associated with GVHD may stem, in part, from injury to thymic epithelium resulting in defective T cell maturation. As a corollary of this hypothesis, it has been suggested that the pathogenesis of some forms of SCID may involve GVHD-associated injury to the thymus by a maternal allograft acquired in utero. This report further documents thymic pathology in human GVHD and discusses these changes in the light of these ideas.     

Enuresis in hyperthyroidism: a temporary lack of central control mechanism leads to nocturnal enuresis

We report on a 9-year-old boy who suffered from hyperthyroidism and a new appearance of enuresis. Bedwetting ceased and prepulse inhibition (PPI) – measured as a parameter of central control – increased during the course of therapy.
Conclusion:  The increase in PPI is an indication that enuresis in hyperthyroidism could be as a result of a temporary loss of central control on brainstem reflexes. The case conveys new insights into the correlation between thyroid hormones and micturition patterns and the aetiology of enuresis.     

甘露聚糖联合胸腺肽治疗小儿反复呼吸道感染160例临床观察

目的 观察临床应用甘露聚糖联合胸腺肽治疗小儿反复呼吸道感染疗效及对免疫功能的影响.方法 160例反复感染的患儿随机分为对照组和治疗组,对照组给予一般的抗炎及对症治疗.治疗组在常规治疗的基础上给甘露聚糖联合胸腺肽.结果 治疗组160例,效果明显优于对照组156例(P＜0.01).治疗后治疗组的Ig值有明显的提高.结论 甘露聚糖联合胸腺肽治疗小儿反复呼吸道感染有很好的效果.     

Coexistent coeliac disease,Graves' disease and diabetes mellitus type 1 in a patient with Down syndrome

A 17-year-old girl with Down syndrome is presented who developed coeliac disease, Graves' disease and diabetes type 1. Her HLA type was A3, A9, B8, B15, DR3, DR5.     

Diffuse mesangial sclerosis: association with unreported congenital anomalies and placental enlargement

A case of diffuse mesangial sclerosis (DMS) associated with a number of undescribed congenital anomalies is reported. The occurrence of additional anomalies, especially ocular anomalies, is a common finding in DMS. However, neither megalocornea, Dandy-Walker malformation, postaxial hexadactyly, rocker-bottom feet, nor atrial septal defect, as observed in our patient, has been reported previously in association with DMS. This case might be considered an atypical manifestation of the Galloway-Mowat syndrome. In contrast to most cases of DMS, the patient revealed intrauterine proteinuria as the placenta was enlarged to 31% of birth weight. This case demonstrates that the large placenta, > 25% of birth weight, is not only pathognomonic of the congenital nephrotic syndrome of the Finnish type but can also occur in DMS.