Recurrent parotid swelling in children: clinical features useful for differential diagnosis of Sjögren''s syndrome

Immunological evaluations were performed in 59 children with at least five episodes of parotid swelling. Autoantibody(ies) was transiently or persistently detected in 12 (20%) of 59 patients with recurrent parotitis. Three of the 12 children with autoantibodies were diagnosed as having Sj?gren's syndrome. The mean age at onset of parotid swelling in Sj?gren's syndrome was significantly higher than that of recurrent parotitis of unknown etiology. The present study and the review of the literature suggest that patients with the onset of parotid swelling at age five years or over deserve screening for underlying systemic immune disorders.     

New insights into juvenile parotitis

AIM: We inquired about the possibility of a familial trend in juvenile parotitis and evaluated the role of SPINK1 mutations in juvenile parotitis. METHODS: The clinical records of all children admitted to the Helsinki University Hospital during 1995 to May 2003 because of swelling in the parotid gland were reviewed. A questionnaire on possible recurrences and on familial cases was mailed. As disturbances in trypsin inhibition might be involved in the pathogenesis, we assessed the SPINK1 gene encoding for Kazal-type trypsin inhibitor in voluntary patients. The study group comprised 133 children (boys 82 girls 51) with juvenile parotitis. The median age at presentation of first symptoms was 6.0 y (range 1-19 y). RESULTS: Recurrent symptoms in the parotid gland were common (57%), and 29% of the children (38/133) had suffered from four or more episodes. A young age at the first episode of symptoms increased the likelihood of recurrences (p<0.0001). Familial cases of parotid swelling were common (22%; response rate 67%). A total of 47 patients (35%) agreed to testing for SPINK1 status. Four children had a major mutation (N34S or P55S), corresponding to an 8.5% (4/47) prevalence, but this was not different from the controls (5%).CONCLUSION: It is likely that inherited factors are involved in the manifestation of juvenile parotitis in a subset of patients. It is tempting to speculate that disturbed proteolytic balance may play a role in the development of symptoms.     

Primary Gougerot-Sj?gren syndrome in a 13-year-old girl]

Sj?gren's syndrome is uncommon in children, and occurs most often in association with autoimmune diseases (secondary Sj?gren's syndrome). We describe the clinical and biological features of a 13-year-old girl with primary Sj?gren's syndrome, revealed by recurrent parotitis. CASE REPORT: This adolescent girl was referred for investigation of multiple episodes of bilateral parotid swelling since age nine, without systemic symptoms. Examination was unremarkable except for enlarged and painless parotid glands. Laboratory investigations, measurement of saliva production, parotid sialography, labial salivary gland biopsy, revealed Sj?gren's syndrome without associated disease. Hydroxychloroquine was prescribed with clinical improvement. CONCLUSION: Recurrent parotitis in children is an uncommon condition. The onset of parotid swelling at five years or over deserves screening for dysimmune disorders, sarcoidosis or Sj?gren's syndrome. Diagnosis of Sj?gren's syndrome is based on laboratory evidence of autoimmune disorders and minor salivary gland biopsy.     

Selective IgA deficiency in children with recurrent parotitis of childhood

Recurrent parotitis of childhood is defined as the relapsing form of juvenile (idiopathic) parotitis and represents a rare inflammatory disorder of the parotid gland with potentially significant morbidity. We reviewed the charts of patients who were diagnosed with inflammatory parotid diseases in our institution between 1992 and 2002. There were 91 patients presenting with juvenile parotitis (1 of 6117 of all clinical visits). Of these 91 cases, 23 patients (28%) had the relapsing form of juvenile parotitis, and the median number of episodes was 5 (range, 2-20). Laboratory investigations revealed that 5 patients had selective IgA deficiency. The prevalence (22%) is different from the cumulative prevalence of IgA deficiency in a healthy population (0.3%; P < 0.001).     

Recurrent parotitis in selective IgA deficiency

Recurrent parotitis is an uncommon condition in children. In most cases the etiology is unknown, although the disease is occasionally associated with viral infections, autoimmune disorders and immunodeficiency. We describe, for the first time, a child with recurrent parotitis and isolated immunoglobulin A (IgA) deficiency, without autoimmune disease. As IgA is the main immunoglobulin secreted into the mucosal surfaces, including that of the respiratory and gastrointestinal tracts, and into the saliva, the lack of IgA may be involved in the pathogenesis of recurrent parotitis. We recommend that IgA and other immunoglobulins be tested in all cases of recurrent parotitis.     

A child with primary Sjögren syndrome and a review of the literature

Primary Sj?gren syndrome (pSS) is an uncommon disease in childhood. Childhood pSS might have different clinical manifestations than adult pSS. We describe a 13-year-old girl with multiple episodes of bilateral parotid swelling lasting 2 years. Her history included severe arthralgia, local edema, and purpura episodes since 9 years of age. During her 3-week hospitalization, 2 episodes of parotid swelling occurred, which both resolved in 48 hours. Ultrasonography and magnetic resonance images of parotid glands showed parenchymal inhomogeneity related to adipose degeneration and nodular pattern. Investigations showed elevated erythrocyte sedimentation rate, the presence of hypergammaglobulinemia, positive antinuclear antibody, and elevated rheumatoid factor, anti-Sj?gren syndrome antigen A, and anti-Sj?gren syndrome antigen B. Histopathologic examination of labial minor salivary glands revealed focal periductal lymphocytic infiltrate and sialoduct ectasia. She was diagnosed as having pSS. Recurrent parotid swelling is a more characteristic feature of disease in children, and this finding should alert the clinician to the possible diagnosis of pSS.     

儿童复发性多软骨炎三例及文献复习

目的 分析儿童复发性多软骨炎(Relapsing Polychondritis,RP)的临床特点.方法 回顾性分析我院3例儿童RP临床特征及诊治,在医学引文索引(Medline)检索到16例RP进行文献复习,并与成人RP进行比较.结果 3例儿童RP发病年龄为10～15岁(文献报道儿童RP发病年龄最小为2岁),从出现症状到确诊时间为3～9个月(文献报道为3个月～2年).3例儿童RP临床主要表现为关节软骨炎、耳软骨炎、鼻软骨炎、眼结膜炎等,3例患儿均因累及咽喉部引起严重呼吸困难而行气管切开术.与成人RP相比,累及呼吸系统的为78.9%,较成人(35.2%)更为常见和严重;关节炎是儿童RP最常见的临床表现和首发症状;较少合并其他自身免疫病.无特异的实验室诊断指标.2例依靠临床标准而明确诊断,1例有组织病理的支持而确诊.呼吸道CT及其三维重建有利于RP的早期诊断.糖皮质激素(尤其是甲泼尼龙)能控制患儿的病情,但容易反复,重组人Ⅱ型肿瘤环死因子受体-抗体融合蛋白成功治疗1例儿童难治性RP.结论 详细的病史、全面的体检以及必要的辅助检查(如:呼吸道CT检查等)有助于减少误诊、明确诊断.组织病理对疾病的诊断非常重要但并非必需.严重呼吸困难时应及时行气管切开术.正确诊断、早期治疗是改善预后的关键.糖皮质激素及免疫抑制剂对儿童RP治疗有效,但不持久,重组人Ⅱ型肿瘤坏死因子受体-抗体融合蛋白可能是治疗儿童难治性RP的一种有益的探索和尝试.     

Cognitive function in Type 1 diabetic children with and without episodes of severe hypoglycaemia

We assessed the effect of diabetes and of episodes of severe hypoglycaemia on cognitive function in 28 diabetic children. Fifteen diabetic children (age 12.9 (SD 2.0) years) had experienced 1–4 episodes of severe hypoglycaemia. Five of these children diseased before the age of 5 years (SH-eod subgroup), and ten diseased after this age (SH-lod subgroup). Thirteen diabetic children (age 13.1 (SD 2.0) years) had not experienced episodes of severe hypoglycaemia (non-SH group). Each diabetic child was compared with a healthy control child of the same age and gender and with a similar social background. Neuropsychological assessment was blinded. The neuropsychological tests were grouped into one of seven cognitive domains. We found no effect on cognitive performance from diabetes per se or from severe hypoglycaemia in children with late-onset diabetes. However, early-onset diabetes was associated with low scores in two cognitive domains: psychomotor efficiency and attention. The SH-eod subgroup had lower scores than the SH-lod subgroup in psychomotor efficiency ( p < 0.05) and also had lower scores than the SH-lod subgroup and the non-SH group in measures of attention ( p < 0.05). Our results may indicate a slight cognitive dysfunction in children with early-onset diabetes who have experienced episodes of severe hypoglycaemia early in childhood.     

Recurrent limb pain in schoolchildren

OBJECTIVES: To determine the prevalence, causes and clinical features of short lasting recurrent limb pain (recurrent limb pain) in children. DESIGN: Population-based study in two stages, with an initial screening questionnaire followed by clinical interviews and physical examination of symptomatic children. SETTING: 67 primary and secondary schools in the city of Aberdeen. SUBJECTS: 2165 children representing a random 10% sample of all schoolchildren aged between 5-15 years. MAIN OUTCOME MEASURES: (a) The causes of limb pain in children, (b) the prevalence of recurrent limb pain in schoolchildren, (c) the relationship of recurrent limb pain to childhood migraine. RESULTS: Sports and playground injuries were the most common cause of limb pain, affecting 9% of all children. The prevalence rate of recurrent limb pain was 2.6% (95% confidence interval 1.9 to 3.4). Episodes of recurrent limb pain had similar trigger factors, associated symptoms, and relieving factors to episodes of headache in children with migraine. CONCLUSIONS: Recurrent limb pain is a common cause of limb pain, with a prevalence rate of 2.6%. The close clinical and epidemiological similarities between recurrent limb pain and childhood migraine suggest a common pathogenesis.     

Recurrent limb pain in schoolchildren.

OBJECTIVES: To determine the prevalence, causes and clinical features of short lasting recurrent limb pain (recurrent limb pain) in children. DESIGN: Population-based study in two stages, with an initial screening questionnaire followed by clinical interviews and physical examination of symptomatic children. SETTING: 67 primary and secondary schools in the city of Aberdeen. SUBJECTS: 2165 children representing a random 10% sample of all schoolchildren aged between 5-15 years. MAIN OUTCOME MEASURES: (a) The causes of limb pain in children, (b) the prevalence of recurrent limb pain in schoolchildren, (c) the relationship of recurrent limb pain to childhood migraine. RESULTS: Sports and playground injuries were the most common cause of limb pain, affecting 9% of all children. The prevalence rate of recurrent limb pain was 2.6% (95% confidence interval 1.9 to 3.4). Episodes of recurrent limb pain had similar trigger factors, associated symptoms, and relieving factors to episodes of headache in children with migraine. CONCLUSIONS: Recurrent limb pain is a common cause of limb pain, with a prevalence rate of 2.6%. The close clinical and epidemiological similarities between recurrent limb pain and childhood migraine suggest a common pathogenesis.     

Clinical and microbiological analysis of six children with acute suppurative parotitis

Six children including two neonates with acute suppurative parotitis are described. They presented typical symptoms and signs of fever (4/6) and swelling (6/6), tenderness (6/6), erythema (4/6) and local warmness (4/6) of the parotid gland affected. Leucocytosis and an elevated erythrocyte sedimentation rate and serum amylase level may be seen. The presence of purulent discharge from the Stensen duct when the parotid gland is externally compressed is pathognomonic of the disease, and the microbiological diagnosis can be made by culture of the pus. Microbiologically, this series highlights the polymicrobic nature and importance of Streptococcus viridans in paediatric suppurative parotitis, indicating, therefore, that the causative bacteria entered the gland from the oral cavity. Our data also suggest that initial antibiotic therapy for such patients should provide adequate coverage for streptococci and staphylococci as well as for anaerobic bacteria.     

The Natural History of Childhood Asthma Through Adolescence

A follow-up of the natural history of childhood asthma at 17-18 years in a randomly selected population of asthmatic and control children who had been studied clinically and physiologically at 7, 10 and 14 years of age, has been conducted by questionnaire. Of the sample still in the study at 14 years of age, 77% of the asthma population replied, representing 65% of the original sample. There had been significant amelioration of asthma in those children who still had asthma at 14 years of age. Of those children with episodic asthma, approximately half had ceased wheezing while the remainder had relatively infrequent episodes. Those children with chronic asthma continued to wheeze but in just over half, asthma appeared to be episodic. Of the children who had ceased wheezing before 14 years of age, 17% had recurrence in the 12 months prior to review. However, in only 1 subject were the episodes of asthma frequent.     

Effects of nutritional status on diarrhea in Peruvian children

OBJECTIVES: We conducted a 4-year (1995-1998) field study in a Peruvian peri-urban community (pueblo joven) to examine the relation between diarrhea and nutritional status in 230 children <3 years of age. METHODS: We followed the birth cohort daily for diarrhea and monthly for anthropometry. We modeled diarrheal incidence with a multivariate time-to-event regression model to account for multiple episodes per child and irregular follow-up periods and diarrheal duration with a mixed-effects gamma regression model to account for disease heterogeneity across children. RESULTS: During 159,551 child-days of follow-up, we identified 1387 diarrheal episodes, which yielded an average incidence of 3.2 episodes per child-year. Diarrhea was seasonal, for example, infants had up to 8 diarrheal episodes during the summer; however, these variations decreased noticeably with age. Nutritional status was significantly associated with diarrheal incidence. The frequency of diarrhea increased by 15% per standard deviation decrease in height-for-age z score. Diarrheal episodes in children <6 months of age lasted significantly longer than episodes among older children. CONCLUSIONS: These results identify infants and children of poor nutritional status as priority risk groups for prevention efforts aimed at reducing the burden of acute childhood diarrhea.     

The acute scrotum in Arab children with familial Mediterranean fever

 Over a period of 7 years, among 175 boys under the age of 16 years with familial Mediterranean fever (FMF), 16 (9%) developed 28 episodes of scrotal swelling that was unilateral in 26 (93%) and bilateral in 2 (7%). Fever and pain were present in 15 (94%) children; fever was characterized by a gradual onset and pain was moderate in intensity. The episodes were self-limiting and lasted from 8 h to 5 days. Scrotal swelling was the presenting feature of FMF in 4 (25%) patients. Six (38%) children underwent surgery; the operative findings, available in 3, showed a normal testis and epididymis and inflammation of the tunica vaginalis. The self-limiting nature of the episodes lasting for a few days was similar to the clinical course of serositis seen in FMF. This strongly suggests that inflammation of the tunica vaginalis, resulting in scrotal swelling, is another feature of FMF serositis. The gradual onset of fever, pain, swelling, and recurrence in a boy of Mediterranean origin, especially in the presence of a relevant family history, strongly points toward the diagnosis of FMF and conservative management. Early diagnosis and prophylactic colchicine therapy are expected to avert recurrences, which may result in ischemic testicular necrosis and FMF nephropathy. Accepted: 4 September 1998     

Clinical, histopathologic and immunohistochemical studies of chronic sialectatic parotitis in childhood and adolescence]

Chronic sialectatic parotitis (CSP) causes problems in differential diagnosis and therapy. CSP shows the typical clinical features of chronic recurrent parotitis and will be investigated histopathologically only after ultimative parotidectomy. The etiology and pathogenesis of these unspecific inflammations is still unknown. Therefore no causal therapy is available and a lot of different trials (sialogoga, gland massage, infrared light, antibiotics, antiphlogistics, Trasylol, duct occlusion, duct ligation, gland denervation, radiotherapy) are not successful in the long run. MATERIAL AND METHOD: The salivary gland registry of the University of Hamburg (1965-1996) contains 22 infants and juvenile patients showing very severe courses of CSP. These cases have been investigated clinical (ultrasound, sialography), histopathological (paraffin embedded sections, histomorphometry of the ectatic duct lumina) and immunohistochemical (CK-MNF, AKTIN, KiM4) in a retrospective study to research the pathogenesis of CSP. RESULTS: Recurrent and always very dolent parotid swelling occurs between the age of 3 and 14 years for the first time. The courses vary from 3 months until 25 years. Local findings as well as ultrasound and sialographic features allow no certain differentiation of chronic recurrent parotitis. Conservative therapy fails in each case and leads to the necessity of surgical treatment. Histopathological three different stages of development can be observed: Initial stages show regular lobular architectonic structure of the parotid gland parenchyme with duct ectasies surrounded by slight inflammation of lymphocytes and plasmacells. Advanced stages are characterized by an increase of periductal inflammation and the appearance of lymphfollicels. Nearly complete lymphatic transformation of the parenchyme with destruction of the lobular formation dominates the terminal "immunologic" stage. Some cases show multiple myoepithelial islands within this lymphatic stroma typically observed in benign lymphoepithelial lesions. Whether bacteria nor primary obstructive changes can be observed. The histomorphometric analyses of the average and maximal luminal duct diameters show marked increase of 39% respectively 46% from and- vanced to terminal stages of CSP. Therefore the pathognomonic duct ectasies seem to depend on the progredient inflammation and are not due to a hereditary malformation of the duct system. Immunohistochemical terminal stages show follicular lymphatic hyperplasia (KiM4) expressing overshooting humoral immune reaction of MALT. CONCLUSION: Concerning the pathogenesis CSP corresponds to a immunopathological disorder of MALT and seems to be a prestage of benign lymphoepithelial lesion. Consequently important changes in the diagnosis and therapy of CSP lead to early histopathological investigation to differentiate the stage of inflammation. In stage III conservative parotidectomy should be carried out because spontaneous healing can not be expected. In contrast initial cases should be treated at first by glucocorticoids and immunosuppressives.     

Chronic non-specific diarrhoea.

Recurrent, unexplained diarrhoea is the most common intestinal complaint in children aged 6 months to 3 years. We studied 27 consecutive children with this complaint and followed them up until the age of 5 years. Diarrhoea began at the mean age of 9 months (range 4 to 16 months) and resolved in 21 children by 3 years of age. Twelve children had had infantile colic earlier. In six patients diarrhoea was caused by food allergy (cows'' milk allergy and allergy to fresh vegetables). Episodes of diarrhoea persisted in four of these six. Twenty one children had unexplained diarrhoea: this resolved in 19. Nutritional deficiencies were rare; only one child had iron deficiency. Relative weights of the children were significantly lower at 2 years than at 1 year of age. At 5 years of age six of the children continued to have episodes of diarrhoea, and abdominal pains, headaches, and atopy occurred more commonly than in the general population. We suggest that there are two major subgroups among children with recurrent diarrhoea--children with food allergy and those who react to environmental stresses with a variety of somatic symptoms.     

小儿阴囊急症的鉴别诊断和手术治疗

目的探讨小儿阴囊急症的鉴别诊断及治疗方法。方法总结分析我院近7年收治的小儿阴囊急症病例资料。结果133例阴囊急症中睾丸附件扭转105例(79.0%),睾丸附睾炎16例(12.0%),睾丸扭转4例(3.0%),其他8例(6.0%)。附件扭转以6～12岁居多,其阴囊肿痛程度较睾丸附睾炎为轻(P<0.005)。在睾丸附件扭转中蓝斑征9例(8.6%),痛性结节23例(21.9%),超声附件检出率为90.5%。手术中发现不完全性坏死附件、哑铃状附件节间扭转、慢性附件扭转等特殊病例。睾丸附睾炎好发于6岁内,可双侧发病或反复发作。睾丸扭转其提睾反射均消失,超声显示睾丸无血流。结论小儿阴囊急症以睾丸附件扭转最常见,睾丸扭转发病最急。病史、体征及超声检查有助于鉴别诊断,主张积极探查手术。     

The clinical picture of juvenile parotitis in a prospective setup

Aim: To characterize the features of juvenile parotitis in a prospective setup and epidemiology. Methods: All children with parotitis admitted to Helsinki University Central Hospital 2005–2010 were recruited. Clinical characteristics, given treatment, outcome, blood leukocyte count, C‐reactive protein, serum amylase and trypsinogen, SPINK‐1 genotype and mumps antibodies were recorded. To map the epidemiology, a questionnaire was sent to 1000 randomly selected 13‐year‐old children. Results: The prospective study included 41 children (aged ≤ 17) with acute parotitis, all in good general condition. Serum amylase, but not trypsinogen, was elevated in majority of the cases (79%) and C‐reactive protein in 68%. Eleven (27%) children had an elevated blood leukocyte count. None had acute mumps. Most children recovered well, 51% being treated symptomatically only. Seven children were treated on ward. Seventeen (46%) children had recurrent symptoms. One child (2.4%) had SPINK P55S mutation. According to the epidemiological questionnaire, 1.1% of the respondents (8/728, response rate 73%) reported a verified episode(s) of parotitis. Conclusion: Juvenile parotitis has a frequency close to 1%. In the majority, the general condition is good during the episode. Serum amylase serves as an additional marker for the disease. Parotitis has a tendency to recur in almost half of the cases.     

Is recurrent parotitis in childhood still an enigma? A pilot experience

AIM: To test the hypothesis that dental malocclusion with mandibular misplacement may be a causative factor for recurrent parotitis (RP) through unbalancing of masticatory muscles. METHODS: Thirteen patients (age 4-14 years) who were referred to a dental clinic for RP and malocclusion were treated by oral appliance positioning for a 6-month period. Monthly visits were scheduled regularly. RESULTS: Symptoms were clearly improved in nine children. No effect was obtained in three patients. One patient was lost at follow-up. CONCLUSION: Occlusal intervention is effective in patients with RP and associated malocclusion. It should be considered an important option for the treatment of such intriguing disorder.     

Hashimoto's thyroiditis in children and adolescents: a retrospective study on clinical, epidemiological and laboratory properties of the disease

Hashimoto's thyroiditis (HT) is the most common cause of goiter and acquired hypothyroidism in children and adolescents in iodine replete areas. To find out the clinical, epidemiological and laboratory characteristics of the disease in childhood, we reviewed files of 162 children and adolescents with HT followed in the Department of Pediatric Endocrinology, Hacettepe University Faculty of Medicine. RESULTS: Female patients constituted 86.4% (n = 140) of all patients with a female:male ratio of 6.4. Mean age at diagnosis was 11.4 +/- 2.97 years (age range 4.4-16.5 years). At the time of diagnosis 43.2% of the patients (n = 70) were euthyroid, 24.1% (n = 39) had subclinical hypothyroidism, 21% (n = 34) had overt hypothyroidism, and 8.6% (n = 14) had overt and 3.1% (n = 5) subclinical hyperthyroidism. CONCLUSIONS: Autoimmune thyroiditis is more frequent in females, and increases in frequency over age during childhood and adolescence. At the time of diagnosis, frequency of overt and subclinical hypothyroidism is similar to that of euthyroid goiter.