Sarcoidosis in children. Clinical manifestations, epidemiology, treatment and prognosis

Sarcoidosis is a granulomatous disease of unknown aetiology, which may affect various organs. The diagnosis is obtained by the demonstration of epytheloid cell granulomas in an affected organ. The incidence of sarcoidosis in Danish children less than 15 years of age is 0.22-0.27/100.000 children/year, corresponding to approximately three new cases in Denmark each year. The disease often takes an asymptomatic course. During the period 1980-1992, three cases of paediatric sarcoidosis were observed in Copenhagen Country. All three had pulmonary involvement, and one had severe hypercalcaemia. In children less than five years of age, the disease is characterized by involvement of lungs, lymph nodes and eyes. Treatment, which is symptomatic, consists of systemic steroids. Due to the risk of growth retardation, the indication for treatment should be carefully considered and steroids administered at the lowest effective dose. Due to the lack of follow-up studies, the long term prognosis is unclarified.     

Germ cell tumors: staging, prognosis, and outcome

Germ cell tumors (GCT) remain the model for solid tumor therapy. Until 1997, GCT staging was based on individual institution systems, which limited comparison of data and collaboration between GCT groups. GCT staging is based on four basic criteria: disease site of origin, histology, secretion of serum tumor markers (STM), and bulk of disease. Within most staging systems developed by investigators, clinical stage I disease is confined to the testis based on radiographic imaging and STM or pathological stage I based on lack of histological disease at retroperitoneal lymphadenectomy. Stages II and III are considered to be disease outside the testis categorized by lymphatic spread to the retroperitoneal lymph nodes or hematological spread to lungs and visceral organs, respectively. The major staging systems previously used include the Indiana University Staging System; Modified Samuels' Classification (M.D. Anderson Cancer Center); Memorial Sloan Kettering Cancer Center Mathematical Model; and the Tumor, Nodal, Metastases (TNM) Staging System (American Joint Committee on Cancer). The most recent evolution in staging systems is the 1997 International Germ Cell Consensus Classification, which is based on prognosis and outcomes. This system allows for comparison of data and collaboration between Germ Cell Tumor Groups.     

Changing prognosis of primary liver cancer: some aspects to improve long-term survival

The following steps are essential in promoting a successful transition. (1) General condition of the patient must be stable. (2) Both parents must give consent to home care. (3) One parent at least must be thoroughly trained in all aspects of care. (4) Medical supplies must be kept in the home, and home modification to provide easy access and movement should be done as much as possible. (5) A medical support system must be established; home visiting nurses must be provided; medical doctors in the neighborhood and the local ambulance dispatch center must be notified of the patient's condition in case of an emergency.     

Brain tumors with symptomatic onset in the first two years of life

Eighty children who in the first 2 years of life had signs and symptoms relating to a cerebral neoplasm were studied over an 18-year period (1970-1987), the mean follow-up being 8.2 years. In each case age at onset, clinical presentation, tumor location and pathological diagnosis, extent of surgical resection, postoperative mortality, adjuvant therapy length of survival and quality of life were assessed. Supratentorial tumors (59%) were more common than infratentorial. The most frequent clinical presenting feature (70%) was increased intracranial pressure. Sixty-three patients (79%) were operated on and in all of these cases a histological diagnosis was obtained. Astrocytomas (41%) and medulloblastomas (20%) were the most common oncotypes. Surgical mortality was 17.4% and the 5-year survival rate was 54%. Quality of life was assessed for all long-term surviving patients using a specifically designed protocol. Normal physical and intellectual performances were found in 46% of cases, and all together 75% of the patients had sufficient autonomy in daily life. The prognosis is more closely related to tumor location and type of treatment than to histological diagnosis or age at onset.     

Sarcoidosis in children. Epidemiology in Danes, clinical features, diagnosis, treatment and prognosis

This paper reviews current knowledge of childhood sarcoidosis with regard to the epidemiology in Danes, clinical presentation, diagnostic procedures, treatment and prognosis. Sarcoidosis is a granulomatous disease of unknown aetiology, with multiorgan involvement. The diagnosis is confirmed by the demonstration of epitheloid cell granulomas in tissue biopsy specimens. During the period 1980-92, three cases of childhood sarcoidosis were recorded in Copenhagen County, which has a total population of 610,000. The approximate incidence of clinically recognized sarcoidosis in Danish children younger than 15 y of age was 0.22-0.27/100,000 children per year, corresponding to approximately three new cases in Denmark each year. The true incidence is unknown, since the disease is often asymptomatic and resolves without a clinical diagnosis being made. In children younger than 5 y of age, the disease is characterized by involvement of skin, eyes and joints, whereas in older children involvement of lungs, lymph nodes and eyes predominate. The mainstay of treatment consists of oral corticosteroids. The risk/benefit ratio of using long-term corticosteroids needs to be evaluated in each individual patient. Some patients may benefit from additional therapy with methotrexate. The long-term prognosis is not well established, but it seems to be poorer in children younger than 5 y. Older children appear to have as favourable a prognosis as young adults.     

Clinical course, diagnosis and treatment of ovarian tumors and cysts in children

The experience of diagnosis and treatment of the ovarial tumor and cyst in 68 girls was summarized. Children were admitted to the hospital with an acute abdomen signs and in a planned order as well. All the patients were operated on. Children with benign ovarial tumor and cyst are alive. Four patients with malignancy have died.     

Peritoneal dialysis in children under 5 years of age

The aim of this study was to examine the effect of intensive practice in table tennis on perceptual coincident timing. The main question was whether the perceptual demands encountered in fast ball sports produce modifications of the perceptual visual system. Expert table tennis players and novices were compared in a perceptual task which consisted of estimating, by pressing a key, the arrival of a moving stimulus at a target. The stimulus, which was presented either at constant velocity or at constant deceleration, reproduced as closely as possible the natural visual demands encountered in table tennis. The difference between the time of response and the time of arrival of the stimulus at a target position was measured over 40 trials for each of the 16 participants. The results showed no effect of expertise under the constant-velocity condition but an effect under the decelerative condition, indicating that experts were less trajectory-dependent than novices. This result was interpreted as reflecting a better adaptation of the perceptual system of experts to the constraints encountered during table tennis and specifically to the perceptual demands resulting from varied and decelerated ball trajectories. Finally, some limitations of the coincidence anticipation procedure are highlighted, concerning its use in practical settings for evaluating athletes or detecting sport talents, and the need for the simulation conditions during testing to reproduce as closely as possible the perceptual demands of real life is discussed.     

Ewing's family of tumors in adults: multivariate analysis of survival and long-term results of multimodality therapy in 182 patients

PURPOSE: To assess the outcome and the prognosis of adults with a neoplasm related to the Ewing's sarcoma family of tumors. PATIENTS AND METHODS: The outcomes of 182 consecutive patients older than 15 years with Ewing's sarcoma or related neoplasms managed from 1982 to 1992 were reviewed, without any selection according to primary tumor site or disease extension. RESULTS: Of 182 patients, 53 had evidence of metastases at presentation (29%). Tumor size was greater than 10 cm in 70 patients (41%). With a median follow-up duration of 66 months, the 5-year overall survival (OS) rate was 41%. In patients with localized disease, 5-year OS rate was 54% and 5-year progression-free survival (PFS) rate, 43%. Late relapses after 5 years accounted for 9% of relapses. Metastasis at presentation (P = .00001), pelvic primary lesion (P = .0025), and tumor size greater than 10 cm (P = .004) were independent prognostic factors for survival. Five-year OS was 67% in patients with nonpelvic tumors < or = 10 cm, 52% in those with pelvic tumors less than 10 cm or extrapelvic tumors > or = 10 cm, 16% in those with pelvic tumors greater than 10 cm, and 9% in those with metastasis (P = .00001). CONCLUSION: Based on our experience and a review of the literature, we concluded that the natural history and the prognosis of the Ewing's family of tumors in adults are not different from that found in children. A greater tumor bulk in adults may explain the less favorable prognosis previously reported by others. Outcome could be adequately monitored by a simple prognostic index.     

Prognosis of brain tumors: epidemiology, survival time and clinical course

The purpose of this study is to investigate the thermophysiological significance of hydrophilic and hydrophobic properties of underwear materials under the influences of profuse sweating produced during severe exercise in the cold. Two kinds of underwear were used: two layers of cotton underwear with two-piece long-sleeved shirt and full-trousers (C), and two layers of polypropylene underwear with two-piece long-sleeved shirt and full-trousers (P). In addition, the subject put on a two-piece ski suit of 100% polyester including 100% polyester padding. Eight adult females volunteered as subjects in this study. The test was performed in a climatic chamber at an ambient air temperature of 2 degrees C and an air velocity of 0.26 m.s-1. The subject exercised on a cycle ergometer at an intensity of 65% maximal oxygen uptake for 30 min and followed by 60 min recovery. The major findings are summarized as follows: 1) The fall of rectal temperature tended to be greater in P during the recovery. 2) The absolute humidity of innermost layer and middle layer was significantly higher in C than in P during the recovery, but the absolute humidity of middle layer and outermost layer was significantly higher in P than in C during the exercise. 3) Clothing microclimate temperature of innermost at back was significantly higher in C during the exercise and recovery. 4) Metabolic heat production for last 30 min during recovery was significantly higher in P. 5) The degree of skin wettedness sensation and sweating sensation for whole body was significantly higher in P during the exercise. It was concluded that the slower evaporation behavior by absorbing of underwear material in the clothing system has a beneficial influence on thermophysiological responses during severe exercise and its recovery in the cold, although the differences were very small.     

Setback phenomenon in autism and long-term prognosis

Early effective management of Pneumocystis carinii pneumonia improves outcome in patients with this disorder. Trimethoprim-sulfamethoxazole remains the agent of choice for treatment of severe P. carinii pneumonia. Pentamidine, trimethoprim-dapsone, atovaquone, and other regimens are useful in selected clinical situations. Adjunctive corticosteroids are indicated in patients with acquired immune deficiency syndrome and P. carinii pneumonia who have moderate to severe P. carinii pneumonia defined as a room air arterial PaO2 less than 70 mm Hg or an alveolar-arterial oxygen gradient of greater than 35 mm Hg. The use of trimethoprim-sulfamethoxazole, dapsone, and aerosolized pentamidine in immunocompromised patients without AIDS is also reviewed.     

Discrimination of syntactical errors in children under two and one-half years.

Tested 32 8-30 mo old children who spoke in either 1- or 2-word utterances for their ability to discriminate between complete grammatical and ungrammatical sentences. The measure of discrimination used was children's listening preferences. Results show that both types of children can discriminate, and illustrate the extent of the lag between children's acquisition of syntax and their use of that syntax in free production. Preferences may provide a methodology for studying the acquisition of syntax independent of production skills. (17 ref) (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Psychiatric interventions in children under five years of age: prevention or care?

We analysed the consultations of children under the age of 5 attending our pedopsychiatric outpatients department over a period of 1 year. Although paediatricians and pedopsychiatrists agree on the necessity to start therapy as early as possible, we found that many children were seen to late, at a time when they had difficulties, and that the principle of early intervention in pedopsychiatric needs to be better applied during infancy in an effort to prevent psychological disturbances.     

Antiaggregatory effects of picotamide in long-term treatment: a 2-year, double-blind placebo-controlled trial

With faster CT scanners, asymmetric/heterogeneous enhancement (ASHE) of the internal jugular veins (IJVs) is frequently encountered in the absence of pathology. We investigated the frequency, side, pattern and significance of ASHE in 200 patients with various head and neck lesions. Non-ionic contrast medium (300 mgI/ml) was infused into a forearm vein (right, n = 100); left, n = 100). Forty seconds after contrast medium injection, contiguous 5-mm-thick sections were obtained craniocaudally from the skull base to the aortic arch. CT machines with two different scanning cycle times (3 s, n = 100; 2 s, n = 100) were used. ASHE of the IJVs was observed in 51 patients (25.5%); the patient group receiving a right-sided injection with a 2-s scan cycle machine, showing a higher frequency (44%). ASHE was divided into four patterns: homogeneous low density, focal low density, heterogenous opacification and fluid-fluid level formation. ASHE was frequently observed in routine contrast-enhanced CT of the head and neck. Frequency of ASHE increased when the scanning cycle was shorter. We should be aware of this phenomenon to avoid its misinterpretation as venous thrombosis or other pathology.     

Augmentation of nutritional intakes in children receiving long-term hemodialysis treatment

One of the major problems confronting children on long-term hemodialysis treatment is an inadequate intake of nutrients. The intake of calories, protein and minerals in children dialyzed for up to 3 years was documented in this study to be low. A simple method of augmenting the nutritional intake that has wide patient acceptibility is presented.     

The long-term course of severe anorexia nervosa in adolescents: survival analysis of recovery, relapse, and outcome predictors over 10-15 years in a prospective study

OBJECTIVE: To assess the long-term course of recovery and relapse and predictors of outcome in anorexia nervosa. METHOD: A naturalistic, longitudinal prospective design was used to assess recovery and relapse in patients ascertained through a university-based specialty treatment program. Patients were assessed semiannually for 5 years and annually thereafter over 10-15 years from the time of their index admission. Recovery was defined in terms of varying levels of symptom remission maintained for no fewer than 8 consecutive weeks. RESULTS: Nearly 30% of patients had relapses following hospital discharge, prior to clinical recovery. However, most patients were weight recovered and menstruating regularly by the end of follow-up, with nearly 76% of the cohort meeting criteria for full recovery. Relapse after recovery was relatively uncommon. Of note, time to recovery was protracted, ranging from 57-79 months depending on definition of recovery. Among restrictors at intake, nearly 30% developed binge eating, occurring within 5 years of intake. A variety of predictors of chronic outcome and binge eating were identified. There were no deaths in the cohort. CONCLUSION: The course of anorexia nervosa is protracted. Predictors of outcome are surprisingly few, but those identified are in keeping with previous accounts. The intensive treatment received by these patients may account for the lower levels of morbidity and mortality when considered in relation to other reports in the follow-up literature.