Primary skeletal non-Hodgkin's lymphoma in the pediatric age group

The authors discuss rare primary skeletal non-Hodgkin's lymphoma in 16 patients treated from 1973 to 1989. The symptoms of these patients related to bone lesions in 95% of the cases. These bone lesions were monostotic or polyostotic, with or without regional and distant metastases. The locations of these lesions were long bones in 13 patients, pelvic bones in seven patients, and skull and vertebral bodies in two patients. The anatomical locations of these lesions in the bones were diaphysis alone in one patient, epiphysis in two patients, metaphysis in three patients, and a combination of diaphyseal, epiphyseal, and metaphyseal lesions in seven patients. Extraskeletal involvement was present in nine patients; extraskeletal sites included regional or distant lymph node involvement in seven cases, the mediastinum in two, lung nodules in two patients, the skin and subcutaneous regions in four patients; bone marrow in three patients, and peripheral nervous system (PNS) in one patient. Two patients had stage I disease, three had stage II disease, eight had stage III disease, and three had stage IV disease. The majority of patients had large noncleaved cell diffuse lymphomas or DHL by Rappaport classification. All patients were treated with the LSA₂-L₂ protocol; six patients received radiation therapy to the affected bone, and ten patients received no radiation therapy. Three patients failed on treatment within the first 4 months of therapy. Two patients developed a second tumor, one in the radiation therapy field and the other in a patient who received no radiation therapy. Eleven patients are alive without evidence of disease, with a median observation time of 6.5 years. The event-free survival for the 16 patients receiving LSA₂-L₂ was 73%, and the lymphoma-free survival was 81%. Extension of disease to multiple sites or location of the primary site was not of prognostic significance, but the finding of central nervous system (CNS), PNS, or bone marrow disease at diagnosis was. Whether the skeletal lesions were uni- or multifocal, with regional or distant metastases, the prognosis for lymphoma-free survival was good. The role of radiation therapy for all patients and intensive treatment for earlystage disease is discussed. The authors conclude that chemotherapy is the most important modality of treatment for any stage, histology, or location of the tumor. This chemotherapy shou ld include drugs that have been shown to be effective in the treatment of pediatric lymphoma, such as cyclophosphamide, cytosine arabinoside, vincristine, methotrexate, and daunomycin, in dosages sufficient to produce marrow suppression and early recovery, allowing for continuous or intermittent therapy. Radiation therapy should be used if there is no response or progression of disease (demonstrated either clinically or by scans, confirmed by a biopsy) within the first few months of treatment. © 1992 Wiley-Liss, Inc.     

儿童非霍奇金淋巴瘤诊疗建议

儿童非霍奇金淋巴瘤(non-Hodgkin lymphomas,NHL)是源于淋巴系统器官和细胞的一系列疾病的总称,包括所有未归类于霍奇金病的恶性淋巴瘤.北美＜15岁的儿童NHL年发病率为8.3/百万.1992-1996年上海市肿瘤登记系统统计结果表明0～14岁组儿童NHL发病率为8.94/百万,仅次于白血病和颅内肿瘤.     

Wegener's granulomatosis in the pediatric age group.

Six cases of Wegener's granulomatosis (WG) occurring in patients younger than 21 years are described. Only 11 other cases in the pediatric age group have been reported, and all 17 of these patients had the onset of the disease during the second decade of life. Wegener's granulomatosis is a systemic disease characterized by a clinical triad of paranasal sinus and nasal mucosa involvement, pulmonary infiltration and cavitation, and renal disease with hematuria. The most common presenting symptoms are malaise and fever, sinusitis, epistaxis, and hematuria. Most patients have roentgenographic evidence of pulmonary and sinus disease and laboratory evidence of renal involvement on initial evaluation. The prognosis of WG was formerly dismal; more than 90% of patients died in less than two years, but with recent therapeutic regimens, more than 50% of these patients are surviving. The treatment we recommend consists of nitrogen mustard with adrenocorticotropic hormone or prednisone for the induction of remission, followed by cyclophosphamide and prednisone as maintenance drugs. This regimen has proved effective in inducing a remission in four of four patients.     

Self-inflicted ocular mutilation in the pediatric age group

Three mentally retarded children with severe self-inflicted ocular injuries are presented. All three suffered from severe ocular injuries including retinal detachment resulting in progressive visual loss and even blindness. Self-inflicted injuries to the eyes, including self enucleation, is an extremely uncommon form of behavior, rarely encountered by pediatricians. The risk of ocular morbidity is high if the diagnosis is overlooked. Technical advances in ophthalmology permit much improvement in some formerly hopeless cases of ocular self-mutilation, but there is still no accurate method to repair destroyed retinal or nervous tissue. Early identification of patients at risk of ocular self-mutilation is essential in order to prevent or minimize such severe ocular injuries.     

Primary subconjunctival lymphoma: an unusual presentation of childhood non-Hodgkin's lymphoma

Of 806 non-Hodgkin's lymphoma cases, only one primary subconjunctival lymphoma case was observed in Hacettepe Children's Hospital over a period of 16 years. The patient was a 12 year-old-boy with a subconjunctival mass on the upper part of his right eye. Biopsy revealed malignant lymphoma. Extensive investigations disclosed no other site of tumor. He was given the modified LSA2-L2 protocol. He has been in remission for 22 months. This may be the first report of a primary subconjunctival lymphoma in the childhood period.     

Measurement of severity for nonhospitalized injuries in the pediatric age group.

To better classify minor injuries in the pediatric outpatient population, we empirically developed a scale to predict functional impairment, one week and one month postinjury, based on data available at the time of care. A sample of 857 injured children aged five to 19 years was randomly split into two subsamples. Using logistic regression techniques, predictive models were developed and confirmed on the subsamples and then applied to the full sample. Age, location of injury, and type of injury were predictive of impairment one week post-injury; however, type of injury was the only consistently significant predictor. Type of injury, specifically fracture, dislocation, and sprain, and location of injury (lower extremity) were significant predictors of functional impairment one month postinjury; however, once again, the type of injury was the only variable consistently significant across all models. Other than the type of injury, the variables available at the time of care were too uniform between disabled and nondisabled patients to be useful in developing a severity scale for minor injuries. We conclude that other outcome measurements or scale development techniques should be explored to develop a useful classification scheme for minor injuries.     

Diagnostic and therapeutic ERCP in the pediatric age group

The role and value of endoscopic retrograde cholangiopancreatography (ERCP) in the pediatric age group is not well established, because pancreatic and biliary diseases are less common in children. This however is not the case in areas like the Eastern Province of Saudi Arabia where sickle cell disease (SCD) and other hemoglobinopathies are common, with increased frequency of cholelithiasis and choledocholithiasis. The purpose of this study was to evaluate the indications, findings, safety and therapies of ERCP in children. One hundred and twenty five children had diagnostic and/or therapeutic ERCP as part of their management at our hospital. Their medical records were reviewed for: age at diagnosis, sex, Hb electrophoresis, indication for ERCP, findings, therapy and complications. There were 77 males and 48 females. Their age at presentation ranged from 5–18 year (mean 13.25 year). The majority of them had sickle cell disease (77.6%). The indications for ERCP were: obstructive jaundice (67.2%), recurrent biliary colic with or without jaundice (10.4%), acute and chronic pancreatitis (7.2%), postoperative bile leak (2.4%), cholangitis with obstructive jaundice (2.4%), hepatitis of unknown etiology (3.2%), cirrhosis of unknown etiology (4%), thalassemia with jaundice (0.8%), hemobilia (0.8%), acute cholecystitis with jaundice (0.8%), and sickle cell disease with ulcerative colitis and obstructive jaundice (0.8%). In six children, ERCP was done following laparoscopic cholecystectomy. ERCP was carried out under sedation in 91 (72.8%) children and under general anesthesia in 34. It was successful in 121 (96.8%) children while cannulation of the Ampulla failed in four. ERCP was normal in 43 children, but eight of them showed evidence of recent stone passage and in six, there were gallstones. In the remaining children, ERCP revealed: normal CBD with stones (18 patients), dilated CBD with stones (17 patients), dilated CBD without stones (19 patients), dilated biliary tree with stones (10 patients), dilated biliary tree without stones (six patients), bile leak (two patients), dilated biliary tree with stones and choledocho-duodenal fistula (one patient), choledochal cyst (two patients), septate gallbladder (one patient), normal ERCP with multiple pancreatic cysts (one patient) and biliary stricture (one patient). The following procedures were carried out: 35 had endoscopic sphincterotomy and stone extraction, 20 had endoscopic sphincterotomy, four had CBD stenting, one underwent removal of a stent, two had insertion of a nasobiliary tube and one had biliary endoprosethesis. There was no mortality. One had bleeding from the site of sphincterotomy which stopped after adrenaline injection. Four patients (3.2%) developed transient mild pancreatitis which settled conservatively. ERCP in the pediatric age group is safe both as a diagnostic and therapeutic procedure. ERCP can provide valuable information which aid in the diagnosis of biliary and pancreatic diseases in children as well as therapy with the technical feasibility of endoscopic sphincterotomy. This is specially so in the era of laparoscopic cholecystectomy, where ERCP should be the treatment of choice in children with CBD stones who are going or have previously undergone laparoscopic cholecystectomy.     

Type 2 diabetes mellitus in the pediatric age group

In the past two decades, type 2 diabetes mellitus has increased in children and adolescents, especially within certain ethnic groups. This increase has been parallel to the rising prevalence of obesity. Because of the overlap between some clinical characteristics, the differential diagnosis between type 1 and 2 diabetes is difficult. Of 300 diabetic patients in our diabetes section, only three (1 %) had type 2 diabetes. Two patients were obese adolescents with a positive family history of type 2 diabetes mellitus; the third patient was a prepubertal, overweight girl with no family history of this disorder. The diagnosis was incidental in two patients and one patient presented with ketoacidosis. The differences between the three patients reveal the great clinical variability of this disorder and suggest that various underlying factors are involved in the pathophysiology of type 2 diabetes.     

Recognition of asthmatic symptoms in the pediatric age group

Asthma produces substantial morbidity in childhood. Under‐diagnosis may lead to inappropriate treatment and prolonged periods of illness and absence from school. The results of a recent International Study of Asthma and Allergies in Childhood (ISAAC) questionnaire study in Malta showed a higher prevalence of wheezy symptoms in the Central North Region. The present study was carried out to test the null hypothesis that there is no correlation between potential exposure to pediatricians (measured as pediatric private clinics/1000 childhood population/week) and percentage of children aged 13–15 years
of age responding positively in the questionnaire to having had wheezy symptoms. A significant, positive correlation was found between potential exposure to pediatricians and percentage of children aged
13–15 years responding positively to having had wheeze in the previous year (p=0.009). A negative correlation was also found for severity of asthma (> 12 attacks in the previous 12 months) and potential exposure to pediatricians (p=0.01). This is the first report of potential exposure to pediatricians influencing the regional incidence of asthma on a national basis. This skew may be caused by the greater exposure of pediatricians to patients with severe forms of asthma in a hospital setting, and hence a higher index of suspicion for the milder forms of the disease. Asthma may be less severe in regions where there are a greater number of pediatricians because of recognition of the condition with appropriate treatment and/or prophylaxis.     

Therapeutic and diagnostic role of bronchoscopy in pediatric age group.

Two hundred and seventy five cases were evaluated bronchoscopically for various respiratory conditions. In 140 cases, a foreign body and in 30 cases mucus plug was removed. In 47 cases there was inflammation of the tracheobronchial tree. Forty patients with empyema thoracis were evaluated bronchoscopically because of persistence of bronchopleural fistula or continued pus discharge from intercostal tubes not responding to the routine treatment. The purpose of the article is to stress the therapeutic as well as diagnostic aspect of bronchoscopy for various respiratory conditions in pediatric age group.