American journal of diseases of children (1960)最新文献_第9页

Fanconi's syndrome with hepatorenal glycogenosis associated with phosphorylase b kinase deficiency. 范可尼综合征伴肝肾糖原症与磷酸化酶b激酶缺乏相关。

American journal of diseases of children (1960) Pub Date : 1993-09-01 DOI: 10.1001/archpedi.1993.02160330047016

S A Sanjad, R E Kaddoura, H M Nazer, M Akhtar, N A Sakati

{"title":"Fanconi's syndrome with hepatorenal glycogenosis associated with phosphorylase b kinase deficiency.","authors":"S A Sanjad, R E Kaddoura, H M Nazer, M Akhtar, N A Sakati","doi":"10.1001/archpedi.1993.02160330047016","DOIUrl":"https://doi.org/10.1001/archpedi.1993.02160330047016","url":null,"abstract":"Objective: To describe two patients with Fanconi's nephropathy secondary to glycogen storage disease and speculate on the possible etiology.Design: Convenience sample.Setting: Tertiary care, referral center.Patients: Two related children referred for failure to thrive, rickets, and hepatomegaly.Intervention: Dietary and therapeutic measures for rickets and renal tubular acidosis.Measurements and results: The main laboratory findings were fasting hypoglycemia and massive glucosuria, with evidence of multiple renal tubular dysfunction characteristic of the Fanconi syndrome. Liver and kidney biopsy specimens were consistent with glycogen storage disease. Enzymatic assay of liver homogenates revealed marked deficiency of phosphorylase b kinase in one patient and absent activity in the other.Conclusion: Phosphorylase b kinase deficiency may be causally related to hepatorenal glycogenosis with the Fanconi syndrome. More patients with this syndrome need to be studied before a definitive causal role is implicated.","PeriodicalId":75474,"journal":{"name":"American journal of diseases of children (1960)","volume":"147 9","pages":"957-9"},"PeriodicalIF":0.0,"publicationDate":"1993-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1001/archpedi.1993.02160330047016","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"19349276","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 21

The morbidity of cardiac nondisease revisited. Is there lingering concern associated with an innocent murmur? 心脏非疾病的发病率再次探讨。是否有挥之不去的担忧与无辜的杂音联系在一起?

American journal of diseases of children (1960) Pub Date : 1993-09-01 DOI: 10.1001/archpedi.1993.02160330065021

P C Young

{"title":"The morbidity of cardiac nondisease revisited. Is there lingering concern associated with an innocent murmur?","authors":"P C Young","doi":"10.1001/archpedi.1993.02160330065021","DOIUrl":"https://doi.org/10.1001/archpedi.1993.02160330065021","url":null,"abstract":"Background: The phenomenon of cardiac nondisease was first reported in 1967 to call attention to the fact that some children were being restricted from their normal activities because their parents believed that they had a heart problem when, in fact, the child's heart was perfectly normal. This influential article and others have suggested that there may be significant morbidity in children who have been found to have innocent cardiac murmurs.Methods: Parents of 71 schoolchildren were interviewed 1 to 5 years after their child had been examined by a pediatric cardiologist. Parents were asked about the presence of a family history of heart disease, their recall of the diagnosis and recommendations made by the cardiologist, and their level of satisfaction with the visit to the cardiologist. Parents were extensively questioned for evidence of restriction of the child's activities and for the presence of a belief that their child's murmur was something to still be concerned about.Results: None of the parents reported restricting their child's activity, but 12 (17%) were still concerned about the murmur. A statistically significant relationship between parents reporting being less than \"very satisfied\" with the visit to the cardiologist and the presence of concern was found (P = .0006 by chi 2, Yates' correction). No other associations were noted.Conclusion: The association with a lower degree of satisfaction suggests that communication issues are relevant to the presence of lingering parental concern. Because the parents of a child with an innocent murmur may consider him or her to be \"vulnerable,\" primary care physicians should follow up with parents of children referred to pediatric cardiologists for evaluation of a murmur so that any potential misunderstandings can be clarified.","PeriodicalId":75474,"journal":{"name":"American journal of diseases of children (1960)","volume":"147 9","pages":"975-7"},"PeriodicalIF":0.0,"publicationDate":"1993-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1001/archpedi.1993.02160330065021","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"19349832","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 32

Alarming heart rates. 惊人的心率。

American journal of diseases of children (1960) Pub Date : 1993-09-01 DOI: 10.1001/archpedi.1993.02160330029010

D C Shannon

引用次数: 0

Aplastic anemia in neonatal lupus erythematosus. 新生儿红斑狼疮再生障碍性贫血。

American journal of diseases of children (1960) Pub Date : 1993-09-01 DOI: 10.1001/archpedi.1993.02160330031012

B Wolach, L Choc, A Pomeranz, Y Ben Ari, D Douer, A Metzker

{"title":"Aplastic anemia in neonatal lupus erythematosus.","authors":"B Wolach, L Choc, A Pomeranz, Y Ben Ari, D Douer, A Metzker","doi":"10.1001/archpedi.1993.02160330031012","DOIUrl":"https://doi.org/10.1001/archpedi.1993.02160330031012","url":null,"abstract":"Objective: To describe an infant with neonatal lupus erythematosus associated with aplastic anemia.Setting: The pediatric department in a tertiary-care hospital.Interventions: Packed red blood cell transfusions and a 3-week course of high-dose steroid therapy.Measurements/main results: The patient presented with severe anemia and a circumscribed, reticular, macular rash on the face and neck at 5 months of age. Skin lesion biopsy revealed epidermic hyperkeratosis, hydropic degeneration of the basal layer, and deposition of immunoglobulins and granular C1q at the dermoepidermal junction. Ro/SS-A antibodies were present in the infant. BFU-E (erythroid progenitor burst-forming unit) colonies in bone marrow increased by about tenfold when suppressor CD8+ T lymphocytes were removed, indicating immune suppression of hematopoiesis. High-dose steroid therapy failed. The infant subsequently developed gram-negative sepsis, severe metabolic acidosis, and consumptive coagulopathy and died.Conclusions: Neonatal lupus erythematosus may present as part of a spectrum. The disease may range from mild and transient to a severe, life-threatening condition requiring immediate intervention, as in the case reported here. This is the first report of neonatal lupus associated with aplastic anemia due to immune-mediated suppression of hematopoiesis.","PeriodicalId":75474,"journal":{"name":"American journal of diseases of children (1960)","volume":"147 9","pages":"941-4"},"PeriodicalIF":0.0,"publicationDate":"1993-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1001/archpedi.1993.02160330031012","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"19349272","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 36

First, do no harm. Low birth weight and adolescent obesity. 第一，不要伤害他人。低出生体重和青少年肥胖。

American journal of diseases of children (1960) Pub Date : 1993-09-01 DOI: 10.1001/archpedi.1993.02160330073023

E R McAnarney, C Stevens-Simon

引用次数: 9

Immunization status and reasons for immunization delay among children using public health immunization clinics. 使用公共卫生免疫诊所的儿童的免疫状况和免疫延迟的原因。

American journal of diseases of children (1960) Pub Date : 1993-09-01 DOI: 10.1001/archpedi.1993.02160330055018

B Abbotts, L M Osborn

{"title":"Immunization status and reasons for immunization delay among children using public health immunization clinics.","authors":"B Abbotts, L M Osborn","doi":"10.1001/archpedi.1993.02160330055018","DOIUrl":"https://doi.org/10.1001/archpedi.1993.02160330055018","url":null,"abstract":"Objectives: To determine whether children attending our local health department clinics were being immunized in a timely manner, and to investigate the reasons for children not being immunized on schedule.Design: Cross-sectional research design.Setting: Five Salt Lake City/County Health Department immunization clinics in Utah.Participants: All patients presenting to the clinics for immunization from November 1990 to March 1991 when minor illness is prevalent.Interventions: Data were gathered through interview and questionnaire.Measurements/main results: Children were mostly white; they came from two-parent households with reasonably high incomes and high parental education level. Only four children were denied vaccination, all for inappropriate timing. None were denied for illness. More than 75% had postponed bringing their children in for immunization. The most common reason given for delay was minor illness in the child.Conclusion: Even in this \"low-risk\" population, parental misperception regarding immunizations is a significant, contributing factor to low immunization rates. Public educational programs directed at increasing parental knowledge must be developed.","PeriodicalId":75474,"journal":{"name":"American journal of diseases of children (1960)","volume":"147 9","pages":"965-8"},"PeriodicalIF":0.0,"publicationDate":"1993-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1001/archpedi.1993.02160330055018","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"19349278","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 45

The art of referral. Pediatricians and the emergency department. 推荐的艺术。儿科医生和急诊科

American journal of diseases of children (1960) Pub Date : 1993-09-01 DOI: 10.1001/archpedi.1993.02160330068022

D P Hamburger, J M Chamberlain, D W Ochsenschlager

引用次数: 4

Monitoring retinopathy of prematurity: a new form for the nursery. 早产儿视网膜病变监测:托儿所的新形式。

American journal of diseases of children (1960) Pub Date : 1993-09-01 DOI: 10.1001/archpedi.1993.02160330017003

R A Gordon, V R Lolley

引用次数: 0

Serious sports injuries requiring hospitalization seen in a pediatric emergency department. 在儿科急诊科看到的需要住院治疗的严重运动损伤。

American journal of diseases of children (1960) Pub Date : 1993-09-01 DOI: 10.1001/archpedi.1993.02160330091027

J M Davis, N Kuppermann, G Fleisher

{"title":"Serious sports injuries requiring hospitalization seen in a pediatric emergency department.","authors":"J M Davis, N Kuppermann, G Fleisher","doi":"10.1001/archpedi.1993.02160330091027","DOIUrl":"https://doi.org/10.1001/archpedi.1993.02160330091027","url":null,"abstract":"Objective: To define the spectrum of serious sports injuries requiring hospitalization seen in a pediatric emergency department.Design: Trauma registry and retrospective chart reviews.Setting: Emergency department, Children's Hospital, Boston, Mass.Study participants: One hundred forty-two patients with sports injuries presenting to the emergency department and requiring hospitalization over 2 years.Measurements/results: Serious sports injuries accounted for 13% of admissions for trauma, 0.6% of visits to the emergency department for trauma, and approximately 0.14% of total encounters. Injuries included fractures (77%), abdominal injuries (7%), multiple trauma (5%), cerebral contusions or hemorrhages (4%), and dislocations (3%). The most frequently injured areas were the extremities (69%), head and neck (16%), and abdomen (3%). Compared with nonsports injuries requiring hospitalization, sports injuries occurred in older patients (11.5 vs 7.3 years, P < .001) and more often in males (83% vs 64%, P < .001). Sports injuries also resulted in higher Injury Severity Scores (10 vs 8.8, P = .021) and in more fractures (77% vs 51%, P < .001) and neck injuries (4% vs 1%, P = .002).Conclusion: Serious sports injuries evaluated in the emergency department affect predominantly male teenagers and result predominantly in fractures involving the extremities. The spectrum of injuries is dissimilar to that resulting from other mechanisms.","PeriodicalId":75474,"journal":{"name":"American journal of diseases of children (1960)","volume":"147 9","pages":"1001-4"},"PeriodicalIF":0.0,"publicationDate":"1993-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1001/archpedi.1993.02160330091027","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"19348590","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 14

Picture of the month. Acute guttate psoriasis. 本月照片。急性口状牛皮癣。

American journal of diseases of children (1960) Pub Date : 1993-09-01 DOI: 10.1001/archpedi.1993.02160330103032

M F Toppare, D A Senşes, I S Kaya, F Kitapci, G Gedikoglu, U Dilmen, W W Tunnessen

引用次数: 0