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目的分析中枢性性早熟(CPP)女童糖脂代谢的特点及脂联素在性早熟女童糖、脂代谢中的作用。方法浙江大学医学院附属儿童医院于2004年6~10月收治50例CPP女童,测量空腹血糖、胰岛素、甘油三酯、胆固醇、脂联素,并做葡萄糖耐量试验和胰岛素释放试验,采用总体胰岛素敏感指数(WBISI)、胰岛素抵抗指数(HOMA-IR)这2个指标来评估胰岛素敏感性和胰岛β细胞功能。并与年龄匹配的正常对照组进行比较。结果(1)CPP女童空腹胰岛素、HOMA-IR明显高于正常对照组(P<0·01)。(2)CPP女童A1组胆固醇较正常对照组明显升高(P<0·05)。(3)CPP女童体重指数(BMI)值均较正常对照组明显升高(P<0·05)。其中超重16%(8/50),肥胖8%(4/50)。(4)CPP女童脂联素均较正常对照组明显下降(P<0·01)。(5)CPP女童BMI值与WBISI显著负相关(r=-0·31,P<0·05),与HOMA-IR显著正相关(r=0·30,P<0·05),与脂联素显著负相关(r=-0·43,P<0·01)。CPP女童脂联素与WBISI显著正相关(r=0·29,P<0·05),多元回归分析显示CPP女童脂联素与WBISI、HOMA-IR无显著相关性。(6)排除12例超重加肥胖CPP女童后再分析显示A1、A2组女童空腹胰岛素、HOMA-IR仍明显高于正常对照组(P<0·01),而脂联素水平3组差异无显著性。结论(1)CPP女童存在不同程度的胰岛素抵抗,尤见于BMI值明显升高的性早熟女童。(2)肥胖或超重的性早熟女童胰岛素抵抗可能与脂联素水平下降有关。     

Assessment and management of the child with excessive thirst

Excessive thirst is termed polydipsia and this is usually accompanied by excessive drinking and an increased urine output. Polyuria is the passage of unusually large volumes of dilute, pale urine. Diabetes mellitus is an important cause, which should always be considered and investigated promptly. Other causes of polydipsia such as electrolyte disturbance, illness and environmental causes should be considered. This review focusses on the polydipsia-polyuria syndromes, which include cranial diabetes insipidus, nephrogenic diabetes insipidus and primary polydipsia. This review includes an approach to assessment of polydipsia and polyuria. The symptoms and signs to consider within the history and examination are outlined. Baseline investigations are summarised. Specialist investigations, such as the water deprivation test, are described, as well as a discussion on when these are needed. The treatment and management are discussed in relation to the underlying cause. This includes the use of desmopressin, specific management of nephrogenic diabetes insipidus, and need for imaging in cranial diabetes insipidus.     

Hemangioma of the Spleen with Thrombocytopenia and Fibrinogen Deficiency

A case of giant splenic hemangioma associated with thrombocytopenia and fibrinogen deficiency is described. The case presented clinically with severe bleeding and splenomegaly. Treatment with prednisone and transfusions of fresh blood and fibrinogen, was unsuccessful in controlling the hemorrhage. Following splenectomy, the patient improved considerably, the bleeding tendency disappeared and the blood findings became normal. The relationship between giant heman-giomata, either visceral or cutaneous, and the disturbed mechanisms of blood clotting is discussed.     

Assessment and investigation of the child with disordered development

Every paediatrician, generalist or specialist, at every level and in every setting will come across the child or young person with disordered development and has a duty of care to ensure that appropriate assessment and investigations are undertaken, if each individual is to be given the best possible opportunities to achieve the highest possible level of participation and enjoy the best possible quality of life. Using a structured approach, all paediatricians have the potential to make a significant positive difference and should seek every opportunity to do so, even if seeing the child for an entirely different reason. Key messages of this article include: (1) each child is unique and requires careful, individual, clinical assessment and thought before any investigations are undertaken; (2) there is no single list of appropriate tests to be done for all children with disordered development; (3) the clinical judgement of the experienced clinician (expert triage) is more helpful than 'guidelines' in deciding which investigations to do; (4) clinical networking with colleagues in paediatric neurodisability, neurology, clinical genetics, metabolic paediatrics, and so on, is essential to achieve the highest possible yield from investigations and to reduce the number, discomfort and expense of inappropriate investigations; (5) the more effort and thought that goes into formulating differential diagnoses, the more appropriate the investigations are likely to be and the higher the likely diagnostic hit rate. Diagnostic hit rates up to 80% have been reported in the literature for those with severe learning disabilities and this is likely to be even higher once microarray comparative genomic hybridisation becomes more widely available.     

A child with esthesioneuroblastoma with metastases to the spinal cord and the bone marrow

Esthesioneuroblastoma is a rare tumor in children, and the correct diagnosis may be difficult, as is demonstrated in this case report. A 5-year-old girl was diagnosed with this tumor, which was incurable and behaved like a neuroblastoma, sending metastases to the bone marrow and invading the cranium and the spinal canal.     

Review of the reliability and validity of myometry with children

Pediatric occupational therapists and physical therapists often measure muscle strength, and muscle strength frequently is cited as an indicator of function in clinical practice and in research. A reliable measure to quantify strength is necessary to accurately assess muscle strength so the values can be used for comparison, study, and indication of change. The hand-held myometer provides an alternative to traditional methods of manual muscle testing, and its reliability and validity have been examined in adults. The purpose of this review is to summarize the available literature relating to the reliability and validity of the hand-held myometer when used with children and to identify directions for future study in the use of myometers with children.     

Ewing's sarcoma of the scapula with metastases to the lung and eye

A small, round cell tumor of the left scapula was found in a 3-month-old female Caucasian child. The histology was consistent with Ewing's sarcoma. Subsequently, a solitary pulmonary metastasis was excised. Thirty-one months after diagnosis, the child presented with leukokoria and a solitary pleural metastasis. The histology of both was identical to that of the original scapular tumor. G-banded karyotypes of the pulmonary metastasis and the ocular tumor revealed the presence of a balanced translocation, 46,XX, t(11;22)(q24;q12), which supported the original diagnosis of Ewing's sarcoma.     

Degree of compliance with the ten steps of the Breastfeeding Friendly Primary Care Initiative and its association with the prevalence of exclusive breastfeeding

ObjectiveTo analyze the association between the degree of compliance with the ten steps of the Breastfeeding-Friendly Primary Care Initiative (BFPCI) and the prevalence of exclusive breastfeeding (EBF) in infants younger than six months in the city of Rio de Janeiro.MethodsThis was a cross-sectional study conducted in a representative sample of 56 primary health care units of this municipality. The assessment of compliance with the ten steps of the BFPCI was carried out by interviewing health care professionals, pregnant women, and mothers; the generated performance scores were classified into tertiles. To obtain the outcome, i.e., the EBF, a data collection questionnaire was applied to mothers of children younger than six months who were followed up at these units in November of 2007. Prevalence ratios were obtained for the EBF using Poisson regression with robust variance.ResultsThe prevalence of EBF was 47.6%. In the multivariate analysis, the upper tertile of performance showed a 34% higher prevalence of EBF (PR = 1.34, 95% CI: 1.24 to 1.44) and the second tertile was 17% higher (PR = 1.17, 95% CI: 1.08 to 1.27) than the first tertile. Mothers who did not work outside home had a 75% higher prevalence of EBF (PR = 1.75, 95% CI: 1.53 to 2.01); assistance in a basic health unit, as opposed to a family health unit, implied a 10% higher prevalence (PR = 1.10, 95% CI: 1.03 to 1.19). The prevalence of EBF decreased 1% for each day of the infant's life (PR = 0.993, 95% CI: 0.992 to 0.993).ConclusionGiven the contribution of BFPCI to the practice of EBF, a greater investment in the expansion and sustainability of this initiative is recommended, as well as its association with other strategies to promote, protect, and support breastfeeding.     

Pre- and Postoperative Exercise Testing of the Child with Coarctation of the Aorta

Exercise studies in patients with coarctation of the aorta (CoA) are reviewed. A brief survey of the natural history of unoperated CoA, the pioneers who reported the first operative repair, and comments on blood pressure measurements are provided. Preoperative and postoperative (postop) exercise studies of patients with CoA are also reviewed. The various etiological factors which may contribute to hypertension found in some post-op CoA patients are discussed.     

Experiences with miconazole in the prevention and therapy of candidiasis

1. The treatment of 26 children suffering from haemoblastoses and tumors by the antimycotic Miconazol is reported on. 2. The application took place prophylactically as well as therapeutically by an oral or intravenous administration. Mycological examinations (stools, urine, throat swab, blood) have been carried through before, during and after the treatment in weekly intervals. 3. The result of treatment is altogether to be called good, invasive mycoses have been prevented and proved Blastomyces infections successfully treated. 4. Side effects like nausea and vomiting were observed only in one case, damages of the haematopoietic system, the liver and kidneys could not have been demonstrated.     

Rectum and bladder duplication with malformations of the VACTERL association

The case of a newborn with duplications of rectum, presenting as a tumor of the labia majora, and duplication of bladder and urethra is reported. In addition malformations from the VACTERL-association (sacral malformation, anal atresia with vestibulum fistula, renal dysplasia, only eleven pairs of ribs with hypoplasia of the eleventh one, diplopodia on the left foot) were found. In the study of urogenital and enteric duplications in cases from the literature this coincidence with VACTERL malformations was found as well.     

Bleeding and surgery in children with Glanzmann thrombasthenia with and without the use of recombinant factor VII a

BACKGROUND: An inherited deficiency of platelet glycoprotein II b/III a (GP II b/III a), Glanzmann thrombasthenia, can lead to excessive bleeding and require platelet transfusion to secure hemostasis. Antibodies to GP II b/III a or HLA may platelet transfusion render ineffective to stop bleeding or to cover surgery. Recombinant factor VII a has been introduced as therapeutic alternative and has been suggested to be effective. PATIENTS AND AIMS OF THE STUDY: In a retrospective evaluation, bleeding episodes and surgery in six patients treated with antifibrinolytics and with and without the additional use of rFVII a were analysed to achieve informations for treatment indication and efficacy. RESULTS: Nineteen mucosal and subcutaneous bleeding episodes, two dental surgeries and seven joint bleeds occurred. In 11 mild to moderate mucocutaneous bleeds treated without rFVII a, seven stopped within 48 hours, three stopped until the fourth day; one showed recurrence. Three bleeds were treated with rFVII a and responded within 24 hours. One severe bleed treated without rFVII a did not stop until the 8 (th) day after cautery. In 4 severe bleeds treated with rFVII a, one stopped within 24 hours, one showed recurrence, one was treated with platelet transfusion concurrently and one did not respond to rFVII a. Clinical signs persisted in one conservatively treated elbow joint bleed, whereas in two episodes treated with rFVII a, the bleeding responded within 5 and 7 days and in four episodes in at least 4 days. Two dental surgeries showed no recurrence after rFVII a over 18 or 36 hours. CONCLUSIONS: In severe mucocutaneous bleeding episodes or joint bleeding rFVII a is of some benefit whereas in surgeries like teeth extraction, prophylactically administered rFVII a seems effective to avoid bleeding. In mild to moderate mucocutaneous bleeding events, antifibrinolytics and local measures were sufficient in most cases and the additional use of rFVII a does not seem to be necessary. Further information is needed to elaborate clear indications for the rational use of rFVII a in bleeding episodes in patients with Glanzmann thrombasthenia compared to standardized baseline treatment. This information may generate a prospective multicenter study to provide clear advice with respect to bleeding site, severity and duration.     

Rhabdoid tumour of the kidney with caval and cardiac extension

Rhabdoid tumour of the kidney is a rare paediatric neoplasm usually associated with a grave prognosis. We report a case of left renal rhabdoid tumour with involvement of the inferior vena cava (IVC) and right atrium in a 13-year-old girl. Left nephrectomy together with removal of the IVC and atrial extension was undertaken on cardiopulmonary bypass and hypothermia. She remained disease-free and in good health for 1 year, however, recent review has shown recurrence in the abdominal lymph nodes.     

The pediatrician and medical care of the child with cancer

The child's pediatrician is an important resource for families regarding a host of issues such as infections, complications and side effects of chemotherapy, school issues, and psychosocial stressors that are common in families of children with life-threatening illness. This article provides guidance for caring for children with malignancies in the primary care setting.