小儿急性血肿型肺吸虫脑病的外科治疗

目的 分析小儿急性血肿型肺吸虫脑病的临床特点和诊治方法,探讨该型与其他类型的区别.方法 回顾性分析16例急性血肿型肺吸虫脑病患儿的临床特点、诊断方法和治疗效果.结果 16例患儿均有高颅压症状,惊厥发作5例,肢体偏瘫或单瘫4例,合并肺部症状4例；流行病学史阳性14例；全部病例肺吸虫抗体检测阳性；CT检查发现单纯脑内血肿12例,伴蛛网膜下腔出血3例,伴脑梗塞1例,血肿以额叶、顶叶为主.手术开颅清除血肿11例,非手术治疗5例,所有病例均口服吡喹酮治疗4～6个疗程,随访2个月至2年,预后良好.结论 小儿急性血肿型肺吸虫脑病与其他类型有较大差别,病程更短、病情更为危重；影像学检查易出现误诊、漏诊；治疗上更倾向于积极手术；规范治疗预后更好.     

Nosokomiale Sepsis bei sehr kleinen Frühgeborenen

Diagnostics

In recent years, options for earlier diagnosis of the late onset sepsis (LOS) in very low birth weight preterm infants have increased considerably. The continuous evaluation of clinical (heart frequency variability, microcirculation) and clinical–chemical (cytokines) parameters are the most sensitive methods nowadays. Protein microarrays are the basis for a cost-effective analysis, yet their clinical use is just starting.

Evaluation of an infection

Due to the different kinetics of the various parameters used for the diagnosis of an infection, a complete evaluation of an infection and decision-making to stop antibiotic therapy is only possible by the measurement of various parameters at the same time. A combination of proinflammatory cytokines (IL-6 or IL-8) with C-reactive protein (CRP) or procalcitonin is currently the best decision.

Therapy

Progress in treatment of LOS can be achieved by optimizing already known strategies. Prophylaxis of hospital acquired infections by consequent application of hygiene procedures (RKI guidelines) is the best way to avoid infections. In case of the clinical suspicion of LOS in preterm infants, antibiotic therapy has to be started on the basis of the known local surveillance data, even before receiving results from the laboratory. Therapy is executed on the principles of the Tarragona strategy. Permanent infection control needs the consequent application of Antibiotic Stewardship Programs in neonatal intensive care units.     

Nebulized budesonide for the treatment of moderate to severe asthma in infants and toddlers*

Maintenance treatment with nebulized budesonide was studied in young children with asthma not controlled without steroids. In a blind parallel-group study for 18 weeks, 102 children, mean age 22 (5–47) months, were randomized for treatment starting with 0.25 or 1 mg b.i.d. The patients were reviewed every 3 weeks, and if symptom control had been achieved the dose was reduced, otherwise it was kept. The clinical effect was very good with both dose regimens. The median time to 7 consecutive days without any asthma symptoms was about 1 month with both, highlighting the importance of the duration of therapy rather than the benefits of a high starting dose. In 18 of 24 children who attained the placebo stage, symptoms had reappeared at the last visit. Although an overall minimal effective maintenance dose could not be demonstrated, 47% achieved symptom control on 0.25 mg b.i.d., i.e. fulfilled criteria for further dose reduction. No significant side effects were seen. On average, 25% of the nominal dose reached the patients.     

Congenital mesoblastic nephroma: a clinicoradiologic study of 17 cases representing the pathologic spectrum of the disease

Congenital mesoblastic nephroma (CMN) is a rare infantile renal tumor with a generally excellent prognosis. We describe 17 tumors that fit into the pathologic spectrum of CMN proposed by Beckwith, which ranges from benign renal tumors, through atypical "gray zone" lesions of more aggressive potential, to "crossover" tumors akin to clear cell sarcoma of kidney. Nine patients with histologically typical CMN were significantly younger and had smaller tumors than did eight patients with atypical CMN. Clinical features did not differ in the two groups of patients. A distinctive "ring sign" on renal sonography was commonly seen in patients with typical intrarenal CMN. All 17 patients were alive with no evidence of disease at a mean follow-up of 10 years. Nephrectomy was adequate therapy for younger infants and for those with typical CMN. Nephrectomy was probably also adequate therapy for infants 3 months of age or younger with atypical CMN, even if the tumor extended to the surgical resection margins and into the perinephric connective tissues. Adjuvant chemotherapy or radiation or both should be reserved for patients older than 3 months who have grossly unresected tumors and for those patients whose tumors have an unequivocally malignant histologic appearance or evidence of aggressive biologic behavior.     

Pneumococcal endocarditis in infants and children

We report a case of pediatric pneumococcal endocarditis (PPE) and review the English language literature on this disease. Thirty-two cases of PPE were identified since 1900. One-fourth of these were reported since 1990. Clinical features differed from adult cases, with mitral valve involvement being more frequent and Osler's triad rarely present in children. Congenital heart disease was the only identifiable risk factor. Medical therapy alone resulted in a high mortality rate that was improved in the group of patients receiving combined medical and surgical interventions. PPE is a rare infection that has been reported more frequently in the era of increasing antibiotic resistance. Unlike typical "subacute" endocarditis caused by viridans streptococci, PPE is an aggressive disease with a high mortality rate. Early surgical intervention might improve survival.     

Inflammatory fibroid polyps in children:A new case report and a systematic review of the pediatric literature

AIM: To study that inflammatory fibroid polyps(IFPs) in children are extremely uncommon tumors that may occur throughout the gastrointestinal tract. METHODS: A systematic review of the pediatric literature and a report of a new case of IFP is also pres-ented. The Pub Med database was searched for original studies on pediatric IFPs since 1960, according to "Preferred reporting items for systematic reviews and meta-analyses" guidelines for systematic reviews. RESULTS: Five studies were finally enclosed, encompassing 6 children with IFPs(mean age 64 mo). Tumors were located in the stomach(2 patients), in the small bowel(2 patients), in the rectum(1 patient) and in the colon(1 patient). Open surgery was performed in all patients and complete excision of the mass was achieved in all cases. All patients are alive and free of symptom. Authors described a further case of a 3-year-old boy with a large duodenal IFP, in whom the tumor was removed by "en block resection". The presence of IFP throughout the gastrointestinal tract and its variable clinical appearances make it difficult to diagnose. An accurate pre-operative assessment is fundamental in order to differentiate IFP from other more aggressive gastrointestinal tumor, enabling unnecessary demolitive surgery. CONCLUSION: When complete resection of the IFP is achieved, the prognosis is excellent.     

Basilar artery occlusion in a 14-year old female successfully treated with acute intravascular intervention: case report and review of the literature

Basilar artery occlusion (BAO) is a rare cause of paediatric stroke that may result in severe neurological disability including a 'locked-in' state. Acute interventional therapy for paediatric BAO is limited to a small number of published case reports. Of 13 previously published cases that have undergone acute intravascular therapy, six made a full neurological recovery, six had residual deficits ranging from mild dysarthria and ataxia to vegetative state and one patient died. The time from symptom onset to intervention was ≥ 12 h in 77% (10/13). We reported a 14-year-old female patient presenting with altered sensorium that progressed to a 'locked-in' state due to idiopathic BAO who made a full clinical recovery after successful mechanical thrombectomy at 24 h following symptom onset. Acute neuro-interventional therapy for paediatric BAO can result in complete neurological recovery despite the presence of severe neurological deficits and a prolonged period of time from symptom onset to clinical diagnosis.     

Chronic diarrhoea as a dominating symptom in two children with cerebrotendinous xanthomatosis

The objective of this study was to describe diarrhoea as a dominating symptom of cerebrotendinous xanthomatosis (CTX), a lipid storage disease, and investigate its cause. Two children with chronic diarrhoea as the dominating symptom of CTX are presented. Before and after therapy with orally administered chenodeoxycholic acid (15 mg kg⁻¹ 24 h, in three divided doses) bile alcohol excretion in urine, serum cholestanol level, serum bile acid patterns and faecal bile acids were measured. All routine gastro-intestinal investigations before therapy were normal. Diarrhoea ceased immediately after starting treatment with chenodeoxycholic acid. Abnormal bile alcohol excretion in urine decreased rapidly during the first days and elevated serum cholestanol level normalized in 2 years. We postulate the presence of bile alcohols in the lumen of the gut as most likely cause for diarrhoea in CTX, since the rapid decrease of bile alcohol excretion is associated with prompt cessation of diarrhoea after starting treatment with chenodeoxycholic acid.     

Eosinophilic Granuloma of Bone

Eosinophilic granuloma of bone is an uncommon granulomatous process for which therapy recommendations vary considerably. Consequently, a survey of the world literature from 1940 to 1974 was undertaken in an attempt to develop more concrete guidelines. This survey revealed that the disease manifests itself primarily in males under 20 years of age of all races. The most common symptom is pain, with or without swelling. Multiple sites in a single case are frequent. The clinical course is generally benign, unlike Letterer-Siwe disease or Hand-Schuller-Christian disease. A remission rate of 95% was noted, with relapses usually occurring as distant disease within the first year. Simple excision or curettage was the most common surgical procedure utilized, and radiotherapy doses varied widely. Excellent local control of the disease can be provided using conservative surgery, low-dose irradiation, or both. Morbidity secondary to pathologic fracture or to overly aggressive treatment are the major concerns, not failure to control the disease.     

Beyond the guidelines: fatal and near-fatal asthma

While asthma is extremely common, fatal and near fatal asthma is rare and often preventable if there is early recognition of symptom progression and appropriate intervention. In the past decade, asthma mortality has progressively declined in association with the widespread use of inhaled corticosteroids and asthma care plans. Management of life threatening asthma requires patient education to ensure the proper use of medications and to enable the patient to recognize when additional therapy for poorly controlled asthma is required. There is some evidence that suggests that the overly aggressive use of asthma medications when treating a severe exacerbation may contribute to morbidity. Because of the risks of air trapping and barotrauma associated with partial airway obstruction, it is best to avoid mechanical ventilation if possible but when this is used, low tidal volumes, longer exhalation times, and permissive hypercarbia can minimize these risks. There is the promise that a better understanding of asthma immunology and severe asthma "phenotypes" will lead to better prevention and therapy.     

Severe GABAA receptor encephalitis without seizures: A paediatric case successfully treated with early immunomodulation

Background

Autoimmune-mediated processes are the driving force behind many neurological diseases. Autoimmune encephalitis, a group of syndromes, mediated by or at least associated with autoantibodies against neuronal tissue, have gained increasing importance especially in paediatric neurology. Since the first NMDAR encephalitis was described a growing number of patients with encephalopathy, seizures and psychiatric symptoms were found to suffer from treatable autoimmune disorders. Recently a severe form of encephalitis associated with GABA_AR antibodies was described showing extensive MRI abnormalities and refractory seizures.

Atypical teratoid/rhabdoid tumor with 26-year overall survival: case report

Atypical teratoid/rhabdoid tumor (AT/RT) is an aggressive embryonic brain tumor predominantly seen in young children. The authors report an unusual case of a fourth ventricle AT/RT in an infant who survived for 26 years. The tumor was resected when the patient was 6 months of age, and radiation therapy (40-Gy total dose) was performed thereafter. The patient was free from the disease for 26 years until a recurrent tumor was found in the spinal cord. The spinal cord neoplasm was a "collision tumor" with 2 components: benign schwannoma and recurrent AT/RT. The patient died of dissemination of the recurrent tumor 5 months after it was excised. This is the longest survival of a patient with AT/RT ever reported and indicates that long-term survival, more than 20 years, can be achieved in infantile-onset AT/RT. Despite intensive treatment, the prognosis for AT/RT is very poor, especially in children younger than 3 years of age. The benefits of upfront radiation therapy for AT/RT should be carefully assessed with respect to its inevitable toxicity in very young children. However, early upfront radiation therapy may be of therapeutic interest to prevent aggressive progression of the disease.     

Secondary abdominal compartment syndrome in a case of pediatric trauma shock resuscitation.

OBJECTIVE: To report a rare case of secondary abdominal compartment syndrome during shock resuscitation in a pediatric trauma patient. DESIGN: Case report and literature review. SETTING: A community hospital and a designated children's trauma hospital. PATIENT: A 17-yr-old trauma patient. INTERVENTIONS: Advanced trauma life support, trauma laparotomy, and superficial temporal artery ligation. MEASUREMENTS AND MAIN RESULTS: A 17-yr-old trauma patient with ongoing blood loss from a lacerated superficial temporal artery received aggressive crystalloid resuscitation before arrival at a designated trauma hospital. His injury severity score was 16. The first hemoglobin drawn was 55 g/L with a hematocrit of 0.16 L/L. Within 3 hrs of the trauma, an abdominal computed tomography scan demonstrated a moderate amount of free peritoneal fluid, edematous bowel with marked enhancement, and a compressed inferior vena cava. Shortly after completion of imaging studies, the patient's abdomen became increasingly tense with poor perfusion to the lower extremities. Urgent laparotomy for abdominal compartment syndrome identified excessive ascites and extensive bowel edema with no blood or traumatic injuries. Abdominal decompression resulted in immediate improvement of hemodynamics and restored lower limb perfusion. Primary abdominal closure was obtained and the patient recovered fully with gentle diuresis. CONCLUSIONS: Secondary abdominal compartment syndrome developed in this pediatric trauma patient with hemorrhagic shock, possibly from aggressive crystalloid resuscitation. This trauma case highlights the importance of early hemorrhagic control with balanced crystalloid/transfusion therapy. Secondary abdominal compartment syndrome in pediatric trauma is rare and may reflect physiologic differences during development, less aggressive resuscitation practices, and/or underrecognition.     

Human monocytic ehrlichiosis in children

BACKGROUND: Human monocytic ehrlichiosis (HME) is a tick-borne illness caused by Ehrlichia chaffeensis. Data about disease in children have been largely derived from case reports or small case series. METHODS: A retrospective review of all medical and laboratory records from 6 sites located in the "tick belt" of the Southeastern United States was carried out. Demographic, history and laboratory data were abstracted from the identified medical records of patients. Bivariate statistical comparisons were performed using Fisher exact test or Wilcoxon rank sum tests. RESULTS: Common clinical signs and symptoms of patients with HME (n = 32) included fever (100%), headache (69%), myalgia (69%), rash (66%), nausea/vomiting (56%), altered mental status (50%) and lymphadenopathy (47%). Only 48% had a complaint of fever, headache and rash. Common laboratory abnormalities included thrombocytopenia (94%), elevated aspartate aminotransferase (90%), elevated alanine aminotransferase (74%), hypoalbuminemia (65%), lymphopenia (57%), leukopenia (56%) and hyponatremia (55%). The median number of days of illness before the initiation of antirickettsial therapy was 6. Patients who received sulfonamides before starting doxycycline therapy developed a rash, were admitted to the hospital, and started doxycycline at a later date. Twenty-two percent of patients were admitted to the intensive care unit with 12.5% of patients requiring ventilatory and blood pressure support. CONCLUSIONS: Although HME has been recognized among children for almost 20 years, there is only a limited knowledge about its clinical course. Even among physicians practicing in endemic regions, few cases are diagnosed each year. More work is needed to understand the true burden of disease and the natural history among asymptomatically and symptomatically infected children.     

Plasmapheresis, intravenous cytomegalovirus‐specific immunoglobulin and reversal of antibody‐mediated rejection in a pediatric renal transplant recipient: A case report

This is a pediatric case report illustrating the development of antibody (Ab)-mediated rejection in a patient with low levels of pretransplant anti-human leucocyte antigen (HLA) panel reactive antibodies (PRA). The clinical course of this patient suggests that aggressive use of a combination of plasmapheresis, monoclonal anti-T-lymphocyte antibody therapy, and intravenous immunoglobulin (IVIG) therapy can reverse Ab-mediated rejection in previously allosensitized pediatric transplant recipients.     

Giant supratentorial atypical teratoid/rhabdoid tumor presentation: a case of a five-year-old child with favorable outcome and review of the literature

Atypical teratoid/rhabdoid tumor of the central nervous system is a highly malignant neoplasm and that usually arises in the posterior fossa, survival from this is frequently poor. We present a unique case in a 21-month-old girl who had an atypical teratoid/rhabdoid tumor with cystic components located in the right fronto-parietal lobe. The patient underwent radical surgical intervention followed by chemotherapy. It consisted of five chemotherapeutic agents, but the patient did not receive any radiotherapy. The postoperative course was uneventful and the patient was followed-up by cranial magnetic resonance imaging every 3 months. Two years later at the last follow-up visit, there was no evidence of a tumor relapse on MRI, and the examination was symptom free. It is possible the favorable outcome of the patient resulted from a rapid diagnosis, prompt management, radical surgical intervention and aggressive chemotherapy.     

Using Social Indicators in Assessing Factors and Numbers of Street Children in the World

Many reports rely on the estimate that there are anywhere from 150 to 100 million street children in the world, although this figure has virtually no basis in empirical evidence. In this article, all the available data on the number of street children and relevant social indicators for 184 countries were gathered and statistically processed in order to produce a more reliable estimate. Aptekar (Cross-Cultural Research 28(3): 195–224, 1994) assumption on common denominators of countries with a high presence of street children was used as a starting point. The results show that there is about 10 to 15 million street children in the world. Two directions for future development are outlined: firstly, a more reliable and unified inductive approach for estimating the number of street children in individual countries is needed along with an appropriate methodological model and secondly, there is a need to create a global estimate of the number of all children living in the streets, which might be achieved by a similar methodological approach to the one used in this article.     

Successful use of posaconazole in a pediatric case of fungal necrotizing fasciitis.

OBJECTIVE: To report a pediatric case of posttraumatic fungal fasciitis treated successfully with a combination including posaconazole. DESIGN: Case report. SETTING: Pediatric critical care unit at Antwerp University Hospital. PATIENT: A 12-yr-old female polytrauma patient, suffering from extensive skin and muscle injuries, developed fasciitis. Two fungi (Mucor and Trichosporon species) were isolated. The necrotic tissues were aggressively excised, hyperbaric oxygen therapy was applied, and oral posaconazole was added to the initial amphotericin B treatment. The patient made an excellent recovery. CONCLUSIONS: A combination of a high index of suspicion, early aggressive surgery, hyperbaric oxygen, and the association of amphotericin B and posaconazole led to a successful outcome in a case of fungal necrotizing fasciitis. After decades of disappointing amphotericin B treatment in spite of acceptable in vitro activity, the combination with the new triazole posaconazole seems promising.     

Symptom development in originally asymptomatic CPAM diagnosed prenatally: a systematic review

Purpose

For the approximately three quarters of patients with a prenatal diagnosis of congenital pulmonary airway malformation (CPAM) who are asymptomatic at birth, the chance of eventually developing symptoms is unknown. We sought to explore the natural history of asymptomatic CPAM.

Methods

We searched EMBASE, MEDLINE, and the first 50 results from Google Scholar. Studies describing the natural history of prenatally diagnosed, initially asymptomatic CPAM were included. For asymptomatic patients initially managed non-surgically, we tabulated the proportion who went on to develop symptoms as well as the median age at symptom development.

Results

We included data from 19 retrospective studies on 353 patients. Of the 128 patients managed expectantly, 31 (24.2%) developed symptoms requiring surgical intervention. The median age at symptom development was 7.5 months (range 15 days–5 years).

Conclusion

The risk for developing respiratory symptoms exists with originally asymptomatic CPAM patients, but the exact risk is difficult to predict. Parents may be given the value of approximately 1 in 4 as an estimate of the proportion of asymptomatic CPAM patients who go on to develop symptoms, which will help them make an informed decision regarding the option of elective surgery.