Local Experience with Extracorporeal Membrane Oxygentaion in Children with Acute Fulminant Myocarditis

To analyze the clinical effect of extracorporeal membrane oxygenation (ECMO) in children with acute fulminant myocarditis, we retrospectively analyzed the data of five children with acute fulminant myocarditis in the intensive care unit (ICU) at the Affiliated Children’s Hospital, Zhejiang University from February 2009 to November 2012. The study group included two boys and three girls ranging in age from 9 to 13 years (median 10 years). Body weight ranged from 25 to 33 kg (mean 29.6 kg). They underwent extracorporeal membrane oxygenation (ECMO) through a venous-arterial ECMO model with an average ECMO supporting time of 89.8 h (40–142 h). Extracorporeal circulation was established in all five children. After treatment with ECMO, the heart rate, blood pressure, and oxygen saturation were greatly improved in the four children who survived. These four children were successfully weaned from ECMO and discharged from hospital machine-free, for a survival rate of 80% (4/5). One child died still dependent on the machine. Cause of death was irrecoverable cardiac function and multiple organ failure. Complications during ECMO included three cases of suture bleeding, one case of acute hemolytic renal failure and suture bleeding, and one case of hyperglycemia. During the follow-up period of 4–50 months, the four surviving children recovered with normal cardiac function and no abnormal functions of other organs. The application of ECMO in acute fulminant myocarditis, even in local centers that experience low incidence of this disease, remains an effective approach. Larger studies to determine optimal timing of placement on ECMO to guide local centers are warranted.     

Long-term Stability of Remission in Nephrotic Syndrome after Treatment with Cyclophosphamide

Fifty-eight children with minimal-change nephrotic lesions who relapsed repeatedly and showed toxic side effects from corticosteroids were treated with cyclophosphamide for an average of 12 weeks. The initial dose was 5 mg/kg/day. Four to seven years (mean 5·8 years) later 20 remained in remission, 34 were still relapsing, and 4 had died (two during relapses, one of measles after cyclophosphamide, and one of a brain-stem astrocytoma). The half time for the relapse-free period after treatment was 2·8 years. There was no relationship between the length of treatment with cyclophosphamide and the stability of remission within the limits studied.     

Why do patients with lupus nephritis die?

Over 20 years 42 of 138 patients with systemic lupus erythematosus "died"--that is, suffered actual death or went into terminal renal failure, or both; data from 41 were available for analysis. In most patients the causes of death were multiple. Twenty seven patients went into terminal renal failure, of whom 25 were offered dialysis treatment. Three regained renal function later, 12 survived on dialysis or with functioning kidney allografts--almost all with inactive lupus--but 13 died after starting dialysis, most within a few weeks or months. The principal causes were active lupus or infection. In those patients with renal failure after rapid deterioration in renal function (n = 14) there were nine deaths, while of 10 patients with a slow evolution into renal failure, only four died. Four patients with impaired and 10 with normal renal function died, again most often from complications of lupus or from infection. Vascular disease was a major cause of death in seven patients, all but two of whom were young; of 15 postmortem examinations, eight showed severe coronary artery atheroma, and three surviving patients required coronary bypass operations. Analysis of the timing of death or entry into renal failure showed that in 12 out of 13 patients who died within two years of onset the lupus was judged to be active, while this was true in only eight out of 19 patients who died later. Six of the seven vascular deaths occurred later than two years from onset, while only nine of 26 renal "deaths" occurred before two years; deaths from infections (n = 13) were distributed equally. Despite this and aggressive treatment of active disease, the principal cause of actual death was uncontrolled lupus.     

Sudden unexpected death in children with congenital heart disease.

Of 18,000 children with organic heart disease evaluated at The Hospital for Sick Children, Toronto between 1940 and 1971, 33 died suddenly and unexpectedly between 1 and 21 years of age. Nine had discrete obstruction of the left ventricular outflow tract and five had muscular narrowing of the left ventricular outflow tract and five had muscular narrowing of the left ventricular outflow tract. Pulmonary vascular disease caused seven sudden deaths, and arrhythmias (usually due to atrioventricular block) caused seven more. Of the five other children who died suddenly three had transposition of the great arteries, one had a complex cyanotic heart defect and one had an anomalous course of the left coronary artery, which originated from the right sinus of Valsalva. With earlier investigation of aortic stenosis, earlier closure of ventricular septal defect to avoid pulmonary vascular disease, better design of artificial pacemakers and better investigation of patients with angina, many of these deaths will be avoided in the future.     

Outcome in colorectal adenocarcinoma: two seven-year studies of a population.

OBJECTIVE--To record every patient with proved colorectal adenocarcinoma presenting from a defined population over two years in 1968-9 and during 1980-2, and to compare treatment and outcome over seven years. DESIGN--Retrospective in 1968-9 and prospective in 1980-2. SETTING--Aberdeen general hospitals. SUBJECTS--Every patient presenting to the four general surgical units with histological proof of colorectal adenocarcinoma. MAIN FINDINGS--On average, one new patient presented each week per 100,000 population. The proportion of patients aged 65 and over rose from 67% to 71%. An operation was performed on 385 patients in 1968-9 and on 399 during 1980-2. At laparotomy the proportion of patients who seemed to be curable and had a radical operation rose slightly, from 56% to 61%, and operative mortality fell from 9% to 5%. In all there were 421 survivors of curative surgery, and seven years later three quarters were either alive (51%) or had died without recurrence (25%). In both studies some 40% of patients were considered incurable when they presented, but the number who had a palliative resection rose from 59% to 85%. CONCLUSIONS--The contribution of radical surgery to the treatment of colorectal adenocarcinoma is substantial, with three quarters of patients so treated showing no evidence of recurrence after seven years. Operative mortality in an elderly population is now low, and improvement in late outcome is more likely to come from developments in adjuvant therapy than in operative technique. A continuing problem is that 40% of patients are not curable when they present, although palliative resection can now be offered to over 80%.     

Subacute bacterial endocarditis; a report on 57 patients treated with massive doses of penicillin

Fifty-seven patients with subacute bacterial endocarditis were treated with doses of penicillin varying from 500,000 to 20,000,000 units per day. Diagnosis was confirmed in some cases by growths on blood culture, in others by postmortem examination. In those cases in which the diagnosis was established by blood culture, the in vitro sensitivity of the organism to penicillin was determined and penicillin then was administered by continuous intramuscular infusion in a dosage calculated to produce blood levels of penicillin four to five times that required for in vitro inhibition. Penicillin was given for a period of 21 days, and blood cultures were made periodically during and after treatment. Of the 57 patients, 38 were cured (66.7 per cent), and 19 died (33.3 per cent). Of the 19 who died, three did so within 48 hours of hospitalization and seven died despite adequate treatment. Of these seven, three died of cerebral emboli, two because of resistance to penicillin and streptomycin, one because of congestive heart failure, and one of undetermined cause. The remaining nine who died were considered to have been inadequately treated in that there was (1) failure to obtain sensitivity, (2) inadequate dossage of penicillin, (3) delay in starting treatment, or (4) failure to recognize mixed infections. There were five patients with repeatedly sterile blood cultures during life. In all of these cases, streptococcus viridans was recovered at postmortem examination. In an attempt to determine how long therapy should justly be withheld in patients with repeatedly sterile blood cultures, 140 cases of subacute bacterial endocarditis in which positive blood cultures had been obtained were reviewed. From the review it was determined that if blood cultures taken during the first two days are reported sterile, the chance of subsequent cultures proving positive is minimal. Therefore, for patients in whom the diagnosis seems otherwise obvious, delaying treatment for more than two days is not justified even though the blood culture be sterile. In cases in which blood cultures are repeatedly sterile, a dosage of 6,000,000 to 10,000,000 units of penicillin daily for 21 days is advisable.High bacterial resistance to penicillin and streptomycin was found in four fatal cases. In one of these, the infecting organism was streptococcus viridans, and in three it was staphylococcus albus. There was one patient with penumococcal meningitis complicated by unrecognized streptococcal viridans bacterial endocarditis.     

SUBACUTE BACTERIAL ENDOCARDITIS—A Report on 57 Patients Treated with Massive Doses of Penicillin

Fifty-seven patients with subacute bacterial endocarditis were treated with doses of penicillin varying from 500,000 to 20,000,000 units per day. Diagnosis was confirmed in some cases by growths on blood culture, in others by postmortem examination. In those cases in which the diagnosis was established by blood culture, the in vitro sensitivity of the organism to penicillin was determined and penicillin then was administered by continuous intramuscular infusion in a dosage calculated to produce blood levels of penicillin four to five times that required for in vitro inhibition. Penicillin was given for a period of 21 days, and blood cultures were made periodically during and after treatment.Of the 57 patients, 38 were cured (66.7 per cent), and 19 died (33.3 per cent).Of the 19 who died, three did so within 48 hours of hospitalization and seven died despite adequate treatment. Of these seven, three died of cerebral emboli, two because of resistance to penicillin and streptomycin, one because of congestive heart failure, and one of undetermined cause. The remaining nine who died were considered to have been inadequately treated in that there was (1) failure to obtain sensitivity, (2) inadequate dossage of penicillin, (3) delay in starting treatment, or (4) failure to recognize mixed infections.There were five patients with repeatedly sterile blood cultures during life. In all of these cases, streptococcus viridans was recovered at postmortem examination. In an attempt to determine how long therapy should justly be withheld in patients with repeatedly sterile blood cultures, 140 cases of subacute bacterial endocarditis in which positive blood cultures had been obtained were reviewed. From the review it was determined that if blood cultures taken during the first two days are reported sterile, the chance of subsequent cultures proving positive is minimal. Therefore, for patients in whom the diagnosis seems otherwise obvious, delaying treatment for more than two days is not justified even though the blood culture be sterile. In cases in which blood cultures are repeatedly sterile, a dosage of 6,000,000 to 10,000,000 units of penicillin daily for 21 days is advisable.High bacterial resistance to penicillin and streptomycin was found in four fatal cases. In one of these, the infecting organism was streptococcus viridans, and in three it was staphylococcus albus. There was one patient with penumococcal meningitis complicated by unrecognized streptococcal viridans bacterial endocarditis.     

Kawasaki disease,or mucocutaneous lymph node syndrome: report of seven cases in North America.

The clinical and laboratory findings in seven children with Kawasaki disease are reviewed. Four of the patients had the more complicated course that has characterized the cases diagnosed in North America. This suggests that the benign forms are often mistaken for other febrile illnesses. The patients were two girls and five boys ranging in age from 4 months to 7 years; six were Caucasian and one was a North American Indian. Fever, redness of the oral mucosa, an erythematous or scarlatiniform rash and cervical adenopathy were seen in all; six patients had the characteristic fingertip desquamation and nonexudative conjunctivitis. Cardiac involvement occurred in four patients, two of whom had coronary artery aneurysm or thrombosis. Arthritis or arthralgia was seen in six patients, and aseptic meningitis occurred in four. Of the three patients with jaundice two underwent laparotomy and excision of a hydropic gallbladder; one of them died from Klebsiella pneumoniae sepsis and disseminated intravascular coagulopathy.     

Plasmapheresis in the initial treatment of insulin-dependent diabetes mellitus in children.

Several factors indicate that autoimmune mechanisms may play a part in the aetiology of insulin-dependent diabetes mellitus. At the onset of the disease in 10 children (aged 11-16 years) plasmapheresis was performed four times over one to two weeks. Seventeen age-matched children with the same clinical features served as controls. The C-peptide concentrations at onset were the same in the two groups, but after one month the children treated with plasmapheresis had significantly higher values. This difference became even more pronounced after three, nine, and 18 months, both during fasting and at the maximum response to a standardised meal. The study group also had a significantly more stable metabolism, longer partial remission, and no higher insulin requirement. Of the 10 treated children islet-cell cytoplasmic antibodies were present in seven before plasmapheresis and in nine during treatment. The antibodies remained detectable in five and six out of nine patients at one and six months respectively after plasmapheresis. Although the mechanisms are obscure, plasmapheresis performed at the onset of insulin-dependent diabetes mellitus may help to preserve beta-cell function.     

Myasthenia Gravis: Comparative Evaluation of Medical and Surgical Treatment

Sixty-eight patients with myasthenia gravis were evaluated and compared to determine the results of medical and surgical treatment; eight patients with thymoma were evaluated separately. In the group of 30 non-thymoma patients treated medically 50% of patients derived moderate to good improvement over a mean follow-up period of 11 years. Ten per cent of patients in this group died from myasthenia.In the group of 30 non-thymoma patients treated by thymectomy, 83% achieved good to excellent improvement. There was no surgical or myasthenic mortality over a mean follow-up period of nine years.The results of treatment in the eight thymoma patients were decidedly inferior and there was no significant difference between the medically and surgically treated patients. Fifty per cent showed only moderate improvement during a mean follow-up of five years and 50%, after initial improvement, deteriorated later and died from myasthenia between three and four years after thymectomy.Two additional patients had thymoma without myasthenia. Neither of them had developed myasthenia, two years following thymectomy in one case and after 25 years in the other, despite recurrence of the tumour with extensive invasiveness in the very long-standing case.     

Mutation Spectrum of RB1 Gene in Unilateral Retinoblastoma Cases from Tunisia and Correlations with Clinical Features

Retinoblastoma, an embryonic neoplasm of retinal origin, is the most common primary intraocular malignancy in children. Somatic inactivation of both alleles of the RB1 tumor suppressor gene in a retinal progenitor cell through diverse mechanisms including genetic and epigenetic modifications, is the crucial event in initiation of tumorigenesis in most cases of isolated unilateral retinoblastoma. We analyzed DNA from tumor tissue and from peripheral blood to determine the RB1 mutation status and seek correlations with clinical features of 37 unrelated cases of Tunisian origin with sporadic retinoblastoma. All cases were unilateral except one who presented with bilateral disease, in whom no germline coding sequence alteration was identified. A multi-step mutation scanning protocol identified bi-allelic inactivation of RB1 gene in 30 (81%) of the samples tested. A total of 7 novel mutations were identified. There were three tumors without any detectable mutation while a subset contained multiple mutations in RB1 gene. The latter group included tumors collected after treatment with chemotherapy. There were seven individuals with germline mutations and all presented with advanced stage of tumor. There was no difference in age of onset of RB based on the germline mutation status. Thus 20% of the individuals with sporadic unilateral RB in this series carried germline mutations and indicate the importance of genetic testing all children with sporadic retinoblastoma. These findings help to characterize the spectrum of mutations present in the Tunisian population and can improve genetic diagnosis of retinoblastoma.     

Canine parvovirus enteritis, canine distemper, and major histocompatibility complex genetic variation in Mexican wolves

The endangered Mexican wolf (Canis lupus baileyi) was recently reintroduced into Arizona and New Mexico (USA). In 1999 and 2000, pups from three litters that were part of the reintroduction program died of either canine parvovirus or canine distemper. Overall, half (seven of 14) of the pups died of either canine parvovirus or canine distemper. The parents and their litters were analyzed for variation at the class II major histocompatibility complex (MHC) gene DRB1. Similar MHC genes are related to disease resistance in other species. All six of the surviving pups genotyped for the MHC gene were heterozygous while five of the pups that died were heterozygous and one was homozygous. Resistance to pathogens is an important aspect of the management and long-term survival of endangered taxa, such as the Mexican wolf.     

Successful Pregnancy Following Strassmann Metroplasty

In a study of 44 infants and young children with staphylococcal pneumonia and pleurisy, cases were found to be divisible into three clinical groups: those with predominantly digestive tract symptomatology (seven); those in acute respiratory distress (28); and those with signs of central nervous system disorder (nine). Characteristic radiographic findings often permitted an early etiologic diagnosis, confirmed later by bacteriologic culture or by the course of the disease. The length of hospitalization was found to be directly proportional to the length of time between the onset of symptoms and the start of adequate therapy. Nineteen patients required thoracotomy, two were drained by a Küss trocar, 13 required repeated pleural aspiration, five underwent spontaneous resorption of the empyema and five had no pleural effusion. In several instances antibiotics were used in very high dosage (three to four times the suggested maximal dose). The main agents used were erythromycin, chloramphenicol and ristocetin; the dosage of the last named never exceeded the maximal recommended dose. Twenty-eight patients were less than one year old; of these seven died. Sixteen were over one year of age, the oldest being six years; all of these were cured.     

Bronchiectasis in acute leukaemia.

Five children in remission from acute lymphoblastic leukaemia developed bronchiectasis when on chemotherapy. Persistent collapse or consolidation on chest radiographs was helpful in suggesting the diagnosis. Necropsy established the diagnosis in one child who died of massive haemoptysis when in complete remission, and bronchography confirmed the diagnosis in three. In a further child the diagnosis was based on clinical and chest X-ray findings alone. The surviving children were treated with prophylactic rotating antibiotics. Routine chest radiographs are recommended in children with acute lymphoblastic leukaemia, as bronchiectasis may otherwise be underdiagnosed.     

Long-term parenteral nutrition

Nineteen patients (11 women and eight men) aged 20-68 received long-term parenteral nutrition, mostly at home, for six to 63 months (mean 19 months). Indications for LTPN were extensive, active Crohn''s disease in three patients, intestinocutaneous fistulas in three, and short-bowel syndrome in the remaining 13 patients. Subclavian or intra-atrial (Broviac) catheters were most commonly used, for which the average life was four and seven months respectively. Complications of long-term parenteral nutrition included pneumothorax in four out of 48 subclavian vein punctures. Catheter-induced thrombosis of central veins was shown by phlebography 17 times in nine patients, and eight episodes of total occlusion occurred. Two of these patients had pulmonary infarction. Nineteen episodes of catheter sepsis occurred in 11 patients, but only one was fatal. Complications related to intestinal disease included intra-abdominal abscesses and intestinal fistulas, and disturbances of liver function. Five patients died, though in only two was death related to long-term parenteral nutrition. One of these patients died from catheter sepsis, the other had subdural haematoma possibly caused by anticoagulant treatment. Eight of the 14 surviving patients still needed parenteral nutrition. All received a disability pension, but six had an acceptable quality of life with almost normal social activities.Despite problems such as difficulties in maintaining standardised infusion programmes, it was concluded that long-term parenteral nutrition at home is practicable and consistent with an acceptable quality of life.     

重度颅脑损伤合并神经原性肺水肿机械通气的观察与护理

目的：探讨重型颅脑损伤合并神经原性肺水肿（NPE）病人机械通气的护理经验,提高此类病人的治疗成功率。方法：回顾性分析2000年～2007年18例颅脑损伤患者合并NPE的临床护理资料,对这些患者进行了机械通气治疗,观察和总结护理的临床效应。结果：18例患者中肺水肿消退11例,死亡7例。11例存活患者经过随访,死亡4例,植物生存1例,重残1例,中残2例,好3例。结论：重型颅脑损伤合并NPE预后不良,死亡率高;机械通气是治疗NPE的有效方法,正确积极的护理可提高患者的治愈率。     

Cyclophosphamide Therapy in the Nephrotic Syndrome in Childhood

Forty-six children with the nephrotic syndrome whose renal biopsy specimens showed minimal changes and whose response to corticosteroid therapy was unsatisfactory were treated with cyclophosphamide. Three patients were completely steroid-resistant from the outset and the remainder were steroid-dependent. In several patients steroids controlled the condition less effectively with time. Most patients showed signs of steroid toxicity, and growth retardation was striking.A moderate leucopenia was induced with cyclophosphamide, and treatment was maintained for three to four months in the majority of cases. Thirty-eight children (83%) have remained in complete remission off all treatment for periods of 3 to 23 months, 33 after one course of cyclophosphamide and five after a second course. Two other patients who remitted but relapsed later are still on treatment. In only six patients was full remission not obtained, and three of these were steroid-resistant from the start. Two died from pneumonia and adrenal failure and four continued to have proteinuria, though in one an impressive reduction occurred.The results indicate that cyclophosphamide therapy is an effective alternative for nephrotic children with normal glomeruli on light microscopy who develop steroid dependence or resistance, and who exhibit toxic effects of steroid therapy.     

Survival of captive and free-ranging Harlequin Ducks (Histrionicus histrionicus) following surgical liver biopsy

We measured intra- and postoperative mortality rates of captive and free-ranging Harlequin Ducks (Histrionicus histrionicus) undergoing surgical liver biopsy sampling for determination of the induction of cytochrome P4501A, a biomarker of oil exposure. Liver biopsies were taken from and radio transmitters were implanted into 157 free-ranging Harlequin Ducks over three winters (55 in 2000, 55 in 2001, and 47 in 2002). No birds died during surgery, but seven (4.5%) died during recovery from anesthesia (three in 2001 and four in 2002). None of the deaths could be attributed directly to the liver biopsy. Four of the 150 (2.7%) birds that were released died in the 2 wk period after surgery. All post-release deaths occurred in 2001; no birds died after release in 2000 or 2002. No mortalities of 36 captive birds occurred during surgery or recovery or in the 2 wk period following surgery. Hemorrhage was a minor problem with one captive bird. Surgical liver biopsies appear to be a safe procedure, but anesthetic complications may occur with overwintering ducks.     

Surgical treatment of infective endocarditis with special reference to prosthetic valve endocarditis.

Patients with native valve endocarditis treated surgically between 1968 and 1978 (n = 15) and all patients presenting with prosthetic valve endocarditis during this period (n = 21) were followed up for at least four years. Five of the patients with native valve endocarditis required urgent early surgical intervention, of whom two died. The remaining 10 underwent valve replacement after a course of antibiotic treatment: all survived, though one required further valve replacement. The 21 patients with prosthetic valve endocarditis suffered 25 attacks. Nine were cured by medical treatment alone; two died before surgical intervention was possible; 11 required valve replacement, of whom three died; and two required valve replacement after a course of antibiotic treatment. The incidence of early prosthetic valve endocarditis--that occurring within two months of operation--was 0.67%, but that of late prosthetic valve endocarditis could not be determined. Medical treatment when started early should cure endocarditis in most patients, but vigilance should be maintained for the appearance of indications for surgery. When such indications exist surgery should not be delayed.