Exchange transfusion to reverse severe pertussis-induced cardiogenic shock

Severe pertussis has a high mortality risk, especially in those with high white blood cell counts and pulmonary hypertension. Exchange transfusion can reduce the leukocyte mass in blood. We report 3 young infants with severe pertussis and hyperleukocytosis who developed cardiogenic shock and pulmonary hypertension. Exchange transfusion was performed. The white blood cell count decreased accompanied by improvement in the cardiopulmonary condition and survival in the 3 infants. Exchange transfusion should be considered in patients with severe pertussis with hyperleukocytosis.     

Malignant pertussis in the young infant]

Pertussis remains in France the first cause of bacterial, infectious death in infant aged 10 days to 2 months. It is especially in this age group that malignant pertussis occurs. CASE REPORT: A 40-day-old infant was admitted in the intensive care unit with symptoms of bronchiolitis along with a 200 bpm permanent tachycardia. He presented a marked leukocytosis with lymphocytosis. On the second day, convulsions and coma occurred, followed rapidly by respiratory failure, with a subsequent deterioration due to the development of severe pulmonary hypertension. Circulatory failure caused the infant's death on the beginning of the 5th day. Pertussis was confirmed by PCR on nasopharyngeal swab. Intra-familial contamination was most likely. COMMENTS: Malignant pertussis is characterized by the very young age of patients, permanent tachycardia sine materia, dyspnea with early respiratory failure, frequent neurological symptoms, severe hyperleukocytosis and hyperlymphocytosis, and deep hyponatremia with oliguria and edema. Mortality remains superior to 75% despite the various treatments and life support measures that have been attempted. Adult pertussis, which represents one third of the cases of prolonged cough in this age group, is the main source of contamination of non-immunized young infants. This mode of transmission stresses the importance of the generalization of pertussis vaccine booster in early adolescence, which is recommended in France since 1998. Its extension towards the adult age is under study.     

Laboratory-confirmed reinfections with Bordetella pertussis

Susceptibility to infection with Bordetella pertussis re-emerges several years after pertussis vaccination. However, the duration of immunity after natural infection with B. pertussis, postulated to be lifelong, is not known. In an ongoing study, the longitudinal course of pertussis antibodies in patients who have had laboratory-confirmed pertussis is being followed using sera obtained at irregular intervals. In 4 patients a reinfection with Bordetella pertussis is described respectively 7 (patient A), 12 (patients B and C) and 3.5 (patient D) y after the first infection. It seems that the longer the interval between the infections the more severe the complaints. Conclusion: To the authors' knowledge. these are the first patients in whom symptomatic reinfection with B. pertussis has definitely been proven by laboratory confirmation of both episodes. Bordetella pertussis infection should be considered in patients with symptoms of typical or atypical whooping cough, irrespective of their vaccination status or previous whooping cough.     

Early complications in children with acute lymphoblastic leukemia presenting with hyperleukocytosis

BACKGROUND: The optimal management of childhood acute lymphoblastic leukemia (ALL) with hyperleukocytosis is unclear, largely because the risk of leukostasis-related complications is poorly characterized. PROCEDURE: We reviewed the presenting characteristics, initial management, and frequency and type of complications in all children seen at St. Jude Children's Research Hospital with previously untreated ALL and an initial leukocyte count >200 x 10(9)/L. RESULTS: A total of 178 children, representing 8% of all children with ALL, had an initial leukocyte count >200 x 10(9)/L; 67 patients had a leukocyte count >400 x 10(9)/L. Sixteen patients (9%) had neurological complications with 12 of these patients experiencing symptoms at presentation. Four patients (2%), all with initial leukocyte counts >400 x 10(9)/L, suffered a CNS hemorrhage. Pulmonary leukostasis occurred in 11 patients (6%). The degree of hyperleukocytosis was significantly predictive of neurological (P = 0.006) and respiratory (P = 0.014) complications. The majority of complications occurred at presentation. Cytoreduction (94 patients) decreased the leukocyte count but delayed initiation of chemotherapy (P = 0.013). CONCLUSIONS: Serious leukostasis-related complications are relatively uncommon in childhood ALL and most occur at presentation. Their incidence increases in proportion to the leukocyte count. A large subset of cases can be managed successfully without cytoreduction. Cytoreduction may be considered for patients with leukocyte counts >400 x 10(9)/L or patients who have complications at presentation.     

Cerebral edema and priapism in an adolescent with acute lymphoblastic leukemia

Priapism and increased intracranial pressure are both rare, but recognized, manifestations of leukemia. However, they have never been reported in the same patient. We report a 15-year-old male with acute lymphoblastic leukemia who presented with hyperleukocytosis, priapism, and increased intracranial pressure. Central nervous system leukostasis and cerebral edema may have been detected earlier, had his history of priapism been known. Management of hyperleukocytosis complicated by priapism and increased intracranial pressure is discussed.     

"Pseudoleukemia" in a newborn infant with trisomy 21]

The increased incidence of leukemia in patients with trisomy 21 is well established. A blood disorder which may be mistaken for acute leukemia may occur in infants with trisomy 21. The authors report a newborn with trisomy 21 and hematologic findings suggesting acute leukemia (hyperleukocytosis, anemia, thrombocytopenia; replacement of bone marrow by blast-like cells). Without treatment spontaneous and complete recovery occurred. Cytochemistry and therapy are discussed in this paper.     

Transitorisch myeloproliferatives Syndrom bei Neugeborenen mit Down-Syndrom

Transient myeloproliferative syndrome (TMS) is a disorder of neonates with Down syndrome (trisomy 21) and occurs at an incidence of up to 10%. Typical for this entity is a proliferation of myeloid blasts with megakaryoblastic and/or erythroblastic features detectable in the peripheral blood. In the majority of cases TMS is asymptomatic, and only a small percentage of children show clinical symptoms due to hyperleukocytosis, thrombocytopenia, anemia, or hepatomegaly. Almost all children will achieve spontaneous remission after 2–4 months (disappearance of myeloid blasts and regression of clinical symptoms). Patients with initial hyperleukocytosis and hepatomegaly, however, carry a particular risk of developing hepatopathy followed frequently by lethal liver fibrosis. Chemotherapy with cytarabine is indicated in patients with clinical symptoms of hyperleukocytosis or early signs of hepatopathy. Within the first 4 years the risk of developing acute myeloblastic leukemia (AML) is high at 20–30%; therefore, regular follow-ups are advised. This analysis is based on one of the largest databases for TMS.     

Pertussis--an illness with typical clinical symptoms?]

There has been a noticeable increase in the incidence of pertussis in West Germany over the last decade. Since the availability of adequate bacteriological diagnosis a much broader clinical spectrum can be attributed to infections with B. pertussis. Three patients with an unusual clinical presentation of pertussis are presented. A three month old infant presented with severe apneic spells without cough as the sole clinical symptoms of the infection. B. pertussis was isolated in the nasopharyngeal swab. A nine month old premature infant with bronchopulmonary dysplasia after long time intubation and artificial ventilation presented with apneic spells, pulmonary and cardiac decompensation and required ventilatory support. The diagnosis was suggested by a massive leucocytosis with lymphocytosis. The diagnosis on the patient was established by serologic methods. Adult contacts of this patient developed longstanding cough and clinical signs of pertussis. The diagnosis of pertussis in these persons was established by nasopharyngeal culture. The third patient with trisomy 21 and a corrected AV canal suffered from nonspecific cough and gradually developed signs of congestive heart failure with pneumonia. B. pertussis was isolated from the nasopharynx. This patient showed neither the typical paroxysmal coughing spells nor disclosed the typical lymphocytosis in his white blood count. Microbiological investigations of patients with symptoms of respiratory tract infections should include the isolation of B. pertussis. Thus, additional cases of pertussis not suspected on the basis of their initial clinical presentation will be detected.     

Spontaneous remission of congenital acute nonlymphoblastic leukemia with normal karyotype in twins

BACKGROUND: Congenital acute nonlymphoblastic leukemia (cANLL) is an extremely rare event and represents only 0.5-1% of the leukemias in the first year of life. It is usually more common among patients with chromosomal abnormalities. Transient myeloproliferative disease (TMD) is an hyperleukocytosis entity that occurs almost exclusively in Down syndrome patients and remits spontaneously. Spontaneous remission of congenital leukemia has been reported and related to the presence of an extra chromosome 21. PROCEDURE: A pair of non-Down syndrome newborn twins presented with a clinical picture of skin rash and hyperleukocytosis. Twin B had full-blown cANLL with bone marrow, peripheral blood, skin, CSF, and placental invasion. Twin A presented transient peripheral blood and skin involvement by the same type of blast cells. No cytotoxic therapy was given. With 2 years follow-up, they continue to do well. RESULTS: Histologic and immunophenotypical analysis of placentas, cord blood, skin, CSF, bone marrows, and peripheral blood revealed a consistent picture of intrautero cANLL in twin B, with transplacental invasion of twin A. Normal and blast cells were found to be karyotypically normal. Spontaneous remission occurred. CONCLUSIONS: cANLL with karyotypically normal blasts can develop a self-limited clinical course, which has resemblances to TMD.     

ADad 3: The Epidemiology of Anxiety Disorders Among Adolescents in a Rural Community Population in India

Hyperleukocytosis is defined as peripheral blood leukocyte count exceeding 100,000/mm³. Acute leukemia is the most common etiology in pediatric practice. Hyperleukocytosis is a medical emergency. The increased blood viscosity, secondary to high white cell count and leukocyte aggregates, results in stasis in the smaller blood vessels. This predisposes to neurological, pulmonary or gastrointestinal complications. In addition, patients are at risk for tumor lysis syndrome due to the increased tumor burden. Initial management includes aggressive hydration, prevention of tumor lysis syndrome, and correction of metabolic abnormalities. A red cell transfusion is not indicated in a hemodynamically stable child, as it adversely affects the blood viscosity. Leukapheresis is the treatment of choice for a very high count, or in patients with symptomatic hyperleukocytosis. The technical expertise required, a relative difficult venous access in younger children, risk of anticoagulation and possible non-availability of the procedure in emergency hours are limitations of leukapheresis. However, it is a rewarding procedure and performed with relative ease in centers that perform the procedure frequently. An exchange transfusion is often a practical option when hyperleukocytosis is complicated with severe anemia. The partial exchange aids in correcting both, without the risk of volume overload or hyperviscosity, which are the limitations of hydration and blood transfusion, respectively. Etiology and management of hyperleukocytosis in relevance to the pediatric emergency room is outlined.     

First experience of the AML‐Berlin‐Frankfurt‐Münster group in pediatric patients with standard‐risk acute promyelocytic leukemia treated with arsenic trioxide and all‐trans retinoid acid

Recently, studies in adults with acute promyelocytic leukemia (APL) showed high cure rates in low‐risk patients treated with all‐trans retinoid acid (ATRA) and arsenic trioxide (ATO), while toxicities were significantly reduced compared to the standard treatment with ATRA and chemotherapy. Here we report about first experience with 11 pediatric patients with low‐risk APL treated with ATRA and ATO. All patients stayed in molecular remission. All suffered from hyperleukocytosis. Two patients experienced reversible severe side effects. One suffered from osteonecroses at both femurs, seizures, as well as posterior reversible encephalopathy syndrome, the other patient had an abducens paresis.     

Bordetella pertussis and mixed infections

AIM: In pertussis-like respiratory infections, once pertussis has been laboratory confirmed, other potential causative pathogens will seldom be looked for. Probably most mixed infections are found accidentally and since these mixed infections might cause a more severe disease we performed a retrospective study of their incidence. METHODS: We selected from 2 groups of patients with serologically confirmed Bordetella (B.) pertussis infection those in whom serology for other respiratory pathogens had also been performed. Group 1 consisted of 50 pertussis patients with 51 episodes of B. pertussis infection selected from 100 patients with serologically confirmed pertussis. They participated in a long-term follow-up after a B. pertussis infection. In group 2, 31 pertussis patients were selected from 98 consecutive patients with positive pertussis serology from one routine practice. RESULTS: In 23 of 82 pertussis infections (28%) serological evidence of 1 (n = 21) or 2 (n = 2) additional infections were demonstrated. These involved para-influenza virus (n = 6), respiratory syncytial virus (RSV) (n = 6), Mycoplasma pneumoniae (n = 5), adenovirus (n = 4), influenza A virus (n = 3) and influenza B virus (n = 1). CONCLUSIONS: We conclude that in patients with B. pertussis infection, coinfection with another respiratory pathogen is often present.     

The role of adenoviruses in the pertussis syndrome.

To define the role of adenoviruses in the pertussis syndrome, a study was done of a group of 134 children with clinical pertussis and a healthy control population of similar age, race, sex, and socioeconomic status. Adenovirus infections occurred in 30 (22.4%) of 134 patients with the pertussis syndrome and 5 (4.9%) of 101 control subjects (p smaller than 0.001). B. pertussis was recovered from 46 (34.3%) patients, and from 18 (39.1%) of these patients adenoviruses were also isolated. Although adenovirus infections also occurred in patients with the pertussis syndrome with negative cultures for B. pertussis, the rate, 12 of 88 patients (13.6%), was significantly lower (p smaller than 0.001). The clinical course was similar irrespective of the results of bacterial or viral cultures. These data substantiate the frequent association of adenoviruses with the pertussis syndrome, It would appear that adenoviruses do not usually have an independent role in the pathogenesis of the pertussis syndrome since we found them so commonly to be one agent in a mixed infection.     

Pertussis outbreak in an elementary school with high vaccination coverage.

BACKGROUND: An outbreak of pertussis in a US elementary school with high vaccination coverage was investigated to evaluate vaccine effectiveness and to identify potential contributing factors. METHODS: Survey and cohort study of all 215 students of an elementary school (including 36 case patients) and 16 secondary cases among contacts. RESULTS: Fifty-two pertussis cases were identified (attack rate among students, 17%). Receipt of <3 doses of pertussis-containing-vaccine compared with receipt of complete vaccination series was a significant risk factor for pertussis [relative risk, 5.1; 95% confidence interval (CI), 3 to 8.6]. The effectiveness of the complete vaccination series was 80% (95% CI 66 to 88). No evidence of waning immunity among students was found. The following contributing factors for the outbreak were identified: multiple introductions of pertussis from the community; delays in identification and treatment of early cases; and high contact rates among students. Antimicrobial treatment initiated >14 days after cough onset was associated with increased risk of further transmission of pertussis (relative risk, 10.1; 95% CI 1.5 to 70.3) compared with treatment within 14 days of onset. CONCLUSIONS: This investigation demonstrated the potential for pertussis outbreaks to occur in well-vaccinated elementary school populations. Aggressive efforts to identify cases and contacts and timely antimicrobial treatment can limit spread of pertussis in similar settings. High vaccination coverage should be maintained, because vaccination significantly reduces the risk of the disease throughout the elementary school years, and to ensure timely diagnosis and treatment health care providers should maintain a high index of suspicion for pertussis among elementary school age children.     

Respiratory viral infections in infants with clinically suspected pertussis

Objectiveto evaluate the frequency of respiratory viral infections in hospitalized infants with clinical suspicion of pertussis, and to analyze their characteristics at hospital admission and clinical outcomes.Methodsa historical cohort study was performed in a reference service for pertussis, in which the research of respiratory viruses was also a routine for infants hospitalized with respiratory problems. All infants reported as suspected cases of pertussis were included. Tests for Bordetella pertussis (BP) (polymerase chain reaction/culture) and for respiratory viruses (RVs) (immunofluorescence) were performed. Patients who received macrolides before hospitalization were excluded. Clinical data were obtained from medical records.ResultsAmong the 67 patients studied, BP tests were positive in 44%, and 26% were positive for RV. There was no etiological identification in 35%, and RV combined with BP was identified in 5%. All patients had similar demographic characteristics. Cough followed by inspiratory stridor or cyanosis was a strong predictor of pertussis, as well as prominent leukocytosis and lymphocytosis. Rhinorrhea and dyspnea were more frequent in viral infections. Macrolides were discontinued in 40% of patients who tested positive for RV and negative for BP.Conclusionthe results suggest that viral infection can be present in hospitalized infants with clinical suspicion of pertussis, and etiological tests may enable a reduction in the use of macrolides in some cases. However, the etiological diagnosis of respiratory virus infection, by itself, does not exclude the possibility of infection with BP.     

Multiple intracranial tumors in Philadelphia chromosome positive acute lymphoblastic leukemia: successful treatment following aggressive supportive care, early cranial radiation, high dose chemotherapy and imatinib

We report the first case of multiple intracranial tumors ("chloromas") at diagnosis of Philadelphia chromosome positive acute lymphoblastic leukemia. The patient presented comatose with signs of cerebral herniation. Initial management of raised intracranial pressure and hyperleukocytosis followed by emergent whole brain radiation therapy reversed the life-threatening neurological signs. High-dose chemotherapy combined with daily imatinib mesylate induced a rapid and sustained bone marrow remission. Ongoing rehabilitation resulted in a near complete neurological recovery within 6 months of diagnosis. This outcome justifies aggressive early management of increased intracranial pressure and hyperleukocytosis in future similar presentations.     

The role of antibiotics, immunizations, and adenoviruses in pertussis.

Sixty-five patients with pertussis were identified by a clinical criterion, and Bordetella pertussis was isolated from 75% of these patients or their symptomatic household contacts. Negative nasopharyngeal cultures were usually associated with either a history of antibiotic therapy with erythromycin or tetracycline (two of three patients), two or more diphtheria and tetanus toxoids with pertussis (DTP) vaccines (six of eight patients), or both (two of three patients). Erythromycin therapy resulted in the elimination of B. pertussis from the nasopharynx in 2 to 7 days (mean, 3.6 days) compared with 7 to 17 or more days (mean, greater than 12 days) in patients treated with no antibiotics, but had no effect on the duration or severity of illness as judged by length of hospitalization. Adenoviruses were recovered from five of 44 patients cultured. Four of these isolates were from throat swabs obtained early in the illness and the remaining isolate was from one of 33 repeated viral cultures obtained two to three weeks later; B. pertussis was also isolated from these five patients. Paired serum samples were obtained from only two of these patients. Neither demonstrated a fourfold rise in adenoviral complement-fixing antibodies. Therefore, in these patients, adenoviral isolation may have been secondary to reactivation of a latent viral infection by infection with B. pertussis.     

百日咳患儿临床特点及危险因素分析

目的 探讨百日咳患儿的临床特点及感染的危险因素。方法 回顾性对比分析253例百日咳住院患儿（百日咳组）与同期314例咳嗽住院患儿（对照组）的临床资料及免疫功能相关实验室指标结果。百日咳鲍特菌DNA检测采用实时荧光PCR法。收集两组患儿疫苗免疫、可疑患者接触史及住址分布的信息行百日咳危险因素分析。结果 对两组患儿进行23项典型临床指标比较,结果百日咳组患儿仅10项典型临床指标不同于对照组（P < 0.05）。两组患儿并发症中,仅百日咳组心肌炎发生率低于对照组（P < 0.05）。百日咳组患儿血清球蛋白、IgM水平显著低于对照组（P < 0.05）。百日咳组患儿百白破联合疫苗未免疫、未及时免疫及可疑患者接触史比率显著高于对照组（P < 0.05）;疫苗未免疫、未及时免疫、可疑患者接触史是百日咳感染的危险因素（P < 0.05）。结论 百日咳患儿常不具备典型的临床特征,实时荧光PCR法检测百日咳鲍特菌DNA有助于不典型百日咳患儿的早期诊断。婴幼儿应及时进行百日咳疫苗免疫接种,并与可疑患者做好隔离防护,降低百日咳发生率。     

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??Objective??To explore the clinical characteristics of coinfections in children with pertussis. Methods??From February 2016 to September 2017??198 cases with pertussis-like symptom were tested for PCR??bacterial culture??respiratory virus antigen and serum mycoplasma pneumoniae antibody in Children’s Hospital of Soochow University. Results??Totally 198 patients were enrolled and 105 patients were B.Pertussis positive. Single infection was in 37 cases??35.2%??. Coinfections were observed in 68??64.8%?? children with pertussis??including co-infection with one pathogen in 51 cases??75.0%??. The most frequent co-infection pathogen was rhinovirus??50.9%??26 cases????followed by Mycoplasma pneumoniae??13.7%??7 cases?? and Streptococcus pneumoniae??11.8%??6 cases??. There was no statistical difference in the coinfection rate among different age groups??P = 0.08??. Pertussis coinfection with MP was increased with age. Coinfections patients were older than those with single infections???11.77±2.32?? months vs. ??6.74±8.07?? months??P = 0.017??. Fever??dyspnea??and positive signs of lung in chest imang were more common in children with mixed infections??0 vs. 10.3%??20.6% vs. 5.4%??76.5% vs. 36.4%??P??0.05??. Chest imaging showed pathy shadow in most cases. There was no significant difference in lab tests??such as white blood cell counts??neutrophil counts??C-reactive protein??CRP????course of disease prior to admission or hospital stay between patients with pertussis only and those with mixed-pathogen infections??P??0.05??. Patients older than 3 months??OR??3.0??95%CI 1.1-8.5??P??0.03?? and fever??OR??2.5??95%CI 1.1-6.7??P??0.03?? were the independent risk factors for mixed infections. Conclusion??There is a higher proportion of coinfection in hospitalized children with pertussis??most commonly co-infected with rhinovirus??followed by Mycoplasma pneumoniae and Streptococcus pneumoniae. Coinfections are found to aggravate pertussis. Fever and being older than 3 months are risk factors of mixed infection.     

Acute lymphoblastic leukemia with hyperleukocytosis, sinus pauses, and hypoxemic respiratory failure in an infant

A 5-month-old female infant presented with new-onset acute lymphoblastic leukemia and hyperleukocytosis with white blood cell count of 352 × 10(9) cells/L. She developed sinus pauses and hypoxemic respiratory failure. A manual single volume exchange blood transfusion was done with complete resolution of sinus pauses and hypoxemia.