Neonatal toxic shock syndrome-like exanthematous disease (NTED)

The author and colleagues recently discovered an emerging neonatal infectious disease: neonatal toxic shock syndrome-like exanthematous disease (NTED), which is induced by the superantigen toxic shock syndrome toxin-1 (TSST-1), produced by methicillin-resistant Staphylococcus aureus (MRSA). The massively expanded Vbeta2+ T cells were rapidly deleted in the peripheral blood of patients with NTED. A marked depletion of Vbeta2+ T cells was also observed in the peripheral blood before the expansion of these T cells. Anergy is specifically induced in the TSST-1 reactive T cells of patients with NTED. Rapid recovery from NTED without complications is expected to be related to the induction of immunologic tolerance in neonatal patients. Anti-TSST-1 IgG antibody of maternal origin was found to play a protective role in preventing the development of NTED. The number of hospitals that have experience caring for patients with NTED has increased threefold in the past 5 years. Most MRSA isolates from neonatal intensive care units in Japan were found to be a single clone of coagulase type II and to possess TSST-1 and staphylococcal enterotoxin C genes. The timing and increased incidence of NTED suggest the emergence of a new MRSA clone. By recognizing that TSST-1 can induce NTED, healthcare providers may give increased attention to this disease in neonatal wards.     

Toxic shock syndrome in burns: diagnosis and management

Toxic shock syndrome (TSS), a toxin-mediated disease, is the most common cause of unexpected mortality in children with small burns. It is a diagnosis that is often missed because of non-specific signs and an ability to mimic other childhood illnesses. Any child with a pyrexia greater than 38.9 degrees C, a rash, or a sudden change in clinical condition within a few days of a burn injury should be monitored closely for TSS. If there is co-incident hyponatraemia or lymphopaenia, or if there is any deterioration in clinical condition, the child should be managed with anti-staphylococcal and streptococcal antibiotics and passive immunity for toxins provided by fresh frozen plasma (FFP) or intravenous immunoglobulin (IVIG). It is essential that all paediatric and emergency departments accepting children with burns are aware of the symptoms, signs and early management of TSS.     

Genetic basis of neonatal methicillin-resistant Staphylococcus aureus in Japan

Methicillin-resistant Staphylococcus aureus (MRSA) is still one of major problems of drug-resistant microorganisms and healthcare-acquired infections. Methicillin-resistant Staphylococcus aureus is highly prevalent in patients in neonatal intensive care units (NICU) in Japan. The most predominant MRSA in NICU is multidrug resistant and produces superantigenic exotoxin, toxic shock syndrome toxin-1 (TSST-1) and staphylococcal enterotoxin C (SEC). These predominant MRSA strains belong to coagulase type II, SCCmec type II, mecA-Tn554 polymorph type I-A and show closely related pulse field gel electrophoresis types. The dissemination of MRSA is wide, and there is a pandemic distribution of a single MRSA clone in the NICU of Japan. Since 1992, the nationwide spread of this clone has also led to the development of a new neonatal disease known as neonatal toxic shock-like exanthematous disease (NTED), which is caused by overactivation of vbeta2+ T cells induced by TSST-1. The spread of MRSA in NICU in Japan has been attributed to overcrowding, high rates of extremely low birthweight babies, understaffing, low control measures of infection and overuse of antibiotics. The environment of NICU and infection control intervention should be improved and a new strategy for control like vaccination or probiotics is required.     

Ilio-psoas abscess caused by methicillin-resistant Staphylococcus aureus (MRSA): a rare but potentially dangerous condition in neonates

We report a case of methicillin-resistant Staphylococcus aureus ilio-psoas abscess (IPA) in a neonate. This case has clinical importance because this neonate had toxic shock syndrome-like exanthematous disease, known as NTED, before developing IPA. A high index of suspicion is required for IPA if a neonate presents with limb disuse and fever of unknown origin. Our case required surgical drainage, since ultrasound-guided percutaneous needle aspiration failed.     

DIAGNOSTIC AND THERAPEUTIC ASPECTS OF DIHYDROBIOPTERIN DEFICIENCY

ABSTRACT. The first Scandinavian hyperphenyialaninaemic patient with a cofactor deficiency is described. By neonatal screening the Guthrie test showed a serum phenylalanine of 302 μmol/I (5 mg/dl), which at age 6 weeks had fallen to high normal values. At age 5 months the serum phenylalanine was around 2000 μmol/l and the child presented with severe neurological symptoms. The diagnosis of defect dihydrobiopterin biosynthesis was made by high performance liquid chromatography of the urine. Loading tests followed by daily treatment of the missing cofactor was able to keep the serum phenylalanine in the normal level. Because of persisting, yet diminishing neurological symptoms neurotransmitter treatment was started. Breast feeding as the cause of the low neonatal levels of serum phenylalanine and the late start of clinical symptoms is proposed and the importance of screening all hyperphenylalaninaemic newborns for defect biopterin metabolism is stressed.     

Diagnostic and therapeutic aspects of dihydrobiopterin deficiency

The first Scandinavian hyperphenylalaninaemic patient with a cofactor deficiency is described. By neonatal screening the Guthrie test showed a serum phenylalanine of 302 mumol/1 (5 mg/dl), which at age 6 weeks had fallen to high normal values. At age 5 1/2 months the serum phenylalanine was around 2000 mumol/1 and the child presented with severe neurological symptoms. The diagnosis of defect dihydrobiopterin biosynthesis was made by high performance liquid chromatography of the urine. Loading tests followed by daily treatment of the missing cofactor was able to keep the serum phenylalanine in the normal level. Because of persisting, yet diminishing neurological symptoms neurotransmitter treatment was started. Breast feeding as the cause of the low neonatal levels of serum phenylalanine and the late start of clinical symptoms is proposed and the importance of screening all hyperphenylalaninaemic newborns for defect biopterin metabolism is stressed.     

Selectively high level of serum interleukin 5 in a newborn infant with cow's milk allergy

Cow's milk allergy (CMA) in the neonatal period is thought to include several clinical conditions, yet the pathophysiology remains unclear. We report here the case of a term newborn infant who showed hematochezia 36 hours after the first feeding with cow's milk formula. His serum immunoglobulin E levels were not elevated, although eosinophils were detected in the stool. Elimination of cow's milk formula resolved the symptoms, and from the clinical course and laboratory data the infant was diagnosed with CMA. The serum interleukin 5 (IL-5) (125 pg/mL) level in this patient was selectively elevated. However, serum levels of other T-helper 2 (Th2) cytokines (including IL-4 and IL-13), Th1 cytokines (including interferon γ), and proinflammatory cytokines (including tumor necrosis factor α) were not elevated. These findings suggest that, for this patient, IL-5 and eosinophils might have played a role in the development of neonatal CMA. Although this finding is reported from only 1 case, it highlights the need for serum IL-5 to be determined in more neonatal patients with CMA to further clarify the pathophysiology of this condition in the neonatal period.     

Toxic shock syndrome: diagnosis and management

Staphylococcal and streptococcal toxic shock syndrome (TSS) is a type of shock that is mediated by superantigens. It is associated with significant morbidity and mortality. Early recognition of this disease is important because the clinical course is fulminant and the outcome depends on the prompt institution of therapy. Management of a child with TSS includes hemodynamic stabilization and appropriate antimicrobial therapy to eradicate the bacteria. The use of immunoglobulin is advised to block the superantigens. The authors describe the definitions of TSS, the clinical presentation, the role of superantigens, and the initial management.     

床旁肺部超声在新生儿肺炎中的诊断价值

目的 探讨床旁肺部超声在新生儿肺炎中的诊断价值。方法 收集2017年3月就诊于成都市妇女儿童中心医院新生儿重症医学科,以呼吸道症状为主诉,并在入院24 h内完善床旁肺部超声的49例新生儿的临床资料。对49例患儿的临床资料、肺部超声资料进行回顾性分析,对床旁肺部超声诊断新生儿肺炎进行诊断价值评价。结果 49例患儿中,根据新生儿肺炎诊断的金标准,44例诊断为新生儿肺炎;根据新生儿肺炎肺部超声诊断标准,38例诊断为新生儿肺炎。肺部超声在有呼吸道症状新生儿中诊断新生儿肺炎的敏感性为 86%,特异性为100%,阳性预测值为100%,阴性预测值为45%。44例金标准诊断的新生儿肺炎患儿中,肺部超声声像图显示,44例（100%）均出现B线,表现为异常胸膜线的比例达75%,部分患儿出现斑片/局限性弱回声区（36%）或肺泡间质综合征（27%）、支气管充气征（20%）等。结论 床旁肺部超声作为新的临床诊断技术,对新生儿肺炎的诊断敏感性高、特异性强,可作为新生儿肺炎诊断的工具。     

Neonatal polycythemia: frequency of clinical manifestations and other associated findings

We report the results of a two-part study examining the frequency of symptoms and other findings associated with neonatal polycythemia. In the first part of the investigation, we evaluated the occurrence and features of the disorder in a cohort of 3,768 infants born at our institution that had been screened for the disorder during a 4-year period. Fifty-five infants (1.46%) had neonatal polycythemia. Of these infants, 85% had features associated with the disorder. Frequent signs and symptoms included "feeding problems" (21.8%), plethora (20.0%), lethargy (14.5%), cyanosis (14.5%), respiratory distress (9.1%), jitteriness (7.3%), and hypotonia (7.3%). Other findings included hypoglycemia (40.0%) and hyperbilirubinemia (21.8%). Of the polycythemic infants, 14.5% had no clinical symptoms or associated laboratory abnormalities. In the second portion of the study, we reviewed the features of polycythemia in all infants so diagnosed who were born in United States Army hospitals, worldwide, during a 5-year period. There were 220,050 infants born during this period; 932 (0.42%) were diagnosed as having neonatal polycythemia. Frequent findings were hyperbilirubinemia (33.5%), hypoglycemia (13.0%), and respiratory distress (6.6%). In this large group, only 13 (1.4%) had necrotizing enterocolitis, and nine (1.0%) were thrombocytopenic. Several findings among the 932 infants were unexpected. Six of the infants (only one premature) had intracranial hemorrhages. Additionally, three of the 932 had gonadal dysgenesis and three had cystic fibrosis. We found that premature infants were not less prone to having polycythemia and that the overall frequency of the disorder was less than that which has been previously reported.     

Neonatal herpes simplex: clinical findings and outcome in relation to type of maternal infection

In 39 mothers of children with neonatal herpes simplex virus infection, maternal infection was serologically characterized retrospectively and was related to maternal clinical symptoms and to the clinical findings and outcome in the child. Thirteen mothers had a primary infection (six type 1, seven type 2), mostly with clinical symptoms. The mean age of onset of the disease of the infants was 7 days and a disseminated disease was most commonly found. Most of the type 1-infected children recovered completely, whereas all but one of the type 2-infected children died. Twenty mothers had a recurrent (2 type 1, 18 type 2) and 4 an intermediate infection (primary type 2, prior infection with type 1), mostly asymptomatic. Their children had a localized disease (of the skin-eye-mouth or the central nervous system) with onset at a mean age of 14 or 13 days, respectively. The frequency of neurological sequelae was high. Two mothers had no serological signs of herpes infection.     

Neonates presenting with temperature symptoms: Role in the diagnosis of early onset sepsis

Background: In this study, we aimed to evaluate the role of fever, hypothermia, and temperature instability in term and preterm newborns during the first 3 days of life and to identify risk factors for early onset sepsis (EOS) among newborns presenting with these temperature symptoms. Methods: In this retrospective cohort study set in our level III neonatal intensive care unit, we included all newborns hospitalized within the first 24 h of life from 2004 to 2007. Results: Of 851 newborns, 127 presented with temperature symptoms during the first 3 days of life (15%): 69 had fever, 69 had hypothermia, and 55 had temperature instability (8%, 8%, and 6%, respectively). Of 127 newborns presenting with temperature symptoms, 14 had culture‐proven EOS/pneumonia (33% of all 42 newborns with culture‐proven EOS/pneumonia), 67 had clinical EOS (30% of all 209 newborns with clinical EOS) and 46 were EOS‐negative (8% of all 600 EOS‐negatives). Factors associated with culture‐proven EOS/pneumonia in newborns presenting with temperature symptoms were maternal fever (P= 0.009), chorioamnionitis (P < 0.001), antibiotic therapy of the mother (P= 0.04), poor skin color (P= 0.001) and syndrome of persistent fetal circulation (P= 0.01). Conclusions: Every seventh newborn hospitalized at our neonatal intensive care unit developed fever, hypothermia and/or temperature instability during the first 3 days of life. Two‐thirds of them had culture‐proven or clinical sepsis. Temperature symptoms were rarely observed in EOS‐negative newborns (8%) but despite low sensitivity, were highly specific for bacterial infection in preterm and term newborns.     

新生儿精神药物撤药综合征的研究进展

妊娠期应用精神药物可导致20％-30％的新生儿出现撤药症状,临床表现主要包括中枢神经系统兴奋症状、胃肠道功能失常症状、呼吸系统症状、自主神经方面症状体征等.由于新生儿精神药物撤药综合征临床表现无特异性,容易误诊,所以应详细询问母亲病史,特别是孕期用药史,严密观察临床症状,采用临床量表进行评定,进行有关的实验室及其他检查,并排除其他疾病.治疗上支持疗法至关重要,病情严重时可给予苯巴比妥等药物.     

Familial asphyxiating thoracic dysplasia: clinical variability and impact of improved neonatal intensive care.

We describe 3 siblings with asphyxiating thoracic dysplasia whose neonatal symptoms range from mild respiratory distress to asphyxia and death. The youngest sibling received aggressive modern respiratory intensive care, survived, and at 2 years showed no respiratory symptoms. Improved neonatal intensive care has implications for clinical decision making and genetic counseling.     

Presentation of congenital heart disease in infancy: implications for routine examination.

AIM: To investigate the performance of routine neonatal and 6 week examinations for detecting congenital heart disease. METHODS: A retrospective review of findings on clinical examination was conducted of a cohort of live born infants with congenital heart disease in one health region in 1987-94. RESULTS: Of 1590 babies with congenital heart disease, 523 (33%) presented before neonatal examination because of symptoms or non-cardiac abnormalities. 1061 underwent routine neonatal examination which was abnormal in 476 (45%), but only 170 were referred directly for diagnosis. Of 876 discharged with no diagnosis, 306 presented or died undiagnosed before 6 weeks. At 6 weeks 252 of 569 babies underwent a second routine examination which was abnormal in 164 (65%). CONCLUSIONS: Routine neonatal examination fails to detect more than half of babies with heart disease; examination at 6 weeks misses one third. A normal examination does not exclude heart disease. Babies with murmurs at neonatal or 6 week examinations should be referred for early paediatric cardiological evaluation which will result either in a definitive diagnosis of congenital heart disease or in authoritative reassurance of normal cardiac anatomy and function.     

新生儿出血性及梗死性脑血管病诊治探讨

目的：提高对新生儿脑血管病的认识,探讨其临床发病特点及诊断方法。方法：选用不同的影像学方法对9例由于脑血管畸形所致的新生儿脑实质出血, 17例不同部位脑梗死患儿进行了检查、确诊,并依据神经系统症状,分析临床发病特点。结果：9例脑实质出血发生于额叶、颞叶和枕叶,其中7例在生后72h内发病; 10例因脑血管发育异常致大脑前动脉、中动脉、后动脉供血区梗死; 7例继发于不同疾病,为分支血管供血障碍所致的继发性脑梗死。典型的临床特征是频繁惊厥。结论：新生儿脑血管病最显著的临床症状是频繁惊厥,影像学检查为确诊提供了科学依据,早期治疗对预后有改善作用。     

A case of maple syrup urine disease misdiagnosed as Tetanus neonatorum on admission

A case of Maple Syrup Urine Disease (MSUD) is presented with clinical signs and symptoms on admission resembling neonatal tetanus. Diagnosis had to be differentiated between MSUD and other metabolic disorders and neonatal infections (especially neonatal tetanus because of severe opisthotonos) and generalized seizures of the patient. Early diagnosis of the MSUD patient is very important for effective therapy and better long-term prognosis as well as genetic counselling and prenatal diagnosis for future pregnancies.     

新生儿乳糜性浆膜腔积液临床分析

目的 探讨新生儿乳糜性浆膜腔积液的临床特征、病因、治疗和转归。方法 回顾性收集并分析21例新生儿乳糜性浆膜腔积液患儿的临床资料。结果 21例患儿中,单纯乳糜性胸腔积液13例,乳糜性腹腔积液2例,乳糜性心包积液2例,乳糜性多浆膜腔积液4例。先天性乳糜性浆膜腔积液患儿（n=5）纳入先天组,多发生在胎儿期,生后即出现症状;后天性乳糜性浆膜腔积液患儿（n=16）纳入后天组,多在中心静脉置管后1周内出现症状（n=14）,2例与败血症相伴出现。先天组患儿浆膜腔积液的比重、白细胞计数、单核细胞百分比、白蛋白水平均高于后天组（P < 0.05）;而葡萄糖、三酰甘油水平均低于后天组（P < 0.05）。置管相关乳糜性浆膜腔积液的发生多与置管位置不当、移位损伤、渗透压过高等因素有关。乳糜性浆膜腔积液患儿的治疗以保守治疗为主（n=20）,有效率达85%。先天组特殊奶粉喂养比例高于后天组,拔除置管比例低于后天组,浆膜腔积液吸收时间长于后天组（P < 0.05）。结论 引发新生儿乳糜性浆膜腔积液的原因和疾病发生的部位不同,症状出现的时间和表现不一。后天性患儿因血管损伤导致静脉高渗液渗出的可能性大。保守治疗效果显著,先天性患儿治疗难度高于后天性患儿。     

Clinical features and epidemiology of septicaemia and meningitis in neonates due to Streptococcus agalactiae in Copenhagen County, Denmark: a 10 year survey from 1992 to 2001

AIM: To elucidate the clinical and biochemical features, and to estimate the incidence and outcome of invasive culture-verified group B streptococcal (GBS) septicaemia/meningitis in neonates in Denmark. METHODS: Clinical microbiology laboratory records in patients 0-3 mo of age were searched for culture-verified GBS during 1992-2001 in Copenhagen County. Clinical records at the neonatal intensive care unit were reviewed retrospectively. Selected clinical and biochemical parameters were evaluated. RESULTS: 61 neonates had culture-verified GBS septicaemia/meningitis. The mean annual incidence was 0.76 cases per 1000 livebirths (range 0.0-1.91). A significant decrease in incidence was observed in the latest 3 y. The male:female ratio was 1.3:1. Eighty percent of the neonates had early-onset GBS within 24 h, 57% with symptoms at birth. Predominant initial symptoms were respiratory (72%), cardiovascular (69%) and neurological (63%). Only 4% developed GBS by day 7-90. Seventy-five percent had maternal or neonatal risk factors for early-onset GBS disease; 21% had clinical asphyxia, 37% of the mothers had premature rupture of membranes and 31% of the mothers were febrile. Initial C-reactive protein (CRP) was low, but increased significantly after more than 12 h duration of symptoms in 82% of patients. Leucopenia was an important initial haematological marker. CONCLUSION: The incidence of early-onset GBS has decreased significantly in Denmark, probably because of preventive measures in pregnancy and during birth. Respiratory symptoms are early signs of early-onset GBS. Initial leucopenia and a late (12-48 h) increase in CRP are valuable markers for invasive GBS.