Congenital syphilis: an emerging emergency also in Brazil

OBJECTIVE: To highlight to the fact that some newborns are not identified as having congenital syphilis, and will present to an emergency room within a few months with a severe disease. METHOD: Review of the charts concerning 3 patients with congenital syphilis, as well as review of the literature using Medline and Lilacs databases, covering the period from 1988 to 1999. RESULTS: We describe the case of 3 infants whose diagnosis of congenital syphilis was only established after the neonatal period, when they presented to the emergency room and were admitted to the pediatric intensive care unit. The first patient had neurosyphilis and nephrotic syndrome, the second had neurosyphilis, and the third had hepatitis. We discuss the clinical aspects of the cases, and comment on other clinical manifestations of congenital syphilis that should be within the purview of pediatric emergency medicine. We analyze the reasons for the failure to diagnose syphilis at birth, and describe some risk factors for gestational syphilis. CONCLUSIONS: Considering the rising incidence of syphilis in Brazil, and the possibility that the congenital infections are not recognized at birth, emergency physicians must keep a high degree of suspicion and an awareness of maternal risk factors, prenatal serology pitfalls, as well as of the several clinical presentations of congenital syphilis that can develop in the first months of life.     

Unusual presentation of congenital syphilis

Congenital syphilis (CS) usually presents in the neonatal period and may involve any organ system. The signs are diverse and a high index of suspicion must be maintained to make the diagnosis. Early congenital syphilis can present at any time before 2 years of age, but a review of the literature over the last 30 years indicates that it usually presents in the neonatal period and seldom later than 3-4 months of life. We report an 8-month-old boy who presented an unusual combination of signs of congenital syphilis, which led to delay in establishing the diagnosis. This case emphasises the importance of maintaining a high index of suspicion for CS throughout early childhood.     

新生儿梅毒螺旋体IgM抗体检测的方法研究与临床应用

目的研究新生儿梅毒螺旋体(TP)-IgM抗体的检测方法,及其在先天性梅毒早期诊断中的价值。方法采用甲苯胺红不加热血清试验(TRUST)和TP酶联免疫吸附试验(TP-ELISA)检测梅毒新生儿及其母亲血清TP-IgM抗体,同时检测血清TRUST滴度。以德国欧蒙试剂为对照,评估去除血清中IgG抗体和IgG型类风湿因子(IgG/RF)、酶标抗IgM抗体和酶标TP抗原等方法对IgM抗体检测效能的影响。结果 108例梅毒新生儿及其母亲TP-ELISA阳性率差异无统计学意义(P>0.05),梅毒母亲的TRUST阳性率显著高于其新生儿(P<0.05),而且梅毒母亲TRUST抗体滴度普遍高于新生儿(P<0.05)。与对照方法相比,IgM抗体检测时去除血清中IgG抗体可获得更好的相关性和一致性(Kappa>0.75),而使用酶标抗人IgM抗体(间接法,Kappa=0.956)比使用酶标TP抗原(双抗原夹心法,Kappa=0.817)的检测结果与对照方法更相近。结论 TRUST和TP-ELISA检测对新生儿先天性梅毒的早期诊断意义较小,而TP-IgM抗体检测方法在先天性梅毒的早期诊断中有较好应用前景。     

IgM antibody to Treponema pallidum in cerebrospinal fluid of infants with congenital syphilis.

OBJECTIVES--To characterize the neonatal IgG and IgM response to specific Treponema pallidum antigens in the cerebrospinal fluid (CSF) of infants with congenital syphilis. DESIGN--Cross-sectional survey. SETTING--Newborn nursery and neonatal intensive care unit of a county hospital in Dallas, Tex. PARTICIPANTS--Twenty-one infants born to mothers with reactive serologic tests for syphilis were enrolled. Group 1 consisted of six infants with clinical and laboratory evidence of congenital syphilis; group 2, six asymptomatic infants born to mothers with untreated syphilis; and group 3, nine asymptomatic infants whose mothers were treated for syphilis before delivery. SELECTION PROCEDURES--Random sample. MEASUREMENTS AND RESULTS--Immunoblotting was used to examine the IgM and IgG reactivities of neonatal serum and CSF against T pallidum antigens. Among serum samples of all group 1 infants, a specific IgM response to T pallidum antigens with apparent molecular masses of 47, 45, and 17 kd was observed. Cerebrospinal fluid IgM reactivity to a 47-kd antigen of T pallidum was seen in four group 1 infants. Serum samples from two group 2 and three group 3 infants demonstrated IgM reactivity against the 47-kd antigen and, in some cases, against the 45-kd antigen of T pallidum. None of 15 group 2 and 3 infants had a positive CSF IgM immunoblot result. The IgG reactivity in CSF was similar in the three groups and was directed against T pallidum antigens with apparent molecular masses of 72, 59, 47, 45, 42, 37, 34, 17, and 15 kd. CONCLUSIONS--A specific IgM response to T pallidum antigens, particularly the 47-kd antigen, was detected in the CSF of some infants with clinical and laboratory evidence of congenital syphilis. The potential usefulness of this test for the diagnosis of congenital neurosyphilis merits further study.     

Herpes simplex virus infections in neonates and early childhood

Of the commonly considered congenital infections, those caused by cytomegalovirus (CMV), syphilis, and herpes simplex virus (HSV) are frequently (CMV, HSV) or exclusively (syphilis) acquired sexually by the mother, with subsequent transmission to the developing fetus. Of the other commonly considered congenital infections, including rubella and toxoplasma infections, the mother is exposed to the infectious agent via interpersonal or environmental contacts. Unlike each of these other pathogens, which are transmitted transplacentally to the developing fetus following maternal infection though, HSV usually is transmitted perinatally as the neonate is exposed to the virus during passage through an infected birth canal. This difference in timing of acquisition of infection has had important consequence in the therapeutic advances achieved during the last 30 years in the management of neonatal HSV infections. Because the time period between the acquisition of infection and initiation of effective antiviral therapy is shorter in neonatal herpes than in congenital toxoplasmosis or CMV infections, the outcomes of therapy have the potential to be markedly different. This article will summarize the current state of neonatal HSV disease presentation, diagnosis, and management.     

新生儿先天性梅毒临床特点及再发危险因素分析

目的探讨新生儿先天性梅毒的临床特点及再发梅毒的危险因素。方法选择2005—2008年本院新生儿重症监护室收治的新生儿先天性梅毒患儿,回顾性分析其临床资料。患儿出院18个月后行问卷调查,根据是否再感染或复发梅毒分为再发组和无再发组,并对问卷结果进行单因素分析和Logistic回归分析。结果先天性梅毒65例,缺乏特异性临床表现,45例（69.2%）来自低文化教育家庭。共有54例完成问卷,其中无再发组45例,再发组9例。单因素分析显示,再发组患儿父母中接受健康教育、积极接受梅毒血清学检查、注意患儿衣物清洗和消毒比例均低于无再发组（分别为22.2%比64.4%、33.3%比75.6%、55.6%比88.9%,P均〈0.05）,而父母梅毒血清学RPR持续阳性的比例高于无再发组（77.8%比13.3%,P〈0.05）;Logistic回归分析提示,患儿父母RPR持续阳性是患儿再发梅毒的高危因素（OR=51.170,P=0.002）,对患儿父母进行健康教育是患儿再发梅毒的保护因素（OR=0.057,P=0.025）。结论目前先天性梅毒患儿主要来源于低文化教育水平家庭,缺乏特异性临床表现,经正规治疗后,仍可能再发,通过进行梅毒相关的健康教育,可减少患儿再发梅毒的机会。     

Curable dementia revealing late congenital syphilis]

SUMMARY: Late congenital syphilis is a rare disease and its neurological expression is exceptional. We report a case revealed by a curable dementia. CASE REPORT: This 17-year-old patient presented for one year progressive dementia, frontal syndrome and extra pyramidal syndrome. The cerebral CT scan showed a diffuse cortical and subcortical atrophy. Blood and CSF positive antibodies confirmed the diagnosis of late congenital meningoencephalitis due to syphilis. The outcome after 13 months follow-up was favourable with penicillin therapy. COMMENTS: Late congenital syphilis is a rare disease, that may be exceptionally revealed by a curable dementia. Evolution may be favourable with early penicillin therapy.     

Chest radiographs of neonates with respiratory failure caused by congenital syphilis

Congenital syphilis still occurs in newborn babies and the prevalence has increased in recent years, especially in developing countries. This has led to an increase in the number of babies with congenital syphilis requiring intensive care for respiratory failure. The early recognition of this disease could lead to the institution of timely and appropriate treatment. In this study the radiological picture of syphilitic pneumonitis is described in 20 neonates admitted to our neonatal intensive care unit requiring ventilation for respiratory failure. The radiological picture of the babies with syphilis was compared to 20 babies with other causes of respiratory distress. The radiological picture in 17 babies demonstrated a coarse nodular pattern in addition to band-like opacities radiating from the hilar regions. The nodular opacities became confluent on follow-up radiographs. In 13 cases, the proximal humeri showed changes typical of congenital syphilis. Two of the three babies with syphilis who did not have the typical chest radiological picture had bony involvement visible on the chest radiograph. Both the sensitivity and specificity of radiographic diagnosis were 75% with a positive and negative predictive value of 75%. The diagnosis of congenital syphilitic pneumonitis can therefore be suspected on chest radiographs and should be included in the differential diagnosis of any baby who presents with an interstitial pattern on chest radiography.     

HIV-1 infection and perinatal mortality in Zimbabwe.

As part of a survey of the causes of perinatal mortality at Mpilo Maternity Hospital, 220 neonatal deaths and the mothers of 221 stillbirths were tested for HIV-1 antibodies. The HIV positive rate in neonatal deaths was 23.6% (95% confidence interval (CI) 18.0 to 29.2%), significantly higher than 15.4% (95% CI 10.6 to 20.1%) in stillbirths. Perinatal deaths from congenital malformations, birth asphyxia, pregnancy induced hypertension, placental abruption, and oFther non-infectious causes had similar low HIV positive rates averaging 8.1% (95% CI 3.9 to 12.3%). Deaths from septicaemia had a significantly greater rate of 39.3% (95% CI 27.0 to 51.6%) and the highest rate of 72.2% (95% CI 51.5 to 92.9%) was found in deaths from congenital infection other than syphilis, indicating that maternal HIV infection predisposes to neonatal septicaemia and congenital infection. Unexplained stillbirths also had a significantly greater rate of 22.4% (95% CI 10.7 to 34.1%), presumably because some died from unrecognised infection. The rate in deaths from congenital syphilis was 17.4% (95% CI 9.6 to 25.2%), indicating a significant but weak association between these two sexually transmitted diseases in Bulawayo. The rate in deaths from hyaline membrane disease was not significantly greater at 15.0% (95% CI 6.0 to 24.0%). By predisposing to infection, maternal HIV infection was estimated to increase the stillbirth rate by 1.6 times and the neonatal mortality rate by 2.7 times. It predisposed equally to early and late onset neonatal septicaemia, but more to infection from streptococci and staphylococci than from Gram negative enterobacteria. HIV positive deaths from congenital infection had respiratory distress and usually intrauterine growth retardation, hepatosplenomegaly, and congenital pneumonia on lung histology.     

Early congenital syphilis presenting with skin eruption alone: a case report

Congenital syphilis is one of the most well-known congenital infections, yet it remains a worldwide public health problem. Congenital syphilis can involve any organ system and present with various symptoms. However, early diagnosis of congenital syphilis is difficult because more than half of the affected infants are asymptomatic, and the signs in symptomatic infants may be subtle and nonspecific. Here, we report a case of congenital syphilis with only a skin rash, which led to a delay in diagnosis. This case indicates that congenital syphilis should be considered throughout early childhood.     

Congenital syphilis: still a serious,under-diagnosed threat for children in resource-poor countries

Background  

With 700 000 to 1.5 million new cases annually, congenital syphilis remains a major infectious cause of morbidity and mortality in neonates, infants and children in resource-poor countries. We therefore analyzed the extent of congenital syphilis in the pediatric patient population at our rural hospital in Tanzania.     

Congenital syphilis surveillance and newborn evaluation in a low-incidence state

OBJECTIVES: To evaluate congenital syphilis surveillance in Minnesota, to assess the evaluation and management of newborns at risk for congenital syphilis, and to assess prenatal syphilis screening. DESIGN: Case ascertainment and medical record review. SETTING: The 7-county Minneapolis-St Paul metropolitan area. PATIENTS: Newborns at risk for congenital syphilis during a 3-year period (1992-1994). MAIN OUTCOME MEASURES: The completeness of congenital syphilis case ascertainment, maternal demographic data, maternal syphilis management, newborn evaluation for and management of congenital syphilis, and hospital syphilis screening practices at delivery. RESULTS: Eighty mother-infant pairs who were at risk for congenital syphilis were identified from 3 sources. Using the Centers for Disease Control and Prevention's congenital syphilis case definition, 36 infants (45%) were classified as probable cases, 42 (53%) were classified as noncases, and 2 (3%) were syphilitic stillbirths. Forty-seven women (59%) had syphilis serologic tests performed in the third trimester; only 37 (46%) had syphilis screening at delivery. Conditions of the mothers of 8 probable cases (22%) were diagnosed at delivery. Most probable cases (86%) were evaluated; only 56% were evaluated adequately. Twenty-five probable cases (69%) were treated. Most hospitals did not have formal policies for syphilis screening at delivery. The Minnesota Department of Health's congenital syphilis registry lacked sensitivity (39%) as a case ascertainment method. CONCLUSIONS: Clinicians should adhere to standardized protocols in the evaluation and management of at-risk newborns. Vigilant screening prenatally and at delivery and adequate follow-up are critical to reduce congenital syphilis. Improved surveillance data and resources are needed for the identification and follow-up of newborns at risk for congenital syphilis.     

母梅毒之新生儿13例临床分析

对母确诊为梅毒的13例新生儿病例进行临床分型、影像学检查、免疫测定和定期随访,探讨母产前梅毒螺旋体治疗情况对新生儿发病的影响,新生儿期先天性梅毒(CS)的主要临床表现。新生儿期确诊为CS共5例,疑诊3例,正常5例;确诊5例中母未治疗4例,产时较治疗前RPR滴度下降<4倍1例,产时RPR滴度>1:4共4例,新生儿RPR滴度≥母4倍3例。CS临床表现以早产、肝脾肿大、皮疹为主。神经系统早期改变主要为脑脊液蛋白增高。13例中失防2例,确诊组和正常组各1例;确诊组1例给予重复治疗,3例RPR滴度不同程度下降;疑诊组RPR滴度均在4个月内阴转;正常组持续阴性。结论:新生儿期的CS症状不典型,需根据临床、X线检查以及血清学等综合分析进行诊断。母产前定期监测、有效治疗可降低CS的发病率,而新生儿科医师对该病的认识和警惕性有利于患儿得到及时治疗。     

Congenital syphilis: a continuing but neglected problem

Congenital syphilis was rare in most affluent countries but there has been a slight resurgence recently in several European countries. In large parts of the world and particularly sub-Saharan Africa congenital syphilis is a significant public health problem. The cornerstone of congenital syphilis control is antenatal screening and treatment of mothers with penicillin, which is a cost-effective intervention. In affluent countries it should be strengthened among those at high risk. Clinicians should be more vigilant for the possibility of babies being born with congenital syphilis, which is often asymptomatic. In developing countries not only does antenatal care screening need to be strengthened by implementing point-of-care decentralised screening and treatment but alternative innovative approaches to controlling congenital syphilis should be explored. There is an urgent need for international health agencies to support focused approaches to tackling the tragedy of continuing congenital syphilis. This could be a part of a pro-poor strategy to meet the Millennium Development Goals.     

Symptomatic congenital syphilis presenting at birth

We report here a case of congenital syphilis presenting in a newborn infant at birth. A negative infant VDRL test, pseudoparesis and more notably, joint swellings (arthritis) were features seen uncommonly. Florid skeletal involvement, which is rarely seen in the early neonatal period, prompted us to draw attention to the varied presentation of this disease, rightly referred to as the “master masquerader”.     

HEMOGLOBIN-BASED OXYGEN CARRIERS: A Long Road from Bench to Bedside

Congenital syphilis is a rare, serious disease that continues to be a major health-care problem. The infected neonate may be asymptomatic or multiple-organ system involvement may occur. Anemia and thrombocytopenia are common hematological findings. Hemophagocytic lymphohistiocytosis (HLH) is a clinicopathological condition characterized by activation and uncontrolled nonmalignant proliferation of T lymphocytes and macrophages. The authors report an infant with congenital syphilis as a very rare cause of hemophagocytic syndrome. The reason for anemia and thrombocytopenia in congenital syphilis is not clear. The authors suggest that hemophagocytosis may play role in pathogenesis of cytopenia, particularly thrombocytopenia in patients with congenital syphilis.     

Congenital syphilis: A guide to diagnosis and management

Although congenital syphilis is a rare disease in Canada, infected infants may experience severe sequelae, including cerebral palsy, hydrocephalus, sensorineural hearing loss and musculoskeletal deformity. Timely treatment of congenital syphilis during pregnancy may prevent all of the above sequelae. However, the diagnosis of suspected cases and management of congenital syphilis may be confusing, and the potential for severe disability is high when cases are missed. The present review provides assistance to practitioners in the diagnosis of suspected cases and management of children with presumed or confirmed infection.     

Congenital syphilis: old disease, new resurgence.

The resurgence of syphilis has a detrimental effect on the health of communities and populations. As this epidemic continues, pediatric nurse practitioners may care for children with congenital syphilis. Children with congenital syphilis are at increased risk for physical abnormalities and developmental delays. Frequent physical and developmental assessments with goal-oriented plans will assist the child and family to achieve their maximum potential. Pediatric nurse practitioners who are knowledgeable about congenital syphilis, its manifestations, and associated nursing care needs will serve as case managers for children and their families with this diagnosis.     

Congenital and infantile nephrotic syndrome in Thai infants

Congenital and infantile nephrotic syndrome reported from the Eastern world is rare and might be a different entity from that in the West. In a retrospective review of 10 nephrotic syndrome in Thai infants (5 girls and 5 boys), 7 were diagnosed with congenital nephrotic syndrome and 3 with infantile nephrotic syndrome. Two had congenital nephrotic syndrome secondary to congenital syphilis. All had edema, ascites, and failure to thrive. Of the 3 patients tested for thyroid function, all showed hypothyroidism. Two patients developed renal failure. Renal tissue was examined from 4 patients from 3 biopsies and 2 autopsies; only 1 patient showed tubular microcysts. Symptomatic therapy was performed concurrently with penicillin therapy in 2 patients having congenital syphilis. Prednisolone, cyclophosphamide, captopril, and enalapril were tried in some patients, with little effect. Five patients died from respiratory failure complicated by later infection, 1 patient died from renal failure, and 4 patients were lost to follow-up. Nephrotic syndrome in the first year of life in the Eastern world is rare. Prognosis of nephrotic syndrome in Thai infants at this time is still poor.     

A premature newborn with vesiculobullous skin lesions

Although the rate of congenital syphilis is declining in Western Europe and in the USA, a significant increase is observed in the developing countries. In this contribution, a newborn with cutaneous manifestations of congenital syphilis is presented. CONCLUSION: Pediatricians should be attentive when confronted with vesiculobullous skin lesions in a newborn, and congenital syphilis should be considered in the differential diagnosis.