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ClinicoPathological Conference

ClinicoPathological Conference. 93-03-24 林世朋 / 蔡明志大夫 三 軍 總 醫 院 小 兒 部. Chief complaints. A 4-day-old girl had suffered from fever since two days ago. Present illness (1).

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ClinicoPathological Conference

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  1. ClinicoPathological Conference 93-03-24 林世朋 / 蔡明志大夫 三 軍 總 醫 院 小 兒 部

  2. Chief complaints • A 4-day-old girl had suffered from fever since two days ago

  3. Present illness (1) • The 4-day-old female newborn was a fullterm and was delivered vaginally through thick meconium, with Apgar score of 9 at 1 minute and 9 at 5 minute. • She developed a fever of 38.6 ‘C at 2 days of age, but was relatively well without cardiopulmonary compromise. • She was sent to hospital for help where septic workup was done including lumbar puncture. Viral cultures were submitted because of several papulovesicular lesions noted on her back. • Ampicillin & gentamicine therapy was started at 2 days of age and acyclovir was added at 4 days of age. • All cultures including viral culture were negative.

  4. Present illness (2) • Then she was transferred to another hospital for further evaluation. • In addition, the mother had mild fever at the time of delivery, and was treated with antibiotics briefly before discharge. • By telephone interview, the mother said that she was still febrile 1 week after delivery with the main symptoms of malaise, abdominal pain and headache. • She denied respiratory symptoms, vomiting, diarrhea and skin rash. The father believed that she was jaundiced.

  5. Present illness (3) • The baby continued to appear pale, ill and febrile. On the baby’s 10th day of life, the mother visited the nursery. • With ill appearance, she told us that she had headache and neck pain. • The father stated that she was having personality changes that began the week before delivery. • The mother was then admitted to our hospital and evaluations revealed the diagnosis.

  6. Past, personal and family histories • Fullterm with gestational age of 38 weeks via vaginal delivery. • Apgar scores : 9 & 9 at 1st & 5th minutes, respectively ; BBW : 3100 g • Mother history : a 24-year-old gravida 3, para 1 to 2 mother. • Contact history : unknown • Traveling history : unknown • Family history : unknown

  7. Physical examinations(4 days of life) • Vital signs :HR : 130 /min ; RR: 40 /min ; BT: 38.6 ‘C • General appearance : pale and ill-looking, fussy but consolable • Skin : several scabbing lesions on her scalp. • Chest : clear and symmetric breathing sound; tachypnea: (-) ; retraction :(-) • Abdomen : palpable liver edge, 4 cm below the right costal margin in the midclavicular line; palpable spleen tip, 3 cm below the left costal margin. • Genitalia : bilateral inguinal lymph nodes 0.5 cm in diameter

  8. Laboratory findings (1) • At 3 days of age :WBC : 13400 /mm3Platelet : 96000 /mm3Neutrophil : 48%Lymphocytes : 17 %Band form : 29% (<15 %) • ALT : 20 U/L (6-50 U/L) • CSF study :WBC: 4 /mm3 (all mononuclear cells)Protein : 91 mg/dl (84 ± 45 mg/dL)Glucose : 49 mg/dl (46 ±10 mg/dL)

  9. Laboratory findings (2) • At 6 days of lifeCBC : essentially unchangedAST : 66 U/L (35-140 U/L)ALT : 54 U/L (6-50 U/L)r- GT : 555 U/L (13-147 U/L)

  10. Major problems : Fever Skin lesions (papulovesicular) over the back; scabbing lesions on the scalp Hepatosplenomegaly Lymphadenopathy over inguinal regions Minor problems : Predominant band form of WBC Thrombocytopenia Heavy meconium stain Problems of neonate

  11. Major problems : Personality changes Fever when delivery s/p antibiotic therapy Still fever, malaise, abdominal pain and headache Headache and neck pain. Minor problems : Jaundice Problems of mother

  12. Questions ?? • Neonate :1. any other abnormal physical findings ?2. head circumference ?3. ophthalmoscopic examination ?4. other laboratory findings (Hg, bilirubin etc..)5. CxR ?6. CNS image studies ?

  13. Question ?? • Mother :1. PROM ? Amnionitis ?2. Liver function ? Bilirubin levels ?3. fever source ? Genital cultures ?4. culture results ?5. physical findings ? Neurologic findings ? 6. Lumbar puncture ? CSF studies results? 7. past & personal histories ? VDRL ?8. travel & contact histories ?9. Image study (CxR, brain CT)? 10. What kind of antibiotics were used when delivery ? Drug history ?

  14. Possible Consequences of Infection of a Mother

  15. Possible organisms • Bacteria :Trepnema pallidum, Mycobacterium tuberculosis • Virus :Rubella, CMV, HSV, HIV, Enterovirus • Protozoa :Toxoplasma gondii, Plasmodium

  16. Clinical Features of Prenatal TORCH Infection

  17. Relative Clinical Features of TORCH Infection

  18. Congenital Rubella Syndrome • Affect virtually all organ systems • Manifestations :1.IUGR (most common)2.cataracts, frequently associated with microphthalmia 3.myocarditis and structural cardiac defects (PDA or pulmonary artery stenosis)4.”blueberry muffin” skin lesions5.hearing loss from sensorineural deafness6.meningoencephalitis7.pneumonia, hepatitis, bone lucencies, thrombocytopenia purpura, and anemia.8.motor and mental retardation • Diagnosis :1.anti-rubella IgM Ab in neonatal serum2.culturing rubella virus from the infant (nasopharynx, urine, or tissue)

  19. Cytomegalic Inclusion Disease • Congenital CMV infection • Only 5% of all congenitally infected infants have severe cytomegalic inclusion disease, another 5% have mild involvement, and 90% are born with subclinical, but still chronic, CMV infection. • Characteristic manifestations : IUGR, prematurity, hepatosplenomegaly and jaundice, thrombocytopenia and purpura, and microcephaly and intracranial calcifications. • Other neurologic problems :chorioretinitis, sensorineural hearing loss, and mild increases in CSF protein • Most symptomatic congenital infections and those resulting in sequelae are caused by primary rather than reactivated infections in pregnant women • Re-infection with a different strain of CMV can lead to symptomatic congenital infection. • Asymptomatic congenital CMV infection is likely a leading cause of sensorineural hearing loss, which occurs in approximately 7% of infected infants.

  20. Human Immunodeficiency Virus (1) • Currently, >95% of children with HIV infection acquire the infection from their mother (vertical transmission); transfusion of contaminated blood or clotting factor concentrates is now rarely observed in the USA • Breastfeeding remains a possible risk for transmission. • Infants born to HIV-infected mothers :1. Risk of infection is 13-39% if no antiretroviral therapy delivered to mother and infant2. With appropriate therapy, risk is < 5%3. Risk factors for vertical transmission include maternal viral load and degree of immunodeficiency and PROM.

  21. Human Immunodeficiency Virus (2) • Clinical manifestations :1. generally asymptomatic for first few months of life; mean age of onset of symptoms is 1 year2. Common manifestations include failure to thrive, hepatosplenomegaly, oral candidiasis, recurrent diarrhea, recurrent bacterial infections, and PCP between 3-6 months of age.3. Anemia, neutropenia, and thrombocytopenia are common

  22. Possible organisms • Bacteria :Trepnema pallidum, Mycobacterium tuberculosis • Virus :Rubella, CMV, HSV, HIV, Enterovirus • Protozoa :Toxoplasma gondii, Plasmodium

  23. Enterovirus---NEONATAL INFECTIONS (1) • Enterovirus infection frequently is with coxsackieviruses B2–B5 and echoviruses 6, 9, 11, and 19. • Transmission via :1. vertically before, during, or after delivery2. horizontally from other infected family members or by transmission in hospital nurseries (sporadic or epidemic). • Infection in utero may be associated with placentitis, fetal demise, neonatal illness, and, possibly, congenital anomalies. • Neonatal infection is associated with a range of clinical manifestations :--- asymptomatic (the majority) --- benign febrile illness --- severe multi-system diseases • Most affected newborns are full-term and previously well; • Maternal history often reveals a recent viral illness, including fever and, frequently, abdominal pain.

  24. Enterovirus---NEONATAL INFECTIONS (2) • Symptoms in the neonate may occur throughout the newborn period, with some beginning as early as day 1 of life; severe disease generally has an onset within the first 2 wk of life. • Clinical manifestations :--- fever or hypothermia, irritability, lethargy, anorexia,--- rash (maculopapular, occasionally petechial or papulovesicular), jaundice,--- respiratory symptoms, apnea, hepatomegaly, abdominal distention, emesis, diarrhea, and decreased perfusion. • Most symptomatic neonates have benign courses, with resolution of fever in an average of 3 days and of other symptoms in about 1 wk. Occasionally, a biphasic course may occur. • Laboratory findings: --- leukocytosis, thrombocytopenia, pleocytosis, --- elevated transaminases and bilirubin, coagulopathy,--- pulmonary infiltrates,--- EKG changes.

  25. Enterovirus---NEONATAL INFECTIONS (3) • Complications include :--- CNS necrosis and generalized or focal neurologic compromise--- arrhythmias, congestive heart failure, myocardial infarction, and pericarditis; --- hepatic necrosis and failure; intracranial or other bleeding;--- adrenal necrosis and hemorrhage; and rapidly progressive pneumonitis. --- Myositis, NEC, SIADH, hemophagocytic syndrome, and sudden death are rare events. • Mortality in those with severe disease is significant and is most often associated with hepatitis and associated bleeding complications, myocarditis, or pneumonitis. • Risk factors for severe disease include :--- illness onset in the first few days of life, --- maternal illness just prior to or at delivery, prematurity, male sex,--- infection by echovirus 11 or a coxsackie B virus, --- positive serum viral culture, --- absence of neutralizing antibody to the infecting virus, --- evidence of severe hepatitis and/or multi-system disease.

  26. Congenital syphilis (1) • Transplacental transmission of Treponema pallidum (spirochetes) • Transmission can occur at any stage of pregnancy; transmission rate approaching 100%. • Fetal and perinatal death occurs in 40% of affected infants. • Clinical manifestations :1.early signs : (during the first 2 yr of life) ---hepatosplenomegaly, jaundice, elevated liver enzymes; bile stasis ---diffuse lymphadenopathy ---Coombs negative hemolytic anemia; thrombocytopenia ---osteochondritis (wrist, elbow,ankle & knee) and periostitis (long bone) ---mucocutaneous rash : maculopapular or bullous lesions, followed by desquamation involving hands and feet ---CNS abnormalities, failure to thrive, chorioretinitis, nephritis2.late signs: (appear gradually during the first 2 decades) ---result primarily from chronic inflammation of bone, teeth and CNS. ---olympian brow, Higoumenakis sign, saber shins, Hutchinson teeth, mulberry molars, saddle nose---Juvenile paresis, Juvenile tabes, Clutton joint

  27. Congenital syphilis (2) • Diagnosis :1. nontreponemal tests : ---VDRL, RPR ---detect Ab against a cardiolipin-cholesterol-lecithin complex ---helpful in screening ---titers elevate when active and decline when treatment is adequate2. treponemal tests : ---TPI, FTA-ABS, and MHA-TP ---measure Ab specific to T. pallidum ---confirmatory testing of positive results from nontreponemal tests.

  28. Malaria • Plasmodium protozoa(Plasmodium ovale, Plasmodium vivax, Plasmodium malariae, Plasmodium falciparum). • Transmitted through :1. an infected female Anopheles species mosquito. 2. blood transfusion 3. congenitally between mother and fetus (rare) • Clinical manifestations :1. asymptomatic during the initial phase (incubation period)2. paroxysms of fever, rigors, sweats, headache, myalgia, back pain,abdominal pain, nausea, vomiting, diarrhea, pallor and jaundice. • Diagnosis :1. identification of organisms on Giemsa-stained smears of peripheral blood2. monoclonal antibody test3. PCR

  29. Congenital malaria • Malaria acquired from the mother prenatally or perinatally. • In endemic areas, congenital malaria is an important cause of abortions, miscarriages, stillbirth, premature births, IUGR and neonatal deaths. • Congenital malaria usually occurs in a nonimmune mother with P. vivax or P. malariae, although it can be observed with any of the human malaria species. • Symptoms and signs most commonly occurs between 10-30 days of age (14hr to several months of age) • Clinical manifestations :fever, restlessness, drowsiness, pallor, jaundice, poor feeding, vomiting, diarrhea, cyanosis and hepatosplenomegaly.

  30. Mycobacteria infection (1) • Mycobacterium tuberculosis complex : M. tuberculosis, M bovis, M africanum, M. microti, and M. canetti • Transmission :1. person to person by airborne mucus droplet (most adults no longer transmit the organism within several days to 2 wk after chemotherapy)2. M. bovis may penetrate GI mucosa or invade lymphatic tissue of oropharynx (human infection is rare as a result of pasteurization of milk & effective TB control program for cattle) • Pregnancy and the newborn :1.congenital tuberculosis is rare because the most common result of female genital tract tuberculosis is infertility.2.primary infection just before or during pregnancy is more likely to cause congenital infection than is reactivation of a previous infection.3.tubercle bacilli first reach fetal liver, then pass into fetal circulation and infect many organs.4.congenital tuberculosis may also be caused by aspiration or ingestion of infected amniotic fluid.

  31. Mycobacteria infection (2) Lymphohematogenous (disseminated) disease : • Tubercle bacilli are disseminated to distant sites, including liver, spleen,skin and lung apices, in all cases of tuberculosis infection. • Lymphohematogenous spread is usually asymptomatic. • Clinical manifestations :---hepatomegaly, splenomegaly---lymphadenitis in superficial or deep nodes---papulonecrotic tuberculids appearing on the skin---bone and joints or kidneys also may become involved---meningitis occurs only late in the course of the disease • Miliary disease (the most clinically significant form of disseminated TB)---occurs when massive numbers of tubercle bacilli are released into bloodstream, causing disease in two or more organs.---occur within 2-6 mo of the initial infection---lesions are often larger and more numerous in the lungs, spleen, liver and bone marrow than other tissues.---chronic or recurrent headache (meningitis)---abdominal pain or tenderness (tuberculous peritonitis)---cutaneous lesions papulonecrotic tuberculids, nodules, or purpura.

  32. Mycobacteria infection (3) Tuberculous Meningitis • Tuberculosis of the CNS accounts for about 5% of extrapulmonary cases. It is seen most often in young children but also develops in adults, especially those who are infected with HIV. • From hematogenous spread or rupture of a subependymal tubercle into the subarachnoid space . • may present subtly as headache and mental changes or acutely as confusion, lethargy, altered sensorium, and neck rigidity. • Typically, the disease evolves over 1 or 2 weeks, a course longer than that of bacterial meningitis; Hydrocephalus is common . • Lumbar puncture is the cornerstone of diagnosis. • In general, CSF reveals a high leukocyte count, a protein content of 100 to 800 mg/dL, and a low glucose concentration. • AFB are seen on direct smear of CSF sediment in only 20% of cases, but repeated lumbar punctures increase the yield. Culture of CSF is diagnostic in up to 80% of cases. • CT and MRI may show hydrocephalus and abnormal enhancement of basal cisterns or ependyma

  33. ---Congenital tuberculosis--- • symptoms may be present at birth but more commonly begin by the 2nd or 3rd wk of life. • clinical manifestations: respiratory distress, fever,hepatic or splenic enlargement, poor feeding, lethargy or irritability, lymphadenopathy, abdominal distention, failure to thrive, ear drainage, and skin lesions. • Many infants have an abnormal chest radiograph, most often a miliary pattern. • Some with no pulmonary findings early in the course of the disease later develop profound radiologic and clinical abnormalities. • Clinical presentation of TB in newborn is similar to that caused by bacterial sepsis and other congenital infection, such as syphilis, toxoplasmosis and CMV. • Diagnosis :acid-fast stain of gastric aspirate, middle-ear discharge, bone marrow, tracheal aspirate, or biopsy tissue (especially liver)

  34. Impression • 1.Mycobacteria tuberculosis • 2.Enterovirus infection

  35. Diagnostic procedure • AFB and culture of CSF from mother & gastric aspirate from neonate • PCR for enterovirus

  36. Thank you !!

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