—  SPECIALTY CONFERENCE  — Infectious Disease Pathology Sunday March 21 , 7:30 PM Harding Infections In the Pediatric Population Moderator: JEANNETTE GUARNER Emory University, Decatur, GA Disclosure: In accordance with ACCME guidelines regarding disclosure, the USCAP policy requires that faculty members who have a significant financial or other relationship with a commercial company, entity, or service (which will be discussed in this Symposium) must disclose this to attendees. The Academy also requires that speakers disclose any products that are not labeled for the use under discussion. The speakers listed below have indicated they have nothing to disclose. Panelists: MIGUEL REYES-MUGICA, Children's Hospital of Pittsburgh, Pittsburgh, PA LILI MILES, Cincinnati Children's Hospital Medical Center, Cincinnati, OH CARLOS R. ABRAMOWSKY, Emory University - Egleston Children's Hospital, Atlanta, GA BEVERLY B. ROGERS, Children's Medical Center, Dallas, TX JEANNETTE GUARNER, Emory University, Decatur, GA Clinical Histories and Still Images are displayed below. Click on slide thumbnail images for an enlarged view. If you have any difficulties viewing these slides, email or call George Clay at +1.724.449.1137. Case 1 Submitted by: Miguel Reyes-Mugica - Children's Hospital of Pittsburgh, Pittsburgh, PA Clinical Summary: In 1995, 6 year-old girl presented with a large left neck mass at the Children's Hospital of Pittsburgh. Her past medical history was relevant for biliary cirrhosis secondary to extrahepatic biliary obstruction (biliary atresia), treated with a Kasai procedure. At one year of age, she underwent an orthotopic liver transplant (OLTx). At the time of her hospital admission, five years after the liver transplant, relevant laboratory results included: , Hb= 12.2; Ht= 35.1; WBC= 7.6K/DL: Plt.=296 K/dL; Neut.= 49%; Lymph.= 31%; Mon.= 9%; Eos.=10%; Bas./Band= 1%. SGTP/SGOT= 44/39 IU/L; ALKP/GGTP= 157/22 IU/L. Renal function tests, electrolytes and glucose were within normal limits. Uric acid= 3 mg/dL. The mass was removed and sent to pathology. Representative photomicrographs are presented for discussion. Case 1 - Figure 1 Diff-Quik Case 1 - Figure 2 Case 1 - Figure 3 Case 1 - Figure 4 Case 1 - Figure 5 CD20 Case 1 - Figure 6 Ki67 Case 2 Submitted by: Carlos R. Abramowsky - Emory University - Egleston Children's Hospital, Atlanta, GA Clinical History: An adequate for gestation newborn female was delivered at term by elective cesarean section, with good Apgar scores. The infant's initial examination was unremarkable. The mother was a 31 year old, well to do African from Liberia and the pregnancy was uncomplicated. However, intractable postpartum bleeding led to a hysterectomy with a diagnosis of retained placenta. The placenta and uterus were examined in the pathology department with extensive sampling of the placenta. A diagnosis of placenta accreta was rendered. In the third day of life, the infant developed fever and leucocytosis and was diagnosed with sepsis. Blood, CSF and urine cultures were taken, followed by antibiotic therapy with no resolution of the clinical picture. At 72 hours antibiotics were discontinued as cultures were negative. At this time, physical exam showed hepatomegaly at 5 cm below costal margin without splenomegaly and fundoscopic examination showed chorioretinitis. A head ultrasound and CT scan were normal. Among other lab studies, her serum IgM level was very high at 250mg/dL; however, specific TORCH serologies (including HIV) and CMV viral cultures were negative. Through her 3d week of life, she appeared mildly ill, still febrile with leucocytosis, lethargy and poor feeding. Her liver was more enlarged and splenomegaly was evident. Examination of blood smears for malaria was negative. A re-review of the many sections of placenta (~15) and uterus to see if there were any clues to explain the infant's illness was conducted and a focal area of villitis and intervillitis with neutrophilic and lymphocytic inflammatory cells was identified (Figures 1 and 2). A chest X-ray of the infant, which had been previously negative, was repeated, and it showed diffuse infiltrates (Figure 3). Because of increasingly abnormal liver chemistries, a liver biopsy was obtained (Figure 4). At that point, the mother developed a respiratory infection with pleural effusion. Case 2 - Figure 1 Case 2 - Figure 2 Case 2 - Figure 3 Case 2 - Figure 4 Case 3 Submitted by: LiLi Miles - Cincinnati Children's Hospital Medical Center, Cincinnati, OH Clinical Summary: This was an infant girl born at 39 weeks gestational age by spontaneous vaginal delivery. The mother was a 34-year-old G5P3(T1P2A0L3). Prenatal testing results were as follows: maternal blood type, O negative; VDRL/RPR, negative; Rubella, non-immune; Hepatitis B surface antigen, negative. At the time of delivery, the Apgars were 8 and 9 at 1 and 5 minutes, respectively. The birth weight was normal for the gestational age, and the placenta was normal grossly. At birth, her lower extremities were atrophic and they did not have spontaneous movement. She started having desaturation episodes with oxygen saturation down into the 40's and questionable apnea, shortly after birth. Neurological workup revealed neurological deficits consistent with lower motor unit disorders. MRI on day 3 of life revealed brain with multifocal enhancement and cystic changes, consistent with previous hemorrhage and/or calcification. Similar changes were also seen in the lumbar spinal cord. Eye examination revealed chorioretinal scarring, OD. Serology was negative for CMV IgG and IgM. She developed extensive apneic spells and subsequently with no spontaneous respiratory effort. She expired at 3 weeks of age after put on DNR by parents due to grave prognosis. The major autopsy findings were microcephaly, hydrocephaly, and extensive necrotizing meningoencephalitis involving cerebrum, cerebellum and brain stem. There was also necrotizing myelitis, especially involving spinal cord distal to L1. Other autopsy findings included focal myocarditis, aspiration pneumonia, mucus plugs in the bronchus, hepatomegaly with pericentral necrosis, splenomegaly with white pulp hyperplasia, and extramedullary hematopoiesis in multiple organs. The immediate cause of death in the case was respiratory failure. The underlying cause of death was congenital infection extensively involving the central nervous system. Representative gross photos and H&E stained slides from brain and spinal cord are submitted for review. Case 3 - Slide 1 Click to view with ImageScope Click to view with a Web-Based Viewer Case 3 - Figure 1 Case 3 - Figure 2 Case 3 - Figure 3 Case 3 - Figure 4 Case 3 - Figure 5 Case 3 - Figure 6 Case 3 - Figure 7 Case 3 - Figure 8 Case 3 - Figure 9 Case 4 Submitted by: Beverly B. Rogers - Children's Medical Center, Dallas, TX Clinical Summary: This 1170 stillborn male fetus was delivered to a 26 year- old G3P2 mother at a gestational age of 27 weeks. The mother had been receiving prenatal care and was referred to Parkland Hospital after documentation of severe fetal hydrops. The fetus was re-imaged, with significant findings including severe fetal hydrops, a markedly enlarged placenta, ascites, a large pericardial effusion, an enlarged heart, and a severe elevation of the middle cerebral artery pressure (maximum peak systolic velocity of 130 cm/sec). The mother was admitted for an intrauterine transfusion and the fetus's hematocrit increased from 5.4 to 10.2 g/dL. Despite intrauterine transfucion, the fetus continued to have severe anemia and decelerations. Therefore, it was decided to induce labor. The infant was delivered by vaginal delivery without complications two days after initial presentation. Apgar scores were 0. Fetal examination showed a 1170 gram male infant with the only significant findings being hydrops and focal skin slippage. Case 4 - Figure 1 A 20X photograph of fetal lung (original magnification) Case 4 - Figure 2 A 100X photograph of fetal lung (original magnification) Case 4 - Figure 3 A 20X photograph of placenta (original magnification) Case 4 - Figure 4 A 100X photograph of placenta (original magnification) Case 5 Submitted by: Jeannette Guarner - Emory University, Decatur, GA Clinical Summary: A 7-year-old female presented with 5 days of fever, headache, abdominal pain, and vomiting, without diarrhea. On admission, her hemoglobin was 9.1 mg/dl and platelets were 41,000 platelets/ microliter. Liver function tests were mildly elevated, with an aspartate amino transferase of 107 U/lt and an alanine amino transferase of 112 U/lt. What is your diagnosis? What is the percent parasitemia? Case 5 - Figure 1 Handouts for all Specialty Conferences will be accessible via the "Educational Materials" section on the homepage the morning after each respective conference. Printed copies of the handout will not be available at the meeting.