Proper use of serum antibody titres against Epstein-Barr virus in nasopharyngeal carcinoma: IgA/virus capsid antigen for diagnosis and EBV-related nuclear antigen-2 for follow-up.

Sera from patients with nasopharyngeal carcinoma (NPC) show high titres of IgA antibodies to Epstein-Barr viral capsid antigen (IgA/VCA). We reported previously that the serum titres for Epstein-Barr virus-related nuclear antigen-2 (EBNA2) correlated with NPC patients' prognosis. To investigate which is better for diagnosing NPC and predicting patient prognosis, the titration of serum IgA/VCA or EBNA2, we examined the same serum titres. Sixteen cases of NPC in which serum EBNA2 antibody titres had been tested, were investigated for the serum IgA/VCA antibody titres before and after radiation treatment. All NPC cases showed positive reactions with indirect immunofluorescence staining, and the median titre was 252. Twelve normal controls, 5 mesopharyngeal carcinoma patients, 4 hypopharyngeal carcinoma patients, 4 laryngeal carcinoma patients and 6 malignant lymphoma were also examined, but they showed negative or relatively low titres. A follow-up study revealed that IgA/VCA titres remained mostly stable. These results indicate a close relationship between IgA/VCA and NPC, however, prognosis correlated better with EBNA2 titres than with IgA/VCA titres.

Expression of latent and replicative-infection genes of Epstein-Barr virus in macrophage.

Unlike other herpesviruses, Epstein-Barr virus (EBV) has not yet been shown to infect macrophages. Six macrophage cultures were isolated from normal and affected samples. Nested polymerase chain reaction revealed the existence of the EBV genome in all these macrophages. EBV latent genes expression in all cultures were detected by mRNA in situ hybridization and immunofluorescence staining. Some cultures also expressed EBV replicative-infection proteins, while in other cultures induction of these proteins was demonstrated. These findings are the first to show expression of several latent and replicative-infection genes of EBV in macrophages, indicating that EBV proliferates in macrophages.

The doughnut sign: an ultrasound finding in pediatric intestinal Burkitt's lymphoma.

This paper describes a doughnut-like ultrasound (US) finding in pediatric intestinal Burkitt's lymphoma. A 9-year-old boy had a fist-sized, hard, non-movable mass in the lower abdomen. US showed a thickened, layered ring like a doughnut. The outer, low-level echoes seemed to be consistent with mucosa and muscle layers and the inner, high-level echoes seemed to be intraluminal air or mucus. The serum lactic dehydrogenase level was high. At surgery, a solid, hard tumor 15 x 10 cm in size was found in the jejunum. The intestinal wall was diffusely thickened with an intact mucosa. From this experience, the US doughnut sign may be a helpful diagnostic finding in pediatric intestinal Burkitt's lymphoma.

Transumbilical resection and umbilical plasty for patent omphalomesenteric duct.

This paper describes a transumbilical resection and umbilical plasty for treatment of a patent omphalomesenteric duct (POMD). In a newborn infant with a POMD, a skin incision was made circumscribing the mucocutaneous junction of the protruded duct. The duct was completely extirpated with a wedge resection of the connection to the intestine and an umbilical plasty was performed. The postoperative appearance was excellent. It appears that transumbilical resection and umbilical plasty may be a satisfactory operation for POMD.

Ileal atresia associated with an omphalomesenteric duct remnant.

This paper describes an unusual case of ileal atresia associated with an omphalomesenteric duct (OMD) remnant. A 1-day-old male developed bilious vomiting and abdominal distension. At laparotomy, the distal portion of the ileum was connected to the umbilicus with a fibrous band. Around this omphalomesenteric band, the ileum was strangulated and necrotic; the end of the ileum was atretic. Apart from these structures, the cecum terminated without mesentery. This experience shows evidence of an OMD remnant as a possible cause of jejunoileal atresia.

Abdominal wall plasty for a premature infant with congenital diaphragmatic hernia.

This paper reports a premature infant with a congenital diaphragmatic hernia (CDH) who underwent an abdominal wall plasty to enlarge the abdominal cavity, one of twin infants born at 32 weeks weighing 1,255 g. After stabilization, the herniated viscera were reduced from the pleural cavity and the abdominal wall muscle and skin layers were replaced by a Gore-tex patch without closure of the diaphragmatic defect. Respiratory and circulatory conditions were stable during the perioperative period. Postoperatively, a roentogenogram showed expansion of the lung. However, his condition deteriorated 24 h after surgery, triggered by intratracheal suction, and he died on the 4th day of life despite the use of high-frequency oscillation, catecholamines, and vasodilators. Postmortem examination showed severely hypoplastic lungs. Abdominal wall plasty may be a less invasive initial procedure, however, further studies, such as comparison with the standard method or conservative management, are needed using a large clinical group or animal models to justify the usefulness of this procedure.

Necrotizing enterocolitis in a term infant with coarctation of the aorta complex.

Necrotizing enterocolitis (NEC) sometimes occurs in term infants with congenital heart disease. This article reports a rare case of a term infant with coarctation of the aorta complex who developed NEC on the 8th day after birth. Spontaneous closure of the ductus arteriosus in the 1st week of life may cause intestinal ischemia and hypoxia with resultant NEC.

Congenital multiple intestinal atresia successfully treated with multiple anastomoses in a premature neonate: report of a case.

We report herein the case of a 2080-g premature male infant born at 34 weeks' gestation with multiple intestinal atresia, for whom multiple anastomoses were successfully performed. A total of 11 atresias were found in the small bowel, and five anastomoses were performed to preserve 59 cm of small bowel and the ileocecal valve. Postoperatively, he developed several episodes of sepsis caused by persistent enterostasis, but was able to be weaned from total parenteral nutrition (TPN) by postoperative day (POD) 106. Thus, multiple anastomoses may be the appropriate procedure to prevent short-gut syndrome for congenital multiple intestinal atresia, even in premature infants.

Infarcted intestinal volvulus detected by prenatal ultrasonography.

This article describes a prenatal ultrasonographic finding of an infarcted intestinal volvulus. Ultrasonography showed polyhydramnios, multiple dilated intestinal loops, increased transverse abdominal area, and ascites. After cesarean section due to premature rupture of membranes and fetal distress, derotation of the infarcted volvulus caused postoperative thrombocytopenia, hyperkalemia, and acidosis and a subsequent resection was required. A high output of intestinal juice from the jejunostomy caused severe hypovolemia and electrolyte imbalance with resultant death. Increased transverse abdominal area caused by marked intestinal dilatation, ascites, fetal distress, and hydrops fetalis may suggest an infarcted intestinal volvulus.

A Ki-1-positive cell line expressing Epstein-Barr virus antigens, established from a child with Ki-1-positive lymphoma.

We report here the establishment of a CD30 (Ki-1) antigen-positive cell line 'Ki-JK' from a child with anaplastic large cell lymphoma. We characterized this cell line and show that: (i) Ki-JK cells do not grow in soft agar but infrequently produce a tumor or suppressed growth when injected into nude mice; (ii) Ki-JK cells expressed Epstein-Barr virus (EBV) antigens, EBV-associated nuclear antigen-2 and latent membrane protein; (iii) Ki-JK cells are labelled by in situ hybridization using an RNA probe derived from the BamHI W fragment of EBV, and (iv) polymerase chain reaction demonstrates the presence of an EBV BamHI W sequence in DNA of lymphoma and Ki-JK cells. These results suggest an etiological role for EBV in the development of some cases of Ki-1-positive lymphomas.

Ultrastructural study on a severe infantile sialidosis (beta-galactosidase-alpha-neuraminidase deficiency).

This study was undertaken to elucidate ultrastructural changes in a severe infantile sialidosis. The materials examined in this study consisted of biopsied rectal mucosa and autopsied small intestine, liver and kidney. In the biopsy sample, axons contained a number of pleomorphic electron dense bodies, and numerous membrane-bound vacuoles were found in Schwann's cells, fibroblasts, endothelial cells, lymphocytes and plasma cells. In autopsy samples, neurons in Auerbach's myenteric plexus of the small intestine were filled with a number of membranous cytoplasmic bodies, pleomorphic dense bodies and vesicles containing dense materials. Hepatocytes in the liver, and glomerular and tubular epithelial cells in the kidney were also extended by a number of membrane-bound vacuoles. These ultrastructural changes in severe infantile sialidosis closely resemble those in GM1-gangliosidosis type 1.

Live varicella vaccine: prevention of nosocomial infection and protection of high risk infants from varicella infection.

For prevention of nosocomial infection, 25 infants including high risk patients received an emergency injection of live varicella vaccine. Three patients developed a rash within 5 days after vaccination, but their symptoms were mild. The other 22 showed no clinical symptoms and gave an immune response. Twenty-two patients receiving immunosuppressive therapy were vaccinated and 20 of them showed a positive response in the varicella skin test. Of 14 vaccinated patients with malignancies, 2 giving a positive skin test, later showed clinical varicella, but their symptoms were not severe. One case with ALL was immunized safely under very poor conditions during the first induction therapy. No complications were observed in any patients.

A severe infantile sialidosis (beta-galactosidase-alpha-neuraminidase deficiency) mimicking GM1-gangliosidosis type 1.

We observed a 3-month-old Japanese female infant with severe psychomotor retardation, coarse facial appearance, hepatosplenomegaly, and dysostosis multiplex. Only beta-galactosidase was found to be deficient when the routine lysosomal hydrolase assay was performed on the patient's lymphocytes at 6 months of age. At first GM1-gangliosidosis type 1 seemed the most likely diagnosis. Later, however, additional studies (hydrolase assay in cultured skin fibroblasts, urinary oligosaccharide analysis, genetic complementation study, etc.) revealed that biochemical data of this case were in agreement with those of severe infantile sialidosis. The only important exception was that alpha-neuraminidase in the patient's lymphocytes showed normal activity but abnormal pH dependence toward 4-methylumbellyferyl substrate. In addition, a severely damaged kidney suggested that his case may be classified as a unique type of severe infantile sialidosis (possible nephrosialidosis). These observations stress the importance of careful biochemical diagnosis of a case with GM1-gangliosidosis type 1 phenotype.