Treating nevoid basal cell carcinoma syndrome.

More than 100 anomalies are associated with this syndrome. In this case, a cyst removed from a 15-year-old male was diagnosed as an odontogenic keratocyst. The long history of this syndrome with its associated problems is described.

Absence of lower extremity ossification centers in term infants with congenital syphilis.

Radiologic evaluation is valuable for confirmation of congenital syphilis. Bony abnormalities are thought to be secondary to active disease or a growth response of bone to systemic illness. Anteroposterior lower extremity radiographs of 85 euthyroid term infants (> or = 38 weeks' gestation; mean weight, 3244 gm; range, 1940-4380 gm) with presumptive congenital syphilis were evaluated for the presence of the distal femoral and proximal tibial secondary ossification centers and other bony abnormalities. Both proximal tibial and distal femoral ossification centers were absent in only 9 of 85 infants with syphilis; the proximal tibial center was absent in 26 of 85 (total 30.6%; 95% confidence limits 0.2 to 0.4). This observation is significant at the p < 0.01 level by chi-square analysis with use of historical controls. Periosteal new bone formation was seen in 2 of 85 infants. Metaphyseal lucent bands were present in 21 of 85 infants, 8 of whom also had absent lower extremity ossification centers. For comparison we reviewed lower extremity radiographs from 12 term infants without congenital syphilis. Ossification centers were absent in one growth-retarded infant, and metaphyseal lucent bands were found in one infant. Although standard texts report nearly universal ossification of the distal femoral epiphysis at term, we observed delayed skeletal maturation in 30.1% of term infants with congenital syphilis. We speculate that this delay may be reflective of systemic infection.

Hepatic spindle cell tumors in HIV positive children.

Spindle cell tumors (leiomyoma, leiomyosarcoma) have been described in HIV infected children involving the gastrointestinal tract, respiratory tract and liver. The same tumor has been reported in immunocompromised children following liver and renal transplantation. A case of hepatic spindle cell tumor in an HIV infected child is discussed.

Choledochal cyst. Imaging by Telepaque-enhanced computed tomography.

A choledochal cyst is an uncommon anomaly of the biliary system requiring surgical intervention. A case of a choledochal cyst imaged by computed tomography following oral administration of cholangiography contrast material (Telepaque) is reported. Telepaque-enhanced computed tomography is an easy, noninvasive method to demonstrate pertinent preoperative anatomy in cases of choledochal cysts, and is especially useful in the pediatric patient.

Viral infections in the dental setting: potential effects on pregnant HCWs.

Occupational exposure of pregnant health care workers to infectious diseases may cause a variety of effects. This article reviews potential effects of viral infections in pregnant health care workers by pathogens transmittable in a dental setting.

Pericardial effusion and cardiac hemangioma in the neonate.

In the absence of hydrops or sepsis, a pericardial effusion is a rare occurrence in the neonate. We report a case of a neonate with a pericardial effusion in which there was an associated intracardiac hemangioma. Our literature review found 32 cases of pericardial effusion without hydrops in infants under 3 months of age; twelve of these cases were associated with intracardiac and pericardial tumors; 20 others were discovered to be randomly associated with other problems.

Is a chest radiograph necessary in the evaluation of every febrile infant less than 8 weeks of age?

This study was designed to examine the relationship between respiratory signs and the likelihood of having an abnormal chest radiograph in a sample of febrile infants less than 8 weeks of age. The sample consisted of 242 infants who were admitted during a 3-year period with temperatures greater than or equal to 38 degrees C (100.4 degrees F) and had a chest radiograph. The house officer recorded the presence of respiratory signs and symptoms including rhinorrhea, tachypnea, cough, rales, wheezes, retractions, and rhonchi. Each chest radiograph was reviewed independently according to predetermined criteria by a senior radiology resident and an attending pediatric radiologist. Interobserver agreement was 91%. Both observers were blind to the infants' respiratory signs. The chest radiograph interpretations were compared with the presence of respiratory signs. Of the 242 cases, 228 had chest radiographs available for interpretation. Of these, 27 chest radiographs (12%) were identified as abnormal, including 6 where there was initial disagreement as to the presence of an abnormality. Twenty-five (31%) of 80 infants with any respiratory signs had an abnormal chest radiograph, whereas only 2 (1%) of 148 asymptomatic infants did. The sensitivity of respiratory signs was 93% (confidence interval = 76% to 99%). These findings suggest that in the absence of respiratory signs, febrile infants are unlikely to have an abnormal chest radiograph.

Seminal vesicle-rectal fistula. Report of a case.

A previously unreported complication of low anterior resection of the rectum, seminal vesicle-rectal fistula, was encountered one month after surgery in an elderly patient with adenocarcinoma of the midrectum. Antibiotic-induced colitis in the immediate postoperative period led to anastomotic leakage with abscess formation and ensuing fistulization to the surgically denuded right seminal vesicle. Pneumaturia, bacteriuria, and right testicular pain were treated by cutaneous vasostomy and antimicrobial therapy. Despite recurrent low-grade urinary sepsis controlled by alternating courses of various antimicrobials, and radiation therapy for local tumor recurrence, the patient remained reasonably healthy until his death two years later due to stroke associated with cerebral metastases.

Aspartylglucosaminuria in a Puerto Rican family: additional features of a panethnic disorder.

We report on 3 Puerto Rican brothers with the clinical and laboratory findings of aspartylglucosaminuria (AGU). Their parents were first cousins. The affected sibs have the "cardinal" manifestations of AGU, including developmental disabilities, progressive "coarsening" of the face, and early onset of hepatosplenomegaly. Biochemical studies showed elevated levels of urinary aspartylglucosamine and very low activity of aspartylglucosaminidase(AGA) in cultured fibroblasts. With long term follow-up, previously undescribed manifestations were noted, including radiographic evidence of spondylolysis and spondylolisthesis in early childhood and development of macro-orchidism during puberty. This family shows that AGU is not limited to individuals of Finnish background, but that the gene is panethnic in distribution and that additional changes, not previously noted, may present with advancing age.

The usefulness of chest radiographs in first asthma attacks.

To assess the value of routine chest radiography during acute first attacks of asthma, we studied 371 consecutive children over one year of age who presented with an initial episode of wheezing. Three hundred fifty children (94.3 per cent) had radiographic findings that were compatible with uncomplicated asthma and were considered negative. Twenty-one (5.7 per cent) had positive findings: atelectasis and pneumonia were noted in seven, segmental atelectasis in six, pneumonia in five, multiple areas of subsegmental atelectasis in two, and pneumomediastinum in one. The patients with positive films were more likely to have a respiratory rate above 60 or a pulse rate above 160 (P less than 0.001), localized rales or localized decreased breath sounds before treatment (P less than 0.01), and localized rales (P less than 0.005) and localized wheezing (P less than 0.02) after treatment; also, these patients were admitted to the hospital more often (P less than 0.001). Ninety-five per cent (20 of 21) of the children with positive films could be identified before treatment on the basis of a combination of tachypnea, tachycardia, fever, and localized rales or localized decreased breath sounds. Most first-time wheezers will not have positive radiographs; careful clinical evaluation should reveal which patients will have abnormal radiographs and will therefore benefit from the procedure.

The use of glucagon in hydrostatic reduction of intussusception: a double-blind study of 30 patients.

Thirty patients were referred for hydrostatic reduction of ileocolic intussusception. Children with suspected gangrenous bowel or sensitivity to glucagon were excluded from the investigation. A standard protocol for the procedure was used in all patients, including the intravenous administration of glucagon or placebo (0.05 mg/kg) when the intussusception was encountered. Successful reduction was achieved in 53% of both control and glucagon-treated patients. Analysis of the length of the procedure and the ease of reduction of the intussusception indicated no difference in the two groups. This multicenter double-blind study failed to show any therapeutic value of glucagon in hydrostatic reduction of intussusception.

Passive immunization for hepatitis B.

Numerous studies have been conducted to assess the effectiveness of gamma globulin in preventing type B hepatitis. These investigations have evaluated the effectivenesses of standard immunoglobulin (IG) and hepatitis B immunoglobulin (HBIG) in pre-exposure and postexposure settings. The current data suggest that HBIG and standard IG containing some antibody to hepatitis B surface antigen may be effective for pre- and postexposure prophylaxis of hepatitis B. It is recommended that HBIG be used for the postexposure prophylaxis of individuals sustaining accidental needle-stick or mucosal exposure to blood known to contain hepatitis B surface antigen. If HBIG is unavailable, then standard IG may be beneficial. While pre-exposure prophylaxis with gamma globulin may be effective, there are appropriate environmental control methods available that may significantly reduce the transmission of hepatitis B, and these should be fully implemented.

Methods of hepatitis B virus transfer in oral surgery.

There are a variety of modes of transmission of the hepatitis B virus. Although the traditional parenteral mechanism of direct transfer of the virus via a needle containing infected serum or plasma is the most common, nontraditional parenteral mechanisms have been documented. These include transfer of the virus via saliva and plasma through minute skin abrasions, through mucosal surfaces, and indirectly via inanimate environmental surfaces. Current evidence does not support an intestinal mode of transmission. The various modes of transmission. The various modes of transfer of hepatitis B virus cause particular problems for people in the dental health care profession. However, knowledge of the different modes of virus transmission will allow the dental practitioner to take appropriate precautions to prevent transfer of hepatitis B virus from patient to dentist.

Hepatitis B virus: an overview for dentists.

Hepatitis B has been singled out as a condition of special concern to dentists as it is frequently severe and debilitating. A review of the literature shows a significant and increasing risk of hepatitis among dentists, especially oral surgeons. Concern is directed toward dentists who contract the disease as well as those who transmit the infection.