Case profile: neuroblastoma in eleven-month-old infant.
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Biomedical subjects
Publications and source records attributed to N B Genieser.
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A case of idiopathic, chronic relapsing pancreatitis arising in childhood is presented and compared with the 12 previously reported cases. This entity is a rare cause of severe, recurrent abdominal pain in childhood. The diagnosis is suggested by elevated serum or urine amylase values or pancreatic calcifications. Ultrasound, computerized tomography, or as in this case, endoscopic retrograde cholangiopancreatography can aid in the diagnosis. Surgery yields excellent results.
Two cases of hydrometrocolpos and polydactyly in female infants with persistent urogenital sinus are reported. The importance of considering hydrometrocolpos as the cause of an abdominal mass in female infants with polydactyly is stressed. Cytologic examination of the vaginal fluid may help in early diagnosis.
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A ring of proliferative osteophytes at the junction of the femoral head and neck has been described as a characteristic finding in patients with ankylosing spondylitis. A review of 55 cases of juvenile rheumatoid arthritis, without evidence of sacroiliitis, revealed an identical ridge of osteophytes in 5.
Atypical measles pneumonia should be considered an explanation for nodular pulmonary lesions in older children and young adults. The disorder can develop in children immunized with inactivated measles virus vaccine who are then exposed to natural measles virus. Individuals given the vaccine when it was used (1961--1968) are now at risk to develop atypical measles pneumonia. This pneumonia may have a lobar or diffuse bronchopneumonia pattern. Three cases of this disorder are described. In one case, radiography showed calcification in the nodules 6 years after the pneumonia. Complicated and possibly hazardous workups can be avoided by awareness of this disease.
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Rupture of a popliteal cyst and dissection of its contents into the calf may produce pain, swelling, a positive Homan's sign and other findings closely resembling thrombophlebitis of the calf. The correct diagnosis is not often made, and the patient is subjected to needless long term anticoagulant therapy with its potential complications. To avoid this, it is essential that this possibility be kept in mind in all patients in whom the diagnosis of thrombophlebitis is considered. The history of preexisting arthritis of the knee, joint effusion and popliteal cyst are strongly suggestive of a ruptured popliteal cyst. This diagnosis can be verified by arthrography, ultrasonography, computed tomography and radionuclide scanning. Arthrography is preferred because it reveals superior anatomic detail thereby making differentiation between an encapsulated calf cyst, with smooth walls, and rupture, with irregular feathery margins, possible. Three illustrative cases are presented and the literature is reviewed.
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Three patients are reported, two of whom had mothers who revealed ingestion of abortifacient drugs during pregnancy. All patients resemble one another and those described earlier with malformations that were probably either aminopterin- or methotrexate-induced. It is likely that Patients I and II have abnormalities associated with the teratogenic action of these folic-acid antagonists. The etiology of Patient III's defects remains uncertain.
Pulmonary disorders occur in 25 to 47 per cent of the patients with Sjogren's Syndrome. These are of two types: 1. related to the major connective tissue disorders which commonly accompany this syndrome, and 2. secondary to the peculiar underlying pathology of Sjogren's Syndrome i.e. sicca disorders of the lung. The latter is the more frequent type, occurring mainly in patients with protected sicca complex unaccompanied by a systemic connective tissue disorder, and consists of chronic bronchitis, subsegmental atelectasis, pneumonia, bronchiectasis, lymphoproliferative pulmonary infiltrates and chronic interstitial pneumonitis which may lead to pulmonary fibrosis. 21 cases of Sjogren's Syndrome, 9 of whom had evidence of pulmonary abnormality, are presented, including a case of interstitial pneumonitis leading to diffuse interstitial pulmonary fibrosis. A review of the literature is included.
Computerized axial tomography is a new radiologic technique that permits noninvasive study of the skull, brain, and cerebral ventricles. This technique has been applied to the study of craniofacial malformations prior to intracranial surgical correction. The assessment of the cerebral ventricles is valuable as a base line study as well as helpful in detecting unsuspected abnormalities in the brain prior to surgery.
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Hypertrophic pulmonary osteoarthropathy is most commonly encountered in association with bronchogenic carcinoma and tumors of the pleura. Its association with pulmonary metastases from extrathoracic neoplasms is rare, with only 44 documented cases in the literature. Three additional cases are reported. Nearly half of the reported cases have been sarcomas, mainly of bone and soft tissues; among the rest are tumors of the nasopharynx and uterus and cervix. It has recently been noted that symptoms can be dramatically relieved by intrathoracic or cervical vagotomy.
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