Alopecia areata universalis in an infant.

BACKGROUND: Alopecia areata (AA) is common during childhood and rarely reported in infants. The four reported cases of AA in infants all exhibited circumscribed patches of alopecia that appeared at birth or shortly thereafter. OBJECTIVE: We report a case of alopecia areata universalis that developed after birth along with fingernail changes of shortening (onychomadesis) and onycholysis. Scalp biopsy at 2 years of age revealed rare, intermediate, terminal follicles in catagen associated with sparse peribulbar lymphocytic infiltrates. RESULTS: This constellation of clinicopathologic features was interpreted as AA. We discuss the differential diagnosis of generalized alopecia in healthy infants, in particular, Clouston's syndrome, a hair-nail (hidrotic) ectodermal dysplasia found in this region. Genetic testing for linked polymorphisms to the Clouston gene locus were negative in this child and his parents. CONCLUSIONS: Alopecia areata should be included in the differential diagnosis of generalized alopecia presenting at or shortly after birth. For purposes of genetic counseling and prognosis, it is crucial that a correct diagnosis be made.

Principles and concepts of brain death and organ donation: the Jewish perspective.

The harvesting of organs for transplantation is dependent on a stringent definition of brain death. Different societies have had to struggle with their cultural heritage, adapting it to conform to the advances in medical science and the need of the sick. In this article, the development of the concept of brain death as it applies to organ transplantation in Judaism is outlined. The ability of traditional Jewish values to address themselves to the challenges of modern medicine can serve as a basis for cultural cross-fertilization and comparison in modern societies.

Principles and concepts of brain death and organ donation: the Jewish perspective.

The harvesting of organs for transplantation is dependent on a stringent definition of brain death. Different societies have had to struggle with their cultural heritage, adapting their traditional attitudes to conform to the advances in medical science and the needs of the sick. In this article, the development of the concept of brain death as it applies to organ transplantation in Judaism is outlined. The ability of traditional Jewish values to address themselves to the challenges of modern medicine can serve as a basis for cultural cross-fertilization and comparison in modern societies.

Variation's in the use of SMAS (superficial musculoaponeurotic system) to prevent Frey's syndrome after parotidectomy.

Since 1979, the SMAS (Superficial Muscular Aponeurotic System) has been used in over 100 parotidectomies to ameliorate the sunken facial appearance, and to prevent Frey's Syndrome. The procedure involves a rhytidectomy incision followed by variations including superior rotation, posterior advancement and plication of the SMAS. Appropriateness of such variations considers the defect from the surgery, and the histology of the tumor.

Reconstruction of the base of the tongue with the microvascular ulnar forearm flap: a functional assessment.

Ten patients with infiltrating carcinomas of the base of the tongue/tonsillar region underwent 30 to 100 percent resection of the base of the tongue and lateral pharyngeal wall. The surgical defect was reconstructed (9 primary, 1 secondary) with a large microvascular ulnar forearm flap that was selectively contoured to provide bulk for the base of the tongue and a thin lining for the pharyngeal wall. Seven patients were evaluated for swallowing and speech 6 weeks to 2 years following the reconstruction. Cineradiographic studies showed excellent base of the tongue and flap mobility allowing glossopharyngeal closure in all patients and complete pharyngeal evacuation in four patients. Four patients who were in good health preoperatively were able to eat a regular diet postoperatively, and the remaining three patients were able to handle soft food. Functional recovery after major tongue base surgery is contingent upon a three-dimensional microvascular reconstruction using a thin forearm flap.

Prevention of Frey's syndrome with superficial musculoaponeurotic system interposition.

Frey's syndrome after parotidectomy has an incidence generally reported to be 50% to 60% and is thought to be due to aberrant reinnervation of sweat glands by postganglionic parasympathetic fibers normally supplying the parotid gland. One hundred twelve patients had interposition of a flap of the superficial musculoaponeurotic system between the parotid bed and the overlying skin. Only one patient developed symptomatic Frey's syndrome, and one other patient had positive results on starch iodine testing. In patients not having the interposition, the incidence of symptomatic Frey's syndrome and positive starch iodine testing was 83% and 87%, respectively.

Secondary microvascular tongue reconstruction: functional results.

Between 1978 and 1991, 56 microvascular composite flaps were used for oromandibular reconstructions: 15 for primary total and subtotal tongue reconstruction and five for secondary major tongue reconstruction. The delayed reconstructions were performed to improve the oral and pharyngeal phases of swallowing. Using a floor of the mouth composite bone grafting technique to reposition the tongue and obliterate the oral dead space intraoral food transport was improved (three of five patients), but aspiration persisted (three of four patients). When compared with 10 patients evaluated for primary total and subtotal tongue reconstruction the primary reconstruction group showed superior swallowing (eight dynamic oral transport, no aspiration) and speech results. The poor results of secondary reconstruction are attributed to scarring and irreversible damage to remaining functional muscles involved in protecting the laryngeal aditus.

Primary sclerosing cholangitis in sarcoidosis.

A 63-year-old patient with sarcoidosis developed a clinical picture compatible with sclerosing cholangitis 20 years later. The uncommon association between these two rare diseases, and possible common pathophysiological-immunological mechanisms are discussed.

Total and subtotal glossectomy: function after microvascular reconstruction.

Twelve patients with advanced carcinoma of the floor of the mouth and tongue were treated with total (five patients) or subtotal (seven patients) glossectomy, partial mandibulectomy, and immediate reconstruction with the microvascular composite groin flap. The osteomusculocutaneous groin flap was used in eleven patients, and the osteomuscular flap was used in one patient. The groin musculocutaneous or muscle flap was designed to resemble the shape of the tongue for dynamic food transport, improved swallowing, and acceptable speech. Eight of the 12 patients who survived more than 1 year were evaluated for speech and swallowing. Eight patients were able to speak intelligibly, six patients could tolerate a soft/pureed diet, and two patients were limited to fluids. Cinefluorographic swallow studies using semisolid contrast material showed voluntary active intraoral transport and propulsive pharyngeal emptying without aspiration in six patients with complete flap to palate contact; the remaining two patients were unable to move the intraoral contrast material effectively for swallowing because of poor palatal contact.

Horizontal versus vertical block resection for early floor of mouth carcinoma.

Although the literature affirms the superiority of block resection over local excision for the surgical treatment of early squamous cell carcinoma of the anterior floor of the mouth, the best method of block resection is not certain. Two methods are in widespread use; a horizontal procedure (HB) and a vertical procedure (VB). This retrospective study compares these two procedures according to outcome in separate series of patients treated between 1970 and 1984. Patients were followed for a minimum of 3 years with an average follow-up of 6 years. Twenty-five patients underwent HB while 27 underwent VB. Control at the primary site was 100% for HB and 74% for VB. Neck metastasis and distant metastasis occurred with equal frequency. Determinate survivals at 3 and 5 years were 96% and 91% for the HB group and 93% and 65% for the VB group. Local recurrence correlated with tumor growth posteriorally toward the ventrum of the tongue, suggesting an explanation for the difference in outcome between the two procedures. We conclude that the HB procedure is the preferable surgical procedure for stage I and II disease.

Characterization of a goldfish antigen during development and regeneration of the visual system.

Normal, regenerating, and developing optic nerves of the goldfish were studied utilizing a monoclonal antibody (mAb) 1E1T which has specificity for Müller cells in the retina, radial glial cells in the tectum, and non-neuronal cells in the optic nerve. Sections of the normal optic nerve revealed longitudinally oriented chains of non-neuronal cells, that were 4-8 cells long. The number of chains in the normal nerve was very few. In addition, short acellular septa, probably the connective tissue septa, were also labeled with mAb 1E1T. Sections of crushed optic nerves showed an increase in the antigen recognized by mAb 1E1T within the septa and new septa were now visualized. Furthermore, the existing septa were longer and extended the length of the optic nerve. The formation and elongation of the septa occurred as early as 3 day postcrush. Between 3 and 11 d postcrush, there was heavy labeling of the septa and a large accumulation of non-neuronal cells at the crush site. At 3 months postcrush, the accumulation of non-neuronal cells labeled by mAb 1E1T were no longer visible but heavy labeling of the septa was still apparent.

A monoclonal antibody specific for the visual system of the goldfish.

Although several monoclonal antibodies (MAbs) have been reported that recognize antigens present in ganglion cells of the mammalian retina, these MAbs do not cross-react in the goldfish. In the current study we have identified a MAb that recognized a 14.4 kDa antigen that is present on the ganglion cells in the retinae of goldfish but is absent from the retinae of all other species that were tested. No other cell type in the goldfish retina or optic nerve was labeled with this MAb. Furthermore, the axons in the optic nerve, optic tract and the terminal layers in the optic tectum were labeled.

Persistent oral antral fistulas.

Oral antral fistulas that have been already subjected to previous attempts at closure have been difficult to treat. Gold foil, bony plugs, and other materials have been used for many years to close these fistulas. Because of all of the various techniques and materials used in this procedure, it has been vexing to evaluate their efficacy. Although the persistent fistula is a rare entity, the surgeons faced with treating this condition must understand the principles of flap closure. In six cases in which multiple attempts at oral antral fistula closure had failed, we used either bilateral or unilateral palatal flaps based on the posterior palatine artery. The patients have been followed up for ten years after surgery and can wear dentures if indicated. There has not been any recurrent fistulization in any of these cases.

Syngeneic monoclonal antibodies to B16 melanoma viral antigens.

Four stable IgM monoclonal antibody-producing hybridomas were generated by fusing mouse myeloma cells with spleen lymphocytes from C57BL/6 mice hyperimmunized against the syngeneic B16 melanoma. All four monoclonal antibodies (R31/15, R37/4, R37/6, and R37/7), in common with polyclonal antiserum from immunized mice, recognized antigens on the same complex of related cell surface molecules specified by endogenous AKR-type murine leukemia virus, designated the B16-gp/70/80/85 antigen complex. Reactivity with this antigen complex was demonstrated by radioimmunoprecipitation. Specificity for viral Mr 70,000 glycoprotein-related antigens was indicated by absorption of antibody activity by endogenous AKR virus and by inhibition of antibody binding to B16 melanoma cells by monospecific antiserum to murine leukemia virus Mr 70,000 glycoprotein. Neither polyclonal nor monoclonal antibodies recognized antigens on fish, guinea pig, swine, or human melanoma cell lines. Polyclonal antiserum reacted with several other mouse melanomas and with certain mouse lymphoma lines induced by, or harboring, endogenous murine leukemia viruses, but the monoclonal antibodies were unreactive except for recognition of antigens on Harding-Passey mouse melanoma cells by antibody R37/4 and on RL male 1 mouse lymphoma cells by antibody R37/7. Only monoclonal R37/7 was cytotoxic for cultured B16 melanoma cells in an antibody- and complement-dependent assay with guinea pig complement, although all antibodies were cytotoxic with rabbit complement. In reflecting the predominant humoral immune response to the B16 melanoma detected in syngeneic mice during tumor growth, these monoclonal antibodies will permit experimental amplification of that response to help determine how that immunity influences tumor growth and metastatic dissemination.

Adaptive mechanisms of speech and swallowing after combined jaw and tongue reconstruction in long-term survivors.

Twelve patients have been studied for speech and swallowing function after major combined jaw and tongue reconstruction with the microvascular iliac bone and groin skin composite flap. Cinegraphic barium swallows demonstrated that for bolus propulsion, it is important to be able to occlude the palate with the flap. Glottic competence prevents aspiration. Speech studies show that although there is loss of certain speech sounds, approximate sounds are substituted. Speech is intelligible when soft-tissue contact to the palate can be accomplished. The adaptive mechanisms in these patients have been compared with the mechanisms used by a patient with uncorrected congenital aglossia and hypomandibulosis who developed excellent speech and swallowing. The mobility of this patient's mouth and pharynx was similar to that in the reconstructed cancer patients who were able to swallow and speak. This procedure has become our reconstruction method of choice for these major defects.