Gastroesophageal Reflux in Severe Cases of Robin Sequence Treated With Nasopharyngeal Intubation

Objective: To study the prevalence of abnormal gastroesophageal reflux in infants with Robin sequence who had severe respiratory obstruction treated with nasopharyngeal intubation and to evaluate the efficacy of nonsurgical treatment. Design: Longitudinal prospective study. Setting: Hospital de Reabilitacao de Anomalias Craniofaciais, University of Sao Paulo, Brazil. Patients: Twenty infants with severe isolated Robin sequence treated with nasopharyngeal intubation. Interventions: We performed 24-hour esophageal pH monitoring on each child at 2, 4, and 6 months of age. Respiratory and feeding status were evaluated. We considered abnormal gastroesophageal reflux as reflux index values above the 95th percentile of the Vandenplas reference for normal children. Results: The prevalence of reflux index above the 95th percentile at the first exam was 6/20, a value significantly higher than the reference (5/103, p < .01). At the second and third exams, reflux index values were decreased. Ninety percent of the infants showed improvement of respiratory difficulty and developed oral feeding capacity. Conclusions: The prevalence of abnormal gastroesophageal reflux is higher in infants with severe cases of Robin sequence than in normal infants. Nonsurgical procedures improved respiratory and feeding difficulties of most of these infants.

Perinatal outcome of fetal atrioventricular block - One-hundred-sixteen cases from a single institution

Background-Fetal atrioventricular (AV) block is an uncommon lesion with significant mortality. Because of the rarity of this disorder, the natural course, extensive evaluation of untreated fetuses, and late follow-up remain unclear. Methods and Results-Of the 116 consecutive cases of fetal AV block studied from 1988 to 2006, only 1 was terminated, and 75% were live births. Fifty-nine cases of AV block were associated with major structural heart disease, mainly left atrial isomerism (n = 40), with only 26% of neonatal survivors. Of the 57 fetuses with normal cardiac anatomy, 41 (72%) were positive for maternal antinuclear antibodies, and 32 of these seropositive mothers did not receive any treatment. This untreated group had live-birth and 1-year infant survival rates of 93% and 90%, respectively. Five fetuses from seronegative mothers showed regression to sinus rhythm during pregnancy. The presence of major structural heart disease, hydrops, an atrial rate <= 120 bpm, and a ventricular rate <= 55 bpm were identified as risk factors for mortality. Logistic regression analysis of the whole group showed that the presence of structural heart disease was the only independent predictor of death (P < 0.001). Conclusions-This long-term study confirms that fetal AV block has a poor outcome when associated with structural heart disease and that spontaneous regression of AV block is possible in seronegative forms. The survival rate of >90% of our untreated patients with isolated forms of AV block raises concerns about any decision to intervene with immunosuppressive agents.

Children and economic development: Family size, gender preferences and human capital formation - theory and Indian cases

In the light of Gary Becker's economic theory of the family, considers how economic cost and benefit factors can influence the size of families that parents decide to have. Some support for the importance of such factors is found from results of structured interviews with wives in Kondh-dominated villages in western Orissa. These results are at variance with the hypothesis of Malthus about population growth. Factors that may alter the optimal family size as development proceeds are discussed. It is found in our sampling that, on the whole, there is a preference for daughters rather than sons although this is not as strong in the Kondh-dominated villages as in poor villages in the Santal tribal belt of West Bengal. While in the Kondh-dominated villages some discrimination in access to education in favour of boys compared to girls is present, little such or no such discrimination occurs in relation to access to food and medical attention. In the villages surveyed in the West Bengal Santal tribal belt, discrimination in favour of boys is more pronounced than in the Kondh-dominated area in Orissa. While economic considerations help to explain gender discrimination between boys and girls, we find that social and cultural factors also play a major role. Parents in a similar economic situation seem to display substantially different patterns of gender discrimination between children depending on their social and cultural content. It seems that the extent to which economic theories of the family explain family preferences and behaviour depend significantly on the social and cultural context in which they are to be applied.

Oral squamous cell carcinoma: status of tight junction claudins in the different histopathological patterns and relationship with clinical parameters. A tissue-microarray-based study of 136 cases

Aims Claudins are integral transmembrane proteins of the tight junctions, critical for maintaining cell adhesion and polarity. Alterations in the expression of individual claudins have been detected in carcinomas and appear to correlate with tumour progression. Methods In this study, a panel of anti-claudin antibodies (anti-claudins 1, 2, 3, 4, 5 and 7) was employed to map claudin expression in 136 cases of oral squamous cell carcinoma (OSCC) organised in a tissue microarray. Results Claudins were expressed in a reticular pattern up to the prickle layer in normal mucosal epithelium. In OSCC, claudins were strongly present in well-differentiated tumours, they presented mild and low expression in moderately differentiated OSCC, and were negative in poorly differentiated OSCC; the absences of claudin 1 (p = 0.002) and claudin 4 (p<0.001) were associated with moderately/poorly differentiated tumours. Strong expression of claudin 4 was associated with decreased perineural infiltration (p = 0.024). Claudins 5 and 7 were mostly negative or weakly expressed in all cases studied. Expression of claudin 7 was associated with the early clinical stages of the disease, whereas loss of claudin 7 tended to be more frequent in advanced stages of OSCC (p = 0.054). Absence of claudin 7 was also associated with absent vascular infiltration (p = 0.045) and with presence of recurrence (p = 0.052). Conclusions Claudin expression patterns showed a strong correlation with histological type of OSCC; claudin expression was decreased in areas of invasion, and negative in poorly differentiated tumours. This pattern may be related to evolution and prognosis of these tumours, especially in the case of claudin 7, which seems to be associated with a poor prognosis in OSCC.

Intravascular papillary endothelial hyperplasia: Report of 4 cases with immunohistochemical findings

Intravascular papillary endothelial hyperplasia (IPEH) is a benign endothelial proliferation, usually intravascular, that may mimic angiosarcoma. In this report, four new cases of IPEH involving the oral region are described. The affected sites were the lower lip, labial comissure and the submandibular region. After clinical evaluation, the complete removal of the lesions showed a circumscribed and soft mass. Histologically, the major feature was a reactive proliferation of endothelial cells composed of small papillary structures with hypocellular and hyalinized cores arising in an organized thrombus. Immunohistochemical staining for CD34 was strongly positive in endothelial cells. Vimentin and laminin immunolabelling were also consistent with a vascular origin. In order to verify the proliferative potential of the lesions, the Ki-67 antibody was used, revealing low percentage of labeled cells (<20%). No immunoreactivity for GLUT-1 was observed. Since the complete removal is curative, no additional treatment was necessary, and no signs of recurrence had been observed until now. Due to the particular features of IPEH, it is important for pathologists and clinicians to become familiar with this lesion. Additionally, the specific histological arrangement, including the absence of cellular pleomorphism, mitotic activity and necrosis, represents a guide to help in the differential diagnosis. Moreover, the vascular origin and the proliferative index should be assessed by immunohistochemistry in order to provide an accurate diagnosis.

Intralesional injection of triamcinolone hexacetonide as an alternative treatment for central giant-cell granuloma in 21 cases

Central giant-cell granulomas are benign, but occasionally aggressive, lesions that traditionally have been treated surgically. 21 cases of central giant-cell granuloma of the jaw were treated with intralesional injection of corticosteroids. The treatment protocol adopted was intralesional injection of 20 mg/ml triamcinolone hexacetonide diluted in an anaesthetic solution of 2% lidocaine/epinephrine 1:200,000 in the proportion 1:1; 1.0 ml of the solution was infiltrated for every 1 cm(3) of radiolucid area of the lesion, totalling 6 biweekly applications. Ten patients had aggressive lesions and 11 nonaggressive. Two patients showed a negative response to the treatment and underwent surgical resection, 4 showed a moderate response and 15 a good response. 8 of the 19 who had a moderate-to-good response to the drug treatment underwent osteoplasty to reestablish facial aesthetics. In these cases, only mature or dysplastic bone was observed, with the presence or absence of rare giant multinucleated cells. The advantages of this therapy are its less-invasive nature, the probable lower cost to the patient, lower risk and the ability to treat the lesion surgically in the future, if necessary.

Localization of small esophageal cancers for radiation planning using endoscopic contrast injection: report on a series of eight cases

Recently, Barrett's esophagus and early adenocarcinomas have been detected increasingly frequently in routine follow-up of patients with gastroesophageal reflux. Although surgery is the treatment of choice, some patients are medically unfit for esophagectomy and, in this case, the only alternative curative therapy is radical chemoradiation therapy. In addition, some patients who present with symptoms have small tumors that cannot be localized accurately using routine imaging techniques. This report describes a series of eight patients with small esophageal cancers in whom the tumors were successfully localized following endoscopic injection of contrast, and treated with chemoradiation therapy. The treatment was successful in seven patients. This method of tumor localization demonstrated that conventional techniques are mostly, unreliable when applied to very early cancers.

Primary Bartholin gland carcinoma: a report of seven cases

This study reviews our experience with 7 patients with primary Bartholin gland cancer (BGC) treated at the Queensland Gynaecological Cancer Centre (QCGC) and compares this with previously published data. A retrospective clinicopathologic review of all patients with primary BGC treated at QCGC from 1988 to 2000 was performed. Of the 7 patients treated, all underwent primary surgery and 5 of the 7 patients received radiotherapy postoperatively. All patients presented with a local swelling or a lump. Two had associated discharge and 2 had associated pain. Of the 7 patients, 2, 3 and 2 respectively were classified as having Stage IB, II or III disease. Five of the 7 patients had squamous cell carcinoma (SCC), one had adenoid-cystic carcinoma and 1 had a small-cell neuroendocrine cancer of the Bartholin gland. None of the patients with SCC developed recurrent disease. The patient with adenoid-cystic carcinoma experienced local recurrences at 4 years and again at 5 years and 3 months. Nine years after primary treatment she was diagnosed with pulmonary metastases. The patient with small-cell neuroendocrine cancer of the Bartholin gland was considered tumour-free after operation. Thorough imaging, including a CT scan of her chest, abdomen and pelvis showed no evidence of disease. She died 1 year and three months after diagnosis from disseminated pulmonary disease. We present the first report, of small cell neuroendocrine cancer of the Bartholin gland. Therapeutic principles in the management of vulval cancer at other sites appear to be appropriate for management of BGC.

A cluster of melioidosis cases from an endemic region is clonal and is linked to the water supply using molecular typing of Burkholderia pseudomallei isolates

Nine cases of melioidosis with four deaths occurred over a 28-month period in members of a small remote Aboriginal community in the top end of the Northern Territory of Australia. Typing by pulsed-field gel electrophoresis showed isolates of Burkholderia pseudomallei from six of the cases to be clonal and also identical to an isolate from the community water supply, but not to soil isolates. The clonality of the isolates found in this cluster contrasts with the marked genetic diversity of human and environmental isolates found in this region which is hyperendemic for B. pseudomallei. It is possible that the clonal bacteria persisted and were propagated in biofilm in the water supply system. While the exact mode of transmission to humans and the reasons for cessation of the outbreak remain uncertain, contamination of the unchlorinated community water supply is a likely explanation.

Impact of malocclusion and orthognathic reconstruction surgery on resonance and articulatory function: an examination of variability in five cases

Articulatory patterns and nasal resonance were assessed before and 6 months after orthognathic reconstruction surgery in five patients with dentofacial deformities. Perceptual and physiological assessments showed disorders of nasality and articulatory function preoperatively, two patients being hyponasal, and one hypernasal. Four patients had mild articulatory deficits, and four had reduced maximal lip or tongue pressures. Operation resulted in different patterns of change. Nasality deteriorated in three patients and articulatory precision and intelligibility improved in only one patient and showed no change in the other four. Operation improved interlabial pressures in three patients, while its impact on tongue pressures varied, being improved in one case, deteriorating in one, and remaining unchanged in the other three. The variability in the results highlights the need for routine assessment of speech and resonance before and after orthognathic reconstruction. (C) 2002 The British Association of Oral and Maxillofacial Surgeons. Published by Elsevier Science Ltd. All rights reserved.

Petrology and geochemistry of the Bandas del Sur Formation, Las Canadas Edifice, Tenerife (Canary Islands)

The Bandas del Sur Formation preserves a Quaternary extra-caldera record of central phonolitic explosive volcanism of the Las Canadas volcano at Tenerife. Volcanic rocks are bimodal in composition, being predominantly phonolitic pyroclastic deposits, several eruptions of which resulted in summit caldera collapse, alkali basaltic lavas erupted from many fissures around the flanks. For the pyroclastic deposits, there is a broad range of pumice glass compositions from phonotephrite to phonolite. The phonolite pyroclastic deposits are also characterized by a diverse, 7-8-phase phenocryst assemblage (alkali feldspar + biotite + sodian diopside + titanomagnetite + ilmenite + nosean-hauyne + titanite + apatite) with alkali feldspar dominant, in contrast to interbedded phonolite lavas that typically have lower phenocryst contents and lack hydrous phases. Petrological and geochemical data are consistent with fractional crystallization (involving the observed phenocryst assemblages) as the dominant process in the development of phonolite magmas. New stratigraphically constrained data indicate that petrological and geochemical differences exist between pyroclastic deposits of the last two explosive cycles of phonolitic volcanism. Cycle 2 (0.85-0.57 Ma) pyroclastic fall deposits commonly show a cryptic compositional zonation indicating that several eruptions tapped chemically, and probably thermally stratified magma systems. Evidence for magma mixing is most widespread in the pyroclastic deposits of Cycle 3 (0.37-0.17 Ma), which includes the presence of reversely and normally zoned phenocrysts, quenched mafic glass blebs in pumice, banded pumice, and bimodal to polymodal phenocryst compositional populations. Syn-eruptive mixing events involved mostly phonolite and tephriphonolite magmas, whereas a pre-eruptive mixing event involving basaltic magma is recorded in several banded pumice-bearing ignimbrites of Cycle 3. The periodic addition and mixing of basaltic magma ultimately may have triggered several eruptions. Recharge and underplating by basaltic magma is interpreted to have elevated sulphur contents (occurring as an exsolved gas phase) in the capping phonolitic magma reservoir. This promoted nosean-hauyne crystallization over nepheline, elevated SO3 contents in apatite, and possibly resulted in large, climatologically important SO2 emissions.

Welding and rheomorphism of phonolitic fallout deposits from the Las Canadas caldera, Tenerife, Canary Islands

The Las Canadas caldera is a nested collapse caldera formed by the successive migration and collapse of shallow magmatic chambers. Among the pyroclastic products of this caldera are phonolitic fallout deposits that crop out in the caldera wall and on the extracaldera slopes. These deposits exhibit an uninterrupted facies gradation from nonwelded to lava-like and record continuous volcanic deposition. Densely welded and lava-like facies result from the extreme attenuation and complete homogenization of juvenile clasts that destroy original clast outlines and any evidence of fallout deposition. Agglutination contributes significantly to the final degree of flattening observed in the welded facies. After deposition, rheomorphic flowage occurs. Emplacement temperatures for one of the welding sequences are calculated from magmatic temperatures and a model of tephra cooling during fallout. Results are 486 degreesC for the nonwelded facies and 740 degreesC for the moderately welded facies. For the same welding sequence, a cooling time between 25 and 54 days is estimated from published experimental and computational data as the possible duration of welding and rheomorphism. Following deposition and agglutination, the lava-like pyroclastic facies had the rheological properties of viscous lavas and flowed down the outer slopes away from the caldera. Some lava-like masses detached from proximal areas to more distal regions. During deposition, the eruptive style evolved from Plinian fallout to fountain-fed spatter deposition. This evolution was accompanied by a decrease in explosive power and a lower height of the eruptive column, which produce higher emplacement temperatures and more effective heat retention of pyroclasts.

A new technique for the quantification of peripheral edema with application in both unilateral and bilateral cases

Current noninvasive techniques for the routine and frequent quantification of peripheral lymphedema in patients are total limb volume measurement (by water immersion or by circumferential measurements) and bioelectrical impedance analysis (BIA). However both of these techniques require standardizing the measurement using a contralateral measurement from the unaffected limb, Hence these techniques are essentially restricted to unilateral lymphedema. This paper describes the results from a preliminary study to investigate an alternative approach to the analysis of the data from multiple frequency BIA to produce an index of lymphedema without the need for normalization to another body segment. Twenty patients receiving surgical treatment for breast cancer were monitored prior to surgery and again after diagnosis with unilateral lymphedema. The data recorded were total limb volume, by circumferential measurements; and BIA measurements of both limbs. From these measurements total limb volumes and extracellular fluid volumes were calculated and expressed as ratios of the affected limb to that of the unaffected limb. An index of the ratio of the extracellular fluid volume to the intracellular fluid volume was determined. This ECW/ICW index was calculated for both the affected and unaffected limbs at both measurement times. Results confirmed that the established techniques of total limb volume and extracellular fluid volume normalized to the unaffected contralateral limb were accurate in the detection of lymphedema (p < 10(-6)). Comparison of the ECW/ICW index from the affected limb after diagnosis with that from the pre-surgery measurement revealed a significant (p< 10(-6)) and considerable (75%) increase. The results of this pilot study suggest that by using multiple frequency bioelectrical impedance analysis, an index of the ECW/ICW ratio can be obtained and this index appears to have an equal, or better, sensitivity, than the other techniques in detecting lymphedema. More importantly, this index does not require normalization to another body segment and can be used to detect all types of peripheral edema including both unilateral and bilateral lymphedema.

Neutrophil dysplasia characterised by a pseudo-Pelger-Huet anomaly occurring with the use of mycophenolate mofetil and ganciclovir following renal transplantation: a report of five cases

Aim: The pseudo-Pelger-Huet (PH) anomaly has been associated with a variety of primary haematological disorders, infections and drugs. Recently, the development of dysgranulopoiesis characterised by a pseudo-PH anomaly has been reported in two patients with the use of mycophenolate mofetil (MMF) in the setting of heart and/or lung transplantation. We present a further five cases of MMF-related dysgranulopoiesis characterised by a pseudo-PH anomaly occurring after renal transplantation. Methods: All patients were receiving standard immunosuppression protocols for renal transplantation, including a combination of MMF, steroids and either cyclosporin or tacrolimus. Oral ganciclovir was also used for cytomegalovirus prophylaxis in each case. Results: Development of dysplastic granulopoiesis occurred a median of 96 days (range 66-196 days) after transplantation. Moderate or severe neutropaenia (