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Anaplastic thyroid cancer with transient thyrotoxicosis: case report and literature review.

A 55-year-old woman with anaplastic thyroid carcinoma presented with hyperthyroidism and neck swelling, hoarseness, and cervical lymphadenopathy. On physical examination, she was found to be clinically hyperthyroid with an enlarged, nontender multinodular goitre. Her serum thyroid hormone levels confirmed hyperthyroidism and technetium-99m pertechnetate scan failed to visualize the thyroid gland. Open biopsy showed an invasion of the thyroid gland by anaplastic thyroid carcinoma. The thyrotoxic phase lasted 60 days with predominantly increased thyroxine level and triiodothyronine/thyroxine (T3/T4) ratio decreased below 15. The thyrotoxic period was followed by subclinical hyperthyroidism and hypothyroidism which continued until she died of lung metastasis.

Biopsy↗

Symptoms of gastro-oesophageal reflux disease in elderly people.

The frequency and the possible age-related characteristics of gastro-oesophageal reflux disease (GORD) were investigated in 195 consecutive elderly subjects (mean age 74 years), referred to endoscopy for abdominal symptoms or sideropenic anaemia. In the 105 of these patients in whom there was any suspicion of GORD, 24-hour pH monitoring was carried out. All the patients were interviewed before the examinations. Erosive or complicated (grade 2-4) oesophagitis was found in 18% of patients. The main symptoms in these patients were dysphagia, respiratory symptoms and vomiting. Chronic cough, hoarseness or wheezing were present in 57% of patients with oesophagitis compared with 33% of those without oesophagitis (p less than 0.001). The occurrence of heartburn and regurgitation did not differ significantly between patients with or without oesophagitis, although the mean symptom scores were higher in those with oesophagitis. Dyspepsia and chest pain were not typical symptoms in oesophagitis. Of patients with oesophagitis 29% had no typical symptoms of GORD; only 24% of patients with regurgitation had oesophagitis. In 24-hour pH monitoring, a significant increase in the occurrence of symptoms was not seen until total reflux time pH less than 4 exceeded 10%. The occurrence of heartburn did not correlate with the extent of reflux in the pH study. In conclusion, typical symptoms of GORD in the aged were regurgitation, dysphagia, respiratory symptoms and vomiting rather than heartburn.

Age Factors↗

Necrotizing sialometaplasia of the larynx. A report of two cases and a review of the literature.

Two cases of necrotizing sialometaplasia of the larynx are reported. One of the cases occurred in the subglottic larynx of a 37-year-old woman and the other occurred in the right false vocal cord of a 59-year-old man. In both of the cases, the sialometaplasia was identified in association with another pathologic process. The female patient presented with dysphagia. Excisional biopsy was performed twice in the span of an 8-day period. Both biopsy specimens showed fibrosis. The subglottic stenosis was felt to be of undetermined origin. There was evidence of necrotizing sialometaplasia in only the second biopsy material. Subsequently, the patient was diagnosed as having relapsing polychondritis, the probable cause of the subglottic stenosis. The male patient presented with increasing hoarseness and was found to have a mass of the right false vocal cord. Biopsy of the mass was diagnostic for a poorly differentiated squamous cell carcinoma with separate foci of necrotizing sialometaplasia. In both cases, it appears that the sialometaplasia occurred secondary to the other pathologic processes with the likely pathogenesis the result of vascular compromise to the affected area.

Adult↗

Amyloid goiter. A clinicopathologic study of 14 cases and review of the literature.

The authors report the clinicopathologic features of 14 cases of amyloid goiter (AG). Eleven patients were males and three females with ages ranging from 23 to 75 years (median, 54 years). Eight patients had secondary amyloidosis and six had primary amyloidosis. Nine cases were identified at autopsy. In symptomatic patients (n = 5), the clinical presentation included a nontender, rapidly enlarging neck mass with associated dysphagia, dyspnea or hoarseness. Clinical or laboratory evaluation failed to detect evidence of thyroid dysfunction. The histologic appearance of the thyroid predominantly consisted of diffuse amyloid deposition surrounding thyroid follicles. In two cases, a nodular pattern of amyloid deposition was seen resulting in compression and distortion of the follicular architecture. Areas of mature adipose tissue and focal lymphocytic thyroiditis with or without foreign-body type-giant cells were seen in approximately one third of the cases. Confirmation of amyloid was made by the presence of congophilia and apple-green birefringence under polarized-light microscopy. Immunohistochemical evaluation demonstrated the presence of amyloid A immunoreactivity. No Immunoreactivity was seen with calcitonin or thyroglobulin. Fine-needle aspiration may facilitate the diagnosis, as occurred in one the patients. In symptomatic patients, thyroidectomy is warranted to alleviate pressure symptoms.

Adult↗

Laryngeal cysts in adults: a clinicopathologic study of 20 cases.

Clinically significant cysts in the region of the larynx are uncommon. In this study, 20 cases were recovered for the period 1969-1983. There were eight males and 12 females whose ages ranged from 28-76 years and whose most common symptom combinations were hoarseness (9), local foreign body sensation (5), and pain (4). Laryngoscopic examination revealed cystic lesions 0.5-3.0 cm. in diameter, all localized to the supraglottis with half in the vallecula . Histologically, 17 cysts were closely associated with tonsillar lymphoid tissue. Nine cysts, having squamous lined crypt-like structures and abundant follicular lymphoid tissue, were designated "tonsillar cysts." Eight cysts had a prominent epithelial component and focal follicular lymphoid aggregates. Three cysts were unassociated with lymphoid tissue. Two of these, located in the ventricles of elderly patients, were oncocytic cysts. Both showed oncocytic changes on the surface mucosa and in adjacent salivary gland tissue. In one case, a biopsy of the contralateral ventricle demonstrated oncocytic metaplasia. Treatment in all cases was by surgical excision. Follow-up on 13 patients ranging from three months to 9 years revealed one recurrence of an oncocytic cyst one year after initial removal. We conclude that follicular lymphoid tissue is present in most laryngeal cysts and may play some pathogenetic role in their formation. Furthermore, oncocytic cysts are different from other laryngeal cysts, having a characteristic location, age group, field effect, and recurrence rate.

Adult↗

Cytologic and immunohistochemical diagnosis of neuroendocrine (Merkel cell) carcinoma in cerebrospinal fluid.

Neuroendocrine (Merkel cell) carcinoma of the skin is a rare entity. Often locally aggressive, this lesion may also metastasize to organ systems, including bone, liver, and brain. The authors report a case of a 64-year-old male who presented with hoarseness and dysphagia 17 months after resection of a primary Merkel cell carcinoma of the nose. Additional studies revealed bilateral vocal cord paralysis secondary to central nervous system dysfunction. Cytologic evaluation of the cerebrospinal fluid revealed malignant tumor cells consistent with metastatic Merkel cell carcinoma. Presented are the cytologic and immunohistochemical findings in a case of metastatic Merkel cell carcinoma involving the central nervous system.

Carcinoma, Merkel Cell↗

Rhabdomyosarcoma arising in mediastinal teratoma in an adult man: a case report.

We report a case of rhabdomyosarcoma which occurred in a mediastinal teratoma in a 44-year-old man. Presentation symptoms were chest pain, hoarseness and a cough. Diagnosis was fortuitous, performed by the histological and immunohistochemical study of a mediastinal tumour biopsy specimen that showed embryonal carcinoma and yolk sac tumour components associated with the rhabdomyosarcoma. After cisplatin-based chemotherapy (bleomycin-etoposide-cisplatin), surgical resection of the residual mediastinal tumour was performed. Histological and immunohistochemical study of this tumour confirmed the presence of mature teratoma and embryonal rhabdomyosarcoma. Evolution was marked by a local extension of the mediastinal tumour, occurrence of multiple metastases and bone marrow involvement. The patient died 8 months after diagnosis despite chemotherapy and radiotherapy. A review of the literature reveals that the development of rhabdomyosarcoma in primary mediastinal teratomas is unusual in adults. The diagnostic, therapeutic and prognostic implications of such an association are reviewed.

Adult↗

Sore throat after operation: influence of tracheal intubation, intracuff pressure and type of cuff.

One hundred and eighty-four patients were studied to elucidate the contribution of intracuff pressure and cuff type to the occurrence of sore throat and hoarseness after operation. The patients were allocated to one of the following groups: A = mask only; B = reusable Rüsch tube with intermittent cuff volume adjustment; C = reusable Rüsch tube without cuff volume adjustment; D = disposable Portex Blue Line tube with intermittent cuff volume adjustment; E = disposable Shiley Low Pressure tube with intermittent cuff volume adjustment. Nitrous oxide was a component of anaesthesia in all patients. Moderate or severe symptoms were recorded in 30-33% of the patients in groups C, D and E, contrasting with group B, in which these sequelae were seen in only 10% of patients (P less than 0.025). All sequelae occurred less frequently in group A than in any of the other groups (P less than 0.025). Women were more likely to develop sore throat after intubation than were men (P less than 0.01). A possible relationship between differences in cuff-trachea contact area is postulated.

Adolescent↗

Alkalinization of intra-cuff lidocaine and use of gel lubrication protect against tracheal tube-induced emergence phenomena.

BACKGROUND: We sought to determine the benefits of using alkalinized lidocaine 40 mg to fill the cuff of a tracheal tube (ETT) in combination with water-soluble gel lubrication to prevent post-intubation sore throat. METHODS: The work included an in vitro study of the diffusion of alkalinized lidocaine solution through the low-pressure, high-volume cuff of an ETT. We also performed a randomized controlled study (n=20 patients in each group) that included a group who received an alkalinized lidocaine-filled ETT cuff with lubrication of the tube using water-soluble gel (Group G), and two control groups who received an alkalinized lidocaine-filled cuff with ETT lubrication with water (Group W) or an air-filled cuff with ETT lubrication with water (Group C). RESULTS: Water-soluble gel lubrication (Group G) produced a lower incidence of sore throat during the 24-h post-extubation period than lubrication with water alone in the cuffs filled with alkalinized lidocaine (Group W), and compared with the air control group. The ability of lidocaine to pass through the cuff of an ETT when water-soluble gel and/or water alone was used as a lubricant was similar, as determined by lidocaine plasma concentrations (C(max) 45 ng x ml(-1)). Cough and restlessness before tracheal extubation were decreased in patients with the alkalinized lidocaine-filled cuffs compared with the air-filled cuffs. After extubation, nausea, vomiting, dysphonia and hoarseness were greater for patients with air-filled cuffs compared with the lidocaine-filled cuffs. No significant difference between the groups was recorded in arterial blood pressure and heart rate. In vitro data suggest that the lower the NaHCO(3) injection volume, the greater the release of lidocaine across a low-pressure, high-volume cuff. CONCLUSIONS: These data show benefits of using an alkalinized lidocaine-filled ETT cuff in combination with water-soluble gel lubrication in preventing post-intubation sore throat.

Adult↗

Clinical, electrophysiological and morphological findings of Charcot-Marie-Tooth neuropathy with vocal cord palsy and mutations in the GDAP1 gene.

Three Spanish families with an autosomal recessive severe hereditary motor and sensory neuropathy, showing mutations in the ganglioside-induced-differentiation-associated protein 1 (GDAP1) gene in the Charcot-Marie-Tooth (CMT) type 4A locus were studied. The disorder started in the neonatal period or early infancy with weakness and wasting of the feet and, subsequently, involvement of the hands, causing severe disability. By the late teens, some patients developed a hoarse voice and vocal cord paresis. Peripheral motor nerve conduction velocity (MNCV) could not be measured in many cases because of the absence of muscle response due to distal atrophy. However, latencies to proximal muscles were in the normal range; median MNCV was >40 m/s in those cases in which it could be measured. Sural nerve biopsy from two patients showed a pronounced depletion of myelinated fibres, regenerative clusters and signs of axonal atrophy. Additionally, a small proportion of thin myelinated fibres and proliferation of Schwann cells forming onion bulb structures were also found. Unmyelinated fibre population was markedly increased. These findings are indicative of a predominant axonal degeneration with some demyelinating features. These Spanish families share in the severe CMT clinical phenotype with some Tunisian families who also presented mutations in the GDAP1 gene and to which the CMT4A locus was originally assigned. However, our families differ in the presence of laryngeal involvement and values of MNCV and pathological features are more in line with CMT2 type. The possibility that GDAP1 gene mutations could be expressed under different phenotypes is a question to be resolved.

Adult↗

Diagnosis of lung cancer in primary care: a structured review.

BACKGROUND: Lung cancer has the highest cancer incidence and mortality in the UK. Despite this, an individual GP encounters only one new presentation approximately every 8 months, so gains relatively little experience of its diagnosis. This is partly addressed by referral guidelines which aim to help GPs in selection of patients for chest X-ray or referral for specialist investigation. OBJECTIVE: The purpose of this study was to review the primary care presenting features of lung cancer, in the light of the UK Referral Guidelines for Suspected Cancer. METHODS: A structured literature review was carried out. RESULTS: Little research has been undertaken in primary care, and the predictive values for most symptoms are unknown. Approximate likelihood ratios could be calculated for six symptoms or signs: haemoptysis 13; fatigue 5.7; cough 5.3; finger clubbing 3.9; weight loss 2.9; and dyspnoea 1.5-5.7, but none of these figures derived from single primary care studies. Three recommendations for urgent investigation of possible lung cancer in the UK Referral Guidelines are questioned: for unexplained dyspnoea, hoarseness or cervical lymphadenopathy. For all these presentations, other serious diagnoses are more likely. CONCLUSION: The UK Guidelines for referral of suspected lung cancer have a weak evidence base.

Diagnosis, Differential↗

Lipoid proteinosis maps to 1q21 and is caused by mutations in the extracellular matrix protein 1 gene (ECM1).

Lipoid proteinosis (LP), also known as hyalinosis cutis et mucosae or Urbach-Wiethe disease (OMIM 247100) is a rare, autosomal recessive disorder typified by generalized thickening of skin, mucosae and certain viscera. Classical features include beaded eyelid papules and laryngeal infiltration leading to hoarseness. Histologically, there is widespread deposition of hyaline (glycoprotein) material and disruption/reduplication of basement membrane. The aetiology of LP is currently unknown. Using DNA from three affected siblings in a consanguineous Saudi Arabian family we performed genome-wide linkage and mapped the disorder to 1q21 (marker D1S498) with a two-point LOD score of 3.45 at theta = 0. A further 28 affected individuals from five other unrelated consanguineous family groups from different geographical regions also showed complete linkage and resulted in a maximum two-point LOD score of 21.85 at theta = 0. Using available markers in the interval between D1S442 and D1S305, the observed recombinants placed the gene in a 2.3 cM critical interval between D1S2344 and D1S2343 (Marshfield genetic map) corresponding to an approximately 6.5 Mb region on the UCSC physical map. Using a candidate gene approach (comparison of control versus LP gene expression in cultured fibroblasts) and subsequent direct sequencing of genomic DNA, we identified six different homozygous loss-of-function mutations in the extracellular matrix protein 1 gene (ECM1). Although the precise function of ECM1 is not known, our findings provide the first clinical indication of its relevance to skin adhesion, epidermal differentiation, wound healing, scarring, angiogenesis/angiopathy and basement membrane physiology, as well as defining the molecular basis of this inherited disorder.

Blood Vessels↗

Chlamydia pneumoniae strain TWAR, Mycoplasma pneumoniae, and viral infections in acute respiratory disease in a university student health clinic population.

Clinical and serologic data were collected on 667 University of Washington students who presented to the David Hall Student Health Center between 1983 and 1987 with acute respiratory disease. Sera were tested for evidence of acute or past infections with Chlamydia pneumoniae strain TWAR, Chlamydia trachomatis, Mycoplasma pneumoniae, influenza A virus, influenza B virus, adenovirus, and respiratory syncytial virus. Pharyngeal swab specimens were cultured for C. pneumoniae and C. trachomatis, but not for the other agents. Evidence of acute infection with C. pneumoniae was found in 20 patients and evidence of an acute infection with M. pneumoniae in 29 patients. C. pneumoniae was associated with 9% and M. pneumoniae with 11% of 149 pneumonias diagnosed clinically, and with 20% and 22%, respectively, of the 59 pneumonias confirmed on chest radiograph. There was no evidence of seasonality in C. pneumoniae or M. pneumoniae infections. Compared with patients with M. pneumoniae, patients with C. pneumoniae were less likely to have a temperature greater than 37.8 degrees C (10% vs. 34%), but were more likely to present with a sore throat (80% vs. 52%) or hoarseness (30% vs. 3%). The mean number of days from onset of symptoms until enrollment was longer in patients with C. pneumoniae infections than in those with M. pneumoniae (12.8 vs. 7.9 days), or those with a viral infection (12.8 vs. 7.3 days), suggesting a more gradual onset of disease caused by C. pneumoniae.

Adolescent↗

A case report of a three-year survivor with advanced non-small cell lung cancer producing alpha-fetoprotein.

An 80-year-old patient with poorly differentiated adenocarcinoma of the left lung with metastasis to both lungs and supraclavicular lymph nodes, stage III M1 (T2N2M1), was treated with cisplatin (cis-diamminedichloroplatinum, CDDP) at a dose of 80 mg/m2 intravenously. He achieved a partial response, however, he could not continue therapy with CDDP because of its renal toxicity. He was then given etoposide and vindesine as a single chemotherapeutic agent, but no response was observed. Therefore, CDDP was administered again in a fractionated regimen, the first course of treatment achieved a minor response, but the second course resulted in stable disease. He therefore received combination chemotherapy consisting of mitomycin, vindesine and CDDP, and tumor regression of more than 30% was observed after one course of this combination chemotherapy. After three years from the initiation of chemotherapy, he had no symptoms except for hoarseness, and has been followed up on an ambulatory basis. In addition, the tumor was producing alpha-fetoprotein (AFP), which was shown by immunohistochemical staining with polyclonal antibody against AFP. The changes in serum AFP level correlated well with the disease status.

Adenocarcinoma↗

Effect of zincum gluconicum nasal gel on the duration and symptom severity of the common cold in otherwise healthy adults.

BACKGROUND: Previous studies suggest that zinc salts may be effective in treating the common cold. Since rhinovirus infections occur primarily in the nasal cavity, an attempt to arrest the infection at the portal of entry seems logical. AIM: To assess the ability of zinc nasal gel to shorten the duration and reduce the severity of the common cold in healthy adults. STUDY DESIGN: Randomized, double blind, placebo-controlled study. METHODS: Of 1087 patients screened by telephone, 80 patients were enrolled, all presenting within 24-48 h of the onset of illness. They received one dose per nostril of a nasal gel spray containing either 33 mmol/l zincum gluconicum, or an identical placebo four times daily until their symptoms resolved, for a maximum of 10 days. RESULTS: Median duration of cold symptoms in the zinc group was significantly shorter than in the placebo group (median [IQR] 4.3 days [2.5-5.5] vs. 6 days [5-8.5], p=0.002). Nasal drainage, nasal congestion, hoarseness, and sore throat were the symptoms most affected. Significant reduction of total symptom scores started from the second day of the study. Adverse effects (mainly nasal stinging) were similar in both groups. DISCUSSION: Zincum gluconicum nasal gel shortens duration and reduces symptom severity of the common cold in healthy adults, when started within 24-48 h of the onset of illness.

Administration, Intranasal↗

Vesicular lesions in a patient with lipoid proteinosis: a probable acantholytic dermatosis.

Lipoid proteinosis is a rare genodermatosis with characteristic clinical and histologic findings later in life. We present a case of lipoid proteinosis in which the diagnosis was originally missed because the patient presented in childhood with vesicles and a hoarse voice and lacked any other manifestations. Microscopic examination of a vesicle revealed extensive non-dyskeratotic acantholysis. Vesiculo-bullous lesions have been described clinically in lipoid proteinosis, but we are unaware of any histopathologic description regarding bullae formation. Although only a single patient is herein described, it seems unlikely that this type of acantholysis is purely coincidental. It is proposed that lipoid proteinosis should be in the differential of acantholytic vesiculo-bullous lesions in a child.

Acantholysis↗

Acute epiglottitis in adults.

We treated four adults whose upper airway was compromised due to acute epiglottitis. We also reviewed the English literature for all reports of this condition in adults (18 years and older). Among the 158 cases, the infectious etiology was identified in 29 (H. influenzae 20, Streptococcus pneumoniae six, H. parainfluenzae two, Streptococcus pyogenes one). In the remaining cases, the etiology was uncertain. Bacteremia was documented in 23/32 patients (71.9%), but extra-epiglottic infections were strikingly rare (X = six). The clinical manifestations were sore throat (100%), fever (88%), dyspnea (78%), dysphagia (76%), anterior neck cellulitis or tenderness (27%), hoarseness (21%), pharyngitis (20%) and anterior cervical lymphadenopathy (9%). Complete airway obstruction ensued in 23 out of the 119 subjects (18.3%) who had respiratory difficulty. Overall mortality rate was 17.6% but it was 6.4% among the patients who were semi-electively tracheostomized or endotracheally intubated. These findings illustrate that antibiotics therapy active against H. influenzae is required in the treatment of acute epiglottitis in adults. Additionally, airway patency should be established when inspiratory stridor appears assuring uncomplicated recovery.

Acute Disease↗

Unusual presentations of hypothyroidism.

Hypothyroidism is a commonly diagnosed endocrine disorder. Typical signs and symptoms of hypothyroidism include lethargy, cold intolerance, hoarseness, dry skin, constipation, delayed relaxation phase of deep tendon reflexes, and bradycardia. Hypothyroidism, presenting with such classic manifestations, usually is readily recognized and, therefore, easy to diagnose. Occasionally patients have less commonly emphasized symptoms, making the diagnosis less apparent. Such atypical presentations may suggest other diseases as the primary problem and, therefore, the initial focus of attention is on a diagnosis other than hypothyroidism. We have observed patients with hypothyroidism with rare manifestations. The diagnosis of primary hypothyroidism was established in all patients by thyroid function tests, and initiation of thyroid hormone therapy resulted in significant improvement of the presenting symptom. We considered it instructive to report about these patients because it shows the need to be aware of the unusual presentations of hypothyroidism and to consider hypothyroidism when confronted with atypical clinical manifestations.

Adult↗