Langerhans' cell histiocytosis with thyroid involvement masquerading as thyroid carcinoma

A 28-year-old woman was admitted to our Hospital with a chief complaint of progressive gingival swelling and loosening of teeth over about a year. According to past history, she had received total thyroidectomy 2 years previously due to thyromegaly. The thyroidectomy specimen was at first interpreted as 'poorly differentiated carcinoma of the thyroid'. One year ago, she began to be aware of gingival swelling and loosening of teeth. A gum biopsy was taken and the pathologic features were similar to her 'thyroid carcinoma'. Subsequent investigations, including immunohistochemical stain, showed the gum was heavily infiltrated with histiocyte-like Langerhans' cells which were positive for S-100 protein. Ultrastructural examination of the cells under electron microscope revealed many typical intra-cytoplasmic Birbeck granules. Langerhans' cell histiocytosis was diagnosed. Langerhans' cell histiocytosis with thyroid involvement is extremely rare and may run a relatively indolent course. Even on a retrospective examination, it may easily be confused with poorly differentiated carcinoma of the thyroid. We suspect that this error may have been made on other occasions and that the occurrence of this condition may be underreported.      

Malnutrition as a prognostic factor in lymphoblastic leukaemia: a multivariate analysis

One hundred and twenty eight Brazilian children with lymphoblastic leukaemia were intensively treated with a Berlin-Frankfurt-Munich based protocol. More children had a white cell count above 50 x 10(9)/l (31%) then observed in developed countries. After a median follow up of 31 months (11-58 months), the estimated probability of relapse free survival was 41% (7%) for the whole group. After adjustment in the Cox's multivariate model, malnutrition was the most significant adverse factor affecting duration of complete remission. Age above 8 years and high peripheral white cell count were also significant adverse factors. Among the nutritional indices, the height for age and weight for age z scores were both significant, whether the cut off points of z-2 or z = -1.28 were chosen to define malnutrition. A strong statistical association between the two indices was found; the contribution of height for age z score to the prediction of relapse free survival was more significant. Children with height for age z score < -2 had a relapse risk of 8.2 (95% confidence interval 3.1 to 21.9) relative to children with z score > -2. The results of this study suggest that socioeconomic and nutritional factors should be considered in the prognostic evaluation of children with leukaemia in developing countries.     

Dynamic computed tomography of the head and neck: differential diagnostic value

A retrospective review of the dynamic CT studies performed in our institution on head and neck lesions, excluding the brain, was carried out. Five basic types of density vs. time curves were obtained. Dynamic CT scanning is valuable in the differential diagnosis, management, and followup of such cases; its usefulness as an imaging modality in diagnosis and followup of hemangiomas is stressed.     

Morphological abnormalities of the terminal neuromuscular apparatus in recent juvenile diabetes

Summary Muscle biopsies from eight cases of acute juvenile diabetes (a few weeks after the beginning of the disease) revealed well marked degenerative changes combined with vigorous regeneration of the terminal neuromuscular apparatus. This observation is in accordance with previous demonstrations of neurophysiological disturbances at this early stage of diabetes.

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Morphologische Anomalien der neuromuskulären Endplatte bei frisch entdeckten juvenilen Diabetikern

Zusammenfassung Muskelbiopsien von 8 Patienten mit akutem juvenilem Diabetes (einige Wochen nach Krankheitsausbruch) zeigten ausgeprägte degenerative Veränderungen mit kräftigen Regenerationserscheinungen an der neuromuskulären Endplatte. Diese Befunde stimmen mit früher mitgeteilten Beobachtungen neurophysiologischer Störungen dieses Frühstadiums des Diabetes überein.

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Anomalies morphologiques de l'appareil neuro-musculaire terminal dans le diabète juvénile récent

Résumé Dans huit cas de diabete juvénile aigu (quelques semaines après le début de la maladie) des biopsies musculaires ont révélé des modifications dégénératives bien marquées, associées á une puissante régénération de l'appareil neuro-musculaire terminal. Cette observation est en accord avec les démonstrations précédentes de troubles neurophysiologiques à ce stade précoce du diabète.

     

Epithelioid sarcoma of the hand

Twenty-eight patients were treated for a primary epithelioid sarcoma of the hand. Twenty-seven patients (96%) had excisions before evaluation, including 11 (39%) with multiple prior excisions with varying diagnoses before epithelioid sarcoma, and all had surgical treatment after referral. The patients' surgical management included three patients with amputation at the forearm, three patients with wide excision, and 21 patients with a partial amputation of the hand. The followup period averaged 120 months (range, 24-276 months). Eighteen patients have no evidence of disease at last followup. Treatment failures included one local recurrence, four regional metastases, and five distant metastases. Five patients died secondary to disease. Two patients are alive with disease, and three are alive with no evidence of disease after additional treatment. After aggressive surgical management with negative margins, 71% of the patients were alive without evidence of disease at the last followup, with a 5- and 10-year survivorship of 85%. Our goal is to review: (1) the effectiveness of preoperative imaging, (2) the role of adjuvant therapy, (3) survival after alternative resections, and (4) function after resection.     

Adenocarcinomas of the jejunum and ileum: A 25-year experience

Adenocarcinomas of the jejunum and ileum are rare gastrointestinal malignancies. Because few large published experiences exist, we reviewed patients with jejunal and ileal adenocarcinoma treated at our institution over the last 25 years. Between January 1976 and December 2001, 77 patients had an operation for a jejunal or ileal adenocarcinoma. Records were retrospectively reviewed for patient, tumor, and treatment variables. Factors affecting disease recurrence and patient survival were investigated. Fifty-two of the adenocarcinomas (67%) occurred in the jejunum and 25 occured in the ileum (33%). Mean patient age was 63 ±14 years. Segmental bowel resection was performed in 50 patients (65%) with curative intent. Palliative operative procedures including resection or bypass were performed in 27 patients (35%). One (1%) patient had stage I, 18 (23%) stage II, 19 (25%) stage III, and 39 (51%) stage IV adenocarcinoma at diagnosis. Postoperatively, 12 patients had palliative and 18 adjuvant chemotherapy (n 5 30), radiation therapy (n = 1), or combination treatment (n = 7). Median patient survival was 19 months. Sixty-six percent of patients who had a curative operation had a tumor relapse. Tumor stage had a highly significant effect (P < 0.0001) on median survival (72 months for stage I and II, 30 months for stage III, and 9 months for stage IV disease). In multivariate analysis of patients having curative treatment, tumor recurrence (P < 0.0001), stage (P < 0.0002), and weight loss (P < 0.001) were significant negative prognostic indicators. Most patients with adenocarcinoma of the jejunum or ileum present with advanced disease. Tumor stage, disease recurrence, and weight loss predicted patient outcome following a curative operation. Early recognition of these tumors requires a high index of suspicion. Presented at the Forty-Fifth Annual Meeting of The Society for Surgery of the Alimentary Tract, New Orleans, Louisana, May 15–19, 2004 (poster presentation).     

The effect of the summer 2003 heat wave on mortality in the Netherlands

In the Netherlands, between 1400 and 2200 deaths in the summer of 2003 may have been heat-related. The fact that the maximum temperatures were lower than in some other European countries, and occurred in less heavily populated areas, may have led to mortality figures that were relatively less dramatic. The temporarily increased death rates are only partly due to a forward shift of mortality. Heat-related mortality was most pronounced among the elderly in nursing homes.     

Toxicity of photomirex with special reference to porphyria,hepatic P-450 and glutathione levels,serum enzymes,histology and residues in the quail and rat

Conclusions Photomirex, given either as single oral doses up to 1000 mg/kg to quails or as 100 ppm diet to quails and female rats for 27 days, caused moderate hepatic changes consisting of an increase in liver weight and cytoplasmic enlargement of hepatocytes. Hepatic cytochrome P-450 contents of quails but not rats were also increased. There were no indications of severe damage to the livers of these animals (alterations in glutathione level and serum profile, porphyria and necrosis).