Kimura's disease

An Asian man aged 42 had a pre-auricular swelling on the left with local skin itching. Owing to the highly probably benign nature at examination, surgical treatment was refrained from. One year later, the swelling had increased while the itching was unchanged, so that surgical excision was performed. Morbid-anatomical examination revealed angiolymphoid hyperplasia with eosinophilia, compatible with Kimura's disease. Kimura's disease is a chronic inflammatory disorder which is very rare in Europe, but which is frequently diagnosed in Asian countries and in immigrants from these countries. Tumours in the head and neck region, enlarged lymph nodes and increased eosinophil counts are typical signs. The IgE level is increased. The cause of this disease is so far not clear, although there are indications that an inflammatory reaction to an unknown allergen plays a part. It is essential that this disease should be distinguished in the diagnostic work-up from presence of a malignancy. Fine-needle aspiration cytology is often inconclusive; as a rule the diagnosis can only be made after surgical excision of the tumour. There is no consensus on the treatment of this disease, but symptomatic therapy usually suffices.     

Coexistence of Kimura's disease and lichen amyloidosus in three patients

Three patients had Kimura's disease with multiple asymptomatic subcutaneous tumors of 11 to 20 years' duration on the head, soft palate, elbow, or inguinal regions associated with enlarged regional lymph nodes. In each patient, lichen amyloidosus had also developed on the extensor surfaces of the extremities during the past 12 to 20 years. Blood eosinophilia (26% to 75%) was present in all cases. The possible pathogenetic relationship between Kimura's disease and primary localized cutaneous amyloidosis is discussed.     

Mast cells in elastic cartilage of the human epiglottis

Light and transmission electron microscopy revealed in the elastic cartilage of the human epiglottis the presence of mast cells. Cytoplasmic granules of these cells contain characteristic cylindrical bodies, whose texture is different from that found in cytoplasmic granules of mast cells from other sites. The presence of mast cells in elastic cartilage, described for the first time, and the different substructure of their cytoplasmic granules, is considered as another proof of the heterogeneity of that cell type.     

Bifid epiglottis: a rare laryngeal anomaly

Two cases of bifid epiglottis are presented: one with an associated laryngeal cyst and another with an associated cricoid stenosis. The occurrence of multiple laryngeal anomalies in association with bifid epiglottis has not previously been described. The occurrence of an extra digit is noted to be statistically significant both in the current series and in a review of the literature. A brief review of the embryologic classification and staging by the Carnegie System, and the correlation of the time sequence of development of the epiglottis is presented. No correlation is made as to the mechanism of the origin of this laryngeal anomaly, as adequate embryologic knowledge of the development of the pharynx is not available at this time.     

The elastic cartilage in the normal rat epiglottis. I. Fine structure

Unilateral electrolytic lesions of the locus coeruleus in rats result in spontaneous ipsiversive rotation, which is then replaced by contraversive rotation. One week after lesioning, when spontaneous turning ceases, apomorphine and d-amphetamine elicit contraversive circling behaviour, which was not affected by noradrenergic receptor blockade but was abolished by dopamine receptor blockade. The drug-induced contraversive circling response was also reproduced by piribedil but not clonidine. Combined unilateral electrolytic locus coeruleus and substantia nigra lesions on the same side resulted in apomorphine- and d-amphetamine-induced ipsilateral rotational behaviour which was indistinguishable from that seen with substantia nigra lesions alone. In rats with unilateral locus coeruleus lesions, the dose of intrastriatally injected apomorphine required to produce circling was less on the lesioned than the non-lesioned side. Direct injection of noradrenaline into one substantia nigra caused contraversive circling. Direct injection of phenoxybenzamine into one substantia nigra followed by apomorphine caused ipsiversive circling. The results suggest that the circling behaviour seen after unilateral locus coeruleus lesions depends on an asymmetry of striatal dopamine receptor activity and are consistent with a proposed coeruleus-nigral noradrenergic pathway, which enhances impulse flow in the dopaminergic nigrostriatal system.     

Congenital laryngeal stridor secondary to flaccid epiglottis, anomalous accessory cartilages and redundant aryepiglottic folds

Most laryngeal anomalies are supraglottic and laryngomalacia is the most common. Cysts, bifid epiglottis and absence of the epiglottis are uncommon. An 18-year-old Caucasian man had long-standing stridor caused by anomalous supraglottic structures: a small floppy epiglottis, enlarged accessory cartilages and redundant aryepiglottic folds. These structures were excised and the airway was improved. The ventral portions of the fourth arches become the aryepiglottic folds and lateral segments of the epiglottis. A disturbance in this portion of the fourth arch may explain the anomaly. The cartilaginous contributions to the epiglottis were possibly isolated as accessory cartilages. Epiglottic anomalies may be associated with other anomalies, especially the digits of the hand. This patient had a short lingual frenulum and mild macroglossia.     

Primary Hodgkin disease of the ileum complicating Crohn disease

BACKGROUND: Primary gastrointestinal lymphoma currently is considered to be an uncommon complication of chronic inflammatory bowel disease. All tumors reported in which recently developed techniques, such as immunohistochemical markers, were used for lymphoma classification proved to be non-Hodgkin lymphomas. Gastrointestinal lymphomas developing in Crohn disease are a very heterogeneous group, with tumors of both B-cell and T-cell lineage represented, along with some tumors of equivocal phenotype. By contrast, gastrointestinal lymphomas complicating ulcerative colitis all have proved to be so-called polymorphic B-cell lymphomas. METHODS: The current report describes another case of primary gastrointestinal lymphoma complicating chronic inflammatory bowel disease occurring in the ileum of a 34-year-old man with a 3-year history of Crohn disease. RESULTS: Histopathologic findings were in keeping with nodular sclerosing Hodgkin disease. Broad birefringent collagen bands divided the tumor into well-defined nodules consisting of typical Reed-Sternberg cells and lacunar variants admixed with a polymorphous lymphoid infiltrate. By immunohistochemical studies, Reed-Sternberg cells and lacunar variants stained positively for Leu-M1 (CD15) and Ber H2 (CD30). The background lymphocytes were primarily of T-cell phenotype. CONCLUSIONS: To the knowledge of the authors, this article reports the first case of primary gastrointestinal Hodgkin disease in association with chronic inflammatory bowel disease that has been confirmed by immunohistochemical studies.     

Ischemic disease of the intestine

With the remarkable progress in diagnostic techniques, the recent increase in the incidence of arteriosclerosis and the gradually aging population, a great deal of attention is now being focused on intestinal ischemia and this disease has come to be regarded as one of the clinically important pathological conditions. From the clinic aspect, special attention should be directed to the artery which is involved in intestinal ischemia. Furthermore acute superior mesenteric arterial ischemia (SMA ischemia), ischemic colitis, non-occlusive mesenteric ischemia (NOMI) require serious attentions. Few specific findings of these pathological conditions can be obtained in general examinations, therefore angiography is the most useful technique for making difinitive diagnoses. SMA ischemia and NOMI are progressive diseases and their prognoses are poor. The progress of ischemic colitis is gradual and the conservative therapy consisting of fasting, drip infusion and antibiotic administration is generally effective in treating this disease.     

Gorham disease of the spine

STUDY DESIGN: This is a case report of Gorham disease of the spine with review of the literature. OBJECTIVE: To review the diagnosis, therapy, clinical course, and prognosis of Gorham disease of the spine. SUMMARY OF BACKGROUND DATA: This is the 17th reported case of spinal involvement by this rare type of idiopathic osteolysis. METHODS: The patient was studied with radiographs, nuclear bone scans, computed tomography scans, magnetic resonance scans, and biopsies. His spine was stabilized by posterior and anterior rods, corpectomies, and bone grafts. RESULTS: The patient's spine had remained stable for 22 months after surgery, but intractable chylothoraces and spreading bone destruction were present. CONCLUSIONS: Spinal Gorham disease has high morbidity and mortality, but the course in an individual patient is difficult to predict. Early spinal stabilization should be considered before irreversible neurologic complications occur.     

Predicting the course of disease

The ability to predict the course of disease and the effect of interventions is critical to effective medical practice and health care management. In this analysis, we sought to test whether available clinical data and analytic methodologies can be used to accurately predict the time course of the probability of death after hospital admission and the probability of readmission following discharge for patients with acute myocardial infarction or pulmonary disease. We grouped patients by selected physiologic characteristics and made time-to-event predictions using multiple regression models. These predictions were compared with observed probabilities calculated using the actuarial or life-table method. Predictions made with the Bailey-Makeham model generally replicated observed experience. They accurately accounted for substantial differences in the patterns of death and readmission and accurately delineated the effects of therapies, after adjustment for patient risk. These results were validated by analyses of resampled populations that differed in case mix from the source population. We believe that using such models to project the course of disease and the effects of treatment on that course in defined classes of patients should facilitate the development of practice guidelines for patient care and the management of health care resources.