Hereditary angioedema. A rare cause of acute abdominal pain with ascites

HISTORY AND CLINICAL FINDINGS: Since the age of 16 years a now 25-year-old woman had been known to have C1-inhibitor (C1-INH) deficiency. She presented herself at the emergency department because of acute severe lower abdominal cramps. A urinary infection had been treated with antibiotics for the previous 4 days. There was marked pain on pressure over the lower abdomen, but there were no signs of peritonitis and bowel sound were normal. There had been no nausea or vomiting and the stools had been normal. INVESTIGATIONS: There was a leukocytosis of 10,200/microliter, moderately elevated C-reactive protein (44.8 mg/l), haemoglobin concentration of 17 g/dl and haematocrit of 51%. Radiology revealed oedema of the duodenum and sonography showed free fluid in the abdomen. TREATMENT AND COURSE: After excluding an acute abdomen and in view of the C1-INH deficiency treatment was symptomatic. All symptoms completely disappeared after 2 days. CONCLUSIONS: Exclusively gastrointestinal symptoms and ascites are rare in patients with hereditary angioedema. But knowledge of this manifestation of the disease is important because patients are sometimes operated under the false diagnosis of acute abdomen. In severe cases symptomatic treatment may have to be supplemented by C1-INH administration. Prevention with attenuated androgens should be started or modified, respectively.     

Hereditary neurocutaneous angiomatosis. Report of four cases

The authors report the coexistence of vascular nevi (hemangiomas and arteriovenous malformations (AVMs) of the skin) with AVMs and venous malformations of the brain in male siblings from two related but nonconsanguineous families of three generations. The proband, his siblings, parents, aunts, uncles, and cousins were examined, underwent magnetic resonance (MR) imaging and MR angiography, and when appropriate, cerebral angiography. A father had vascular nevi and a mother, his sister, had an azygos anterior cerebral artery. No other cutaneous or cerebrovascular malformations were present in the parents. Each of the two families had two boys and one girl, 9 to 18 years of age. All the children had vascular nevi and all of the boys had coexisting cerebrovascular malformations: AVMs in three, and a venous malformation in another. One boy had three cerebral AVMs. Two boys had a cerebral hemorrhage, and one also had focal motor seizures. The skin lesions were not those of the Sturge-Weber-Dimitri, Rendu-Osler-Weber, or Wybum-Mason syndromes. The association of cutaneous and cerebrovascular malformations was seen only in males in these families. but females have also been reported in the literature. The results obtained in these families and three other families reported from Western and Central Europe indicate that the association of cerebral and cutaneous vascular hamartomas constitutes a distinct, hereditary clinicopathological entity with autosomal dominant inheritance and variable penetrance. The clinical manifestations of this syndrome are visible, painful vascular nevi, epilepsy, cerebral hemorrhage, and focal neurological deficits. The preponderance of male patients with the full expression of the syndrome suggests a possible hormonal influence on the expression of the gene.     

Fetomaternal macrotransfusion--a cause for decreased fetal movements. 2 cases

Two cases of fetomaternal macrotransfusion in otherwise normal pregnancies are reported. In both cases the main symptoms observed by the patients were decreasing fetal movements. Cardiotocography revealed a highly pathologic pattern an immediate cesarean section was performed. The cause of massive fetomaternal hemorrhage (390 and 635 ml fetal blood) remains unclear. Delayed treatment leads to severe anemia followed by hypovolemic shock and ultimately to stillbirth. Pregnancies complicated by fetomaternal hemorrhage develop normally until signs of fetal decompensation begin to appear. Decreasing fetal movements in the most common symptom reported by the patients. In addition to a contraction-stress-test, blood from the pregnant women should be looked at for fetal erythrocytes, a test that can be performed very quickly and easily. If the fetus is viable, immediate delivery should be performed and blood transfusions to the newborn should be administered. In preterm pregnancies cordocentesis and intrauterine blood transfusion may be considered.     

Primary pancreatic lymphoma. A rare cause of pain-free icterus

Toxocariosis is a zoonosis which has been widely studied in dogs. However, not much is known about this parasitosis in cats. The aim of the present work was to determine the frequency of Toxocara cati in domestic cats in Mexico City and the State of Mexico. Feces of 660 domestic cats were studied by the Faust concentration-floating test; 401 samples were from cats living in Mexico City of which 308 lived in houses and 93 in apartments; 231 were from urban areas of the State of Mexico and 28 from a rural area of the same state. The total frequency of T. cati eggs obtained from domestic cats in Mexico City was 42.9%; in cats living in apartments it was 18.3% and in cats living in houses it was 50.3%. In domestic cats from the State of Mexico, T. cati frequency was 36.4% in the urban and 21.4% in the rural areas. We consider that toxocariosis frequency observed at the two studied sites is high and that the need to prevent dissemination of the infectious forms of T. cati is urgent, as is the necessity of informing the population of the risk of living with T. cati parasited animals and of the anatomopathological alterations caused by T. cati in man.     

Esophageal cyst--a rare cause of backache

BACKGROUND: The duration of action of muscle relaxants is poorly correlated to the rate of decay of their plasma concentration. The plasma concentration of mivacurium may rapidly decrease below its active concentration because of the extensive hydrolysis of mivacurium. By inflating a tourniquet on one upper limb for 3 min after the administration of atracurium, mivacurium or vecuronium, we studied the influence of the initial decline of their plasma concentration on their effect. METHODS: In 50 patients anaesthetised with thiopental, isoflurane and fentanyl, the effect of bolus doses of 0.15 or 0.25 mg.kg-1 mivacurium (MIV 15, MIV 25), 0.3 or 0.5 mg.kg-1 atracurium (ATR 30, ATR 50) and 0.06 or 0.1 mg.kg-1 vecuronium (VEC 06, VEC 10) were measured on both arms (evoked response of the adductor pollicis to train-of-four stimulation every 12 s), a tourniquet being applied on one arm just before and during 3 min after the muscle relaxant bolus. RESULTS: Tourniquet inflation of 3 min almost abolished the neuromuscular effect of mivacurium. In the vecuronium groups and in the ATR 50 group, tourniquet inflation did not modify the maximum degree of depression of the twitch response. Also, the duration of action of vecuronium was unaffected by the tourniquet. In the ATR 30 group, times to return of the twitch response to 25% (duration 25%) and 75% (duration 75%) of control response were significantly shorter in the cuffed arm, 23 min vs 27 min, and 41 min vs 45 min, respectively. In the ATR 50 group, only duration 25% was significantly shorter in the cuffed arm (41 min vs 45 min). CONCLUSION: The results suggest that the rate of decline of the plasma concentration of mivacurium is so rapid, that a very low and almost clinically ineffective concentration is present as soon as 3 min after its administration. The results also indicate that the recovery from a mivacurium-induced neuromuscular blockade is not influenced by the rate of decay of its plasma concentration in patients with genotypically normal plasma cholinesterase.     

Dieulafoy colonic ulcer. A rare cause of lower gastrointestinal hemorrhage

Dieulafoy's disease is an unusual cause of gastrointestinal hemorrhage, reported to account for less than 2% of acute gastrointestinal bleeding episodes. Bleeding occurs from a defect in an unusually large submucosal artery, through a minute mucosal erosion. Endoscopic diagnosis is sometimes difficult, but primary endoscopic therapy may be successful and should be attempted. In most cases the lesion is found in the proximal stomach. Sixteen cases of Dieulafoy's lesion located in the colon have been reported in the literature but only nine have been confirmed by histology. We present the case of a 63 year-old male with Dieulafoy's lesion of the transverse colon which was diagnosed by endoscopy and confirmed by histology.     

A rare cause of urinary frequency]

Induction and maintenance of peripheral tolerance are important mechanisms to maintain the balance of the immune system. In addition to the deletion of T cells and their failure to respond in certain circumstances, active suppression mediated by T cells or T-cell factors has been proposed as a mechanism for maintaining peripheral tolerance. However, the inability to isolate and clone regulatory T cells involved in antigen-specific inhibition of immune responses has made it difficult to understand the mechanisms underlying such suppression. Here, we show that chronic activation of both human and murine CD4+ T cells in the presence of interleukin (IL)-10 gives rise to CD4+ T-cell clones with low proliferative capacity, producing high levels of IL-10, low levels of IL-2 and no IL-4. These antigen-specific T-cell clones suppress the proliferation of CD4+ T cells in response to antigen, and prevent colitis induced in SCID mice by pathogenic CD4+CD45RBhigh splenic T cells. Thus IL-10 drives the generation of a CD4+ T-cell subset, designated T regulatory cells 1 (Tr1), which suppresses antigen-specific immune responses and actively downregulates a pathological immune response in vivo.     

Intestinal ganglioneuromatosis--a rare cause of chronic diarrhea

A case of intestinal ganglioneuromatosis is reported. The symptoms were watery diarrhoea and abdominal pain of several months duration. Endoscopic examination of the oesophagus, ventricle, duodenum, colon and rectum was normal. Mucosal biopsies from colon and rectum revealed ganglia cells and thin nerve fibres in the lamina mucosa, giving the diagnosis ganglioneuromatosis. As a consequence of the diagnosis thyroid scintigraphy, CT-scanning of the thyroid and adrenal glands and measurement of serum calcitonin and gastrin were performed. The tests revealed an intrathoracic nodular struma, and beyond this no abnormalities. The relation of intestinal ganglioneuromatosis to Multiple Endocrine Neoplasia type II b is discussed and the necessity of performing mucosal-biopsy from endoscopically normal colonic mucosa in cases of chronic diarrhoea is emphasised.     

Autoimmune hepatitis--a rare cause of elevated transaminases

Autoimmune hepatitis is a rare chronic hepatitis of unknown etiology. Immunological changes are conspicuous, with tissue antibodies found in a high proportion of patients. There is a female preponderance of 2-3:1 and the disease often affects young people. The presenting symptoms are vague, fatigue being the most important general symptom. The diagnosis is often made at a late stage of the disease when symptoms of liver decompensation and cirrhosis are obvious. We present two cases of AIH in young women diagnosed at a late stage with advanced cirrhosis, for whom symptoms of liver disease had been intermittently present for 12 and 9 months, but wrongly attributed to presumed acute viral hepatitis acquired on holiday in the Mediterranean. In order not to miss the diagnosis, it is important to confirm suspected viral hepatitis serologically, to look for liver disease stigmata in patients with abnormal liver enzymes, and to be especially aware of the possibility of AIH in young women with concommittant or family history of other autoimmune diseases.     

A rare cause of dysphonia: laryngeal rhabdomyoma

The authors report their finding concerning the frequency of postoperative pulmonary thromboembolism in an autopsy series of subjects who had died with 15 days of surgery. In all cases the relationship was evaluated between death and age, sex, the underlying pathology and the type of surgery.     

Mesenteric cysts: rare cause of abdominal pain

For about two years a now 47-year-old woman had been suffering from heartburn, diarrhoea and constipation, at times also abdominal colic and a pressure sensation in the right lower abdomen. Physical examination and biochemical tests were according to age, except for an raised erythrocyte sedimentation rate of 25/50 mm. Ultrasound revealed an intraperitoneal echo-free multi-chambered structure, 11 x 5 x 2 cm, with dorsally increased echo density and smooth entry echo, apparently arising from the mesentery. Computed tomography confirmed these findings and at laparoscopy a cyst, 12 x 8 x 5 cm, was identified. It was filled with yellow clear fluid and arose from the mesentery of the ascending colon. The cyst was scooped out surgically. Histological examination showed a single-layered mesothelial coat. The postoperative course was unremarkable. At re-examination 8 months later the patient had remained symptom-free.     

Melanocanthoma: a rare cause of oral hyperpigmentation

The oral features of a black woman with melanocanthoma of the oral mucosa are detailed, and the current literature of melanocanthoma of the oral mucosa is briefly reviewed.     

Bilateral massive macronodular adrenal gland hyperplasia. A rare cause of Cushing's syndrome

A 46-year-old man with known arterial hypertension for 10 years had, over the last two years, developed increasing obesity, particularly of the trunk, with other symptoms typical of Cushing's syndrome. Hormone analysis demonstrated hypercortisolism and decreased plasma ACTH concentration. The dexamethasone inhibition test failed to show any significant suppression of serum cortisol. Plasma ACTH was not increased in the corticotrophin-releasing hormone and the metyrapone tests. In the short ACTH test there was an excessive cortisol increase. Abdominal computed tomography revealed both adrenals to be enlarged (6 x 4 cm) and coarsely nodular. Adrenolytic treatment with ketoconazole (400 mg daily) caused symptoms of adrenal insufficiency, but a reduced dosage of 200 mg daily lowered the cortisol level to between 5 and 11 micrograms/dl and normalized the blood pressure and clinical signs of Cushing's syndrome disappeared. Subsequent bilateral adrenalectomy confirmed the diagnosis of massive macronodular adrenal hyperplasia. Substitution treatment with twice daily 25 mg cortisone acetate and 0.05 mg fludrocortisone was started postoperatively.     

Formation cause,composition analysis and comprehensive utilization of rare earth solid wastes

Based on practical situation of rare earth industrial chain,production process and rare earth materials that could produce solid wastes on batch were discussed.Formation cause,formation volume,composition analysis and comprehensive utilization of the solid wastes of rare earth hydrometallurgy slag,electrolysis slag,Fe-based rare earth permanent magnetic materials,Co-based rare earth permanent magnetic materials,rare earth hydrogen storage materials,rare earth polishing powders and rare earth catalysts were ...