Hereditary Angioedema: Implications of Management.

Hereditary angioedema (HAE) is a genetic condition that is characterized by frequent episodes of localized angioedema. It is a rare disorder that a primary care provider, otolaryngologist, dermatologist, or rheumatologist may encounter only occasionally. This disease is being reviewed because of the significant advances in further understanding the genetics, biology, and therapeutic management surrounding the condition. Histamine-mediated angioedema responds to steroids, antihistamines, and epinephrine, whereas bradykinin-mediated angioedema is resistant to those interventions and requires specialized therapy. Previously used medications have significant adverse effects. Approved medications for HAE have been effective in decreasing morbidity and mortality in patients with this condition. We review the presentation, diagnosis, and available pharmaceutical options for HAE and explore the limitations of implementing recommended therapy.

The silent sinus syndrome: protean manifestations of a rare upper respiratory disorder revisited.

Silent Sinus Syndrome (SSS) is known to be a rare clinical condition, characterized by spontaneous and progressive enophthalmos and hypoglobus associated with atelectasis of the maxillary sinus and alteration of the orbital floor. Most of the patients with this syndrome present with ophthalmological complaints without any nasal sinus symptoms, and it typically has a painless course and slow development, ergo the term "silent." Here we present a case report of a patient with occasional coughing spells as the presenting symptom of Silent Sinus Syndrome, which has not been previously described in the literature. The CT scan findings suggested chronic rhinosinusitis. The radiological findings were suggestive of maxillary sinus hypoplasia, with evidence of maxillary sinus atelectasis. Awareness of this syndrome is important for specialists who work with nasal sinus disease, since its management is different than chronic rhinosinusits.

Chronic cough as the presenting symptom of hydrocephalus.

Chronic cough is defined as a daily cough lasting for more than eight weeks. We report an unusual case of chronic cough as the primary manifestation of obstructive hydrocephalus. Chronic cough in our case was determined to be of neurogenic origin only after exhaustive investigations failed to reveal a systemic cause, and, in particular, after a positive response to treatment of the hydrocephalus was observed. To the best of our knowledge, this is the first report of hydrocephalus presenting as chronic cough. We believe this case will remind physicians of the importance of considering neurological disease as a cause of chronic cough after common causes are excluded.

Seasonal distribution in newly diagnosed cases of eosinophilic esophagitis in adults.

OBJECTIVES: The etiology of eosinophilic esophagitis (EoE) is not well understood. It has been proposed that eosinophils migrate to the esophagus in response to various ingested and inhaled allergens. Recent reports in children found an increased proportion of cases of EoE during months with higher outdoor aeroallergens. To our knowledge, this has not been evaluated in adults. We aimed to determine whether there is a seasonal distribution in the number of newly diagnosed cases of EoE in an adult population. METHODS: We conducted a retrospective review of consecutive adult cases newly diagnosed with EoE in 1 year. Cases were grouped based on the index month when the diagnosis was made at endoscopy. To test the consistency of the observations, a second cohort of consecutive cases of similar sample size diagnosed at a different period in time was also analyzed. RESULTS: In total, 41 patients were diagnosed with EoE at our center during the study period, providing an annual prevalence of 0.98%. More cases were diagnosed with EoE during the months of April and May than any other month (P<0.001). When patients were grouped seasonally, there was a significant increase of EoE cases in spring and summer months (n=28) when compared with the fall and winter months (n=13) (P=0.019). Analysis of the second cohort of cases (n=37) from 2002 to 2006 confirmed a similar seasonal diagnostic pattern for EoE during the outdoor seasons. CONCLUSIONS: Our data demonstrate that EoE has a seasonal prevalence in adults. The seasonal variation pattern found in newly diagnosed EoE cases in adults supports the potential role of aeroallergens in the pathogenesis of EoE.

ACE inhibitors: upper respiratory symptoms.

Cough and angioedema are well-known adverse reactions of ACE inhibitors. However, other adverse effects of upper airways such as postnasal drainage, rhinitis and nasal blockage, are less frequently recognised. These might share the same pathophysiological mechanism: bradykinin accumulation. We present two patients with ACE inhibitor-induced upper respiratory symptoms that improved after the discontinuation of ACE-inhibitors and substitution with angiotensin II receptor blockers. The incidence of these adverse events is not accurately known, since these are not required to be reported, but it is estimated to be low. This presents challenges to the physician and demonstrates the importance of keeping it as a differential diagnosis. Most physicians are aware of ACE inhibitor-induced cough but not of ACE inhibitor-induced nasal blockage, rhinitis or postnasal drainage. Identifying these can avoid unnecessary diagnostic tests and inappropriate treatment.

Successful progressive challenge after a cutaneous reaction to imatinib mesylate (Gleevec): a case report and review of the literature.

The use of imatinib mesylate (Gleevec), a selective tyrosine kinase inhibitor, has become the new gold standard for the treatment of chronic myeloid leukemia. Unfortunately, the medication has been commonly associated with mild to severe cutaneous reactions, which has limited its use for some patients. We describe a case of a successful progressive challenge of a patient with a drug-induced exanthem from imatinib mesylate and a review of the literature.

Diagnosis and treatment of postprandial nausea, allergy, and eosinophilia.

We report a case of a 16-year-old male patient who presented with postprandial fullness and nausea. He had a history of seasonal allergies, asthma, and peripheral eosinophilia. Endoscopy of the stomach with mucosal biopsies revealed predominate eosinophils. A diagnosis of eosinophilic gastroenteritis was made. The patient's disease course and management is described in this article.