Extremely Delayed Diagnosis of Type II Hereditary Angioedema: Case Report and Review of the Literature.

We present a case with extremely late diagnosis of type II hereditary angioedema (HAE). Given recent advances in HAE treatment, we want to bring physician awareness to this condition and aid in earlier detection. HAE is a disorder associated with episodes of angioedema of the face, larynx, lips, abdomen, or extremities. Late diagnosis of HAE can lead to significant morbidity and is severely impairing due to recurring attacks. The diagnosis of HAE is ordinarily made during childhood and adolescence. Delayed diagnoses in early and middle adulthood have been documented in the literature. Gastrointestinal symptoms are common features of HAE and can be misdiagnosed as disease of primary gastrointestinal pathology, such as irritable bowel syndrome, recurrent pancreatitis, or appendicitis. These attacks are characterized by recurrent attacks of subcutaneous and submucosal edema without the presence of urticaria.We present a case of an elderly veteran whose diagnoses was extremely delayed into the eighth decade of life subsequent to unexplained abdominal symptoms. After diagnosis, the patient's symptoms were well controlled with medication due to advances in HAE treatment. To prevent further atypically delayed diagnoses, physicians should consider HAE in patients with recurrent attacks of unexplained abdominal pain.

Treatment of primary and secondary hypertension in children.

The incidence of pediatric hypertension (HTN) is increasing, mainly due to an increase in primary (essential) HTN, or PH. There are only a limited number of studies assessing the characteristics and treatment efficacy of PH versus secondary HTN (SH). We conducted a retrospective analysis of 158 pediatric patients (mean age: 10.8 years; sex ratio: 51.1% female, 48.9% male) with HTN of whom 34.4% had PH and 65.6% had SH. The vast majority were either African-American or Caucasian. Among all patients, therapy induced a significant decrease in systolic blood pressure (SBP) and diastolic BP (DBP) (both p<0.0001). SBP (p<0.0001) and DBP (p=0.002) declined significantly in PH patients. PH and SH patients with a body mass index (BMI) >95th percentile (%) had a significantly higher post-therapy SBP (both p<0.05) than those with a BMI <95th%. SBP declined similarly in PH patients treated with calcium-channel blockers (CCB) and angiotensin-converting enzyme inhibitors (ACEI). DBP declined only in PH patients treated with ACEI. SBP and DBP (both p<0.0001) declined significantly in SH patients. Post-therapy BP was similar in SH patients treated with either CCB or ACEI. Post-therapy SBP and DBP were significantly lower in SH patients than in PH patients; moreover, therapy induced a greater decline in SBP and DBP in the SH patients. Compared to PH patients, SH patients were twofold more likely to achieve a SBP less than the 95th% after therapy. We conclude that (1) significant lowering of BP with either CCB or ACEI is achievable in most children with HTN, and (2) SH patients respond better to therapy than those with PH.