Under-recognition of Low Blood Pressure Readings in Patients with Duchenne Muscular Dystrophy.

Duchenne muscular dystrophy (DMD), a recessive sex-linked hereditary disorder, is characterized by degeneration, atrophy, and weakness of skeletal and cardiac muscle. The purpose of this study was to document the prevalence of abnormally low resting BP recordings in patients with DMD in our outpatient clinic. The charts of 31 patients with DMD attending the cardiology clinic at Rush University Medical Center were retrospectively reviewed. Demographic data, systolic, diastolic, and mean blood pressures along with current medications, echocardiograms, and documented clinical appreciation and management of low blood pressure were recorded in the form of 104 outpatient clinical visits. Blood pressure (BP) was classified as low if the systolic and/or mean BP was less than the fifth percentile for height for patients aged ≤17 years (n = 23). For patients ≥18 years (n = 8), systolic blood pressure (SBP) <90 mmHg or a mean arterial pressure (MAP) <60 mmHg was recorded as a low reading. Patients with other forms of myopathy or unclear diagnosis were excluded. Statistical analysis was done using PASW version 18. BP was documented at 103 (99.01 %) outpatient encounters. Low systolic and mean BP were recorded in 35 (33.7 %) encounters. This represented low recordings for 19 (61.3 %) out of a total 31 patients with two or more successive low recordings for 12 (38.7 %) patients. Thirty-one low BP encounters were in patients <18 years old. Hispanic patients accounted for 74 (71.2 %) visits and had low BP recorded in 32 (43.2 %) instances. The patients were non-ambulant in 71 (68.3 %) encounters. Out of 35 encounters with low BP, 17 patients (48.6 %) were taking heart failure medication. In instances when patients had low BP, 22 (66.7 %) out of 33 echocardiography encounters had normal left ventricular ejection fraction. Clinician comments on low BP reading were present in 11 (10.6 %) encounters, and treatment modification occurred in only 1 (1 %) patient. Age in years (p = .031) and ethnicity (p = .035) were independent predictors of low BP using stepwise multiple regression analysis. Low BP was recorded in a significant number of patient encounters in patients with DMD. Age 17 years or less and Hispanic ethnicity were significant predictors associated with low BP readings in our DMD cohort. Concomitant heart failure therapy was not a statistically significant association. There is a need for enhanced awareness of low BP in DMD patients among primary care and specialty physicians. The etiology and clinical impact of these findings are unclear but may impact escalation of heart failure therapy.

The use of intracardiac echocardiography during percutaneous pulmonary valve replacement.

High-quality live imaging assessment of cardiac valves and cardiac anatomy is crucial for the success of catheter-based procedures. We present our experience using Intracardiac echocardiography (ICE) during transcatheter Percutaneous Pulmonary Valve replacement (tPVR).This is a retrospective study that included 35 patients who underwent tPVR between April 2008 and June 2012. Thirty-one of these patients had the procedure performed under continuous ICE guidance. Pre-procedure transthoracic echocardiography (TTE) was obtained in all patients. ICE was performed at baseline, during the procedure, and at the conclusion of the procedure. Comparisons between the pre-procedure TTE and baseline ICE data and between post-procedure ICE data and the following day TTE were performed. Total of 35 patients had tPVR during the above-mentioned time period. Twenty-one patients received the Edwards Sapien valve and 14 patients had the Melody valve. Thirty-one patients had the procedure performed under continuous ICE guidance. The mean Pre-TTE peak gradient (PG) and Pre-ICE-PG were 45.5 ± 20 vs 33 ± 13 mmHg (p < 0.001) and the mean Pre-TTE mean gradient (MG) and Pre-ICE-MG were 27.7 ± 13 vs 21 ± 18 mmHg (p < 0.001). The mean Post-TTE- PG and Post-ICE-PG were 24.3 ± 11 vs 15.3 ± 7 mmHg (p < 0.001) and the mean of the Post-TTE-MG and Post-ICE-MG were 14.2 ± 7 vs 8.4 ± 4 mmHg (p < 0.001). There was a good correlation between peak ICE and TTE gradient at baseline and after valve placement. For the degree of pulmonary regurgitation, there was no significant difference between TTE and ICE. ICE is an important modality to guide tPVR in patients with dysfunctional homograft valve between the right ventricle and pulmonary artery and should be used to assess valve function before, during and immediately after the procedure.

Post-transfusion hypertension, convulsion and intracranial haemorrhage in ß-thalassemia major.

The haematologic disorder ß-thalassemia major is common in Pakistan. We describe a patient with undiagnosed thalassemia presenting with hypertension and convulsions and found to have cerebral haemorrhage on neuro-imaging. He had been transfused 2 weeks before this illness. Our experience is similar to a few case reports described in literature that were found to have cerebral haemorrhages post-mortem after a similar clinical presentation. All patients had a blood transfusion within 2 weeks prior to the presentation so association with transfusion has been proposed. We have reviewed the several mechanisms presented and discussed the findings.

Cardiac tamponade and pleural effusion complicating varicella: a case report.

Varicella is a common infectious disease, usually benign and self-limited, and complications are believed to be rare. Most cases occur in healthy children younger than 14 years. Serious complications are uncommon, although high-risk groups have been identified, such as immunosuppressed patients, neonates, and adults. Cardiac tamponade after pericardial effusion occurring as a complication of varicella infection has been very infrequently reported. We describe an 8-month-old infant presenting with cardiac tamponade after varicella infection secondary to purulent bacterial pericardial effusion. He required a pericardial window formation. He also developed pleural empyema, another uncommon complication requiring thoracostomy drainage. This illustrative case, management, and unique features will be presented along with a review of all cases of varicella complicated by pericarditis in the English literature.

Acute transverse myelitis in children.

A case of Acute Transverse Myelitis (ATM) is presented. ATM is a rare disease in childhood. The diagnosis stems from the clinical presentation, cerebrospinal fluid findings, appearance of the spinal cord on imaging and ruling out differential diagnoses like Guillain-Barre syndrome (GBS) and Poliomyelitis. The proposed treatment is intravenous (IV) methyl prednisolone with variable chances of recovery. A controlled multicenter study is suggested to assess epidemiology, etiology and prognosis of ATM.

Chest radiography in children aged 2-59 months diagnosed with non-severe pneumonia as defined by World Health Organization: descriptive multicentre study in Pakistan.

OBJECTIVES: To evaluate the chest radiographs of children diagnosed with non-severe pneumonia on the basis of the current World Health Organization guidelines (fast breathing alone) for radiological evidence of pneumonia. DESIGN: Descriptive analysis. SETTING: Outpatient departments of six hospitals in four cities in Pakistan. PARTICIPANTS: 2000 children with non-severe pneumonia were enrolled; 1932 children were selected for chest radiography. INTERVENTIONS: Two consultant radiologists used standardised WHO definitions to evaluate chest radiographs; no clinical information was made available to them. If they disagreed, the radiographs were read by a third radiologist; the final classification was based on agreement between two of the three radiologists. MAIN OUTCOME MEASURES: Presence or absence of pneumonia on radiographs. RESULTS: Chest radiographs were reported normal in 1519 children (82%). Radiological evidence of pneumonia was reported in only 263 (14%) children, most of whom had interstitial pneumonitis. Lobar consolidation was present in only 26 children. The duration of illness did not correlate significantly with the presence of radiological changes (relative risk 1.17, 95% confidence interval 0.91 to 1.49). CONCLUSION: Most children diagnosed with non-severe pneumonia on the basis of the current WHO definition had normal chest radiographs.