Right heart pressure determination by Doppler in infants with tricuspid regurgitation

Doppler and direct measurements of right ventricle to right atrial pressure drop were made during cardiac catheterisation on 28 occasions in 26 infants with congenital heart disease. Age was 10 days to 12 months (median 4.5 months), and weight was 3.1 to 9.0 kg (median 4.7 kg). We measured peak velocity of tricuspid regurgitation by continuous wave Doppler, and the pressure drop was calculated using the modified Bernoulli equation (delta p = 4v2). There was a high correlation (r = 0.95) between direct and Doppler measurements. Doppler values tended to underestimate the right ventricle to right atrial pressure drop, but this was not of clinical significance (mean 2 mm Hg). The 95% confidence interval for the Doppler velocity was -0.41 to +0.26 m/sec, and was consistent across the range of pressures studied. Variability between observers was tested, by two observers performing sequential paired examinations on 16 newborn babies with tricuspid regurgitation. The coefficient of repeatability was 6.3 mm Hg (95% confidence interval 4.7 to 9.5 mm Hg) or 0.26 m/sec (0.18 to 0.50 m/sec). This method of right ventricular pressure estimation, validated previously only in older children and adults, is a reproducible and accurate technique in infants with tricuspid regurgitation.     

Failure of ablation of a mediastinal parathyroid adenoma by repeated contrast staining

In a patient with primary hyperparathyroidism an attempt was made to ablate a middle mediastinal parathyroid gland by forceful staining with radiographic contrast material. The gland was stained on two separate occasions, two weeks apart. Both times the serum calcium level temporarily fell to the normal range but reverted to abnormal levels. The patient ultimately required surgery for correction of hypercalcemia. The mechanism of staining and possible reasons for failure as well as potential complications are discussed.     

The relationship between frontal sinusitis and localization of the frontal sinus outflow tract: a computer-assisted anatomical and clinical study

OBJECTIVE: To evaluate the relationship between frontal sinusitis and the localization of the frontal sinus outflow tract medial or lateral to the superior attachment of the uncinate process (UP). DESIGN: A retrospective anatomical and clinical study. SETTING: An ear, nose, and throat clinic in i?li Etfal Teaching and Research Hospital, Istanbul, Turkey. PATIENTS: Paranasal sinus computed tomographic scans of 486 sides of the frontal sinuses (hereafter referred to as sides) of 243 patients who had chronic sinusitis were evaluated. In 125 sides (26%), the superior attachment of the UP could not be identified. In the remaining 361 sides (74%), the prevalence of superior attachment of UP types and the presence of frontal sinusitis in each side were recorded. Localization of the frontal sinus outflow tract was determined according to the superior attachment of the UP. Drainage of the frontal sinus to the middle meatus (medial to the superior attachment of the UP [types 1-3]) was classified as group 1, and drainage of the frontal sinus to the ethmoid infundibulum (lateral to the superior attachment of the UP [types 4-6]) was classified as group 2. RESULTS: Frontal sinusitis was found in 125 (35%) of 361 sides. The distribution of frontal sinusitis was 97 (41%) of 237 in group 1 and 28 (23%) of 124 in group 2. Group 1 drainage had a statistically significant presence of frontal sinusitis (chi(2) = 12.11; P<.001). The prevalence of superior attachment of UP types was 63% for type 1/2, 3% for type 3, 12% for type 4, 14% for type 5, and 8% for type 6. CONCLUSIONS: Frontal sinus outflow tract, which is medial to the superior attachment of the UP, is more common than the lateral one. There is a statistically significant relation between the presence of frontal sinusitis and the frontal sinus outflow tract, which is medial to the superior attachment of the UP.     

Intracranial abscesses associated with chronic suppurative otitis media

Intracranial abscesses are serious complications of chronic suppurative otitis media (COM). This study included 32 patients presenting with intracranial abscesses from 780 patients hospitalized for treatment of COM. The 32 patients had 59 intracranial complications. Perisinus abscess (13 of 32) was the most common intracranial abscess, followed by temporal lobe abscess (8 of 32), epidural abscess (7 of 32), cerebellar abscess (6 of 32) and subdural empyema (2 of 32). Headache (93%), fever (87%) and altered mental status (62%) were the most common presenting symptoms and signs, along with symptoms of COM. All patients were treated with intravenous antibiotics and canal wall down mastoidectomy. Cholesteatoma with granulation tissue and bony defects at the sinus plate and/or dural plate were seen in most of the patients. Gram negative bacilli and anaerobes were the most common organisms cultured from the abscesses. Three patients had neurological sequels. One patient died. The early diagnosis of these complications requires a high index of suspicion and imaging studies. A multidisciplinary and coordinated approach is important for the management of these patients.The authors did not receive any financial support for the present study.     

The use of transdermal fentanyl in chronic non-malignant pain

Twelve non-malignant pain patients were followed-up for pain, improvement in the quality of life, satisfaction for pain therapy and side effects for a mean of 95.25 days with the use of transdermal fentanyl (TDF). During this period a mean of 32.04 mg/gr TDF was used. While the score of pain at rest was decreased by 52.6%, mean pain score on movement was decreased by 45.2% (p= 0.002). Quality of sleep improved and impairment of daily living by pain was decreased significantly (p= 0.002). Satisfaction by the pain therapy was 83.3% and the most common side effect was nausea (16.8%). TDF may be a good alternative in the therapy of chronic non-malignant pain if patients were selected carefully.     

Frequencies of inherited organic acidurias and disorders of mitochondrial fatty acid transport and oxidation in Germany

The lack of epidemiological data on the frequency and/or burden of organic acidurias (OA) and mitochondrial fatty acid transport and oxidation disorders (mtFATOD) is one reason for hesitation to expand newborn screening (NBS) by tandem mass spectrometry (MS-MS). From 1999 to 2000, the frequency of ten potentially treatable OA and mtFATOD was assessed by active nation-wide surveillance on cases presenting with clinical symptoms using the German Paediatric Surveillance Unit (ESPED) system. Case ascertainment was complemented by a second independent source: 3-monthly inquiries in the metabolic laboratories performing secondary selected screening for OA and mtFATOD. Frequency estimates for clinically symptomatic cases older than 7 days in a birth cohort of 844,575 conventionally screened children was compared to the frequency found in a cohort of 382,247 screened by MS-MS in Bavaria and Baden-Württemberg. The overall frequency of the ten conditions considered was 1:8,000 (95% CI 1:11,000–1:6,000) by MS-MS as compared to 1:23,000 (95% CI 1:36,000–1:17,000) in symptomatic cases presenting mainly with metabolic crisis. The contributions of medium-chain acyl-CoA dehydrogenase deficiency (MCADD), other mtFATOD and OA were 29, 4 and 13 among the 46 cases identified by MS-MS, and 19, 1 and 13 among the 33 clinically symptomatic cases, respectively. Acute metabolic crisis, with a lethal outcome in four patients, was reported for 22/33 clinically symptomatic cases. No clinically symptomatic cases were reported from cohorts with screened by MS-MS. Conclusion: ten potentially treatable organic acidurias and mitochondrial fatty acid transport and oxidations disorders were more common than phenylketonuria with organic acidurias accounting for 28% of the cases detected by newborn screening and 39% of the cases identified on high risk screening. These conditions were related to considerable morbidity and mortality. Considerations for their inclusion in expanded newborn screening programmes might be warranted.Abbreviations ESPED Erhebungseinheit für seltene pädiatrische Erkrankungen in Deutschland (German Paediatric Surveillance Unit) - MCADD medium-chain acyl-CoA dehydrogenase deficiency - MS-MS tandem mass spectrometry - mtFATOD mitochondrial fatty acid transport and oxidation disorders - NBS newborn screening - OA organic acidurias     

Seizures in the developing brain cause adverse long-term effects on spatial learning and anxiety

PURPOSE: Seizures in the developing brain cause less macroscopic structural damage than do seizures in adulthood, but accumulating evidence shows that seizures early in life can be associated with persistent behavioral and cognitive impairments. We previously showed that long-term spatial memory in the eight-arm radial-arm maze was impaired in rats that experienced a single episode of kainic acid (KA)-induced status epilepticus during early development (postnatal days (P) 1-14). Here we extend those findings by using a set of behavioral paradigms that are sensitive to additional aspects of learning and behavior. METHODS: On P1, P7, P14, or P24, rats underwent status epilepticus induced by intraperitoneal injections of age-specific doses of KA. In adulthood (P90-P100), the behavioral performance of these rats was compared with that of control rats that did not receive KA. A modified version of the radial-arm maze was used to assess short-term spatial memory; the Morris water maze was used to evaluate long-term spatial memory and retrieval; and the elevated plus maze was used to determine anxiety. RESULTS: Compared with controls, rats with KA seizures at each tested age had impaired short-term spatial memory in the radial-arm maze (longer latency to criterion and more reference errors), deficient long-term spatial learning and retrieval in the water maze (longer escape latencies and memory for platform location), and a greater degree of anxiety in the elevated plus maze (greater time spent in open arms). CONCLUSIONS: These findings provide additional support for the concept that seizures early in life may be followed by life-long impairment of certain cognitive and behavioral functions. These results may have clinical implications, favoring early and aggressive control of seizures during development.