Staged surgical repair of functional single ventricle in infants with unobstructed pulmonary blood flow

Objective: The infant with a functional single ventricle (SV) and unobstructed pulmonary blood flow (UPBF) requires early protection of the pulmonary vascular bed to ensure suitability for a subsequent Fontan procedure. Systemic obstruction by aortic arch obstruction, subaortic stenosis, or combination of both, has been widely recognized as an important risk factor for poor outcome in children with SV–UPBF who are palliated with pulmonary artery banding (PAB). We reviewed our experience with primary PAB in the subset of patients with SV–UPBF to identify risk factors for subsequent palliative procedures and Fontan completion. Methods: Between January 1990 and May 2004, 80 patients (median age, 14 days) with functional SV and UPBF underwent PAB as their primary palliative procedure. Thirty-five neonates had concomitant aortic coarctation or interrupted aortic arch repair (44%). A Damus–Kaye–Stansel procedure was subsequently performed in 19 patients, and subaortic resection or ventricular septal defect or bulboventricular foramen enlargement was performed in five. Results: There were 4 operative deaths, and 15 late deaths. The actuarial overall survival is 84% at 1 year, 76% at 5 and 15 years. Follow-up is complete in all but six children at a mean interval of 4.9±3.7 years (range, 2 months–15 years). Thirty-seven patients (49%; 37 of 76) have undergone the hemi-Fontan procedure (with three hospital deaths) and 40 patients (53%; 40 of 76; 12 children without previous hemi-Fontan) have undergone the completion Fontan procedure without mortality or Fontan takedown. Conclusion: In infants with single ventricle physiology with or without systemic outflow obstruction and unobstructed pulmonary blood flow, a strategy of pulmonary artery banding carries acceptable operative and mid-term mortality in a high-risk group of patients. Pulmonary artery banding does not compromise performance of subsequent Damus–Kaye–Stansel procedure or completion Fontan palliation.     

Case report: Concordant traumatic brainstem contusion delayed diagnosis in a young man with Wilson's disease

Wilson's disease is a hereditary autosomal recessive disorder of copper metabolism. The corresponding gene locus has been localized on the long arm of chromosome 13. Three different clinical variants of the disease can be distinguished: hepato-cerebral, abdominal/hepatic, and central nervous type. The heterogeneity of symptoms can cause problems in differential diagnosis, especially when another concordant disorder can also explain the pathogenesis of symptoms. The case report of a young man who suffered from brainstem contusion demonstrates the possibilities of misinterpretation because presenting symptoms could be attributed either to traumatic brain injury followed by adjustment disorder or Wilson's disease. Clinical signs included leftsided hemiparesis, bilateral gaze direction nystagmus, marked dysarthria with consecutive pervasive mutism, choreo-athetoid movements, spasmodic torticollis and diplopia dependent on gaze direction. Slit lamp examination showed Kayser-Fleischer's corneal ring. EEG- and computer assisted tomography investigations revealed non-specific findings. The patient was treated with D-Penicillamine. Alternative treatment with oral zinc preparations is discussed.

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Zusammenfassung Beim Morbus Wilson handelt es sich um eine autosomal rezessiv vererbte Störung des Kupferstoffwechsels. Der Genort konnte auf dem langen Arm des Chromosoms 13 lokalisiert werden. Klinisch können aufgrund ihrer Symptomatik drei Verlaufsformen (hepato-zerebraler, abdominalhepatischer und zerebraler Typ) unterschieden werden. Die Vielfalt der Symptome kann differentialdiagnostische Schwierigkeiten bereiten. Das Beispiel eines jungen Mannes mit einer traumatischen Hirnstammkontusion zeigt, wie die Diagnose der hepato-lentikulären Erkrankung dadurch erschwert wurde, daß die Pathogenese der Symptome durch die Hirnstammkontusion und darauf folgende Anpassungsstörungen erklärt worden war. Die Symptomatik bestand aus linksseitiger Hemiparese, lateralem Blickrichtungsnystagmus, Dysarthrie mit nachfolgendem universalem Mutismus, choreo-athetodischen Bewegungsstörungen, Torticollis spasmoidicus und blickrichtungsabhängigem Auftreten von Doppelbildern. Bei der Spaltlampenuntersuchung stellte sich der Kayser-Fleischer Ring dar. EEG- und computertomographische Untersuchungen erbrachten nur unspezifische Befunde. Die Behandlung erfolgte mit D-Penicillamin. Die alternative Behandlung mit oraler Gabe von Zinksalzen wird diskutiert.

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Résumé La maladie de Wilson est une affection héréditaire autosomale recessive concernant le métabolisme cuivré. Le locus du gène a été situé sur le bras long du chromosome 13. Du point de vue clinique on distingue trois formes symptomatologiques: le type hepato-cérébral, hepato-abdominal et cérébral. La diversité des signes cliniques peut poser des problèmes de diagnostic différentiel, car d'autres affections peuvent se présenter avec cette même Symptomatologie. Nous rapportons ici l'exemple d'un homme jeune, porteur d'une maladie de Wilson et victime d'une contusion traumatique du tronc cérébral, dont les signes cliniques ainsi que les troubles du comportement pouvaient été autant rapportés à la contusion du tronc cérébral qu'à l'affection métabolique.La Symptomatologie comprenait une hemiparesie gauche, un nystagmus lateralisé, une dysarthrie avec mutisme secondaire universel, des mouvements choréo-athétosiques, un torticolis spasmodique et une diplopie dépendante de la direction du regard. L'examen à la lampe à fente permettait à mettre en evidence un anneau de Kayser Fleischer. L'EEG et le scanner cérébral ne montraient pas d'anomalies specifique.Le traitement a consisté en l'administration de D-Penicillamine. Traitment alternative avec les sels de zinc est discuté.

     

Bündelhaar-Follikulitis

Zusammenfassung Bei der Bündelhaar-Follikulitis (tufted hair folliculitis) handelt es sich um eine durch Staphylococcus aureus verursachte, chronisch entzündliche und vernarbende Haarbodenerkrankung, die charakterisiert ist durch das umschriebene Auftreten von Bündelhaaren. Histopathologisch findet sich eine perifollikul?re Entzündung im oberen Bereich der Haarfollikel unter Aussparung der tiefen Follikelanteile. Innerhalb der Entzündungsareale münden mehrere Haare aus getrennten Haarwurzeln in ein gemeinsames Infundibulum mit dilatiertem Ostium. Das Krankheitsbild ist als eine Erscheinungsform der Folliculitis decalvans capillitii Quinquaud anzusehen, bei der die Auffassung besteht, da? die Infektion mit Staphylococcus aureus den pathogenetischen Initialfaktor darstellt, und Besonderheiten der Follikelanatomie und Immunantwort die individuelle Morphologie bestimmen: Dabei ist anzunehmen, da? die Ausbildung fl?chig atropher Narben mit vollst?ndigem Untergang der Hautanhangsgebilde (im Falle der Folliculitis decalvans) oder das Auftreten von Haarbündeln (im Falle der Bündelhaar-Follikulitis) von der Tiefe und dem Destruktions-Potential des Entzündungsinfiltrates abh?ngen. Die Behandlung der Bündelhaar-Follikulitis ist nicht ganz unproblematisch: Die prolongierte und wiederholte Gabe staphylokokken-wiksamer Antibiotika vermag den Krankheitsverlauf zu stabilisieren. Bereits vorhandene Haarbündel mit einer besonders hohen Rezidivneigung sollten aber – wie im vorgestellten Fall – nach M?glichkeit chirurgisch exzidiert werden.

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Summary A case of tufted hair folliculutis presenting as circumscribed, tender and inflamed areas in the occiput with residual tufted follicles in a 28-year old man is reported. Tufted hair folliculitis is a characteristic localized scarring bacterial folliculitis of the scalp due to Staphylococcus aureus. Histopathological studies reveal perifollicular inflammation around the upper portions of the follicles sparing the hair root level. Within areas of inflammation, several follicles converge toward a common follicular duct with a widely dilated opening. Currently, tufted hair folliculitis is considered a variant of folliculitis decalvans of Quinquaud. Staphylococcal infection is believed to be an initial causative factor, and underlying differences in follicular anatomy or host response may be important in determining which reaction pattern occurs in an affected individual. The development of atrophy with loss of adnexal structures (in folliculitis decalvans) or of hair tufts (in tufting folliculitis) may depend upon the depth and destructive potential of the inflammatory process. The therapeutic approach is problematic; prolonged treatment with oral antibiotics may stabilize the disease, but good and at times more definitive results (as in the presented case) have been reported after radical surgical excision of the involved areas.

Eingegangen am 2. April 1996 Angenommen am 7. Juni 1996     

Perception of maternal behavior by elementary school children of alcoholic mothers

Elementary-school-aged children of alcoholic mothers more often perceive their mothers to be using guilt as a means of psychological control and using lax disciplinary methods than similar children with nonalcoholic mothers. This outcome is discussed as a unique profile resulting from denial in the alcoholic family and the possible projection of guilt felt by the alcoholic mother. Implications for prevention programming and further areas for empirical investigation are suggested.     

Population-based randomized controlled trial of a stage-targeted physical activity intervention

Background: Intervention trials with self-selected participants have shown that mailed stage-targeted print materials can increase participation in physical activity in the short term. We examined the effects of a mailed stage-targeted print intervention designed to promote physical activity, in a random sample of adults living in a regional city.Method: Participants (n = 462, 40–60 years of age) were randomly allocated to an intervention in - 227) or control group (n - 235). Measures included validated 2-week physical activity recall and stage of motivational readiness for physical activity. The intervention consisted of a single mailing of a letter and full-color stage-targeted booklets (specific to precontemplation, contemplation, preparation, and action/maintenance) 1 week postbaseline. Follow-up interviews were conducted at 2 and 6 months postbaseline.Results: After 2 months, participants in the intervention group were significantly More likely to meet the current American College of Sports Medicine/Centers for Disease Control and Prevention recommendation for sufficient physical activity than those in the control group (adjusted odds ratio [OR] - 2.40; 95% confidence interval [CI] = 1.44–3.99). After 6 months, intervention participants who reported receiving and reading the intervention materials were significantly more likely to be meeting the sufficient physical activity criterion compared with the control group (adjusted OR = 2.03; 95% Cl = 1.16–3.56).Conclusions: The stage-targeted print intervention was effective in promoting short-term increases in physical activity and was most effective for participants who recognized and used the materials. This low-cost, generalizable intervention has demonstrated potential as a practical population-based physical activity promotion strategy. Further research is required before widespread dissemination would be justified, as additional strategies may be required to ensure sustained change. This project was supported by a National Heart Foundation of Australia Research Project Grant. David Crawford was supported by a Nutrition Research fellowship from the National Heart Foundation.     

Unusual presentation of common acute lymphoblastic leukemia antigen-positive extramedullary disease in childhood. Two patients with isolated masseter muscle involvement.

Two children with unusual extramedullary common acute lymphoblastic leukemia antigen (CALLA)-positive (CD10) disease are reported. Isolated masseter infiltration with CD10/CD19-positive lymphoblasts was present in both patients with no other evidence of disease. One child had relapse of common acute lymphoblastic leukemia, and the other had primary disease. Disease may have spread from lymph nodes overlying the masseter muscle. Immunophenotyping and immunogenotyping provided a rapid and accurate diagnosis for both children.     

Is spironolactone safe for dialysis patients?

BACKGROUND: Spironolactone is useful in heart failure, but is not given to dialysis patients for fear of hyperkalaemia. This study evaluated the safety of spironolactone administration in haemodialysis patients. METHODS: Fifteen haemodialysis outpatients with mean serum potassium <5.6 mEq/l over the preceding 4 months were treated with spironolactone 25 mg daily for 28 days. Serum potassium was measured before every haemodialysis during the study. Aldosterone and renin were measured at the beginning and end of the study. Patients were monitored for side effects. Data were examined with a paired t-test, with patients serving as their own controls and P < 0.05 considered significant. A sample size of 14 was required to achieve a power of 0.8 and a P = 0.05 to detect a potassium difference of 0.5 +/- 0.6 mEq/l. All patients were analysed as intention-to-treat. RESULTS: The mean potassium level was 4.6 +/- 0.6 mEq/l at baseline and 4.9 +/- 0.9 mEq/l at study completion (P = 0.14). Thirteen patients completed the trial with no potassium levels >6.0 mEq/l. Four patients had potassium levels between 5.5 and 6.0 mEq/l. One patient was withdrawn at day 20 after developing hyperkalaemia (7.6 mEq/l). Another patient was withdrawn at day 25 after missing a dialysis treatment. There were no differences in either baseline or 28 day aldosterone or renin levels (16.8 +/- 28.8 vs 11.7 +/- 6.1 ng/dl and 3.5 +/- 3.9 vs 3.5 +/- 3.5 ng/ml/h, respectively). Infrequent side effects included dry mouth, nosebleed, pruritis, gynecomastia and diarrhoea. No significant leukopenia or anaemia was noted. CONCLUSIONS: Spironolactone may be considered as a treatment option for selected chronic haemodialysis patients with heart disease.