Acute and Chronic Kernicterus: MR Imaging Evolution of Globus Pallidus Signal Change during Childhood.

BACKGROUND AND PURPOSE: Despite its rarity in Western countries, kernicterus resulting from severe neonatal hyperbilirubinemia and its associated neurologic consequences still persists. Subtle MR imaging patterns may be overlooked, leading to diagnostic and prognostic uncertainties. The study systematically analyzes MR imaging pattern over time. MATERIALS AND METHODS: A retrospective MR imaging study was conducted in Departments of Pediatric Neurology at the University Children's Hospitals in Leipzig, Germany, or Tübingen, Germany, between 2012 and 2022 in patients who presented beyond the neonatal period suspected of having chronic kernicterus. RESULTS: Eight patients with a total of 15 MR images were identified. The clinical diagnosis of kernicterus was confirmed in all cases on the basis of typical MR imaging findings: Bilateral, diffuse hyperintensity of the globus pallidus was observed in the neonatal period on T1WI (1 MR imaging, at 2 weeks), in infancy on T2WI (4 MR images, at 9-26 months). In children 2 years of age and older, bilateral hyperintensity on T2WI was limited to the borders of the globus pallidus (8 MR images, at 20 months -13 years). Notably, 2 children exhibited normal initial MR imaging findings at 2 months of age. Hence, MR imaging depiction of kernicterus pathology evolves with time, first evident on T1WI, subsequently on T2WI, with a "blind window" during early infancy. The T2WI signal change initially involves the entire globus pallidus and later is limited to the borders. Kernicterus had not been diagnosed in any except 2 patients by previous investigators. CONCLUSIONS: All patients presented with a characteristic clinical history and signs and an evolving MR imaging pattern. Nonetheless, the diagnosis of kernicterus was frequently missed. Abnormalities on later MR images appear to be underrecognized.

Monitoring selective etching of self-assembled nanostructured a-Si:Al films.

Nanoporous and nanowire structures based on silicon (Si) have a well recognized potential in a number of applications such as photovoltaics, energy storage and thermoelectricity. The immiscibility of Si and aluminum (Al) may be utilized to produce a thin film of vertically aligned Al nanowires of 5 nm diameter within an amorphous silicon matrix (a-Si), providing a cheap and scalable fabrication method for sub 5 nm size Si nanostructures. In this work we study functionalization of these structures by removal of the Al nanowires. The nanowires have been etched by an aqueous solution of HCl, which results in a structure of vertically aligned nanochannels in a-Si with admixture of SiO x . The removal of Al nanowires has been monitored by several electron microscopy techniques, x-ray diffraction, Rutherford backscattering spectroscopy, and optical reflectance. We have established that optical reflectance measurements can reliably identify the complete removal of Al, confirmed by other techniques. This provides a robust and relatively simple method for controlling the nano-fabrication process on a macroscopic scale.

Fulminant hepatic failure due to chemotherapy-induced hepatitis B reactivation: role of rituximab.

Hepatitis B virus reactivation during immunosuppressive therapies can lead to liver failure with very limited treatment options available. We report here on two cases of severe hepatitis B reactivation during chemotherapy including rituximab for B cell lymphoma which were treated with liver or liver-cell transplantation. Liver function was normal and HBV infection was unknown in both patients before chemotherapy was started. Impaired liver function became apparent after 4 and 6 courses of chemotherapy, respectively, and both patients experienced fulminant hepatic failure despite antiviral treatment with lamivudine or entecavir. Patient A underwent liver transplantation after documentation of complete remission of the lymphoma and survived without any evidence for hepatitis B recurrence. Patient B received 4 courses of hepatocyte transplantation but did not survive. These cases underline the importance of anti-HBc screening in patients receiving immunosuppressive treatments in particular when rituximab is given. Pre-emptive antiviral treatments should be administered since delayed antiviral treatment is frequently unable to prevent liver failure.

Applications of combined EXAFS and powder diffraction analysis in solid state chemistry.

Recent applications of combined EXAFS/powder neutron and X-diffraction analysis are reviewed and provisional results for three additional compounds are presented. Criteria for successful refinements are suggested. The new results relate to the materials: CoAl2O4, La(6.4)Ca(1.6)Cu6Ni2O20 and Pr(0.5)Sr(0.5)FeO(2.75).

Two domains in dihydropyridine receptor activate the skeletal muscle Ca(2+) release channel.

The II-III cytoplasmic loop of the skeletal muscle dihydropyridine receptor (DHPR) alpha(1)-subunit is essential for skeletal-type excitation-contraction coupling. Single channel and [(3)H]ryanodine binding studies with a full-length recombinant peptide (p(666-791)) confirmed that this region specifically activates skeletal muscle Ca2+ release channels (CRCs). However, attempts to identify shorter domains of the II-III loop specific for skeletal CRC activation have yielded contradictory results. We assessed the specificity of the interaction of five truncated II-III loop peptides by comparing their effects on skeletal and cardiac CRCs in lipid bilayer experiments; p(671-680) and p(720-765) specifically activated the submaximally Ca2+-activated skeletal CRC in experiments using both mono and divalent ions as current carriers. A third peptide, p(671-690), showed a bimodal activation/inactivation behavior indicating a high-affinity activating and low-affinity inactivating binding site. Two other peptides (p(681-690) and p(681-685)) that contained an RKRRK-motif and have previously been suggested in in vitro studies to be important for skeletal-type E-C coupling, failed to specifically stimulate skeletal CRCs. Noteworthy, p(671-690), p(681-690), and p(681-685) induced similar subconductances and long-lasting channel closings in skeletal and cardiac CRCs, indicating that these peptides interact in an isoform-independent manner with the CRCs.

[Intraocular metastases of metastatic breast carcinoma in the woman. Incidence, risk factors and therapy]. / Intraokulare Metastasen bei metastasiertem Mammakarzinom der Frau. Inzidenz, Risikofaktoren und Therapie.

BACKGROUND: The breast cancer metastasis is the most common intraocular tumor in females. Aim of this study was to determine incidence and risk factors for intraocular metastasis and to evaluate the benefit of an early treatment. PATIENTS AND METHODS: 151 patients suffering from metastatic breast cancer were screened for the presence of intraocular metastasis. The medical history and the tumor status at the time of screening were evaluated and risk factors for intraocular metastasis were determined. In case of choroidal metastasis external beam radiotherapy was performed. RESULTS: Intraocular metastasis was found in 7 out of 151 patients screened (4.6%). In one patient metastasis was located in the iris, in 6 patients in the choroid. Intraocular metastasis was only found in patients with more than one other organ system involved into disease (p = 0.002). In this subgroup of patients (n = 65) prevalence of intraocular metastasis was 10.8%. Other risk factors for intraocular metastasis were presence of lung metastasis or brain metastasis. In 5 out 6 patients with choroidal metastasis external beam radiotherapy was performed, resulting in durable regression of metastasis and stabilization or improvement of visual acuity. CONCLUSION: The prevalence of intraocular metastasis in metastatic breast cancer was determined to be 4.6%. It is most commonly located in the choroid and develops in the course of advanced metastatic disease. Since early external beam radiotherapy of choroidal metastasis prevents functional loss, patients at risk should be given an ophthalmological screening for intraocular metastasis.

CRH test prior to discontinuation of long-term low-dose glucocorticoid therapy.

Often long-term low-dosage glucocorticoid therapy cannot be terminated. This is due to the fact that even low doses which are within the physiological replacement range can cause a detectable, though clinically insignificant suppression of the adrenal gland function, resulting in "corticosteroid withdrawal syndrome". Another reason is the fact that it is necessary to be able to suppress undesirable inflammatory reactions caused by the underlying disease. ACTH testing of the adrenal capacity is widespread, but repeated testing may lead to undesirable side effects, such as allergic reactions. This study investigates the usefulness of testing the function of the pituitary-adrenal axis in predicting withdrawal problems. In 21 patients with chronic inflammatory disease who were treated with glucocorticoid doses of 5 to 10 mg prednisolone equivalent daily for a period of 2 to 131 months, stimulation with 100 microg hCRH (human corticotrophin-releasing hormone) was performed prior to the gradual withdrawal of medication. Blood samples were taken at baseline and after 45 minutes to measure ACTH and cortisol levels. Four weeks after steroid withdrawal the patients were reevaluated for signs of a relapse of the underlying disease in order to establish the necessity of reintroducing steroid therapy. This reevalution comprised clinical criteria, laboratory tests and the patients' own assessment of his/her well-being. In sixteen patients who later successfully withdrew from glucocorticoid therapy, a significant increase in cortisal levels was noticed after stimulation with CRH (p < 0.05). In five patients, with whom steroid withdrawal was not successful, baseline levels of cortisol were significantly lower than in the others (p < 0.05) and no sufficient increase was achieved after stimulation with CRH. These results show that successful withdrawal of a long-term low-dosage glucocorticoid therapy depends on the integrity of the pituitary-adrenal axis. Therefore CRH testing for evaluation of the pituitary-adrenal axis can be helpful in identifying patients in whom glucocorticoid withdrawal would be troublesome.

Reliability of the Agitated Behavior Scale.

OBJECTIVE: The objective of this study was to investigate the interrater reliability of the Agitated Behavior Scale. DESIGN: Ratings made by research assistants and nursing staff were compared. PARTICIPANTS AND SETTING: Forty-five persons with brain injury and 23 persons with progressive dementia were studied at an acute rehabilitation unit and a long-term-care facility. RESULTS: Ratings of persons with brain injury by research assistants yielded a correlation coefficient for the Total score of.920. The correlation coefficients for the factors Disinhibition, Aggression, and Lability were.902,.909, and.726, respectively. Lower coefficients were obtained when the ratings of the research assistants and nursing staff were correlated; these ranged from.364 to.604. The ratings by research assistants of long-term-care facility residents yielded coefficients ranging from.860 to.906 for the Total and factor scores. CONCLUSION: This study shows that the Agitated Behavior Scale is a reliable instrument for measuring agitation in persons with traumatic brain injury, as well as with long-term-care facility residents experiencing dementia.

Somatic function, mental health and psychosocial functioning in 22 adolescents with bladder exstrophy and epispadias.

PURPOSE: We report the long-term somatic outcome, mental health and psychosocial adjustment in adolescents with bladder exstrophy and epispadias. MATERIALS AND METHODS: A total of 22 adolescents 11 to 20 years old (median age 14.5), including 19 with bladder exstrophy and 3 with epispadias, were assessed for urogenital status, stoma, renal and bowel function, anorectal physiology, mental health and psychosocial functioning by physical examinations, semistructured interviews and standardized questionnaires. The parents of 21 patients were interviewed and completed questionnaires. Information was also obtained on control groups. RESULTS: Of the 22 patients 9 (41%) had no urinary diversion and were urinary incontinent, 6 (27%) had persistent fecal staining and anal canal pressures that were lower than the controls, 10 (59%) were dissatisfied with the penile appearance and 11 (50%) met the criteria for psychiatric diagnoses. The main predictors of mental health were parental warmth and patient genital appraisal in the 11 to 14-year age group, and parental warmth and urinary continence function in the 15 to 20-year age group. Psychosocial dysfunction was predicted by fecal incontinence in the younger group and worries about future sexual relationships in the older group. CONCLUSIONS: The present multimodal outcome study revealed that adolescents with bladder exstrophy and epispadias had significant physical and mental problems. Genital malformation, and urinary and fecal incontinence may have a negative impact on mental health and psychosocial functioning. Our findings emphasize the need to include psychosocial experts on health care teams to reveal the amount of distress caused by these anomalies and to offer psychosocial support.

Frequency of asymptomatic choroidal metastasis in patients with disseminated breast cancer: results of a prospective screening programme.

AIM: To determine the frequency of visually asymptomatic choroidal metastasis in patients with disseminated breast cancer and its dependence on the incidence of metastasis by number and site of other organ metastases. METHODS: From January 1995 until April 1997 120 patients irradiated for disseminated breast cancer underwent ophthalmological screening for choroidal metastasis. No patient was symptomatic for ocular disease. 68 out of 120 patients were found to have metastases in one organ and 52 patients had metastases in more than one organ. 80% of the patients had bone metastases, 25% lung metastases, 22% liver metastases, 15% brain metastases, and 22% had metastases in other organs. RESULTS: Six patients (5%) were found to have asymptomatic choroidal metastases. Five patients had unilateral and one patient bilateral metastases. 52 patients with more than one involved organ had a significantly higher risk for asymptomatic choroidal metastasis (6/52, 11%) than 68 patients with metastases in only one organ (0/68) (p = 0.006). In univariate analysis a significantly higher risk was seen for patients with lung metastases (14% choroidal metastases versus 2% in patients without lung metastases, p = 0.03) and for patients with brain metastases (17% choroidal metastases versus 3% in those without brain metastases, p = 0.04). CONCLUSION: In disseminated breast cancer the incidence of asymptomatic choroidal metastases was 5% and increased to 11% when more than one organ was involved in metastatic spread. Risk factors for choroidal metastases were dissemination of disease in more than one organ and the presence of lung and brain metastases.