Lumbar disc herniation in three patients with cystic fibrosis: a case series.

INTRODUCTION: To date, lumbar disc herniation has not been reported in the context of cystic fibrosis even though back pain and musculoskeletal problems are very common in patients with cystic fibrosis. CASE PRESENTATION: We report on three patients with cystic fibrosis who experienced lumbar disc herniation in the course of their disease at ages 19 to 21 years (a 22-year-old Caucasian man, a 23-year-old Caucasian man, and a 21-year-old Caucasian woman). Our third patient eventually died because of her deteriorated pulmonary situation, which was influenced by the lumbar disc herniation as it was not possible for her to perform pulmonary drainage techniques properly because of the pain. CONCLUSIONS: Lumbar disc herniation can lead to a vicious cycle for patients with cystic fibrosis as it may promote pulmonary infections. This report highlights the need to investigate patients correctly.

A new method to analyse the pace of child development: Cox regression validated by a bootstrap resampling procedure.

BACKGROUND: Various perinatal factors influencing neuromotor development are known from cross sectional studies. Factors influencing the age at which distinct abilities are acquired are uncertain. We hypothesized that the Cox regression model might identify these factors. METHODS: Neonates treated at Aachen University Hospital in 2000/2001 were identified retrospectively (n = 796). Outcome data, based on a structured interview, were available from 466 children, as were perinatal data. Factors possibly related to outcome were identified by bootstrap selection and then included into a multivariate Cox regression model. To evaluate if the parental assessment might change with the time elapsed since birth we studied five age cohorts of 163 normally developed children. RESULTS: Birth weight, gestational age, congenital cardiac disease and periventricular leukomalacia were related to outcome in the multivariate analysis (p < 0.05). Analysis of the control cohorts revealed that the parents' assessment of the ability of bladder control is modified by the time elapsed since birth. CONCLUSIONS: Combined application of the bootstrap resampling procedure and multivariate Cox regression analysis effectively identifies perinatal factors influencing the age at which distinct abilities are acquired. These were similar as known from previous cross sectional studies. Retrospective data acquisition may lead to a bias because the parental memories change with time. This recommends applying this statistical approach in larger prospective trials.

Intrathecal synthesis of anti-viral antibodies in pediatric patients.

INTRODUCTION: Detection of intrathecal synthesis of specific antibodies (antibody index (AI)) is an established method to prove cerebral viral infection. Experience on its clinical application in large patient groups, however, is sparse. METHODS: Retrospective analysis of pediatric patients with positive viral AI treated at RWTH Aachen University Hospital between 1999 and 2005. RESULTS: 63 patients were studied, including 14 with encephalitis, 12 with neuritis, nine with cerebral vasculitis, six with multiple sclerosis (MS), five with severe cephalgia, five with psychiatric symptoms, three with hearing loss, two with seizures, three with white matter diseases, two with movement disorders, one with meningococcal meningitis and one with sinus venous thrombosis. Seven had several positive AI among them only one patient with MS. Of the 51 patients with a single positive AI and not having MS, 16 showed a positive AI for herpes simplex-, 13 for varicella zoster-, nine for Epstein-Barr-, four for cytomegalo-, four for mumps-, three for rubella- and two for measles virus. Frequent combinations were varicella zoster virus (VZV) and vasculitis (n = 8), herpes simplex virus (HSV) and neuritis (n = 6), Epstein-Barr virus (EBV) (n = 5), respectively, VZV (n = 4) and encephalitis as wells as mumps virus (n = 2) and hearing loss. Matched polymerase chain reaction (PCR) and AI data were available in 25 patients. PCR was simultaneously positive in three cases only. DISCUSSION: AI testing identifies a similar spectrum of pathogens as known from cerebrospinal fluid (CSF) PCR studies. It complements the PCR and increases the chance for adequate diagnosis and treatment of patients with assumed cerebral viral infections.

[Prevalence of optic atrophy and associated ocular and systemic diseases in a department of paediatric ophthalmology]. / Prävalenz der Optikusatrophie und assoziierte okuläre und systemische Erkrankungen in einer kinderophthalmologischen Sprechstunde.

BACKGROUND: Optic atrophy is one of the most common causes of severe visual impairment in children. So far an analysis of ocular and systemic findings comparing patients with and without optic atrophy has not performed. PATIENTS AND METHODS: Ocular and systemic findings of a total of 1042 patients (examination of all patients in May 1995 [N = 485] and May 2001 [N = 557]) of the department of paediatric ophthalmology in Homburg/Saar were retrospectively evaluated and diagnoses of patients with and without optic atrophy were compared. Optic atrophy was diagnosed ophthalmoscopically and in 1/3rd of the patients by VEP as well. RESULTS: 18 % of all patients (N = 188; 87.2 % [N = 164] were children) had optic atrophy. Nearly half of these children were prematurely born (46.7 %). 53.2 % of patients with optic atrophy (N = 88) showed nystagmus (without atrophy: 10.7 %), especially sensory defect nystagmus. Median of visual acuity level was 0.2 with optic atrophy and 0.8 without. Albinism and buphthalmia were common findings. 69.5 % of all patients with optic atrophy suffered from systemic diseases (without atrophy: 25.2 %), especially mental retardation, neurologic findings and oculocutaneous albinism. In 10.4 % more than two systemic diagnoses could be found. 55.3 % of all the patients with optic atrophy were disabled, 31.9 % multiply disabled. CONCLUSIONS: Sequelae of prematurity, peripartal asphyxia and congenital brain damages are the main findings in patients with optic nerve atrophy. Such children are worst-case patients of any paediatric ophthalmology department with a high prevalence of severe visual impairment, mental retardation and multiple disability. Treatment in a specialised center is therefore necessary for an efficient therapy. In addition, the statistical survey shows that in children who survived the critical phases of prematurity secondary damages can lead to persistent impairment and to alterations of visual and general development.