Brain abscess due to Klebsiella pneumoniae in a liver-transplanted child.

Brain abscesses are a rare, severe complication of orthotopic liver transplantation (OLT). They are almost exclusively due to fungi, Nocardia, or Toxoplasma, and usually occur within months of surgery. Here we report the case of an adolescent who developed a brain abscess due to Klebsiella pneumoniae 11.5 years after OLT. Fever was absent and laboratory parameters were not indicative of infectious disease, and therefore the diagnosis of a central nervous system neoplasm was considered. Subsequent magnetic resonance imaging and spectroscopy led to a diagnosis of a brain abscess, and to prompt appropriate antibiotic treatment. This case shows that K. pneumoniae may cause a brain abscess long after liver transplantation. The appearance of neurological symptoms should alert clinicians to consider a brain abscess even in the absence of overt clinical/laboratory signs of inflammation, which may be blunted by chronic immunosuppression.

Clinical utility of electrophysiological evaluation in Crigler-Najjar syndrome.

We evaluated the neurological and neurophysiological features in ten patients with genetically characterized Crigler-Najjar (CN) syndrome: four with typical type I CN had undergone orthotopic liver transplantation (OLT); six had type II CN, and three of them developed severe hyperbilirubinemia with a limited response to phenobarbital leading to an intermediate phenotype I/II. Clinical neurological and multimodal electrophysiological evaluations [electroencephalogram (EEG), visual (VEPs), motor (MEPs) and brainstem auditory (BAEPs) evoked potentials] were performed. Neurological examinations showed mild hand tremor in four patients (one pre-OLT and one post-OLT type I, two type I/II). EEG revealed high voltage paroxysmal discharges in four patients (three type I/II, and one type I with a marked improvement after OLT). VEPs showed P100 wave increased latency in five patients (three type I, and two type I/II considered for OLT evaluation). MEPs showed prolonged central motor conduction time in five patients (two type I; one type I/II; two type II). Only EEG and VEPs findings showed a correlation with high bilirubin levels. BAEPs were normal. In conclusion, VEPs and EEG contribute to identify and monitor bilirubin neurotoxic effects, and may play a decisional role in some cases of severe hyperbilirubinemia without overt neurologic damage.

Macro-creatine kinase and macro-lactate dehydrogenase in a girl with ulcerative colitis.

We report the case of a girl affected by ulcerative colitis with high serum levels of creatine kinase and lactate dehydrogenase due to the presence of macroenzymes. Our observation suggests that the knowledge of this association may be helpful when evaluating ulcerative colitis patients with unexplained isolated elevation of one or more serum enzyme activities.

Life saving cyclophosphamide treatment in a girl with giant cell hepatitis and autoimmune haemolytic anaemia: case report and up-to-date on therapeutical options.

We report the case of a girl affected by giant cell hepatitis associated with autoimmune haemolytic anaemia. Both conditions were severe with a number of life-threatening episodes of liver failure and anaemia unresponsive to several immunosuppressant drugs but cyclophosphamide. After a low-dose long-term treatment with this drug the patient is stably well without any therapy. A review of therapeutical options in this condition is also presented.

Screening haemodialysis patients for infection with human immune deficiency virus (HIV).

A group of 484 patients having regular haemodialysis was tested for the presence of antibodies to the human immunodeficiency virus (HIV). With a commercial enzyme-linked immunoassay kit, the serum of 17 appeared positive. When these 17 samples were retested by a different method, however, none was found to contain antibodies to the virus. Furthermore, evaluation of the clinical state of these 17 patients for the presence of any prodromal symptoms associated with the acquired immune deficiency syndrome was negative. It is therefore suggested that patients having regular haemodialysis are presently at low risk of contracting infection by HIV. By contrast, 81% of these patients had antibodies to cytomegalovirus.