Guillain-Barré syndrome complicating acute hepatitis B. A case with detailed electrophysiological and immunological studies.

A 61-year old man was seen for Guillain-Barré syndrome (GBS) complicating acute type B hepatitis. Results of detailed electrophysiological studies were characteristic of a severe peripheral neuropathy of the segmental demyelinating type. Immunological studies demonstrated cell-mediated sensitization to peripheral nerve basic protein. Twenty-six cases of GBS complicating viral hepatitis were analyzed for age, sex, temporal relationship of the onset of the hepatitis to that of the polyneuritis, and outcome.

Familial poliodystrophy, mitochondrial myopathy, and lactate acidemia.

We describe a 16-year-old boy who has a progressive dementia and seizures. On investigation, he was found to have a mitochondrial myopathy and elevated lactate levels in the blood and cerebrospinal fluid. His sister died at 18 years of age of a similar condition.

Muscle carnitine deficiency and fatal cardiomyopathy.

A 23-month-old boy with progressive muscle weakness and severe cardiomyopathy was found to have oil red O positive vacuoles predominantly in type 1 muscle fibers. Serum carnitine was normal, but muscle carnitine content was decreased. Both parents were clinically normal, but the muscle carnitine level was low in the father. Despite oral treatment with carnitine, the condition progressed and was fatal. At autopsy, cardiac muscle showed borderline low carnitine content and numerous mitochondria, but no lipid accumulation.

Hemifacial spasm. Occurrence in multiple sclerosis.

We present six patients with hemifacial spasm and multiple sclerosis. To our knowledge, this association has not been described previously in the North American literature. Magnetic resonance imaging was obtained in all the patients and plaques consistent with multiple sclerosis were identified. In two patients the plaques were seen in the area of the facial nucleus on the involved side. We suggest that hemifacial spasm can be a manifestation of multiple sclerosis. These cases illustrate the utility of magnetic resonance imaging in the investigation of hemifacial spasm. Our findings also support a central (nuclear) origin in multiple sclerosis associated with hemifacial spasm.

Clinical experience with i.v. Mannitol in the treatment of ciguatera.

To assess the effectiveness of i.v. Mannitol treatment for Ciguatera Poisoning, 35 patients were treated from the Miami-Caribbean area with symptoms of acute and chronic Ciguatera Poisoning. Information was collected on demographics, fish type and location, timing and type of symptoms, and response to treatment. Iv Mannitol (1 g/kg infused 3-4 hours) given within 48 hours dramatically decreased the acute morbidity of Ciguatera Poisoning without serious side effects. Treatment also appears to be safe and effective in chronic cases up to eight weeks from ingestion of toxic fish.

A pilot study of a new ELISA test for ciguatoxin in humans.

The major impediment to the thorough study of Ciguatera in human populations has been the lack of definitive diagnostic ability. However, recently an ELISA test was developed which can diagnose Ciguatera qualitatively and quantitatively in human fluids, as well as in contaminated fish tissue. This study proposes to evaluate this new ELISA test in human subjects with the clinical diagnoses of acute and chronic Ciguatera Poisoning. The contaminated fish from exposed subjects, and the blood and urine of exposed and controls, will be examined using the new ELISA. The ELISA performance will be compared to traditional bioassays for the fish testing. In addition, a distinct diagnostic profile will be developed using serial questionnaires, physical examinations, and nerve conduction tests. Ultimately this ELISA test can be used not only in establishing the correct diagnosis of Ciguatera Poisoning, but also in the treatment and clinical prognosis, and in epidemiologic studies of Ciguatera Poisoning in human populations. We hope that this protocol will serve as a model for the study of the effects of other marine toxins on human populations.

Spectrum of systemic bacterial infections during febrile neutropenia in pediatric oncology patients in tertiary care pediatric center.

BACKGROUND: Outcome of pediatric cancers has significantly improved with modern chemotherapy and good supportive care. However, febrile neutropenia remains one of the important limiting factors in these patients especially with the emergence of resistant organisms. Choosing appropriate antimicrobials is possible only if we understand the local microbial spectrum and their sensitivity pattern. AIMS: To study the likely etiologic agents and their antibiotic sensitivity pattern among systemic infections in children with cancer. SETTINGS AND DESIGN: This is a prospective study. MATERIALS AND METHODS: The study was conducted at a tertiary care center for pediatrics, in which culture samples representing blood stream infections and others like urinary tract infections sent from the Oncology services of the Hospital during the year of 2013 were analyzed. The microbiological profile and antibiotic sensitivity pattern of these isolates were studied. RESULTS: There were 89 isolates that represented blood and urinary tract infections in neutropenic patients with cancer.Out of 89 positive cultures 76 were gram negative isolates. The most common gram negative bacterial isolates were Escherichia coli 33 (37%), followed by Pseudomonas 21 (23.5%). Acinetobacter grew in 2 patients (2.2%). Extended spectrum beta-lactamases (ESBL's), carbepenem resistant and pan-resistant organisms seen in 28 (31.4%), 5 (5.6%) and 2 cases (2.3%) respectively. Over all Gram-positive organisms were 13/89 (12.3%). Staphylococcus was the most common Gram-positive organism and methicillin resistant Staphylococcus aureus seen in 5 each. CONCLUSION: Gram-negative organism is a common isolate in cancer children with febrile neutropenia, which is resistant to first-line antibiotic cefepime. Meropenem is most sensitive antibiotic and ESBL's are sensitive to piperacillin-tazobactam.

Sigmoidal volvulus in childhood: report of two cases.

Volvulus of the sigmoid colon is a very uncommon cause of acute obstruction in children. Although common in adults in India, it was found to account for only 0.8 per cent of all acute obstructions in infants and children in this institution. It causes a proximal torsional obstruction of the colon with an acute onset of symptoms. The onset of volvulus is characterized by colicky pain over the left lower quadrant, vomiting, tenderness, and rigidity in te left lower quadrant. A scout film of the abdomen may be inconclusive, but a barium-enema examination is diagnostic. The number of cases reported is too small to allow conclusions about the best treatment for children who have sigmoidal volvulus.

Spinal cord compression in Paget's disease.

Three cases of spinal cord compression secondary to Paget's disease of the spine are reported. In two of the cases a relatively short history with pain as a prominent feature suggested initially a diagnosis of extradural malignancy. The usual clinical features of this rare disorder are discussed and the characteristic radiological findings are emphasized.

Transfer factor treatment in bullous pemphigoid. Impaired T cell function.

A patient with bullous pemphigoid, not responding to prednisone, had impaired cellular immunity. Transfer factor (TF) was given in doses of 10 U/m2 intramuscularly to improve T cell immunity, at a frequency of 3 times to once a week. Clinical improvement fluctuated with changes in the frequency of TF injections. The skin has been clear of the lesions for the past 9 months. Her T cell functions became normal and serum IgE decreased from 900 U/ml to 270 U/ml. She has received a total of 1,732 U of TF. This report is intended to encourage investigation into T cell functions in patients with bullous pemphigoid and controlled trials of TF in this disease.