Association of Eosinophilic fasciitis and epileptic seizure.

Visceral involvement in eosinophilic fasciitis (EF) is a well known but rare event. With regard to neurological manifestations, both carpal tunnel syndrome and peripheral neuropathy have been described. We report the case of a 40-year-old woman with EF who had a major motor seizure. This association might not be fortuitous and be related to the known neurotoxin of eosinophils.

[Amyotrophic lateral sclerosis in a patient with Waldenstrom's macroglobulinemia]. / Sclerosi laterale amiotrofica in un paziente con macroglobulinemia di Waldenstrom.

Although a peripheral neuropathy is the best known neurological complication of Waldenstrom's Macroglobulinemia (WM), the association of Amyotrophic Lateral Sclerosis (ALS), or other Motor Neuron Diseases (MND) with monoclonal gammopathies is described. We report the case of a male patient (41 years old) with WM and ALS. Whether monoclonal gammopathies play a role in the pathogenesis of MND is unclear but is la possible that patients might have antibodies against motor neurons. In our reported case neurologic symptoms were the first and the most important manifestations of the underlying hemopathy and despite plasmapheresis and immunosuppressive treatment ALS syndrome progressed. The neurologic disease worsened despite the improvement of WM.

Treatment of hemodialysis-related porphyria cutanea tarda with small repeated phlebotomies.

Phlebotomy is generally thought to be contraindicated in porphyria cutanea tarda (PTC) associated with hemodialysis for the anemia which is often present in uremic patients. We report a patient with a severe form of hemodialysis-related PTC and biochemical parameters suggestive of iron overload in whom treatment with small repeated phlebotomies was well tolerated without any significant worsening of the anemia and gave marked biochemical improvement and complete clinical remission.

Lithium-induced sinus node dysfunction.

A 74-year-old woman, on lithium carbonate treatment, developed symptomatic sinus node dysfunction and central nervous toxicity, which disappeared after discontinuation of the drug and when serum lithium fell from 2 to 1.27 mEq/L. Our review of the literature demonstrates that sinus node abnormalities can occur both in presence of therapeutic and toxic serum lithium levels and are frequently asymptomatic and completely reversible. All patients receiving lithium should have their pulse regularly recorded and the drug should be promptly discontinued if severe bradycardia or other rhythm disturbances appeared during the treatment.

[Neonatal necrotizing enterocolitis (NEC). Observations of 21 cases studied from 1977 to 1980]. / Enterocolite necrotizzante del neonato (NEC). Rilievi su 21 casi osservati negli anni 1977-1980.

We have carried out a study together with neonatologists, paediatricians, paediatric surgeons and paediatric radiologist on the etiopathogenic, clinical-statistical, therapeutical and evolutionary aspects of NEC during the period from 1977 to 1980 in the Neonatology Clinic and in the Paediatric Surgery Department University of Bologna. The most important data were as follows: a high percentage of full-term newborns, the apparently unfavourable action of artificial feeding, a little amount of infectious etiology, an absence of predisposing factors in more than half the cases, an uniform symptomatology in a lot of patients with adverse evolution in the cases of secondary colic stenosis. The mortality was 33 percent, specially among the small for gestational age infants. The surgical therapy during four years period was modified technically, i.e. there was an orientation towards conservative surgery. The final surgical operation has recently been made right after the fourth month from the acute crisis as it has recently been demonstrated that secondary stenosis always occurs during this period of time. Long - term follow - ups showed: completely satisfactory progress in patients treated only with medical therapy, particularly unfavourable evolution in patients after a more demolishing - type of operation (resectioning of the last ansa of ileum and ileum - caecum valve), considerable improvement in auxological and nutritional parameters also in the latter cases after the final surgical operation.

Free-cortisol assay by immunoextraction: comparison with an equilibrium dialysis procedure.

We describe a new method for directly determining the apparent free cortisol concentration in plasma samples by use of an antibody-coated test-tube RIA. Buffer-diluted plasma or standard serum is added to antibody-coated test tubes, incubated at 4 degrees C for 4 h, and the solutions are aspirated. 125I-labeled cortisol is added to each tube and incubated for 3 h at 4 degrees C. Then the insides of the tubes are washed and their radioactivities counted. The standard curve is in terms of free cortisol, the standard serum solutions having been measured with an equilibrium dialysis procedure. Plasma samples (n = 155) from normal subjects and from various patients, measured with the new immunoextraction method and the equilibrium dialysis technique, gave results that correlated well (r = 0.847, p less than 0.001). Results by this direct RIA also correlate well with the clinical adrenocortical status of patients for whom data on total plasma cortisol may be misleading. This simple, easy RIA is suited to be the routine method for free cortisol in plasma.

Hypoglycemia associated with a leiomyosarcoma of the small bowel.

A patient with recurrent hypoglycemic episodes associated with a leiomyosarcoma of the small bowel is described. Fasting plasma insulin levels were consistently low and a subnormal insulin response to provocative stimuli (oral glucose and i.v. glucagon) was shown. After removal of the tumor, hypoglycemic episodes disappeared, fasting plasma insulin concentrations rose to normal values and insulin response to both OGTT and glucagon test was markedly enhanced. The possible mechanisms responsible for the hypoglycemia and the causes of subnormal insulin response to provocative stimuli are discussed.

[Urinary guanidinosuccinic acid in uremic children]. / L'acido guanidinosuccinico urinario in soggetti uremici in eta' pediatrica.

Urinary guanidinosuccinic acid (GSA) was measured in pediatric age: six normal subjects, six chronically uremic patients and five with acute renal failure. Urinary GSA was increased in uremic patients as compared to that in normal subjects: our levels was less than in the growth-up people. These differences might be correlated with different protein metabolism in children.

[4 cases of tricholeukocytic leukemia]. / Osservazioni su 4 casi di leucemia tricoleucocitica.

The clinical, haematological, cytomorphological, cytochemical, ultrastructural, and bone marrow biopsy histological data observed in 4 cases of tricholeukocytic leukaemia are reported, along with some unusual findings. Stress is placed on the varied course of this disease and the difficulties associated with its treatment. Some criteria for the choice of therapy are also proposed.

Prenylamine-induced ventricular arrhythmias and syncopal attacks with Q-T prolongation. Report of a case and comment on therapeutic use of lignocaine.

A 70-year-old man, on prenylamine for exertional angina, complained of syncopal attacks which seemed to be caused by bursts of ventricular tachycardia associated with Q-T prolongation. These symptoms disappeared after treatment with lignocaine, and the Q-T interval gradually returned to normal when prenylamine was stopped. This communication emphasizes the possibility of the occurrence of Q-T prolongation with associated ventricular arrhythmias in patients treated with prenylamine and the usefulness of a cautious trial with lignocaine in ventricular arrhythmias linked to Q-T prolongation.