[Primary lymphoma of the small intestine. A clinical case report and review of the literature]. / Il linfoma primitivo dell'intestino tenue. Descrizione di un caso clinico e revisione della letteratura.

The authors present a case of small bowel primitive lymphoma found in a 66-years-old female. After having briefly reviewed the literature they direct their attention to the clinical-instrumental diagnostic difficulties of this neoplasm which is often discovered only when it has already invaded various organs. Therefore surgical therapy is not always radical. This fact in addition to the fatally rapid postoperative course led the authors to discuss the problems involved in a correct treatment of this pathology.

[The laparoscopic approach to the polycystic liver and adnexal cysts]. / Approccio laparoscopico alla policistosi epatica e alle cisti annessiali.

Having re-examined the nosographic data acquired on the subject of hepatic polycystosis and uterine adnexal cysts, the authors report the results of their experience. On the basis of the evaluation of these results (no laparotomic conversion, no postoperative complications, reduced hospital stay), which are comparable to those reported in the literature, the authors conclude that the modern technology may be used to advantage in all cases where laparoscopic surgery is not difficult, does not require numerous tests and does not entail a high risk of complications.

[The utility of isolating the recurrent nerve and parathyroids in the prevention of complications in thyroid surgery. The role of the superior laryngeal nerve and C cells]. / Utilità dell'isolamento del nervo ricorrente e delle paratiroidi nella prevenzione delle complicanze in chirurgia tiroidea. Il ruolo del laringeo superiore e delle cellule C.

The authors emphasize systematic isolation of the recurrent nerve and of the parathyroids, to prevent complications in surgery of the thyroid. Moreover they try to underline the significance of the superior laryngeal nerve and the role of parafollicular cells (today unknown) for a rational and modern therapy of the non-neoplastic diseases of the thyroid.

[Low ileorectal anastomosis in the surgery of ulcerative rectocolitis]. / L'ileorettoanastomosi bassa nella chirurgia della rettocolite ulcerosa.

The authors report their experience of 23 patients with ulcerative colitis treated with surgery from 1991 to 1994. At admission 8 patients had a high-grade illness, 10 patients had a middle-grade illness, 5 patients had a toxic megacolon. All the patients were treated with systemic medical management (mesalazine + methylprednisolone) and topical medical management with rectal steroid (methylprednisolone) and rectal mesalazine by enema, and with nutritional support (Total Parenteral Nutrition). The 5 patients with toxic megacolon, after 48 hours of unsuccessful medical management, underwent surgery with deferred urgency; the other 18 patients underwent surgery after one-four months. The authors prefer the ileorectal anastomosis (IRA), since the rectal lesions are more susceptible to topical therapy. A single-stage IRA was performed in 17 patients. A sigmoid resection has been employed in the first stage in the 18th patient with a local development of the disease in the sigma; a following relapse of the lesions required a total colectomy with a low ileorectal anastomosis. A first-stage subtotal colectomy with ileostomy and Hartmann closure of the rectum with low ileorectal anastomosis at a later date was performed in the 5 patients with toxic megacolon. One of these, 18 years old, died after a heart failure. After surgery, as soon as the patients start moving their bowels all of these had again a systemic therapy with mesalazine for a short period and after they had a topical therapy with mesalazine + methylprednisolone by enema, for a long period. The average postoperative period of admission was 16.3 days. The functional results have been encouraging with an average of 1.6 bowel movements daily and an average of 0.4 nocturnal bowel movements. All the patients had a normal anal sphincter function with an acceptable stool frequency. In male patients there no urinary or sexual defects. In one case of these, there was a low grade of dysplasia, revealed by endoscopic biopsy. The authors conclude that today surgical treatment of ulcerative colitis is not well established.

[Neutropenia in glycogenesis I B]. / Neutropenia nella glicogenosi IB.

Type IB Glycogen storage disease (GSD) is a new variant of type I Glycogen storage disease. It is characterized by same clinical findings: hepatomegaly, fasting hypoglycemia, hyperlipidemia, hyperuricemia, lactic acidosis, renal enlargement, short stature; but it distinguish for normal glucose-6-phosphatase hepatic activity in vitro. The involvement is in G-6-P transport system. Recently has been described in some patients with GSD IB, neutropenia and defective neutrophil mobility. In this report the authors described two family cases of GDS IB that one characterized by severe neutropenia.

[Lawrence Moon Biedl Bardet, a polymorphic syndrome]. / Laurence Moon Biedl Bardet, una sindrome polimorfa.

The Laurence Moon Biedl Bardet syndrome is a polymorphous disease whose pathogenesis is still obscure. It is characterize by obesity, oligophrenia, polidactylia, retinitis pigmentosa, hipogonadism, but often there are various others symptoms. AA describe two cases. After a short explanation of de main features of this disease, they dwell upon the study of clinical objective symtomatology and upon instrumental and laboratory parameters regarding hormonal, metabolic and functional order of various organs and apparatuses. Both cases present all classic symptoms of this disease. In the first case we have noticed a deficit in LH and FSH, besides we have also noticed an asymmetry of the lateral ventricles of the brain prevalently on the right and a small increase in 17-KS and 17-OH-KS urinary. On the contrary in the second case we have noticed an EEG of epileptic type.

[The Silver-Russel syndrome]. / La sindrome di Silver-Russel.

The authors report a case of Silver-Russel syndrome with severe deficit growth observed since intrauterine life. They describe the major features, that are constant and the minor features, that are variable and numerous. The features not always are present at birth. The A. exclude in this case a genetic cause and suggest a possible motherly cause.

Measurement of rapamycin in whole blood using reverse-phase high-performance liquid chromatography.

Rapamycin (RAPA) is an immunosuppressant with a potency similar to FK 506 and up to 100-fold greater than cyclosporine. To date, no data have been reported on the pharmacokinetics and disposition of the drug, primarily due to a lack of suitable methods for its analysis. We describe here an evaluation of a reverse-phase high-performance liquid chromatographic (HPLC) method for analysis of the drug in whole blood. The method showed excellent analytical recovery of the drug, close to 100%. The sensitivity method was found to be 1.0 micrograms/L with between-run coefficients of variation of 14.4% and 9.8% at 10 and 50 mg/L. The stability of RAPA was investigated with this method in whole-blood specimens. It was found that the drug was stable for 30 days at 4 degrees C and for 55 days at -40 degrees C.

Abbott TDx monoclonal antibody assay evaluated for measuring cyclosporine in whole blood.

We report here the evaluation of the Abbott TDx assay with a monoclonal antibody for selectively quantifying cyclosporine (CsA) in whole blood. Over the clinically relevant concentration ranges, results with this assay demonstrated within- and between-run CVs of less than 2.5% and 5%, respectively; sensitivity of 25 micrograms/L; good analytical recovery (100.3%); and linearity with whole-blood specimens. The percentage cross-reactivity of the major CsA metabolites varied from 15.3% for AM9 (M-1), 8.2% for AM1 (M-17), and 3.7% for AM4N (M-21), to less than 3% for the other metabolites tested. Results with the TDx assay (y) correlated well with those by the Sandimmune selective RIA (x; Sandoz) with blood specimens from 44 renal-transplant recipients (n = 44, x= 187.3, y = 198.9, y = 5.49 + 1.03x, r = 0.987). The TDx values were on average 24% higher than those by HPLC (x') with the same patients' specimens (n = 44, x' = 159.9, y = 198.9, y = 15.9 + 1.14x', r = 0.967). We conclude that the Abbott TDx monoclonal antibody assay provides a rapid, precise, and accurate means for quantifying CsA in whole blood.

Distribution of cyclosporin G (NVa2 cyclosporin) in blood and plasma.

We report here on the distribution of cyclosporin G (CsG), an analog of cyclosporin A, in whole blood. CsG has a temperature-dependent distribution between erythrocytes (RBCs) and plasma. After 30 min at 37 degrees C, the plasma/whole blood and plasma/RBC ratios were relatively constant (0.7-0.8) up to CsG at 1000 micrograms/L. At 5000 micrograms/L, this ratio increased to 1.0-1.1 for plasma/whole blood and 1.7 for plasma/RBC. The primary CsG metabolites GM1 and GM9 were sequestered within RBCs to a greater extent than was the parent drug. In whole blood, approximately 2% of CsG was bound to granulocytes, 6% to lymphocytes, and 50-55% to RBC, and 35-40% was found in the plasma fraction. The free fraction of the drug as determined by ultracentrifugation was 5-6% and 13-17% of total drug at 37 and 4 degrees C, respectively. In plasma the drug was primarily associated with high-density lipoprotein (50-60%) and to a lesser degree with low-density (20-30%) and very-low-density (10%) lipoproteins.

Double diagnostic meaning of serum transferrin receptor in hemodialysis patients: two case reports.

Hemodialysis patients on maintenance erythropoietin need an adequate supply of iron to optimize therapy and achieve and maintain target levels of hemoglobin. Evaluation of iron stores and early detection of iron deficiency are essential for management of erythropoiesis in chronic renal failure, but there is still no single biochemical or hematological parameter that is sensitive or specific enough to completely describe the distribution of iron in the body. Serum transferrin receptor (sTfR) is a marker of iron that is available for erythropoiesis. We selected 2 clinical cases in which hemodialysis patients were receiving maintenance erythropoietin. To suggest how sTfR can be used in its double diagnostic meaning according to the clinical context of the patient, sTfR was evaluated in one case as a marker of iron deficiency and in the other as a marker of erythropoiesis. The association of sTfR with hematological parameters of iron-deficient erythropoiesis (reticulocyte hemoglobin content, percentage of hypochromic erythrocytes, ratio of reticulocyte hemoglobin content to hemoglobin content) and parameters of stimulated erythropoiesis (absolute reticulocyte count, immature reticulocyte fraction) increases the accuracy of sTfR in its double diagnostic power.