[Primary empty sella syndrome (ESS). Clinical observations on 20 cases]. / Empty sella syndrome (ESS) primitiva. Osservazioni cliniche su 20 casi.

The authors report 20 cases of primary empty sella syndrome (ESS) observed during the last 5 years in their Department of Internal Medicine. Clinical data and particular syndromes are discussed and compared with data of partially reviewed literature. The authors discuss the classification of primary and secondary ESS with their possible endocrine, ophthalmological and neurological symptoms, or--on the contrary--without clinical signs as more frequently observed. The usual diagnostic and therapeutic procedures are also reported.

[Gastrointestinal cancer-associated antigen (GICA) and intravascular hemolysis. Report of 2 cases and review of the literature]. / Gastrointestinal cancer-associated antigen (GICA) ed emolisi intravascolare. Presentazione di due casi e revisione della letteratura.

GICA or CA 19-9, monosialyl Lewis (a) antigen, is a ganglioside, component of cellular wall. GICA is known as a neoplastic marker with high correlation, specificity and sensibility for the gastrointestinal neoplasms, mainly of the pancreas. Two cases of intravascular haemolysis are reported (a case in woman with Moschowitz's syndrome and a case in man with mitral prosthetic valve and mild congestive heart failure) with elevated serum levels of GICA. First in the literature, the Authors hypothesize a strict correlation between the aspecific elevation of GICA serum levels and diffuse membrane cell damage in several body districts, and its significance (1-6%) in hemolytic, non neoplastic pathology. Its serum evaluation will be useful in the early diagnosis and follow-up of hemolytic pictures associated with several diseases (e.g.: thrombotic microangiopathies, as TTP, HUS, HELLP, etc; hemolysis by prosthetic valves, congenital cardiopathies, etc.; liver diseases; hemolytic anemias caused by intra- and extra-corpuscular defects). The literature is widely reviewed about significance of GICA serum elevation in neoplastic pathology, its diagnostic pitfall even in the presence of neoplastic diseases, aspecific elevation in non neoplastic diseases and its correlation with other illnesses.

[Antiemetic protocol in oncohematologic polychemotherapy]. / Protocollo antiemetico in corso di polichemioterapia oncoematologica.

Authors, in an open and preliminary study, evaluate the beneficial effects on chemotherapy-induced nausea and vomiting of an association of three antiemetic drugs parenterally administered. Twenty-six patients receiving chemotherapy for Lymphomas (195 cycles) were treated with: Chlorpheniramine maleate 10 mg IM 45' before, Sulpiride 200 mg in normal saline 100 ml, at XXX gtt/m', 20' before, and 6-Methyl-Prednisolone 500 mg IV over 2'-3' immediately before chemotherapy. Patients were evaluated for emesis over 48 hours after chemotherapy: they were interviewed on nausea, vomiting and other possible side-effects. The antiemetic activity observed was: complete response in 73.1% (19/26); partial response 27.9% (7/26). Failure of response was never observed. 166/195 cycles were well tolerated. Our results demonstrate the antiemetic efficacy of this antiemetic regimen. Further studies should investigate its use in randomized trials.

[Thrombotic thrombocytopenic purpura (Moschowitz syndrome). A clinical case and review of the literature]. / Porpora trombotica trombocitopenica (sindrome di Moschowitz). Caso clinico e revisione della letteratura.

A case of young woman affected by relapsing Thrombotic Thrombocytopenic Purpura (TTP), probably beginning in September 1986 and relapsing after 6 years, in June 1992, is described. After a first Complete Remission due to Plasma Exchange and Antiplatelet Agents, the patient relapsed during maintenance therapy with Ticlopidine and, over 1 month, developed two relapses which were resistant to traditional therapy. Showing resistance even to Iloprost, the patient had a Complete Remission only after a 2nd IV bolus of Vincristine according to Gutterman's scheme (modified), with prolonged therapeutic wellbeing. Many authors say that Plasma Exchange and Vincristine therapy must first be used in TTP and tried in refractory TTP. This one experience of ours should confirm that. From this TTP case, the authors begin a wide review of the literature, examining history, etiopathogenetic and therapeutic debate, possible related diseases, differential diagnosis, evolution, prognosis and future developments of this mysterious and rare illness.

[Pseudothrombocytopenia: clinical comment on 37 cases]. / Pseudopiastrinopenia. Considerazioni cliniche su 37 casi.

BACKGROUND: Pseudothrombocytopenia (PTCP) is characterized by in vitro EDTA-, pH-, temperature- and time-dependent clumping which gives rise to spurious piastrinopenia at platelet count in automatized analyzers. It is caused by serum immunoglobulins recognizing some cryptoantigens on the platelet surface membrane. Aim of this study was to evaluate the prevalence of PTCP in a series of patients and its clinical significance. METHODS: From all the inpatients, in Internal Medicine Division, during 29 months, we have considered as PTCP the thrombocytopenias persistent in EDTA and contemporaneously, missing with ACD at the automatized counter, after microscopic examination. Then we estimated: PTCP rate in regard to all patients, sex, age and morbid associations both in PTCP group and in all patients. These frequences were compared with the chi-square test. RESULTS: 37 cases of PTCP are observed from a cohort of 2922 inpatients (1.26%): 23 were males, 17 females; median age was 73.7+/-11.2. The frequency of the following associations was significantly higher in the PTCP group: solid tumors in 9/37 (24.3%), myelo- and lymphoproliferative syndromes in 7/37 (18.9%), autoimmune disease in 4/37 (10.8%). CONCLUSIONS: This study suggests that PTCP is one of the more frequent thrombocytopenias: it is important to identify it, to avoid clinical and therapeutic mistakes. Moreover PTCP, in our study, is often correlated with important diseases. We can conclude that in hospitalized old patients, PTCP could represent a signal to search more severe illnesses.

[Pregnancy complication in homozygous (delta beta) zero-thalassemia. A clinical case]. / Gravidanza complicata in (delta beta) zero-talassemia omozigote. Caso clinico.

A case of a pregnant woman suffering from homozygous (delta beta) zero-thalassemia clinically running as thalassemia Intermedia is described. In this patient pregnancy could not follow its normal course and was interrupted in the 24th week, because of endouterine death of the foetus due to complications such as serious haemolytic anaemia following transfusional alloimmunisation and the presence of portal vein thrombosis. In this article the literature is reviewed and the need is shown for all thalassemic patients to be tested for the various blood antigenic systems, so that transfusional reactions can be avoided. Stress is laid on the importance of erythropoietin both in the pathogenesis of haematological and rheological modifications in thalassemic patients and also as a trigger in serious thrombotic complications.

[Sweet's syndrome (acute febrile neutrophilic dermatosis) associated with Sjögren's syndrome. A clinical case]. / Sindrome di Sweet (dermatosi neutrofila acuta febbrile) associata a sindrome di Sjögren. Un caso clinico.

A case of Sweet syndrome associated with Sjögren syndrome is reported. After a review of the literature, the clinical and pathologic patterns of the disorder are described. The possible underlying or associated diseases, and the problems on diagnosis and prognosis are discussed. In spite of the several etio-pathogenetic problems still unsolved, the syndrome can be considered as a primary, immuno-mediate disorder.

[Lyme disease. Case contribution and review of the literature]. / Malattia di Lyme. Contributo casistico e revisione della letteratura.

Lyme borreliosis is a systemic disease. The etiologic agent is a tick-bite borne spirochete (Borrelia burgdorferi) of which men are casual hosts. Although the illness is reported worldwide, it is more frequent in the northern parts of Countries. Some isolated clinical pictures are reported in the past, but only in 1975 the illness was well described at Old Lyme (Connecticut, Usa) and then well defined in its pathogenesis and clinical course in three stages. Lyme disease, involving skin, heart, nervous system and joints, is frequently protean and misdiagnosed, so that it was called "the new great imitator". In Italy the first case is reported in 1985. The authors report three (3) new cases, mostly with neuro-articular signs and symptoms. Literature is partially reviewed, for a better definition and outline of some epidemiological, etiopathogenetic, historical and clinico-therapeutic aspects of Lyme disease.

[Celiac disease (familial) associated with sarcoidosis. Clinical case and review of the literature]. / Associazione di malattia celiaca (familiare) e sarcoidosi. Caso clinico e revisione delle letteratura.

The authors report a case of a 42 year-old patient, female, already suffering in 1990 from pulmonary sarcoidosis at 0 radiological stage, together with uveitis and relapsing erythema nodosum, with dyspepsia and weight loss which benefited from corticosteroidal therapy, repeatedly applied at the relapses of Erythema Nodosum. This therapy induced clinical recovery and marked weight gain. After 3 years (in 1993), the appearance of chronic diarrhoea, weight loss, oedemas of the lower limbs and altered laboratory findings which suggested malabsorbtive syndrome, made us verify with clinical-instrumental examinations (serum AGA IgA and IgG, Xilose test, perendoscopic jejunal biopsy) the diagnosis of fully clinically expressed adult coeliac disease. The screening of close relatives, showed that the patient's brother, HLA like (HLA A1-B8), was suffering from a less expressive coeliac disease. After a wide review of the literature, authors emphasize particular aspects of both diseases, reporting clinical manifestations, possible morbid linkage and prognostic factors. They underline epidemiological, pathogenetic and genetic/immunological similarity. bound to support a possible non-causal linkage of the diseases, even within the family. The authors think this linkage to be underestimated, because it is not often searched for on identified.

[Primary non-Hodgkin's lymphoma of paranasal sinuses. Review of the literature and a case report]. / Linfoma maligno non Hodgkin primitivo dei seni paranasali. Revisione della letteratura e caso clinico.

A case of primary extranodal non-Hodgkin's lymphoma arising in paranasal sinuses, in an 89 year-old woman is reported. Histologically it was lymphoplasmacytoid, pleomorphic, diffuse, at intermediate grade of malignancy according to "Working formulation for clinical usage" in I EB stage. Complete resolution was obtained with polychemotherapy according to the scheme ProMACE-CytaBOM. After a wide review of the literature, the Authors emphasize incidence, prevalence, clinical symptoms (especially local), the different beginning points, prognostic and therapeutic factors, and the most frequent associations with other illnesses. Agreeing with other Authors, the necessity of using aggressive polychemotherapy is stressed, also in elderly patients, even if at the beginning of the illness.

[Empty sella syndrome (ESS) associated with primary hyperparathyroidism. A clinical case and review of the literature]. / Sindrome della sella vuota (ESS) associata ad iperparatiroidismo primario. Caso clinico e revisione della letteratura.

The report describes a patient with coexisting primary hyperparathyroidism due to adenoma of the left superior lobe and the primary empty sella syndrome (ESS). Pathogenetic mechanisms, clinical pictures, associated illnesses and similarity between these diseases are discussed. A complete diagnostic procedure and follow-up is necessary even if the illnesses are oligosymptomatic. In fact this association may be part of multiple endocrine neoplasia (MEN) or other endocrine diseases.

[Hashimoto's thyroiditis, idiopathic thrombocytopenic purpura (Werlhof's disease) and Steinert's dystrophia myotonica. A clinical case]. / Tiroidite di Hashimoto, porpora trombocitopenica idiopatica (morbo di Werlhof) e distrofia miotonica di Steinert. Un caso clinico.

The authors report a rare association: Hashimoto's thyroiditis, thrombocytopenic idiopathic purpura (Werlhof's disease) and Steinert's myotonic dystrophia. The authors outline the fingerprints of each disease, report particular syndromes and compare that association to data of partially reviewed literature. They suppose a suggestive pathogenetic connection among these diseases, due to the coexistence of altered genetic, endocrine and autoimmune factors and consequently impaired cell membrane permeability.

[43 cases of primary empty sella syndrome: a case series]. / Quarantatre casi di "empty sella syndrome" primitiva: contributo casistico.

Primary empty sella syndrome (ESS) is an anatomo-radiological picture characterized by the presence of an arachnoid herniation filled with liquor that compresses the pituitary against the sellar wall. ESS occurs particularly in obese, hypertensive, cephalalgic women. It is often asymptomatic but may be associated with ophthalmologic, neurologic and non-characterizing endocrine disorders. We report here 43 cases of primary ESS observed and assessed in our Departments of Internal Medicine from June 1983 to May 1993. The following endocrinological diagnostic procedures were carried out: hormonal (RIA) basal profile: FT3, FT4, TSH, PRL, ACTH, FSH, LH, 8.00 a.m. and p.m., blood cortisol, aldo, PRA, DHEA-S, FTe, E2, P, PTH, CT, and calcemia and phosphoremia; provocative tests: TRH, GnRH, etc.; inhibition tests: high dose dexamethasone. Clinical, neurologic (skull radiographs, sellar stratigraphy, computed tomography scan and magnetic resonance), and ophthalmologic (fundus, visual fields) assessments were also made. Our findings fit with the data in the literature concerning common symptoms of ESS, associated endocrinopathies and other illness. We found obesity (62.7%), oligo-amenorrhea (16.6%), galactorrhea (14.6%), hyperPRL (11.6%), hypopituitarism (9.3%), hypogonadism (4.6%), diabetes insipidus (2.3%), (micro-)polycystic ovary syndrome (19%), hyperACTH (2.3%). In 9.3% of the cases, endocrinopathy referred to pituitary adenomas. Moreover, we noted a high frequency of psychological disorders, to our knowledge not previously reported in the literature, including anxiety or dysthymic disorders with altered behavior (chiefly oral compulsion). We also make the hypothesis that obesity (occurring in 62.7% of our patients) and hypertension (62.7%) may be related to hypothalamic alterations.

[Primary empty sella syndrome. Observations on 71 cases]. / Sindrome della sella turcica vuota primitiva. Osservazioni su 71 casi.

Primary empty sella syndrome (ESS) is an anatomo-radiological picture characterized by the presence of an arachnoid herniation filled with liquor that compresses the pituitary against the sellar wall. ESS occurs particularly in obese, hypertensive, cephalgic women, it is often asymptomatic but it may be associated with ophthalmologic, neurologic and sometime non-characterizing endocrine disorders. We report here 71 cases of primary ESS observed and assessed during the last fourteen years. The following endocrinological diagnostic procedures were carried out: hormonal (RIA) basal profile: FT3, FT4, TSH, PRL, ACTH, FSH, LH, 8.00 a.m. and p.m. cortisolemia, Aldo, PRA, DHEA-S, FTe, E2, P, PTH, CT, and calcemia and phosphoremia; provocative tests: TRH, GnRH, insulin hypoglycemia, etc.; inhibition tests: "overnight" and high dose dexamethasone. Clinical, radiological (skull radiographs, CT and/or MRI) and ophthalmologic (fundus, visual fields) assessment were made. We found principally cephalgia (52/71: 73.2%), hypertension (42/71: 59.1%), obesity (47/71: 66.1%). But we found especially mental disorders (57/71: 80.2%), in our knowledge not previously reported in the literature, as anxiety or dysthymic disorders with behavioural disturbances (chiefly oral compulsion). We found endocrinopathies in 36/71 (50.7%), isolated or coexisting in some patients: hyperPRL (14%), hypopituitarism (10.4%), hypogonadism (7%), diabetes insipidus (2.8%), hyperACTH (1.4%), hypoGH (15.4%), pituitary adenomas (8.4%). Several hypothalamic illness show a clinical picture including mental disorders and obesity. The Authors hypothesize that the ESS may be a "new" hypothalamic syndrome (compression/stretching on hypophysis and/or hypophyseal stalk by arachnoidocele; disorder of some hormones and neurotransmitters as leptin, neuropeptide Y, orexins, POMC-derived peptides, etc).

Nicotinic and M1-, M2-muscarinic cholinergic control of ACTH response to insulin-induced hypoglycaemia in man.

The possible mediation of muscarinic and/or nicotinic-cholinergic receptors in the response of ACTH to insulin-induced hypoglycaemia was evaluated in 18 normal men. Subjects were tested with the insulin (0.15 U/kg) tolerance test (ITT) in basal conditions and in the presence of the M1- and M2-muscarinic antagonist atropine (600 micrograms iv just before insulin injection (time 0) plus 600 micrograms 20 min later in 6 subjects) or the M1-muscarinic receptor blocker pirenzepine (40 mg iv 10 min before ITT or 20 mg at time 0 plus 30 mg at time 20 in 6 subjects). The remaining 6 men were treated with the nicotinic receptor antagonist trimethaphan (0.3 mg/min x 30 min before ITT). ACTH rose 4.7 times in response to hypoglycaemia. The ACTH response to hypoglycaemia did not change after pirenzepine administration, whereas it was significantly increased by atropine and decreased by trimethaphan treatment. These data indicate that nicotinic and muscarinic (M2 but not M1) receptors participate in a different manner in the regulation of the hypoglycaemia-induced ACTH release.