Imported occupational lead poisoning: report of four cases.

BACKGROUND: In most industrialized countries, occupational lead poisoning has become increasingly rare, however this metal remains a serious health hazard in the rest of the world. REPORT OF CASES: We observedfour male patients (aged 35 / 54 years) who had suffered recurrent abdominal pain due to recent lead exposure (for 7 to 13 months) in two Chinese battery recycling plants. On their return to Italy, three of them presented normocytic, normochromic anaemia. The diagnosis was confirmed by high lead levels in the blood and urine, decreased erythrocyte delta-aminolevulinic acid dehydratase (ALA-D), raised erythrocyte zinc protoporphyrin (ZP), and elevated urinary excretion of b-aminolevulinic acid (ALA-U) and porphyrins. Chelation with EDTA resulted in increased urinary lead excretion, improvement of the clinical picture, decreased ZP, and progressive normalization of the other lead biomarkers (Pb-B, ALA-D, ALA-U, urinary porphyrins). CONCLUSIONS: Temporary work in developing countries may result in imported lead poisoning. Differential diagnosis of this unusual condition requires careful medical history collection and specific toxicological analysis. Preventive measures for workers going abroad are needed.

Divry-Van Bogaert syndrome. Clinical and ultrastructural findings.

A case of a progressive disease with epilepsy, marble skin, and roentgenographic evidence of tapering of the distal carotid branches with corticomeningeal angiomatosis was studied. The clinical course, angiographic findings, and skin biopsy results justified the diagnosis of noncalcifying venous capillary angiomatosis, or Divry-Van Bogaert syndrome.

Amiodarone induced phospholipidosis. Biochemical, morphological and functional changes in the lungs of rats chronically treated with amiodarone.

Amiodarone, an antiarrhythmic drug, causes pulmonary fibrosis in some patients during chronic treatment but the mechanism is unknown. We studied the effects of amiodarone on pulmonary biochemistry, morphology and function at doses of 25 and 50 mg/kg/12 hr given to rats by gavage for four weeks. Plasma and pulmonary phospholipids were significantly augmented, 13% and 88% respectively, in the group given amiodarone 50 mg/kg/12 hr compared to pair-fed controls. Typical phospholipidosis-like light and electron microscopic alterations were seen in the lung, their severity related to the extent of biochemical changes induced by amiodarone. Pulmonary function tests revealed mild but not significant changes in O2 and CO2 alveolar exchange efficiency and lung compliance (P-V curve) of treated animals in comparison to pair fed controls. Plasma average concentrations of amiodarone and its main metabolite, desethylamiodarone, after four weeks were 2.46 +/- 0.18 and 0.73 +/- 0.13 micrograms/ml, respectively, in the 50 mg/kg/12 hr group. In the same group amiodarone and desethylamiodarone concentrations in lung were 163 +/- 26 and 569 +/- 153 times higher than those in plasma. A highly significant correlation was found between amiodarone concentrations in plasma and lung and phospholipid content in the lung. A subgroup of animals received amiodarone 50 mg/kg/12 hr for 8 weeks. The pulmonary phospholipidosis-like lesions were similar to those observed after one month of treatment, no fibrosis was evident on light microscopic examination.

Neuropsychological evaluation in transient ischaemic attack and minor stroke.

Two groups of patients with transient ischaemic attack and minor stroke without detectable haemodynamic stenotic lesions were evaluated by neuropsychological tests and compared with a control group. The mean values of the scores adjusted for age and educational background demonstrated that the patients with transient ischaemic attack did not have a worse performance than normal subjects in any of the tests, the patients with minor stroke had a worse performance than normals, particularly in Rey's figure-copying test (P less than 0.025), and the latter test was not affected by educational background or age of the subjects. The results are discussed with reference to other case series, and the importance of age and cerebral damage in causing intellectual impairment evaluated by neurophysiological tests is stressed.

Friedreich's ataxia in northern Italy: I. Clinical, neurophysiological and in vivo biochemical studies.

Eighteen patients with the presumptive diagnosis of Friedreich's ataxia were studied. Clinical, neurophysiological and biochemical data were concordant in 14 patients and led to the diagnosis of typical Friedreich's ataxia in this group of patients. The remaining 4 patients differed from the typical patients in several respects, but mainly in the cardiological findings. It is concluded that no single clinical or laboratory finding is typical of F.A. Multidisciplinary approaches are essential to the diagnosis of Friedreich's ataxia.

Electrophysiological study of subjects occupationally exposed to lead and with low levels of lead poisoning.

The relationship between the length of exposure to lead, blood lead level (PbB), and peripheral nerve damage in a population occupationally exposed to low lead levels was evaluated. Sixty-two foundry workers were studied whose length of exposure ranged from 5 months to 10 a; their mean PbB in the last 2 a had not exceeded 50 microgram/100 ml (2.4 mumol/l). The referents were 27 hospital employees, of comparable ages, not occupationally exposed to lead. The electrophysiological examination consisted of the determination of the motor conduction velocity (MCV) of the median, and peroneal nerves, sensory conduction velocity (SCV) of the median and sural nerves, and the latency of the T and H reflexes. The mean MCV, SCV and the amplitude of the sensory action potential (SAP) of the median nerve were significantly lower in the group of exposed workers than in the reference group. When the exposed subjects were divided into three groups according to length of exposure, no difference was observed between the three groups. When they were divided into different groups according to the maximum PbB (max PbB) value, the observed differences with respect to the reference groups were already present in those with a max PbB of less than 50 microgram/100 ml (less than 2.4 mumol/l), but were more evident in the subjects with a max PbB of greater than 70 microgram/100 ml (greater than 3.4 mumol/l). Within the group of subjects with a max PbB of 50-69 microgram/100 ml (2.4-3.3 mumol/l), the differences with respect to the reference values were more evident for subjects with a short length of exposure (less than 2 a). The MCV of the median nerve correlated with the time since the PbB exceeded 50 microgram/100 ml (2.4 mumol/l). In conclusion, the electrophysiological changes observed in lead-exposed subjects do not seem correlated with the length of exposure. Such alterations seem more evident in those subjects whose PbB has been greater than 70 microgram/100 ml (3.4 mumol/l) at least once during the preceding 2 a and in those subjects who, at the time of the neurophysiological examination, have a PbB greater than 50 microgram/100 ml (2.4 mumol/l). The apparently greater sensitivity to lead in new employees, compared to workers with a longer exposure time, remains to be clarified.

Relationship between clinical and electrophysiological findings and indicators of heavy exposure to 2,3,7,8-tetrachlorodibenzo-dioxin.

In this study the prevalence rate of peripheral neuropathy in a population living in an area polluted with 2,3,7,8-tetrachlorodibenzo-dioxin (dioxin-TCDD) was determined. Of the 723 subjects invited to the first screening in 1977, 470 (65%) attended. At the second screening in 1978, of the 710 invited subjects, 319 (45%) attended. Prevalence rate ratios for peripheral neuropathy and the associated 95% confidence limits were calculated for subgroups determined by the presence of (i) predisposing factors to neuropathy (alcoholism, diabetes, occupational exposure to neurotoxic agents, etc) or (ii) conditions thought to result from exposure to dioxin-TCDD such as chloracne or abnormal serum hepatic enzyme levels. The prevalence rate of peripheral neuropathy among those subjects with predisposing factors and among those with chloracne or abnormal serum hepatic enzyme levels was nearly three times greater than among those without these manifestations. The results derived from this study may be useful qualitative pointers for identifying subjects at risk in the neurological follow-up.

Immunohistochemical evaluation of a panel of monoclonal antibodies for the diagnosis of small cell lung cancer.

Monoclonal antibodies (mAbs) with a high sensitivity and specificity for small cell lung cancer (SCLC) may be of potential diagnostic and therapeutic use. We selected five different mAbs generated against SCLC cell lines and tested them on paraffin-embedded SCLC and non SCLC (NSCLC) clinical samples in a retrospective study using immunohistochemical techniques. The results showed that the contemporary use of mAbs that react with different antigens allowed all the examined SCLC to be detected indicating that this panel of mAbs was extremely sensitive. Analysis of the reactivity of these mAbs with NSCLC showed that 4 of the 5 antibodies reacted also with a few NSCLC. Using higher dilutions of these mAbs their specificity improved substantially. The 5 different mAbs presented marked heterogeneity of antigenic expression within and between SCLC. This panel of antibodies may be very useful in the diagnosis of SCLC whereas their application, in vivo, in therapeutic trials, is limited by the heterogeneity of antigenic expression.

Cytologic detection of metastatic malignant melanoma in urine. A report of three cases.

BACKGROUND: The presence of melanoma cells in urinary specimens is a rare event in either primary or metastatic melanoma of the genitourinary tract. CASES: Melanoma cells were observed in urinary specimens from three white males aged 50, 67 and 51 years, with a previous history of cutaneous melanoma in the first two cases; in the last one autopsy showed a primary melanoma of the gallbladder. The first patient, treated with palliative therapy, survived 10 months, the second died a few days after the cytologic diagnosis, while the third survived 1 month. All patients had widespread metastases at the time of cytologic diagnosis. CONCLUSION: The presence of melanoma cells in urinary specimens may be an important marker for assessing the spread of metastatic disease. When the cytologic diagnosis is made, widespread dissemination is present, and only palliative treatment is suggested.

Solid and cystic tumor of the pancreas--case report.

Pancreatic tumors are rarely present in childhood. The authors present a case of papillary-cystic tumor in a 13-year-old girl, treated by partial pancreatoduodenectomy, with preservation of the pylorus. The histologic pattern was of a papillary cystic tumor without evident atypical nuclei. One year after operation, the girl is well without any finding of disease.

[ANCA-associated vasculitis]. / Vasculiti anca-associate.

ANCA-associated vasculitis. The term "antineutrophil cytoplasm antibody (ANCA)- associated vasculitis" (AASV) ihighers generally used to include primary vasculitis syndromes in which circulating ANCA against proteinase 3 (PR3) and myeloperoxidase (MPO) are commonly found. AASV syndromes include Wegener's granulomatosis, microscopic polyangiitis, idiopathic pauci- immune necrotizing crescentic glomerulonephritis and Churg-Strauss syndrome (CSS). AASV syndromes share some general clinical-histological manifestations, such as rapidly progressive renal failure and focal necrotizing glomerulonephritis with extracapillary proliferation in the absence (or in the presence of modest) immunoglobulins deposits (pauci- immune). Untreated AASV follow a progressive course with a fatal outcome due to vital organ failure. The combination of cyclophosphamide and prednisone is now established as the treatment of choice for patients with AASV, but there is considerable debate over the duration of therapy and the best way to administer cyclophosphamide. Treatment of AASV can be divided into two phases: an induction of remission and a maintenance of remission phase. Patients with AASV and renal involvement (serum creatinine less than 500 ml/L or 5.6 mg/dl) should be treated with a combination of oral prednisone with gradual tapering and cyclophosphamide. Once remission is achieved, usually after 3-6 months, azathioprine should replace cyclophosphamide. It is not known for how long treatment should be continued but at least one year of treatment after remission is warranted. When serum creatinine is than 500 ml/L (5.6 mg/dl) and/or oliguria is present, the addition of methylprednisolone pulses and/or plasma exchange should be considered.

Recurrent pulmonary embolism secondary to popliteal vein aneurysm with intraluminal wall ulcer.

The popliteal vein is the most frequent site of venous aneurysm. Surgical treatment is indicated above a 2.5 cm diameter to prevent complications, notably deep venous thrombosis and pulmonary embolism (PE). Here we report a case of recurrent episodes of severe PE, leading to cardio-circulatory shock caused by a popliteal vein aneurysm (PVA) despite oral anticoagulant therapy. When surgical correction of the aneurysm was performed, we found an ulcerative lesion in the inner aspect of the vein that was acting as a 'thrombogenic focus' inside the aneurysm. An accurate inspection of the intimal wall is always important during surgery of PVA, particularly when tangential resection is performed.

The pseudotumor cerebri.

Pseudotumor cerebri is the most appropriate term yet devised for classifying clinical patterns marked by papillary edema, uni- or bilateral, without major clinical signs and with normally sized cerebral ventricles, in which clinical or radiological investigations have ruled out the presence of a space occupying lesion of infective process. Because pseudotumor cerebri is a syndrome and its etiologies are many and varied, it is still difficult to make a diagnosis other than by exclusion. However, the knowledge that has accumulated as a result of epidemiological, clinical, neuroradiological, and experimental research enables us to say that the explanation of pseudotumor cerebri lies, on the one hand, in a sluggishness of the intracranial venous circulation with a consequent increase in cerebral blood volume and slowing of cerebrospinal fluid absorption and, on the other, in the onset of cerebral edema, either hypo-osmolar or vasogenic. The differing extent to which these three components participate in the clinical pattern depends on the etiology, but the resulting syndrome is always the same, though varying in clinical expression and pathogenesis.