Hemoglobinopathies in the Hamilton region. I. A 4-year survey.

A regional laboratory for the diagnosis and investigation of hemoglobinopathies was established by the Hamilton District Program in Laboratory Medicine in October 1970. Specimens from patients suspected of having a hemoglobinopathy were referred to the regional laboratory from all the hospitals participating in the program. Between October 1970 and October 1974, 3547 specimens were screened for an abnormal hemoglobin and thalassemia; 758 cases of thalassemia, 165 cases of abnormal hemoglobin and 14 mixed cases were diagnosed. Before 1970, 110 cases of thalassemia and 12 cases of abnormal hemoglobin were on record in the Hamilton region. Regionalization of laboratory services provides a more effective means of screening for abnormalities in hemoglobin structure and synthesis and facilitates the opportunity for improving diagnostic procedures.     

V.A.T.E.R. association and its limits

Comparative investigation of 92 cases of V.A.T.E.R. syndrome (4 personal cases) and 62 cases of caudal regression (Duhamel syndrome) (2 personal cases) are performed. There is much analogy between these two entities. Initial impairment would be an early dysfunction of mesoderm setting up located on esophagus in V.A.T.E.R. syndrome and on kidneys in Duhamel syndrome. Etiopathogenic factors remain unknown. Genetic counseling is good. Detection of only one mesodermal malformation leads to inquire other unnoticed anomalies (kidneys, heart, spine, alimentary duct).     

Origin of the v. portae and variability of its tributaries in laboratory animals. II. The guinea pig (Cavia aperea f. porcellus)

The authors investigated the origin of the v. portae and variability of its main tributaries in 30 adult guinea pigs (Cavia aperea f. porcellus). The venous bed was visualized by means of blue-dyed latex. The v. portae was formed the most frequently - in 17 cases (56.7%) - by the confluence of three veins - the v. mesenterica cranialis, the v. lienalis and the v. gastroepiploica dextra; less often it was formed from four or five tributaries and in one case only it was formed by union of the v. mesenterica cranialis and the v. lienalis. The v. gastrica sinistra was a tributary of the v. lienalis in 24 cases (80.0%). It arose on the ventral and the dorsal surface of the stomach, in the region adjacent to the curvatura minor ventriculi. In 21 cases (70.0%) it was joined by an independent v. cardiaca. The v. lienalis originated in the hilus lienis, usually as a result of the union of two venous trunks. Inter-organ anastomoses were found in 26 cases (86.7%); in 10 cases (33.3%) they occurred simultaneously between the spleen and the stomach and between the spleen and the pancreas, while in 16 cases (53.3%) they connected the superior pole of the spleen with the stomach. An independent v. gastroepiploica dextra was present in 19 cases (63.3%) as a tributary of the v. portae. In 22 cases it was joined by the v. pylorica. A v. pancreaticoduodenalis cranialis was present in 27 cases (90.0%). In 18 cases (60.0%) it united with the v. gastroepiploica dextra to form a common trunk, the v. gastroduodenalis, which joined the v. portae; in two cases (6.7%) it was an independent tributary of the v. portae. In six cases it was connected with the v. lienalis and in three cases (10.0%) it was replaced by a few vv. pancreaticae emptying into the v. lienalis. A typical v. pancreaticoduodenalis caudalis, as a tributary of the a. mesenterica cranialis, was found in 19 cases (63.3%). In two cases, together with the v. pancreaticoduodenalis cranialis, it formed a single trunk (the v. pancreaticoduodenalis communis). A comparison of the v. portae and its tributaries in the guinea pig and the cat has so far not brought to light any significant morphological differences relative to their different zoological classification and their different modes of life.     

Solitary nodular goiter. Review of cytomorphologic features in 441 cases.

OBJECTIVE: To study the cytomorphologic features of solitary nodular goiters (SNG). STUDY DESIGN: May-Grünwald-Giemsa-stained smears in 441 SNG diagnosed by ultrasonography and fine needle aspiration (FNA) and found to have optimum cellular material at review were subjected to detailed cytologic assessment. The age of the patients ranged from 11 to 75 years, with a median of 35. Male: female ratio was 69:372. The parameters for cytologic assessment included cellularity, colloid content, acinar formation, papillary formation, intranuclear cytoplasmic inclusions, nuclear grooves, marginal vacuoles, Hürthle cells and various inflammatory cells. Histopathology reports on thyroidectomy specimens were available in 27 cases from two Delhi hospitals. RESULTS: Hyperplastic nodules (68 cases) differed significantly from colloid goiters (269 cases) by having more cases with excessive cellularity, acinar formation and marginal vacuoles (P < .001). There was also a significant difference with respect to papillary formation and moderate-to-excessive colloid content (P < .001). As compared to hyperplastic nodules, neoplasms (60 cases) had a significantly higher number of cases with papillary formation, intranuclear inclusions and nuclear grooves but lower number of cases with marginal vacuoles (P < .01-.001). Among neoplasms, usual papillary carcinoma (19 cases) differed from follicular neoplasms (20 cases) with respect to acinar formation, papillary formation and nuclear grooves (P < .001). A significant difference was also observed with respect to colloid content and nuclear inclusions. Follicular variant of papillary carcinomas (FVPC) (10 cases) emerged as a distinct cytologic entity following review and differed from usual papillary carcinomas in having a higher number of cases with acinar formation, tubular formation and marginal vacuoles (P < .01-.001) and lower number of cases with nuclear grooves (P = .05). FVPC also differed from follicular neoplasms with respect to papillary formation, tubular formation, intranuclear inclusions and nuclear grooves (P < .01-.001). Overall cytohistologic agreement was achieved in 24 of 27 (88.9%) cases. CONCLUSION: Detailed cytologic assessment of FNA smears-in SNG was helpful in highlighting parameters that differentiate between various types of goiters.     

Origin of the v. portae and variability of its tributaries in laboratory animals. III. The laboratory rat (Rattus norvegicus var. alba)

The authors studied the origin of the v. portae and the variability of its tributaries in 30 adult laboratory rats (Rattus norvegicus v. alba) after injecting blue-dyed latex into the venous bed. In 21 cases (70.0%) the v. portae was formed from three tributaries. In 18 of these cases (60.0%) they were the v. mesenterica cranialis, v. lienalis and v. pancreaticoduodenalis cranialis. In eight cases the v. portae was formed from four or five tributaries and in only one case was it formed from two--the v. mesenterica cranialis and v. lienalis. In 21 cases (70.0%) the v. gastrica sinistra was a tributary of the v. lienalis. It collected blood from the anterior and posterior surface of the stomach and sometimes originated as far away as the curvatura major ventriculi. A v. cardiaca was present in all 30 cases (100%). As a rule (in 28 cases, i.e. 93.3%), it was a tributary of the v. gastrica sinistra. There was likewise a v. pylorica in every case; most frequently this united with the v. pancreaticoduodenalis cranialis, but was also connected with other veins in the vicinity. A v. pancreaticoduodenalis cranialis was always present. In 22 cases (73.3%) it was an independent tributary of the v. portae. In 11 cases (36.7%), there were no vv. gastroepiploicae or equivalent vessels and blood from the stomach was drained mainly by the v. gastrica sinistra. A v. gastroepiploica dextra was present in 11 cases (36.7%), mostly as a tributary of the v. lienalis; in two cases (6.7%) there was a v. gastroepiploica sinistra and in six cases (20%) the place of these veins was taken by vv. gastricae as tributaries of the v. lienalis. In 21 cases (70.0%) the v. lienalis was joined by the v. gastrica sinistra. In 22 cases (73.3%) inter-organ anastomoses between the spleen, stomach and pancreas were found. A v. pancreaticoduodenalis caudalis was always present and in 20 cases (66.7%) it was an independent tributary of the v. mesenterica cranialis. A comparison of the origin of the v. portae and the organization and variability of its tributaries in the rat, cat and guinea pig shows that, in agreement with their position in the zoological system and their mode of life, there was greater similarity between the rat and the guinea pig as regards practically all the veins examined.     

Q. & A. on C. P. S

Encephalitis is a challenging public health problem in Kern County and in the San Joaquin Valley area. During the last ten years the authors have studied the epidemiology of encephalitides due to arthropod-borne viruses and the methods used in the differential dignosis of these conditions. To incriminate a virus it is necessary to demonstrate a rise in antibody titer in the blood over a period of seven to fourteen days, the first specimen to be taken as soon as possible after onset of symptoms. A variety of tests may be necessary in some cases. Among the patients included in this presentation the greater number of those with encephalitis and also of those with poliomyelitis were under 30 years of age. The age range was five weeks to 49 years. The median annual mortality rate was 4.3 deaths per 100 cases for poliomyelitis and 3.9 for encephalitis. The greatest incidence occurred during the summer months.The clinical manifestations usually observed in encephalitis are described, but it is emphasized that wide variations and even completely atypical clinical phenomena are encountered. Four representative cases are reported and treatment is discussed. Vaccination and vector control are considered as the most promising means of combating the disease.     

Fine needle aspiration cytology diagnosis of tuberculous thyroiditis. A report of eight cases.

Among 1,283 cases of thyroid lesions subjected to fine needle aspiration cytology (FNAC) over a period of two years, 8 cases (0.6%) were found to be having cytologic features consistent with tuberculous thyroiditis. The ages of the patients ranged from 14 to 65 years, with a median of 30. The male:female ratio was 4:4. Six cases clinically presented with solitary nodules of the thyroid and two cases as abscesses in the thyroid region. Three patients had concomitant cervical lymphadenopathy, and only two patients were known cases of tuberculosis on treatment. Ultrasonography, done in seven cases, confirmed solitary nodules in four; in one case the differentiation between an extrathyroid nodule and cystic isthmic nodule was difficult, and in the remaining two cases the lesions were found to be extrathyroid. Fine needle aspirates from thyroid swellings showed epithelioid granuloma with necrosis in five cases and necrosis without epithelioid granuloma in three cases. The number of cases positive for acid-fast bacilli in these two groups were two and three, respectively. Lymph node aspiration, done in three cases, revealed necrotic material in two; both were positive for acid-fast bacilli, and the third case showed epithelioid granuloma without necrosis.     

Pulmonary fine needle aspiration biopsy. Assessing the negative diagnosis.

OBJECTIVE: To assess the significance of the "negative for malignancy" category when applied to pulmonary transthoracic fine needle aspiration biopsy (FNAB). STUDY DESIGN: Transthoracic lung FNABs diagnosed as "negative for malignancy" were identified from the files of Barnes-Jewish Hospital's South and North Campus over a period of five and nine years, respectively. Histologic correlation and clinical follow-up were obtained. RESULTS: Of the 1,181 lung FNABs performed during the study period, 108 cases (9%) had a negative cytologic diagnosis. Histologic correlation was available in 46 cases (43%), of which 23 cases had benign histologic findings, and 19 cases were malignant. Thirty-five of the 62 cases without histologic correlation had clinical follow-up consistent with a benign process. CONCLUSION: Based on the histologic correlation and clinical data available, the negative predictive value was 77%. Inadequate sampling was responsible for all false negative cytologic diagnoses in this series.     

Climatological correlates of screwworm (Cochliomyia hominivorax) abundance in Texas, U.S.A.

The causes of screwworm (Cochliomyia hominivorax) outbreaks in North America are not well understood, but the literature suggests that climate was historically important. Screwworm case incidence in each of seven climatological divisions of Texas was examined for the years 1962-83, the period when sterile-male releases were made. Weak but statistically significant correlations were found between winter and summer cases and mean winter and summer rainfall and temperature when the independent variables were examined one at a time. Multiple regression of log case incidence on previous quarterly cases and current rainfall and temperatures showed a significant, negative effect of temperature on summer cases. Lagged screwworm cases accounted for most of the variation in quarterly cases. No climatic effects were detected in the other seasons. Rainfall was statistically unrelated to screwworm abundance in any season even in an arid region. The analysis does not support a climatic genesis of screwworm outbreaks or eradication. The sterile-male method is a credible explanation for screwworm disappearance.     

Neonatal pneumopericardium.

Summary: Pneumopericardium developed in three newborn infants, including a set of twins, with respiratory distress syndrome. The rarity of this condition and its occurrence in two newborns suggest an anatomic predisposition, especially in premature infants requiring assisted ventilation. Two of the infants died; one had undergone pericardiocentesis. From a review of the literature and from our cases we conclude that conservative therapy appears warranted in cases of isolated pneumopericardium although the number of cases reported is too small to provide a definite answer.     

Tuberculous pleurisy.

A review of the records for all cases of tuberculous pleurisy notified in Canada from 1970 through 1974 and in British Columbia from 1967 through 1976 showed that in the periods studied the annual incidence of this condition was low, just under nine cases per million population, and was declining. The disease commonly occurred a few months after a primary infection with tubercle bacilli. Bacteriologic confirmation of the diagnosis was possible in only 40% of the cases since biopsy specimens were not consistently cultured.     

The positive pleural effusion. A retrospective study of cytopathologic diagnoses with autopsy confirmation.

We performed an investigation focusing on the distribution of tumor types responsible for positive pleural effusions in 143 patients who died of malignancy and underwent autopsy. The principal malignant tumors were lung carcinoma (41 cases, 51.2%) and pleural mesothelioma (23 cases, 28.7%) in males and breast carcinoma (24 cases, 38.2%) and lung carcinoma (13 cases, 20.6%) in females. Histologically, most of the cases belonged to the adenocarcinoma category. The first morphologic diagnosis was a cytologic one in 86 cases (60.1%), especially regarding lung cancer. In breast cancer a positive pleural effusion always preceded recurrent disease with a rapidly progressive course, even a long time after the initial surgery. The results of this study, based on both cytomorphologic features and postmortem data on the tumor sites, may be a useful working framework for the cytologist dealing with a positive pleural effusion.     

Image analysis of mesothelioma. I. differentiation of mesothelioma from adenocarcinoma of the lung

OBJECTIVE: To explore the usefulness of nuclear micromorphometric analysis for the differentiation between epithelial mesothelioma and metastatic adenocarcinoma in the chest wall. STUDY DESIGN: High-resolution images of 2,100 nuclei from 27 cases of epithelial mesothelioma and 15 cases of adenocarcinoma of the lung were recorded. Stepwise discriminant analysis and a nonparametric classifier were applied to derive estimates for a case diagnosis correct classification rate. RESULTS: Nuclei from epithelial mesothelioma and adenocarcinoma of the lung showed statistically significantly different properties, but there was a region of overlap in feature space such that approximately 15-20% of cases could not be correctly classified. The lesion signatures derived from the mesothelioma cases with discriminant function scores that might result in case misclassification and the cases of adenocarcinoma of the lung spanned a similar range of degree of nuclear abnormality. However, the distribution of nuclear abnormality values for the mesothelioma cases has a mode at 0.87 SD from normal, whereas the distribution seen in lung adenocarcinoma cases had a mode at about 3.7 SD. CONCLUSION: Cases of epithelial mesothelioma and adenocarcinoma of the lung have nuclei with a wide range of deviation from normal in the spatial and statistical distribution of their nuclear chromatin. For approximately 80% of cases, correct case classification can be provided by nuclear micromorphometric analysis. Cases of epithelial mesothelioma with highly abnormal nuclei overlap in feature space with nuclei from adenocarcinoma of the lung. However, it is possible that characterization by a lesion signature may allow correct assignment for those cases.     

The comedo subtype of intraductal carcinoma. Cytologic characteristics.

Smears from 10 intraductal carcinomas of the comedo type without microinfiltration were compared with smears from 10 similar tumors with suspected or proven microinfiltration and smears from 10 invasive comedo carcinomas. Microinvasive tumors could not be separated from purely intraductal cases unless tumor cell infiltration in mammary fat was seen in the smear. The tumor cells in most of the intraductal cases were shed in cohesive groups and clusters, lying in necrotic cellular debris and with few or no scattered, single, dissociated tumor cells. Smears from invasive comedo carcinomas invariably showed tumor cell clusters and scattered, single, dissociated tumor cells, often with atypia in excess of what was seen in the intraductal cases. Also, in most cases the invasive tumors did not show a background of necrotic cellular debris.     

Gonorrhea in homosexual men.

Extragenital gonorrhea was seen in 65% of 54 cases of gonorrhea in 43 homosexual men attending a venereal diseases clinic between 1974 and 1977; in 21 cases the infection was extragenital only. This type of gonorrhea was often asymptomatic and was associated with a high rate of failure of initial treatment in 6 of the 50 cases in which the patient returned for follow-up assessment, and in 5 of the 6 the persistent infection was extragenital. Syphillis was seen concomitantly or had previously occurred in 6 of the 54 cases. Fifteen of 28 cases of primary, secondary or early latent syphilis seen in men during the same study period had occurred in homosexuals. Appropriate testing for extragenital gonorrhea and for syphilis is important in homosexual men who present for examination, and homosexuality with the possibility of extragenital gonorrhea should be considered in a man with syphilis of recent onset.     

General practice observed. Child abuse and general practice.

In a general practice of 9250 patients with 1841 children under 10 there were 12 cases of actual abuse during 1973-6. In March 1976 30 children were at risk. A preventive scheme was set up and the short-term outcome was good. There were no cases of serious abuse among the children at risk.     

The Dyggve-Melchior-Clausen syndrome.

Two new cases of Dyggve-Melchior-Clausen syndrome are described; they belong to the fourth family from Lebanon in which this disease has been recognized. There is no genealogical linkage between these four families. A particular feature in these cases is a striking rhizomelic shortness of the arms especially in one case. Clinical and radiological findings, progression of the skeletal changes are studied, along with the review of the cases in the literature. Cytological and biochemical data indicate that the DMC syndrome is not a mucopolysaccharidosis.     

Penicillinase-producing Neisseria gonorrhoeae in California. Report of a major outbreak and control recommendations.

A major outbreak of penicillinase-producing Neisseria gonorrhoeae occurred in California beginning in late 1986. After a state public health emergency was declared in April 1987, a statewide, cooperative federal-state-local treatment and control program was implemented. It was successful in reducing the reported incidence by 59%. Despite the success of this effort, penicillinase-producing N gonorrhoeae is not controlled in California, and, while not nearly the magnitude reported in Florida, an increasing number of cases are again being reported in California. We present recommendations for clinicians and local public health officials, including the epidemiologic and empiric treatment of suspected gonorrhea cases with ceftriaxone when the incidence of penicillinase-producing gonococcal cases in a community exceeds 1% of all reported gonorrhea cases.     

Superior sagittal sinus thrombosis. Still a killer.

Following treating a case of superior sagittal sinus thrombosis, we did an extensive search of the literature, eliciting 795 cases of the disorder. An analysis showed that even after the introduction of antibiotics, the preponderance of these cases have been diagnosed at autopsy. Our findings raise questions about the current methods of diagnosis and management of superior sagittal sinus thrombosis: Can the correct diagnosis be made earlier? Does a distinction between partial and complete thrombosis call for a different management?     

Linkage analysis of chromosome 1q markers in 136 prostate cancer families. The Cancer Research Campaign/British Prostate Group U.K. Familial Prostate Cancer Study Collaborators.

Prostate cancer shows evidence of familial aggregation, particularly at young ages at diagnosis, but the inherited basis of familial prostate cancer is poorly understood. Smith et al. recently found evidence of linkage to markers on 1q, at a locus designated "HPC1," in 91 families with multiple cases of early-onset prostate cancer. Using both parametric and nonparametric methods, we attempted to confirm this finding, in 60 affected related pairs and in 76 families with three or more cases of prostate cancer, but we found no significant evidence of linkage. The estimated proportion of linked families, under a standard autosomal dominant model, was 4%, with an upper 95% confidence limit of 31%. We conclude that the HPC1 locus is responsible for only a minority of familial prostate cancer cases and that it is likely to be most important in families with at least four cases of the disease.