Biomedical subjects
Publications and source records attributed to O Kiekara.
Septic phlebitis of the portal vein, or pylephlebitis, is a rare but potentially severe complication of abdominal sepsis. The authors present a case of pylephlebitis after perforated retrocecal appendicitis in a child and discuss the etiology, presentation, diagnosis, and treatment of this disorder in the modern era.
Though appropriate glucocorticoid substitution therapy should abolish both cortisol deficiency and adrenal androgen excess in patients with 21-hydroxylase deficiency (21-OHD), the long-term outcome is not always satisfactory. There are several reports on low adult height in both male and female patients, and impaired fertility has been reported in females with 21-OHD. There are only few reports on gonadal function of adult male patients with 21 -OHD. In this study, we calculated the child rate of all the 29 diagnosed adult Finnish males with classical 21-OHD and compared it with the mean child rate of the whole Finnish male population with equal age distribution. Sixteen males with 21-OHD and their age-matched healthy controls were further examined in a cross-sectional study. Auxology and pituitary gonadal axis were examined in both patients and controls. Testicular ultrasonography of the patients was also performed. The mean child rate of the 29 males with 21-OHD was 0.07 which was significantly lower (p<0.001) than that in the Finnish male population of the same age (0.34). In the cross-sectional study, males with 21-OHD had serum testosterone, inhibin B, LH and FSH concentrations comparable to those of healthy controls and reference values. Serum DHEA-S concentrations were remarkably low, even in the undersubstituted males with 21-OHD (p<0.001, compared with the healthy controls). In the patient group, serum inhibin B concentration did not correlate with serum FSH concentration. Adrenal rest tumors of the testicles were found in two undersubstituted males with 21-OHD. In conclusion, our study suggests normal pituitary and gonadal function but reduced child rate in adult males with 21-OHD. This might be explained by suboptimal psychosocial adaptation to the chronic disease. However, the patients in this study were young and the final child rate may become essentially higher.
BACKGROUND: Ovulatory disorders and decreased fertility rates have been found in females with classical 21-hydroxylase deficiency (21-OHD). We analyzed the pregnancies of 29 females with classical 21-hydroxylase deficiency and examined 16 of these women in a cross-sectional study. METHODS: The child rate of the 29 patients was compared with that in the general Finnish female population with equal age distribution.The cross-sectional study of the 16 patients included a standardized questionnaire, clinical examination, ultrasonography of the internal genitalia, basal measurements of serum steroids, sex hormone binding globulin, PRL, gonadotropins and plasma ACTH. RESULTS: The child rate was significantly lower than that in the general population (0.34 vs 0.91; p<0.001). A total of 13 pregnancies could be registered. From these pregnancies, ten healthy children were born, all for females with a simple virilizing disease. None of the nine females with salt wasting 21-OHD had any children or had sought treatment for infertility. In the cross-sectional study, females with regular menses (5/16; 31%) had well controlled adrenal androgen secretion and they had normal serum progesterone concentrations whereas the females with irregular menses (11/16; 69%) were often undersubstituted and presented with high serum progesterone concentration in the follicular phase. Polycystic ovaries could not be detected in any of these patients. CONCLUSIONS: Females with simple virilizing 21-OHD often have irregular menses but their final prognosis for fertility seems to be better than previously reported. On the contrary, our study confirms the extremely low child rate in the salt wasting group.
The aim of the present study was to assess the diagnostic accuracy and clinical utility of the first anteroposterior chest radiograph taken within the first hours of life from 99 neonates admitted consecutively from the delivery room to the neonatal intensive care unit because of mild to moderate respiratory distress. Before the radiograph was taken, all necessary emergency life support measures were undertaken. These included intubation, if indicated, and placement of intravenous and intra-arterial cannulae and nasogastric tubes. Intratracheal surfactant was never, however, administered before the radiograph. We examined the agreement between the clinicians' and the pediatric radiologist's opinion regarding the pulmonary and cardiac findings in these radiographs. Seventy-four radiographs exhibited definite pulmonary or cardiac pathology. In 66 patients the initial assessment was in agreement with the radiologic reevaluation. Clinicians' and radiologist's diagnoses of respiratory distress syndrome (RDS) agreed in 95%, of pneumothoraces in 78%, but, ultimately, of transient tachypnea of the neonate (TTN) in only 48%. The benefits of having the chest radiographs reviewed by a consultant radiologist experienced in neonatology are evident. The first chest radiograph taken early during the course of the disease had the greatest impact in the care of neonates with mild respiratory distress. In such cases, potentially dangerous conditions (e.g., pneumothoraces) could be detected before severe clinical deterioration occurred.
During 12 months in 1981-82, 201 children were hospitalized due to radiologically verified definite or probable pneumonia. In 1985, 194 chest radiographs (anteroposterior views) were re-evaluated jointly by two radiologists, and classified into three categories: alveolar, interstitial and probable pneumonia. In 127 cases definite pneumonia was diagnosed on both occasions, alveolar in 48 cases and interstitial in 79 cases. Variation between the two evaluations 3 years apart was observed in 46 (24%) of the 194 cases; the adjusted kappa (0.47) was in the modest region. Factors contributing to this variation were young age, less than 12 months, and the presence of interstitial infiltration, bronchial obstruction and low C-reactive protein. Factors associated with less marked variation were the presence of alveolar infiltration, auscultatory fine rates and elevated C-reactive protein. The microbial aetiology of infection, assessed by viral and bacterial antigen and antibody assays, showed no association with diagnostic variation. A lateral view of the chest radiograph was obtained from 158 patients; it was positive in 99 (91%) of the 109 cases with definite pneumonia. In only three cases the diagnosis was based on the lateral view alone. Our results show that the radiological diagnosis of pneumonia is difficult in children, especially in young children with interstitial pneumonia.
Sixty-one children were treated in hospital from 1981 to 1982 because of both radiologically and microbiologically verified viral or bacterial pneumonia. The chest radiographs were interpreted by two radiologists, not familiar with the clinical data, on two occasions three years apart, and only those patients with a definite alveolar (n = 27) or interstitial (n = 34) pneumonia at both evaluations were included in the present analysis. In addition, all patients had viral (n = 20), mixed viral-bacterial (n = 21) or bacterial (n = 20) infections diagnosed by viral or bacterial antibody or antigen assays. Viral infection alone was seen in 7 (26%), mixed viral-bacterial infection in 8 (30%) and bacterial infection alone in 12 (44%) of the 27 patients with alveolar pneumonia. The respective figures were 13 (38%), 13 (38%) and 8 (24%) for the 34 patients with interstitial pneumonia. C-reactive protein concentration was greater than 40 mg/l (a screening limit for viral and bacterial infections) in 15 (56%) of the patients with alveolar and in 11 (32%) of the patients with interstitial pneumonia. Thus 74% of the patients with alveolar and 62% with interstitial pneumonia had bacterial infection, either alone or as a mixed viral-bacterial infection. Our results suggest that the presence of an alveolar infiltrate in a chest radiograph is a specific but insensitive indicator of bacterial pneumonia. We conclude that patients with alveolar pneumonia should be treated with antibiotics. In patients with interstitial pneumonia, however, both viral and bacterial aetiology are possible. In those, the decision concerning antibiotic treatment should be based on clinical and laboratory findings.
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We examined 72 children aged 6-9 years to assess the effects of prematurity and ventilator treatment on subsequent lung function and respiratory morbidity. The preterm study group (n = 42) was divided into children with a history of bronchopulmonary dysplasia (BPD) (n = 10), children who had had neonatal respiratory treatment but no BPD (n = 19), and children without severe neonatal respiratory problems (n = 13). The BPD children as a group had markedly lower specific airway conductance and larger residual volume than did the full-term control group, but there were no significant differences in spirometric measurements. The BPD group also had higher respiratory morbidity requiring hospitalizations, particularly during the first 2 years of life, than did the children in the other study groups. Therefore, BPD may improve but does not disappear as age increases. Non-BPD children who had had ventilator treatment as neonates recovered well and suffered no severe respiratory problems after infancy. Pulmonary function parameters in prematurely born children without neonatal ventilator treatment did not differ from those in the full-term control group. Thus, neonatal lung disease seems to be a more important determinant of abnormal pulmonary function at a later age than is prematurity alone.
Photofluorography using a large-field image intensifier (Siemens Optilux 57) was applied to scoliosis radiography and compared with a full-size rare-earth screen/film technique. When scoliosis radiography (PA-projection) was performed on 25 adolescent patients, the photofluorographs were found to be of comparable diagnostic quality with full-size films. A close correspondence between the imaging techniques was found in the Cobb angle measurements as well as in the grading of rotation with the pedicle method. The use of photofluorography results in a radiation dose reduction of about one-half and considerable savings in direct imaging costs and archive space. In our opinion the method is particularly well-suited for follow-up and screening evaluation of scoliosis, but in tall patients the image field size of 40 x 40 cm restricts its usefulness as initial examination.
The occurrence, predictive factors and associated morbidity of bronchopulmonary dysplasia (BPD) was examined in a preterm birth cohort of 712 children, born before 37 weeks of gestation to residents of a geographically defined area between 1978-82. All cases of BPD (N = 16) were born at or before 32 weeks of gestation. The incidence of BPD, based on status at the age of 28 days, was 1 per 1000 live births, but 135 per 1000 live preterms born at or before 32 weeks. Most cases of BPD developed following respiratory distress syndrome (RDS), only one case developing after minimal respiratory symptoms was observed. BPD infants had higher neonatal morbidity, even when compared with preterms of equal gestational maturity, but only a few variables had predictive value with respect to the future development of BPD. Radiologic grading of RDS and associated early cardiologic signs did not increase their predictivity regarding the subsequent development of BPD. Two (12.5%) of the 16 BPD infants died postneonatally. The unfavorable effects of BPD on the health status of preterm infants extended far beyond the neonatal period. The BPD group, which consisted only of 18% of neonatal survivors born at less than or equal to 32 weeks, consumed 53% of all hospital days used by these preterms during the first two years of life. In particular, BPD survivors had markedly more respiratory infections (63%), more neurologic sequelae (37%) and more cases of retrolental fibroplasia (12%) than their non-BPD counterparts.
Angiographically determined reduction of vascular lumen was correlated with lower-limb systolic pressure ratios in a series of 62 patients with intermittent claudication or suspected aneurysmal or vasospastic disease of the lower limbs. Statistically highly significant, though not particularly close, correlations were observed between the angiographic findings and the thigh-arm, calf-arm and ankle-arm pressure ratios (r = -0.732, -0.711 and -0.731, respectively). A mathematical model of the type employed in this study seems to be useful for correlating angiographic findings with systolic pressures in the lower limbs.
Postnatal regression of prenatally or neonatally detected multicystic dysplastic kidney disease has been widely documented. However, renal cysts can regress during gestation, although they usually become larger in utero. We present a case of prenatally detected multicystic dysplastic kidney with an atypical course. During the third trimester, unilateral multicystic renal lesions in the fetus first enlarged and later involuted; by the second postpartum year, the kidney had become hypoplastic and nonfunctional. Our case also shows that before birth, blood flow in the affected kidney, measured with Doppler imaging, was normal until the cysts involuted.