A true hermaphrodite with some unusual features

A fifteen-month-old boy with ambiguous external genitalia was found to have a 46XX karyotype, an ovotestis in the right labioscrotal fold, and an absent left gonad. He also had a rudimentary uterus and fallopian tubes and a blind-ending vagina lined with squamous epithelium. These features are compared with those more commonly found in true hermaphroditism.     

The glycosaminoglycans of the gubernaculum during testicular descent in the fetus

Tissues were obtained from 387 male pig fetuses ranging from 60 to 120 days of gestation. The relative wet mass and water content of the gubernaculum increased during and decreased after the period of testicular descent. The extracellular glycosaminoglycans (GAG) were assayed to determine whether these polyanionic macromolecules are responsible for the increased water content of the gubernaculum. The total GAG/wet tissue mass in the gubernaculum decreased during and increased after descent, while the total GAG/dry mass decreased during and after descent, indicating an accumulation of water during descent, with a loss of water and an increase in less hydrated tissue components after descent. The major GAG fraction in the gubernaculum was dermatan sulfate, but the percentage hyaluronate in the gubernaculum was two times higher than in striated muscle or umbilical cord, indicating that this GAG fraction may be responsible for the increased water content of the gubernaculum, which probably serves to dilate the inguinal canal and scrotum, thus facilitating descent.     

Exstrophy of the testis

Among the anomalies of testicular descent exstrophy of the testis appears to be the most rare. We describe a full-term male newborn with the right testis and spermatic cord prolapsed from a skin defect at the neck of the scrotum. The cause of the anomaly is unknown.     

Three-dimensional computed tomographic imaging of the pelvicaliceal system: analysis of factors influencing image quality.

We analyzed 3-dimensional (3D) computed tomographic images of the pelvicaliceal system obtained in 25 patients after opacification of the collecting system by retrograde ureteric or intravenous (IV) infusion of sodium iothalamate. 3D images were reconstructed using the data from nonoverlapping 5-mm sections taken on an Elscint 2400 scanner. Factors contributing to poor image quality were: artifacts caused by contrast in the collecting tubules, underfilling or overdistension of the pelvicaliceal system with contrast, movement of the patient and severe hydronephrosis. Satisfactory 3D images of the pelvicaliceal system can be obtained by retrograde ureteric infusion of 5% w/v sodium iothalamate at 20 cm H2O, or by IV injection of 50 ml of 70% w/v sodium iothalamate. Abdominal compression should be applied 10-15 min after the IV injection of contrast, and the computed tomographic examination should commence 20-30 min after the injection. It is imperative that the patient lie completely still and breathe normally to avoid movement of the kidneys. 3D computed tomographic imaging of the pelvicaliceal system may become a valuable adjunct to the preoperative planning and performance of percutaneous nephrolithotomy.     

Hyperplasia and hypertrophy of the gubernaculum during testicular descent in the fetus

The role of the gubernaculum during testicular descent was investigated in 115 male pig fetuses ranging from 68 to 109 days of gestation. Descent of the testis through the inguinal canal occurred between 77 and 94 days. There was a marked increase in the total and relative wet mass as well as the wet/dry mass ratio of the gubernaculum, denoting an increase in the water content of this structure during descent of the testis. Similar changes were not seen in other fetal tissues. Asymmetrical descent of the testes occurred in seven of the 40 fetuses between 81 and 92 days of gestation. Descended testes had gubernacula with a greater relative wet mass than the gubernacula of undescended testes. These findings indicate that the increase in mass of the gubernaculum plays an important part in testicular descent. An increase in both the DNA content (hyperplasia) and the RNA/DNA ratio (hypertrophy) was noted in the gubernaculum during descent of the testis. Hyperplasia in the gubernaculum occurred at a rapid rate initially and ceased after the completion of descent. Hyperplasia was also seen in the testis and epididymis, but occurred at a slower rate initially, with a rapid acceleration after the completion of descent. Cellular hypertrophy was not noted in the testis and epididymis, but did occur in umbilical cord tissue. If gonadotropins acting via androgen secretion by the testis provide the stimulus for these changes, the observed difference in response between the gubernaculum and testis could be due to a difference in end-organ sensitivity. However, it is also possible that some unidentified non-androgenic gubernaculotropin, possibly secreted by the fetal testis, provides the stimulus for gubernacular growth.     

Platelet aggregation in chronic idiopathic thrombocytopenic purpura.

A method for washing platelets by albumin density gradient separation has been modified to prepare platelet rich plasma of thrombocytopenic patients for platelet aggregation studies. The concentration procedure, consisting of centrifuging platelets into a specific gravity gradient between plasma and 40-45% aqueous solution of bovine albumin, does not affect platelet aggregation adversely. Platelet aggregation in eight patients with chronic idiopathic thrombocytopenic purpura was determined by this method. On the basis of the results the patients could clearly be divided into two groups: four patients with normal aggregation and four with a qualitative platelet defect. In contrast to the other patients, the group with an in vitro platelet functional defect all had more prolonged bleeding times and the presence of a serum antiplatelet antibody.     

Evaluation of mathematic models to assess platelet kinetics

Twelve mathematic methods used to calculate the mean platelet survival time were compared by determining the "goodness of fit" of the models to the platelet survival curves of 15 reference subjects and 54 patients. Platelets were labeled with [111In]oxine. The linear (LN), exponential, weighted mean, multiple hit (MH), Dornhorst (DH), Meuleman (ML), alpha order (AO), and polynomial (PO) mathematic models were investigated. The goodness of fit for the exponential model was determined by the nonlinear least squares method (EP), and also by the linear least squares method on logarithmically transformed data (EX) as is recommended. The modified weighted mean (MWM) and the usual weighted mean method (WM) obtained with these exponential models were tested. The Dornhorst (DH10) and Meuleman (ML10) models, where the potential age-dependent platelet survival times were kept constant at 10 days, were also evaluated. The goodness of fit results, expressed as % s.d. indicated that the LN (5.2%), EX (5.0%), EP (4.4%), WM (3.7%), DH10 (3.7%), and ML10 (3.7%) models all fitted the data significantly worse than the MWM, MH, DH, ML, AO, and PO models (range 3.2-3.3%). The mean platelet survival time determined with the MH model differed significantly from the results with the DH, ML, and AO models. The results of mean platelet survival time calculated with different mathematic models cannot, therefore, be compared directly. The models that fitted the platelet survival curve well varied slightly in sensitivity to noise as is indicated by the coefficient of variation of the mean platelet survival time estimates for the reference subjects (range 7.9-12.0%). Fitting data to at least two mathematic models has definite advantages. Data on which the calculations are based are probably invalid if the following are true: (a) if the mean platelet survival time estimated with the alpha order model is shorter than that estimated with the EP, MWM, or MH models, or (b) the mean platelet survival time estimated with either the DH, ML, AO, or PO models, is longer than the LN, MWM, or MH estimate of the mean platelet survival time. We conclude that the mean platelet survival time can be reliably estimated by fitting the data to either the MWM method (if limited computing facilities are available) or the MH model. Confidence in the result will be increased if considered in conjunction with the finding obtained with one other model; in those cases where the platelet survival time is very short, the alpha order model is recommended.(ABSTRACT TRUNCATED AT 400 WORDS)