Follicular large cell lymphoma localized to the testis in children.

PURPOSE: Primary follicular lymphoma of the testis in childhood is rare with only 6 cases previously reported. We present 3 additional cases. MATERIALS AND METHODS: We extensively analyzed primary follicular lymphoma of the testis in 3 boys. Clinical data were obtained by reviewing patient charts. RESULTS: The patients were 4, 5 and 11 years old, respectively. Two patients presented with painless unilateral testicular enlargement and 1 presented with unilateral hydrocele. Laboratory findings were within normal limits in all patients. Radical orchiectomy was done in all cases. The excised testes were partially or completely replaced by tumor. In all cases the features were those of follicular, large cell-type malignant lymphoma. Tumor cells in all cases were CD20 and CDw75 positive, focally CD23 positive and bcl-2 negative, while in 2 they were CD10 positive and bcl-6 positive. Surface Ig was absent in the 2 cases studied. Karyotyping in 1 case showed a normal karyotype. Staging revealed no evidence of extratesticular disease. All patients underwent combination chemotherapy and were in complete remission 7 to 59 months after therapy. CONCLUSIONS: We present 3 cases of pediatric primary follicular lymphoma of the testis. Pathological findings and clinical features were similar to those in the 6 previously reported cases and suggest that primary pediatric testicular follicular lymphoma may represent unique subset of follicular lymphoma with a particularly good prognosis.

Grading nephroureteral dilatation detected in the first year of life: correlation with obstruction.

To understand better the significance of pediatric idiopathic nephroureteral dilatation the renal ultrasound images of patients less than 1 year old with hydronephrosis or hydroureteronephrosis were graded and compared to the radiological diagnosis of obstruction as determined by diuresis renography and/or urography. The study included 73 boys and 30 girls with hydronephrosis (76 patients) or hydroureteronephrosis (27). For hydronephrosis obstruction was diagnosed in 56 children (74%) and involved 61 of 97 kidneys (63%). For obstructed kidneys the mean grade of hydronephrosis (3.4 +/- 0.7 standard deviation) was statistically different from that of nonobstructed kidneys (1.6 +/- 0.8 standard deviation) (p less than 0.05). When the value to predict obstruction was set at grade 3 hydronephrosis or greater there was an 88% sensitivity and 95% specificity. For hydroureteronephrosis obstruction was diagnosed in 15 of 27 children (56%) and involved 17 of 34 kidneys (50%). The degree of dilatation was weighted as a score to assess the grades of hydronephrosis and ureteral dilatation, namely hydroureteronephrosis score equals grade of hydronephrosis plus grade of ureteral dilatation. In obstructed megaureters the mean hydroureteronephrosis score (5.8 +/- 1.0) was statistically different from that for nonobstructed megaureters (mean hydroureteronephrosis score 2.7 +/- 1.9) (p less than 0.001). When the value to predict obstruction was set at hydroureteronephrosis score of 5 or greater there was a 94% sensitivity and 80% specificity. Although ultrasound examination alone cannot be used to diagnose urinary obstruction, the radiological diagnosis of obstruction is linked with the grade of hydronephrosis or score of hydroureteronephrosis.

Favorable experience with pre-emptive renal transplantation in children.

We retrospectively examined our experience with live-related donor kidney transplants in 66 children during the 7-year period 1984-1990. We compared the clinical courses of 26 children who did not receive any dialytic therapy prior to transplantation with 40 children who were dialyzed (27 via peritoneal dialysis and 13 via hemodialysis). We did not find any statistically significant differences in patient or graft survival between these three groups with a follow-up period of 6-87 months. Based on our results, we conclude that pre-emptive transplantation is an acceptable treatment for children who will inevitably require renal replacement therapy.

Radiologic evaluation of foreign bodies.

The correct selection of a radiologic imaging modality along with knowledge of indirect radiologic findings can help determine the presence and location of a foreign body. Plain radiographs should be the initial screening modality for a suspected foreign body. Whereas most metal and glass foreign bodies are detectable on radiographs, many foreign bodies, including wood, are not. We do not advocate using xeroradiography for the detection of foreign bodies. When a suspected superficial foreign body is not delineated on radiographs, ultrasonography should be the next modality of choice. CT should be reserved for deep foreign bodies or when foreign bodies are not seen on radiographs or ultrasonography but are suspected.

Azoospermia: a complication of hydrocele repair in a fertile population.

Although minor complications have been reported, hydrocelectomy generally is considered to be a safe procedure. We recently treated 3 patients who became azoospermic after hydrocele repair. Of the patients 2 underwent bilateral and 1 unilateral hydrocelectomy. All 3 patients had iatrogenic injury to the vas deferens or epididymis after hydrocele repair.

Beemer-Langer syndrome with manifestations of an orofaciodigital syndrome.

We report on an infant girl with hydrops, macrocephaly, high forehead, flat face, hypertelorism, broad nasal bridge, median cleft lip and alveolar ridge, grooved palate, accessory frenula, small tongue, milia, severe rib and limb shortness, brachydactyly, talipes equinovarus, Dandy-Walker malformation, accessory spleen, unfixed mesentery, ectopic pancreas, and renal cysts. This patient resembles seven previously reported patients with the Beemer-Langer syndrome, a distinct lethal short rib syndrome characterized by hydrops, markedly short ribs and limbs, median cleft lip with or without cleft palate, flat face, and macrocephaly. Polydactyly is usually absent. Our patient's oral anomalies suggest an orofaciodigital syndrome, but the severe rib and limb shortness distinguish it from those disorders.

Detection of soft-tissue foreign bodies by plain radiography, xerography, computed tomography, and ultrasonography.

Detection of a soft-tissue foreign body is common yet often difficult, particularly when the foreign material is not radiopaque. Various imaging modalities have been advocated for detecting foreign bodies that are not revealed by plain radiography. The abilities of plain radiography, xerography, computed tomography, and ultrasonography to detect glass, wooden, and plastic foreign bodies in an in vitro preparation are compared. While all of these imaging techniques demonstrated a glass foreign body, only ultrasonography clearly identified wooden and plastic foreign bodies.

Pediatric musculoskeletal magnetic resonance imaging.

MRI has demonstrated clinical applicability and improved diagnostic capability in the musculoskeletal system to a degree rivaling its success in the neurologic system. For certain regions such as the joints, the superficial nature of these structures is ideal for the application of localized radiofrequency coils, enabling considerably improved signal, spatial resolution, and detectable contrast. The multiplanar capability is often invaluable in assessing such regions as the weight-bearing surfaces of the hips. It has been shown to be the most sensitive single test for diagnosing avascular necrosis, and has yielded very high resolution images of the internal architecture of the joints and soft tissues in an entirely noninvasive manner and without the need for the administration of exogenous contrast agents. Short TE images are quite useful in evaluating fatty background regions of interest, such as the bone marrow and subcutaneous tissue, whereas long TR and TE studies are frequently more suitable for soft tissue pathology. The lack of ionizing radiation and the continued failure of intensive research to disclose any deleterious effects at the levels used for clinical diagnostic imaging make this tool especially appealing for application to the pediatric population. With the continual development of new pulsing sequences, contrast agents, and further advances in imaging/spectroscopic correlation, magnetic resonance imaging of the musculoskeletal system is ensured of a solid role in the clinical diagnostic evaluation of the pediatric population.

Identical twins with malrotation and type IV jejunal atresia.

Identical twins are reported, who associated with maternal anaphylactic shock at 101/2 weeks gestation, acquired a type IV jejunal atresia with total absorption of the small bowel beyond the third portion of the duodenum and persistence of the most terminal length of ileum in the ileocolic artery in an apple peel configuration. Both infants survived a tapered jejunoileostomy and have undergone a successful transition to a regular diet. This accidental occurrence in two infants at the same time and in an identical fashion is a rare event.

Effects of 2,4,6-triaminopyrimidine on the electromechanical properties of guinea-pig myocardium.

The effects of 2,4,6-triaminopyrimidine (TAP) were studied in vitro on transmembrane potential and contractility in guinea-pig myocardium. In electrically driven Langendorff-perfused hearts, the addition of 1 and 5 mM TAP produced an initial dose-dependent positive inotropic response with no change in resting tension. The activation process was studied by means of microelectrode and tension recording from isolated electrically driven guinea-pig left atrial preparations. TAP (1-10 mM) produced dose-dependent changes in both electrical and mechanical properties of the tissue. In particular, 1,5 and 10 mM TAP increased the action potential duration by approximately 20, 40 and 60% while also increasing contractile strength by approximately 30, 60 and 90%, respectively. The mechanical effects of TAP were also observed in the presence of 10 muM propranolol. Experiments on catecholamine-restored hearts which were depolarized by potassium indicated that the inotropic action of TAP was associated with slow calcium influx channels. The inotropic effects of TAP were reduced by D600, a calcium antagonistic agent. These data are consistent with a hypothesis that the inotropic actions of TAP result from a decrease in potassium conductance as a result of the inhibition of Ca++-K+ interactions at the inner surface of the myocardial membrane.