A case of retroperitoneal neuroblastoma in an adult with extensive bone marrow metastasis

We present a case of retroperitoneal neuroblastoma in a 27-year-old male with extensive bone marrow metastasis at the first presentation. After the simple excision of the tumor, adjuvant multi-drug chemotherapy, consisting of vincristine, actinomycin-D, ifosfamide, doxorubicin, carboplatin and etoposide, was carried out for 17 months, leading to complete remission. Ten months after completion of the chemotherapy, the tumor recurred with bone marrow metastasis. He further developed thoracic vertebral metastases resulting in paraplegia, and died of the disease 41 months after the presentation. The clinical course of this case, with its emphases especially on the effect of the chemotherapy, is described in this report. Since the clinical characteristics and treatment strategies for adult neuroblastoma have not yet been well established, they remain to be investigated in detail.     

Restoration of fragile histidine triad (FHIT) expression induces apoptosis and suppresses tumorigenicity in breast cancer cell lines

The fragile histidine triad (FHIT) gene at chromosome 3p14.2 is a tumor suppressor gene that is altered mainly by deletion in a large fraction of human tumors, including breast cancers. To evaluate the potential of FHIT gene therapy in this type of cancer, we have studied the biological effects of adenoviral FHIT transduction (Ad-FHIT) in breast cancer cell lines. The results showed that, after FHIT restoration in BT-549, MDA-MB-436, and HCC1806 cells, they underwent apoptosis by activation of the intrinsic pathway. In all three cell lines infected with Ad-FHIT, we have found activation of caspase-2, which is required for permeabilization of mitochondria, release of cytochrome c, and apoptosis. Furthermore, Fhit overexpression produces alteration in cell cycling properties, as well as reduction of the tumorigenic potential in nude mice.     

Neonatal hydronephrosis detected on routine health check-up

BACKGROUND/PURPOSE: Prenatal dilatation of the urinary tract may be caused by obstructive defects, and it is known that 20% of normal fetuses have some degree of renal pelvic or calyceal dilatation, determined by sonographic examination. We analyzed the clinical course of patients found to have dilatation of the renal pelvis by ultrasound 1 month after birth, and compared prenatal and postnatal sonograms of each patient. METHODS: Between January 1996 and December 1998, renal ultrasounds were performed on 2,071 children at their 1 month routine health checks in our hospital. We found dilatation of the renal pelvis in 92 kidneys in 84 children and then compared these neonatal sonograms with the prenatal ultrasounds for each child. Ultrasound examinations were performed at 28 weeks of gestational age and 1 month after birth. Our criterion for diagnosis of hydronephrosis at 1 month of age was a renal pelvis measuring greater than 7 mm at the central echo complex. The medical records of patients found to have hydronephrosis were then reviewed. RESULTS: Eight patients had bilateral hydronephrosis, 2 had dilatation only in the right kidney and 74 had this finding only in the left kidney. Examination of 36 (39%) of these 92 kidneys revealed renal pelvic dilatation to be present both prenatally and neonatally. The dilatation ranged from 7 to 43 mm. No dilatation of the renal pelvis was seen on the other 56 fetal examinations. During the follow-up period, 2 patients (1 with bilateral and the other with right-sided hydronephrosis) were diagnosed with vesicoureteral reflux, the 1 patient with bilateral pelvic dilatation was found to have a vesico-ureteral junction obstruction. After full evaluation, the other children were found to have no anatomic abnormalities. CONCLUSIONS: We found 84 of 2,071 children showed dilatation of the renal pelvis on ultrasound examination performed at 1 month of age. Three (3.3%) of the 84 children required surgery to correct the neonatal hydronephrosis detected via this imaging modality. Interestingly, 88% of the children had only left-sided hydronephrosis, which did not predict an adverse outcome during the follow-up period. We conclude that neonatal hydronephrosis appears to be a relatively benign condition and the requirement of surgery is relatively slight.     

Effect of beraprost sodium on pulmonaryvascular resistance in candidates for a Fontan procedure: A preliminarystudy

OBJECTIVE: Although long-term prostacyclin(PGI2) therapy in patients with severe pulmonary hypertension (PH)reduces pulmonary vascular resistance (PVR), there have been no reports on its therapeutic effects in patients with mild PH. We investigated the chronic effect of beraprost sodium (BPS), an oral PGI2 analog, in children with mild PH. METHODS: We studied 20 patients who were destined for a Fontan procedure with a mean pulmonary arterial pressure(PAP) of>20 mmHg and/or PVR of>3.0 Wood units. Both the PAP and the PVR in these cases were too high for patients to undergo a successful Fontan procedure. Seven patients received BPS (PG group) and 13 did not (control group). All patients underwent repeat cardiac catheterization to examine pulmonary hemodynamics. RESULTS: In the PG group, the pulmonary-to-systemic flow ratio (Qp/Qs) did not change after BPS administration(1.1 +/- 0.6 vs 1.3 +/- 0.9);however, the mean PAP decreased significantly (25.3 +/- 8.2 vs 19.9 +/- 6.5 mmHg; P < 0.05),as did PVR (3.7 +/- 1.3 vs 2.3 +/- 0.9 Wood units; P < 0.05), whereas the pulmonary artery (PA) index increased significantly (312 +/- 136 vs 375 +/- 165; P < 0.05). In the control group, the mean PAP decreased significantly (24.9 +/- 4.7 vs 19.8 +/- 6.3 mmHg; P < 0.05)and the PA index increased significantly (295 +/- 72 vs 362 +/- 114; P < 0.05). No significant changes in Qp/Qs (1.5 +/- 0.8 vs 1.4 +/- 0.6)or PVR (2.9 +/- 1.3 vs 2.5 +/- 0.8 Wood units) were observed. CONCLUSION: We conclude that long-term BPS administration probably reduces PVR in potential candidates for a Fontan procedure with mild PH. This treatment would reduce the risks associated with the Fontan procedure and would also improve its outcome.     

In Vitro Release Profile of Mitomycin C from Albumin Microspheres: Extrapolation from Macrospheres to Microspheres

To analyze the in vitro release profiles of mitomycin C from albumin microspheres prepared by chemical denaturation in a multiparticulate system, a method to calculate the total cumulative amount of mitomycin C released from a batch of microspheres was developed. Mitomycin C-loaded albumin macrospheres (diameter in mm range) were prepared, and the in vitro release kinetics of mitomycin C from individual macrospheres were determined. Then the relationship between the kinetic parameters and the physical parameters (e.g., diameter, weight) was investigated under the assumption that macrospheres and microspheres behave identically. Further, the size distribution of microspheres was measured, and the total cumulative amount of mitomycin C released from albumin microspheres was calculated. The release profiles of mitomycin C from individual macrospheres fitted first-order release kinetics better than spherical matrix kinetics. The calculated initial mitomycin C contents and first-order release rate constants for individual macrospheres were correlated with the weight and reciprocal of surface area of the macrospheres, respectively. The observed in vitro release profile for the microspheres agreed with the calculated values. These results suggest that this method is valid for calculating drug release from albumin microspheres.     

Somatotopic organization of motoneurons innervating the pronators, carpal and digital flexors and forepaw muscles in the dog: a retrograde horseradish peroxidase study

Central distribution of motor neurons innervating the individual forearm and forepaw muscle through the median and ulnar nerves was studied in the dog using the retrograde horseradish peroxidase (HRP) method. All HRP-labeled cells were seen ipsilaterally in restricted parts within the dorsolateral (DL) and retrodorsolateral nucleus (rDL) of the ventral horn at levels from the cranial tip of C7 to the cranial third of T2. The results showed that motoneurons situated more cranially supply the more proximal muscles and those situated more caudally supply the more distal ones, and that the pronator motoneurons occupy longitudinally the central part of the DL; the carpal flexor motoneurons, the medial part of the DL; the digital flexor motoneurons, the dorsal central part of the DL and the lateral fourth of the rDL; and the forepaw muscle motoneurons, the medial three-fourths of the rDL. These somatotopic arrangements of each motor pool could be correlated with the location and action of the muscles.     

Stenting of a stenosed major aortopulmonary collateral artery in a baby with pulmonary atresia and a ventricular septal defect: Rescue from critical hypoxia in the immediate postoperative stage of unifocalization supported by extracorporeal membrane oxygenation

A 4‐month baby with pulmonary atresia, ventricular septal defect, major aortopulmonary collateral arteries (MAPCAs) and an extremely hypoplastic central pulmonary artery developed critical hypoxia following right unifocalization combined with a right Blalock‐Taussig shunt. To increase pulmonary blood flow we stented the left lower MAPCA during extracorporeal membrane oxygenation (ECMO) support. He was successfully weaned from ECMO 2 days after stenting. Percutaneous intervention for a stenosed MAPCA is an effective means of increasing pulmonary blood flow in critically hypoxic patients following unilateral unifocalization, even in the immediate postoperative stage. © 2008 Wiley‐Liss, Inc.