Factor V deficiency in Philadelphia-positive chronic myelogenous leukemia

Factor V deficiency has been identified in 8 of 8 patients 7--20 yr of age, with Philadelphia-positive (Ph1+) chronic myelogenous leukemia (CML). In these 8 patients, factor V deficiency was not due to hepatic dysfunction, factor V inhibitors, or disseminated intravascular coagulation. In 3 patients, factor V activity rose 10%--12% (0.10--0.12 U/ml) after the infusion of 28--31 ml/kg body weight of fresh frozen plasma (FFP). The rise persisted less than 14 hr. The mean measured postinfusion rise in factor V was 18% of the expected rise calculated from the volume of FFP infused in the patients' plasma volume. In 4 patients, a small transient rise in factor V activity occurred after splenectomy or plateletpheresis. Factor V deficiency was completely corrected after a marked reduction in bone marrow cellularity in 2 patients with Ph1+ CML treated with extensive chemotherapy, total body irradiation, and bone marrow transplantation. Factor V deficiency was retrospectively observed in 6 of 20 patients, ages 20--80 yr, with Ph1+ CML and 3 of 6 patients with other myeloproliferative disorders. The factor V deficiency appears to be associated with the large myeloid- megakaryocytic cell mass characteristic of CML and other myeloproliferative disorders.     

Urethral ratio on voiding cystourethrogram: A comparative method to assess success of posterior urethral valve ablation

ObjectiveTo develop a simple, objective and reproducible quantitative measurement to assess success of posterior urethral valve ablation.MethodIn 30 patients with posterior urethral valves the diagnosis was confirmed by voiding cystourethrogram (VCUG). Our protocol was to perform valve ablation, and repeat VCUG at 12 weeks postoperatively. Urethral ratio was calculated by dividing the posterior urethral diameter by the anterior urethral diameter. Thirty males undergoing VCUG for urinary tract infections were evaluated as normative controls.ResultsMedian age of controls was 12 months (2 days–6 years) and of study group was 13 months (1 day–11 years). Mean urethral ratio in pre-fulguration group was 4.94 (±2.97) and in post-fulguration group was 2.134 (±1.19) (P < 0.001). The mean urethral ratio in the control group of 1.73 (±0.577) was significantly different from the pre-fulguration group result (P < 0.001), but not significantly different in comparison to the post-fulguration group (P = 0.104).ConclusionCalculation of urethral ratio on VCUG as a method of assessment of outcome of fulguration is objective, reproducible, and allows preoperative and postoperative VCUG from different facilities to be compared. A post-fulguration urethral ratio of 2.5–3 represents an acceptable result postoperatively.     

Carotid sheath lipoma: first case report in the English literature

Lipomas are the most commonly encountered benign mesenchymal tumour, arising in any location where fat is normally present. Lipomas in the head and neck are rare in all age groups. Cases of vascular sheath lipomas in the femoral region have only been reported in adults. In children, vascular sheath lipomas have not been described to date. We report the first case of a carotid sheath lipoma in a seven-year-old boy. He had a non-tender soft mass with ill defined borders occupying the left upper part of the neck. Magnetic resonance imaging showed a mass at the bifurcation of the left common carotid artery without involving the same. The mass was hyperintense on T1 and T2 weighted sequences, suggestive of a lipoma. The lipoma was enucleated after incising the carotid sheath, safeguarding its contents. Histopathology confirmed it as a lipoma.     

Pneumothorax preceding pulmonary blastoma in a child

Serial plain radiographic, ultrasound and CT findings of an unusual case of pulmonary blastoma are described with a review of the literature.     

UREAPLASMAS UREALYTICUM AND HUMAN INFERTILITY: EFFECT ON SPERMATOZOA MORPHOLOGY

Seminal fluids of 197 males with complaints of involuntary infertility were examined for spermatozoal counts, morphological changes in the spermatozoa and cultured for ureaplasmas and mycoplasmas. In 12, no spermatozoa were present, 29 had a count of less than one million and 156 had more than one million spermatozoa per mL of the seminal fluid. Various morphological changes were detected in the spermatozoa in some cases. U urealyticum and M hominis were grown in 43.15% and 16.75% in comparison to control figures of 15.9% and 11.4% respectively. There was no correlation between growth of ureaplasmas and the spermatozoal count. Among the morphological changes, presence of coiled tails, presence of a fuzzy coat around the tail and microcolonies were highly specific for culture positivity (98.2, 98.2 and 97.35% respectively) but of low sensitivity (55.2%, 14.1% and 8.2% respectively).KEY WORDS: Mycoplasma, Spermatozoa, Ureaplasma     

Laparoscopic management of persistent mullerian duct syndrome

Persistent mullerian duct syndrome (PMDS), characterized by the presence of mullerian structures in a virilized male, frequently presents as undescended testis, either intraabdominal or within a hernial sac. We describe a 10-month-old infant with PMDS successfully managed by the laparoscopic approach. At the age of 1.5 months, the patient presented with a left inguinal hernia and bilateral nonpalpable gonads in another center and underwent left inguinal exploration. The uterus and a gonadlike structure along with the hernia sac were found in the inguinal canal. Left inguinal herniotomy was performed after reduction of the uterus and gonadlike structure. No gonadal biopsy was obtained. The patient was further investigated in the same center. His karyotype was 46,XY. Magnetic resonance imaging of the abdomen and pelvis revealed a uterinelike structure posterior to the urinary bladder, but neither testis nor ovaries were visualized. At 10 months of age, he was referred to our department for further management. A laparoscopic single-stage orchiopexy was performed. Both testes were identified and brought to the scrotum by splitting the uterus in the midline and then bringing the testes with the vas and attached uterine tissue into the scrotum. The aim of placement of well-vascularized testes in the scrotum was achieved as confirmed on follow-up color Doppler ultrasound study 6 months postoperatively, which showed normal vascularity. Laparoscopic surgical techniques for this condition are also discussed.