Disappearance of Tophi in Familial Juvenile Hyperuricemic Nephropathy after Kidney Transplantation

A 40-year-old man who had been on hemodialysis for 25 months due to familial juvenile hyperuricemic nephropathy (FJHN) received a kidney transplant. Biopsy of his native kidney had shown tubulo-interstitial nephropathy. Genetic analysis confirmed abnormal uromodulin expression due to a mutation in the exon 4 of the UMOD gene. He had multiple tophi on the day of transplantation, including some on his fingers. He received immunosuppressive treatment including polyclonal antilymphocyte antibodies, mycophenolate mofetil, steroids and cyclosporine and achieved excellent renal function, with serum creatinine at 13 mg/L on day 10 posttransplantation and 9.4 mg/L at 6 months. His uric acid excretion rate increased from 4.4% at day 2 posttransplantation to 7.7% 6 months after transplantation. The number and sizes of the tophi were reduced 3 months posttransplantation, and nearly disappeared at month 6. Serum uric acid level decreased slowly from 650 mumol/L before transplantation to 300 mumol/L. Reduction of tophi was probably due to the absence of the mutated UMOD gene in the transplanted kidney.     

HP26PSEUDO‐ACHALASIA FOLLOWING A SLIPPED LAPAROSCOPICALLY PLACED ADJUSTABLE GASTRIC BAND: A CASE REPORT

Purpose To demonstrate that laparoscopic adjustable gastric banding may promote oesophageal dilatation or interfere with oesophageal motility. Methodology We report a case of a 67 year old female with a complex medical history who developed secondary achalasia from a slipped laparoscopic adjustable gastric band for weight loss. This led to recurring episodes of aspiration pneumonia requiring multiple admissions at North Shore Hospital, Auckland, New Zealand. Results A decision was made to remove the gastric band, five years after its initial insertion. At one month follow up, she was swallowing normally and oesophageal manometry had returned to normal. Conclusion Oesophageal dysmotility is sometimes seen in patients who have bands that are adjusted too tightly or in whom the band has slipped. This can lead to serious complications if unrecognized and incorrectly treated. Oesophageal symptoms in patients with adjustable bands must be considered secondary to the band until proven otherwise ie removal of the band or complete deflation.     

Fontan completion without surgery.

OBJECTIVE: There are several modifications introduced in the preparation for a subsequent non-surgical transcatheter completion of the Fontan procedure. We report our experience with one type of the modification and the short-term results following its implementation. METHODS: During bidirectional cavopulmonary connection (BCPC) an intra-atrial lateral tunnel is additionally created, as intended for a Fontan procedure but fenestrated with a 10-14 mm aperture. The cardiac end of the superior vena cava (SVC) is then patched to maintain the physiology of BCPC. During the interventional transcatheter completion procedure, the SVC patch is perforated using radio-frequency (RF) energy, balloon-dilated, and stented as well. The aperture is closed with a device when required. Paired t-test was used to compare data before and after the Fontan completion. RESULTS: From June 2003 to February 2006, 16 patients (9 boys and 7 girls, mean age 12 months) underwent the surgical procedure described. The mean bypass time was 137 min and the mean ischemic time was 77 min. There were no operative deaths. One patient with bilateral SVC required a take down due to recurrent effusions. Ten months later, nine patients underwent completion (mean age 20 months, mean weight 10.6 kg). The stents were dilated to a mean diameter of 14.4mm. All except one aperture was closed with a device. The mean fluoroscopy time was 41 min. Oxygen saturation increased from 85 to 94% (p=0.001). Pulmonary artery pressures remained normal (16 mmHg before and 19 mmHg after, p=0.12). No patients required mechanical ventilation and none developed pleural effusions or arrhythmias. All were discharged from hospital within 6 days of the Fontan completion. Twenty-two months after Fontan, all were well. Echocardiography revealed no gradients across the stents. Two patients had minor leaks across the aperture. One underwent further stent dilatation a year later. CONCLUSIONS: Fontan completion without surgery is suitable in patients with single ventricles with lower mortality and morbidity, avoids multiple surgical interventions while maintaining the staged approach and allows for successive dilatation of the Fontan pathway to accommodate for growth.     

Retroperitoneoscopic approach to the adrenal glands and retroperitoneal tumours in children: where do we stand?

Choosing the best way to approach adrenal gland and retroperitoneal tumours is still difficult. We reviewed our first 10 cases operated on by retroperitoneoscopy and compared this approach with other possible ways described in the literature. There were 2 intraoperative complications: 1 opening of the diaphragm and 1 bleeding. Tumour resection was always complete. There was no conversion. There were no postoperative complications. The retroperitoneoscopic approach for adrenalectomy and retroperitoneal tumour resection is increasingly being used. In children, operation is quite fast, without much blood loss and with spectacular postoperative recovery results. Even for the right side we advocate this approach, due to the particular anatomy (small tumour size, less fat, thinner muscle layers) in this age group. Trained surgeons are, of course, mandatory.     

Decidua menstrualis

Archives of Gynecology and Obstetrics -     

Status of Women and Infants in Complex Humanitarian Emergencies

Women and children bear the greatest burden in the midst of war and long‐term disasters. Complex humanitarian emergencies are characterized by social disruption, armed conflict, population displacement, collapse of public health infrastructure, and food shortages. Humanitarian assistance for refugees and internally displaced populations requires particular attention to the common issues affecting morbidity and mortality in women and infants. Gender‐based violence and reproductive health concerns are discussed within the context of populations affected by conflict and forced migration. Recommendations for midwives and women's health care providers engaging in care for women and children in complex humanitarian emergencies are discussed.     

Transarterial embolization of vein of Galen aneurysmal malformation after unsuccessful stereotactic radiosurgery

The authors present three cases of vein of Galen aneurysmal malformations (VGAMs) diagnosed in infancy and submitted by the referring teams for stereotactic radiosurgery as the initial therapy (therapeutic doses ranging between 20–25 Gy and 40–50 Gy to the peak dose). After the conventional follow-up of 18–24 months, no change could be detected in the angioarchitecture of the lesions. All three cases were then referred for endovascular treatment and underwent embolization by the transarterial route using liquid adhesives (N-butyl cyanoacrylate). This resulted in complete anatomical exclusion of the lesion. Regardless of the theoretical efficiency of radiosurgery in the management of brain arteriovenous malformations, the present authors believe that transarterial embolization remains the treatment of choice in VGAMs. It offers a high rate of morphological cure and the best chances for normal neurocognitive development. The time required by radiosurgery to achieve a significant result is too long for developing and maturing brain and may not prevent the negative effects of the lesion, mainly in regard to hemo- and hydrodynamic disorders (atrophy, subcortical calcifications, etc.) created by the VGAM, thus leading to irreversible mental retardation.     

Benign osteoblastoma of the parietal bone

A 5-year-old girl presented with a 1-year history of a subcutaneous swelling in the right parietal region. There were no cerebral symptoms or signs. Plain X-ray of the skull showed a lytic bony lesion with sclerotic margin. Computed tomography showed a hypodense, osteolytic lesion with thinned and bulged inner and outer skull tables with intact continuity. There was no intracranial lesion. At operation, the mass was found to be pink and granular, and was totally enucleated. Histology revealed it to be a benign osteoblastoma.     

Influence of urbanization of the western coast of the United Arab Emirates on trace metal content in muscle and liver of wild Red-spot emperor (Lethrinus lentjan).

We hypothesized that increased ambient concentrations of metals, as a consequence of escalating urbanization and industrialization of the Gulf region will respond in increased contamination of edible fish species. In this study, we report concentrations of chromium, manganese, cobalt, copper, zinc, arsenic, cadmium, mercury and lead in meat and liver of wild Red-spot emperor (Lethrinus lentjan) from three sampling points at the UAE coast. Analysis was performed by the ICP-MS/microwave digestion. Our study has shown that meat and liver metal content was significantly higher in areas with higher industrial activity, although metal values did not exceed permitted levels of fish for human consumption.     

Endonasal carbon-dioxide laser assisted dacryocystorhinostomy verses external dacryocystorhinostomy

This is a prospective, non-randomized study to evaluate and compare the results, morbidity and surgical time for endonasal carbon-dioxide laser assisted dacryocystorhinostomy and external dacryocystorhinostomy. 70 consecutive patients of chronic dacryocystitis with nasolacrimal duct obstruction were selected for the study. 36 patients under went endonasal CO2 laser assisted dacryocystorhinostomy and 34 had external dacryocystorhinostomy. Selection of the type of operation was left to the patient's choice. All the patients had preoperative counseling and both the procedures were explained in detail with their advantages and disadvantages. Patients not willing for the external incision were selected for endonasal laser assisted dacryocystorhinostomy and others were operated via external approach. Silicone tubes were put in all the patients for three months after surgery. The final follow up was 12 months after the removal of silicone tubes. The patency of the lacrimal passage was confirmed by irrigation, and patients were questioned about their symptoms. The success rates, 12 months after removal of silicone tubes were 100% in endonasal CO2 laser assisted dacryocystorhinostomy and 88.24% in external dacryocystorhinostomy. The surgical time of endonasal laser assisted dacryocystorhinostomy was 38 minutes as compared to 62 in external dacryocystorhinostomy. Complication rate in both groups was almost equal. Thus, we came to the conclusion that Endonasal CO2 laser assisted dacryocystorhinostomy is a better surgical option to external dacryocystorhinostomy in cases of chronic dacryocystitis with nasolacrimal duct obstruction, with shorter surgical time.