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K Sharif

Publications and source records attributed to K Sharif.

At least 19 recordsLinked to original sources

Recurrent mesenchymal hamartoma associated with 19q translocation. A call for more radical surgical resection.

Hepatic mesenchymal hamartoma is a rare benign tumour in children. It is often large and centrally located in the liver at diagnosis, making surgical resection difficult; thus non-radical resection has been proposed in the past as acceptable management. However, a literature survey and a case with recurrence associated with cytogenetic anomalies suggest that radical liver surgery (resection with a margin of normal liver parenchyma, as for malignant tumour) should be recommended for mesenchymal hamartoma.

Chromosomes, Human, Pair 19↗

Management of end-stage central venous access in children referred for possible small bowel transplantation.

UNLABELLED: The 3-year survival after small bowel transplantation (SBTx) has improved to between 73% and 88%. Impaired venous access for parenteral nutrition can be an indication for SBTx in children with chronic intestinal failure. AIM: To report our experience in management of children with extreme end-stage venous access. SUBJECTS: The study consisted of 6 children (all boys), median age of assessment 27 months (range, 13-52 months), diagnosed with total intestinal aganglionosis (1), protracted diarrhea (1), and short bowel syndrome (4), of which gastroschisis (2) and malrotation with midgut volvulus (2) were the causes. All had a documented history of more than 10 central venous catheter insertions previously. All had venograms, and 1 child additionally had a magnetic resonance angiogram to evaluate venous access. Five of 6 presented with thrombosis of the superior vena cava (SVC) and/or inferior vena cava. METHODS: Venous access was reestablished as follows: transhepatic venous catheters (5), direct intra-atrial catheter via midline sternotomy (4), azygous venous catheters (2), dilatation of left subclavian vein after passage of a guide wire and then placing a catheter to reach the right atrium (1), radiological recanalization of the SVC and placement of a central venous catheter in situ (1), and direct puncture of SVC stump(1). Complications included serous pleural effusion after direct intra-atrial line insertion, which resolved after chest drain insertion (1), displacement of transhepatic catheter needing repositioning (2), and SVC stent narrowing requiring repeated balloon dilatation. OUTCOME: Four children with permanent intestinal failure on assessment were offered SBTx, 3 of which were transplanted and were established on full enteral nutrition; the family of 1 child declined the procedure. In the remaining 2 children in whom bowel adaptation was still a possibility, attempts were made to provide adequate central venous access as feeds and drug manipulations were undertaken. One of them received liver and SBTx nearly 3 years after presenting with end-stage central venous access, because attempts to achieve independence from parenteral nutrition had failed. The other child died immediately after a transhepatic venous catheter placement, possibly from a nutritional depletion syndrome as no physical cause of death was found. Direct intra-atrial catheters in transplanted children proved to be adequate for the management of uncomplicated transplantation, although the usual infusion protocol had to be modified considerably, and the lack of access would have been critical if massive blood transfusion had been required during the transplant procedure. CONCLUSION: It was possible to reestablish central venous access in all cases. However, this was time consuming and difficult to assemble a skilled team consisting of one of more: surgeon, cardiologist, interventional radiologist, and transplant anesthetist. Small bowel transplantation is easier and safer with adequate central venous access, and we advocate liaison with an SBTx center at an early stage.

Catheterization, Central Venous↗

Role of rescue IVF-ET treatment in the management of high response in stimulated IUI cycles.

Rescue in-vitro fertilisation and embryo transfer (IVF-ET) has been used in high response gonadotrophin intrauterine insemination (IUI) cycles to minimise the risks of ovarian hyperstimulation and multiple gestation. Such unplanned IVF treatment increases the cost of treatment. But can this added cost and the risks associated with IVF be justified? We present our experience with this treatment using clinical pregnancy and live birth rates as the primary outcomes. Between 1998 to 2001, 40 women undergoing IUI cycles who over responded (>3 follicles measuring >15 mm in diameter on the planned day of hCG administration) to gonadotrophin were offered the choice of conversion to IVF-ET or cancel the cycle. 17/40 declined rescue IVF/ET and had their cycles cancelled. 23/40 converted to IVF/ET and underwent transvaginal oocyte retrieval. 21/23 had embryo transferred. The clinical pregnancy and live birth rates were 52% and 48%, respectively. Rescue IVF-ET offers excellent clinical pregnancy and live birth rates in high responders. However, affordability can be an obstacle in the utilization of this treatment option.

Adult↗

Management of hepatic epithelioid haemangio-endothelioma in children: what option?

Hepatic epithelioid haemangio-endothelioma (HEHE) is an endothelium-derived tumour of low-to-medium grade malignancy. It is predominantly seen in adults and is unresponsive to chemotherapy. Liver transplantation is an accepted indication when the tumour is unresectable. Hepatic epithelioid haemangio-endothelioma is very rare in children and results after transplantation are not reported. The aim of this study is to review the experience of three European centres in the management of HEHE in children. A retrospective review of all paediatric patients with HEHE managed in three European centres is presented. Five children were identified. Four had unresectable tumours. The first had successful resection followed by chemotherapy and is alive, without disease 3 years after diagnosis. One child died of sepsis and one of tumour recurrence in the graft and lungs 2 and 5 months, respectively, after transplant. Two children who had progressive disease with ifosfamide-based chemotherapy have had a reduction in clinical symptoms and stabilisation of disease up to 18 and 24 months after the use of platinum-based chemotherapy. HEHE seems more aggressive in children than reported in adults and the curative role of transplantation must be questioned. Ifosfamide-based chemotherapy was not effective. Further studies are necessary to confirm if HEHE progression in children may be influenced by platinum-based chemotherapy.

Adolescent↗

A postal survey of hydrosalpinx management prior to IVF in the United Kingdom.

BACKGROUND: In the last decade, numerous studies have demonstrated concern about the presence of hydrosalpinx and its management in patients undergoing IVF. We evaluated the current management of hydrosalpinx prior to IVF treatment in the UK. METHODS: A total of 117 postal survey, anonymous, sealed questionnaires were sent to all IVF centres in the UK, to determine the policy for the management of hydrosalpinx in infertile women prior to IVF treatment. RESULTS: There were 88 (75%) responders, of which 80 (91%) indicated that they discussed the effect of hydrosalpinx on IVF outcome. Ten (12%) units did not recommend treatment of hydrosalpinx prior to IVF treatment, while 30 (36%), 27 (33%) and 16 (19%) recommended treatment weakly, strongly and very strongly respectively. The treatment options offered by clinicians were laparoscopic salpingectomy (75%), open salpingectomy (45%), salpingostomy (40%), proximal tubal occlusion (34%), transvaginal songraphic (TVS) aspiration during oocyte collection (23%) and TVS aspiration before oocyte collection (10%). The frequency of use varied from one option of treatment to another. Only 28% of the responders had a protocol or guidelines for the management of hydrosalpinx. CONCLUSIONS: More attention should be given to patients with hydrosalpinx prior to IVF treatment and patients should be counselled about the negative effect of hydrosalpinx on IVF outcome. There is a wide variation in the management of hydrosalpinx prior to IVF treatment in the UK and many treatment options may be questionable, as they are not yet based on evidence.

Ambulatory Care Facilities↗

Are the anticipated benefits of myomectomy achieved in women of reproductive age? A 5-year review of the results at a UK tertiary hospital.

Fibroids are the most common benign tumours of the pelvis in women, with a prevalence estimated at 20-50%. They are more common towards the end of the reproductive years. There is a racial preponderance, being more common in black than white women. This may relate to the aetiology, which is still poorly understood. Generally, fibroids do not cause symptoms but some sufferers do complain about pressure symptoms, abnormal vaginal bleeding and infertility. For these reasons, myomectomy is often resorted to after failure of medical interventions on the premise that it brings about improvement/cure of symptoms and enhancement of fertility. However, the evidence for these indications for surgery is hazy. An analysis of the 109 medical records of symptomatic patients who had myomectomy over a 5-year period at a tertiary centre revealed the following. Single-symptom presentation in 41 (38%), menorrhagia in 20 (18%) being the most common. Only 52 (48%) patients had medical treatment of one form or another before myomectomy. Additional operative findings included pelvic adhesions, evidence of PID and endometriosis. Thirty-four (31%) had an estimated blood loss 500 ml and 23 of these patients needed blood transfusion. There were four cases of unscheduled hysterectomies due to uncontrollable bleeding. Pyrexia was the most common (38%) postoperative complication followed by superficial wound infection in 5%. We observed improvement of symptoms, assessed over a range of 2-24 months, in 34 cases (68%) in patients without fertility symptoms who accounted for 50 of these women. The symptomatic benefit was less (36%) in the 'infertility group'. Following an observation period of over 12-36 months, 17 patients in the 'infertility group' were lost to follow-up. Two (14%) of the 14 patients who attempted in vitro fertilisation (IVF) were successful. In the non-IVF group, 13 (46%) of the 28 achieved natural conception. These results suggest that symptomatic improvement and fertility enhancement may be possible in some patients with fibroids. In view of the risks and potential failure of treatment associated with myomectomy these results, yet again, support the fact that patients should be properly counselled before embarking on myomectomy and we strongly advocate local data to form the basis of the advice given during the consultation rather than what obtains in the literature.

Adult↗

Bile duct of Luschka leading to bile leak after cholecystectomy--revisiting the biliary anatomy.

Bile ducts of Luschka (also called supravesicular ducts) are small bile ducts in the gallbladder bed. Although they do not drain any liver parenchyma, they can be a source of bile leak or biliary peritonitis after cholecystectomy in both adults and children, as shown in this case report. As a reminder, variations of biliary anatomy in the gallbladder bed and cholecysto-hepatic triangle of Calot, are reviewed.

Bile Ducts↗

New perspective for the management of near-total or total intestinal aganglionosis in infants.

BACKGROUND/PURPOSE: Extensive intestinal aganglionosis is rare. The diagnosis and treatment are known to be difficult and it had been considered to be fatal. The aim of this study was to review our experience with children with extensive intestinal aganglionosis. METHODS: Retrospective analysis was conducted in patients referred to the intestinal transplantation unit since 1993. Presentation and outcome were analysed looking at 2 groups who had either undergone previous subtotal intestinal resection (group I) or no or limited resection (group II). RESULTS: Eight children were selected (3 patients in group I and 5 in group II). Group I was remarkable in that patients all were referred early in age with progressing liver failure. Parents of one patient refused to accept transplantation as treatment, and he died one month later. Two noncirrhotic patients were maintained in the parenteral nutrition programme and currently progress well with enteral feedings. The other 5 patients underwent transplant, and 4 of 5 are alive after transplantation with a mean follow-up of 22.2 months (range 0.4 to 63.6). CONCLUSIONS: Subtotal resection of intestine at the time of diagnosis must be avoided. Conservative management with parenteral nutrition may be associated with long-term good outcome. Small bowel transplant may open new perspective in the management of patients with end-stage liver disease.

Female↗

Spontaneous disappearance of a normal adnexa associated with a contralateral polycystic-appearing ovary.

BACKGROUND: Absence of the adnexa may be congenital or acquired. However, the etiology is often uncertain. CASE: A 27-year-old woman presented with a 3-year history of subfertility. Her irregular menstruation was associated with acne vulgaris, alopecia, and elevated body mass index. Transvaginal ultrasonography of the pelvis showed a normal uterus, a normal right ovary, but a polycystic-appearing left ovary. A hysterosalpingogram demonstrated a normal uterine cavity, prompt filling and spilling of contrast material from the left fallopian tube, but no filling on the right. Subsequent laparoscopy showed an unexpected absence of right adnexa and presence of a solitary rounded free-floating mass enshrouded in the omentum. She did not have a history of abdominal pain or surgery. CONCLUSION: The evidence suggests that the patient might have had an asymptomatic infarction of the right adnexa.

Adnexa Uteri↗

Strategy for hepatoblastoma management: Transplant versus nontransplant surgery.

BACKGROUND: Liver transplantation now is proposed for managing selected hepatoblastoma cases. Indications are not yet well defined. METHODS: The case records of 34 children with hepatoblastoma treated over a period of 10 years (1991 to 2000) were reviewed retrospectively. RESULTS: All patients benefited from preoperative chemotherapy. Twenty patients underwent major hepatic resections. Twelve patients, in absence of residual metastasis, underwent liver transplant because the tumour remained unresectable after chemotherapy. Two patients who presented with recurrence after a right hepatectomy, benefited from transplant as a second option. Two other patients did not undergo surgery because of widespread disease or resistance to chemotherapy. Disease-free survival rates were 95% after surgical resection, 100% when primary transplant was performed in patients with good response to chemotherapy, 60% after transplantation in patients with poor response to chemotherapy, 50% in patients with transplant as second option, and 0% in patients not undergoing surgery. CONCLUSIONS: Transplantation is a potentially curative option for unresectable hepatoblastoma when chemosensitive (decrease in alpha-fetoprotein and decrease in tumour size). In this context, also favourable cases with good response but difficult resections with doubtful margins of resection may best be proposed for primary transplantation. Patients with recurrent or resistant disease are not good candidates.

Antineoplastic Agents↗

Benefits of early diagnosis and preemptive treatment of biliary tract complications after major blunt liver trauma in children.

BACKGROUND/PURPOSE: Nonoperative management of blunt liver trauma may delay diagnosis of related biliary complications leading to delayed surgical intervention and related morbidity. The aim of this study was to see whether technetium (tc) 99 trimethylbromo-im-indolacetic acid (TBIDA) nuclear scan would allow noninvasive early diagnosis of bile leak and pre-emptive management. METHODS: Retrospective analysis of the patient records and radiologic investigations of 7 patients admitted between April 1998 and December 2000 with "major" blunt liver trauma (parenchymal fracture of less than 4 cm on computed tomography [CT] scan or involving porta hepatis) and various types of biliary complications. Patients with or without early TBIDA diagnosis were compared. RESULTS: There were 7 patients. The first 2 patients were treated conventionally without TBIDA, and late diagnosis was associated with further related problems (sepsis, life-threatening hemorrhage in both cases) and prolonged hospital stay. The subsequent 5 consecutive patients benefited from early diagnosis (TBIDA scan, 2 to 4 days after trauma), and preemptive management was done (tailored to each case). There was no further or related morbidity. All 7 patients currently are alive and well. CONCLUSIONS: A TBIDA nuclear medicine scan was efficient in providing an early diagnosis of biliary leakage, thus, allowing adequate preemptive management. In turn, this may have helped avoid related added morbidity compared with cases of late diagnosis. Early TBIDA scan should be performed routinely when the initial CT scan confirms liver trauma graded as "major."

Adolescent↗

Long term safety of fluoroscopically guided selective salpingography and tubal catheterization.

BACKGROUND: The irradiation of the ovaries of reproductive age women during fluoroscopically guided selective salpingography and tubal catheterization has raised concern about the safety of the procedure. In addition to the risk of cancer induction, which exists with the irradiation of all tissues, with the gonads, the induction of hereditary disorders is possible. The objective of this study was to estimate these risks and present them in a clinically meaningful way. METHODS: Retrospective analysis was undertaken of 366 consecutive cases of selective salpingography and tubal catheterization performed at the Birmingham Women's Hospital, UK. The radiation doses of different types of procedure were compared with the background annual radiation dose. The risks of cancer and genetic disorders induction were calculated using conversion coefficients published by the International Commission on Radiological Protection. RESULTS: The radiation dose women were exposed to during selective salpingography and tubal catheterization under fluoroscopic guidance was a fraction of the background annual radiation dose. The excess lifetime risks of cancer and hereditary disorders were in the order of four to 13 and two to six per million procedures respectively. CONCLUSIONS: The long term risks of selective salpingography and tubal catheterization under fluoroscopic guidance are low.

Adult↗