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Undifferentiated (embryonal) sarcoma of the liver in a six-year old: a case report

Rahman Jamal, Sharifah, N.A., Zulfiqar, A., Zakaria, Z.

Malaysian Journal of Child Health, 1997;9(2):199-202.
MyJurnal

We report a rare case of undifferentiated (embryonal) sarcoma of the liver in a six-year-old girl who at presentation, had fever, right hypochondrium pain and hepatomegaly. The diagnosis was clinched by fine needle aspiration cytology and was subsequently reconfirmed by histopathological examination of the resected tumour. Pre-operative chemotherapy was given because primary resection was deemed not possible. The patient underwent a successful extensive hepatectomy followed by continuation chemotherapy
Fulltext Intravenous immunoglobulin in the treatment of acute disseminated encephalomyelitis

Kanaheswari Y, Baizura J, Zulfiqar A

Med J Malaysia, 2004 Mar;59(1):103-7.
PMID: 15535344

We describe a case of acute disseminated encephalomyelitis in a child. This case is unusual in that the illness was characterised by recurrent episodes rather than a monophasic course and that the choice of treatment was intravenous immunoglobulin over corticosteroids. The rapid and remarkable recovery is highlighted and a review of the treatment for this rare condition is discussed.
Magnetic resonance imaging in anterior ectopic anus

Thambidorai CR, Raghu R, Zulfiqar A

Pediatr Surg Int, 2008 Feb;24(2):161-5.
PMID: 17985137

Different criteria have been used in literature to describe the anterior ectopic anus (AEA) anomaly, resulting in uncertainty over its prevalence, association with constipation and definition of the indications for surgery. It has been recently proposed that the term AEA should be restricted to anomalies in which a normal appearing anal orifice is located in the perineum in a more anterior location than normal, with an anal canal of normal calibre that is shown by electrical stimulation to be surrounded by the voluntary external anal sphincter (EAS). We report about four infants, three females and one male, who presented with constipation and had an anteriorly located anal orifice of normal calibre. The anal position index measured clinically was less than 0.34 in all the female patients and 0.44 in the male patient. In preoperative magnetic resonance imaging (MRI), the EAS was distributed all around the circumference of the anal canal, including the ventral aspect of the anal canal, in all the patients. Preoperative MRI documentation of sphincter distribution is recommended for the diagnosis of AEA, as it would help in better definition of its association with constipation and the results of surgical management.
Fulltext Computed tomography (CT) of orbital pseudotumour

Zulfiqar A, Abdul-Samad S, Alias D, Norizan A

Med J Malaysia, 1993 Jun;48(2):160-5.
PMID: 8350791

Orbital pseudotumour is a non-specific inflammatory disease. Its highly variable clinical and radiographic presentation makes specific diagnosis difficult. This study on 31 proven cases of pseudotumour shows that unilateral involvement, retroorbital fat infiltration and enlargement of multiple extraocular muscles with involvement of the tendinous insertions are key computed tomography CT features which help to establish the diagnosis of orbital pseudotumour.
Fulltext Ultrasound (US) and computed tomographic (CT) appearances of large (giant) hepatic cavernous hemangiomas

Samad SA, Maimunah A, Zulfiqar A, Zaharah M

Med J Malaysia, 1995 Mar;50(1):82-6.
PMID: 7752982

The sonographic and CT appearances of 9 large cavernous hemangiomas of the liver were studied. On sonography, 6 masses (67%) exhibit heterogenous echo pattern; where in 2 patients the echotexture was a mixture of hypoechoic and isoechoic areas and in 4 patients there are varying amounts of bright hyperreflective areas similar to the texture typical of small hemangiomas. The masses were predominantly hypoechoic in the remaining 3 patients (33%). Incremental bolus or bolus-infusion dynamic CT showed peripheral contrast enhancement of varying intensities and thickness in all patients. The lesions were incorrectly diagnosed as hepatomas in 4 patients, suspected as hemangiomas with a differential diagnosis of hepatomas in 4 patients and an early liver abscess in 1 patient. It is concluded that large cavernous hemangiomas of the liver do not exhibit the typical homogenous hyperreflective echotexture as exhibited by small lesions and they mimic primary and secondary hepatic neoplasms. However, the diagnosis of hemangioma should be entertained when such a mass contains bright hyperechoic areas within its heterogenous echo pattern and exhibit peripheral enhancement on contrast enhanced CT. In addition to correlation with appropriate clinical information, confirmation of diagnosis include delayed scanning during a routine incremental bolus dynamic CT, single-slice dynamic contrast enhanced CT, angiography or isotope scintigraphy and magnetic resonance imaging depending on the availability of facility.
Fulltext Currarino's triad: Intraoperative ultrasound image-guided surgery

Thambidorai CR, Adbel LK, Zulfiqar A

J Indian Assoc Pediatr Surg, 2010 Oct;15(4):137-8.
PMID: 21170197 DOI: 10.4103/0971-9261.72438

This is a report on the use of transperineal intraoperative ultrasound imaging in a case of Currarino's triad for the first time in the literature.
Malignant hypertension in a child with phakomatosis pigmentovascularis type II b

Kanaheswari Y, Hamzaini AH, Wong SW, Zulfiqar A

Acta Paediatr, 2008 Nov;97(11):1589-91.
PMID: 18671691 DOI: 10.1111/j.1651-2227.2008.00971.x

Phakomatosis pigmentovascularis (PPV) is a rare congenital syndrome characterized predominantly by cutaneous vascular malformations and pigmentary naevi. The most frequently reported form, Type II b, is associated with systemic involvement. Sturge-Weber Syndrome (SWS) with concomitant glaucoma, Klippel-Trenaunay Syndrome (KTS) and naevus of Ota have been frequently described, but there have only been two case reports with asymptomatic renal anomalies.
Fulltext Solitary vascular malformation of the clitoris

Haritharan T, Islah M, Zulfiqar A, Thambi Dorai CR

Med J Malaysia, 2006 Jun;61(2):258-9.
PMID: 16898327 MyJurnal

Isolated involvement of the clitoris by vascular malformation (VM) is very rare. Clinically, the lesion simulates female pseudohermaphroditism. A five-year-old girl presented with clitoromegaly and a clinical diagnosis of solitary VM of the clitoris was made. Magnetic resonance imaging showed characteristic features and confirmed the diagnosis and the extent of the VM. This is the first reported case of isolated involvement of the clitoris by VM to be diagnosed preoperatively.
Fulltext Anorectal anomalies in adult females corrected by posterior sagittal anorectoplasty

Thambidorai CR, Qureshi MA, Shukri J, Zulfiqar A

Med J Malaysia, 2005 Jun;60(2):226-8.
PMID: 16114166

Posterior sagittal anorectoplasty (PSARP) is preferred by most pediatric surgeon and intermediate types of anorectal anomalies (ARA) in infants. In this report, we describe two girls who presented in their late teens with ARA and were treated by PSARP. Prior to this report, only two adult females with congenital rectovaginal fistulae treated by PSARP have been reported. Megarectum is a feature in late presentation of ARA and requires rectal tapering during PSARP. The functional outcome in late presentation of ARA is discussed.
Currarino triad with dual pathology in the presacral mass: report of a case

Thambidorai CR, Muin I, Razman J, Zulfiqar A

Dis Colon Rectum, 2003 Jul;46(7):974-7.
PMID: 12847376

PURPOSE: Currarino triad, which comprises anorectal stenosis, anterior sacral defect, and a presacral mass, is an uncommon cause of constipation in children and adults. The presacral mass in this triad is most often caused by an anterior sacral meningocele, a teratoma, or an enterogenous cyst, but rarely may be caused by dual pathology. A neonate with Currarino triad and dual pathology in the presacral mass is described in this report.
METHOD: A male Chinese neonate, who presented with abdominal distention and constipation on the second day of life, was found to have features of Currarino triad. Colostomy was done in the neonatal period, and the presacral mass was excised by posterior sagittal perineal approach at the age of six months.
RESULTS: The excised presacral mass consisted of an anterior meningocele and a teratoma. The patient continued to have constipation during follow-up and required anorectoplasty to correct residual anorectal stenosis. At the time of this report the patient was three years old and growing normally with normal anorectal function.
DISCUSSION: Of a total of about 200 cases of complete Currarino triad found in the literature, in only 22 patients did the presacral mass contain both meningocele and teratoma. The features of these 22 patients and the current views on the surgical management of Currarino triad are discussed.
Computed tomography and ultrasonography: a comparative study in the diagnosis of analgesic nephropathy

Segasothy M, Abdul Samad S, Zulfiqar A, Shaariah W, Morad Z, Prasad Menon S

Nephron, 1994;66(1):62-6.
PMID: 8107955

Computed tomography (CT) and ultrasonography (US) were performed in 40 patients who had consumed excessive quantities of analgesics (> 1 kg) to compare their value in the diagnosis of analgesic nephropathy (AN). The computed tomography and sonographic features were renal papillary calcifications. Renal papillary necrosis (RPN) was documented in 20 of 40 patients by US and in 14 of 40 patients by CT. In 11 patients, both US and CT were positive. In 9 patients, US was positive whilst CT was negative. In 3 patients, CT was positive whilst US was negative. Prevalence of RPN was 50% using US and 35% using CT. Using US as a gold standard, sensitivity of CT was 55%, specificity 85%, positive predictive value 78.6% and negative predictive value 34.6%. Percent agreement with CT and US was 70%. Cohen's kappa statistic adjusting for chance agreement was 40%. Based on these results, it is found that US yielded a higher percentage of positive cases of RPN.
Analgesic use and chronic renal disease in patients with headache

Rahman A, Segasothy M, Samad SA, Zulfiqar A, Rani M

Headache, 1993 Sep;33(8):442-5.
PMID: 8262786

The pattern of analgesic use, abuse and incidence of analgesic-associated nephropathy in 79 patients with chronic headache was studied. Sixty-eight of these patients had migraine. Most patients had consumed a combination of analgesics (81%) while 19% had taken single analgesics for their headache. Nonsteroidal anti-inflammatory drugs were the most commonly used analgesics (96.2%) followed by paracetamol (70.9%) and aspirin, phenacetin and caffeine compounds (5.1%). Mefenamic acid was the commonest nonsteroidal anti-inflammatory drug consumed (97.4%). Analgesic abuse which was defined as a minimum total of 1 kg of analgesics such as paracetamol or aspirin, phenacetin and caffeine compounds or 400 capsules/tablets of nonsteroidal anti-inflammatory drugs was noted in 65 patients. Nonsteroidal anti-inflammatory drugs were the most commonly abused analgesics (89.2%) followed by paracetamol (38.5%). Forty-five of the 65 analgesic abusers had an intravenous urogram or ultrasound performed and renal papillary necrosis was documented in one patient. Three (4.6%) of the analgesic abusers had mildly raised serum creatinine levels. Mild proteinuria of less than 1 gm/litre was present in 27.7% of abusers. In conclusion, although analgesic use and abuse is common in patients with chronic headache, the short term incidence of analgesic-associated nephropathy (2.2%) and renal impairment (4.6%) was low. Prolonged observations will be necessary to ascertain the safety of these drugs for long term use.
Fulltext The use of interventional radiology in ruptured solitary kidneys

Lei CC, Khairullah A, Zulfiqar A, Samad AS

Med J Malaysia, 1992 Dec;47(4):320-2.
PMID: 1303487

This case report illustrates how a life-threatening renal bleeding which has failed to be controlled by open surgery can be elegantly managed by a minimally invasive technique of interventional radiology. It also allows maximal conservation of renal tissue so that the patient can avoid chronic dialysis or renal transplantation.
Fulltext Maternal, neonatal, and child health in southeast Asia: towards greater regional collaboration

Acuin CS, Khor GL, Liabsuetrakul T, Achadi EL, Htay TT, Firestone R, et al.

Lancet, 2011 Feb 05;377(9764):516-25.
PMID: 21269675 DOI: 10.1016/S0140-6736(10)62049-1

Although maternal and child mortality are on the decline in southeast Asia, there are still major disparities, and greater equity is key to achieve the Millennium Development Goals. We used comparable cross-national data sources to document mortality trends from 1990 to 2008 and to assess major causes of maternal and child deaths. We present inequalities in intervention coverage by two common measures of wealth quintiles and rural or urban status. Case studies of reduction in mortality in Thailand and Indonesia indicate the varying extents of success and point to some factors that accelerate progress. We developed a Lives Saved Tool analysis for the region and for country subgroups to estimate deaths averted by cause and intervention. We identified three major patterns of maternal and child mortality reduction: early, rapid downward trends (Brunei, Singapore, Malaysia, and Thailand); initially high declines (sustained by Vietnam but faltering in the Philippines and Indonesia); and high initial rates with a downward trend (Laos, Cambodia, and Myanmar). Economic development seems to provide an important context that should be coupled with broader health-system interventions. Increasing coverage and consideration of the health-system context is needed, and regional support from the Association of Southeast Asian Nations can provide increased policy support to achieve maternal, neonatal, and child health goals.
Fulltext Spontaneous closure of a persistent post-traumatic pancreatic fistula in a child: incidental therapeutic effect of ERCP

Thambidorai CR, Ismail S, Chan KY, Haron A, Zulfiqar A

Med J Malaysia, 2004 Mar;59(1):120-2.
PMID: 15535349

A child with a post-tramatic pancreatic pseuodocyst developed pancreatic fistula, following percutaneous drainage of the cyst. The fistula output was about 120ml/day and lasted for a month. A diagnostic ERCP revealed main pancreatic duct disruption. The fistula output ceased over the next two days following the ERCP. The patient has been well for the past four years. This patient's immediate recovery after ERCP could possibly be due to dislodgement of a mucus plug by the contrast injected during ERCP. Such a mechanism can explain the previous sporadic reports of spontaneous cure of persistent pancreatic fistulae in children.
Fulltext Ultrasound in the assessment of the palpable breast mass

Hasni H, Meah FA, Norlia A, Sharifah NA, Zulfiqar A

Med J Malaysia, 2004 Oct;59(4):486-94.
PMID: 15779581

The aim of the study was: to obtain the profile of patients (with regards to age and family history of breast cancer) with a palpable breast mass. To determine the validity of ultrasound in the assessment of the palpable breast mass by determining the sensitivity, specificity, positive predictive value, negative predictive value and accuracy of ultrasound in distinguishing a malignant mass. To determine the most discriminating ultrasound characteristics for differentiating benign and malignant masses. Seventy patients who had fine needle aspiration cytology of a palpable breast mass were subjected to an ultrasound assessment of the mass. The ultrasound findings were classified as benign, indeterminate or malignant. These findings were then compared with either the cytology or histology results in cases that eventually had surgical excision. The age of the patients ranged from 15 to 66 years old The majority was in the third and fourth decades with an average age of 25 years. The 8 patients with a proven malignant breast mass were aged between 39 and 66 years old. They did not have any family history of breast cancer. Only 4 patients had a family history of breast carcinoma and all proved to have a benign breast lesion. Ultrasound had a sensitivity of 100%, specificity of 85.7%, positive predictive value of 50%, negative predictive value of 100% and accuracy of 87.5% for distinguishing a malignant mass. For benign masses: 93.7% had well-defined margins, 81.3% had homogenous internal echoes, 91.7% had depth-width ratio of less than 1.0 and 89% were compressible. For malignant masses: 87.5% had either ill-defined or irregular margins, 87.5% had inhomogenous internal echoes and mixed posterior echoes, and 100% were incompressible. The majority of patients with a palpable breast mass were aged below 40 years old. Most of the patients with a malignant breast mass were aged 40 years and older. Neither a positive nor a negative family history of breast cancer had any significance on outcome. Ultrasound had high sensitivity, specificity and accuracy in distinguishing a malignant mass. The most discriminating benign ultrasound characteristic was compressibility. The most discriminating malignant ultrasound characteristic was ill-defined and irregular margins.
Fulltext Interstitial lung disease in children--a report of four cases

Norzila MZ, Azizi BH, Deng CT, Zulfiqar A

Med J Malaysia, 1997 Dec;52(4):429-32.
PMID: 10968122

Interstitial lung disease (ILD) is very rare in children. In the majority of cases the aetiology is unknown. Very little is known about the clinical course of this condition in children. Prognosis may be influenced by sex, age of onset of symptoms, radiographic features, presence of right ventricular hypertrophy and histopathology. We report our experience in managing four children with interstitial lung disease. All these children presented in early infancy with cough, respiratory distress, cyanosis and failure to thrive. Three of these children had finger clubbing and right ventricular hypertrophy. All patients received oral steroids. Chloroquine was added in two patients who showed no response. A trial of oral cyclophosphamide was started in one patient who failed with both drugs. One child is oxygen independent while another is on home oxygen therapy. The other two patients eventually died.
Local versus WHO/International Council for Control of Iodine Deficiency Disorders-recommended thyroid volume reference in the assessment of iodine deficiency disorders

Foo LC, Zulfiqar A, Nafikudin M, Fadzil MT, Asmah AS

Eur. J. Endocrinol., 1999 Jun;140(6):491-7.
PMID: 10366404

Iodine deficiency endemia is defined by the goitre prevalence and the median urinary iodine concentration in a population. Lack of local thyroid volume reference data may bring many health workers to use the European-based WHO/International Council for Control of Iodine Deficiency Disorders (ICCIDD)-recommended reference for the assessment of goitre prevalence in children in different developing countries. The present study was conducted in non-iodine-deficient areas in Malaysia to obtain local children's normative thyroid volume reference data, and to compare their usefulness with those of the WHO/ICCIDD-recommended reference for the assessment of iodine-deficiency disorders (IDD) in Malaysia.
Fulltext Prevalence, clinical predictors and diagnosis of gastro-oesophageal reflux in children with persistent respiratory symptoms

Siti Mazliah K, Norzila MZ, Deng CT, Zulfiqar A, Azizi BHO

Med J Malaysia, 2000 Jun;55(2):180-7.
PMID: 19839146

Objectives: This was a cross sectional study conducted in the Paediatric Institute among infants and children with chronic respiratory symptoms with the following objectives: i) to determine the prevalence of gastro-oesophageal reflux in children with persistent respiratory symptoms, ii) to identify the clinical predictors of GOR (Gastro-oesophageal reflux) in children with persistent respiratory symptoms and iii) assess the validity of abdominal ultrasound, barium oesophagogram and chest radiograph in diagnosing GOR in these patients.
Materials and Methods: Forty-four patients were recruited over a period of six months. All the presenting symptoms were identified. The patients were subjected to chest radiograph, abdominal ultrasound, barium oesophagogram and 24-hour pH oesophageal monitoring.
The predictive validity of clinical symptoms, chest radiograph, abdominal ultrasound and barium oesophagogram were assessed. Twenty-four hours oesophageal pH was the gold standard to diagnose GOR.
Results: The mean age of patients was 9.1 months (1-58 months). Thirty-one patients (70.5%) were confirmed to have GOR by pH study. Respiratory symptoms alone were not useful to predict GOR. Cough had the highest sensitivity of 51.6%. stridor, wheeze and choking each had a specificity of 76%. Wheeze, vomiting, choking and stridor were identified to have high specificity (90-100%) in diagnosing GOR when any two symptoms were taken in combination.
Collapse/consolidation was the commonest radiological abnormality but had low sensitivity (35.5%) and specificity (53.8%). However hyperinflation on chest radiograph had a specificity of 92.3% with positive predictive value at 80% in diagnosing GOR. Barium oesophagogram has low sensitivity (37.9%) and moderate specificity (75%) in diagnosing GOR in children with respiratory symptoms.
Abdominal ultrasound was a valid mode of diagnosing GOR when there were three or more reflux episodes demonstrated during the screening period with a specificity of 90.9%. However the sensitivity was low ie 20-25%. The specificity increased to 90-100% when two positive tests were taken in combination (abdominal ultrasound and barium oesophagogram). However the sensitivity remained low (10-20%). Chest radiograph did not improve the predictive value when considered with the above tests. Combination of clinical symptoms were useful as clinical predictors of GOR. In the absence of a pH oesophageal monitoring, a combination of barium oesophagogram and ultrasound may be helpful in diagnosing GOR.
Chronic nephrotoxicity of anti-inflammatory drugs used in the treatment of arthritis

Segasothy M, Chin GL, Sia KK, Zulfiqar A, Samad SA

Br J Rheumatol, 1995 Feb;34(2):162-5.
PMID: 7704463

We determined the consumption of non-steroidal anti-inflammatory drugs (NSAIDs) and the prevalence of chronic renal impairment and renal papillary necrosis (RPN) in patients with various types of arthritis. Ninety-four patients with chronic arthritis who had consumed more than 1000 capsules and/or tablets of NSAIDs were studied. Renal profiles and radiological investigations such as intravenous urogram (IVU), ultrasonography (US) and computed tomography (CT) were performed to look for evidence of RPN. Twelve patients did not complete the study. Ten of the 82 patients who had completed the study (12.2%) had radiologic evidence of RPN. Five out of 53 patients (9.4%) with rheumatoid arthritis, three out of 11 patients (27.3%) with gouty arthritis and two out of seven patients (28.6%) with osteoarthritis had RPN. Renal impairment (serum creatinine levels of 125-451 mumol/l) was found in 20 patients (24.4%). The patients had consumed 1000-26,300 capsules and/or tablets over a period ranging from 1 yr to more than 30 yr. Patients with chronic arthritis who consume excessive amount of NSAIDs are at risk of developing RPN and chronic renal impairment.

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