Some diseases may underlie finger clubbing. However, there is a dearth of information about early stage of finger clubbing because only few researchers have shown interest in it. We determined the Digital Index of normal, healthy subjects by using thread and manual Vernier calipers, the time used for the procedure, and its interrater reliability. The value of Digital Index was 8.86 ± 0.29 (Mean ± SD) with a range of 8.15 to 9.41. Interrater reliability was excellent with Pearson's correlation coefficient of 0.966. Overall, the time taken to measure the Digital Index ranged from 21.93 to 68.80 minutes with an average of 35.97 ± 9.16 (Mean ± SD). Determining Digital Index need much time, but this can be overcome if we use Digital Index Quantitator (DIQ). Availability of DIQ in the hospital wards will be of much benefit. DIQ can also be used to accurately quantify the progression or regression of the clubbing process. This article proves that we need morphometry of digital clubbing as well as the correlation of the physical sign of clubbing with Digital Index.
INTRODUCTION: Acute Promyelocytic Leukaemia (APL) is associated with devastating coagulopathy and life threatening condition which requires immediate medical attention. It is crucial to establish an expedited diagnosis as early therapeutic intervention has led to optimal patient management. In this study, we assessed the type and frequency of antigen expressions in APL and correlated these findings with genetic studies.
METHODS: Multiparametric immunophenotyping was performed on 30 samples and findings were correlated with karyotypes, FISH for t(15;17) translocation and RT-PCR for PML-RARΑ for detection of breakpoint cluster regions (bcr1,bcr2 and bcr3).
RESULTS: On SSC/CD45, APL cells displayed high to moderate SSC, with the expression of CD33 (100%), CD13 (96.8%), cMPO (71%) but lacked CD34 (3.2%) and HLA-DR (9.7%). Aberrant expression of CD4 was seen in 12.9% and CD56 in 6.5% of the cases. A significant association between cumulative aberrant antigen expression and bcr1 were observed bcr1 (X2(2) =6.833,p.05) and (X2(2)=4.599,p>.05) respectively.
CONCLUSIONS: Flow cytometry is a rapid and effective tool in detecting APL. It is interesting to note that there is significant association between cumulative aberrant antigen expression and genotype analysis. Further validation is required to corroborate this relationship.
Congenital vallecular cyst is an uncommon, benign but potentially dangerous condition causing respiratory distress and stridor. It is associated with sudden upper airway obstruction resulting in death due to its anatomical location in neonates and infants. We reported a rare case of 2 months old male infant presented with respiratory obstruction with failure to thrive with polydactyly (rarest finding) and managed timely with appropriate surgical marsupialization.
Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are a group of uncommon diseases. Given its rarity and multisystem clinical presentation which are usually serious and potentially fatal, prompt recognition and early treatment are of utmost importance. We report a case of AAV that presented with digital infarcts, cutaneous vasculitis, rapidly progressive glomerulonephritis, mononeuritis multiplex, eosinophilia and positive myeloperoxidase (MPO)-ANCA antibody. Apart from renal damage, there was complete recovery in other systems following intravenous cyclophosphamide and high-dose glucocorticoids albeit the response was delayed. This response obviates the need for second-line therapy with newer agent such as rituximab (anti-CD20 monoclonal antibody). We would also like to highlight that this is the first case of AAV that is associated with autoimmune thyroid disease to be reported from Malaysia.
Langerhans Cell Histiocytosis (LCH) is an uncommon neoplastic disorder characterized by accumulation of histiocytes in various tissues. The clinical manifestation is highly variable, ranging from an isolated skin rash or a single bony lesion, to fatal multi-organ failure. Due to its rarity and systemic involvement, the epidemiology of LCH is still not fully understood and most studies focus on the paediatric population. The extent of clinical involvement has important prognostic implications. Treatment options may be local or systemic depending on disease extent. Here we describe a rare case of adult onset LCH who presented with recurrent pneumothoraces, diabetes insipidus and papules and plaques over the scalp and forehead.
Traumatic chest injury with complete tracheo- bronchial disruption is uncommon and occurs in approximately 1% of motor vehicle accidents (MVA) (1,2). Such injuries carry a high mortality and patients rarely survive transfer to hospital. A high index of suspicion facilitates early diagnosis. Early operative intervention is vital for survival. We describe a rare case of traumatic complete disruption of the right mainstem bronchus (RMB) due to blunt chest trauma. The transected airway was reanastomosed emergently avoiding a lung resection.
OBJECTIVE: We here report the first study on the distribution of red cell antigens and phenotype frequencies of various blood group systems in Maldives.
METHOD: Randomly selected 123 regular blood donors of O group were phenotyped for seven blood group systems by direct tube agglutination and or indirect antiglobulin tests. Blood group systems studied were Rh, Kidd, Duffy, Lewis, Kell, P and MNS system.
RESULTS: Rh blood grouping showed, 7.3% donors were Rh(D) negative, 92.7% were Rh(D) positive with the predominance of genotype complex of DCe/DCe (39.0%). The incidence of Jk(a+b+) phenotype was the most common in Kidd system. In Duffy system, the incidence of Fy(a+b+) phenotype was 50.4%. Lewis system was predominated by Le(a-b+) phenotype accounting to 80.5% of the donors. In the Kell system only two phenotypes were present, K+k- (5.7%) and k+k+ (94.3%), in the Maldivian blood donors. P system was represented by P1, P2 and P2k phenotypes with an incidence of 28.5%, 70.7% and 0.8% respectively. In the MNS system, MNss and MNSs phenotypes summed up to 48.8% of blood donors.
CONCLUSION: The detail knowledge of red cell antigen composition and their frequencies in the Maldivian population will be helpful in terms of population genetic perspectives, in establishing a donor data-bank for in-house production of indigenous screening and identification cell panels, and facilitate availability of antigen negative compatible blood for patients with previously identified multiple alloantibodies.
INTRODUCTION: Percutaneous endoscopic gastrostomy (PEG) placement in patients with ventriculo-peritoneal shunt (VPS) may be associated with complications. This study reports our experience of PEG in patients with VPS.
MATERIALS AND METHODS: Consecutive patients undergoing PEG insertion in a gastroenterology unit over 18 month's period were retrospectively analyzed. All patients were evaluated by an attending gastroenterologist for fitness for procedure. Instructions were given for routine antibiotic prophylaxes before the procedure and continued for 48 hours. Patients were followed for immediate complications in particular, wound infection, signs of meningitis, deterioration in neurological state and shunt malfunction. Post discharge, patients were given routine follow-up for review.
RESULTS: Of 86 patients who had PEG inserted during the study period, 14 had VPS including 2 of which had VPS after PEG. The main common indications for VPS were intracerebral bleed and head trauma and for PEG were requirement of long term enteral feeding. Twelve patients had PEG at a mean interval of 61 days (range 1-187 days) after VPS. Of these, eight received prophylactic antibiotic or were on antibiotic for other indications before PEG. Two patients developed mild PEG site infections within a week of insertions, including one patient who was not given antibiotic prophylaxis, both treated successfully with antibiotics. The latter patient developed worsening hydrocephalus secondary to VPS blockage. At a mean follow-up period was 140 days (range 20-570 days), there were no death or further complications encountered.
CONCLUSIONS: Although safe in the majority of patients with VPS, PEG infection can lead to intracranial complications. We recommend antibiotic prophylaxis for VPS patients before PEG.
INTRODUCTION: Paraquat is a quaternary nitrogen herbicide which is highly toxic to human. Death is usually from respiratory failure and may occur within days up to a month after exposure. It is easily available and commonly abused to commit suicide.
METHODOLOGY: This is a retrospective study describing the demographic characteristics, clinical features and outcomes of paraquat poisoning cases admitted to Hospital Taiping from 1st January 2008 to 30th October 2011. Medical records of 79 patients were reviewed.
RESULT: Majority were of the Indian ethnicity (72.2%) followed by Chinese (13.9%) and Malay (10.1%). Majority was male (73.4%) and between 20 to 29 years old (34.2%). The median age of the patients was 30 years old. The mean length of stay was 6.2 days. Most exposures were intentional (69.6%) and presented to the hospital early at less than 6 hours after exposure (72.2%). Patients with positive urine paraquat result had significantly higher mortality rate compared to patients with negative results (47.4% vs 15.2% respectively). We found that neither hemofiltration nor immunosuppressive therapies help to improve survival.
CONCLUSION: The non-survivor characteristics of patients with paraquat poisoning are intentional exposure, delay from exposure to hospital admission, urine paraquat positivity and manifestation of respiratory failure. The demographic characteristics, reasons for exposure and mortality rate are similar to previous reports. Urine paraquat may be used to assess severity of the exposure as well as prognosis. Hemofiltration and immunosuppression therapy do not improve patients' survival and paraquat remains a lethal killer.
INTRODUCTION: Spontaneous rupture is a dramatic presentation of HCC and it carries high mortality rate. To study the outcomes of ruptured HCC patients managed at a tertiary referral centre in Malaysia.
METHODS: A retrospective review of all ruptured HCC patients managed as inpatient at the Department of Hepatobiliary Surgery, Hospital Selayang between January 2001 and December 2010. Data was retrieved from the hospital electronic medical records, Powerchart (Cerner Corporation Inc., USA) and supplemented with registry from Interventional Radiology record of chemoembolization and registry from hepatobiliary operative surgery records.
RESULTS: There were 22 patients admitted with confirmed diagnosis of ruptured HCC over 10 years period. The common clinical findings on presentation were abdominal pain and presence of shock (36.4%). The mortality rate was 81.8% with only four patients noted to be alive during the follow up. One year overall survival for ER and DR are 40.0% and 72.7% respectively and the median survival in patients treated with DR was 433.3 days whereas it was 212.5 days in ER group.
CONCLUSIONS: This study supports the clinical practice of TAE should be the first line treatment followed by staged surgery in suitable candidates with ruptured HCC.
INTRODUCTION: Diabetes care at different healthcare facilities varied from significantly better at one setting to no difference amongst them. We examined type 2 diabetes patient profiles, disease control and complication rates at four public health facilities in Malaysia.
MATERIALS AND METHODS: This study analyzed data from diabetes registry database, the Adult Diabetes Control and Management (ADCM). The four public health facilities were hospital with specialist (HS), hospital without specialist (HNS), health clinics with family physicians (CS) and health clinic without doctor (CND). Independent risk factors were identified using multivariate regression analyses.
RESULTS: The means age and duration of diabetes in years were significantly older and longer in HS (ANOVA, p< 0.0001). There were significantly more patients on insulin (31.2%), anti-hypertensives (80.1%), statins (68.1%) and antiplatelets (51.2%) in HS. Patients at HS had significantly lower means BMI, HbA1c, LDL-C and higher mean HDL-C. A significant larger proportion of type 2 diabetes patients at HS had diabetes-related complications (2-5 times). Compared to the HS, the CS was more likely to achieve HbA1c ≤ 6.5% (adjusted OR 1.2) and BP target < 130/80 mmHg (adjusted OR 1.4), the HNS was 3.4 times more likely not achieving LDL-C target < 2.6 mmol/L.
CONCLUSION: Public hospitals with specialists in Malaysia were treating older male Chinese type 2 diabetes patients with more complications, and prescribed more medications. Patients attending these hospitals achieved better LDL-C target but poorer in attaining BP and lower HbA1c targets as compared to public health clinics with doctors and family physicians.
Down syndrome (Trisomy 21) is the most common chromosomal abnormality among liveborn infants. It is the most frequent form of intellectual disability caused by a microscopically demonstrable chromosomal aberration. Management requires a multidisciplinary approach to the ongoing evaluation and monitoring for associated congenital anomalies and acquired disorders.Trisomy 21 is characterized by a variety of dysmorphic features, congenital anomalies and associated medical conditions. Knowledge of these associated conditions are important for clinicians involved in the management of these patients. Appropriate radiologic imaging with prompt, accurate interpretation plays an important role in the diagnosis and management of these diseases. The primary goal of this pictorial review is to unravel the radiological findings of these associated conditions.
In the past two decades, Fusarium species have been increasingly recognized as serious pathogens in immunocompromised patients. The outcome of fusariosis in the context of severe persistent neutropaenia has been almost universally fatal. The treatment of fusariosis in immunocompromised patients remains a challenge and the prognosis of systemic fusariosis in this population remains poor. This report presents a case of fatal fusariosis in a 37- year-old patient who was diagnosed with precursor-B cell Acute Lymphoblastic Leukaemia (ALL).
OBJECTIVE: Inherited anti-thrombin deficiency is an autosomal dominant disorder which is associated with increased risk for venous thromboembolism (VTE). This condition is very rare in Malaysia and there has been no documented report. Thus, the aim of the present study is to investigate the type of an inherited anti-thrombin deficiency mutation in a 25-year-old Malay woman who presented with deep vein thrombosis in her first pregnancy.
METHODS: DNA was extracted from the patient's blood sample and buccal mucosal swabs from family members. Polymerase chain reaction(PCR) assays were designed to cover all seven exons of the serpin peptidase inhibitor, clade C (antithrombin), member 1 (SERPINC1) gene; and the products were subjected to DNA sequencing. Sequences were referred to NCBI Reference Sequence: NG_012462.1.
RESULTS: A heterozygous substitution mutation at nucleotide position 13267 (CCT->ACT) was identified in the patient and two other family members, giving a possible change of codon 439 (Pro→Thr) also known as anti-thrombin Budapest 5. The genotype was absent in 90 healthy controls.
CONCLUSION: The study revealed a heterozygous antithrombin Budapest 5 mutation in SERPINC 1 giving rise to a possible anti-thrombin deficiency in a Malay-Malaysian family.