Idiopathic pulmonary hemosiderosis - A rare cause of chronic anemia.

Idiopathic pulmonary hemosiderosis (IPH) is a rare disease marked by alveolar bleeding and accumulation of hemosiderin in the lungs. Here we present three cases of IPH. The first case is of a 26-year-old male with anemia, hemoptysis and dyspnea. Bronchoscopy confirmed diffuse alveolar hemorrhage (DAH). A diagnosis of IPH was made after ruling out other causes of DAH and observing good response to steroids. The patient's condition improved with prednisolone and azathioprine. The second case is of 26-year-old female with severe anemia. Imaging suggested IPH and lung biopsy confirmed it. She died shortly afterwards. The third case is of a 7-year-old male with chronic anemia. CT was suggestive of IPH and lung biopsy confirmed the diagnosis. Later, patient developed posterior reversible encephalopathy syndrome (PRES). This patient is stable on azathioprine and prednisolone. We aim to emphasize the importance of considering IPH as a differential in patients with DAH or chronic anemia.

Needs assessment for curricular reform of a post graduate paediatric training programme at a private university hospital in Karachi, Pakistan.

In order to make and sustain changes in the curriculum, needs assessment is the key. We conducted a need assessment survey using a questionnaire based on a 7 point Likert Scale by residents and alumni. Over all response rate in the study was 77.5%. Fifty eight percent participants were females. Highest level of agreement (mean score 5.7) was found between the alumni and residents regarding the need to revise the content, teaching and assessment strategies in the programme. Twenty seven alumni (n= 38, 71%) had cleared their FCPS exams. The alumni thought that the existing teaching and assessment strategies do not facilitate in passing professional certificate exams (mean score 3.6). The mean attempts required by the alumni for clearing this exam was 3.3, and 84.2% considered their weak clinical skills as the reason for their recurrent failure.

Spectrum, management and outcomes of structural and functional uropathies in children attending a tertiary care center in Karachi; Pakistan.

Causes and outcomes of children diagnosed with hydronephrosis in resource-limited countries with a low utilization of antenatal ultrasonography remain unexplored. We performed a retrospective, crosssectional study of all paediatric patients diagnosed with hydronephrosis and managed at a tertiary care center in Karachi, Pakistan between 2005 and 2010. Data relating to demographics, clinical features, etiologies and treatment modalities were systematically collected. Of a total of 234 cases (74.4% male), 83 (35.5%) and 42 (17.9%) were neonates and infants respectively. Congenital urinary tract pathologies were noted in 192(72.2%) patients, of which only 96(50%) had undergone foetal ultrasonography and 77(40.1%) first presented after the age of 1 year. At a median follow-up of 4 years, 24(12.5%) of these patients had evidence of renal dysfunction. Worse urologic outcomes in this study were most likely attributable to delayed diagnosis of congenital urinary tract abnormalities.

MUMPS MYOCARDITIS: A FORGOTTEN DISEASE?

Mumps is an acute viral illness that follows a self-limiting course but up to 10% of cases have a complicated course with the involvement of other organ systems. Myocarditis is reported as a complication but the incidence has greatly fallen ever since the development of the mumps vaccine. A child presented to our department with parotid swelling and fever. Persistent tachycardia with irregular pulse led to further cardiac work up which showed decreased ejection fraction and raised serum cardiac enzymes, indicating myocardial damage. With ionotropic agents and supportive care, there was complete normalization of ejection fraction and serum cardiac enzyme levels. He was discharged within a week of admission. This case highlights the importance of suspecting myocarditis in the setting of mumps, a diagnosis that precludes early suspicion in mumps patients suffering from cardiac symptoms not explained by other potential aetiologies. Early suspicion and timely supportive care are essential to ensure favourable outcomes.

Comparison of clinical and CSF profiles in children with tuberculous and pyogenic meningitis; role of CSF protein: glucose ratio as diagnostic marker of tuberculous meningitis.

OBJECTIVES: To compare the clinical and laboratory features of tuberculous meningitis with bacterial meningitis and to determine if protein glucose ratio in the cerebrospinal fluid can be predictive of the condition. METHODS: It was a retrospective descriptive study at the Paediatric Ward of Aga Khan University Hospital, Karachi, for which records of 46 patients with tuberculous meningitis and 33 with bacterial meningitis were reviewed. All records related to the study period that was from January 2005 to October 2010. The records were retrieved in December 2010. Tuberculous meningitis was defined as illness > or = 14 days, basal enhancement or tuberculoma on computerised tomography scan and response to anti-tuberculous therapy. Pyogenic meningitis was defined by the presence of a pathogenic isolate inthe cerebrospinal fluid (bacterial culture) or by positive latex particle agglutination or a pathogen on cerebrospinal fluid gram stain and clinical improvement with antibiotics. Logistic regression was used to model the probability of having tuberculous meningitis. To define the optimal protein-glucose ratio, cutoff point for the diagnosis of tuberculous meningitis, a receiver operating characteristic curve was applied. All analysis were done using SPSS 16. RESULTS: Features predictive of a diagnosis of tuberculous meningitis were protein:glucose ratio of > or = 2 (OR 21 95% CI 4.7-93); cerebrospinal fluid total leukocyte count < 800 (OR 58, 95% CI 5-649); and the presence of hydrocephalus (OR 19, 95% CI 3.3-109). CONCLUSION: A set of simple clinical, laboratory and radiological criteria can help in predicting tuberculous meningitis. The value of cerebrospinal fluid protein:glucose ratio needs to be validated in larger studies with bacteriologically-confirmed cases of tuberculous meningitis.

Trend of complementary therapies in paediatric age group.

OBJECTIVE: To assess the knowledge of mothers regarding commonly used Complimentary alternative medicines, their usage and various therapeutic choices for common ailments. METHODS: A cross-sectional Survey on a predesigned proforma was done from July 2007- August 2007 in the out patient department of a tertiary care hospital. Consent was taken from the participants and 375 complete proformas were included in the study out of 500 interviewed. RESULTS: Of the 375 mothers who were interviewed, 237 (63.2%) believed in alternative medicine whereas 138 (36.8%) did not believe in it. Complimentary alternative medicines (CAM) was used most often in children with respiratory tract and gastro intestinal symptoms, the most frequently used therapy was Honey 58.9% and Joshanda 31.2%, followed by Heeng 11.3% and brandy being used in 2.8% for respiratory complaints. Gastrointestinal symptoms were treated with Saunf water 28.4%, mint 20.6% and ajwain 17.7%. Higher education level was associated with more complementary medicine use. Majority of mothers used it for gastrointestinal problems and respiratory problems 24% and 22.2% respectively. CONCLUSIONS: Mothers are aware of various available CAM and are using them for different ailments like honey for respiratory illnesses, Saunf and Ajwain for gastrointestinal problems as they have been found to have linctus and antispasmodic effects respectively. It has also been observed that the CAM was used in combination with other therapeutic modalities and not alone.

Brown-Vialetto-Van Laere syndrome: a novel diagnosis to a common presentation.

Brown-Vialetto-Van Laere syndrome (BVVLS) or riboflavin transporter deficiency (OMIM 211530) is a rare treatable autosomal recessive neurodegenerative disorder. This condition is associated with progressive pontobulbar palsy. We describe the clinical course of a 16-month-old boy with BVVLS and a novel homozygous mutation from Pakistan. Our patient presented with stridor and respiratory insufficiency. Hearing loss which is the most common sign of this condition was absent, making it an unusual presentation of BVVLS. His examination revealed ptosis and tongue fasciculation. His riboflavin receptor mutational analysis showed the homozygous mutation in the SLC52A3 gene. Per oral riboflavin was administered, and subsequently, he was able to be weaned off the ventilator. Now the child is improving and attaining developmental milestones.

Frequency of cardiorenal syndrome type-I in hospitalized children with acute heart failure in a tertiary-care hospital.

OBJECTIVE: To determine the frequency of cardiorenal syndrome in hospitalized children with acute heart failure. STUDY DESIGN: Descriptive study. PLACE AND DURATION OF STUDY: Paediatric Intensive Care Unit, The Aga Khan University Hospital, Karachi, from December 2010 to December 2011. METHODOLOGY: Sixty eight (68) children with acute heart failure fulfilling the selection criteria were evaluated for worsening of renal function (WRF). Serum creatinine was done at baseline and repeated at 72 hours to see the worsening of renal function. Estimated serum creatinine clearance was calculated by Schwartz formula. RESULTS: Mean age of patients was 43.6 ± 55.2 months. There were 43 (63%) males, 70% were under 57 months of age. Mean weight on admission was 14.7 ± 19.13 kg and mean height was 83 cm (± 31.08 SD). Mean serum creatinine on admission was 0.77 mg/dl (± 1.18 SD). Worsening renal function was noted in 55 (81%) of children, out of those, majority 36 (70.5%) were under 5 years of age. CONCLUSION: Worsening renal function was found in 81% of children admitted with the diagnosis of acute heart failure. Majority (70.5%) were under 5 years of age indicating a closer observation of renal status in younger age group to reduce, morbidity and mortality.

Treatment of Burn Wound Infection Using Ultraviolet Light: A Case Report.

A 9 year old girl presented to us with complaints of fever and pain in burn wounds with deteriorating health for one month. According to Lund and Broder's chart, burns spanned the posterior trunk (13%), right arm (1.5%), left arm (1.5%), and buttocks (2.5%). The wounds showed improper healing. She had previously underwent split-thickness skin grafting, using skin harvests from thighs and antimicrobial therapy with vancomycin, fluconazole and colomycin with limited clinical improvement. Analgesia was administered. Blood cultures and tissue cultures from the burns indicated polymicrobial wound infection and sepsis, including methicillin resistant Staphylococcus aureus, Klebsiella pneumoniae and Pseudomonas aeruginosa. Despite broad-spectrum antibiotics, fever persisted and condition deteriorated. Antifungals were also administered with no clinical improvement. Eventually another split-thickness skin grafting was done to provide fresh grafts. In due course, ultraviolet light exposure, of wavelength 32-40 nm/W/cm(2), was considered for treatment. In prone position, the wounds were exposed to ultraviolet phototherapy 6-8 h daily for 8 days. Eventually, wound healing and sepsis improved. Antibiotics were optimized and high protein diet was started. Eventually the wounds showed fresh margins and visible signs of healing. With remarkable clinical improvement and no further fever spikes, the patient was eventually discharged. She was advised to shower regularly, apply bandages with acetic acid. On her last outpatient follow up, 2 weeks after discharge, she was doing well, with no complaints of pain or fever. Examination of burns showed clean wounds, with clear margins and good graft uptake. She did not require any further grafting or surgical procedures thereafter.

Prevalence of hypertension in healthy school children in Pakistan and its relationship with body mass index, proteinuria and hematuria.

To determine the prevalence of high blood pressure (BP) in healthy school Pakistani children and its association with high body mass index (BMI), asymptomatic hematuria and proteinuria, we studied 661 public school children and measured their body weight, height and BP and urine dipstick for hematuria performed on a single occasion. Hypertension (BP >95 th centile) and pre-hypertension (BP >90 th centile) were defined based on the US normative BP tables. Over-weight and obesity were defined according to the World Health Organization (WHO) classification of BMI. The mean age of the children was 14 ± 1.3 years. The mean BMI was 18.5 ± 4.3 kg/m 2 . The majority (81.8%) of the children were found to be normotensive (BP <90 th centile). Pre-hypertension was observed in 15% and hypertension in 3% of the children. Overweight was observed in 7.7% and obesity in 1% of the children. The independent risk factors for hypertension and pre-hypertension were age of the child (RR 1.2 95% CI 1-1.4), gender (RR 2.0 for being female 95% CI 1-4.4), BMI >25 (RR for BMI b/w 25-30 = 2.6, RR for BMI >30 = 4.3), positive urine dipstick for proteinuria (RR = 2.3 95% CI 0.7-7.7) and positive urine dipstick for hematuria (RR 1.0 95% CI 0.2-8.3). Hypertension in children is strongly correlated with obesity, asymptomatic proteinuria and hematuria. Community based screening programs for children should include BP recording, BMI assessment and urine dipsticks analysis and approach high-risk groups for early detection and lifestyle modifications.