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.

Frequency of Early-onset Neonatal Sepsis Following Prolonged Rupture of Membranes.

Introduction In developing countries, sepsis and associated mortality rates in neonatal patients is a serious concern. To improve the outcomes and mortality posed by sepsis, physicians need to know the local epidemiology of the microbial pathogens and their resistance patterns to antimicrobial agents. Therefore, our aim was to determine the frequency of early-onset neonatal sepsis (EONS) following prolonged rupture of membranes (PROM). Materials and methods After approval from the ethical review committee, this cross-sectional study was conducted at a tertiary care hospital of a developing country, and informed consent was taken from patients' parents. All neonates born to a mother with PROM after 24 weeks of gestation up to seven days of life were included. Demographic features, signs of sepsis, blood culture results, and laboratory markers of sepsis were recorded. All data were analyzed by using IBM SPSS Statistics for Windows, Version 20.0 (IBM Corp., Armonk, NY). Results A total of 124 patients were enrolled in the study. Seven neonates (5.6%) developed EONS and positive cultures were seen in four neonates (3.2%) with a maternal history of PROM. The organisms identified in cultures were Klebsiella pneumonia, group B streptococcus, Staphylococcus aureus, and Streptococcus species in EONS caused by prolonged PROM. Conclusions Early recognition of risk factors, recognition of clinical conditions with prompt laboratory screening for infection, and early establishment of empirical antibiotic treatment are effective preventive measures. Such approaches would be a secure and efficient strategy, particularly in developing countries.

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.

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.

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.

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.

Unusual Presentation of Dengue Fever: A child with acute myocarditis.

Dengue fever (DF) is an acute febrile illness that follows a self-limiting course. However, some patients suffer from complications, including myocarditis, due to the involvement of other organs. A child presented at the Aga Khan University Hospital in Karachi, Pakistan, in June 2013 with a high-grade fever, malaise and epigastric pain radiating to the chest. Positive DF antigen and immunoglobulin M assays confirmed the diagnosis of DF. Persistent bradycardia with low blood pressure led to further cardiac investigations which showed a decreased ejection fraction and raised serum cardiac enzymes, indicating myocardial damage. With supportive care and use of inotropes, the spontaneous normalisation of cardiac enzyme levels and ejection fraction was observed. The child was discharged five days after admission. This case highlights the importance of identifying myocarditis in DF patients suffering from cardiac symptoms that are not explained by other potential aetiologies. Awareness, early suspicion and supportive care are essential to ensure favourable outcomes.

Liddle's Syndrome.

Hypertension in paediatric age group is commonly secondary to a known cause. It is crucial to identify the cause of hypertension and treat it before development of any associated complications to prevent morbidity and mortality. Paediatric Hypertension is one of the important clinical finding in a child with certain clinical syndrome. We are presenting a case of a 10 month old child presenting with hypertension and hypokalaemia, after excluding all identifiable causes and her positive response to therapy, that is amiloride, along with supportive biochemical data she was diagnosed as a case of monogenic type of hypertension known as Liddle's syndrome.

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.

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.

Oesophageal duplication cyst presenting as haemoptysis.

Duplications of the alimentary tract include a variety of cysts, diverticula, and tubular malformations, all believed to have embryological origin. The cysts are most commonly found in children, and the diagnosis is made in infancy in the majority of patients. We report a case of a two-and-a-half year old child, presenting with the history of repeated episodes of haematemesis. Upper GI endoscopy was unremarkable and the chest x-ray showed no pathology. Computed tomography (CT) angiogram revealed soft tissue density lesion in the right chest at the level of T6. Right thoracotomy suggested a cystic mass close to the oesophagus which was shown on histopathology to be lined with gastric mucosa consistent with oesophageal duplication cyst. To the best of our knowledge, this is the first case of its kind reported from Pakistan.

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.

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.

Child with idiopathic pulmonary hemosiderosis: a case report from Pakistan with multiple ethical and moral issues.

This report discusses the case of a young Pakistani child diagnosed with idiopathic pulmonary hemosiderosis (IPH). The key features of IPH were iron deficiency anemia and pulmonary symptoms due to recurrent pulmonary hemorrhages. The child showed complications of the disease process because of late diagnosis. Because various ethical and moral issues were associated with the diagnosis and management of IPH, this case provides insights about the care burden of health care professionals and a child's parents in a Pakistani pediatric setting. During the course of the child's treatment at one of the private tertiary care settings of Karachi, Pakistan, the key challenges were as follows: declaring the diagnosis to the parents, dealing with the request of the child's parents for withdrawal of ventilatory support and withholding treatment, deciding the code status of the child, and ensuring the quality of the child's life after discharge from the hospital. It was learned from this case report that shared decision making and open communication with the child's family enabled the pediatric health care professionals to determine what was in the best interest of the child, resulting in provision of effective palliative care to the child. Moreover, it was realized that early detection of the disease and availability of hospice care can facilitate palliative care of children diagnosed with IPH.

Idiopathic pulmonary haemosiderosis presenting as severe iron deficiency anaemia--a case from Pakistan.

Idiopathic pulmonary haemosiderosis is an uncommon disorder, which is characterized by iron deficiency, recurrent haemoptysis and diffuse parenchymal infiltration on chest radiograph. We report an 8 year old child who had past history of multiple blood transfusions with diagnosis of iron deficiency anaemia and recurrent chest infection since the age of 21/2 year. At the age of 8 years, the child presented with fever and severe respiratory distress requiring intubation and ventilation. On Chest X-ray, bilateral white out was found and CT scan lung showed diffuse alveolar involvement. Lung biopsy confirmed haemosiderin-laden macrophages. Child was put on steroids and despite severe anaemia (Hb 3.2 g/dl), he showed improvement and survived. To our knowledge, this is the first case of idiopathic pulmonary haemosiderosis reported from Pakistan.

Significance of pyuria in the diagnosis of urinary tract infections in neonates.

OBJECTIVES: To determine the significance of pyuria as a predictor of culture proven urinary tract infections (UTI) in neonates and to assess the frequency of urinary tract anomalies in neonates with pyuria. METHODS: Prospective study conducted at the neonatal intensive care unit of a tertiary care hospital; (Liaquat National Hospital Karachi) for a period of 4 months from April 2008 to August 2008. One hundred and ten consecutive infants < or = 28 days of age admitted to the NICU of Liaquat National Hospital for medical reasons were included in the study. Information regarding age, gender, antenatal history, birth weight, clinical examination, laboratory findings and outcome were recorded on a questionnaire. Septic work up was performed and urine samples were collected using urethral catheterization. Patients showing any number of white blood cells on microscopy were included and their urine sent for culture. All patients had Ultrasound done during their hospital stay and those patients with any renal abnormality were further investigated with Micturating cyestoretherogram (MCUG) at 6 weeks. RESULTS: Out of one hundred and ten patients admitted, thirty five patients showed the presence of pus cells in urine and were included in the study. Of the 35 neonates with pyuria, 71.4% had no growth in urine cultures and 38.2 % neonates with insignificant pyuria (< or = 9 cells in urine) showed a positive culture. The renal ultrasound was normal in 51.4% neonates with pyuria although it was abnormal in 100% of the subjects with higher number of pus cells in urine (> 20 pus cells). CONCLUSION: Pyuria is not a useful marker for the diagnosis of culture proven UTI in neonates it cannot be used as an indicator of underlying renal abnormality, though it may have some utility in neonates with > 20 /numerous pus cells.

Aluminium phosphide poisoning: a case report.

This paper reports the case of a family in which three children were presented at Emergency Room (ER) with poisoning after the use of a pesticide at home. Initially, the cases were managed as routine cases of organophosphorus poisoning; however, the death of two children made the health team members realise that the poison's effects were delayed and devastating. Later, the compound was identified as Aluminium Phosphide (ALP), and the life of the last surviving child in the family was saved.

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.

Rosai Dorfman disease.

We report an unusual presentation of sinus histiocytosis with massive lymphadenopathy (SHML), also called Rosai Dorfman disease. The child presented with bilateral proptosis with massive cervical lymphadenopathy. The patient had fair response to steroid therapy but relapsed during the course of the disease.