7-Month-Old Infant with Cushing's Disease

Cushing's disease in children is not rare but in infants it is quite rare and an important medical condition needing proper line of investigations and management options. Craniopharyngioma as a cause of Cushing's disease is well reported and practical inference of the condition is of clinical importance. Craniopharyngioma generally affects children at 5 - 10 years of age and is rarely seen in infancy. It usually manifests as endocrinological deficits such as short stature, delayed puberty, and obesity. We report the case of a 7 months old infant who presented with obesity and Cushing's disease associated with Craniopharyngioma

Pheochromocytoma: a rare cause of childhood hypertensive encephalopathy

Pheochromocytomas are rare neuroendocrine tumours of chromaffin tissues. They are catecholamine secreting tumours which cause severe hypertension and other systemic disturbances. Of all the causes of childhood hypertension, pheochromocytoma constitutes less than 1%. We report the case of a 12 years old child who presented with hypertensive encephalopathy, confirmed histologically to be secondary to pheochromocytoma, and cured with meticulous critical care and surgical resection

Ventilator-associated pneumonia in children

To determine the frequency of Ventilator-Associated Pneumonia [VAP] and to identify the associated factors, causative organisms and outcome of VAP in children admitted to ICU. Cross-sectional, observational study. Medical ICU [MICU] of the Children/'s Hospital and Institute of Child Health, Lahore, from August 2008 to March 2009. All children admitted to MICU and requiring ventilation during the study period were included and monitored for any features suggestive of VAP. Partial septic screen was done in all suspected cases. VAP was labelled when any patient on the ventilator for more than 48 hours had at least 2 of the following features of nosocomial infection - fever > 101'F, TLC < 4000 or > 15000 per mm[3], neutrophils > 85%, CRP > 48 mg/L or new findings on chest examination suggestive of pneumonia' and radiological evidence of new or progressive and persistent infiltrates. Percentages were compared using chi-square test with the significance at p-value less than 0.05. Of the 93 children requiring mechanical ventilation during the study period, 16 developed VAP [17%]. Almost half [46%] were younger than 1 year with male to female ratio of 1.2:1. Children developing VAP required ventilation for 13.5 [+ 10.1] days compared to 7.7 [+ 5.5] days in those who did not develop VAP. The common organisms isolated were Pseudomonas, Klebsiella and E. coli. Factors associated with increased frequency of VAP included age less than 1 year, unplanned emergency intubation and use of continuous intravenous sedation. Features that strongly suggested underlying VAP included purulent tracheal secretions compared to increased secretions alone, CRP > 48 mg/L, positive radiological findings and positive tracheal aspirate culture. Overall mortality was 23% among the ventilated cohort. Thirty two percent of them had VAP compared to only 13% among those who survived to discharge [p = 0.03]. The frequency of VAP was 17% in this series. Factors significantly associated with VAP were age less than 1 year, unplanned intubation and continuous sedation. The important predictors of VAP included purulent tracheal secretions, high CRP and persistent new radiological findings

Clinical profile of subacute sclerosing panencephalitis

To describe the clinical manifestations of subacute sclerosing panencephalitis in children. Case series. This study was conducted in the Department of Neurology at The Children's Hospital and the Institute of Child Health, Lahore, from April 2005 to April 2007. Fifty patients were diagnosed as subacute sclerosing panencephalitis during the study period. Their diagnosis was based on a detailed history, clinical examination, presence of antimeasles antibodies in Cerebrospinal Fluid [CSF] and typical electroencephalogram [EEG]. The findings were described as average, mean and percentages. Fifty patients were included in this study. The average age of the patients was 8 years. Thirty-eight [76%] were males and 12 [24%] were females. The average duration of symptoms before presentation was 66.72 days. History of measles infection was present in 31 patients [62%] and measles vaccination in 43 patients [86%]. Motor regression was present in all [100%] patients and cognition decline in 43 patients [86%]. Seizures were focal [10%], generalized tonic-clonic [16%] and myoclonic [74%]. Burst-suppression pattern Electroencephalogram [EEG] and the antimeasles antibody in CSF were positive in 100% of patients. SSPE is an indicator of high incidence of measles infection among the paediatric population even among vaccinated children. Males are more common sufferers. SSPE can present with different types of seizures, cognition decline and motor regression being supported by suggestive EEG and presence of anti-measles antibodies in CSF

Predictors of intractable childhood epilepsy

To determine the prognosis of seizures in epileptic children and identify early predictors of intractable childhood epilepsy. Case-control study. The Epilepsy Centre of the Children's Hospital Lahore, from February 2005 to April 2007. All children [aged 1 month to 16 years] with idiopathic or cryptogenic epilepsy who were treated and followed at the centre during the study period were included. The patients who had marked seizures even after two years of adequate treatment were labeled as intractable epileptics [cases]. Children who had no seizure for more than one year at last follow-up visit were the controls. Adequate treatment was described as using at least three anti-epileptic agents either alone or in combination with proper compliance and dosage. Records of these patients were reviewed to identify the variables that may be associated with seizure intractability. Of 442 epileptic children, 325 [74%] intractable and 117 [26%] control epileptics were included in the study. Male gender [OR=3.92], seizures onset in infancy [OR=5.27], >/= 10 seizures before starting treatment [OR=3.76], myoclonic seizures [OR=1.37], neonatal seizures [OR=3.69], abnormal EEG [OR=7.28] and cryptogenic epilepsy [OR=9.69] and head trauma [OR=4.07] were the factors associated with intractable epilepsy. Seizure onset between 5-7 years of age, idiopathic epilepsy, and absence seizures were associated with favourable prognosis in childhood epilepsy. Intractable childhood epilepsy is expected if certain risk factors such as type, age of onset, gender and cause of epilepsy are found. Early referral of such patients to the specialized centres is recommended for prompt and optimal management

Brain Abscess in children

To find out the clinical presentation, radiological characteristics, various underlying predisposing conditions and causative organisms of brain abscess in children in our setup. Descriptive study. The Children's Hospital and the Institute of Child Health, Lahore, over two years from September 2001 to August 2003. All children [<16 years] presenting with brain abscess were included to study demographic, clinical and radiological features. In addition, attempts were made to find out underlying predisposing conditions and causative organisms. Twenty-five children with brain abscess were managed over 2 years. The mean age was 7.8 years [range 9 months to 16 years]. Male to female ratio was 2.1:1. Most patients [43%] presented with 4 weeks history of illness, with mean duration of illness at presentation of 29.3 days. Main presenting complaints were fever [72%], vomiting [48%], headache [44%] and convulsions [32%]. Five patients [20%] had papilledema at presentation, another 4 [16%] had paresis/paralysis and 3 [12%] had cranial nerve palsies. Majority [64%] had solitary abscess, located in parietal, temporal, frontal and occipital lobes in order of frequency. No underlying predisposing condition was identified in 8 [32%] cases; while 8 [32%] had cyanotic congenital heart disease, 5 [20%] patients had otic infection [mastoiditis], 2 [8%] were postoperative cases and one each developed brain abscess secondary to ventriculo-peritoneal [VP] shunt infection and pulmonary tuberculosis. Causative organisms were isolated in 40% cases, which included Staphylococcus aureus, Staphylococcus epidermidis, Streptococcal species, Klebsiella, E.coli and Proteus. Awareness of predisposing factors, early recognition of clinical features and understanding of the prevalent microbial profile is imperative for better management of children with brain abscess