Aplasia cutis congenita with rare association and unusual presentation

Aplasia cutis congenita [ ACC] is a congenital absence of skin most commonly affecting scalp. In very rare instances it can involve extremities and trunk. It is usually diagnosed clinically at birth. Most cases are sporadic with a few reports of familial occurrence. Here, we present two cases of which one had scalp involvement correlating to antenatal methimazole intake and the other one had unusual presentation with involvement of deep tissues up to the periosteum of right lower limb. Both cases were managed conservatively and successfully discharged home. But unfortunately were lost to follow-up. ACC is a rare condition with varying degree of severity depending upon the extent and depth of the involvement of the skin and underlying tissues. Mild cases do not need specific intervention while severe cases need to be managed by a multidisciplinary team of pediatrician, dermatologist and plastic surgeon. Methimazole is a known teratogen associated with this condition hence should be avoided in pregnancy

Gullian-barre syndrome with bilateral facial nerve palsy.a rare entity

Bilateral facial nerve paralysis, as compared to unilateral palsyis an extremely rare presentation with serious aetiologies including post infectious like in Guillain-Barre syndrome, infectious including Lyme disease, Infectious Mononucleosis, autoimmune, malignancy [leukaemia] and traumas

We present a rare case of bilateral facial paralysis due to Guillain-Barré syndrome which was successfully managed at Department of Paediatrics, Liaquat National Hospital. Patient presented in June 2015, she is on regular follow-up with last visit in march 2016

Success of locally devised continuous positive pressure ventilation [CPAP] alone or in combination with surfactant therapy in the management of respiratory distress syndrome in preterm neonates

Objective: To study the effect of implementing locally devised CPAP alone or in combination with INSURE protocol [Intubation, Surfactant, Extubation] in preterm neonates with respiratory distress syndrome in a tertiary care hospital

Methods: The study was conducted from August 2012 to August 2014 in Neonatal Intensive Care Unit [NICU] of Liaquat National Hospital, Karachi. Total 113 neonates were enrolled in the study. They were grouped in two categories on the basis of severity of respiratory distress syndrome [RDS]. Infants with clinical evidence of respiratory distress and radiological evidence of patchy reticulogranularpattern were given a trial of bubble CPAPalone. Neonates with severe RDS were given INSURE protocol along with bubble CPAP. The intervention was considered to be successful if respiratory distress improved and the baby could be successfully weaned off from CPAP

Results: In this study 113 neonates were enrolled. Fifty neonates were initially managed with bubble CPAP alone out of which 29 [58%] improved. Neonates with severe RDS were 63 and received bubble CPAP along with INSURE protocol out of which 45 [71.4%] improved

Conclusion: Locally devised Bubble CPAP along with selective surfactant therapy is a safe and effective intervention in the management of respiratory distress syndrome in preterm. It decreases the need for mechanical ventilation and its subsequent complication

Vitamin D levels in children with growing pains

To estimate the serum levels of vitamin D in children with growing pains and determine the relationship between serum vitamin D levels, parathormone and routine biochemical markers. Cross-sectional study. Department of Paediatrics, Liaquat National Hospital, Karachi, from October 2008 to September 2009. Hundred children, aged 5-12 years presenting in Paediatric Outpatient Department of Liaquat National Hospital, Karachi, with limb pains, fulfilling the diagnostic criteria of growing pains, were included. Children with any systemic illness, organic cause of pain, rheumatologic disorders and signs of rickets were excluded from the study. Children were investigated for serum total calcium, inorganic phosphorus, alkaline phosphatase, vitamine D3 [25-hydroxecholecalciferol] and parathromone levels. On the basis of serum vitamin D3 level, patients were divided into 3 groups; group 1 with normal level of vitamin D3 [> 75 nmol/L], group 2 with vitamin D insufficiency [level between 50-75 nmol/L], and group 3 with vitamin D deficiency [level <50 nmol/L]. Significance of group proportions was determined using chi-square test with significance at p< 0.05. The mean age of the participants was 8.05 years with the majority [59%] being females. Only 6% had normal vitamin D levels. Over 95% of the children with vitamin D insufficiency had normal alkaline phosphatase and parathormone levels. Hypovitaminosis D may have a role in pathogenesis of growing pains. All children with unexplained limb pains without identifiable organic pathology should be tested for vitamin D status, and treated, if necessary. Routine biochemical markers alone are not sufficient to detect all cases of hypovitaminosis D

Caesarean section on demand? should we do it?

This article gives food for thought regarding the implications of the rising rate of caesarean section on demand. The obstetricians concerned should curtail the rising trend. Of this practice by a balanced decision making, keeping the women informed of all the implications instead of giving in to the demands of the patient

childhood asthma being treated appropriately

It was a descriptive study done in the department of Pediatrics, Jinnah Medical College hospital Karachi from October 2005 to September 2006. All children between 1 to12 years, diagnosed as asthma [on clinical grounds] were enrolled. Information regarding asthma severity and use of regular drugs in past 6 months was recorded in a pre-de-signed Proforma. Disease severity was classified according to GINA guidelines as intermittent and persistent. Persistent group was further subdivided into mild, moderate and severe categories. Treatment in accordance to GINA guidelines was considered as a standard therapy. A total of 150 patients were enrolled, out of which only 4.7% were of intermittent category while 95.3% were of persistent type [39.3% mild persistent, 44% moderate persistent and 12% severe persistent]. Patient in intermittent group were not on any regular long term medication. Seventy six percent of the patients with persistent asthma were on quick relief drugs, while controller drugs were being used in19.3% cases only. Limited use of inhalers with or with out spacer device and nebulizers was observed [14% cases]. Inappropriate drugs according to GINA guidelines, e.g.: antihistamines, cough and cold preparations, mast cell stabilizers and drugs from hakims and homeopaths, were not included in the study results. Majority of the patients was not found to be treated according to GINA guidelines. Controller medications were being under prescribed while in its place reliever medication was being inappropriately advised on regular basis

Pattern of congenital malformations and their neonatal outcome

To determine the pattern of major congenital malformations in neonates admitted in a tertiary care NICU and evaluate their early outcome. Descriptive Study. Department of Pediatrics, Liaquat National Hospital and Medical College Karachi, from January to December 2009. A total of 431 neonates were admitted in NICU including those referred from outside. Children with major congenital malformations were identified by clinical examination and confirmed by appropriate radio-diagnostic methods. These neonates were immediately referred to the surgical team for intervention. A total of 57 neonates were admitted during the study period. Thirty one were males and 26 females. Fetal anomalies were diagnosed correctly in 17 cases out of a total of 19 inborn deliveries on maternal ultrasound while it was missed in one fetus and incorrectly diagnosed in one case. A total of 48 patients had surgery out of which 4 [8.3%] died in the neonatal period. Five cases were booked for elective surgery beyond the neonatal period. Out of 4 neonates with congenital heart disease one case was referred outside, one neonate died preoperatively while 2 infants were managed conservatively. Due to detection of fetal anomalies, early surgical intervention, and intensive neonatal care, most infants can be rescued after a successful primary operation

Auto immune hemolytic anemia in a child precipitated by chicken pox

Auto Immune Hemolytic Anemia [AIHA] is a rare entity in children. We report a case of an adolescent girl with AIHA, which was precipitated by chicken pox. Clinical course over 3 years, till remission is described

Effects of water and sanitation on childhood nutrition in Pakistan

To examine the impact of type of household water supply and toilet facilities on the nutritional status of children < 3 yrs of age. Descriptive study. Rural areas of district Malir, Karachi. From November 2000 to April 2001. 400 children < 3 years of age were assessed by anthropometric examination. A pre-designed questionnaire was filled, collecting information on type of water supply and toilet facilities in each of the selected household. Only 190 out of 400 families interviewed had access to piped water and only 185 of 400 families had proper flush toilets. Lack of piped water and toilet facilities had a significant correlation. Statistics for stunting were not significant, probably because it represents a more chronic process. Provision of unpolluted water supply with proper toilet and sewerage facilities play an important role in preventing childhood malnutrition and solutions to address this problem are the need of the day

Rotavirus as the etiological agent of diarrhea in hospitalized children

Diarrheal diseases are a significant cause of mortality in children under five years of age and rotavirus is considered to be one of the major causes of childhood gastroenteritis. As it is a sig-nificant contributor to childhood mortality in developing countries, WHO recommends epidemiological studies regarding rotavirus especially in developing countries. As it is a vaccine preventable disease these researches can have a significant impact on the future recommendation of vaccines. To identify rotavirus in the stool analysis of children less than five years of age, presenting with acute diarrhea. To describe the age distribution and clinical features of rotavirus gastroenteritis. Hospital based, cross-sectional study. Analysis of 40 fecal samples was done for rotavirus by immunochromatography; rotavirus was identified in 6 [15%] samples. The majority of thechildren with rotavirus gastroenteritis were younger than 2.5 years. The children who tested positive for rotavirus had a better nutritional status than the children who were negative for the virus. No significant difference was seen in the duration of diarrhea and of hospitalization in the two groups. Diarrhea is a major public health problem in developing countries. Rotavirus as the etiological agent was detected in 15% of cases in the present study, which is in close approximation to several other studies from Pakistan. Still large-scale studies are required to determine the prevalence of rotavirus gastroenteritis that can have important implications for rotavirus vaccination. Meanwhile our strategy s-hould focus on good sanitation and clean drinking water to save our children from this democratic disease

Management of Acute Asthma in children using metered dose inhaler and small volume nebulizer

To determine whether the administration of 32 - agonist by Metered Dose inhaler [MDI] with accessory device [AD] is a as effective as the administration of 32 - agonist by small volume nebulizers [SVN] for the treatment of acute asthma. A cross sectional study was conducted at Emergency Room [ER] of National Institute of Child Health [NICH], Karachi, between October 2000 to March 2001. This study included 150 children, 6 months and older with a history of wheeze and presenting with an acute asthma exacerbation. Children were categorized into mild, moderate and severe asthma according to medical scoring system. Children were assigned randomly into group A and B to receive standard dose of 32 - agonist [salbutamol] by MDI/AD [group A] or SVN [group B]. Baseline : characteristics and asthma severity were recorded. All variables [dyspnoea, use of accessory muscles, cyanosis, respiratory rate, heart rate, blood pressure, oxygen saturation, pulsus paradoxus, and wheeze] and Peak Expiratory Flow Rate [PEFR] in children 5 years and older, were determined at pre and post inhalation therapy. Both groups did not differ in demographic characteristics. There were no significant differences in outcome measures. In children treated with MDI/ADs and SVNs. PEFR increased significantly in both the groups after completion of treatment, but PEFR was not statistically significant when compared in between groups. The data suggested that MDI/AD is an effective alternative to nebulizer for the treatment of children with acute asthma exacerbation in the ER

Effect of weaning period on nutritional status of children

To determine the effect of age of introduction and the type of weaning foods used, on the nutritional status of children under three years of age. Cross-sectional, community-based study. The study was conducted in rural areas of District Malir in Karachi, Pakistan, comprising of 900 villages spread over 1200 square kilometer, from November 2000 to April 2001. Patients and Three hundred and fifty-nine children under 3 years were included in the study. Based on the age of commencement of weaning, the children were categorized as children weaned at appropriate and inappropriate age. For the type of weaning foods, the children were grouped into two categories based on the nature of food items. Children for whom weaning was started at the recommended age of 4-6 months were found to be significantly less malnourished on anthropometric measurements as compared to the other set in which weaning was started at an earlier or later age. Significant statistical difference was noted in the children weaned on nutritious home-made items compared to children having commercial foods. Malnutrition is of multi-faceted origin beyond only poverty and food shortage. Inappropriate feeding practices can result in growth faltering as noted in the present study. Programmes should be launched for education of mothers regarding weaning practices

Determinants of bottle use amongst economically disadvantages mothers

There is documented evidence of increased risk of infant mortality in formula fed infants versus breast fed babies. The hazards of bottle use for infant feeding, especially in underprivileged communities, are also well known. As the issue of bottle use is directly related to childhood survival and mortality, there is legislation against propagation of bottle-feeding in Pakistan. To obtain information about determinants of bottle use for infant feeding in a low-income periurban community. Community based cross-sectional study. 150 mothers with infants up to one year of age were included in the study period from October 03 to March 04. Out of these 102 mothers were found using bottle for infant with or without breast-feeding. The tendency to use the bottle increased in relation to child's increasing age. Only 17% of the infants under the age of 3 months were offered bottle, it was 69% between 4 to 6 months and it increased to 76%.in infants from 7 months to 1 year. The attributes associated with increased bottle use were mother's older age, illiteracy and increased parity. Bottle use is a public health issue in poor and illiterate mothers of developing countries. While, in Pakistan, laws are enacted against its propagation, we need community-based strategies to bring about a socio-cultural shift in the growing prevalence of bottle use found in the study

Weaning practices in peri-urban low socioeconomic groups

To assess the current weaning practices in infants [children under one year of age] in a low socioeconomic peri-urban area in Karachi. Design: Cross-sectional study. Place and Duration of Study: 'Well Baby Clinic' in Jinnah Medical College Hospital situated in Korangi Industrial area of Karachi from October 2003 to March 2004. Patients and One hundred and fifty infants were included in the study. Their feeding pattern and weaning practices were studied using a questionnaire. They were grouped in three categories according to their age of commencement of weaning. Ninety [60%] infants were receiving semi-solids/solids [weaning foods] in addition to milk. The recommended age [between 4 to 6 months] was noted in 37 [25%] cases, while early weaning was present in 9 [6%] cases and delayed weaning in 44 [29%] cases. Even if weaning was started at the correct age, several problems were noted. These included infrequent feeding, use of expensive commercial cereals given in diluted form as compared to home-made foods, and improper food preparation. The quality, type and choice of food was not ideal for adequate growth. Incorrect weaning practices is an issue of public health in developing countries. The identification and analysis of weaning practices prevalent in low socioeconomic households can lead to remedial strategies for improving the nutrition status of infants and, thereby, help reduce infant and morbidity rate

Value of partogram in prediction of outcome of delivery in primigravidae - A study of 100 cases

A study was carried out over a period of 6 months from June - December 2000 at Lady Aitchison Hospital, Lahore which is affiliated to King Edwards Medical College, Lahore. 100 primigravidae whose gestational period was 36 weeks or above having spontaneous or induced labour were evaluated on a partogram. The study highlighted the value of partogram in the prediction and detection of the likely course of labour