Non-Ketotic Hyperglycinemia: A Rare Presentation with Neurological and Skeletal Abnormalities.

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Role of Citicoline in Treatment of Moderate to Severe Birth Asphyxia: A Pilot Project.

An observational cohort study was performed at Neonatal Intensive Care Units of two military hospitals to see the effects of citicoline in neonates diagnosed to have moderate to severe hypoxic ischemic encephalopathy. Twenty newborns fulfilling the inclusion criteria were selected for the study and were given injection citicoline via the IV route. The outcomes in the immediate newborn period, including neurological features and mortality, were studied. Only one baby, who was given injection citicoline, died during the study period and 19 babies were discharged and sent home after establishment of oral feeds. Citicoline was found promising in treatment of newborns with moderate to severe birth asphyxia. Key Words: Hypoxia, Ischemia, Birth asphyxia, HIE.

Neural tube defects, their implications and solutions in Muslim society.

Neural Tube Defects (NTDs) are serious congenital abnormalities and most of them are incompatible with life. The extremely debilitating quality of life, if one survives, calls for actions to prevent such sufferings. Experts agree on the role of Folic Acid in primary prevention of NTDs, yet, despite best efforts, the use of Folic Acid has reduced NTDs by only 50%. These cases too can be prevented by employing secondary preventive measures. These involve timely interruption of pregnancy -- a decision which, in addition to a medical judgment, is based on ethics, social, cultural and Muslim religious value systems in Pakistan. Indeed, it is a complex issue but empathic understanding and strong co-ordination, once established between different disciplines, can help parents to decide and opt for necessary secondary prevention by interruption of malformed foetus within the given time frame mandated by medical and religious authorities.

Effect of oxytocin infusion on reducing blood loss during abdominal myomectomy: A randomized controlled trial.

OBJECTIVE: To assess oxytocin infusion efficacy in terms of mean blood loss in patients undergoing abdominal myomectomy. METHODS: The single-blind randomised control trial was conducted at the Obstetrics and Gynaecology Department of Military Hospital, Rawalpindi, Pakistan, July 15, 2017, to January 15, 2018, and comprised women with intramural fibroids of American Society of Anaesthesia class I and II who were candidates for elective abdominal myomectomy. The women were randomised into study and control groups. In the study group, an infusion of 30 units of oxytocin in 1000ml normal saline was given at the rate of 15 units/hour during surgery. In the control group, pure normal saline was given. The main outcome measure was intra-operative blood loss. Data was analysed using SPSS 21. RESULTS: Of the 60 women, there were 30(50%) in the study group with a mean age of 37.10±4.35 years, and 30(50%) in the control group with a mean age of 36.67±3.70 (p>0.05). Mean intra-operative blood loss in the study group was 409.67±181.29ml which was significantly lower than the control group 875.33±284.71 (p<0.05). The mean surgery time also showed statistically significant difference between the two groups (p<0.05). In the study group, 3(10%) patients required blood transfusion, while blood was transfused to 11(36.6%) patients in the control group (p=0.046). CONCLUSIONS: Oxytocin, when given as an infusion, was found to be effective in reducing blood loss during abdominal myomectomy.

Waardenburg-Shah syndrome (WS type IV): a rare case from Pakistan.

Waardenburg-Shah syndrome is a rare autosomal recessive [AR] inherited disorder characterized by the presence of Hirschsprung's disease with a high likelihood of aganglionic megacolon, due to which the mortality is high. The management of the condition involves surgical intervention for the removal of the aganglionic segment of the colon. Here, we report a neonate that presented with a white forelock, white eyelashes, iris hypopigmentation, and sensorineural deafness associated with bilious vomiting, refusal to feed, and failure to pass meconium indicating intestinal obstruction.

Role of Corticosteroids in Reducing Respiratory Morbidity in Neonates Delivered by Elective Cesarean Section before 39 Weeks.

OBJECTIVE: To determine the effect of dexamethasone administration to women between 37 to 39 weeks of gestation on neonatal outcome in terms of respiratory morbidity and the need for NICU admission. STUDY DESIGN: An experimental study. PLACE AND DURATION OF STUDY: Department of Obstetrics and Department of Neonatology, Military Hospital, Rawalpindi, Pakistan, from February to July 2017. METHODOLOGY: Women with singleton pregnancies, undergoing elective cesarean section between 37-39 weeks. Neonates were evaluated for any respiratory morbidity and need for NICU admission as well as need of mechanical ventilation, length of hospital stay and final outcome. RESULTS: Out of 535 patients, 240 (44.8%) patients received steroid cover before their elective cesarean section (group 1). Two hundred and ninety-five (55.2%) patients who did not receive steroid cover, were allocated group 2. Mean age of mothers in group 1 was 29.20 +4.50 years and in group 2, it was 29.34 +4.50 years. The mean gestational ages were 37.56 +0.66 weeks and 38.11 +0.79 weeks in groups 1 and 2, respectively. In group 2, 14 (4.74%) newborns developed transient tachypnea of newborn (TTN), which was higher than the newborns in group 1 (04 (1.66%), p = 0.049). Also the number of neonates being admitted to NICU was greater in group 2 than in group 1 [23 (7.79%) vs. 06 (2.5%) respectively, p = 0.007].However, there was no statistically significant difference between the two groups with regard to final outcome, requirement for mechanical ventilation, length of hospital stay and APGAR scores at one and five minutes. CONCLUSION: Steroid cover significantly reduced the risk of respiratory morbidity in babies delivered by elective cesarean section between 37-39 weeks.

Granulocyte-colony stimulating factor in neonatal sepsis with leukopenia: A prospective cohort study.

OBJECTIVE: To observe the duration for normalization of the Total Leucocyte Count (TLC) with adjuvant Granulocyte-Colony Stimulating Factor (G-CSF) treatment in leukopenic neonatal sepsis, and to compare the neutrophilic response to G-CSF in neutropenic vs non-neutropenic subgroups. METHODS: This prospective cohort study was carried out at the Neonatal Intensive Care Unit at Military Hospital Rawalpindi (NICU) from 1st August 2015 to 25th January 2017. Fifty one newborns with sepsis and leucopenia were sampled judgmentally from a population of 5666 admitted to NICU during the study period. The sample was then divided into neutropenic (exposed) and non-neutropenic (non-exposed) subgroups on basis of the absolute neutrophil count (ANC). Adjuvant G-CSF was given to all subjects and stopped once TLC normalized. SPSS v22 was used to calculate mean G-CSF treatment duration and rise in ANC. A Pearson correlation coefficient and simple linear regression were computed to assess the relationship between pre-GCSF ANC and the duration of treatment with GCSF. Comparison of subgroups with respect to rise in ANC was done using independent samples T-test. RESULTS: The mean duration of G-CSF treatment was 1.82±0.81 days (1.0 - 4.0). Neutropenic neonates constituted 49% (n=25). The Pearson correlation coefficient showed a positive but negligible and non-significant correlation between the two variables, r = 0.070, n = 51, p = 0.625. A non-significant regression equation was found (F(1,49) = 0.242,p=0.625) with an R2 of 0.005. There was a 7.06±4.5 fold rise in ANC in the neutropenic subgroup compared to the 4.5±3.1 fold rise in the non-neutropenic subgroup (p=0.04). CONCLUSIONS: The mean duration for recovery from leukopenia with G-CSF treatment in neonatal sepsis was less than 2 days and had no significant relationship with pre-GCSF absolute neutrophil count. The neutrophilic response was significantly higher in neutropenic compared to non-neutropenic neonates. As GCSF made no difference to the outcome in terms of mortality, its routine use is not recommended in leukopenic neonatal sepsis. .

Hybrid Lesion of Congenital Cystic Adenomatoid Malformation and Bronchopulmonary Sequestration.

Hybrid congenital cystic lesions of lung comprising of both congenital cystic adenomatoid malformation (CCAM) and broncho-pulmonary sequestration (BPS) are very rare congenital malformations of the lung. We present a case of a newborn who presented to us with severe respiratory distress and later on found to have a hybrid lesion of the lung. It is a very rare occurrence and no such case has been reported from Pakistan so far.

Epigastric Heteropagus Conjoined Twins.

Heteropagus twins are an extremely rare form of asymmetrical conjoined monochorial, monoamniotic twins with an estimated incidence of less than one per one million live births. An often used synonym is parasitic twins. We report a very rare case of epigastric heteropagus twins with a large omphalocoele. The parasite had fully formed pelvis, lower limbs and upper limbs with male genitalia and it was attached to the autosite in the epigastrium.

Comparison of Serum Magnesium Levels in Overweight and Obese Children and Normal Weight Children.

Purpose Abnormalities in serum magnesium levels have been seen in obesity and its related diseases. Our aim is to determine the mean magnesium levels in overweight and obese children as compared to the levels in normal weight controls to study its relationship with obesity and overweight. The study was done at a tertiary care hospital. Methods A case-control study was conducted at the Department of Pediatrics, Combined Military Hospital, Peshawar, over a 12-month period from August 7, 2015 to August 6, 2016. A total of 140 children between 2-14 years of age were included in the study. They were divided into two equal groups of 70 children each. Both of the groups were matched according to their age and sex. Children with a body mass index (BMI) greater than or equal to 85th centile and 95th centile were placed in the overweight and obese category, respectively, and termed as cases while the other 70 children with a BMI greater than or equal to 5th centile but less than 85th centile were categorized as the normal weight group and termed controls. The serum magnesium levels of both case and control groups were calculated. Results The serum magnesium levels were significantly lower in the overweight and obese group (2.08 ± 0.211 mg/dl) as compared to the normal weight group (2.55 ± 0.155 mg/dl, p<0.001). A significantly strong inverse relationship was seen between serum magnesium levels and body mass index. Conclusion Mean serum magnesium levels in overweight and obese children are lower than those in normal weight children. Further studies are required to see the effect of supplementation of diet with this essential micronutrient on the weight of children.

Caudal Regression Syndrome with Bilateral Microtia.

Caudal regression syndrome (CRS) is a very rare and unusual disorder affecting the lower part of the body along with multiple systems'involvement. It is mostly found in association with maternal diabetes mellitus during pregnancy; but there is a rare familial form of the disorder, as well. We describe a newborn who presented with the classic clinical and radiologic features of CRS along with bilateral microtia. Our search of the available English language literature did not reveal any such association of CRS with microtia.

A Case Of 13-Year-Old Girl With Prolidase Deficiency.

Prolidase deficiency is a rare autosomal recessive disorder characterized by recurrent and nonhealing skin ulcers along with facial dysmorphism and mental retardation. We report a 13-year-old girl who has clinical manifestation of Proliodase deficiency. It is a very rare disorder and no such case has been reported so far from Pakistan.

Efficacy of Trichloro-Acetic Acid Peel Alone Versus Combined Topical Magnesium Ascorbyl Phosphate for Epidermal Melasma.

OBJECTIVE: To compare the efficacy in terms of reduction in melasma area and severity index (MASI) score by more than 10 of a combination of 20% trichloro-acetic acid peel plus 5% topical magnesium ascorbyl phosphate versus 20% trichloroacetic acid peel alone in the treatment of epidermal melasma. STUDY DESIGN: Randomized controlled trial. PLACE AND DURATION OF STUDY: Department of Dermatology, Lady Reading Hospital (LRH), Peshawar, from May 2012 to May 2013. METHODOLOGY: Patients aged 18 - 65 years, with Fitzpatrick skin type III-V were divided into two equal groups having 74 patients each. Detailed history was taken and Wood's lamp examination done to rule out mixed and dermal melasma. Melasma area and severity index (MASI) score was calculated for every patient. Priming was done for all patients with tretinoin cream applied once daily at night for 2 weeks, and to use a broad spectrum sun block cream before sun exposure. Patients in group Awere subjected to combined treatment, i.e. trichloro-acetic acid peel 20% (weekly) plus magnesium ascorbyl phosphate cream (applied once daily), while patients in group B were subjected to trichloro-acetic acid peel 20% (weekly) alone. Treatment was continued for 6 weeks. After completion of treatment, MASI score was recalculated. Proportion of patients with significant MASI score reduction was compared using chi-square test with significance at p < 0.05. RESULTS: Male and female patients were 11 (14.9%) and 63 (85.1%), respectively in group A, whereas 13 (17.6%) and 61 (82.4%) in group B. The mean age in group Awas 30.28 ±8.08 years, and 29.36 ±6.84 years in group B. Significant MASI score reduction in group Awas seen in 60 (81.1%) patients and in group B 49 (66.2%, p= 0.040). CONCLUSION: Combination of trichloro-acetic acid peel and topical magnesium ascorbyl phosphate cream was significantly more effective than trichloro-acetic acid peel alone in treatment of melasma.

Efficacy of Chloroquine as a first line agent in the treatment of uncomplicated malaria due to Plasmodium vivax in children and treatment practices in Pakistan: A Pilot study.

OBJECTIVE: To ascertain the efficacy of chloroquine as first line agent in treatment of uncomplicated malaria -caused by Plasmodium vivax in children---and to determine its current treatment practice in Pakistan. METHODS: This pilot study was conducted at the Paediatrics Department of Combined Military Hospital (CMH), Lahore, Pakistan. Forty-eight children between six months and twelve years of age having positive blood film for Plasmodium vivax were included. They were treated with chloroquine as a drug of - choice. Efficacy of chloroquine was assessed by clinical response, absence of parasitaemia on day seven and twenty-eight after initiation of therapy. A survey was also conducted to determine the first line therapeutic choice of Paediatricians in the treatment of uncomplicated Plasmodium vivax malaria in children in Pakistan. RESULTS: The results showed 100% efficacy of chloroquine in treating uncomplicated malaria caused by Plasmodium vivax in children. Artemisin was preferred by 74.28% Paediatricians' in combination therapy as 1st line treatment. CONCLUSIONS: Guidelines proposed by Malaria Control Programme Pakistan (MCPP) in collaboration with World Health Organization (WHO) are comprehensive but not being adhered to. The recently reported resistance of Plasmodium vivax to artemisin should urge measures to implement WHO guidelines.

Cryptosporidiosis Among Children Of District Skardu, Pakistan.

BACKGROUND: Cryptosporidiosis is a common cause of persistent diarrhoea in children in developing countries. Previously it was thought to be a major pathogenic organism in immuno-compromised children, but recent studies have shown it to be a common infectious agent in immune-competent children as well. METHODS: This descriptive case-series was carried out at Combined Military Hospital Skardu, Pakistan from August 2014 to June 2015. Fifty-three children aged 12 months to 12 years with history of persistent watery diarrhoea for more than 2 weeks, with no blood or mucous in it and no other systemic findings, were included in the study. RESULTS: We examined 53 stool specimens from children with persistent diarrhoea for the presence of cryptosporidium as well other pathogenic organisms. There were 22 (41.5%) females and 31 (58.5%) males. Out of 53 samples, 11 (20.8%) samples were found to be positive for cryptosporidium. Twenty-seven (50.9%) samples were found to be negative for any intestinal pathogen. CONCLUSIONS: It is concluded from this study that cryptosporidium is a very common infectious organism of persistent diarrhoea in this part of the country.