Use of N-acetylcysteine in children with fulminant hepatic failure caused by acute viral hepatitis

To determine the efficacy of N-acetylcysteine [NAC] in children aged > 1 month to 16 years admitted with Fulminant Hepatic Failure [FHF] secondary to Acute Viral Hepatitis [AVH] in a tertiary care center of a developing country. Analytical study. Department of Paediatrics, The Aga Khan University Hospital, Karachi, Pakistan, from January 2007 to December 2011. Medical records of children [> 1 month - 16 years] with FHF admitted with AVH of known etiology who received NAC were reviewed retrospectively. Liver function tests [mean +/- SD] at baseline, 24 hours after NAC and before or at the time of discharge/death were recorded and compared via using repeated measures ANOVA[r-ANOVA]. Efficacy of NAC is defined in improvement in biochemical markers, liver function test and discharge disposition [survived or died]. Mortality associated risk factors were identified by using logistic regression analysis. P-value and 95% confidence interval were recorded. Forty children [mean age was 80 +/- 40 months] with FHF secondary to AVH received NAC. Majority were males [n=25; 63%]. Vomiting [75%] and jaundice [65%] were the main presenting symptoms, one-third had hypoglycemic, while 40% had altered sensorium at the time of admission. There was significant statistical difference in liver enzymes and prothrombin time on admission comparing at discharge in children received NAC [p < 0.001]. Fifteen [38%] children died. Severe vomiting [Odds Ratio [OR] 0.22, 95% Confidence Interval [CI] 0.05 - 0.8], jaundice [OR 9.3, CI 1.1 - 82.6], inotropic support [OR 20.6, CI 3.5 - 118.3] and mechanical ventilation [OR 4.3, CI 1.1 - 16.6] at the time of admission are associated with risk factors for mortality in children with FHF secondary to AVH. NAC used in children with FHF secondary to AVH is associated with markedly improved liver function tests and recovery. FHF with complications is high risk for mortality

Empyema thoracis in children: clinical presentation, management and complications

To determine the etiology, clinical manifestation, management [medical and surgical] and complications of children with empyema thoracis in a tertiary care hospital from Karachi, Pakistan. Descriptive, analytical study. Department of Surgery, The Aga Khan University Hospital, Karachi, from January 1996 to December 2010. Medical records of admitted children aged > a month to 15 years with discharge diagnosis of empyema thoracis and data was collected on demographic features, clinical manifestation, management and complications. Children managed medically were compared with those managed surgically by using interquartile range and median comparison. Mann-Whitney U test was used to compare age in months, weight [kg] and length of stay in days and presenting complaint, duration of illness; chi-square test was used to compare thrombocytosis in between groups and p-value was calculated. Among the 112 patients, 59 [53%] were younger than 5 years of age. Males [n=83, 74%] were predominant. Fifty [45%] children were admitted in winter. Thirty [27%] children found unvaccinated and one fourth [n=27; 24%] were severely malnourished. Fever, cough, and dyspnea were the major presenting symptoms. Sixty-six [59%] were on some antibiotics prior to admission. Staphylococcus aureus [n=13] and Streptococcus pneumoniae [n=5] were the commonest organism isolated from blood and pleural fluid cultures. Majority of the children required some surgical intervention [n=86]. Surgically managed children were younger [p=0.01]; had less weight [p=0.01] and prolonged fever [p=0.02]; and stayed longer in hospital [p < 0.001] as compared to medically managed children. Requiring readmission [n=8], subcutaneous emphysema [n=5] and recollection of pus [n=5] were the major complications. Staphylococcus aureus was the major organism associated with paediatric empyema thoracis. Early identification and empiric antibiotic as per local data is essential to prevent short and long-term complications. Younger, lower weight children with prolonged fever required surgical management

Severe combined immune deficiency syndrome

To determine the clinico-demographic features and laboratory parameters of children with severe combined immunodeficiency [SCID]. Case series. Department of Paediatrics and Child Health, the Aga Khan University, Karachi, from July 2006 to July 2011. Thirteen infants who were discharged with a diagnosis of SCID were inducted in the study. Their clinico-demographic features and laboratory parameters were determined. Descriptive statistics has been used for computing frequency and percentage. The median age at diagnosis was five months; 5 infants presented within 3 months of life. Three-fourth [77%] were males. Most of the infants were severely malnourished [85%] at the time of presentation. More than two-thirds [69%] were products of consanguineous marriages. All subjects had severe lymphopenia [absolute lymphocyte count [ALC] ranging between 170 - 2280 and low T and B lymphocyte counts. SCID should be considered in infants presenting with severe and recurrent infections. Low ALC [< 2500/mm[3]], is a reliable diagnostic feature of SCID. These infants should be promptly referred to a facility where stem cell transplant can be done

Acute lymphoblastic leukemia in a child with fanconi's anaemia

Fanconi anaemia [FA] is an autosomal recessive inherited disorder with progressive bone marrow failure, associated congenital malformation and solid and haematological malignancies. Acute myeloid leukemia is the commonest haematological malignancy followed by myelodysplastic syndrome in children with FA. FA transformed into acute lymphoblastic leukemia [ALL] is a rare phenomenon and one of the rarest haematological malignancies associated with this disorder. We are reporting a 13 years old girl with FA and positive chromosomal breakage. She required regular blood product transfusion. She was planned for haematopoietic stem cell transplantation [HSCT] but the sibling-matched donor was found to have chromosomal breaks as well. Later on, her peripheral smear showed blast cell. Bone marrow showed pre-B ALL. She was started on chemotherapy but died shortly due to complications of the treatment. For this rare condition conservative management is indeed essential, however, safe and appropriate chemotherapy regimen is needed

Incidence of angiographic patterns of diabetic maculopathy

The present study was carried out to observe the incidence of different types of diabetic maculopathy on fluorescein angiography to offer the appropriate treatment modality to the patients. In this interventional study the patients with diabetic retinopathy were selected by simple random technique. From August to December 2009. In present study 130 eyes of 65 patients were included; 30 males and 35 females with age ranging from 43 to 59 years. These patients were examined in out patient department and were diagnosed as cases of diabetic retinopathy with maculopathy. They were advised Fundus Fluorescein Angiography [FFA] which was performed in Diagnostic and Research Centre, Department of Ophthalmology, Allied Hospital, Faisalabad. The angiographic patterns of different types of diabetic maculopathy were observed. Out of 130 eyes, different angiographic patterns of diabetic maculopathy were seen in 115 eyes; Diffuse maculopathy in 77 eyes, Focal type in 23 eyes and Ischaemic type in 15 eyes. No maculopathy was seen in 14 eyes while in 1 eye premacular haemorrhage obscured the view

Factor XIII deficiency in children-clinical presentation and outcome

To determine the demographic features and clinical outcome of children with Factor XIII deficiency. Observational case series. The Aga Khan University Hospital, Karachi, from January 1996 to December 2006. Records of all hospitalized pediatric patients with discharge diagnosis of FXIII D, on the basis of factor XIII assay 5 mol/L urea test were retrospectively reviewed and abstracted on a pre-specified proforma. Demographic features, coagulation profile, family history and outcomes were noted. A total of 10 charts were reviewed. There were 5 boys and 5 girls. Almost all the children [9/10] were less than 5 years of age, out of whom 5 [50%] were infants, and 3 were neonates. Bruises and prolonged bleeding after trauma was the major presenting complaints in 80%, followed by prolonged bleeding from the umbilical stump in 2 patients. Nine patients had past history of prolonged umbilical bleeding. Two patients had history of FXIII D in siblings, while 2 had history of prolonged bleeding in other family members [cause unknown]. Consanguinity was present in 80% of the families. Initial coagulation screen were normal in all patients. Two patients had intracranial hemorrhage, proved on neuro-imaging, were managed with plasma infusions and required craniotomy. The rest were managed conservatively with plasma transfusions. All were discharged alive in good clinical condition. Almost all were followed regularly in clinic with monthly cryoprecipitate transfusions. Although factor XIII deficiency is a rare genetic disorder in children with history of brui sing, prolonged umbilical bleeding, family history of bleeding and consanguinity with normal initial coagulation screen [PT, APTT and platelets], FXIII D should be ruled out