Type II Vitamin D Dependent Rickets: A Case Report.

Vitamin D-dependent rickets Type II is a rare autosomal recessive disorder. Alopecia of the scalp or the body is seen in some families with Vitamin D-dependent rickets Type II. We report a child with this disease, and review the salient features of this disease with emphasis on the associated alopecia. Due to lack of facilities for estimation of 1, 25(OH)2 D and parathyroid hormone, alopecia remains the only clue to the diagnosis of this rare syndrome in association with resistant rickets.

Meconium Stained Amniotic Fluid – A Potential Predictor Of Meconium Aspiration Syndrome.

Objective: To identify potential predictors of Meconium Aspiration Syndrome (MAS) in pregnancies complicated by meconium-stained amniotic fluid (MSAF) & to review the incidence, morbidity and mortality of Meconium Aspiration Syndrome (MAS). Methods: In the period of 2003 to 2006,175 pregnancies with thick meconium-stained AF were delivered; of these, 15 neonates developed MAS and 160 did not. The two groups were compared retrospectively according to maternal findings, pregnancy outcome, and neonatal complications, using univariate analysis (P < 0.05 considered significant) and stepwise multiple logistic regression analysis to identify independent significant factors for prediction of MAS. Results: Incidence of MSAF was 13.97% and that of MAS was 8.57%. All deliveries associated with thick MSAF had developed MAS. 40% mothers were associated with PROM & prolonged labour. Most common & significant risk factors associated with MAS were increased gestational age, increased cesarean section (LSCS) & low Apgar scores at 1 minute and 5 minute. Mortality rate was 6.66% & mechanical ventilation was used in only 1(6.66%) case. Conclusion: MAS are associated with higher incidence of LSCS, lower 1 minute & 5 minute Apgar score & higher gestational weeks. 40% mothers were associated with PROM & prolonged labour. The neonatal morbidity & mortality is significantly more frequent in relation to thick meconium stained amniotic fluid. Specific delivery room resuscitation procedure, early diagnosis & proper management can prevent development of MAS as well as morbidity & mortality.

Childhood Middle Ear Tuberculosis–A Rare Case Report.

Tuberculosis rarely affects the middle ear cleft; the disease is a curiosity and not often considered in the differential diagnosis of otorrhea. The diagnosis is thus made too late, with resulting complications such as irreversible hearing loss and facial nerve paralysis. A case report with review of the literature is presented, emphasizing that tuberculosis should be considered in the differential diagnosis of chronic ear infection in children. In our case direct nosocomial spread of tuebrculous bacilli has been attributed.

An Overview of Renal Diseases in Children in Pokhara.

Objective: To determine the current pattern and prevalence of renal diseases in childhood in this region of Nepal. Material and Methods: A retrospective study of the renal diseases in children attending the Pediatric OPD and those hospitalised in Manipal Teaching Hospital, Pokhara was done over a period of 6 years (September 2000- September 2006). A detailed clinical and laboratory evaluation was performed at baseline. The children were managed according to disease diagnosed. These cases are under follow up and some have undergone surgical treatment. Results: 228 children (123 boys & 105girls) were diagnosed to have renal disease. Among them 39.5% had urinary tract infection (UTI), 30.7 % were suffering from acute glomerulonephritis (AGN), 17.5% were cases of nephrotic syndrome (NS) and 12 % had some other problems for example, 6.14% had genetic defects, 2.63% had renal Stone, 2.2% had pre-renal acute renal failure, unexplained recurrent hematuria in 1.3%. All the cases of UTI underwent through investigation and were treated accordingly. All cases of AGN are planned for follow up for 1½ yrs and among them 3 required biopsy till date. All cases of NS are under regular follow-ups and 2 have undergone biopsy. Renal stone was operated successfully. All cases of acute and chronic renal failures had required dialysis. Out of 5 (2.5%) chronic renal failures, 2 with end stage renal disease expired after repeated hemodialysis and three are still requiring dialysis. Among the obstructive uropathies, 43 % had renal stone, 36 % had posterior urethral valve and 21% VUR. Conclusion: It can be concluded that renal disease is not uncommon in children. It can be completely cured with proper and adequate treatment. Sometimes it has a bad prognosis when it reaches end stage renal disease. Early recognition, timely treatment and regular follow up are mandatory in management of children with renal diseases.

Efficacy of Albendazole and Short-Course Prednisolone Treatment in Children with One or Two Ring-Enhancing Lesions of Neurocysticercosis: A Randomized Controlled Trial.

Context: Neurocysticercosis is a endemic disease in Nepal causing social and financial burden on society and developmental problem in children. Aims: To determine the efficacy of albendazole plus oral prednisolone in children with 1 or 2 ring-enhancing lesions (by CT) on resolution of lesions and recurrence of seizure. Setting and Design: Randomized controlled open trial. Methods and Materials: Children with 1 or 2 ring-enhancing lesions <20 mm in diameter on computed tomography scan, likely to have Neurocysticercosis, were assigned to treatment & control groups. Children assigned to the treatment group (n = 50) were given 2.0 mg/kg per day prednisolone orally for 5 days plus 15 mg/kg per day albendazole on third day for 28 days. Anti epileptic drugs were given to both groups {including Control group (n = 51)}. Statistical Analysis: The results were analysed with the use of Epi Info version 6.04 and Stata version;7 software. Results: The lesions resolved completely or partially in more children in the treated group compared with the control group (p = .04 & p = 0.03). The proportion of children who had seizures was significantly lower in the treated group compared with the control group at 6 months (10% versus 33%; p = .006) and 12 months (14% versus 38%; p = .003). Conclusion: Albendazole plus Prednisolone increased resolution of lesions on computed tomography scan and reduced the risk of subsequent recurrence of seizures among children with Neurocysticercosis.

Neonatal sepsis bacterial isolates and antibiotic susceptibility patterns at a NICU in a tertiary care hospital in western Nepal: a retrospective analysis.

BACKGROUND: Neonatal sepsis is one of the commonest causes of neonatal mortality in the developing world. The neonatal intensive care units (NICUs) today face one common problem of tackling sepsis and neonatologists remain constantly baffled by the changing patterns of microbial flora and their sensitivity patterns. With the neonatal services coming of age in Nepal this retrospective analysis spread over a period of six years has become very pertinent. MATERIALS AND METHODS: We conducted a retrospective study over a period of six years to study the prevalence of different organisms causing septicaemia in the community and at our hospital and the antibiotic susceptibility pattern. In all 265 cases of suspected sepsis were screened using a panel consisting of CRP, ANC and I/T ratio and subsequently confirmed by cultures. The cases were early onset (n=44), late onset (n=56) and nosocomial groups (n=40). The data for the intramural (n=32) and extramural (n=68) cases was analysed separately. RESULTS: One hundred nineteen cultures out of the 131 positives were obtained from blood (44.92%) and the remaining were isolated from urine (6.11 %) and CSF (4.58 %). The most common organism to be isolated was staphylococcus aureus (42.75%) followed by klebsiella pneumoniae (18.32%)and escherechia coli (12.21%). Staphylococcus was isolated from 36.84%, 45.16% and 31.81% of the cultures obtained from neonates in the in-born, out-born and the nosocomial groups respectively while klebsiella pneumoniae [18.32 %] was seen in 21.05 %, 17.39 % and 36.36 % in each of the three groups. Pseudomonas aeruginosa [6.11 %] was isolated from 13.64 % of the nosocomial cultures compared to 8.7 % of the out-borns while it was not seen in the in-borns. Other organisms isolated were much less in number, included - pathogenic streptococci, acinetobacter and enterobacter species. Coagulase negative staphylococci (CoNS) was seen in 4.39 % [n=4] and 9.09 % [n=4] of the same groups respectively. The gram positive organisms displayed a high degree of resistance to most penicillins and cephalosporins but glycopeptides and monobactams were effective in them. There was a high incidence of resistance noted with most third generation cephalosporins and aminoglycosides amongst most gram negative organisms where-in cefepime and imepenem were effective in most cases. CONCLUSIONS: Staphylococcal sepsis is not only common in community acquired infections but also in nosocomial sepsis. There is an emerging resistance to cephalosposrins probably attributable to extended spectrum betalactamases. Further large-scale multicentre studies are required to generalise the data for the whole country.

Enteric fever: a retrospective 6-year analysis of 82 paediatric cases in a teaching hospital.

OBJECTIVE: To evaluate the clinical and laboratory properties, to see the response to therapy, incidence of antimicrobial resistance and complications of Enteric Fever in children. METHODS: This is a retrospective study of 82 cases of enteric fever admitted in department of pediatrics, Manipal Teaching hospital, Pokhara, Nepal. Study period was six years from (Jan 2000 to Dec 2005). RESULTS: Total of 82 cases of Salmonella infections were admitted .There were 50 (60%) males and 32 (40%) females. Most of the patients were above the age of five. The leading clinical feature were Fever (100%) , GI symptoms (73%), followed by splenomegaly (60%), hepatomegaly (58%), chills & rigor (41%), headache(33%),coated tongue(17%), lymphadenopathy (13%), Respiratory signs (13%), toxic look (7%). The laboratory reports revealed leucopenia in 26% and leukocytosis in 16%. Widal test was positive in 83%, Blood culture was positive in 37 %.Bone marrow was done in 8 cases, out of which 5(62.5%) were culture positive. Out of 35 culture positive cases 32 were Salmonella typhi and 3 were Salmonella paratyphi A. Regarding the treatment 55% were treated with ciprofloxacin, 29 % with ceftriaxone, 7% with ampicillin, 6% with cefotaxime and 2.4 % with chloramphenicol. Response to therapy was assessed by day of defervescence after antibiotics. Best response was observed with ciprofloxacin (4.7 days) followed by ceftriaxone (5 days), ampicillin (5.5 days), cefotaximee (6.4 days), chloramphenicol (10 days) respectively. In the antibiogram resistance was 43% with chloramphenicol, 37% with ampicillin, 31% with trimethoprim- sulfamethoxazole, 5.7%with ciprofloxacin and 4% with cefotaxime. Resistance was 0% with ceftriaxone, cefuroxime, and ofloxacin. Gentamycin was found to show high sensitivity (91%). The complications observed were anemia in 10%, 5% had neurologic signs like clouding of consciousness and 3.7% had CNS irritability. CONCLUSION: It is important to include Enteric fever in the differential diagnosis of febrile patients with abdominal symptoms. Though blood culture is the definite test, Widal test plays supportive role in diagnosis of enteric fever, especially when patients come after a course of antibiotics. Sometimes when both blood culture and Widal tests are negative Bone marrow can be the diagnostic tool for the diagnosis. Based on this analysis ciprofloxacin is still a good drug for the treatment of Enteric Fever. Ceftriaxone, Cefuroxime and Ofloxacin can be considered as first line treatment for Enteric fever since resistance was nil with these drugs on culture reports.

Clinico-Radiological Spectrum of Neuronal Migrational Disorders: A Study of Paediatric Patients in the Western Region of Nepal.

Objective: This study was conducted in a tertiary care paediatric hospital to ascertain the spectrum of clinical and radiological features of Neuronal Migrational Disorders in children. The role of inheritance in Neuronal Migrational Disorders is under intense investigation. Studies on Neuronal Migrational Disorders (NMDs) in children from developing countries are lacking. Method: Retrospective analysis of records of diagnosed cases by neuroimaging as Neuronal Migrational Disorders in the Department of Paediatrics. Results: Eighteen Children (2days to 8years age) with different types of neuronal migrational disorder based on neuro-imaging were included. Observed anomalies included Lissencephaly (33.3%), Pachygyria (16.6%), Polymicrogyria (5.5%), Heterotopia (11.1%), Schizencephaly (22.2%) and Hemimegalencephaly (5.5%). Focal Seizure in 5 (27.7%) cases, Generalised Tonic Clonic Seizures in 3 (16.6%) and Myoclonic Seizure in 2 (11.1%) cases were the types of seizure present in 10 (55.5%) patients. Five patients presented with Quadriparesis, two with Hemiplegia and one with Congenital Talipes Equinovarus. All the eighteen patients had some degree of Cognitive Developmental Delay. Conclusion: Lissencephaly is the most common type of Neuronal Migrational Disorder followed by Schizencepahly. Focal Seizure and Quadriparesis were the common manifestations. Family history of similar cases with parental consanguinity in Schizencephaly cases gives a clue to the autosomal recessive mode of inheritance. Family history of similar cases of Schizencephaly without any history of consanguinity indicates an autosomal pattern of inheritance.

Thyroid dysfunction in Down syndrome.

BACKGROUND: Down syndrome is associated with various forms of thyroid dysfunction, hypothyroidism being the most common. The additive effects of both co-morbid conditions lead to further amplification of the clinical problems in these children with Down syndrome. OBJECTIVE: The purpose of this prospective study was to know the prevalence of thyroid dysfunction in Down Syndrome children below the age of 14 years and to correlate the features of Down Syndrome with those of thyroid dysfunction. METHODS: In all 32 Down syndrome children were grouped as euthyroid, compensated and uncompensated hypothyroidism on the basis of their T3, T4 and TSH levels and the features of were compared using the student's t-test. RESULTS: Hypothyroidism was seen in 5 out of 32 cases (15.6%) of which 1 (3.1%) had uncompensated while the other 4 (12.5%) had a compensated hypothyroidism. Hyperthyroidism was not observed in any of the cases. The prevalence of hypothyroidism of 16.7% on the age group 0-1 year could well be a reflection of congenital hypothyroidism while 20% prevalence in the age group 9-12 could imply acquired hypothyroidism. The mean values of the developmental quotient (D.Q.) and the Rao's index in Down syndrome cases with hypothyroidism was 49 5.1 and 0.15 0.06 respectively while that of euthyroid Down syndrome patients were 52 5.54 and 0.17 0.04 respectively ('p' value > 0.05), the differences though obvious yet not statistically significant. CONCLUSION: It thus seems necessary to screen all Down syndrome children for thyroid dysfunction.