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Aga Khan University

Department of Paediatrics and Child Health

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Articles 571 - 592 of 592

Full-Text Articles in Medicine and Health Sciences

Genetic Analysis Of Cystic Fibrosis In Pakistan: A Preliminary Report, Z A. Bhutta, T Moattar, U Shah Jul 2000

Genetic Analysis Of Cystic Fibrosis In Pakistan: A Preliminary Report, Z A. Bhutta, T Moattar, U Shah

Department of Paediatrics and Child Health

Objective: Evaluation of the incidence of common delta F508 mutation in a cohort of Pakistani children with Cystic Fibrosis (CF).
Methodology: The presence of the CFTR gene mutation at position 508 (delta F508) was evaluated in 15 consecutively presenting children with CF, proven by clinical features and an unequivocally positive sweat chloride test. The DNA from mononuclear cells was extracted and the delta F508 mutation assessed by the amplification refractory mutation system using the polymerase chain reaction.
Results: The delta F508 mutation was found in 9 cases (60%), of which 5 were homozygous for the disorder.
Conclusion: In this preliminary …


Self-Reported Feeding Advice By Physicians For Common Childhood Illnesses, S Q. Nizami, Z A. Bhutta Dec 1999

Self-Reported Feeding Advice By Physicians For Common Childhood Illnesses, S Q. Nizami, Z A. Bhutta

Department of Paediatrics and Child Health

Background: A nutritious diet is important for recovery during illnesses. Dietary advice by physicians and consumption of food by the patients are often based upon their hot and cold concepts and beliefs about various foods rather than on scientific basis.
Objectives: To look at the food-advising behaviour of physicians during illnesses and to know the maternal concepts about various foods being hot or cold.
Methods: A questionnaire was served to the physicians participating in a continuous medical education session held at the Aga Khan University and Hospital, asking them to write the foods they advise or restrict during different illnesses …


Dysphagia In Hereditary Sensory Autonomic Neuropathy Type Iv, U Shah, M Arshad, T Mozaffar May 1999

Dysphagia In Hereditary Sensory Autonomic Neuropathy Type Iv, U Shah, M Arshad, T Mozaffar

Department of Paediatrics and Child Health

No abstract provided.


Myelofibrosis In Severe Vitamin D Deficiency Rickets, Mehnaz Atiq, Zehra Fadoo, Fouzia Naz, Mohammad Khurshid Jan 1999

Myelofibrosis In Severe Vitamin D Deficiency Rickets, Mehnaz Atiq, Zehra Fadoo, Fouzia Naz, Mohammad Khurshid

Department of Paediatrics and Child Health

Vitamin D deficiency is prevalent in infants and children in the underdeveloped countries1. Infants with mild deficiency tend to present with complications like hypocalcemic seizures whereas occurrence of pathological features in advanced rickets is well elucidated2. Secondary myeloflbrosis has been reported as a complication of severe rickets and in these children anemia, myeloid metaplasia and bone marrow aplasia strongly suggested myelofibrosis. Despite the high prevalence of rickets in the Indo-Pakistan subcontinent, there has been no report on this complication.We present a case of severe clinical rickets who had developed secondary myelofibrosis with hepatosplenomegaly. The purpose of …


Cholera In Children In Karachi From 1990 Through 1995: A Study Of Cases Admitted To A Tertiary Care Hospital, S Q. Nizami, B J. Farooqui Jun 1998

Cholera In Children In Karachi From 1990 Through 1995: A Study Of Cases Admitted To A Tertiary Care Hospital, S Q. Nizami, B J. Farooqui

Department of Paediatrics and Child Health

Although cholera is an endemic disease in Bangladesh, India and other countries, it was never a significant cause of gastroenteritis in Pakistan before 1988. Since then, cases of cholera are identified each year, both in adults and children in Pakistan. In order to see the contribution of Vibrio cholerae as a cause of gastroenteritis in children, we reviewed the cases of cholera admitted in the pediatric ward of the Aga Khan University Hospital, Karachi, Pakistan. Of 4346 children hospitalized with gastroenteritis during 1990 through 1995, 348 children (8%) were confirmed to have cholera. The youngest child with cholera was seven …


Haemolytic Uraemic Syndrome In Childhood: An Experience Of 7 Years At The Aga Khan University, S H. Ibrahim, Z A. Bhutta, I A. Khan Apr 1998

Haemolytic Uraemic Syndrome In Childhood: An Experience Of 7 Years At The Aga Khan University, S H. Ibrahim, Z A. Bhutta, I A. Khan

Department of Paediatrics and Child Health

We reviewed the case records of all children admitted to the Aga Khan University Hospital (AKUH) with a diagnosis of Haemolytic Uremic Syndrome (HUS) over a 7 year period (July, 1988-June, 1995). A total of 33 patients were admitted to the Pediatric ward at (AKUH) with a median age of 16 months (range 4 months-9 years). 97% cases identified were of the classic variety and no familial case was identified. The mean duration of illness was 27 days, 39% had an illness > 28 days. Diarrhoea and prior antibiotic used was in 97% patients. Oliguria at admission was seen in 52.5% …


Profile And Risk Factors For Congenital Heart Disease, I Hassan, A A. Haleem, Z A. Bhutta Mar 1997

Profile And Risk Factors For Congenital Heart Disease, I Hassan, A A. Haleem, Z A. Bhutta

Department of Paediatrics and Child Health

Congenital heart disease is an important cause of infant mortality and disability. The frequency, spectrum and contributory risk factors for significant cardiovascular malformations among live-births was retrospectively evaluated at the Aga Khan University Hospital. Of a total of 8331 live births between July, 1987 and December, 1992 34 babies were diagnosed to have congenital heart disease in the neonatal period giving a prevalence of 4 per 1000 live births. Ventricular septal defects was the most common (n = 10, 29%) abnormality. Eight cases had associated chromosomal abnormality, the most common being Trisomy 21. Maternal abortions, still-births, consanguinity and diabetes mellitus …


The Role Of Zinc In Health And Disease: Relevance To Child Health In Developing Countries, Z A. Bhutta Feb 1997

The Role Of Zinc In Health And Disease: Relevance To Child Health In Developing Countries, Z A. Bhutta

Department of Paediatrics and Child Health

No abstract provided.


Lactose Intolerance In Persistent Diarrhoea During Childhood: The Role Of A Traditional Rice-Lentil (Khitchri) And Yogurt Diet In Nutritional Management, Z A. Bhutta, S Q. Nizami, Z Isani Jan 1997

Lactose Intolerance In Persistent Diarrhoea During Childhood: The Role Of A Traditional Rice-Lentil (Khitchri) And Yogurt Diet In Nutritional Management, Z A. Bhutta, S Q. Nizami, Z Isani

Department of Paediatrics and Child Health

Lactose intolerance is frequently encountered in children with persistent diarrhoea (PD). Selection of an appropriate milk-based formulation is a major management problem in the developing world. In a consecutive series of studies, we evaluated the role of feeding a traditional rice-lentil (khitchri) diet alone (KY) or in combination with either soy formula (KY-Soy) a dilute buffalo milk (KY-B), in children (age 6 months-3 years) with PD. Serial observations of stool output, caloric intake and weight gain of these children over a 14 day period indicated satisfactory tolerance of the KY diet with adequate weight gain. The weight gain and stool …


Paediatric Prescribing In Karachi, S Q. Nizami, I A. Khan, Z A. Bhutta Jan 1997

Paediatric Prescribing In Karachi, S Q. Nizami, I A. Khan, Z A. Bhutta

Department of Paediatrics and Child Health

To assess amount of drug overuse we studied drug prescribing for common childhood problems by 65 general practitioners (GPs) and 29 paediatricians. A total of 2433 encounters between GPs or paediatricians and children under five years of age were observed. The presenting complaints were fever in 18%, cough in 9%, both fever and cough in 21%, vomiting in 20% and diarrhoea in 41% of encounters. Antibacterials were prescribed in 49% of encounters, analgesics and antipyretics in 29%, antiemetics in 8% and injectables in 15%. Antidiarrhoeals were prescribed in 41% encounters with children reported to have diarrhoea. Ampicillin and cotrimoxazole were …


Pneumococcal Sepsis In The Newborn--An Emerging Problem?, Z A. Bhutta Aug 1996

Pneumococcal Sepsis In The Newborn--An Emerging Problem?, Z A. Bhutta

Department of Paediatrics and Child Health

No abstract provided.


Moyamoya Disease Of Childhood As A Cause Of Recurrent Cerebral Ischemic Attacks--A Case Report, S Ibrahim, S S. Hyder Jan 1996

Moyamoya Disease Of Childhood As A Cause Of Recurrent Cerebral Ischemic Attacks--A Case Report, S Ibrahim, S S. Hyder

Department of Paediatrics and Child Health

No abstract provided.


Ciprofloxacin In Multi-Resistant Infections In Childhood: An Audit, D M. Khan, Z A. Bhutta Jun 1995

Ciprofloxacin In Multi-Resistant Infections In Childhood: An Audit, D M. Khan, Z A. Bhutta

Department of Paediatrics and Child Health

Ciprofloxacin is a new orally administrable fluoroquinolones, with considerable efficacy against multiresistant organisms. Its use in the paediatric age group however, is controversial because of the risk of potential articular toxicity. We retrospectively reviewed ciprofloxacin usage over a 32 week periods (June, 1991-September, 1993) in paediatric inpatients at The Aga Khan University Hospital. Ciprofloxacin was used in 21 cases, singly in 11 (52%) and in combination with other antibiotics in a further 10 (48%). The response to therapy was adjudged as 'good' or 'fair' in 13 (62%) cases. Ciprofloxacin was the only sensitive antibiotic in 4 (19%) and resistance to …


Inherited Metabolic Disorders In Pakistan: Presentation, Diagnosis And Outcome Of Congenital Hyperammonemias, P Mufti, I Ahmed Oct 1994

Inherited Metabolic Disorders In Pakistan: Presentation, Diagnosis And Outcome Of Congenital Hyperammonemias, P Mufti, I Ahmed

Department of Paediatrics and Child Health

A total of 21 patients were admitted to Aga Khan University Hospital with suspected congenital hyperammonemias during the period 1989 to 1992. There were 11 patients with acidosis and 10 patients were without acidosis. Prominent clinical manifestations included positive family history (76%), onset in the first week of life (67%) and neurological manifestations (76%). Of patients with hyperammonemia and acidosis, 4 had severe metabolic acidosis with anion gap of 30mEq/L and above. Of patients with hyperammonemia without acidosis, 4 had ammonia level ranging from 1600-2000 mg/dl. Diagnosis was confirmed in only 1 patient and that was also done abroad. Overall …


Diagnosis And Outcome Of Congenital Hyperammonemias, P Mufti, I Ahmed Nov 1993

Diagnosis And Outcome Of Congenital Hyperammonemias, P Mufti, I Ahmed

Department of Paediatrics and Child Health

A total of 21 patients were admitted to Aga Khan University Hospital with suspected congenital hyperammonemias during the period 1989 to 1992, 11 with acidosis and 10 without acidosis. Prominent clinical manifestations included positive family history (76%), onset in the first week of life (67%) and neurological manifestations (76%). Of patients with hyperammonemia and acidosis, 4 had severe metabolic acidosis with anion gap of 30mEq/L and above. Of patients with hyperammonemia without acidosis, 4 had ammonia level ranging from 1600-2000 micrograms/dl. Diagnosis was confirmed in only 1 patient and that was also done abroad. Overall mortality was 71%. These disorders …


Necrotizing Enterocolitis In Infants Weighing Less Than 2000 G, P Mufti, Z A. Bhutta Feb 1992

Necrotizing Enterocolitis In Infants Weighing Less Than 2000 G, P Mufti, Z A. Bhutta

Department of Paediatrics and Child Health

Over a 4 year period, nine of 180 (5%) infants weighing less than 2000 G, admitted to the Aga Khan University Hospital (AKUH) developed necrotizing enterocolitis (NEC). An outbreak of NEC occurred in 1989, during which six infants developed the clinical illness. Overall incidence was 1.1%. Thirty-one birth weight and gestation matched controls were selected for comparison. Risk factors usually considered as predisposing factors, i.e., low 5 min Apgar score, rate of maternal complications, respiratory distress syndrome, mechanical ventilation, umbilical catheterisation, patient ductus arteriosus, use of antibiotics and feeding practices were found with equal frequency in both cases and controls. …


Current Concepts In The Diagnosis And Management Of Childhood Asthma, Z A. Bhutta Aug 1991

Current Concepts In The Diagnosis And Management Of Childhood Asthma, Z A. Bhutta

Department of Paediatrics and Child Health

No abstract provided.


Transcutaneous Bilirubinometry In Pakistani Newborns: A Preliminary Report, Z A. Bhutta, K Yusuf Jul 1991

Transcutaneous Bilirubinometry In Pakistani Newborns: A Preliminary Report, Z A. Bhutta, K Yusuf

Department of Paediatrics and Child Health

Neonatal hyperbilirubinemia is a frequently encountered problem in the neonatal period and carries a potential risk of encephalopathy. Early detection and quantification is important, and transcutaneous bilirubinometry (TcB) has been recommended as a non-invasive method for rapid screening. We prospectively compared the efficacy of TcB in 65 normal Pakistani jaundiced newborns undergoing simultaneous serum bilirubin measurements. Although the correlation between the two methods was significant (r = 0.66, P less than 0.01), the scatter was wide and the specificity only 53%. Although the technique offers the potential for non-invasive early screening of neonatal hyperbilirubinemia, it requires further validation in a …


Fulminant Hepatic Failure With Typhoid Fever In Childhood, Z A. Bhutta Jun 1991

Fulminant Hepatic Failure With Typhoid Fever In Childhood, Z A. Bhutta

Department of Paediatrics and Child Health

Although hepatic dysfunction has been described among adults with typhoid, there are few reports of significant hepatic functional impairment in children with typhoid. Of 355 children with culture proven typhoid seen at the Aga Khan University Hospital, hepatomegaly was noted in 118 (33%) and isolated right hypochondrial tenderness in 30 (8.5%). The liver function tests were normal in 78% and 47% of these children respectively and significant hepatic dysfunction was seen only in 26 (7.3%). However, children with typhoid and significant hepatic dysfunction had higher mortality (P less than 0.001) and two patients presented with a picture of fulminant hepatic …


Successful Eradication Of Flavobacterium Meningosepticum Neonatal Meningitis With Ceftizoxime, Z A. Bhutta, S H. Naqvi Jun 1991

Successful Eradication Of Flavobacterium Meningosepticum Neonatal Meningitis With Ceftizoxime, Z A. Bhutta, S H. Naqvi

Department of Paediatrics and Child Health

No abstract provided.


Chloramphenicol Therapy Of Typhoid Fever, Z A. Bhutta, S H. Naqvi, S Durrani, A Suria Feb 1991

Chloramphenicol Therapy Of Typhoid Fever, Z A. Bhutta, S H. Naqvi, S Durrani, A Suria

Department of Paediatrics and Child Health

In a prospective study we compared two different dosage regimens of IV chloramphenicol succinate (100 mg/kg/day and 75 mg/kg/day) in children with culture proven typhoid. Trough and peak blood samples, obtained at 48 hrs, were analysed for free chloramphenicol by high pressure liquid chromatography (HPLC). Although the mean trough (8.8 +/- 7.7 versus 5.4 +/- 2.6 mcg/ml) and peak (19.9 +/- 12.2 versus 15.4 +/- 6.1 mcg/ml) chloramphenicol concentrations were comparable in both groups, a significantly wider range was found in the group receiving 100 mg/kg/day. Potentially toxic levels (greater than 30 mcg/ml) developed in two patients with liver dysfunction. …


Neonatal Screening For Congenital Hypothyroidism In Pakistan, Murntaz Lakhani, Mohammad Khurshid, Shehla H. Naqvi, Mohammad Akber Jan 1989

Neonatal Screening For Congenital Hypothyroidism In Pakistan, Murntaz Lakhani, Mohammad Khurshid, Shehla H. Naqvi, Mohammad Akber

Department of Paediatrics and Child Health

Congenital hypothyroidism is a preventable cause of mental retardation. Since clinical signs of congenital hypothyroidism do not generally become obvious before three months of age, screening programmes have been introduced in North America and Europe, which consist of T4 or TSH screening on newborn infants on the third day of life. The screening for congenital hypothyroidism was initiated in Pakistan by the Aga Khan University Hospital (AKUH) in March 1987. By April 1988, 5000 neonates were screened and five cases of congenital hypothyroidism were diagnosed. The study revealed the incidence of hypothyroidism to be one case per 1000 newborns which …