A Tribute to Professor Musallem EIBuaIy: A Pioneer in Child Health Services

Losing a hero in the early hours of June 25th 1997 filled our heart with sorrow, but also with a great sense of pride for having known and worked with this extraordinary man who transformed health care for children in the Sultanate.It has not been an easy task for Child Health Services to reach where it is now for this program hardly existed before the 80's. Much of what has been achieved, we owe to Prof. Musallam ElBualy. A U.K. medical graduate from Durham University, Prof. ElBualy moved to the USA where he undertook his postgraduate qualifications in Paediatrics. Being an Omani with a deep sense of moral obligation, Prof. ElBualy soon joined the Ministry of Health (MOH) in 1981 where, as Chief of Child Health services, he committed himself to the improvement of Maternal and Child Health Care in the country. In 1986, he was appointed as the first Omani Professor of Paediatrics at Sultan Qaboos University and was involved in the setting up of the curriculum. In 1989 in addition to his teaching responsibilities, he was appointed Director of Curative Services at MOH. His brilliant ideas and missionary real contributed to a number of developments, including the rapid decline in Infant Mortality, expansion of the immunization program, and anti-smoking drives. Thanks in great part to his dedication and hard work, Oman achieved significant advances in all the three areas. His strong views on breast feeding policies led to the success of the “Baby Friendly Hospital Initiative" throughout the country. Besides his participation in a number of national Committees, he was also a Coordinator in many research activities, including Growth Studies in Omani Children, work in Nutritional Status, and a Gulf Health Survey. He was involved in the National Survey on Iodine Deficiency Disorder, a study on Vitamin A Deficiencies, and the National Survey on Genetic and Hereditary Blood Disorders. He established links in training programs with the Institute of Child Health, London, U.K. He was closely associated with theRoyal College of Physicians, U.K. and as a result, Oman became a Centre for Diploma in Child Health (DCH) and later MRCP Examinations. He was nominated as a member and an examiner for the Arab Board. He had an excellent working relationship with WHO and UNICEF and received the WHO Award for Anti- smoking Campaign. His achievements were hailed throughout the Gulf Countries, and he was conferred with the AGCC Medal of honor award in Medicine for his contribution and a Merit Award of the MOH by His Majesty Sultan Qaboos. The Centre for Disease Control (CDC) in Atlanta awarded himfor his excellent contribution to research related to the control of communicable diseases. its 4 Prof. ElBualy’s illness never interfered with his academic enthusiasm and clinical dedication. He was a good friend to others and a fatherly figure, inspiring great affection from ,his junior staff. His high expectations and straight forward, “no nonsense” attitude kept one in fear of not coming up to his gear - standards. Many a time he would rumble when you dared make mistakes. We still hear those roars echoing  across the corridors - something to keep us going. something to remember him by. This was a talented man with excellent leadership qualities and a wealth of knowledge and rich experience. In January 1996, he was diagnosed as having a Glioblastma. a problem which he typically kept from his friends and family with braveness and calmness. His happy spirit always made his physical problems seem distant. to the extent that few realised his illness had returned and progressed after treatment with surgery and radiotherapy. He died in his beloved home at the age of 58.This remarkable man will be missed by all

Incidence and Outcome of Severe Hyponatremia in Children and Young Adults: A Single Institution Experience

Objective: Our two main objectives are to assess the incidence and the outcome of severe hyponatremia in young hospitalized patients. Method: We retrospectively reviewed the incidence and outcome of severe hyponatremiac (Na <125 mmol/l) inpatients less than 18 years of age, admitted as consecutive admissions during one calender year. Psuedohyponatremia and artifactual hyponatremia were excluded. Patients’ demographics, clinical features, laboratory, treatment and outcomes were recorded. Results: Of 3561 admissions of patients less than 18 years of age, 20 developed severe hyponatremia. Nausea, vomiting, irritability, clouded sensorium and seizures were the most common symptoms and signs. Underlying central nervous system disease, pneumonia and malignancy were major co-morbid conditions. The initial volume status was determined as hypervolemia (n=7), hypovolemia (n=7) and euvolemia (n=6). Iatrogenic (diuretics 5 and hypotonic fluids 7) hyponatremia accounted for 60% of all cases. Mortality was 20%. Conclusion: Patients receiving intravenous hypotonic fluids should be closely monitored for the development of hyponatremia. The common etiology of hyponatremia in our studied cohort of patients is iatrogenic.

Visceral leishmaniasis and haemophagocytic syndrome in an Omani child

The paper reports the case of a previously healthy 4-year-old-girl who presented with pallor, fever and hepatosplenomegaly. Laboratory findings included pancytopenia, hypertriglyceridemia and hyperferritinemia. Initial diagnosis of kala-azar could not be confirmed because of the absence of clinical evidence, negativity of bone marrow aspiration or specific serology for visceral leishmaniasis. Repeated marrow aspiration, performed due lack of clinical response, revealed histiocytes showing haemophagocytosis consistent with haemophagocytic lymphohistocytosis (HLH) and appropriate treatment was started. She continued to have high-grade fever, and a third bone marrow aspiration ultimately revealed presence of Leishmania amastigotes with evidence of active haemophagocytosis. The girl was treated with liposomal amphotericin (AmBisome) for 5 days, following which she recovered rapidly with definitive remission. 

The Smallest Miracle Baby that Survived in Oman

Extreme prematurity is a major cause of perinatal morbidity and mortality. Management of such babies is a big challenge to neonatologists. Here we report the smallest baby ever survived in Oman

Characterization of PRF1, STX11 and UNC13D genotype-phenotype correlations in familial hemophagocytic lymphohistiocytosis

Familial hemophagocytic lymphohistiocytosis (FHL) is a rare autosomal recessive lethal condition characterized by fever, cytopenia, hepatosplenomegaly and hemophagocytosis. The hallmark of FHL is defect apoptosis triggering and lymphocyte cellular cytotoxicity. Thus far three disease-causing genes (PRF1, UNC13D, STX11) have been identified. We performed a genotype-phenotype study in a large, multi-ethnic cohort of 76 FHL patients originating from 65 unrelated families. Biallelic mutations in PRF1, UNC13D and STX11 were demonstrated in 13/74 (18%), 6/61 (10%) and 14/70 (20%) patients, respectively. In 27/60 (45%) patients analyzed for all three genes, no molecular diagnosis was established. STX11 mutations were most common in Turkish families (7/28, 25%), whereas in Middle East families, PRF1 mutations were most frequent (6/13, 46%). No biallelic mutation was identified in most families of Nordic origin (13/14, 93%). Patients carrying PRF1 mutations had higher risk of early onset (age < 6 months) compared to patients carrying STX11 mutations [adjusted odds ratio 8.23 (95% confidence interval [CI] = 1.20-56.40), P = 0.032]. Moreover, patients without identified mutations had increased risk of pathological cerebrospinal fluid (CSF) at diagnosis compared to patients with STX11 mutations [adjusted odds ratio 26.37 (CI = 1.90-366.82), P = 0.015]. These results indicate that the disease-causing mutations in FHL have different phenotypes with regard to ethnic origin, age at onset, and pathological CSF at diagnosis