Hereditary bleeding disorders in Riyadh, Saudi Arabia.

The result of an eight-year retrospective analysis of patients with hereditary bleeding disorders (HBD) at King Khalid University Hospital, Riyadh, is presented. One hundred and sixty-eight patients referred for investigation for suspected bleeding disorders had bleeding symptoms which fulfilled the criteria for HBD and were categorized as follows: 1) coagulation factor deficiencies: 41 patients had hemophilia A, while 16 had hemophila B; two patients each had factors XI and XII deficiency; four patients each had factors V and VIII deficiency and one patient had factor VII deficiency. There were two patients with dysfibrinogenemias and one with afibrinogenemia. 2) Von Willerbrand's disease was the second most common cause of HBD-25 patients were encountered in 15 different families. 3) Qualitative platelet disorders consisted of Glanzmann's thrombasthenia, with 18 patients, Bernard-Soulier disease, with five patients, and other qualitative platelet disorders, with 33 patients. 4) In 14 patients who presented with a history of bleeding, the only abnormality noted was prolongation of the bleeding time and normal coagulation and platelet function, and no definitive diagnoses could be established. The distribution of hereditary bleeding disorders obtained in this study resembles what has already been established in Western countries, with the exception of an increase of platelet disorders, mostly due to the increased rate of consanguinity in the community.

Acute viral hepatitis, glucose-6-phosphate dehydrogenase deficiency and prolonged acute renal failure: a case report.

A 10-year-old boy with glucose-6-phosphate dehydrogenase deficiency developed acute renal failure during the icteric phase of non-fulminant hepatitis A infection. He needed peritoneal dialysis for 54 days. Acute tubular necrosis was confirmed by percutaneous renal biopsy. He had complete recovery of his renal function when he was discharged.

The haematological manifestations of visceral leishmaniasis in infancy and childhood.

The haematological manifestations were reviewed in 94 patients (55 males and 39 females) with visceral leishmaniasis. Their ages ranged from 4 months to 12 years (mean per cent 1.8 years). All patients had splenomegaly and were anaemic, while (73 per cent) were neutropenic and (56 per cent) thrombocytopenic. Coagulation abnormalities were encountered in 10 (11 per cent) patients; in four patients this was associated with disseminated intravascular coagulopathy. Bone marrow was hypercellular in (90 per cent), normocellular in (5 per cent), and hypocelluar in (4 per cent). Also variable degrees of erythrophagocytosis and leukophagocytosis were noted with preponderance of histiocytes (46 per cent) and granulomatous formation (25 per cent). Low haemosiderin content in the bone marrow was noted, which together with the finding of high serum ferritin is consistent with anaemia of chronic inflammation. Hypersplenism, haemophagocytosis and granulomatous lesions of the bone marrow, chronic inflammation, and dietary factors appear to be the most important factors in the causation of the haematological changes in visceral leishmaniasis.

Congenital dyserythropoietic anaemia type I. Report of two siblings from Saudi Arabia.

This study describes two siblings (a boy and a girl) affected with congenital dyserythropoietic anaemia type I. It is the seventh familial occurrence reported. The girl presented in the neonatal period with anaemia, jaundice and hepatosplenomegaly and required 4 blood transfusions in the first 7 months of life, while her brother was discovered to be anaemic and jaundiced only at the age of 2 years and did not receive any blood transfusion.

Factors influencing linear growth in Saudi Arabian children aged 6-24 months.

This study was carried out to identify possible factors influencing the growth pattern of Saudi children in the first 2 years of life. The body length and weight of 400 healthy babies attending the well-baby care clinic of King Khalid University Hospital, Riyadh, Saudi Arabia, were measured at various determined ages in a cross-sectional study design. Nutritional indicators were calculated and the proportions with low height-for-age, low weight-for-height and low weight-for-age were compared with the reference population. The results showed a prevalence of 2 per cent of wasted children and 18 per cent of children with stunted growth. A univariate and multivariate analysis of personal and nutritional variables with the standard nutritional indicators identified age, sex, bottle feeding, and type of solid foods as significant risk factors for stunted growth. Stunting increases with age and males have poorer growth pattern than females. Genetic factors and poor feeding practices of the children are suggested reasons for the observed faltering growth between 6 and 24 months of age. A public health education programme for good nutrition is highly recommended.

Pharmacokinetics of methotrexate in children with acute lymphocytic leukemia.

Plasma methotrexate (MTX) levels were measured in 10 children (age 3-12 years) who received a total of 43 infusions of MTX. The total dose (1,000 mg/m2) was administered as a bolus of 200 mg/m2 with 24-hour infusions of 800 mg/m2. The clearance was fast at 158 +/- 81 ml/min/m2 and the elimination half-life (t1/2 beta) 3.0 +/- 0.7 h (mean +/- SD). The inter- and intrapatient variations in the steady state were wide, up to 6 times, suggesting the need for dose adjustment during infusion. The patients were at low risk for toxicity with a predicted MTX concentration at 39 h (5 half-lives) postinfusion of 0.28 +/- 0.10 mumol/l (mean +/- SD). None of them required leucovorin rescue 72 h postinfusion. An additional assessment of the MTX level may be useful as a guide to the duration of leucovorin rescue, which may be more or less than the routine 72 h postinfusion. The time suggested for this assessment was 48 h postinfusion. The mean +/- SD concentration at this time was 0.23 +/- 0.12 mumol/l and it correlated (r = 0.841) with the level measured 36 h postinfusion (n = 10). The value of this level needs to be investigated on a larger number of infusions.

Childhood idiopathic thrombocytopenic purpura: Experience at King Khalid University Hospital, Riyadh.

Forty-nine children (30 boys and 19 girls) with idiopathic thrombocytopenic purpura (ITP) seen at King Khalid University Hospital (KKUH) Riyadh, during a 7-1/2 year period were retrospectively reviewed with regard to their clinical characteristics, management, and outcome. The age of onset of the disease ranged from 8 months to 11 years with a mean of 4.26 years. Twenty-seven cases presented as acute ITP while 22 cases presented as chronic ITP. The clinicopathological features and response to treatment of the cases were essentially similar to findings in other parts of the world. In a search for a predictor of chronic disease, the platelet counts at four weeks and three months after diagnosis were found to be a significant factor for prediction of chronicity of ITP. If the platelet counts are less than 100x10(9)/L during these periods, the risk of chronic ITP is increased and vice versa. There was, however, no correlation in our patients between duration of symptoms prior to presentation and outcome of the disease as has been reported by investigators in Europe and Australia.

Pancytopenia in children with brucellosis: clinical manifestations and bone marrow findings.

Among 276 paediatric cases of brucellosis seen over a 7-year period, 16 patients (5.8%) with pancytopenia were identified. The most frequent presentations were fever, malaise, anorexia, weight loss, arthralgia, and hepatosplenomegaly. Fourteen patients (87.5%) had positive blood and/or bone marrow cultures for Brucella melitensis. Bone marrow aspiration specimens showed hypercellularity in 14 patients and normocellularity in 2. Histiocytes, eosinophils and plasma cells were increased in every marrow aspirate, and haemophagocytosis was observed in 14 patients (87.5%). Non-caseating granulomas were present in the bone marrow biopsy of 11 patients (68.8%). The pancytopenia was transient, and resolved on treatment of the Brucella infection.

Severe congenital dysfibrinogenemia (fibrinogen-Riyadh): a family study.

Congenital severe dysfibrinogenemia was discovered in a small Saudi family. Their single child exhibited abnormal severe bleeding tendency since birth and his coagulation profile revealed evidence of severe dysfibrinogenemia. The parents who were first-degree cousins and completely asymptomatic showed evidence of dysfibrinogenemia but to lesser degree than in their son. The child presented with large cephalohematoma and evidence of intracranial hemorrhage and left hemiparesis. He was treated with cryoprecipitate and his hematoma resolved, but his neurological deficit remained.

Fatal myeloencephalopathy due to intrathecal vincristine administration.

Vincristine was accidentally given intrathecally to a child with leukaemia, producing sensory and motor dysfunction followed by encephalopathy and death. Separate times for administering vincristine and intrathecal therapy is recommended.

Septicaemia in febrile neutropenic children with cancer in Saudi Arabia.

The pattern of sepsis among 56 children admitted for various forms of cancer to the King Khalid University Hospital, Riyadh, Kingdom of Saudi Arabia during a 6-year period, was retrospectively reviewed. A total of 148 febrile neutropenic episodes occurred and 55 of these, in 40 patients, were associated with positive blood cultures. The isolates were Gram-positive bacteria in 54% of instances, Gram-negative bacteria in 39.4% and Candida in 6.6% and polymicrobial sepsis occurred in five patients. Profound neutropenia (neutrophil counts less than 0.1 x 10(9) l-1) significantly predisposed to Gram-negative sepsis (P less than 0.02), which was responsible for about one-third of deaths in this series. Central venous catheters were present prior to 49% of the septicaemic episodes, but were not significantly associated with either increased Gram-negative or Gram-positive bacterial sepsis. However, all four cases of candidaemia occurred in patients with a central venous catheter in situ, and it is recommended that early empirical treatment for candida be considered in all febrile neutropenic cancer patients with central venous catheters.

Henoch-Schönlein syndrome in Saudi Arabia.

A retrospective study was carried out on 40 children from Saudi Arabia with Henoch-Schönlein syndrome to delineate its clinical pattern. More than 50 per cent of the cases occurred in winter. There was no apparent causal relationship with B-haemolytic streptococcal infection. All patients had skin rash. Fifty-eight per cent had gastrointestinal manifestations, 58 per cent had joint manifestations and 38 per cent had renal manifestations. The main clinical features of Henoch-Schönlein syndrome in Saudi children are comparable to those reported elsewhere.

Childhood brucellosis: a study of 102 cases.

One hundred two children, 45 days to 14 years of age, with proven brucellosis were studied to illustrate the epidemiologic, clinical and laboratory findings and to assess the outcome of antimicrobial therapy. The main source of infection was the consumption of raw milk in 80% of the patients. The predominant presenting symptoms and signs were fever, arthralgia, malaise, weight loss, arthritis, hepatosplenomegaly and lymphadenopathy. Brucella melitensis was isolated from 75% of 87 patients. Diverse hematologic and biochemical abnormalities were found. Different durations and combinations of trimethoprim-sulfamethoxazole or tetracycline plus streptomycin or rifampin were used for therapy. Eight-five patients were followed for an average of 14 months. Twelve (85.7%) of 14 patients treated with two-antibiotic combinations for 3 weeks relapsed, as did 5 (8%) of 62 patients treated for at least 6 weeks (P less than 0.001). No relapses occurred in 9 patients treated with trimethoprim-sulfamethoxazole and rifampin for 8 to 12 weeks plus streptomycin for the first 3 weeks. Longer duration and combination of antibiotic therapy seem warranted to improve outcome and to prevent relapses.

Schistosomiasis associated with a mediastinal mass: case report and review of the literature.

A 13-year-old girl presented with fever, night sweat, weight loss, abdominal pain, haematuria and hepatosplenomegaly. Urinalysis revealed many Schistosoma haematobium ova, but rectal snip examination was negative for schistosomal ova. X-ray and CT scan of the chest revealed enlargement of the anterior, superior, mediastinal and left suprahilar lymph node with an adjacent left pulmonary parenchymal opacity and small peripheral lesions on the right side. A bone marrow aspiration and biopsy was normal. The patient was treated with Praziquantel for her urinary schistosomiasis. Because of her clinical and radiological chest findings, the possibilities of lymphoma and tuberculosis were considered. Therefore, she underwent a thoracotomy and biopsy of her thoracic lesions. The histopathology revealed pulmonary granulomas surrounding schistosoma ova with reactive mediastinal lymph adenitis.

Cryptococcal meningitis in a child with systemic lupus erythematosus.

This is a case report of fatal cryptococcal meningitis in a child with systemic lupus erythematosus being treated with prednisolone and azathioprine. It is believed to be the first case of cryptococcal meningitis recorded in a child in Saudi Arabia.