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.

Bone marrow transplantation from matched siblings in patients with fanconi anemia utilizing low-dose cyclophosphamide, thoracoabdominal radiation and antithymocyte globulin.

Nineteen patients with Fanconi anemia (FA) and bone marrow failure underwent bone marrow transplantation (BMT) from matched siblings. Median age at BMT was 8.7 years. Conditioning consisted of low-dose cyclophosphamide (CY 5 mg/kg x 4 days) and thoracoabdominal irradiation (TAI 400 cGy). Graft-versus-host disease (GVHD) prophylaxis was cyclosporin A (CsA) in 13 patients and CsA plus methotrexate in 6 patients. Antithymocyte globulin (ATG) was added in the pretransplant as well as the post-transplant period. All patients received high-dose acyclovir from day 2 pre-BMT to day 28 post BMT, and intravenous immunoglobulins (IVIG), 500 mg/kg weekly from day 7 pre-BMT to day 90 post BMT. No fungal prophylaxis was given. All patients engrafted, (median, 14 days for an absolute neutrophil count > or =0.5 x 10(9)/l; median, 37 days for platelet count > or =20 x 10(9)/l). Fourteen (74%) patients are alive with sustained engraftment and are transfusion independent. Three (16.6%) patients developed acute GVHD; none developed chronic GVHD. Five (26%) patients developed invasive fungal infections, and two (10%) developed fatal CMV disease. We believe the addition of ATG may have contributed to the increased incidence of severe life-threatening fungal and viral infections in our series.

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.

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.

Hypocalcemia due to hypoparathyroidism in beta-thalassemia major patients.

BACKGROUND: This is a retrospective analysis of case records of AA(2)-thalassemia major patients who developed hypoparathyroidism (HPT). The objective of this study was to assess the prevalence of hypocalcemia and hypoparathyroidism in AA(2)-thalassemia major patients being followed at King Khalid University Hospital (KKUH), Riyadh, Saudi Arabia. PATIENTS AND METHODS: Diagnosis was based on low serum calcium (S/Ca), high serum phosphate (Po4), normal serum magnesium and alkaline phosphatase, and low serum parathyroid hormone levels. Other parameters analyzed included age, sex, serum ferritin levels, age of onset of HPT, any symptoms of hypocalcemia, and presence of other complications in these patients. RESULTS: Out of 40 patients, eight (20%) were diagnosed to have HPT. The mean age at diagnosis was 13.6 years (range 11-16 years), mean serum calcium was 1.88 mmol/L (range 1.58-2.04), mean serum ferritin was 7490 AA(1/4)g/L (range 2000-23,064) and mean serum phosphate was 1.88 mmol/L (range 1.50-2.73). Serum parathyroid hormone (PTH) levels were low in most of the patients. Only two patients (25%) had mild symptoms of hypocalcemia. Growth retardation was present in all patients, while four patients had liver dysfunction, two had diabetes mellitus and two had cardiac dysfunction. CONCLUSION: HPT due to iron overload may develop in a significant number of thalassemia major patients, especially when chelation therapy is not optimal, therefore, all thalassemics should be carefully watched for this complication from early in their second decade.

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.

Laparoscopic cholecystectomy: The treatment of choice for cholelithiasis in infancy and childhood.

Twelve consecutive laparoscopic cholecystectomies (LC) were performed between January 1994 and October 1996 at King Khalid University Hospital. In all patients the indication for cholecystectomy was symptomatic gallstones. Among the 12 children, six had sickle cell disease. The operating time ranged between 65 and 135 minutes (mean=897plusmn;21.06). There was no major morbidity or mortality. The average duration of postoperative parenteral analgesia (pethidine hydrochloride) required was 0.47+/-0.19 day (ranged between 0.3 and one day). The average postoperative stay was 1.67+/-0.44 days (ranged between 1 and 2.5 days). In conclusion, LC is safe, effective and the preferred approach for cholelithiasis in children, with the advantages of short postoperative analgesia requirement, shorter hospitalization, and therefore, an early return to normal daily activities.

Decline in hepatitis B infection in sickle cell anaemia and beta thalassaemia major.

Seventy five Saudi children, 55 with sickle cell anaemia and 20 with beta thalassaemia major, who were negative for all hepatitis B virus (HBV) markers five years ago were recently investigated for exposure to HBV and hepatitis C virus (HCV) infection. Of the 55 patients with sickle cell anaemia and 20 with beta thalassaemia major, 20 and five patients respectively had been vaccinated against HBV earlier and all of them still had protective antibody (anti-HBs 42-96 IU) 3-5 years after vaccination and there was no vaccine failure. Among the non-vaccinated children the exposure rates to HBV were 14.3% among those with sickle cell anaemia and 26.7% among those with beta thalassaemia and this was not statistically significant when compared with the exposure rate to HBV among the general paediatric population (20.1%). Anti-HCV positivity among those with beta thalassaemia major and sickle cell anaemia was 70% and 18.2%, respectively, and this was significantly higher than anti-HCV positivity among the control group (0.8%). Anti-HCV positivity was directly related to the amount of blood transfused and to the duration of transfusion. The results of the study show that although the exposure rates to HBV among patients with sickle cell anaemia and beta thalassaemia major were not significantly different than that among the general paediatric population, infection with HBV still takes place among non-vaccinated patients despite strict precautionary measures taken. Hence early vaccination against HBV would probably be the only effective way of controlling HBV infection. For HCV infection, and because a vaccine against HCV is still not available, preventive measures such as blood screening for anti-HCV before transfusion and stringent infection control measures are crucial steps to be implemented for the control of spread of HCV among these groups of patients.

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.

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.

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.

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.

Iron-endocrine pattern in patients with beta-thalassaemia.

Patients with beta-thalassaemia (major, minor, and in association with sickle cell gene) frequently suffer from growth retardation and delayed sexual development. This is believed to be due, at least in part, to a direct effect of iron overload on the endocrine mechanisms of puberty. The role of iron overload remains speculative in relation to the disease severity and ethnic variations. This study determined the level of testosterone, cortisol, luteinizing hormone (LH), follicle stimulating hormone (FSH), free thyroxine (T4), tri-iodothyronine (T3), growth hormone (GH), iron, ferritin, and haematological parameters in 44 beta-thalassaemia patients (21 = beta-thal. major, 23 beta-thal minor), 25 Hb S/beta zero-thalassaemia patients, and 50 normal controls with age range 2-15 years. The iron endocrine status and haematological findings were evaluated, and the results were correlated and compared with age- and sex-matched controls. In comparison with controls the beta-thalassaemia-major and the Hb S/beta zero-thalassaemia patients had a significantly higher level of plasma ferritin (P < 0.01) while the mean level of total haemoglobin was significantly lower. The level of LH, FSH, testosterone, and plasma cortisol were lower in both beta-thalassaemia-major and Hb S/beta zero-thalassaemia patients with a negative correlation with plasma ferritin level. Free T3 and T4 were raised, but the difference was not statistically significant. The data demonstrate the occurrence of impaired endocrine function in the beta-thalassaemia and Hb S/beta zero-thalassaemia patients.

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.

Endocrine functions in sickle cell anaemia patients.

In this study, 80 male and female sickle cell patients, aged 4-50 years, with mild (severity index, SI < 6) and severe (SI > or = 6) forms of the disease were investigated). The levels of luteinizing hormone (LH), follicle stimulating hormone (FSH), testosterone, cortisol, growth hormone (GH), free thyroxine (T4), and free triiodothyronine (T3) were determined. The results were evaluated and the mean +/- 2 SD values were compared with those obtained in age- and sex-matched normal controls. The findings indicated gonadal hypofunction in the sickle cell patients, but with varied deviations from the mean results. Patients with the severe form of the sickle cell disease showed more frequent abnormalities of LH, FSH, cortisol and testosterone in comparison with the patients with a mild disease. The LH, FSH, cortisol and testosterone levels were lower, while T3 and T4 did not show significant differences between patients and the controls. The results suggest that the sickle cell gene abnormality has an adverse effect on endocrine functions. Follow-up and appropriate management of endocrine dysfunctions are advocated in such patients.

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.