The association of leukocyte adhesion defect type I and persistent hyperinsulinemic hypoglycemia of infancy in a Saudi Arabian family.

The authors describe 2 female sibling infants diagnosed with leukocyte adhesion defects CD11 and CD18. Both had successful bone marrow transplants from identical siblings. One of the patients was found to have persistent hypoglycemia of infancy.

Neurobrucellosis--a rare complication of renal transplantation.

Brucellosis is an intracellular bacterial infection contracted by consuming raw milk or by contact with infected cattle. Neurobrucellosis is a rather rare manifestation of brucellosis and has protean clinical presentations characterized by meningoencephalitis, myelitis, myelopathies, subarachnoid hemorrhage and psychiatric manifestations. A depressed immune status is believed to be a risk factor for developing neurobrucellosis.

Spontaneous Obstruction of Interatrial Communication in Infants with Atrioventricular Valve Atresia or Stenosis in the Neonatal Period: Serial Echocardiographic-Doppler Study.

BACKGROUND AND METHODS: The objective of this serial echocardiographic study was to document the critical size of the interatrial communication in infants with atrioventricular valve atresia, with which spontaneous obstruction is most likely to occur. Spontaneous obstruction of interatrial communication (patent foramen ovale) has been reported to occur in infants with atrioventricular valve atresia or severe stenosis. Atrial septostomy or septectomy therefore may be considered during initial palliation of these infants.

Evidence for autosomal recessive inheritance of infantile dilated cardiomyopathy: studies from the Eastern Province of Saudi Arabia.

Familial dilated cardiomyopathy is being increasingly recognized, but affected individuals <10 y are rarely identified. We describe the natural history of dilated cardiomyopathy and evaluate the mode of inheritance among infants of Arab descent from the Eastern Province of Saudi Arabia. We evaluated 55 consecutive cases of dilated cardiomyopathy in patients <10 y of age seen during a 5-y interval.

Polypoid endocervical stromal sarcoma with heterologous elements. Report of a case with review of the literature.

A 44 year old woman presented with six recurrent endocervical polyps within the span of 28 months. The last two of these polyps exhibited sarcomatous changes within the endocervical stroma with heterologous cartilagenous elements. Abdominal hysterectomy with bilateral salpingo-oophorectomy was performed.

Laparoscopic cholecystectomy and appendectomy with sickle cell disease.

Thirty-six pediatric and adult patients with sickle cell disease (SCD), cholelithiasis, and repeated abdominal crises underwent laparoscopic cholecystectomy (LC) and appendectomy with minimal complications. We prefer using LC to the open method for patients with SCD, and we propose adjustments to the positions of the four trocars to facilitate both procedures without adversely affecting the patient.

Discontinous splenogonadal fusion mimicking testicular tumor.

Full text is available as a scanned copy of the original print version.

Role of initial near total (95%) pancreatectomy in persistent neonatal hyperinsulinism (PNH).

A retrospective analysis of data on all infants with persistent neonatal hyperinsulinism (PNH) in our hospital during a nine-year period (July 1983 to June 1992) was carried out. The extent of pancreatectomy and outcome based on long-term follow-up were reviewed. Fourteen of the 18 cases, diagnosed at our facility according to the established criteria of PNH, underwent surgery.

Incidence and features of galactosaemia in Saudi Arabs.

Over a 12-year period (1983 to 1994), 11 cases of classical galactosaemia were identified in the Saudi Aramco Medical Services Organization (SAMSO). This yields an incidence of 12 cases per 100,000 live births in the Easter Province of Saudi Arabia. A higher proportion of infants presented with infections compared to reports in the literature.

Laparoscopic varicocelectomy for infertility. An initial report from Saudi Arabia.

Objective: To evaluate laparoscopic varicocelectomy for the treatment of infertility in Saudi males.

Methods: Between January 1992 and July 1994, laparoscopic varicocele repair was performed at Saudi Aramco Dhahran Health Center, in 48 patients, 8 for pain and 40 for infertility.

Results: The 8 patients with pain improved postoperatively.

Aggressive angiomyxoma of the vagina.

Aggressive angiomyxoma is a recently described pathological condition which affects mainly the soft tissues of the pelvis and perineum in women. It is neither encapsulated nor circumscribed and has a tendency for local recurrence. We report a 50-year-old woman who presented with a mass arising within the vaginal wall and extending to the perineum which appeared cystic on ultrasound.

An epidemic of aplastic crisis caused by human parvovirus B19.

Human parvovirus B19 has been associated with several diseases. Aplastic crisis in patients with chronic hemolytic anemia, erythema infectiosum, hydrops fetalis and arthritis are among the common diseases caused by this virus infection. In the period between July, 1991, and March, 1992, 48 patients with aplastic crises were hospitalized at Saudi Aramco-Dhahran Health Center, Dhahran, Saudi Arabia.

Agnogenic myeloid metaplasia in children.

Agnogenic myeloid metaplasia is a chronic myeloproliferative disorder characterized by splenomegaly, leukoerythroblastosis, extramedullary hematopoiesis, teardrop-shaped red blood cells, and fibrosis of the bone marrow. It is a disease of adults, with only one case report in the pediatric literature. The symptoms of the patient in this case clearly fit the diagnostic criteria of this disease.

Convulsion as a possible manifestation of vitamin D deficiency rickets in infants one to six months of age.

During the 42 months of January 1985 through June 1988, five infants between the ages of one and six months had hypocalcemic convulsions associated with vitamin D deficiency rickets. Despite abundant sunlight, convulsions as a manifestation of vitamin D deficiency in young infants are not uncommon in Saudi Arabia and should be considered as a probable cause for hypocalcemic seizure in breast fed infants.

Reversal of severe neurological abnormalities after vitamin B12 replacement in the Imerslund-Grasbeck syndrome.

Vitamin B12 malabsorption in the ileum has been postulated as the underlying cause of the Imerslund-Grasbeck syndrome comprising megaloblastic anemia, proteinuria, and multiple neurological abnormalities. A young Saudi child with spasticity, truncal ataxia, cerebral atrophy, megaloblastic anaemia and proteinuria is described. Replacement therapy with parenteral vitamin B12 resulted in the complete resolution of his neurological findings and brain atrophy.