We describe the clinical patterns and case-fatality rate associated with severe Rift Valley fever (RVF) in patients who were admitted to the Gizan regional referral hospital during an outbreak of RVF in Saudi Arabia from September through November 2000. A total of 165 consecutive patients (136 men and 29 women) were prospectively studied; all were identified according to a strict case definition, were confirmed to have RVF by serologic testing, and were treated according to a predetermined protocol. The major clinical characteristics of RVF included a high frequency of hepatocellular failure in 124 patients (75.2%), acute renal failure in 68 patients (41.2%), and hemorrhagic manifestations in 32 patients (19.4%). Sixteen patients had retinitis and 7 patients had meningoencephalitis as late complications in the course of the disease. A total of 56 patients (33.9%) died. Hepatorenal failure, shock, and severe anemia were major factors associated with patient death.

Rift Valley Fever (RVF) is a viral disease transmitted to humans by mosquito bite and contact with animals or their infected tissues. Other modes of transmission include aerosol inhalation and possibly ingestion of raw milk from infected animals. We present a 5-day-old neonate with fatal RVF. Onset of the infant's illness on the 2nd day of life combined with positive RVF-IgM and serological evidence of maternal disease supports vertical transmission.

Mutations in the myosin 5β, syntaxin-binding protein 2, and syntaxin 3 genes lead to microvillus inclusion disease (MVID), an autosomal recessive congenital enteropathy. This rare disease is characterized by lack of microvilli on the surface of enterocytes in the small intestine, the presence of pathognomonic intracellular microvillus inclusions, and vesicular bodies within these enterocytes. This pathology leads to the characteristic intractable, life-threatening, watery diarrhea. In the more common early-onset form, affected patients present in the first few days after birth, whereas in the late-onset form, clinical manifestations appear at approximately 2 to 3 months of age. Genetic testing can confirm the diagnosis, but the infant’s medical history, clinical presentation, and small intestinal biopsy results are strongly suggestive of the diagnosis. The prevalence of MVID is thought to be higher in countries with a high degree of consanguinity. Patients with MVID cannot tolerate feedings and require continuous total parenteral nutrition. Mortality is extremely high in the early-onset type with reports of survival in patients treated with small intestinal transplantation. Medical counseling for parents of infants with MVID needs to reflect our current understanding of the various genetic forms of this disease, the feasible management, and anticipated outcomes.

Background and objectivesTo study the epidemiology, clinical presentation and outcome of all patients diagnosed with neonatal tetanus and to provide a recommendation for maternal and neonatal tetanus elimination.Design and settingRetrospective study of all cases of neonatal tetanus admitted from 1991 to 2013.Neonatal intensive care unit, King Fahad Central Hospital, Jazan, Saudi Arabia.ResultsThirty patients were diagnosed with neonatal tetanus over 22 years. Eighteen (60%) of the patients were born to Saudi mothers, and 12 (40%) were born to non-Saudi mothers. Twenty-seven (90%) deliveries occurred at home. Most of the mothers lived in the mountainous zone of the region. Two (10%) of the mothers had only a single dose of the tetanus toxoid; the status of the remaining pregnant women was unknown or unimmunized before or during conception. In 18 of the 30 patients (60%), the umbilical cord was severed using household knife, razor blade or plain scissors. Most of the patients presented with muscle spasms (96.7%), refusal to eat and abnormal posture. All of the patients were intubated and receiving mechanical ventilation. Six (20%) of the patients died.ConclusionIt is essential to begin campaigns or integrate complete maternal tetanus toxoid immunization at primary health centers (PHC) during antenatal care. Immunization needs to be arranged so pregnant women can be educated regarding the importance of ANC and the risks of unhygienic home delivery, and immunization should be addressed with adequate information. Pregnant women and those of childbearing age in mountainous areas should be the first targets for these activities.

Fraser syndrome (cryptophthalmos syndrome) is a rare autosomal recessive disorder. 1 The most consistent features are cryptophthalmos, a broad and depressed nasal bridge, malformed ears, syndactyly of fingers and toes, abnormal genitalia, craniofacial skeletal defects, renal agenesis, and laryngeal stenosis. These anomalies were first documented by Fraser in 1962. 2 We report the case of a full-term newborn who had multiple abnormalities consistent with cryptophthalmos syndrome. In addition, the newborn had a total anomalous pulmonary venous return (TAPVR), which is a new finding in patients with this syndrome. To the best of our knowledge, this is the first report of Fraser syndrome with cardiac lesion (TAPVR) in a Saudi infant. Case ReportA 29-year-old Saudi woman, gravida 7 para 4, had a history of two abortions and three healthy children. She also had a history of a spontaneous vaginal delivery of a male infant with multiple malformations, including complete cryptophthalmos in both eyes, small malformed ears, syndactyly of all fingers, and cleft lip. The infant died 30 minutes after birth in a peripheral hospital, and thus, details were not available.The parents of this infant were second-degree cousins, and his cousin on the paternal side died immediately after birth with similar features. Ultrasound examination of the mother at 28 weeks' gestation demonstrated fetal ascites and unidentified urinary bladder and absent kidneys. At 33 weeks' gestation, ultrasound showed ascites, small left kidney, absent right kidney, thick-walled bladder, oligohydramnios, and increased echogenicity of the right lung. Fetal echocardiogram showed evidence of TAPVR and pericardial effusion. Screening for toxoplasmosis, rubella, syphilis, herpes simplex and cytomegalovirus was negative. Chromosomal study revealed normal male karyotype (46,XY). Pregnancy was induced at 39 weeks and the mother gave birth to a dysmorphic infant who never established spontaneous respiration. Bag and mask ventilation was initiated, but the infant died 20 minutes after delivery. The birth weight was 3.13 kg, length was 47 cm, and head circumference was 32 cm. He was found to have complete cryptophthalmos on the right side and partial cryptophthalmos in the left eye with no eyelashes or eyebrows, and the skin passed unbroken from the brow to cheek (Figure 1). A broad and depressed nasal bridge, soft tissue syndactyly of all fingers and toes and hypoplastic scrotum with bilateral cryptorchidism, were noted in the infant (Figure 2). Furthermore, severe laryngeal stenosis resulted in failure of several attempts of intubation with 2.5 mm endotracheal tube. The chest x-ray showed unareated lung with an opaque lesion in the right middle and lower lobes. Postmortem examination was not permitted by the family.