Methicillin-resistant Staphylococcus aureus development in intensive care patients. A case-control study.

OBJECTIVES: To determine the factors associated with the development of methicillin-resistant Staphylococcus aureus (MRSA), hospital stay and mortality, and early versus late MRSA infection. METHODS: Cases (n=44) were intensive care unit (ICU) patients admitted to King Fahd Specialist Hospital, Al-Qassim, Saudi Arabia between 2015 and 2019 who developed MRSA during their hospital stay. Controls (n=48) were patients from the same place and period who did not develop MRSA. Data were abstracted from hospital records. RESULTS: Admission with sepsis (case: 46% vs. control: 2%, p less than 0.001) and having at least one comorbid condition (case: 95% vs. control: 46%, p less than 0.001) were significantly associated with the development of MRSA. Age (mean ±SD: case: 65±18, control: 64±18, p=0.7) and gender (% male, case: 52%, control: 56%, p=0.70) were not associated with the development of MRSA. Approximately 73% of all MRSA cases developed within the first 2 weeks of admission. Among the early cases, 44% died during their ICU stay; the corresponding percentage among the late cases was 42% (p=0.69). There was no difference between early and late MRSA cases in terms of non-sepsis admissions (50% vs. 67%, p=0.32) or comorbid status (at least one: 97% vs. 92%, p=0.17).  Conclusion: Sepsis and comorbid conditions were significant risk factors for MRSA development among hospital patients.

Superior sagittal sinus thrombosis associated with raised intracranial pressure in closed head injury with depressed skull fracture.

A case of delayed signs of intracranial hypertension following closed head injury with a depressed cranial fracture and superior sagittal sinus thrombosis is reported. Conservative treatment of intracranial hypertension, including just repeated lumbar puncture and oral acetazolamide, was performed. Spontaneous recanalization of the superior sagittal sinus was observed. Pathogenesis and different modalities of treatment are discussed.

Elevated B-lymphocyte levels in lesional tissue of non-arthritic psoriasis.

Psoriasis is a chronic inflammatory skin disorder characterized clinically by maculopapular skin lesions and on the cellular level by increased T-lymphocyte activation in the peripheral blood and migration of activated T-lymphocyte into the lesions. The lymphocyte subpopulations in peripheral blood from 21 Kuwaiti patients showed elevated levels of the T-lymphocyte activation marker CD25, as well as increased expression of HLA-DR compared with a group of age and sex-matched controls, confirming published findings on psoriasis. In addition, there was a tendency towards a significant increase in the CD4+/CD45RO+ (memory cell) population that was also consistent with peripheral T-lymphocyte activation. Immunohistological studies showed a heavy infiltrate of all cell types into the lesional tissue including, as expected, activated T-lymphocytes. An unexpected finding was significantly higher levels of B-lymphocytes infiltrating the psoriatic lesions; they numerically exceeded the T-lymphocyte infiltrate. This has previously been reported only in cases of psoriasis with concurrent arthritis. None of the subjects had arthritis, suggesting an immunopathological variant of psoriasis possibly specific to this population group.

beta-Thalassaemia major in Kuwait.

A combined retrospective and prospective study of 129 beta-thalassaemia major patients seen between 1965 and 1995 in Sabah Hospital, Kuwait has been carried out. The age range at diagnosis was 2 to 84 months, median 9 months. In approximately 80 per cent, the patients were outcomes of first- or second-cousin marriages. Nine (7 per cent) of the patients were HBsAg positive, while 42 (33 per cent) were hepatitis C seropositive. Eleven (9 per cent) patients had had bone marrow transplantation (BMT). There was no BMT-related mortality, but there were three graft rejections and two cases of chronic graft-versus-host disease (GVHD).

Peripheral T-cell activation in non-segmental vitiligo.

Vitiligo is a hypopigmentary dermatosis of probable autoimmune origin. Previously reported aberrations in peripheral blood mononuclear cells (PBMC), especially T cells and T cell subsets, have been inconsistent. Lymphocyte subpopulations were examined using flow cytometry and monoclonal antibodies against CD4, CD8, CD20, CD25, CD45RA, and HLA-DR in 34 patients with non-segmental vitiligo. Twelve patients had not received any previous treatment and 22 had previously received at least one course of PUVA therapy that was discontinued at least four months prior to our study. Compared to matched controls, we found significant increases in CD25 and HLA-DR in vitiligo patients (p = 0.000). An inverse correlation was observed between HLA-DR and patient status with regard to treatment (p = 0.001). These results suggest a role for T cells in the pathogenesis of vitiligo and imply that previous PUVA therapy may be reflected by an alteration in circulating DR +ve cells.

An epidemiologic, clinical, and therapeutic study of childhood Guillain-Barré syndrome in Kuwait: is it related to the oral polio vaccine?

We studied Guillain-Barré syndrome, affecting children 12 years old or less, throughout Kuwait, in the period between January 1, 1992, and March 31, 1997. Nineteen children had the diagnostic criteria of Guillain-Barré syndrome, with an overall annual incidence rate of 0.95/100,000 population at risk. Female patients outnumbered male patients with a sex ratio of 1.4:1. There was a clustering of cases in winter and spring and in the year 1996. The disease symptoms were relatively severe in our patients because only 16% (3 of 19) of them were able to walk at the height of their illness, whereas the rest were bed or chair bound or needed assisted ventilation. Two patients had the electrodiagnostic features of axonal neuropathy and both had residual deficits on follow-up, whereas the rest recovered fully. All the patients received intravenous immunoglobulin. The mean time to walk unaided was 23.5 days (range, 2-84 days) after intravenous immunoglobulin and excluding the two patients with axonal neuropathy, and full recovery was achieved in a mean time of 103 days (range, 30-300 days). Contrary to previous studies, we found no correlation between oral polio vaccine administration and Guillain-Barré syndrome in 2 successive years (1995 and 1996) during a nationwide campaign targeting children less than 5 years old.

Kenny-Caffey syndrome is part of the CATCH 22 haploinsufficiency cluster.

We report four sibs with Kenny-Caffey syndrome in a consanguineous Bedouin family. The first two died in the neonatal period while the remaining affected brother and sister had all the characteristic clinical, biochemical, and radiological abnormalities of the syndrome. These included severe pre- and postnatal growth retardation, cortical thickening of the tubular bones with medullary stenosis, eye abnormalities, facial dysmorphism, hypocalcaemia, and low levels of parathyroid hormone. The children also showed intracranial calcification, impaired neutrophil phagocytosis, increased proportion of B lymphocytes, reduced CD4 and CD8 subpopulations of T lymphocytes, and inhibited transformation in response to Candida antigen. Fluorescence in situ hybridisation (FISH) was applied to blood lymphocyte metaphase spreads from these two Bedouin sibs and their parents using probe D22S75 (Oncor), specific for the DiGeorge critical region on chromosome 22q11.2. The presence of 22q11.2 haploinsufficiency was identified in the affected sibs, which was transmitted from the phenotypically normal mother. The present report widens the spectrum of CATCH 22 microdeletion to accommodate Kenny-Caffey syndrome.

Lateral femoral cutaneous nerve block for split skin grafting.

Wound with loss of skin needs grafting for early healing and to prevent deformity and disability. For skin grafting lateral femoral cutaneous nerve block can be used as regional anesthesia. The efficacy of 55 lateral femoral cutaneous nerve block was assessed in 52 patients of 10 to 70 years of age. The nerve block was found effective in all cases. 90.91% showed excellent results. The procedure seemed to be easy, safe and less costly for the purpose of skin grafting.

Non-haematological traits associated with congenital dyserythropoietic anaemia type 1: a new entity emerging.

Dysmorphic features in three sibs with congenital dyserythropoietic anaemia type 1 are described. These findings include growth retardation/short stature, congenital ptosis, abnormal tarsal bones, metatarsal duplication/hypoplasia, nail/phalangeal hypoplasia of fingers and toes, Madelung deformity, syndactyly of toes, and hallux valgus. The patients also showed a very low mitotic index of their peripheral blood lymphocyte cultures. Phenotypic heterogeneity was elicited amongst the three Bedouin sibs. The present report confirms the association between a subset of congenital dyserythropoietic anaemia type 1 and a specific form of distal limb anomalies and suggests that other traits, congenital ptosis and low mitotic index, could represent part of the syndrome profile.

Genotypic/phenotypic heterogeneity of selective vitamin B12 malabsorption (Grasbeck-Imerslund syndrome) in two Bedouin families.

We report on seven patients in two unrelated consanguineous Bedouin families with Grasbeck-Imerslund syndrome. Pedigree analysis in Family 1 was suggestive of an X-linked mode of inheritance. Intra- and inter-familial heterogeneity was elicited among the affected children in both families. Two of the affected sibs in each family had raised Hb A2 (>4%) while a third in Family 1 had a raised level of Hb F before treatment. One of the patients developed subacute combined degeneration of the cord at the age of 17 years before the correct diagnosis was made. All abnormalities were corrected following the institution of parenteral cobalamin therapy.

Another Arab patient with overlap of Váradi-Papp/Opitz trigonocephaly syndromes?

We describe a Bedouin boy with multiple congenital anomalies/mental retardation (MCA/MR). He has frontal bossing, ridged metopic suture, bilateral ptosis, right squint, depressed nasal bridge, small nose, anteverted nostrils, lobulated tongue, polydactyly of both hands, microphallus, hypoplastic scrotum, microtestes, dysgenesis of corpus callosum, and a Dandy-Walker variant. This phenotype overlaps both the Varadi-Papp syndrome (OFD VI) and Opitz trigonocephaly (C syndrome). This phenotypic overlap is discussed in light of the concept of splitting and lumping in genetic diseases.

Febrile urinary tract infection in children: Role of 99MTc-dimercaptosuccinic acid (DMSA) scan and other imaging techniques.

Fifty children (37 females and 13 males) with first febrile urinary tract infections were studied to assess the value of 99MTc-dimercaptosuccinic acid (DMSA) scan in detecting inflammatory changes of acute pyelonephritis (APN). These findings were compared with renal ultrasonography (US). We also evaluated the reliability of clinical and laboratory observations in diagnosing acute pyelonephritis (APN). All children had micturating cystourethrography (MCUG). DMSA-documented acute pyelonephritis was present in 29 (58%) patients. Only four children (8%) demonstrated changes suggestive of APN on renal ultrasonography. Vesicoureteric reflux (VUR) was documented in 17 (47%) of the total group and in 13 (45%) of those with abnormal DMSA scan. Follow-up DMSA scan in 15 children with initial abnormal findings showed complete recovery in seven (47%). Our data have shown that DMSA renal scan is the most useful investigational procedure in children with febrile UTI. The diagnosis of APN, depending on clinical and laboratory data, is unreliable. Renal US alone can miss serious renal defects. MCUG remains the most sensitive procedure to detect VUR and it should be performed in all children with UTI and abbormal DMSA scan. Early detection of acute pyelonephritis allows the prompt introduction of antimicrobial agents in those children and can prevent or decrease renal damage and its complications.

Femoral hypoplasia-unusual facies syndrome with bifid hallux, absent tibia, and macrophallus: a report of a Bedouin baby.

A male Bedouin baby with the clinical profile of femoral hypoplasia-unusual facies syndrome is described. The phenotype includes bilateral asymmetrical lower limb hypoplasia/aplasia with short remnants of both femora, absent right tibia, bifid right big toe, dysmorphic facies, thoracic/pelvic abnormalities, macrophallus, and bilateral cryptorchidism. This report re-emphasises the previously described rare association of femoral hypoplasia-unusual facies syndrome with preaxial polydactyly and suggests that the clinical spectrum of the syndrome could be stretched further to accommodate other unusual traits, for example, macrophallus and absent tibia.

Right upper limb bud triplication and polythelia, left sided hemihypertrophy and congenital hip dislocation, facial dysmorphism, congenital heart disease, and scoliosis: disorganisation-like spectrum or patterning gene defect?

A Somali female baby with right upper limb triplication, polythelia, left sided hemihypertrophy, congenital hip dislocation, facial dysmorphism, congenital heart disease, and scoliosis is described. It seems that the above described pattern of anomalies has not been reported before. The possible developmental genetic mechanism responsible for this phenotype is briefly discussed.

HLA antigens in Arab children with steroid-responsive nephrotic syndrome.

Forty-eight Arab patients with steroid-responsive childhood nephrotic syndrome were studied for the frequency of HLA-A, -B, -C and -DR antigens. HLA-DR7 was significantly increased in the patient group (63% vs. 28%, P = 0.0002) confirming reports of a DR7 association in other ethnic groups and indicating a universal association with this antigen. HLA-CW6 was also significantly increased (44% vs. 21%, P corr. = 0.042). HLA-DQW1 was significantly reduced in the patients (29% vs. 57%, P corr. = 0.012) as was HLA-CW4 (6% vs. 24%, P corr. = 0.042).