Dapsone-induced Methemoglobinemia in a Child with End-stage Renal Disease: A Brief Review.

We report a child who was on immunosuppressive therapy for renal transplant rejection, with end-stage renal disease, and who developed methemoglobinemia while receiving dapsone for Pneumocystis jiroveci infection prophylaxis.

Budd-Chiari Syndrome Diagnosed in a Patient Listed for Liver Transplant and Considered to be Contraindicated for the Operation.

We report the clinical case of 23-year-old patient with liver cirrhosis of unknown genesis, significant resistant ascites, and 2 episodes of bleeding from esophageal varices. Evaluation did not find any cause of liver disease, and the patient was placed on the transplant wait list due to subcompensated liver function (Model for End-Stage Liver Disease score of 16, Child-Pugh class B) and poorly controlled severe portal hypertension. After treatment with diuretics, largevolume paracentesis, antibiotics, and vasoconstrictors, hepatorenal syndrome and spontaneous bacterial peritonitis resolved and liver function improved significantly. Because the patient showed consistently good liver function and resistant portal hypertension, liver transplant was delayed with decision to perform transjugular intrahepatic portosystemic shunting instead. During the attempt of shunting, occlusive thrombosis of the iliac veins, inferior vena cavae, and hepatic veins were diagnosed and the procedure was stopped. Therefore, considering preserved liver function and severe portal hypertension, diagnosis of Budd-Chiari syndrome with subsequent development of liver cirrhosis was made. The patient was recommended to undergo evaluation to exclude thrombophilia as a cause of thrombosis.

Simultaneous Acquired Self-limited Hemophagocytic Lymphohistiocytosis and Kikuchi Necrotizing Lymphadenitis in a 16-Year-Old Teenage Girl: A Case Report and Review of the Literature.

OBJECTIVES: The aim of this study was to increase education and awareness among pediatric practitioners of possibility of simultaneous hemophagocytic lymphohistiocytosis and Kikuchi-Fujimoto disease/Kikuchi disease occurring in the pediatric population and the diagnostic dilemma it can present. We describe a case presentation of acquired and self-limited simultaneous hemophagocytic lymphohistiocytosis and Kikuchi-Fujimoto disease in a 16-year-old in the United States who presented with fevers, night sweats, and joint pain, along with tiredness and decreased appetite along with pancytopenia and elevated lactate dehydrogenase. To the best of our knowledge, simultaneous hemophagocytic lymphohistiocytosis and Kikuchi-Fujimoto in the pediatric population has not been described in North America but remains fairly common in Asia. The literature on both diseases and their simultaneous occurrence is comprehensively reviewed. METHODS: This was a case report and review of the literature. RESULTS: The patient was diagnosed with both hemophagocytic lymphohistiocytosis and Kikuchi-Fujimoto disease based on bone marrow aspiration/biopsy and axillary node biopsy, respectively. Both illnesses resolved completely. CONCLUSIONS: Benign causes of pancytopenia and elevated lactate dehydrogenase exist, but they may not be always straightforward diagnostically. Bone marrow aspiration and lymph node biopsy may be helpful in ascertaining the diagnosis. Hemophagocytic lymphohistiocytosis and Kikuchi-Fujimoto disease may represent a continuum of illness.

Sphingopyxis panaciterrae sp. nov., isolated from soil from ginseng field.

A Gram-negative, strictly aerobic, motile bacterial strain, designated Gsoil 124T, was isolated from a soil sample taken from a ginseng field in Pocheon Province (South Korea). The isolate contained Q-10 as the predominant lipoquinone, plus C18:1 7c and summed feature 4 (C16:1 6c and/or iso- C15:0 2-OH) as the major fatty acids. The G+C content of the genomic DNA was 68.1 mol%, and the major polar lipids consisted of sphingoglycolipid, phosphatidylglycerol, phosphatidylcholine, and phosphatidylethanolamine. A comparative 16S rRNA gene sequence analysis showed that strain Gsoil 124T was most closely related to Sphingopyxis chilensis (98.7%), Sphingopyxis alaskensis (98.2%), Sphingopyxis witflariensis (98.2%), Sphingopyxis taejonensis (98.0%), and Sphingopyxis macrogoltabida (97.6%). However, the DNA-DNA relatedness between strain Gsoil 124T and its phylogenetically closest neighbors was less than 22%. Thus, on the basis of its phenotypic properties and phylogenetic distinctiveness, strain Gsoil 124T should be classified as representing a novel species in the genus Sphingopyxis, for which the name Sphingopyxis panaciterrae sp. nov. is proposed. The type strain is Gsoil 124T (=KCTC 12580T=LMG 24003T).

Is high-frequency ventilation more beneficial than low-tidal volume conventional ventilation?

The ventilator goals of the ICU clinician faced with caring for a critically ill child who has ALI/ARDS remain relatively simple: provide adequate ventilation and oxygenation without overdistending alveoli or furthering lung injury. How one obtains these goals is much less simple. The current use of CV calls for the use of relatively low V(T)s and limiting peak inspiratory pressure and plateau pressure while accepting a certain degree of respiratory acidosis. The ICU team can also often achieve these same goals with HFOV. How, then, does one use evidenced-based medicine to pick the best mode of mechanical ventilation for a particular patient? The answer is controversial, to say the least. Does one start with a gentle, open-lung mode of CV then switch to HFOV if the child deteriorates? Or does one use HFOV from the very early stages of ALI? Animal data appear to point to advantages of HFOV when used early in the course of ALI. Most of these studies report a beneficial effect of HFOV when applied on expanded lungs in the early stages of the disease process. These beneficial effects encompass improved gas exchange, oxygenation, lung tissue morphology and pulmonary mechanics. The studies by Arnold and colleagues in the pediatric population also help to answer our questions. In their work, the early initiation of HFOV was associated with improved gas exchange and a trend toward a lower mortality. In adults, Derdak and colleagues demonstrated the superiority of HFOV in terms of gas exchange and oxygenation; however, no statistical significant difference was found for mortality. So, where is the clinician left after a review of these data? It would appear that (1) low-V(T) CV remains a cornerstone of therapy for the pediatric patient who has ALI/ARDS; (2) HFOV is a safe and well-tolerated mode of mechanical ventilation; (3) early use of HFOV (as opposed to the rescue use of this mode) may be of benefit based on animal and human data; and (4) like so many areas of pediatric critical care, clinicians must await new data and trials that will help them continue to improve the care they provide.

Haplotype analysis of the growth hormone releasing hormone receptor locus in three apparently unrelated kindreds from the indian subcontinent with the identical mutation in the GHRH receptor.

The growth hormone releasing hormone receptor (GHRHR) plays a critical role in growth. We identified three nominally unrelated kindreds harboring the identical mutation (E72X) in GHRHR, the gene that encodes GHRHR; all three families originated in the Indian subcontinent. Because of the relative geographic proximity of these populations, we employed haplotype analysis in the region of GHRHR to determine the likelihood that this mutation occurred in a common ancestor rather than having occurred on separate occasions in different individuals. Members of all three kindreds segregating the E72X mutation were genotyped for highly polymorphic dinucleotide repeat microsatellites in a 15.5 centimorgan (cM) region around GHRHR on chromosome 7p15. We conclude that the affected individuals share a common ancestor, and we use the association with linked markers to estimate the age of this unique mutation.