Living donor liver transplantation for giant cavernous hemangioma of liver in a child

Sood D, Mohan N, Singh A, Buxi TBS, Nundy S, Soin AS. Living donor liver transplantation for giant cavernous hemangioma of liver in a child.
Pediatr Transplantation 2011: 15: E135–E139. © 2010 John Wiley & Sons A/S. Abstract: A 10‐yr‐old girl presented with a seven‐month history of upper abdominal discomfort and weight loss. Physical examination revealed an abdominal lump in the right hypochondrium and epigastrium. Ultrasound examination and a computerized tomographic scan showed a large lobulated mass arising from segments I, 1V, and VIII of liver with arteriovenous shunting and multiple small masses in segments VI and VII. An initial diagnosis of hemangioendothelioma with metastasis was made elsewhere following which she received chemotherapy. She had persistent abdominal discomfort because of which she became dependent on narcotics. The patient had fever because of tumor necrosis and also developed peripheral neuropathy. Finally, owing to progressively worsening of symptoms, she underwent left lobe living donor liver transplantation. Histopathological examination showed the mass to be a cavernous hemangioma, and the patient is now well.     

Trends in detection of new leprosy cases at two centres in Himachal Pradesh, India: a ten-year study

An impressive decline in leprosy prevalence rate (PR) in all endemic districts of India is seen in the post-MDT era. However, the new case-detection rate, an important statistical indicator in leprosy control programmes, has not shown significant decline in spite of all efforts. In Himachal Pradesh, a decline in PR from 7.8 to 0.56/10000 between 1991 to 2000 is seen, and recently the State has won national acclaim for having achieved the goal of elimination of leprosy in all the districts. The vertical leprosy programme has been integrated into general health services of the state. An analysis of data from 1991 to 2000 of two leprosy control units of Himachal Pradesh, the Urban Leprosy Clinic in Shimla (ULC-S) and the District Leprosy Control Unit in Mandi (DLCU-M), showed no significant decline in the new cases detected. 277 and 271 new cases were detected at these centres respectively; these included 2.2% and 1.5% children of less than 14 years of age. Almost 75% of these cases were males and of MB type. A steadily increasing trend in the annual detection of new cases was seen at both the centres during the decade. The cases registered at DLCU-M were mainly indigenous to the district. At ULC-S, 45 migrant cases from other endemic areas-mainly from Nepal, Bihar, and Uttar Pradesh-had also contributed to the increased number of new cases. Other possible causes discussed for this higher new case detection, e.g. overdiagnosis, detection of backlog "hidden cases" and voluntary reporting of patients, do not differ from those seen in other parts of the country or the world.     

Chilblain lupus erythematosus mimicking acrofacial vitiligo

Chilblain lupus erythematosus with depigmentation is a rare presentation of lupus erythematosus that may simulate vitiligo. A 52-year-old lady with such a manifestation is being reported.     

Does Residency Selection Criteria Predict Performance in Orthopaedic Surgery Residency?

Background

More than 1000 candidates applied for orthopaedic residency positions in 2014, and the competition is intense; approximately one-third of the candidates failed to secure a position in the match. However, the criteria used in the selection process often are subjective and studies have differed in terms of which criteria predict either objective measures or subjective ratings of resident performance by faculty.

Questions/purposes

Do preresidency selection factors serve as predictors of success in residency? Specifically, we asked which preresidency selection factors are associated or correlated with (1) objective measures of resident knowledge and performance; and (2) subjective ratings by faculty.

Methods

Charts of 60 orthopaedic residents from our institution were reviewed. Preresidency selection criteria examined included United States Medical Licensing Examination (USMLE) Step 1 and Step 2 scores, Medical College Admission Test (MCAT) scores, number of clinical clerkship honors, number of letters of recommendation, number of away rotations, Alpha Omega Alpha (AOA) honor medical society membership, fourth-year subinternship at our institution, and number of publications. Resident performance was assessed using objective measures including American Board of Orthopaedic Surgery (ABOS) Part I scores and Orthopaedics In-Training Exam (OITE) scores and subjective ratings by faculty including global evaluation scores and faculty rankings of residents. We tested associations between preresidency criteria and the subsequent objective and subjective metrics using linear correlation analysis and Mann-Whitney tests when appropriate.

Results

Objective measures of resident performance namely, ABOS Part I scores, had a moderate linear correlation with the USMLE Step 2 scores (r = 0.55, p < 0.001) and number of clinical honors received in medical school (r = 0.45, p < 0.001). OITE scores had a weak linear correlation with the number of clinical honors (r = 0.35, p = 0.009) and USMLE Step 2 scores (r = 0.29, p = 0.02). With regards to subjective outcomes, AOA membership was associated with higher scores on the global evaluation (p = 0.005). AOA membership also correlated with higher global evaluation scores (r = 0.60, p = 0.005) with the strongest correlation existing between AOA membership and the “interpersonal and communication skills” subsection of the global evaluations.

Conclusions

We found that USMLE Step 2, number of honors in medical school clerkships, and AOA membership demonstrated the strongest correlations with resident performance. Our goal in analyzing these data was to provide residency programs at large a sense of which criteria may be “high yield” in ranking applicants by analyzing data from within our own pool of residents. Similar studies across a broader scope of programs are warranted to confirm applicability of our findings. The continually emerging complexities of the field of orthopaedic surgery lend increasing importance to future work on the appropriate selection and training of orthopaedic residents.     

A rare penetrant TIMP3 mutation confers relatively late onset choroidal neovascularisation which can mimic age-related macular degeneration

Purpose

To perform a genotype–phenotype correlation for three patients heterozygous for a missense mutation in the tissue inhibitor of metalloproteinase 3 (TIMP3) gene.

Methods

Retrospective, observational case series. The medical records and photographs were reviewed for three patients diagnosed at the time with neovascular age-related macular degeneration (AMD). All were later found to carry a predicted C113G mutation in the TIMP3 gene, other known mutations in which are associated with Sorsby''s fundus dystrophy.

Results

All three patients developed drusen and bilateral choroidal neovascularisation with subsequent disciform scarring and atrophy. Visual acuity rapidly deteriorated to <6/60 in both eyes. The age of onset varied from 56 to 64 years and the interval to contralateral eye involvement varied from 4 to 6 years. Two of the three patients had a family history of AMD. All three patients were heterozygous for the C113G nucleotide change, resulting in a Ser38Cys change at the N terminus of the TIMP3 protein.

Conclusion

This case series suggests the C113G TIMP3 variant may represent a novel highly penetrant mutation causing choroidal neovascularisation of relatively late onset for Sorsby''s fundus dystrophy, mimicking early onset AMD.