Journal scan: A review of 13 recent papers of immediate clinical significance, harvested from major international journals

From the desk of the Editor-in-chief

1). McCune–Albright Syndrome

Vijaya Sarathi, Published November 6, 2024,N Engl J Med 2024;391:1734,DOI: 10.1056/NEJMicm2406568,VOL. 391 NO. 18,Copyright © 2024

A 3-year-old boy presented with an 18-month history of bowing of the left leg that impaired his ability to walk. In addition, testicular enlargement and café au lait spots were found on examination.

2). Intravascular large B-cell lymphoma presenting with non-occlusive mesenteric  ischaemia

Kimihiko Nakamura et al, https://www.thelancet.com/journals/lancet/article/PIIS0140-6736(24)02316-X/fulltext

An 81-year-old woman with a 2-day history of vomiting and abdominal distension attended our hospital. The patient reported no fever, recent travel abroad, or change of bowel habit. She had a 3-year history of dementia—diagnosed as Alzheimer’s because she had presented with gradually declining short-term memory—and hysteromyoma resection at age 40 years; she was prescribed rivastigmine.

On examination she was mildly distressed and appeared unwell; her blood pressure was 104/70 mm Hg, pulse was 138 beats per min, and oxygen saturation 88%. Her temperature was 36·6oC. Abdominal examination found diffuse tenderness observed without rebound tenderness; she had no splenomegaly.

Laboratory examinations showed a white blood cell count of 5·2 × 109 per L (normal range 4·5–11·0) with 4047 neutrophils per μL (normal range 1450–7500), haemoglobin concentration 7·2 g/dL (normal range 12–16), lactate dehydrogenase concentration 775 U/L (normal range 140–280), and fibrinogen concentration 527 mg/dL (normal range 200–400).

An abdominal CT scan showed segmental reduced enhancement with mural thickening in the small intestine—indicative of possible intestinal ischaemia (figure). The absence of intestinal obstruction or occlusion in the superior mesenteric vessels led us to make a working diagnosis of nonocclusive mesenteric ischaemia.

An exploratory laparotomy found intestinal necrosis and so we carried out a segmental small bowel resection—removing a 110 cm section of the small intestine (figure).

Histopathological examination of a sample of the resected bowel showed micro vessels filled with large lymphoid cells (figure); fibrin thrombi with neoplastic lymphocytes were found in the larger vein. No changes were observed in the lymph nodes located in the mesentery near the intestine.

Immunohistochemistry found a proliferation of CD20-positive, CD5-negative, and CD3-negative tumour cells (appendix). Additionally, the soluble interleukin-2 receptor concentration level was raised at 2145 U/mL (typical range 158–623).

Considering the findings together, we concluded that the patient had intravascular large B-cell lymphoma which had caused mesenteric ischaemia.

We had arranged for a bone marrow biopsy, but the patient lost consciousness because of a cerebral infarction and encephalitis; she died 29 days after the operation on her bowel.

Intravascular large B-cell lymphoma is a rare and aggressive disease characterised by selective growth of lymphoma cells within the lumen of blood vessels of all sizes; the diagnosis is difficult. Abdominal symptoms and CT findings indicating nonocclusive mesenteric ischaemia are rare. In Asian countries, patients commonly present with haemophagocytic syndrome—in Japan half of all patients have this presentation—which has a particularly rapid onset and progression with fever, bone marrow involvement, hepatosplenomegaly, and thrombocytopenia.

Regarding mesenteric ischaemia, the cause may be mesenteric artery embolism, usually secondary to atrial fibrillation; mesenteric artery thrombosis, caused by atherosclerosis; mesenteric vein thrombosis, commonly caused by hypercoagulability states; or non-occlusive mesenteric ischaemia—as in this case—occurring in situations with low blood flow.

3). Pancreaticopleural fistula presenting with a massive right-sided pleural effusion after pancreatitis

Christopher James Shephard, https://www.thelancet.com/journals/langas/article/PIIS2468-1253(24)00315-7/abstract

A 62-year-old male with psoriatic arthritis, chronic pancreatitis, and alcohol-related liver disease presented with 3 days of increasing breathlessness, chest discomfort, and non-productive cough 2 weeks after admission for acute-on-chronic alcohol-induced pancreatitis (managed conservatively). Examination was pertinent for tachypnoea 28 breaths per min, 1 L/min oxygen requirement, and absent breath sounds on auscultation over the right hemithorax. His abdomen was soft and non-tender. Laboratory studies revealed leukocytosis (15·4 × 109/L), elevated C-reactive protein (65 mg/L), hypoalbuminaemia (27 g/L), deranged liver enzymes (alkaline phosphatase 166 units [U]/L, gamma-glutamyl transferase 267 U/L, aspartate aminotransferase 62 U/L, and alanine aminotransferase 30 U/L) and hyperlipasaemia (1720 U/L). Chest radiography showed a massive right-sided pleural effusion (figure A). Intercostal catheter insertion provided symptomatic relief following initial drainage of 1550 mL haemoserous effusate. Pleural fluid analysis confirmed an exudative process, elevated concentrations of amylase (19 224 U/L) and lipase (88 220 U/L), and a normal triglyceride level (0·4 mmol/L). Culture and cytology were negative.

  • Chest radiograph obtained at presentation showing a massive right-sided pleural effusion with associated lung collapse and leftward mediastinal shift; this is new compared with imaging performed 2 months earlier for right clavicular fracture. (B) Contrast-enhanced CT (axial view) and (C) T2-weighted MRI (coronal view) showing a retroperitoneal peripancreatic fluid collection as depicted by green arrows. (D) T2-weighted MRI (axial view) identifying a linear fluid collection deep to the right diaphragmatic crus as depicted by green arrows, which was seen to communicate with the right pleural cavity and extended inferiorly along the medial margin of the caudate lobe of the liver to communicate with the retroperitoneal peripancreatic fluid collection anterior to the aorta and inferior vena cava.

4). The brain summons deep sleep for healing from life-threatening injury

Mariana Lenharo, https://www.nature.com/articles/d41586-024-03491-2

A heart attack unleashes immune cells that stimulate sleep neurons, leading to restorative slumber.

Immune cells rush to the brain and promote deep sleep after a heart attack, according to a new study1 involving both mice and humans. This heavy slumber helps recovery by easing inflammation in the heart, the study found.

The findings, published today in Nature, could help to guide care for people after a heart attack, says co-author Cameron McAlpine at the Icahn School of Medicine at Mount Sinai in New York City, who studies immune function in the cardiovascular and nervous systems. “Getting sufficient sleep and rest after a heart attack is important for long-term healing of the heart,” he notes.

The implications of the study go beyond heart attack, says Rachel Rowe, a specialist in sleep and inflammation at the University of Colorado Boulder. “For any kind of injury, your body’s natural response would be to help you sleep so your body can heal,” she says.

The heart needs its sleep

Scientists have long known that sleep and cardiovascular health are linked. People who sleep poorly are at a higher risk of developing high blood pressure, for example, than are sound sleepers. But how cardiovascular disease affects sleep has been less explored.

To learn more, the authors induced heart attacks in mice and investigated the animals’ brainwaves. The researchers found that these mice spent much more time in slow-wave sleep — a stage of deep sleep that has been associated with healing — than did mice that hadn’t had a heart attack.

Next, the authors sought to understand what was causing that effect. One obvious place to look was the brain, which controls sleep, notes McAlpine. After a heart attack, immune cells trigger a massive burst of inflammation in the heart, he says, and the researchers wondered whether these immune changes also occurred in the brain.

The team found that, after a mouse’s heart attack, immune cells called monocytes flooded its brain. These cells produced large amounts of a protein called tumour necrosis factor (TNF), which is an important regulator of inflammation and also promotes sleep.

To confirm that these cells were linked to the increased sleep, researchers prevented monocytes from accumulating in the rodents’ brains. As a result, “the mice no longer had this increase in slow-wave sleep after their heart attack,” McAlpine says, supporting the theory that the influx of monocytes to the brain contributes to the post-heart-attack sleep boost. Similar experiments confirmed TNF’s role as a messenger to sleep-inducing brain cells.

Slumbering towards recovery

To understand the purpose of the extra sleep, the researchers repeatedly interrupted slow-wave sleep in mice that had had a heart attack. The team found that these mice had more inflammation in both the brain and the heart, and had a much worse prognosis than mice that were allowed to sleep undisturbed after a heart attack.

The authors also studied humans who had experienced acute coronary syndrome, a term for conditions, including heart attack, that are caused by a sudden reduction of blood flow to the heart muscle. Those who reported poor sleep in the weeks following such an episode had a higher risk of developing heart attacks and other serious cardiovascular problems over the next two years than did those who were good sleepers.

Given the findings, “clinicians need to inform patients of the importance of a good night’s sleep” after a heart attack, says Rowe. This should also be considered at the hospital, where tests and procedures would ideally be conducted during the daytime to minimize sleep interruptions.

The findings highlight the bidirectional relationship between sleep and the immune system. “When your grandma says, ‘if you don’t get enough sleep, you’ll get sick’, there’s a lot of truth to that.”

5). Atrophying Pityriasis Versicolor

Jiawen Chen, Published November 13, 2024,N Engl J Med 2024;391: e42,DOI: 10.1056/NEJMicm2405351,VOL. 391 NO. 19,Copyright © 2024

6). Extensive Tinea Corporis and Tinea Cruris from Trichophyton indotineae

Ziyang Xu etal, Published November 9, 2024,N Engl J Med 2024;391:1837,DOI: 10.1056/NEJMicm2409010,VOL. 391 NO. 19,Copyright © 2024

7). Images in Clinical Medicine

Small-Bowel Obstruction and Intestinal Fistula from Accidental Ingestion of Magnets

Authors: Gaurav Prasad, M.Ch. https://orcid.org/0000-0003-1228-1981, and Vishesh Jain, M.Ch. Published November 20, 2024, N Engl J Med 2024;391: e48,DOI: 10.1056/NEJMicm2406137,VOL. 391 NO. 20, Copyright © 2024

Abstract

An 18-month-old girl was evaluated for sudden-onset abdominal distention, diarrhea, and vomiting. An abdominal radiograph showed three circular radiopaque objects in the intestines and dilated loops of bowel.

8). Images in Clinical Medicine

Anaplasmosis

Zeni Kharel et al, Published November 23, 2024,DOI:10.1056/NEJMicm2408847,Copyright © 2024

Abstract

A man in upstate New York presented with fatigue, fever, poor appetite, headache, and yellow discoloration of the eyes. A peripheral-blood smear revealed band and segmented neutrophils with abnormal intracytoplasmic inclusions.

9). Images in Clinical Medicine

Eiffel-by-Night Sign

Kundian Guo,  Published November 16, 2024,N Engl J Med 2024;391:1936,DOI: 10.1056/NEJMicm2408573,VOL. 391 NO. 20,Copyright © 2024

Abstract

A 50-year-old man presented with a 1-year history of progressive headaches. On examination, there was neck stiffness and dysmetria in both arms. An MRI of the head showed Eiffel-by-night sign.

10). Haematuria in children

Alok Godse etal, BMJ 2024; 387 doi: https://doi.org/10.1136/bmj-2022-072501 (Published 25 November 2024,BMJ 2024;387:e072501

What you need to know

Visible haematuria in children can be caused by many individually rare conditions; a small number of investigations can help to identify those which require urgent action

Isolated non-visible haematuria is common and usually transient; the yield from investigations is very low

Ongoing symptomatic visible haematuria should be referred urgently for hospital investigation. Non-visible haematuria with proteinuria should also be referred

A 12 year old boy presented with a history of recurrent frank haematuria. He reported three or four episodes in the past three months, with each episode quickly fading after a couple of days. On detailed questioning, he revealed that, during each episode, he experienced transient mild dysuria, urinary frequency, urgency, and central abdominal discomfort. Abdominal examination revealed no tenderness, his foreskin was retractable, and no meatal inflammation or excoriation was visible. In clinic his urine looked clear yellow with no visible blood. Urine dipstick revealed 3+of blood.

What is haematuria?

Visible haematuria (macroscopic) is visible bloody discoloration of urine. With easy availability of urine dipstick tests, the incidental discovery of persistent (defined as more than 6 months) non-visible haematuria (microscopic haematuria or NVH) may also occur. Visible haematuria is rare, and its incidence is unknown, whereas non-visible haematuria has been found in up to 5% of school children on mass screening in Asian schools, with up to 0.5% persisting three to six months later.

Unlike in adults, underlying malignancy as a cause of haematuria in children is extremely rare (<0.1%). Although the underlying cause cannot be determined by whether the haematuria is visible or non-visible, isolated non-visible haematuria is most commonly idiopathic, whereas visible haematuria may stem from the kidney (such as IgA nephropathy or autoimmune disease) or the urinary tract (such as posterior urethritis, urinary tract stones, balanitis, or urinary tract infection).

11). Images in Clinical Medicine

Phytophotodermatitis

Vishaka Ravishankar Hatcher, ,Published November 27, 2024,N Engl J Med 2024;391:2035,DOI: 10.1056/NEJMicm2410140,VOL. 391 NO. 21,Copyright © 2024

Abstract

A 40-year-old man presented with a 2-day history of a burning rash on both hands. One day before the rash developed, he juiced limes by hand and spent time outdoors without sunscreen on his hands.

12). Images in Clinical Medicine

Mycosis Fungoides of the Palms and Soles

Chen Li, Published November 30, 2024,N Engl J Med 2024;391:2147,DOI: 10.1056/NEJMicm2401673,VOL. 391 NO. 22,Copyright © 2024

Abstract

A 38-year-old man presented with a 15-year history of a waxing and waning rash on his palms and soles. A skin biopsy revealed dense lymphocytic infiltration of the superficial and middle dermis.

13). Images in Clinical Medicine

Spontaneous Coronary-Artery Dissection

Yehia Saleh, Published December 4, 2024,N Engl J Med 2024;391: e54,DOI: 10.1056/NEJMicm2407274,VOL. 391 NO. 22,Copyright © 2024

Abstract

A 32-year-old woman presented with severe chest pain. An ECG showed ST-segment elevations. Coronary angiography showed an abrupt caliber change in the right coronary artery.

Kauvery Hospital