Description of Demographics, Gastrointestinal Features, Surgical Findings, Treatment, and Digestive Course in 10 Patients With MKD With Severe IBD-Like Features
| Patient . | Sex . | Age at MKD Diagnosis/IBD Onset . | Clinical Features . | Surgical Findings . | Surgery . | Medical Treatment (mo from IBD to onset of efficacious treatment) . | Digestive Status (from IBD to last follow-up) . |
|---|---|---|---|---|---|---|---|
| P1 | F | 4 y/5 y | Abdominal pain, vomiting, acute abdomen | Small-bowel occlusion, multiple abdominal adhesions, ileal necrosis (60 cm) | Debridement of adhesions, ileal resection, appendectomy | Corticosteroids* (0.1) | CR (15 y, lost to follow-up) |
| P2 | M | 3 mo/1 mo† | Abdominal pain, diarrhea, vomiting, acute abdomen, septic shock (enterocolitis) | Jejunum perforation and dilatation, abdominal adhesions | Debridement of adhesions, intestinal resection, jejunostomy | Corticosteroids* (0.1), anakinra* (0.2), canakinumab*,‡ (35) | CR on canakinumab (8 y) |
| P3 | F | 31 y/2 mo | Abdominal pain, vomiting, acute abdomen | Sterile peritonitis, abdominal adhesions | Debridement of adhesions | Corticosteroids* (0.1) | CR off treatment (14 y, lost to follow-up) |
| P4 | M | 6 y/6 y | Abdominal pain, diarrhea, vomiting, acute abdomen | Small-bowel occlusion and perforation, abdominal adhesions | Debridement of adhesions, right hemicolectomy, ileostomy | Corticosteroids (0.1), anakinra*,‡ (12) | CR on anakinra (10 y) |
| P5 | F | 14 mo/1 mo† | Abdominal pain, diarrhea† | None performed | — | Corticosteroids, * infliximab, anakinra, * canakinumab*,‡ | CR on canakinumab (8 y) |
| P6 | M | 5 mo/5 mo† | Abdominal pain, vomiting, acute abdomen, anal fistulae | Bowel occlusion and abdominal adhesions | Debridement of adhesions | Steroidsa (0.1) | CR after surgery and corticosteroids (20 y, lost to follow-up) |
| P7 | M | 4 mo/1 mo | Abdominal pain, diarrhea, vomiting, anal fistulae | Rectal fistulae, rectal abscess | Surgical rectal abscess and fistula drainage | Anakinra, * canakinumab*,‡ | CR on canakinumab (5 y) |
| P8 | M | 28 y/10 mo† | Abdominal pain, diarrhea, vomiting | None performed | — | Corticosteroids, * etanercept, adalimumab, anakinra* (0.1), canakinumab*,‡ | CR on canakinumab (5 y) |
| P9 | M | 1 mo/NN† | Diarrhea, vomiting | None performed | — | Anakinra* (310), canakinumab*, ‡ | CR on canakinumab (4 y) |
| P10 | M | 13 y/3 y | Abdominal pain, diarrhea, vomiting, acute abdomen | Appendicitis, abdominal adhesions | Debridement of abdominal adhesions | Anakinra, * canakinumab,‡ colchicine | CR on canakinumab (13 y) |
| Patient . | Sex . | Age at MKD Diagnosis/IBD Onset . | Clinical Features . | Surgical Findings . | Surgery . | Medical Treatment (mo from IBD to onset of efficacious treatment) . | Digestive Status (from IBD to last follow-up) . |
|---|---|---|---|---|---|---|---|
| P1 | F | 4 y/5 y | Abdominal pain, vomiting, acute abdomen | Small-bowel occlusion, multiple abdominal adhesions, ileal necrosis (60 cm) | Debridement of adhesions, ileal resection, appendectomy | Corticosteroids* (0.1) | CR (15 y, lost to follow-up) |
| P2 | M | 3 mo/1 mo† | Abdominal pain, diarrhea, vomiting, acute abdomen, septic shock (enterocolitis) | Jejunum perforation and dilatation, abdominal adhesions | Debridement of adhesions, intestinal resection, jejunostomy | Corticosteroids* (0.1), anakinra* (0.2), canakinumab*,‡ (35) | CR on canakinumab (8 y) |
| P3 | F | 31 y/2 mo | Abdominal pain, vomiting, acute abdomen | Sterile peritonitis, abdominal adhesions | Debridement of adhesions | Corticosteroids* (0.1) | CR off treatment (14 y, lost to follow-up) |
| P4 | M | 6 y/6 y | Abdominal pain, diarrhea, vomiting, acute abdomen | Small-bowel occlusion and perforation, abdominal adhesions | Debridement of adhesions, right hemicolectomy, ileostomy | Corticosteroids (0.1), anakinra*,‡ (12) | CR on anakinra (10 y) |
| P5 | F | 14 mo/1 mo† | Abdominal pain, diarrhea† | None performed | — | Corticosteroids, * infliximab, anakinra, * canakinumab*,‡ | CR on canakinumab (8 y) |
| P6 | M | 5 mo/5 mo† | Abdominal pain, vomiting, acute abdomen, anal fistulae | Bowel occlusion and abdominal adhesions | Debridement of adhesions | Steroidsa (0.1) | CR after surgery and corticosteroids (20 y, lost to follow-up) |
| P7 | M | 4 mo/1 mo | Abdominal pain, diarrhea, vomiting, anal fistulae | Rectal fistulae, rectal abscess | Surgical rectal abscess and fistula drainage | Anakinra, * canakinumab*,‡ | CR on canakinumab (5 y) |
| P8 | M | 28 y/10 mo† | Abdominal pain, diarrhea, vomiting | None performed | — | Corticosteroids, * etanercept, adalimumab, anakinra* (0.1), canakinumab*,‡ | CR on canakinumab (5 y) |
| P9 | M | 1 mo/NN† | Diarrhea, vomiting | None performed | — | Anakinra* (310), canakinumab*, ‡ | CR on canakinumab (4 y) |
| P10 | M | 13 y/3 y | Abdominal pain, diarrhea, vomiting, acute abdomen | Appendicitis, abdominal adhesions | Debridement of abdominal adhesions | Anakinra, * canakinumab,‡ colchicine | CR on canakinumab (13 y) |
*Treatment that resulted in complete clinical digestive remission of MKD, alone or in association with abdominal surgery.
†MKD-revealing manifestation.
‡Ongoing treatment at last news.
CR indicates complete remission; NN, neonatal.
Description of Demographics, Gastrointestinal Features, Surgical Findings, Treatment, and Digestive Course in 10 Patients With MKD With Severe IBD-Like Features
| Patient . | Sex . | Age at MKD Diagnosis/IBD Onset . | Clinical Features . | Surgical Findings . | Surgery . | Medical Treatment (mo from IBD to onset of efficacious treatment) . | Digestive Status (from IBD to last follow-up) . |
|---|---|---|---|---|---|---|---|
| P1 | F | 4 y/5 y | Abdominal pain, vomiting, acute abdomen | Small-bowel occlusion, multiple abdominal adhesions, ileal necrosis (60 cm) | Debridement of adhesions, ileal resection, appendectomy | Corticosteroids* (0.1) | CR (15 y, lost to follow-up) |
| P2 | M | 3 mo/1 mo† | Abdominal pain, diarrhea, vomiting, acute abdomen, septic shock (enterocolitis) | Jejunum perforation and dilatation, abdominal adhesions | Debridement of adhesions, intestinal resection, jejunostomy | Corticosteroids* (0.1), anakinra* (0.2), canakinumab*,‡ (35) | CR on canakinumab (8 y) |
| P3 | F | 31 y/2 mo | Abdominal pain, vomiting, acute abdomen | Sterile peritonitis, abdominal adhesions | Debridement of adhesions | Corticosteroids* (0.1) | CR off treatment (14 y, lost to follow-up) |
| P4 | M | 6 y/6 y | Abdominal pain, diarrhea, vomiting, acute abdomen | Small-bowel occlusion and perforation, abdominal adhesions | Debridement of adhesions, right hemicolectomy, ileostomy | Corticosteroids (0.1), anakinra*,‡ (12) | CR on anakinra (10 y) |
| P5 | F | 14 mo/1 mo† | Abdominal pain, diarrhea† | None performed | — | Corticosteroids, * infliximab, anakinra, * canakinumab*,‡ | CR on canakinumab (8 y) |
| P6 | M | 5 mo/5 mo† | Abdominal pain, vomiting, acute abdomen, anal fistulae | Bowel occlusion and abdominal adhesions | Debridement of adhesions | Steroidsa (0.1) | CR after surgery and corticosteroids (20 y, lost to follow-up) |
| P7 | M | 4 mo/1 mo | Abdominal pain, diarrhea, vomiting, anal fistulae | Rectal fistulae, rectal abscess | Surgical rectal abscess and fistula drainage | Anakinra, * canakinumab*,‡ | CR on canakinumab (5 y) |
| P8 | M | 28 y/10 mo† | Abdominal pain, diarrhea, vomiting | None performed | — | Corticosteroids, * etanercept, adalimumab, anakinra* (0.1), canakinumab*,‡ | CR on canakinumab (5 y) |
| P9 | M | 1 mo/NN† | Diarrhea, vomiting | None performed | — | Anakinra* (310), canakinumab*, ‡ | CR on canakinumab (4 y) |
| P10 | M | 13 y/3 y | Abdominal pain, diarrhea, vomiting, acute abdomen | Appendicitis, abdominal adhesions | Debridement of abdominal adhesions | Anakinra, * canakinumab,‡ colchicine | CR on canakinumab (13 y) |
| Patient . | Sex . | Age at MKD Diagnosis/IBD Onset . | Clinical Features . | Surgical Findings . | Surgery . | Medical Treatment (mo from IBD to onset of efficacious treatment) . | Digestive Status (from IBD to last follow-up) . |
|---|---|---|---|---|---|---|---|
| P1 | F | 4 y/5 y | Abdominal pain, vomiting, acute abdomen | Small-bowel occlusion, multiple abdominal adhesions, ileal necrosis (60 cm) | Debridement of adhesions, ileal resection, appendectomy | Corticosteroids* (0.1) | CR (15 y, lost to follow-up) |
| P2 | M | 3 mo/1 mo† | Abdominal pain, diarrhea, vomiting, acute abdomen, septic shock (enterocolitis) | Jejunum perforation and dilatation, abdominal adhesions | Debridement of adhesions, intestinal resection, jejunostomy | Corticosteroids* (0.1), anakinra* (0.2), canakinumab*,‡ (35) | CR on canakinumab (8 y) |
| P3 | F | 31 y/2 mo | Abdominal pain, vomiting, acute abdomen | Sterile peritonitis, abdominal adhesions | Debridement of adhesions | Corticosteroids* (0.1) | CR off treatment (14 y, lost to follow-up) |
| P4 | M | 6 y/6 y | Abdominal pain, diarrhea, vomiting, acute abdomen | Small-bowel occlusion and perforation, abdominal adhesions | Debridement of adhesions, right hemicolectomy, ileostomy | Corticosteroids (0.1), anakinra*,‡ (12) | CR on anakinra (10 y) |
| P5 | F | 14 mo/1 mo† | Abdominal pain, diarrhea† | None performed | — | Corticosteroids, * infliximab, anakinra, * canakinumab*,‡ | CR on canakinumab (8 y) |
| P6 | M | 5 mo/5 mo† | Abdominal pain, vomiting, acute abdomen, anal fistulae | Bowel occlusion and abdominal adhesions | Debridement of adhesions | Steroidsa (0.1) | CR after surgery and corticosteroids (20 y, lost to follow-up) |
| P7 | M | 4 mo/1 mo | Abdominal pain, diarrhea, vomiting, anal fistulae | Rectal fistulae, rectal abscess | Surgical rectal abscess and fistula drainage | Anakinra, * canakinumab*,‡ | CR on canakinumab (5 y) |
| P8 | M | 28 y/10 mo† | Abdominal pain, diarrhea, vomiting | None performed | — | Corticosteroids, * etanercept, adalimumab, anakinra* (0.1), canakinumab*,‡ | CR on canakinumab (5 y) |
| P9 | M | 1 mo/NN† | Diarrhea, vomiting | None performed | — | Anakinra* (310), canakinumab*, ‡ | CR on canakinumab (4 y) |
| P10 | M | 13 y/3 y | Abdominal pain, diarrhea, vomiting, acute abdomen | Appendicitis, abdominal adhesions | Debridement of abdominal adhesions | Anakinra, * canakinumab,‡ colchicine | CR on canakinumab (13 y) |
*Treatment that resulted in complete clinical digestive remission of MKD, alone or in association with abdominal surgery.
†MKD-revealing manifestation.
‡Ongoing treatment at last news.
CR indicates complete remission; NN, neonatal.
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